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Objective: As increasing number of young people with renal diseases are reaching adulthood and issues related to their transition to adult units are getting more important. Failure to transfer adolescents under a well-designed transfer program can lead to a decline in attendance to an adult unit. We aim to share our experience and the views of the patients who went through a transition program. Material and Methods: We conducted a telephone questionnaire with 88 patients, who were asked to evaluate their con cerns before their transition and their experience during the visit in the adult nephrology unit after the transition process. Results: The mean age of participants was 19.21±0.97 years. Thirty patients (34%) had chronic kidney disease, and three of them were on dialysis. Three patients (3.4%) had a renal transplant at the time of transition. Fifty-one patients (58%) were felt anxious before the transition process. After their transfer to the adult unit, although 84 patients (95.5%) were satisfied to be in the adult unit, there were still 65 patients (74%) ready to return to the pediatric unit, if it was possible. Conclusion: Transition to the adult unit is still a cause of concern for adolescent patients, even if they are transferred under a well-designed program. It is important to raise awareness about transition programs among pediatricians and to develop a pedi atric-adult transition program for the medical and psychological well-being of patients.

Objective: Classical Bartter syndrome (cBS) and Gitelman syndrome (GS) are genotypically distinct, but there is a phenotypic overlapamong these two diseases, which can complicate the accurate diagnosis without genetic analysis. This study aimed to evaluate thecorrelation between clinical and genetic diagnoses among patients who have genetically defined cBS and GS.Patients and Methods: The study included 18 patients with homozygous/compound heterozygous CLCNKB (NM_000085) (n:10/18)and SLC12A3 (NM_000339) (n:8/18) mutations. Biochemical, clinical and radiological data were collected at presentation and at thelast visit.Results: In cBS group age at diagnosis, median plasma potassium and chloride concentrations were significantly lower and medianplasma HCO3 and blood pH values were significantly higher. Patients with GS had significantly lower median plasma magnesiumconcentrations and urinary calcium/creatinine ratio. One child with GS had normocalciuria, two children with cBS had hypocalciuriaand hypomagnesemia. Low estimated glomerular filtration rate (eGFR) (ml/dk/1.73m2) and growth failure were more evident in cBSgroup. In patients with cBS, nine different CLCNKB gene mutations were detected, five of them were novel. Novel mutations were:one nonsense (c.66G>A, p.Trp22*), one missense (c.499G>A, p.Gly167Ser) and three splice-site (c.867-2delA; c.499-2insG; c.1930-2A>C) mutations. In patients with GS, six different SLC12A3 gene mutations were found.Conclusions: It may not always be possible to clinically distinguish cBS from GS. We suggest to perform a genotypic classification ifgenetic analysis is possible.

Background Cubilin is one of the receptor proteins responsible for reabsorption of albumin in proximal tubules and is encoded by the CUBN gene. We aimed to evaluate clinical and genetic characterization of six patients with proteinuria who had CUBN mutations. Methods Patients’ characteristics, serum creatinine, albumin, vitamin B12 levels, urine analysis, spot urine protein/creatinine, microalbumin/creatinine, beta-2 microglobulin/creatinine ratios, estimated glomerular fltration rates (eGFR), treatments, kidney biopsies, and genetic analyses were evaluated. Results Six patients (2 female, 4 male) with an incidental finding of proteinuria were evaluated. Mean admission age and follow-up time were 7.3 ± 2.9 and 6.5 ± 5.6 years, respectively. Serum albumin, creatinine, and eGFR were normal; urine analysis revealed no hematuria, and C3, C4, ANA, and anti-DNA were negative; kidney ultrasonography was normal for all patients. Urine protein/creatinine was 0.9± 0.3 mg/mg, and microalbumin was high in all patients. Serum vitamin B12 was low in two patients and normal in four. Kidney biopsy was performed in four patients, three demonstrated normal light microscopy, and there was one focal segmental glomerulosclerosis (FSGS). Genetic tests revealed four homozygous and two compound heterozygous mutations in the C-terminal part of cubilin. All patients had normal eGFR and still had non-nephrotic range proteinuria at last visit. Conclusions CUBN gene mutations should be considered in patients with isolated non-nephrotic range proteinuria and normal kidney function. Diagnosing these patients, who are thought to have a better prognosis, is important in terms of avoiding unnecessary treatment and predicting prognosis. CUBN gene mutations may also present as FSGS which extends the spectrum of renal manifestation of these patients.

