86 results on '"John W. Foreman"'
Search Results
2. Leadership Wise: Why Business Books Suck, but Wise Leaders Succeed
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John W. Foreman and John W. Foreman
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- 2023
3. Smart Data statt Big Data: Wie Sie mit Excel-Analysen das Beste aus Ihren Kundendaten herausholen
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John W. Foreman, Jutta Schmidt and John W. Foreman, Jutta Schmidt
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- 2015
4. Tailored use of belatacept in adolescent kidney transplantation
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Annette M. Jackson, Del Wigfall, John W. Foreman, Rasheed Gbadegesin, Allan D. Kirk, Eileen Tsai Chambers, Shashi K. Nagaraj, Annabelle N. Chua, Rebecca E Sadun, and Kathryn H. Blew
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Adult ,Graft Rejection ,Nephrology ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Calcineurin Inhibitors ,Urology ,030230 surgery ,Belatacept ,Abatacept ,03 medical and health sciences ,0302 clinical medicine ,Maintenance therapy ,Internal medicine ,medicine ,Humans ,Immunology and Allergy ,Pharmacology (medical) ,Child ,Kidney transplantation ,Transplantation ,business.industry ,Graft Survival ,Immunosuppression ,medicine.disease ,Kidney Transplantation ,Calcineurin ,Sirolimus ,Alemtuzumab ,business ,Immunosuppressive Agents ,medicine.drug - Abstract
Adolescent transplant recipients are at risk for nonadherence, development of de novo donor-specific antibody (dnDSA), and allograft loss. Belatacept, a selective T cell costimulatory blocker, is associated with reduced dnDSA, improved renal function, and prolonged allograft survival when compared to calcineurin inhibitor-based regimens in adults; however, its use in children is scant. Three adolescents were initiated on belatacept between August 2017 and September 2018 at the time of kidney transplantation. Selection criteria included age ≥ 14 and EBV IgG + serostatus. Intraoperative alemtuzumab and methylprednisolone were given as induction therapy. Tailored maintenance therapy included steroid-free belatacept and sirolimus for two patients. One patient was initially maintained steroid-free on belatacept and belimumab, an inhibitor of B cell activating factor to treat concurrent systemic lupus erythematous; steroids were added subsequently. Renal function, biopsy-proven rejection, dnDSA, allograft survival, infection, nonadherence, and proteinuria were monitored. Renal function was 86, 73, 52 mL/min/1.73 m2 at 20, 20, and 8 months, respectively. There was 100% adherence to therapy and no development of dnDSA. All patients had treatable infections. One developed steroid-responsive acute cellular rejection. Belatacept-based regimens can be tailored for adolescent recipients with good short-term clinical outcomes.
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- 2020
5. Fanconi Syndrome
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John W, Foreman
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Diagnosis, Differential ,Pediatrics, Perinatology and Child Health ,Humans ,Child ,Fanconi Syndrome - Abstract
Fanconi syndrome, also known as the DeToni, Debré, Fanconi syndrome is a global dysfunction of the proximal tubule characterized by glucosuria, phosphaturia, generalized aminoaciduria, and type II renal tubular acidosis. Often there is hypokalemia, sodium wasting, and dehydration. In children, it typically is caused by inborn errors of metabolism, principally cystinosis. In adults, it is mainly caused by medications, exogenous toxins, and heavy metals. Treatment consists of treating the underlying cause and replacing the lost electrolytes and volume.
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- 2019
6. Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings
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David T. Selewski, Tarak Srivastava, Shashi K. Nagaraj, Eileen D. Brewer, Jennifer D. Varner, Rachel M Engen, Christoph Licht, Cynthia Silva, John Barcia, Christoph P. Hornik, Annabelle N. Chua, Delbert R. Wigfall, Rasheed Gbadegesin, Adam Bensimhon, Natasha Jawa, Patricia L. Weng, Caroline Straatmann, Michelle N. Rheault, Jonathan H. Pelletier, Scott E. Wenderfer, Karan R. Kumar, Eileen Tsai Chambers, T. Keefe Davis, Keisha L. Gibson, Mahmoud Kallash, John W. Foreman, and Larry A. Greenbaum
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Adult ,Graft Rejection ,Male ,medicine.medical_specialty ,Nephrotic Syndrome ,Adolescent ,Biopsy ,medicine.medical_treatment ,Drug Resistance ,030232 urology & nephrology ,030204 cardiovascular system & hematology ,Kidney ,Gastroenterology ,Article ,Young Adult ,03 medical and health sciences ,0302 clinical medicine ,Focal segmental glomerulosclerosis ,Recurrence ,Risk Factors ,Internal medicine ,medicine ,Humans ,Minimal change disease ,Child ,Glucocorticoids ,Kidney transplantation ,Retrospective Studies ,medicine.diagnostic_test ,Glomerulosclerosis, Focal Segmental ,business.industry ,Nephrosis, Lipoid ,Infant, Newborn ,Infant ,Immunosuppression ,Prognosis ,medicine.disease ,Kidney Transplantation ,Transplantation ,Nephrology ,Child, Preschool ,Preoperative Period ,Pediatrics, Perinatology and Child Health ,Female ,business ,Nephrotic syndrome ,Kidney disease - Abstract
BACKGROUND AND OBJECTIVES: Steroid resistant nephrotic syndrome (SRNS) due to focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is a leading cause of end stage kidney disease in children. Recurrence of primary disease following transplantation is a major cause of allograft loss. The clinical determinants of disease recurrence are not completely known. Our objectives were to determine risk factors for recurrence of FSGS/MCD following kidney transplantation, and factors that predict response to immunosuppression following recurrence. STUDY DESIGN: Multicenter study of pediatric patients with kidney transplants performed for ESKD due to SRNS between 1/2006–12/2015. Demographics, clinical course, and biopsy data were collected. Patients with primary-SRNS (PSRNS) were defined as those initially resistant to corticosteroid therapy at diagnosis, and patients with late-SRNS (LSRNS) as those initially responsive to steroids who subsequently developed steroid resistance. We performed logistic regression to determine risk factors associated with nephrotic syndrome (NS) recurrence. RESULTS: We analyzed 158 patients; 64 (41%) had recurrence of NS in their renal allograft. Disease recurrence occurred in 78% of patients with LSRNS compared to 39% of those with PSRNS. Patients with MCD on initial native kidney biopsy had a 76% recurrence rate compared with a 40% recurrence rate in those with FSGS. Multivariable analysis showed that MCD histology (OR; 95% CI: 5.6; 1.3–23.7) compared to FSGS predicted disease recurrence. CONCLUSIONS: Pediatric patients with MCD and LSRNS are at higher risk of disease recurrence following kidney transplantation. These findings may be useful for designing studies to test strategies for preventing recurrence.
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- 2018
7. Genetic Diseases of the Kidney
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John W. Foreman
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Kidney ,Pathology ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Urology ,Prenatal diagnosis ,Disease ,Gene mutation ,urologic and male genital diseases ,Bioinformatics ,medicine.disease ,Renal tubular acidosis ,Cystic kidney disease ,medicine.anatomical_structure ,Nephrology ,medicine ,Renal biopsy ,business ,Genetic testing - Abstract
The number of genes associated with renal disease is increasing every day and this has led to a clearer understanding of the pathophysiology of renal disease in many disorders. It is also appreciated now that a genetic mutation(s) underlie many renal syndromes. Genetic testing may also offer the possibility to diagnose some renal diseases without the need for a renal biopsy. It also allows the prenatal diagnosis of certain renal diseases in at risk fetuses or identification of potential renal disease before it has become manifest. Finally, identification of a specific gene mutation holds the possibility of correction though gene therapy in the future. It is increasingly clear that many renal disorders in pediatrics are a consequence of genetic mutations. In the future, genetic testing will become as easy and as common as ordering a serum creatinine today.
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- 2015
8. Rare variants in tenascin genes in a cohort of children with primary vesicoureteric reflux
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Himani Vaidya, Sherry S. Ross, John S. Wiener, Shan Elahi, Adebowale Adeyemo, Shashi K. Nagaraj, Delbert R. Wigfall, Rasheed Gbadegesin, Patrick D. Brophy, Indra R. Gupta, Guanghong Wu, Jonathan C. Routh, John W. Foreman, Gentzon Hall, C. Egla Rabinovich, Alison Homstad, Peter J. Conlon, and Jennifer Stout
- Subjects
Joint Instability ,Male ,0301 basic medicine ,Joint hypermobility ,Pathology ,medicine.medical_specialty ,Mutation, Missense ,Tenascin ,Vesicoureteral reflux ,Gastroenterology ,Article ,03 medical and health sciences ,Internal medicine ,medicine ,Humans ,Missense mutation ,Family history ,Child ,Vesico-Ureteral Reflux ,Reflux nephropathy ,biology ,business.industry ,Infant ,medicine.disease ,Pedigree ,030104 developmental biology ,Nephrology ,Child, Preschool ,Mutation ,Pediatrics, Perinatology and Child Health ,biology.protein ,Etiology ,Female ,business ,Kidney disease - Abstract
Primary vesicoureteral reflux (PVUR) is the most common malformation of the kidney and urinary tract, and reflux nephropathy is a major cause of chronic kidney disease in children. Recently, we reported mutations in the tenascin XB gene (TNXB) as a cause of PVUR with joint hypermobility. To define the role of rare variants in tenascin genes in the etiology of PVUR, we screened a cohort of patients with familial PVUR (FPVUR) and non-familial PVUR (NFPVUR) for rare missense variants inTNXB and the tenascin C gene (TNC) after excluding mutations in ROBO2 and SOX17. The screening procedure identified 134 individuals from 112 families with PVUR; two families with mutations in ROBO2 were excluded from further analysis. Rare missense variants in TNXB were found in the remaining 110 families, of which 5/55 (9 %) families had FPVUR and 2/55 (4 %) had NFPVUR. There were no differences in high-grade reflux or renal parenchymal scarring between patients with and without TNXB variants. All patients with TNXB rare variants who were tested exhibited joint hypermobility. Overall we were able to identify causes of FPVUR in 7/57 (12 %) families (9 % in TNXB and 3 % in ROBO2). In conclusion, the identification of a rare missense variant in TNXB in combination with a positive family history of VUR and joint hypermobility may represent a non-invasive method to diagnose PVUR and warrants further evaluation in other cohorts.
