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Neisseria mucosa is part of the normal nasopharyngeal flora and rarely pathogenic in humans. Reports of serious infections associated with this pathogen are very unusual. A 17-year-old boy with end-stage renal disease due to IgA nephropathy presented with acute, spontaneous, symptomatic peritoneal dialysis-associated peritonitis without reported break in sterility or PD catheter exit site infection. beta-lactamase-negative N. mucosa was isolated from the dialysate effluent. Intraperitoneal antibiotic treatment with cephalothin/gentamicin for 5 days and subsequent ceftriaxone led to complete resolution of the infection. This case demonstrates that "non-pathogenic" Neisseria species can cause clinically severe peritonitis with high intraperitoneal neutrophil counts, elevated C-reactive protein levels in the peritoneal effluent (in the presented case, 27,600/mul and 3.6 mg/l, respectively) and impaired peritoneal membrane transport function. To our knowledge, this is the first case of N. mucosa peritonitis complicating chronic peritoneal dialysis in an adolescent patient.

Purpose: Retrospective review of authors' experience with percutaneous transcatheter renal ablation in patients with uncontrolled hypertension and/or nephrotic syndrome., Materials and Methods: Between April 1987 and September 1995, renal ablation was performed on 11 patients aged 10 months to 21 years. All patients had end-stage renal disease (ESRD) with uncontrolled hypertension (10 patients) and/or nephrotic syndrome (four patients). Uncontrolled hypertension was defined as diastolic pressure greater than 90 mm Hg despite multidrug antihypertensive therapy. Nephrotic syndrome was defined as proteinuria exceeding 960 mg/m2 per day, serum albumin level less than 3 g/dL, and generalized edema. Embolization was performed with absolute ethanol from a common femoral artery approach. In most cases, a balloon catheter was used to prevent alcohol reflux into the aorta or nontarget renal artery branches, such as the adrenal arteries. Angiographic stasis of contrast material in the renal arteries was the endpoint., Results: All patients experienced a postembolization syndrome of 3-5 days duration, clinically manifested by variable degrees of nausea, vomiting, fever, and pain. Long-term improvement in hypertension was observed in nine patients. Improvement in hypertension was defined as diastolic blood pressure below 90 mm Hg while the patient received the same or fewer antihypertensive medications. The four patients with nephrotic syndrome were cured of their proteinuria and edema., Conclusions: Transarterial renal ablation with alcohol is efficacious for treatment of uncontrolled hypertension and nephrotic syndrome in patients with ESRD. The morbidity and mortality in our series were less than those reported for surgical nephrectomy.

Objective: We hypothesized that the absolute value of an immature lecithin/sphingomyelin ratio, in addition to gestational age and birth weight, contributes significantly to predicting the clinical course of the neonate. The lecithin/sphingomyelin value may therefore enhance clinical decision-making regarding timing of delivery and use of tocolytics in high-risk pregnancies., Study Design: One hundred four mother-baby pairs with delivery within 72 hours of an immature lecithin/sphingomyelin determination in the 3-year period immediately before the initiation of surfactant therapy in our hospital were retrospectively reviewed. Stepwise regression was performed for the independent variables of gestational age, birth weight, and lecithin/sphingomyelin values in a linear model to predict total days of respiratory support (oxygen, continuous positive airway pressure, or mechanical ventilation)., Results: In the patients without preterm premature rupture of membranes the lecithin/sphingomyelin value was the best predictor of duration of respiratory support (R2 = 0.2426, F = 12.4908, p = 0.011). After gestational age was controlled for, there was a significant inverse correlation between the lecithin/sphingomyelin value and days of respiratory support (F = 4.634, p = 0.031). In the patients with preterm premature rupture of membranes with vaginally collected fluid, however, lecithin/sphingomyelin values did not contribute significantly in predicting duration of respiratory support. Rupture-to-delivery interval and gestational age were the best predictors in this group., Conclusion: Although a mature lung profile is the ideal situation, preterm delivery may be indicated in pregnancies complicated by maternal disease or evidence of possible fetal compromise. We conclude that in patients without premature rupture of membranes the absolute value of the immature lecithin/sphingomyelin ratio is a better predictor than gestational age or birth weight of duration of respiratory support and should be considered in timing of delivery.

We report on 15 children with proteinuria, at the nephrotic level in the majority of cases, who had no histologic glomerular alterations (eight cases), or focal and segmental glomerular scarring with (three cases) or without (four cases) mesangial proliferation. In all cases, immunofluorescence (IF) microscopy showed prominent mesangial C1q deposits with variable amounts of immunoglobulins. Ultrastructurally, most had conspicuous mesangial electron-dense deposits. Cases with no glomerular histologic alterations were histologically indistinguishable from minimal change disease (MCD), yet they uniformly had an unsatisfactory response to oral prednisone. Thus, the presence of immune deposits with a prominent C1q contribution identifies a group of cases that respond poorly to steroids and that, if light microscopy is considered in isolation, might otherwise be designated MCD.