C3 glomerulopathy (C3G) is characterized by heterogeneous clinical presentation, outcome, and predominant C3 accumulation in glomeruli without significant IgG. There is scarce outcome data regarding childhood C3G. We describe clinical and pathological features, treatment and outcomes, and risk factors for progression to chronic kidney disease stage 5 (CKD5) in the largest pediatric series with biopsy-proven C3G. Sixty pediatric patients with C3G from 21 referral centers in Turkey were included in this retrospective study. Patients were categorized according to CKD stage at last visit as CKD5 or non-CKD5. Demographic data, clinicopathologic findings, treatment, and outcome data were compared and possible risk factors for CKD5 progression determined using Cox proportional hazards model. Mean age at diagnosis was 10.6 ± 3.0 years and follow-up time 48.3 ± 36.3 months. Almost half the patients had gross hematuria and hypertension at diagnosis. Nephritic-nephrotic syndrome was the commonest presenting feature (41.6%) and 1/5 of patients presented with nephrotic syndrome. Membranoproliferative glomerulonephritis was the leading injury pattern, while 40 patients had only C3 staining. Patients with DDD had significantly lower baseline serum albumin compared with C3GN. Eighteen patients received eculizumab. Clinical remission was achieved in 68.3%. At last follow-up, 10 patients (16.6%) developed CKD5: they had lower baseline eGFR and albumin and higher frequency of nephrotic syndrome and dialysis requirement than non-CKD5 patients. Lower serum albumin and eGFR at diagnosis were independent predictors for CKD5 development. Children with C3G who have impaired kidney function and hypoalbuminemia at diagnosis should be carefully monitored for risk of progression to CKD5. Graphical abstract

Background: Nephrotic Syndrome (NS) is one of the most common renal diseases in children. Clinical response to corticosteroids is an important prognostic factor for long-term renal outcome. We aimed to evaluate the clinical parameters, histopathological findings and complications of NS patients and factors predicting steroid resistance in a single tertiary center. Methods: A hundred and sixty two children (57 girls, 105 boys) with NS followed between 1998 and 2018 in Department of Pediatric Nephrology were analyzed in a retrospective cohort. Secondary causes and infantile NS cases were excluded. Results: The median (IQR; range) age of the children at presentation and follow up time were 4.9(5.7; 0.1-16.8) and 5.5(5.4; 0.1-20.3) years, respectively. A hundred and thirty four (82.7%) patients were steroid-sensitive nephrotic syndrome (SSNS) and 28(17.3%) were steroid-resistant nephrotic syndrome (SRNS). Age at the first presentation was lower in SSNS group (p=0.002). Hypertension, macroscopic and microscopic hematuria were higher in SRNS group (pConclusion:The late onset is still an important sign for SRNS. Microscopic and macroscopic hematuria and hypertension may also give a hint for potential steroid resistance. We emphasize that FSGS is a biopsy finding that may reveal to different diagnosis in SRNS.

Objective: It has been aimed to report the experience of our center regarding diagnosis and treatment experience in children with Nutcracker syndrome (NCS).Materials and Methods: The medical records of the seven patients who have admitted to the Department of Pediatric Nephrology of our hospital between February 2017 and March 2020 were evaluated retrospectively. The diagnosis of NCS was confirmed with renal Doppler ultrasound (RDUS) and magnetic resonance angiography (MRA) in these patients who have admitted with the complaints of hematuria and proteinuria. The patient data such as clinical characteristics, radiological findings, radiological signs and information about the applied medical treatment at baseline and the last control examination were recorded. Results: The mean levels of 24-h urine protein excretion in all the patients at baseline and the last control examination were 15,25±9,19 mg/m2/h and 9,8±3,94 mg/m2/h, respectively. The mean levels of 24-h urine protein excretion in the patients treated with ACE (angiotensin converting enzyme) inhibitor at baseline and the last control examination were 20±11,53 mg/m2/h and 9,6±6,44 mg/m2/h, respectively. (p=0,073). The mean levels of 24-h urine protein excretion were 11.7±6.39 mg/m2/h and 9.95±1.84 mg/m2/h in the patients not receiving ACE inhibitor treatment, at baseline and the last control examination respectively (p= 0,61). The mean angle value of the left renal vein in the aortomesenteric distance measured by RDUS examination performed in the upright position was 14,71±4,46 degrees. The anteroposterior diameter of the left renal vein (hilar/aortomesenteric) measured in the upright position was 6,4. Conclusion: The benign nature of NCS in young patients requires maintaining conservative approach.