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- 2015
9. Variability in phenotype induced by the podocin variant R229Q plus a single pathogenic mutation
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David N. Howell, Michelle P. Winn, John W. Foreman, Paul J. Phelan, Gentzon Hall, Andrew F. Malone, Delbert R. Wigfall, Shashi K. Nagaraj, and Rasheed Gbadegesin
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NPHS2 ,Disease ,medicine.disease_cause ,Compound heterozygosity ,medicine ,Genetics ,Transplantation ,Mutation ,Proteinuria ,biology ,business.industry ,nephrotic syndrome ,Hereditary and Rare Nephropathies ,familial ,Heterozygote advantage ,medicine.disease ,Phenotype ,3. Good health ,FSGS ,Nephrology ,Podocin ,biology.protein ,Contents ,medicine.symptom ,proteinuria ,business ,Nephrotic syndrome ,hereditary - Abstract
Background Mutations in podocin (NPHS2) are the most common cause of childhood onset autosomal recessive steroid-resistant nephrotic syndrome (SRNS). The disease is characterized by early-onset proteinuria, resistance to immunosuppressive therapy and rapid progression to end-stage renal disease. Compound heterozygous changes involving the podocin variant R229Q combined with another pathogenic mutation have been associated with a mild phenotype with disease onset often in adulthood. Methods We screened 19 families with early-onset SRNS for mutations in NPHS2 and WT1 and identified four disease-causing mutations (three in NPHS2 and one in WT1) prior to planned whole-exome sequencing. Results We describe two families with three individuals presenting in childhood who are compound heterozygous for R229Q and one other pathogenic NPHS2 mutation, either L327F or A297V. One child presented at age 4 years (A297V plus R229Q) and the other two at age 13 (L327F plus R229Q), one with steadily deteriorating renal function. Conclusions These cases highlight the phenotypic variability associated with the NPHS2 R229Q variant plus pathogenic mutation. Individuals may present with early aggressive disease.
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- 2015
10. Correction to: Recurrence of nephrotic syndrome following kidney transplantation is associated with initial native kidney biopsy findings
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Patricia L. Weng, Keisha L. Gibson, Larry A. Greenbaum, Karan R. Kumar, Eileen D. Brewer, Tarak Srivastava, Michelle N. Rheault, Shashi K. Nagaraj, John W. Foreman, Jennifer D. Varner, Natasha Jawa, Eileen Tsai Chambers, Annabelle N. Chua, Delbert R. Wigfall, Cynthia Silva, Caroline Straatmann, David T. Selewski, John Barcia, T. Keefe Davis, Rachel M Engen, Christoph Licht, Jonathan H. Pelletier, Scott E. Wenderfer, Mahmoud Kallash, Rasheed Gbadegesin, Adam Bensimhon, and Christoph P. Hornik
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Nephrology ,medicine.medical_specialty ,business.industry ,General surgery ,030232 urology & nephrology ,MEDLINE ,030204 cardiovascular system & hematology ,medicine.disease ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Pediatric nephrology ,Native kidney ,business ,Nephrotic syndrome ,Kidney transplantation ,Biopsy findings - Abstract
The original version of this article unfortunately contained a mistake. The subtitle “A Midwest Pediatric Nephrology Consortium (MWPNC) study” was missing. The correct title including subtitle is given above.
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- 2018
11. Hypercalcemia, Hypercalciuria, and Elevated Calcitriol Concentrations with Autosomal Dominant Transmission Due toCYP24A1Mutations: Effects of Ketoconazole Therapy
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Ronald L. Horst, Peter J. Tebben, Dawn S. Milliner, John W. Foreman, Ravinder J. Singh, Peter C. Harris, Rajiv Kumar, Yanhong Wu, and Paul R. Chelminski
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Male ,medicine.medical_specialty ,endocrine system diseases ,Calcitriol ,Bone disease ,Endocrinology, Diabetes and Metabolism ,Hypercalciuria ,Clinical Biochemistry ,Biology ,Gene mutation ,medicine.disease_cause ,Biochemistry ,Endocrinology ,CYP24A1 ,Internal medicine ,medicine ,Humans ,Vitamin D3 24-Hydroxylase ,Mutation ,Biochemistry (medical) ,Cytochrome P450 ,JCEM Online: Brief Reports ,medicine.disease ,Ketoconazole ,14-alpha Demethylase Inhibitors ,Steroid Hydroxylases ,Hypercalcemia ,biology.protein ,medicine.drug - Abstract
Background: Mutations of the CYP24A1 gene, which encodes the 1,25-dihydroxyvitamin D-24-hydroxylase cytochrome P450, Cyp24A1, are predicted to result in elevated 1,25-dihydroxyvitamin D concentrations, hypercalcemia, hypercalciuria, nephrolithiasis, and bone disease. Treatment of hypercalcemia associated with CYP24A1 gene mutations has not been described. Methods: The genetic basis of a syndrome in a 44-yr-old Caucasian male characterized by intermittent hypercalcemia, hypercalciuria, elevated serum 1,25-dihydroxyvitamin D, undetectable serum 24,25-dihydroxyvitamin D, metabolically active nephrolithiasis, and reduced bone mineral density of the lumbar spine was examined. Sequencing of the CYP24A1 gene and biochemical and genetic analysis of seven family members in three generations was carried out. Because of hypercalcemia, hypercalciuria, and metabolically active nephrolithiasis, the patient was treated with a cytochrome 3A inhibitor, ketoconazole, 200 mg orally every 8 h, for 2 months. Results: The sequ...
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- 2012
12. Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities
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Ira Davis, Beatrice Goilav, Detlef Bockenhauer, Jeffrey J. Fadrowski, Ben A. Semmekrot, Trevor Cole, Lynn M. Boyden, Shrikant Mane, Margo Whiteford, Robert D. Bjornson, Giacomo Colussi, Tracy E. Hunley, Joseph R. Tucci, Fiona E. Karet, Craig C. Porter, Mohan Shenoy, Murim Choi, Priya Vaidyanathan, John W. Foreman, Jai Radhakrishnan, Stephanie Dewar, Alain Poujol, Sami A. Sanjad, Keith A. Choate, Carol Nelson-Williams, Kim M. Keppler-Noreuil, Richard D. Gordon, Matti Välimäki, Majid Rasoulpour, Richard P. Lifton, Maury Pinsk, Ali G. Gharavi, Craig W. Belsha, Hania Z. Al-Shahrouri, Sudhir K. Anand, Irina Tikhonova, Maria Elisabetta De Ferrari, Jim R. Stockigt, Marcel Lebel, Raoul D. Nelson, Howard Trachtman, Anita Farhi, Michael Gutkin, Alberto Bettinelli, Farook Thameem, and Hakan R. Toka
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Male ,Models, Molecular ,Pseudohypoaldosteronism ,Water-Electrolyte Imbalance ,Blood Pressure ,Sodium Chloride ,030204 cardiovascular system & hematology ,medicine.disease_cause ,Cohort Studies ,Electrolytes ,Mice ,0302 clinical medicine ,Locus heterogeneity ,Homeostasis ,Exome sequencing ,Genes, Dominant ,Genetics ,0303 health sciences ,Mutation ,Multidisciplinary ,Microfilament Proteins ,Exons ,Hydrogen-Ion Concentration ,Cullin Proteins ,WNK1 ,Disease gene identification ,3. Good health ,Phenotype ,Hypertension ,Female ,Genotype ,Molecular Sequence Data ,Genes, Recessive ,Biology ,Article ,03 medical and health sciences ,medicine ,Animals ,Humans ,Amino Acid Sequence ,Adaptor Proteins, Signal Transducing ,030304 developmental biology ,Phenocopy ,Base Sequence ,Gene Expression Profiling ,medicine.disease ,Potassium ,Carrier Proteins - Abstract
Hypertension affects one billion people and is a principal reversible risk factor for cardiovascular disease. Pseudohypoaldosteronism type II (PHAII), a rare Mendelian syndrome featuring hypertension, hyperkalaemia and metabolic acidosis, has revealed previously unrecognized physiology orchestrating the balance between renal salt reabsorption and K(+) and H(+) excretion. Here we used exome sequencing to identify mutations in kelch-like 3 (KLHL3) or cullin 3 (CUL3) in PHAII patients from 41 unrelated families. KLHL3 mutations are either recessive or dominant, whereas CUL3 mutations are dominant and predominantly de novo. CUL3 and BTB-domain-containing kelch proteins such as KLHL3 are components of cullin-RING E3 ligase complexes that ubiquitinate substrates bound to kelch propeller domains. Dominant KLHL3 mutations are clustered in short segments within the kelch propeller and BTB domains implicated in substrate and cullin binding, respectively. Diverse CUL3 mutations all result in skipping of exon 9, producing an in-frame deletion. Because dominant KLHL3 and CUL3 mutations both phenocopy recessive loss-of-function KLHL3 mutations, they may abrogate ubiquitination of KLHL3 substrates. Disease features are reversed by thiazide diuretics, which inhibit the Na-Cl cotransporter in the distal nephron of the kidney; KLHL3 and CUL3 are expressed in this location, suggesting a mechanistic link between KLHL3 and CUL3 mutations, increased Na-Cl reabsorption, and disease pathogenesis. These findings demonstrate the utility of exome sequencing in disease gene identification despite the combined complexities of locus heterogeneity, mixed models of transmission and frequent de novo mutation, and establish a fundamental role for KLHL3 and CUL3 in blood pressure, K(+) and pH homeostasis.