Renal abscess, the accumulation of infected fluid in the kidney, is a rare condition seen in children as well as adults. It leads to long term hospital admission and antibiotic use. Early diagnosis is an important factor in the outcome of renal abscess because the management may differ. Urinalysis test results and radiologic imaging findings of the patients who are admitted to hospital with complaints of fever, vomiting, abdominal pain or flank pain are important for the early diagnosis. Undertreated cases have high risk for renal scar formation. In this paper, we aim to present three pediatric patients, who showed the complication of renal abscess and were treated with long term antibiotic use without a need for surgical drainage.

Tam Metin / Full Text Q4 PMID: 34174796 WOS:000724833600003 SCI-Expanded Nephrocalcinosis (NC) is defined as calcium deposition in the kidney parenchyma and tubules. This study aims to determine the etiology, risk factors, and follow-up results of patients with NC in Turkey. Materials and methods: Patients diagnosed with NC in the pediatric nephrology Department Units of 19 centers from all geographical regions of Turkey over a 10-year period (2010–2019) were included in the study. The medical records from the centers were reviewed and demographic data, admission complaints, medical history, systemic and genetic disorders, risk factors for NC, treatment details, and presence of NC after one-year follow-up, were recorded retrospectively. Results: The study sample included 195 patients (88 females, 107 males). The mean age at diagnosis was 39.44 ± 47.25 (0.5–208) months; 82/190 patients (43.2%) were diagnosed incidentally; 46/195 patients (23.6%) had an underlying disease; idiopathic hypercalciuria was detected in 75/195 (38.4%) patients. The most common systemic diseases were distal renal tubular acidosis in 11/46 patients (23.9%), primary hyperoxaluria in 9/46 patients (19.6%) and Bartter syndrome in 7/46 patients (15.3%). After one year of follow-up, NC resolved in 56/159 patients (35.2%) and they all did not have an underlying systemic disease. Conclusion: The most common presentation of NC was incidental. Distal renal tubular acidosis and primary hyperoxaluria were the main systemic diseases leading to NC, while hypercalciuria was the most common metabolic risk factor. Nephrocalcinosis was found to remain in most of the patients at a one-year follow-up. It may resolve particularly in patients with no underlying systemic disease. Keywords: Bartter syndrome, hypercalciuria, nephrocalcinosis, renal tubular acidosis

We aimed to present five patients presenting with hypertension and diagnosed as renal artery stenosis. Five patients with renal artery stenosis were studied retrospectively. Two of our cases were diagnosed as fibromuscular dysplasia, other cases were diagnosed as Takayasu arteritis and Moyamoya disease, and one of them had congenital vascular abnormality. Renovascular hypertension is one of the most important causes of secondary hypertension in children, and renal artery stenosis constitutes a significant proportion of renovascular disease. We must consider renal artery stenosis in children presenting with resistant hypertension.

Objective: Renal cystic diseases consist of a broad spectrum of hereditary or acquired conditions that may lead to end stage renal disease. We aimed to evaluate our patients diagnosed as renal cystic disease in terms of their diagnosis, demographic findings and clinical follow-up. Materials and Methods: The patients followed between 1993-2015 in our pediatric nephrology outpatient department with renal cystic diseases were evaluated retrospectively. Results: In 237 patients, 110 (46.41%) were female, 127 (53.59%) were male. One hundred-eight (45.56%) patients were diagnosed antenatally, the mean age at diagnosis was 7.23 +/- 4.72 (0-17) years in 129 patients. The diagnosis were simple-cyst in 36 (15.18%), multicystic displastic kidney disease in 112 (47.25%), autosomal dominant polycystic kidney disease in 56 (23.62%), autosomal recessive polycystic kidney disease in 22 (9.28%), cyst hydatic in three (1.26%), Joubert sydrome in two, nephronophthisis in one, tuberosclerosis in two, Bardet-Biedl syndrome in three patients. Five patients (2.1%) died and ten (4.21%) patients progressed to chronic kidney injury. Proteinuria was found in 15 (6.32 %) and hypertension in 10 (4.21%) patients. Conclusion: Renal cystic disease is an important group that can lead to proteinuria, hypertension and end stage kidney failure. Periodic follow-up is important in these patients to avoid and treat the complications early and properly.