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- 2012
13. Data Smart : Using Data Science to Transform Information Into Insight
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John W. Foreman and John W. Foreman
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- Web sites--Design, Data mining, Web usage mining
- Abstract
Data Science gets thrown around in the press like it's magic. Major retailers are predicting everything from when their customers are pregnant to when they want a new pair of Chuck Taylors. It's a brave new world where seemingly meaningless data can be transformed into valuable insight to drive smart business decisions. But how does one exactly do data science? Do you have to hire one of these priests of the dark arts, the'data scientist,'to extract this gold from your data? Nope. Data science is little more than using straight-forward steps to process raw data into actionable insight. And in Data Smart, author and data scientist John Foreman will show you how that's done within the familiar environment of a spreadsheet. Why a spreadsheet? It's comfortable! You get to look at the data every step of the way, building confidence as you learn the tricks of the trade. Plus, spreadsheets are a vendor-neutral place to learn data science without the hype. But don't let the Excel sheets fool you. This is a book for those serious about learning the analytic techniques, the math and the magic, behind big data. Each chapter will cover a different technique in a spreadsheet so you can follow along: Mathematical optimization, including non-linear programming and genetic algorithms Clustering via k-means, spherical k-means, and graph modularity Data mining in graphs, such as outlier detection Supervised AI through logistic regression, ensemble models, and bag-of-words models Forecasting, seasonal adjustments, and prediction intervals through monte carlo simulation Moving from spreadsheets into the R programming language You get your hands dirty as you work alongside John through each technique. But never fear, the topics are readily applicable and the author laces humor throughout. You'll even learn what a dead squirrel has to do with optimization modeling, which you no doubt are dying to know.
- Published
- 2014
14. Glutamine Transport into Isolated Renal Membrane Vesicles from Normal and Acidotic Rats
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John W. Foreman, Stanton Segal, Robert Reynolds, and Louise M. Pepe
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Glutamine ,Kidney cortex ,Cell membrane permeability ,Biochemistry ,business.industry ,Medicine ,Membrane vesicle ,Mitochondrion ,medicine.symptom ,business ,Glutamine transport ,Acidosis - Published
- 2015
15. Divalent Ions in the Young
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Kay Latta, John W. Foreman, and James C. M. Chan
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chemistry.chemical_classification ,chemistry ,Inorganic chemistry ,Pediatric nephrology ,Divalent - Published
- 2015
16. Neutropenic enterocolitis (typhlitis) in a pediatric renal transplant patient. A case report and review of the literature
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John W. Foreman, Kathleen A. McGann, Jonathan H. Pelletier, Delbert R. Wigfall, Rasheed Gbadegesin, and Shashi K. Nagaraj
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Male ,Abdominal pain ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Neutropenia ,03 medical and health sciences ,Cecum ,Postoperative Complications ,0302 clinical medicine ,Edema ,medicine ,Humans ,Transplantation ,business.industry ,Neutropenic enterocolitis ,Enterocolitis, Neutropenic ,Immunosuppression ,medicine.disease ,Kidney Transplantation ,Surgery ,Diarrhea ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Pediatrics, Perinatology and Child Health ,030211 gastroenterology & hepatology ,medicine.symptom ,business - Abstract
NE (typhlitis) is a potentially life-threatening disease process characterized by bowel wall edema, ulceration, and hemorrhage in an immunosuppressed patient. We report a 15-year-old boy status post deceased donor renal transplantation who presented with fever, abdominal pain, and diarrhea. Laboratory studies revealed neutropenia 5 days prior to admission, and abdominal computed tomography revealed bowel wall thickening in the cecum consistent with NE. He was treated with piperacillin-tazobactam and gentamicin and recovered. To our knowledge, this is the first report of a case of NE in a pediatric kidney transplant recipient.
- Published
- 2017
17. HLA-DQA1 and PLCG2 Are Candidate Risk Loci for Childhood-Onset Steroid-Sensitive Nephrotic Syndrome
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Atif Awan, John W. Foreman, Shenal Thalgahagoda, Tracy E. Hunley, Deepa H. Chand, Arundhati S. Kale, Halima S. Janjua, Vladimir Belostotsky, Catherine O'Brien, Michelle P. Winn, Guanghong Wu, William E. Smoyer, Tarak Srivastava, Yi Cai, Adebowale Adeyemo, Joanna R. Ghali, Patrick D. Brophy, Alison Homstad, Nicholas J. A. Webb, Jen Jar Lin, Kathy Nicholls, Gina Marie Barletta, Gentzon Hall, Delbert R. Wigfall, Arvind Bagga, Rasheed Gbadegesin, Abiodun Aderogba Omoloja, Shashi K. Nagaraj, Elizabeth Abraham, Larry A. Greenbaum, A S Abeyagunawardena, Michelle N. Rheault, Aditi Sinha, David D. Milford, and Debbie S. Gipson
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Male ,Nephrotic Syndrome ,Genotype ,Mutation, Missense ,Single-nucleotide polymorphism ,Human leukocyte antigen ,Biology ,HLA-DQ alpha-Chains ,Cohort Studies ,Age Distribution ,Clinical Research ,Missense mutation ,Humans ,Genetic Predisposition to Disease ,Allele ,Age of Onset ,Sex Distribution ,Child ,Exome ,Alleles ,Sri Lanka ,Genetics ,Phospholipase C gamma ,Incidence ,Case-control study ,General Medicine ,Odds ratio ,Nephrology ,Case-Control Studies ,Child, Preschool ,Immunology ,Female ,Steroids ,Age of onset - Abstract
Steroid-sensitive nephrotic syndrome (SSNS) accounts for >80% of cases of nephrotic syndrome in childhood. However, the etiology and pathogenesis of SSNS remain obscure. Hypothesizing that coding variation may underlie SSNS risk, we conducted an exome array association study of SSNS. We enrolled a discovery set of 363 persons (214 South Asian children with SSNS and 149 controls) and genotyped them using the Illumina HumanExome Beadchip. Four common single nucleotide polymorphisms (SNPs) in HLA-DQA1 and HLA-DQB1 (rs1129740, rs9273349, rs1071630, and rs1140343) were significantly associated with SSNS at or near the Bonferroni-adjusted P value for the number of single variants that were tested (odds ratio, 2.11; 95% confidence interval, 1.56 to 2.86; P=1.68×10(-6) (Fisher exact test). Two of these SNPs-the missense variants C34Y (rs1129740) and F41S (rs1071630) in HLA-DQA1-were replicated in an independent cohort of children of white European ancestry with SSNS (100 cases and ≤589 controls; P=1.42×10(-17)). In the rare variant gene set-based analysis, the best signal was found in PLCG2 (P=7.825×10(-5)). In conclusion, this exome array study identified HLA-DQA1 and PLCG2 missense coding variants as candidate loci for SSNS. The finding of a MHC class II locus underlying SSNS risk suggests a major role for immune response in the pathogenesis of SSNS.
- Published
- 2014
18. Prevelance and clinical correlates of glomerulopathy in children with sickle cell disease
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Delbert R. Wigfall, John W. Foreman, Russell E. Ware, Margaret Burchinal, and Thomas R. Kinney
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medicine.medical_specialty ,Proteinuria ,urogenital system ,Anemia ,business.industry ,Renal function ,urologic and male genital diseases ,medicine.disease ,Sickle cell nephropathy ,Gastroenterology ,female genital diseases and pregnancy complications ,Sickle cell anemia ,Acute chest syndrome ,Surgery ,Nephropathy ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,medicine.symptom ,business ,Kidney disease - Abstract
Objectives: Glomerular disease and renal failure cause substantial morbidity for patients with sickle cell disease (SCD). Proteinuria is an early manifestation of sickle nephropathy, but the prevalence of proteinuria and its clinical correlations in children with SCD are unknown. Study design: Data were collected prospectively on children with SCD for 10 years including physical measurements, laboratory test results, and clinical complications. Persistent proteinuria was defined as ≥1 + protein on urinalysis for at least 6 months. The glomerular filtration rate was estimated with serum creatinine concentration and height. Proteinuria was correlated with other variables by χ 2 analysis. Results: Proteinuria occurred in 20 of 442 pediatric patients including 15 (6.2%) with sickle cell anemia. Proteinuria increased with age, affecting 12% of older teenagers with sickle cell anemia. Proteinuria was significantly associated with lower hemoglobin concentration, higher mean corpuscular volume, and higher leukocyte count. For children of some ages, proteinuria was associated with complications including stroke, acute chest syndrome, cholelithiasis, and hospitalizations. Glomerular filtration rate hyperfiltration occurred early in life, followed by normalization. Conclusions: Sickle nephropathy, manifested as persistent proteinuria, begins early in life, occurs in all forms of SCD, and is associated with severity of disease. Early detection of proteinuria may allow therapy to prevent progressive renal insufficiency.
- Published
- 2000
19. TNXB mutations can cause vesicoureteral reflux
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Alison Homstad, John S. Wiener, Sherry S. Ross, Sara E. Miller, Gentzon Hall, Settara C. Chandrasekharappa, Indra R. Gupta, Michelle P. Winn, Patrick D. Brophy, John W. Foreman, Angelica Selim, Charles N. Rotimi, Adebowale Adeyemo, C. Egla Rabinovich, Jason C. Clarke, Peter Lavin, Guanghong Wu, Bartlomeij Bartkowiak, Katherine Westreich, David S. Hains, Danniele G. Holanda, Rasheed Gbadegesin, and Yutao Liu
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Heterozygote ,Tenascin ,Genome-wide association study ,Biology ,medicine.disease_cause ,Kidney ,Vesicoureteral reflux ,Focal adhesion ,Cell Movement ,medicine ,Cell Adhesion ,Humans ,Urinary Tract ,Vesico-Ureteral Reflux ,Mutation ,Kidney metabolism ,Heterozygote advantage ,General Medicine ,Sequence Analysis, DNA ,medicine.disease ,Pedigree ,Nephrology ,Ureterovesical Junction ,biology.protein ,Female ,Genome-Wide Association Study - Abstract
Primary vesicoureteral reflux (VUR) is the most common congenital anomaly of the kidney and the urinary tract, and it is a major risk factor for pyelonephritic scarring and CKD in children. Although twin studies support the heritability of VUR, specific genetic causes remain elusive. We performed a sequential genome-wide linkage study and whole-exome sequencing in a family with hereditary VUR. We obtained a significant multipoint parametric logarithm of odds score of 3.3 on chromosome 6p, and whole-exome sequencing identified a deleterious heterozygous mutation (T3257I) in the gene encoding tenascin XB (TNXB in 6p21.3). This mutation segregated with disease in the affected family as well as with a pathogenic G1331R change in another family. Fibroblast cell lines carrying the T3257I mutation exhibited a reduction in both cell motility and phosphorylated focal adhesion kinase expression, suggesting a defect in the focal adhesions that link the cell cytoplasm to the extracellular matrix. Immunohistochemical studies revealed that the human uroepithelial lining of the ureterovesical junction expresses TNXB, suggesting that TNXB may be important for generating tensile forces that close the ureterovesical junction during voiding. Taken together, these results suggest that mutations in TNXB can cause hereditary VUR.
- Published
- 2013
20. Effects of low-dose dopamine on urine output in normotensive very low birth weight neonates
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Chi D. Hornik, C M Cotten, John W. Foreman, P B Smith, James L. Wynn, J L Crouchley, and Ronald N. Goldberg
- Subjects
Male ,medicine.medical_specialty ,media_common.quotation_subject ,Dopamine ,Urination ,Urine ,Kidney ,Article ,Internal medicine ,Medicine ,Humans ,Infant, Very Low Birth Weight ,Urine output ,media_common ,Retrospective Studies ,business.industry ,Low dose ,Infant, Newborn ,Obstetrics and Gynecology ,Low birth weight ,Endocrinology ,medicine.anatomical_structure ,Renal physiology ,Pediatrics, Perinatology and Child Health ,Female ,medicine.symptom ,business ,medicine.drug - Abstract
To determine the effects of low-dose dopamine on urine output (UOP) in very low birth weight premature neonates.Retrospective cohort study of all low-dose (3-5 μg kg(-1) per min) dopamine infusions24-h duration in neonates 1500 g and 32 weeks gestation from August 2009 through September 2011. Linear regression was used to estimate the impact of covariates on UOP.We identified 91 episodes of low-dose dopamine use in 65 neonates. Increased UOP occurred in 64% of episodes. Low-dose dopamine use was associated with a 0.6 ml kg(-1) h(-1) increase in UOP (P0.001) and a 1.3 ml kg(-1)h(-1) increase when baseline UOP was1.5 ml kg(-1) h(-1) (P0.001). The improvement remained statistically significant after controlling for medications (diuretics and hydrocortisone) and fluid intake.Low-dose dopamine use was associated with increased UOP in very low birth weight neonates.
- Published
- 2012
21. Insulin-like growth factor-I gene expression in the tibial epiphyseal growth plate of growth hormone-treated uremic rats
- Author
-
James D. Hanna, Fernando Santos, James C. M. Chan, John W. Foreman, and Victor K.M. Han
- Subjects
Male ,medicine.medical_specialty ,medicine.medical_treatment ,Long bone ,Biology ,Chondrocyte ,Rats, Sprague-Dawley ,Insulin-like growth factor ,Internal medicine ,Gene expression ,medicine ,Animals ,Tibia ,Growth Plate ,RNA, Messenger ,Insulin-Like Growth Factor I ,Saline ,In Situ Hybridization ,Uremia ,Growth factor ,medicine.disease ,Immunohistochemistry ,Rats ,medicine.anatomical_structure ,Endocrinology ,Nephrology ,Growth Hormone ,Cell Division - Abstract
Insulin-like growth factor-I gene expression in the tibial epiphyseal growth plate of growth hormone-treated uremic rats. To identify the molecular mechanisms involved in long bone growth of uremic animals, we evaluated the effects of recombinant human growth hormone (rhGH) supplementation on whole body growth, growth plate morphometries, and insulin-like growth factor-I (IGF-I) gene expression in the tibial epiphyseal growth plates of uremic rats. Uremia was induced by a two-stage subtotal nephrectomy (Nx) of 30-day-old rats, followed by rhGH (N = 6) or saline (N = 6) treatment from day 56 to day 70 of age. Controls (N = 4) were sham decapsulated. Treatment with rhGH on Nx animals caused: (1) a significant increase in weight, (2) longitudinal growth similar to controls, and (3) increased total growth plate width predominantly due to an increase in hypertrophic zone width. rhGH increased IGF-I mRNA abundance in both zones, but the increase was greater in the proliferative zone. These changes were accompanied by concomitant alterations in IGF-I immunoreactivity. In uremic animals, therefore, rhGH treatment induces local IGF-I gene expression in the growth plate and increases the hypertrophic zone width but not the proliferative zone width. The latter suggests resistance to IGF-I action in that zone.
- Published
- 1995
- Full Text
- View/download PDF
22. Disorders of Distal Tubular Transport of Sodium and Potassium
- Author
-
John W. Foreman and Sami A. Sanjad
- Subjects
Chemistry ,Sodium ,Potassium ,Biophysics ,chemistry.chemical_element - Published
- 2012
23. Renal Tubular Acidosis
- Author
-
Rasheed A. Gbadegesin and John W. Foreman
- Published
- 2011
24. Acid–Base in Health and Disease
- Author
-
Rasheed Gbadegesin and John W. Foreman
- Subjects
Chemistry ,Stereochemistry ,Base (exponentiation) - Published
- 2011
25. PATHOGENESIS AND THERAPY OF FOCAL SEGMENTAL GLOMERULOSCLEROSIS: AN UPDATE
- Author
-
Rasheed Gbadegesin, Michelle P. Winn, John W. Foreman, and Peter Lavin
- Subjects
Nephrology ,medicine.medical_specialty ,Pathology ,Bioinformatics ,urologic and male genital diseases ,Kidney ,Article ,Podocyte ,Pathogenesis ,Focal segmental glomerulosclerosis ,Risk Factors ,Molecular genetics ,Internal medicine ,medicine ,Humans ,Genetic Predisposition to Disease ,business.industry ,urogenital system ,Glomerulosclerosis, Focal Segmental ,Podocytes ,Glomerulosclerosis ,medicine.disease ,female genital diseases and pregnancy complications ,medicine.anatomical_structure ,Treatment Outcome ,Pediatrics, Perinatology and Child Health ,business ,Nephrotic syndrome ,Immunosuppressive Agents ,Kidney disease ,Stem Cell Transplantation - Abstract
Focal and segmental glomerulosclerosis (FSGS) is an important cause of steroid-resistant nephrotic syndrome in adults and children. It is responsible for 5–20% of all cases of end-stage kidney disease (ESKD) in the United States. The pathogenesis of FSGS has not been fully elucidated; however, data from molecular studies of familial cases in the last two decades suggest that FSGS is a defect of the podocyte. The therapeutic agents available for treatment of FSGS are not very effective and only a small percentage of affected individuals will achieve complete remission. Recent data from molecular biology and molecular genetics has provided insight into the mechanisms of action of old agents and also identification of other novel therapeutic targets. This review focuses on recent advances in the molecular pathogenesis of FSGS and currently available therapeutic agents as well as potential novel therapies.