WOS:000357358000012 PubMed: 26761851 This study was designed to determine the usability of lemon fiber (LF-2%, 4%, 6%) and carrot fiber (CF-2%, 4%, 6%) to produce low-fat beef hamburgers. To that end, a certain amount of fat was replaced with each fiber. The proximate composition, pH value, cholesterol content, cooking characteristics, color, texture profile, and sensory properties of low-fat beef hamburgers were investigated. LF increased moisture content and cooking yield due to its better water binding properties, while CF caused higher fat and cholesterol contents owing to its higher fat absorption capacity (p

Invasive aspergillosis caused by A. Fumigatus, almost occurs in immunocompromised hosts and has a poor prognosis. We report a case of invasive Aspergillosis in a 15-year-old boy with ESRD. He was initially diagnosed as lobar pneumonia and peritonitis. When he complained for lower extremity weakness and had convulsions, a solid mass originated from right lung compresses the spinal cord and intracranial hemorrhagic abscesses were found on MRI. The biopsy specimen showed hypae of aspergillus-spp and he died on 12th day. Keywords: Aspergillosis, End stage renal disease, Peritoneal dialysis

Background: The causative agent spectrum and resistance patterns of urinary tract infections in children are affected by many factors. Aims: To demonstrate antibiotic resistance in urinary tract infections and changing ratio in antibiotic resistance by years. Study Design: Retrospective cross-sectional study. Methods: We analysed antibiotic resistance patterns of isolated Gram (-) bacteria during the years 2011-2014 (study period 2) in children with urinary tract infections. We compared these findings with data collected in the same centre in 2001-2003 (study period 1). Results: Four hundred and sixty-five uncomplicated community-acquired Gram (-) urinary tract infections were analysed from 2001-2003 and 400 from 2011-2014. Sixty-one percent of patients were female (1.5 girls : 1 boy). The mean age of children included in the study was 3 years and 9 months. Escherichia coli was the predominant bacteria isolated during both periods of the study (60% in study period 1 and 73% in study period 2). Bacteria other than E. coli demonstrated a higher level of resistance to all of the antimicrobials except trimethoprim-sulfamethoxazole than E. coli bacteria during the years 2011-2014. In our study, we found increasing resistance trends of urinary pathogens for cefixime (from 1% to 15%, p0.05). Conclusion: In childhood urinary tract infections, antibiotic resistance should be evaluated periodically and empiric antimicrobial therapy should be decided according to antibiotic sensitivity results.

Objectives. Procalcitonin is a propeptide ofcalcitonin and has been increasingly used as a biomarker of infection. The aimof this study was to evaluate correlation of serum C-reactive protein (CRP) andprocalcitonin (PCT) levels of kidney transplant patients hospitalized due toinfection. Methods. There were 121 patients who had kidney transplant inour center between September 2012 and February 2017 and patients with adiagnosis of infection or rejection were included in the study. Simultaneous106 serum CRP and PCT levels at the beginning or during any time of treatmentfor post-transplant infection, cytomegalovirus (CMV) positivity, BK viremia andrejection were evaluated. Results. Median andinterquartile ranges of CRP and PCT serum levels were 40 mg/l [24.7-64.9] and 0.19ng/ml [0.1-0.61], respectively. A significant positive correlation betweenserum CRP and PCT levels of the patients were observed (r=0.490, p100 mg/l, correlations with serumPCT levels were as r=0.461 (p100 mg/l did not show a statistically significant correlation.Stability of PCT levels in viral infections and rejections might be anadvantage for the follow-up of solid organ transplants. We need prospectivetrials of PCT measurements for the evaluation of post-transplant infections.