- Published
- 2010
26. Fanconi Syndrome and Other Proximal Tubule Disorders
- Author
-
John W. Foreman
- Subjects
Pathology ,medicine.medical_specialty ,medicine.anatomical_structure ,business.industry ,medicine ,Fanconi syndrome ,Proximal tubule ,medicine.disease ,business - Published
- 2010
27. Contributors
- Author
-
Sharon Adler, Horacio J. Adrogué, Venkatesh Aiyagari, Robert J. Alpern, Charles E. Alpers, Gerald B. Appel, Fatiu A. Arogundade, Stephen R. Ash, Arif Asif, Pierre Aucouturier, Phyllis August, George L. Bakris, Adam D. Barlow, Rashad S. Barsoum, Chris Baylis, Aminu Bello, Tomas Berl, Suresh Bhat, Gemma Bircher, Joseph V. Bonventre, Josée Bouchard, Nicholas R. Brook, Christopher Brown, Mark A. Brown, Emmanuel A. Burdmann, David A. Bushinsky, Daniel C. Cattran, Matthew J. Cervelli, Steven J. Chadban, Karen E. Charlton, Yipu Chen, Ignatius K.P. Cheng, John O. Connolly, William G. Couser, Paolo Cravedi, Vivette D. D’Agati, Gabriel M. Danovitch, Simon J. Davies, John M. Davison, Wayne Derman, Gerald F. DiBona, Tilman B. Drüeke, Jamie P. Dwyer, Kai-Uwe Eckardt, Jason Eckel, Frank Eitner, Mohsen El Kossi, Marlies Elger, Elwaleed A. Elhassan, Pieter Evenepoel, June Fabian, Ronald J. Falk, John Feehally, Evelyne A. Fischer, Jonathan S. Fisher, Jürgen Floege, Giovanni B. Fogazzi, John W. Foreman, Toshiro Fujita, F. John Gennari, Evangelos G. Gkougkousis, Richard J. Glassock, Philip B. Gorelick, Barbara A. Greco, Peter Gross, Lisa M. Guay-Woodford, Nabil Haddad, Kevin P.G. Harris, Peter C. Harris, Lee A. Hebert, Peter Heduschka, Charles A. Herzog, Thomas Hooton, Walter H. Hörl, Peter F. Hoyer, Jeremy Hughes, Christian Hugo, Enyu Imai, Ashley B. Irish, Bertrand L. Jaber, Sunjay Jain, David Jayne, J. Ashley Jefferson, J. Charles Jennette, Vivekanand Jha, Richard J. Johnson, Nigel S. Kanagasundaram, John Kanellis, S. Ananth Karumanchi, Clifford E. Kashtan, Carol A. Kauffman, Bisher Kawar, Bryan Kestenbaum, Markus Ketteler, Jeffrey Kopp, Peter Kotanko, Wilhelm Kriz, Martin K. Kuhlmann, Dirk R. Kuypers, Jonathan R.T. Lakey, Estelle V. Lambert, William Lawton, Andrew S. Levey, Nathan W. Levin, Jeremy Levy, Andrew Lewington, Julia B. Lewis, Felix F.K. Li, Stuart L. Linas, Friedrich C. Luft, Jan C. ter Maaten, Iain C. Macdougall, Etienne Macedo, Nicolaos E. Madias, Colm C. Magee, Christopher L. Marsh, Mark R. Marshall, Kevin J. Martin, Philip D. Mason, Ranjiv Mathews, Tej K. Mattoo, Ravindra L. Mehta, Herwig-Ulf Meier-Kriesche, J. Kilian Mellon, M. Reza Mirbolooki, Rebeca D. Monk, Bruno Moulin, William R. Mulley, Meguid El Nahas, Saraladevi Naicker, Masaomi Nangaku, Guy H. Neild, M. Gary Nicholls, Michael L. Nicholson, Philip J. O’Connell, W. Charles O’Neill, Biff F. Palmer, Chirag Parikh, Phuong-Chi T. Pham, Phuong-Thu T. Pham, Son V. Pham, Richard G. Phelps, Raimund Pichler, Tiina Podymow, Wolfgang Pommer, Charles D. Pusey, Hamid Rabb, Brian Rayner, Hugh C. Rayner, Giuseppe Remuzzi, A. Mark Richards, Bengt Rippe, Eberhard Ritz, R. Paul Robertson, Bernardo Rodriguez-Iturbe, Claudio Ronco, Pierre M. Ronco, Edward A. Ross, Jerome A. Rossert, Piero Ruggenenti, Sean Ruland, Graeme R. Russ, Martin A. Samuels, Pantelis A. Sarafidis, F. Paolo Schena, Jesse D. Schold, Robert W. Schrier, Victor F. Seabra, Mark S. Segal, Julian Lawrence Seifter, Shani Shastri, David G. Shirley, Visith Sitprija, Titte R. Srinivas, Peter Stenvinkel, Lesley A. Stevens, Stephen C. Textor, Joshua M. Thurman, Li-Li Tong, Peter S. Topham, Jan H.M. Tordoir, Vicente E. Torres, Dace Trence, A. Neil Turner, Robert J. Unwin, Henri Vacher-Coponat, R. Kasi Visweswaran, Haimanot Wasse, Moses D. Wavamunno, I. David Weiner, David C. Wheeler, Bryan Williams, John D. Williams, Charles S. Wingo, Michelle Winn, Alexander C. Wiseman, Gunter Wolf, Karl Womer, Graham Woodrow, David C. Wymer, Li Yang, and Xueqing Yu
- Published
- 2010
28. Growth in chronic renal disease
- Author
-
John W. Foreman, James D. Hanna, and James C.M. Chan
- Subjects
Nephrology ,medicine.medical_specialty ,business.industry ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Chronic renal disease ,business - Published
- 1992
29. Inhibition of calcium oxalate crystal growth in vitro by uropontin: another member of the aspartic acid-rich protein superfamily
- Author
-
H Shiraga, W Min, C H Terrell, Craig T. Przysiecki, John W. Foreman, D Miner, M D Clayman, Joseph R. Sherbotie, W J VanDusen, and Eric G. Neilson
- Subjects
Sialoglycoproteins ,Molecular Sequence Data ,Calcium oxalate ,chemistry.chemical_compound ,Affinity chromatography ,Aspartic acid ,Humans ,Amino Acid Sequence ,Osteopontin ,Peptide sequence ,Aspartic Acid ,Urinary Bladder Calculi ,Multidisciplinary ,Calcium Oxalate ,biology ,Chemistry ,Antibodies, Monoclonal ,Proteins ,Protein superfamily ,In vitro ,Biochemistry ,Multigene Family ,biology.protein ,Crystallization ,Sequence Alignment ,Research Article ,Biomineralization - Abstract
The majority of human urinary stones are primarily composed of calcium salts. Although normal urine is frequently supersaturated with respect to calcium oxalate, most humans do not form stones. Inhibitors are among the multiple factors that may influence the complex process of urinary stone formation. We have isolated an inhibitor of calcium oxalate crystal growth from human urine by monoclonal antibody immunoaffinity chromatography. The N-terminal amino acid sequence and acidic amino acid content of this aspartic acid-rich protein, uropontin, are similar to those of other pontin proteins from bone, plasma, breast milk, and cells. The inhibitory effect of uropontin on calcium oxalate crystal growth in vitro supports the concept that pontins may have a regulatory role. This function would be analogous to that of other members of the aspartic acid-rich protein superfamily, which stereospecifically regulate the mineralization fronts of calcium-containing crystals.
- Published
- 1992
30. Thrombocytopenia and absent radii (TAR) syndrome associated with horseshoe kidney
- Author
-
John W. Foreman, A. Bradshaw, and Lane F. Donnelly
- Subjects
Adult ,Male ,Nephrology ,medicine.medical_specialty ,Clubfoot ,Genes, Recessive ,Kidney ,Kidney Function Tests ,Internal medicine ,Parenchyma ,Humans ,Medicine ,Abnormalities, Multiple ,Ultrasonography ,Genitourinary system ,business.industry ,TAR syndrome ,Horseshoe kidney ,Syndrome ,Anatomy ,medicine.disease ,Thrombocytopenia ,Radius ,Agenesis ,Pediatrics, Perinatology and Child Health ,business ,Kidney disease - Abstract
The TAR syndrome is an inherited disorder characterized by limb abnormalities, especially absent radii, and hypomegakaryocytic thrombocytopenia. Previous reports have included two infants with genitourinary abnormalities. We report a newborn with bilaterally absent radii and foreshortened ulnae, hypoplastic humeri, a left clubfoot, a ventricular septum defect, and persistent thrombocytopenia. This constellation of abnormalities is consistent with the TAR syndrome. In addition, he had a horseshoe kidney with parenchyma of normal appearance. This is the first report of horseshoe kidney in association with the TAR syndrome.
- Published
- 2000
31. Renal Transplantation in Children
- Author
-
H.M. Lee, John W. Foreman, James C.M. Chan, M.P. Posner, James D. Hanna, and F. Santos
- Subjects
Biomaterials ,Transplantation ,medicine.medical_specialty ,Text mining ,business.industry ,Biomedical Engineering ,Medicine (miscellaneous) ,Medicine ,Bioengineering ,General Medicine ,business ,Intensive care medicine - Published
- 1991
32. Effect of cystine loading and cystine dimethylester on renal brushborder membrane transport
- Author
-
John W. Foreman and Linda L. Benson
- Subjects
Male ,Proline ,Cystinosis ,Biophysics ,Cystine ,Biochemistry ,chemistry.chemical_compound ,medicine ,Animals ,Molecular Biology ,Incubation ,Cells, Cultured ,Renal tubule ,Microvilli ,Vesicle ,Biological Transport ,Rats, Inbred Strains ,Cell Biology ,Membrane transport ,Fanconi Syndrome ,medicine.disease ,Rats ,Kidney Tubules ,Membrane ,chemistry - Abstract
The effect of loading renal tubule cells with cystine was studied by incubating them with cystine dimethylester. Proline uptake into brushborder membrane vesicles isolated from the cystine loaded cells was not different from that observed into brushborder vesicles isolated from tubules incubated in buffer alone. Incubating brushborder membranes with 2 mM cystine dimethylester for 10 minutes reduced the uptake of proline by 27% after 15 seconds of incubation and by 21% after 60 seconds of incubation. There was no effect after 20 minutes of incubation. Pre-incubating brushborder membrane vesicles with cystine dimethylester had no statistically significant effect on the affinity of priline for the carrier, but did reduce the maximal rate of proline uptake by 49%.
- Published
- 1990
33. Quality control of the nutritional component of the Growth Failure in Children with Renal Diseases Study
- Author
-
C. Frederic Strife, Martha D. Massie, John W. Foreman, and James C.M. Chan
- Subjects
Quality Control ,medicine.medical_specialty ,Dietetics ,MEDLINE ,law.invention ,Eating ,Randomized controlled trial ,law ,medicine ,Humans ,Multicenter Studies as Topic ,Child ,Intensive care medicine ,Patient compliance ,Growth Disorders ,Randomized Controlled Trials as Topic ,Data collection ,Anthropometry ,business.industry ,Data Collection ,Dietary intake ,Reproducibility of Results ,Quality control ,Diet ,Surgery ,Clinical trial ,Nutrition Assessment ,Pediatrics, Perinatology and Child Health ,Kidney Failure, Chronic ,business - Abstract
Quality control procedures are critically important in a clinical trial in which dietitians in geographically separated units perform collaborative nutritional intervention and data collection during a period of several years. Typically, the quality control measures developed within the nutritional component of a clinical trial encompass the following areas of activity: 1. Uniform collection and coding procedures for dietary intake records 2. Collection of accurate and reliable anthropometric measurements 3. Nutritional assessment, intervention, and counseling of patients 4. Assessment of patient compliance with dietary intervention Accordingly, the functions of the core nutritional unit of a multicenter clinical trial are as follows: 1. To ensure the collection of uniform, accurate nutrient and anthropometric data in participating centers using standardized procedures 2. To provide consistent nutritional education to ensure standardized counseling of the patient, the primary caretaker, or both The major objective of the nutritional component of this clinical trial is to identify changes in nutriture and appetite as evidenced by food consumption records, appetite assessment, and anthropometric measurements, and to intervene as study guidelines permit. From December 1984 to June 1989, the core nutritional
- Published
- 1990
34. Effect of cystine loading on substrate oxidation by rat renal tubules
- Author
-
John W. Foreman and Linda L. Benson
- Subjects
Male ,Nephrology ,medicine.medical_specialty ,Cystine ,Butyrate ,In Vitro Techniques ,chemistry.chemical_compound ,Oxygen Consumption ,Internal medicine ,medicine ,Animals ,Cysteine ,Incubation ,business.industry ,Substrate (chemistry) ,Rats, Inbred Strains ,Succinates ,Metabolism ,Carbon Dioxide ,medicine.disease ,Rats ,Butyrates ,Glucose ,Kidney Tubules ,Endocrinology ,chemistry ,Pediatrics, Perinatology and Child Health ,Cystinosis ,Lactates ,business ,Oxidation-Reduction - Abstract
Isolated rat renal tubules were loaded with cystine by incubating them with 2 mM cystine dimethylester. The oxidation of 1 mM glucose and lactate was significantly decreased after 20 and 30 min of incubation, in the cystine-loaded tubules compared with control tubules. The oxidation of 1 mM butyrate was significantly decreased after 10 and 30 min of incubation in the cystine-loaded tubules. Cystine loading decreased the oxidation of 1 mM succinate at all time points examined. The O2 consumption of renal tubules was reduced 59% with cystine loading by the addition of 2 mM cystine dimethylester, and by 37% with 1 mM cystine dimethylester. These data indicate that loading normal renal tubule cells with cystine impairs their ability to oxidize metabolic fuels. This impairment in metabolism may explain the decreased transport observed previously in cystine-loaded tubules and may have implications for the human disorder, cystinosis.
- Published
- 1990
35. Drug-induced granulomatous interstitial nephritis in a pediatric patient
- Author
-
James E. Tong, David N. Howell, and John W. Foreman
- Subjects
Nephrology ,medicine.medical_specialty ,Pathology ,Adolescent ,Interstitial nephritis ,Biopsy ,Antigen-Antibody Complex ,Kidney ,Basement Membrane ,Internal medicine ,medicine ,Humans ,Glucocorticoids ,Tetralogy of Fallot ,Cefuroxime ,Granuloma ,medicine.diagnostic_test ,business.industry ,Kidney metabolism ,Acute Kidney Injury ,medicine.disease ,Anti-Bacterial Agents ,Giant cell ,Pediatrics, Perinatology and Child Health ,Nephritis, Interstitial ,Prednisone ,Female ,Renal biopsy ,business ,Nephritis - Abstract
Acute interstitial nephritis (AIN) is a known cause of acute renal failure in children. In most instances, drug therapy is the offending agent. Although granuloma formation has been observed in drug-induced interstitial nephritis, it is not a commonly associated manifestation. This is a case of a 15-year-old white female with Tetralogy of Fallot and pulmonary atresia who developed acute renal failure secondary to drug-induced interstitial nephritis and renal granulomas. In addition to interstitial edema with eosinophils and lymphocytes, her renal biopsy showed interstitial granulomas, immune complexes within tubular basement membranes, and the unusual feature of multinucleated giant cells engulfing tubules. Her acute renal failure resolved after the withdrawal of antibiotics and the initiation of intravenous steroid therapy.
- Published
- 2006
36. American Society of Pediatric Nephrology position paper on linking reimbursement to quality of care
- Author
-
Barbara A. Fivush, Jennifer Shevchek, John W. Foreman, Neil R. Powe, Sharon Andreoli, Sandra L. Watkins, and Eileen D. Brewer
- Subjects
Nephrology ,Quality Control ,medicine.medical_specialty ,urologic and male genital diseases ,Pediatrics ,Reimbursement Mechanisms ,Internal medicine ,Research Support as Topic ,Fee Schedules ,Outcome Assessment, Health Care ,Medicine ,Pediatric nephrology ,Humans ,Quality of care ,Intensive care medicine ,Child ,Reimbursement ,Societies, Medical ,Quality of Health Care ,Health Services Needs and Demand ,Evidence-Based Medicine ,business.industry ,Public health ,General Medicine ,medicine.disease ,female genital diseases and pregnancy complications ,United States ,Treatment Outcome ,El Niño ,Position paper ,Kidney Failure, Chronic ,Health Services Research ,business ,Kidney disease - Abstract
The pediatric ESRD patient is a member of a unique subpopulation of ESRD patients. The cause of ESRD in the pediatric patient differs markedly from the adult patient; treatment modality in the pediatric ESRD patient differs substantially from the adult patient; and outcomes such as growth
- Published
- 2005
37. Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination
- Author
-
Tomoko Obara, Julia Hoefele, Hubert Nivet, Bernhard Schermer, Tom Strachan, Marie F. Gagnadoux, John F. O'Toole, Corinne Antignac, Frank Beekmann, Judith A. Goodship, Andreas Kispert, Rainer G. Ruf, Gerd Walz, Adelheid M. Mueller, Matthias T.F. Wolf, Thomas Benzing, Karl S. Hiller, Daniel Landau, Friedhelm Hildebrandt, Edgar A. Otto, John W. Foreman, and Iain A. Drummond
- Subjects
Genetics ,Cilium ,TMEM67 ,Senior–Løken syndrome ,Biology ,medicine.disease ,Article ,Cell biology ,Cystic kidney disease ,Nephronophthisis ,RPGRIP1L ,medicine ,Polycystic kidney disease ,Heart looping - Abstract
Nephronophthisis (NPHP), an autosomal recessive cystic kidney disease, leads to chronic renal failure in children. The genes mutated in NPHP1 and NPHP4 have been identified, and a gene locus associated with infantile nephronophthisis (NPHP2) was mapped. The kidney phenotype of NPHP2 combines clinical features of NPHP and polycystic kidney disease (PKD). Here, we identify inversin (INVS) as the gene mutated in NPHP2 with and without situs inversus. We show molecular interaction of inversin with nephrocystin, the product of the gene mutated in NPHP1 and interaction of nephrocystin with β-tubulin, a main component of primary cilia. We show that nephrocystin, inversin and β-tubulin colocalize to primary cilia of renal tubular cells. Furthermore, we produce a PKD-like renal cystic phenotype and randomization of heart looping by knockdown of invs expression in zebrafish. The interaction and colocalization in cilia of inversin, nephrocystin and β-tubulin connect pathogenetic aspects of NPHP to PKD, to primary cilia function and to left-right axis determination.