Amaç: Bu çalışmanın amacı, ülkemizde 1975 yılında uygulamaya geçen ve hastanemizde ancak 2012 yılında başlatılan böbrek nakli programının sonuçlarını retrospektif olarak değerlendirmektir. Yöntemler: Eylül 2012-Aralık 2016 tarihleri arasında Organ Nakli Merkezimizde böbrek transplantasyonu uygulanan 115 hasta demografik veriler, immunsüpresif protokolü, rejeksiyon durumu, greft kaybı, hasta kaybı, postoperatif cerrahi komplikasyonlar, BK viremi (BKV) ve nefropatisi (BKN), cytomegalovirus (CMV) infeksiyonu, tüberküloz (TBC) ve posttransplant malignensi açısından değerlendirildi. Bulgular: On iki olguya (%10,4) kadavradan, 103 olguya (%89,6) canlı donörden böbrek transplantasyonu uygulandı. Alıcıların ve donörlerin ortalama yaşı sırası ile 34.65 (12-68) ve 42 (23-72) idi. Erkek/Kadın oranı alıcı ve donörlerde sırası ile 65/50 ve 48/67 idi. Postoperatif median ve çeyreklerarası aralık (ÇAA) taburculuk kreatini 1.03 [0.55-1.48] mg/dl idi. Yedi (%6) olgu akut rejeksiyon tanısı aldı. Dört olguda (%3,4) greft kaybı gözlendi. Mortalite gözlenmedi. Sonuç: Böbrek transplantasyonu programımız gelişim aşamasındadır. Ancak kısa dönem sonuçlar literatür ile uyumlu görünmektedir. Uzun süreli takip sonuçları ile daha anlamlı sonuçlar elde edilebilir. Objectives: The aim of this study is to retrospectively evaluate the results of the transplantation program that started in 2012 in our hospital. Methods: We examined the demographic variables, immunosuppressive protocols, rejection rates, graft survival, mortality, postoperative surgical complications, BK viremia (BKV) and BK virus-assoiciated nephropathy (BKN), cytomegaloviruse (CMV) infection rates, tuberculosis (TBC) rates and posttransplant malignancy rates of 115 patients who received kidney transplant in our transplantation center between September 2012 and December 2016. Results: Twelve (10.4%) patients received kidney from cadaver donors and 103 (89.6%) paients received kidney from living donors. The Mean donor age was 42 (23-72) and the mean recipient age was 34.65 (12-68). Male to female ratio was 65/50 for the recipients and 48/67 for the donors. Median discharge creatinine was 1.03 mg/dl [interquartile range=0.55-1.48]. Seven patients (6%) suffered from acute rejection and graft loses occurred in four (3.4%) patients. There was no mortality. Conclusion: Our kidney transplantation program is in the developmental stage. However, short-term outcomes are consistent with the literature. More meaningful results can be obtained with long-term follow-up results.

Subacute sclerosing panencephalitis (SSPE) is a chronic encephalitis of childhood and young adolescence due to persistant measles virus infection of the central nervous system. In majority of cases onset occurs from 5–15 years of age.1 In a nonimmunized population the average onset is 8 years. Children with SSPE had experienced natural infection with the rubeola virus at an early age, half before age 2 years.2 SSPE generally occurs 5–10 years after measles infection. In the early stages of the disease behavioral and personality changes is followed by myoclonic jerks and convulsions. In late stages dementia, stupor and coma develops. Diagnosis is achieved by typical clinical findings, meales antibody titer increase in cerebrospinal fluid (CSF) and serum, high amplitude, slow, sharp waves in EEG. Prognosis is poor and death ensues in about 3 yr after the diagnosis. Here it is presented a 7-years-old boy with involuntary movements in both hands, drop attacks while walking, ataxia and stupor. Due to suggestive radiological and clinical findings and a history of recent mumps infection he was thought to have acute disseminated encephalomyelitis initially and given treatment. But due to clinical deterioration and detection of anti measles IgG in serum, and CSF, SSPE diagnosis was confirmed. With this SSPE case presenting initially as ADEM, the authors tried to emphasize that presentation of SSPE may clinically and radiologically beddiverse and a thorough differential diagnosis is mandatory for a definite diagnosis.