- Published
- 2003
38. Appearance of immune complex glomerulonephritis following the onset of type I diabetes mellitus in a child
- Author
-
David N. Howell, Lisa A. Robinson, Delbert R. Wigfall, and John W. Foreman
- Subjects
Male ,medicine.medical_specialty ,Nephrotic Syndrome ,Time Factors ,Biopsy ,Kidney ,Gastroenterology ,Diabetic nephropathy ,Diagnosis, Differential ,Glomerulonephritis ,Diabetes mellitus ,Internal medicine ,Immunopathology ,medicine ,Humans ,Immune Complex Diseases ,Diabetic Nephropathies ,Proteinuria ,business.industry ,medicine.disease ,Immune complex ,Diabetes Mellitus, Type 1 ,Nephrology ,Child, Preschool ,Immunology ,medicine.symptom ,business ,Nephrotic syndrome ,Kidney disease - Abstract
Renal disease is a frequent late complication of type I diabetes mellitus, occurring almost entirely in adult patients. Typical diabetic nephropathy is characterized by proteinuria, and by the histological lesions of mesangial expansion and basement membrane thickening. We report an interesting case of a 3-year-old boy who developed immune complex glomerulonephritis with nephrotic syndrome 2 months after the onset of insulin-dependent diabetes mellitus.
- Published
- 1997
39. Nutritional intake in children with renal insufficiency: a report of the growth failure in children with renal diseases study
- Author
-
C. F. Strife, C. L. Abitbol, James C.M. Chan, M. D. Massie, L. G. Feld, R. M. Boyle, John W. Foreman, E. H. Garin, and H. Trachtman
- Subjects
medicine.medical_specialty ,Medicine (miscellaneous) ,Physiology ,Renal function ,Body size ,Body weight ,Internal medicine ,medicine ,Humans ,Renal Insufficiency ,Child ,Growth Disorders ,Nutrition and Dietetics ,business.industry ,Body Weight ,Infant ,Vitamins ,Protein intake ,medicine.disease ,Caloric intake ,Body Height ,Diet Records ,Diet ,Endocrinology ,El Niño ,Dietary Reference Intake ,Child, Preschool ,Dietary Proteins ,business ,Child Nutritional Physiological Phenomena ,Energy Intake ,Kidney disease - Abstract
This study was designed to assess sequentially the nutrient intake in children with chronic renal insufficiency and its relationship to body size, the level of renal failure, and growth velocity.The nutrient intake from 401 4-day food records obtained from 120 children with renal insufficiency over a 6-month observation period was analyzed. The height and weight were measured at the beginning and end of the observation period. The glomerular filtration rate was estimated from the height and serum creatinine.The mean caloric intake in these children was 80 +/- 23% (mean +/- SD) of the Recommended Dietary Allowance (RDA) for age. Fifty-six percent of the food records obtained from these children revealed a caloric intake that was less than 80% of the RDA. Caloric intake expressed as the %RDA for age decreased with increasing age. However, the mean caloric intake when factored by body weight was in the normal range. There was no correlation between caloric intake and height velocity. The mean protein intake in these children was 153 +/- 53% of the RDA. Further, 45% of the food records indicated a protein intake greater than 150% of the RDA. There was no relationship between the degree of renal insufficiency and caloric or protein intake. Calcium, vitamin, and zinc intakes were also low.Children with chronic renal failure consume less calories than their age matched peers, but the majority of these children appear to ingest adequate amounts for their body mass. This reduction in caloric intake occurs early in renal insufficiency. They also ingest inadequate amounts of calcium, zinc, vitamin B6, and folate.
- Published
- 1996
40. The peritoneal equilibration test in children
- Author
-
Jean Wolfrum, James C.M. Chan, John W. Foreman, Todd W.B. Gehr, James D. Hanna, and Joyce Ruddley
- Subjects
Adult ,Blood Glucose ,medicine.medical_specialty ,Adolescent ,medicine.medical_treatment ,Urology ,Renal function ,Peritoneal equilibration test ,Peritoneal dialysis ,chemistry.chemical_compound ,Peritoneal Dialysis, Continuous Ambulatory ,Dialysis Solutions ,Medicine ,Ascitic Fluid ,Humans ,Child ,Tidal volume ,Creatinine ,business.industry ,Continuous ambulatory peritoneal dialysis ,Infant ,Middle Aged ,Surgery ,Glucose ,Volume (thermodynamics) ,chemistry ,Nephrology ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Ambulatory ,Kidney Failure, Chronic ,business - Abstract
Eight children, aged 15 months to 17 years 9 months, maintained by continuous ambulatory peritoneal dialysis (CAPD)/continuous cycling peritoneal dialysis and nine adults, aged 20–59 years, managed by CAPD were compared using a standardized peritoneal dialysis protocol, the peritoneal equilibration test (PET). The peritoncal glucose concentration tended to equilibrate with the serum glucose more rapidly in children, but the percentage of the glucose load absorbed was not different between the two age groups. There was an inverse trend between the percentage of glucose absorbed and age in children. Peritoneal creatinine clearance scaled to surface area in children was significantly less than that of the adults; however, the clearances became similar when adjusted for body weight. Peritoneal creatinine clearace scaled to surface area bore a positive and significant relationship to age which, when expressed per kilogram body weight, disappeared. Children had a significantly higher dialysate/plasma (D.P) creatinine ratio after the first 2 h of the PET, but this ratio approached unity by 4 and was not different from adults. The fractional change in the creatinine D/P ratio during the PET was not different between the two age groups. Drain volume adjusted to surface area was significantly less in children than adults. This difference was reversed when drain volume was factored by weight. Similarly drain volume scaled to surface area demonstrated a significant and positive relationship to age, which disappeared when drain volume was expressed per kilogram body weight. Ultrafiltration, whether factored by weight or scaled to surface area, did not differ between the two groups. The post-PEt residual volume corrected for body weight was significantly larger in the children, but bore no relationship to age. It is possible that this larger residual volume in children functions as a tidal volume enhancing solute equilibration.
- Published
- 1993
41. Chronic renal insufficiency in infants and children
- Author
-
James D. Hanna, James C.M. Chan, and John W. Foreman
- Subjects
medicine.medical_specialty ,Pediatrics ,business.industry ,MEDLINE ,Renal function ,Infant ,Surgery ,03 medical and health sciences ,0302 clinical medicine ,Text mining ,El Niño ,030225 pediatrics ,Child, Preschool ,Pediatrics, Perinatology and Child Health ,Medicine ,Chronic renal insufficiency ,Humans ,Kidney Failure, Chronic ,business ,Child - Published
- 1991
42. Diagnostic workup of renal disorders
- Author
-
Orejas G, John W. Foreman, James C.M. Chan, and Fernando Santos
- Subjects
Diagnostic Imaging ,Groin ,Flank pain ,business.industry ,Nausea ,Periorbital Edema ,Anorexia ,urologic and male genital diseases ,medicine.disease ,Kidney ,Kidney Function Tests ,Uremia ,medicine.anatomical_structure ,Anesthesia ,Edema ,Pediatrics, Perinatology and Child Health ,Vomiting ,Medicine ,Humans ,Kidney Diseases ,medicine.symptom ,business ,Child - Abstract
A careful, thorough history is probably the most important step in the workup of a child with a renal disorder. This includes careful monitoring as to the color, volume, and frequency of the urine output, as well as any pain on voiding or difficulty with initiating or stopping micturition. The presence of flank pain or loin pain radiating to the groin may indicate a renal disorder, as can a history of edema, either peripheral, especially periorbital edema, or pulmonary. Less obvious clues are the symptoms of uremia, which include persistent nausea and/or vomiting, anorexia, lassitude, changes in mental status, headache, and even seizures.
- Published
- 1991
43. Renal Calculi
- Author
-
Mitsuro Nakano, Gad Kainer, John W. Foreman, and James C. M. Chan
- Published
- 1990
44. 64-P: Immune cell response in pediatric kidney transplant recipients
- Author
-
John W. Foreman, Barbara O. Burgess, Dong-Feng Chen, Nancy L. Reinsmoen, and Linda Peel
- Subjects
Nephrology ,medicine.medical_specialty ,Immune system ,business.industry ,Internal medicine ,Immunology ,Immunology and Allergy ,Medicine ,Cell response ,General Medicine ,business ,Kidney transplant - Published
- 2007
45. Cystine and lysine transport in cultured human renal epithelial cells
- Author
-
Beatrice States, Dorothy Harris, John W. Foreman, Stanton Segal, and Judithann Lee
- Subjects
Ornithine ,Arginine ,Endocrinology, Diabetes and Metabolism ,Lysine ,Cystine ,Biology ,Kidney ,Binding, Competitive ,Epithelium ,chemistry.chemical_compound ,Endocrinology ,Humans ,chemistry.chemical_classification ,Sodium ,Kidney metabolism ,Biological Transport ,Amino acid ,Kinetics ,chemistry ,Biochemistry ,Cell culture ,Lysine transport - Abstract
The transport of the amino acids, cystine and lysine, was studied in epithelial cell lines propagated from human kidney cortex. Cystine uptake data were reproducible in different cell lines and did not vary over several cell passages of an individual cell line. The transport of this disulfide amino acid was sodium-dependent with kinetic analysis showing one apparent Kt system of 0.09 mmol/L and Vmax of 0.054 mmol/L cell water/min. Studies of the kinetics of lysine transport, however, revealed two uptake systems with apparent high and low affinities with Kt of 0.14 mmol/L and 5 mmol/L and Vmax of 0.041 and 0.167 mmol/L cell water/min, respectively. Glutamate appeared to be the most potent inhibitor of cystine uptake by these cultured human renal cells and this interaction was competitive. Although cystine did not inhibit lysine uptake, arginine and ornithine were shown to be major inhibitors, thus providing evidence for the presence of a shared dibasic amino acid transport system.
- Published
- 1987
46. Holocarboxylase synthetase deficiency: A biotin-responsive organic acidemia
- Author
-
John W. Foreman, Ronald Rothman, William Yang, Stanton Segal, and Karl S. Roth
- Subjects
medicine.medical_specialty ,Biotin ,Ligases ,chemistry.chemical_compound ,Internal medicine ,medicine ,Humans ,Carbon-Nitrogen Ligases ,Amino Acids ,Renal Aminoacidurias ,Amino Acid Metabolism, Inborn Errors ,Holocarboxylase synthetase deficiency ,chemistry.chemical_classification ,Cultured skin ,business.industry ,Infant, Newborn ,Infant ,Chromatography, Ion Exchange ,medicine.disease ,Pyruvate carboxylase ,Endocrinology ,Enzyme ,chemistry ,Respiratory failure ,Shock (circulatory) ,Organic acidemia ,Pediatrics, Perinatology and Child Health ,medicine.symptom ,Apoproteins ,business - Abstract
The clinical and biochemical features of an infant affected by holocarboxylase synthetase deficiency are presented. The patient was the sibling of the deceased child in whose cultured skin fibroblasts the precise enzymatic disorder was first determined. This fact permitted administration of specific therapy in the form of oral biotin, resulting in immediate improvement from impending respiratory failure and shock. The clinical response to biotin was accompanied by recovery of the biochemical mechanisms known to be biotin-dependent, as manifested by disappearance of intermediates in urine and blood. The variability of biotin responsiveness and the diversity of clinical presentation in the patients originally thought to have a deficiency of beta methylcrotonylCoA carboxylase, a biotin-dependent enzyme, raises the question of a separate, specific apocarboxylase defect.
- Published
- 1980
47. The Effects of Exogenous Rat Growth Hormone Therapy on Growth of Uremic Rats Fed an 8% Protein Diet
- Author
-
Gad Kainer, Mitsuro Nakano, James C.M. Chan, Daijin Ko, and John W. Foreman
- Subjects
medicine.medical_specialty ,Protein diet ,medicine.medical_treatment ,Serum albumin ,Weanling ,Growth ,Biology ,Growth hormone ,chemistry.chemical_compound ,Low-protein diet ,Internal medicine ,medicine ,Animals ,Serum Albumin ,Uremia ,Creatinine ,Body Weight ,Metabolic disorder ,Rats, Inbred Strains ,medicine.disease ,Diet ,Rats ,Disease Models, Animal ,Endocrinology ,chemistry ,Growth Hormone ,Pediatrics, Perinatology and Child Health ,biology.protein - Abstract
Although the mechanisms underlying the inhibitory effects of chronic renal insufficiency on growth are poorly understood, large doses of growth hormone (GH) have been used to improve growth. The present study examines the effects of rat GH and a reduced (8%) protein diet on 75% nephrectomized weanling rats by measuring changes in growth parameters, food utilization, serum albumin concentration, and muscle water content. Significantly greater improvement in growth was found in the GH-treated uremic rats compared with the uremic controls. The mean percent change in wt, length (nose to tail tip), and cranial biparietal diameter was significantly increased in the GH-treated uremic rats, compared with the uremic controls, but foot length and femur length showed only moderate improvement. Food utilization efficiency and serum albumin concentration were significantly higher in GH-treated uremic rats compared with uremic controls, achieving levels that were not different from sham-operated rats. Muscle water content was not significantly different between GH-treated uremic rats, uremic controls, and sham-operated rats. Thus, rat GH treatment administered at an early age in mild renal insufficiency significantly improved overall growth, food efficiency, and serum albumin concentrations, despite a low protein diet, suggesting that further evaluation of this form of therapy for growth failure of uremia is warranted.
- Published
- 1989
48. Nonsurgical management of obstructive aortic thrombosis complicated by renovascular hypertension in the neonate
- Author
-
John W. Foreman, Stephen Baumgart, Henrietta Kotlus Rosenberg, and Seth W. Malin
- Subjects
Male ,medicine.medical_specialty ,Renal Artery Obstruction ,Aortic disease ,Umbilical Arteries ,Renovascular hypertension ,Catheters, Indwelling ,medicine.artery ,medicine ,Humans ,Intensive care medicine ,Aorta ,business.industry ,Infant, Newborn ,Thrombosis ,Umbilical artery ,Aortic Valve Stenosis ,medicine.disease ,Infant newborn ,Surgery ,Hypertension, Renovascular ,Pediatrics, Perinatology and Child Health ,business ,Thrombotic complication ,Aortic thrombosis - Abstract
The use of umbilical artery catheters has become commonplace in the intensive, care nursery and has facilitated management in the very ill newborn infant. Thrombotic complications associated with the use of such catheters may be as high as 30%. The successful, nonsurgical management of either complete or partial aortic thrombosis, associated with renovascular hypertension, is reported in three infants. Aggressive supportive medical management resulted in a satisfactory outcome in all three infants.
- Published
- 1985
49. Cystine-glutamate transport interactions in rat renal cortical tubules, brushborder vesicles, and cultured renal tubule cells
- Author
-
Judithann Lee, John W. Foreman, Claire Rea, Stanton Segal, Pamela D. McNamara, and Margaret Ann Bowring
- Subjects
Male ,Kidney Cortex ,Lysine ,Biophysics ,Cystine ,Glutamic Acid ,Biochemistry ,chemistry.chemical_compound ,Glutamates ,Culture Techniques ,Animals ,Drug Interactions ,Membrane vesicle ,Molecular Biology ,Cells, Cultured ,Renal tubule ,Microvilli ,Chemistry ,Vesicle ,Cell Membrane ,Glutamate receptor ,Biological Transport ,Rats, Inbred Strains ,Cell Biology ,Rats ,Cell biology ,Chemically defined medium ,Kidney Tubules - Abstract
Glutamate had no significant effect on the uptake of 0.025 mM cystine by isolated rat renal cortical tubules and brushborder membrane vesicles in contrast to lysine which significantly inhibits cystine transport. Glutamate, however, markedly inhibited cystine uptake by rat renal tubule cells grown in a serum-free, hormonally defined media for 5 days. Lysine also inhibited cystine transport in these cultured renal tubule cells.
- Published
- 1986
50. Developmental Aspects of Cystine Transport in the Dog
- Author
-
John W. Foreman, Marvin S. Medow, Stanton Segal, and Kenneth C. Bovee
- Subjects
Kidney ,medicine.medical_specialty ,biology ,Reabsorption ,Fissipedia ,Cystine ,Biological Transport ,medicine.disease ,biology.organism_classification ,Excretion ,chemistry.chemical_compound ,Dogs ,medicine.anatomical_structure ,Tubule ,Endocrinology ,Animals, Newborn ,chemistry ,Aminoaciduria ,Internal medicine ,Pediatrics, Perinatology and Child Health ,medicine ,Animals ,Cysteine - Abstract
Increased amino acid excretion is a characteristic of developing rat, dog and man. The mechanisms underlying this physiologic aminoaciduria are unclear. We have shown faster cystine entry into isolated renal tubule cells from newborn rats compared to adults, despite impaired in vivo reabsorption. To see if these observations extend to other animals, we examined the fractional reabsorption (FRc) of cystine in developing dogs and cystine uptake by isolated renal cortical tubule fragments from newborn (NB) and adult dogs. FRc was 67% of the filtered load in five day old dogs but reached the adult value of 99% by 21 days of life. Isolated renal cortical tubules from NB dogs progressively accumulated label when incubated for 60 min. with a physiologic concentration of cystine (0.025 mM), although this uptake was lower than that observed in adults. Nearly complete conversion of transported labelled cystine to cysteine occurred in the NB and adult as previously noted for rat tubules. Kinetic analysis of uptake indicated two systems for cystine entry in both adult and NB. The affinity constants (Km) for the newborn systems were Km1 = 0.08 ± 0.01 and Km2 = 0.33 ± 0.03 mM. The corresponding values for adult were significantly higher - Km1 = 0.14 ± 0.02 and Km2 = 0.66 ± 0.09 mM. The maximal uptake rate for each NB system was only 1/3 that of the adult. These data suggest that, in contrast to the rat, the impaired cystine reabsorption by NB dog kidney in vivo is related in part to a slower flux of cystine into the renal tubule cells.
- Published
- 1986
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