We proposed and demonstrated architectural tuning of color chromaticity by controlling photoluminescence decay kinetics through nonradiative Förster resonance energy transfer in the heterostructure of layer-by-layer spaced CdTe nanocrystal (NC) solids. We achieved highly sensitive tuning by precisely adjusting the energy transfer efficiency from donor NCs to acceptor NCs via controlling interspacing between them at the nanoscale. By modifying decay lifetimes of donors from 12.05 to 2.96 ns and acceptors from 3.68 to 14.57 ns, we fine-tuned chromaticity coordinates from (x,y) = (0.575,0.424) to (0.632, 0.367). This structural adjustment enabled a postsynthesis color tuning capability, alternative or additive to using the size, shape, and composition of NCs. © 2009 American Institute of Physics.

In recent years, precisely tuning shades of white across color chromaticity diagram has become critically important in solid state lighting, especially to achieve application specific spectrally tuned illumination [1]. In this respect, semiconductor quantum dot nanocrystals (NCs) have attracted significant attention for their use as luminophors in light-emitting diodes (LEDs). This is mainly because it is possible to conveniently control the electronic structure of these nanocrystals by adjusting their size, shape, and composition [2]. However, such tuning is only limited to the process of nanocrystal synthesis in which the size, shape, and composition are controlled. Here we propose and demonstrate an alternative, post-synthesis, architectural approach to tune the color of nanocrystal emitters by controlling their radiative decay kinetics and steady state spontaneous emission using nonradiative Forster resonance energy transfer (FRET). For the proof-of-concept demonstration of such FRET-controlled tuning, we constructed nanocrystal solids that are layer by layer assembled with interlayer spacings between them precisely controlled at the nanoscale [3].

We present light harvesting of aqueous colloidal quantum dots to nonradiatively transfer their excitonic excitation energy efficiently to dye molecules in water, without requiring ligand exchange. These as-synthesized CdTe quantum dots that are used as donors to serve as light-harvesting antennas are carefully optimized to match the electronic structure of Rhodamine B molecules used as acceptors for light harvesting in aqueous medium. By varying the acceptor to donor concentration ratio, we measure the light harvesting factor, along with substantial lifetime modifications of these water-soluble quantum dots, from 25.3 ns to 7.2 ns as a result of their energy transfer with efficiency levels up to 86%. Such nonradiative energy transfer mediated light harvesting in aqueous medium holds great promise for future quantum dot multiplexed dye biodetection systems. (C) 2010 Optical Society of America.

Sekonder psödohipoaldosteronizm (PHA), böbrek tübüllerininaldosterona duyarsızlığı ile karakterize, hiperkalemi,hiponatremi ve metabolik asidoz ile kendini gösteren nadirbir durumdur. Obstrüktif üropatisi olan bebeklerde vepiyelonefrit sırasında görülebilir. Gelişimi devam eden erkenbebeklik dönemi böbreği, üriner sistemin obstrüktif anomalisive enfeksiyonu sekonder PHA hastalarında tübüluslardaaldosteron direncine neden olan üç önemli faktördür. Genelliklehiponatremi, hiperkalemi ve metabolik asidoz ilk bulgulardır,tetkiklerde belirgin olarak yükselmiş plazma renin ve aldosteronseviyelerinin saptanması ile tanı konur. Süt çoçuklarındabiyokimyasal incelemede hiperpotasemi, hiponatremi vemetabolik asidoz saptandığında konjenital adrenal hiperplaziayırıcı tanısında PHA göz önünde bulundurulmalı ve buhastalarda üriner ultrasonografisi (USG) ve idrar analizi rutinolarak yapılmalıdır. Bu yazımızda sekonder PHA gelişenposterior üretral valv (PUV)‘lı iki hasta sunuldu., Secondary pseudohypoaldosteronism (PHA) is a rare conditionthat presents with hyperkalemia, hyponatremia, and metabolicacidosis, which develops due to renal tubular unresponsivenessto aldosterone. It may be seen in infants with obstructiveuropathy and acute pyelonephritis. Three important reasons ofthe aldosterone unresponsiveness of the tubules are early infancykidney, obstructive uropathy, and urinary tract infection. Besidesother findings, diagnosis is established with highly increasedplasma aldosterone and renin levels. In infants who present withhyperkalemia, hyponatremia, and metabolic acidosis, PHA shouldbe considered in the differential diagnosis of congenital adrenalhyperplasia. Urinary ultrasonography and urine analysis should beperformed in these patients. Herein, we present two patients withsecondary PHA caused by posterior urethral valves.