The murine monoclonal antibody muromonab CD3 is currently used to reverse acute renal graft rejection. We report a case of systemic anaphylaxis during a muromonab CD3 infusion despite pretreatment with systemic antihistamines and corticosteroids. Rapid intravenous desensitization was performed the following day without untoward reactions and daily muromonab CD3 infusions were successful in reversing renal graft rejection. A second rapid desensitization to CD3 was performed 1 month later without any complications. Serum muromonab CD3-specific IgG and IgE antibodies were detected in serum samples obtained after the anaphylactic reaction. The anaphylactic reaction to muromonoab CD3 monoclonal antibody could have been due to allergen-specific antibodies noted in postreaction serum or a cross-reactive antibody to mouse antigens or both. More importantly, this case illustrates that rapid desensitization can be performed successfully without serious complications; therefore, systemic anaphylaxis can develop in susceptible atopic individuals receiving muromonab CD3 monoclonal antibody for renal graft rejection.

The cases of two brothers with sickle-cell anemia complicated by the nephrotic syndrome and membranoproliferative glomerulonephritis are presented. The literature related to this infrequent association is reviewed and possible explanations for the occurrence of the latter in two brothers are discussed.

Acute renal failure developed in a 3-year-old boy with acute pyelonephritis. Renal biopsy showed acute interstitial infiltration of neutrophils and macrophages. There were also glomerulitis and capillary tuft thrombosis. He required peritoneal dialysis, but subsequently recovered renal function. Prompt antimicrobial therapy is crucial to insure a favorable outcome. Pyelonephritis is an unusual cause of acute renal failure in infants and children.

The effect of adenosine-3',5'-cyclic monophosphate (cyclic AMP) and N(6),O(2)-dibutyryl adenosine-3',5'-cyclic monophosphate (dibutyryl cyclic AMP) on renal tubular permeability was studied by microinjection techniques in anesthetized diuretic rats. Radioactive inulin and mannitol were microinjected simultaneously into superficial proximal and distal convolutions and recovery of the isotopes was measured in the urine.During control conditions, mannitol and inulin recovery was essentially complete. However, during infusion of cyclic AMP or dibutyryl cyclic AMP, mannitol recovery was significantly less than control after early proximal and late proximal microinjections, averaging 79 and 85%, respectively. There was no loss of mannitol from the nephron after microinjection into distal convolutions. Inulin recovery was complete after all microinjections during cyclic AMP or dibutyryl cyclic AMP infusion. Simultaneous clearances of mannitol and inulin as well as peritubular capillary microinjections studies demonstrated bidirectional fluxes of mannitol across the proximal tubular epithelium during infusion of cyclic AMP or dibutyryl cyclic AMP. Intratubular pressures were not different during control and experimental periods. These studies demonstrate a change in the permeability characteristic of the proximal convoluted tubule during infusion of cyclic AMP and dibutyryl cyclic AMP. This change in permeability of the proximal tubule could account for the effects of cyclic AMP on proximal tubular transport processes.

Microinjection experiments were performed in anesthetized rats to study the tubular absorptive capacity for glucose (TG) in normal and chronically altered tubules, i.e., tubules that sustained anatomical repair and compensatory changes of their previously normal configuration after acute damage. TG was complete when the glucose load injected in normal or altered early or late proximal convolutions was less than or equal to 1.5 or less than or equal to 10 pmol . s-1, respectively. In normal tubules, TmG was 10 and 5 pmol . s-1 after early and late proximal microinjections, respectively. After early proximal microinjections in altered tubules, different levels of TmG were found. They related to the degree of compensatory growth. TmG was approximately 12, 24, or 35 pmol . s-1, respectively, in altered tubules with minor, moderate, or major compensatory growth. After late proximal microinjections, TmG was 15 pmol . s-1. TG variability was greater in altered than in normal tubules. Correlation between structural and functional compensatory changes was thus demonstrated. Furthermore, an important, albeit latent, absorptive capacity for glucose of the proximal straight tubule was found.

After using a cellular digestion technique to extract cells from the basement membranes of frozen kidney tissue, we employed scanning electron microscopy to examine the acellular glomerular basement membranes (AGBM) from normal kidneys and from kidneys of patients with idiopathic membranous glomerulopathy (MGN). This method revealed, in the AGBM, previously unrecognized three-dimensional patterns of pathologic changes. These patterns correlated with increasing MGN stage as defined by Ehrenreich and Churg. On the epimembranous AGBM surface these patterns were composed of ridge-like trabeculae, irregular plaques, and reticulated crater-like deformities. The endothelial AGBM surfaces were smooth in stages I and II MGN, but in stage III MGN the endothelial surfaces were irregular and perforated. In contrast to lupus-related MGN, where some immune-complex-like material remained after cellular extraction, epimembranous immune-complex-like material in idiopathic MGN was extracted.

After digestion removed the cells from glomeruli of frozen kidney tissue, we employed scanning electron microscopy to examine the acellular glomerular basement membranes (AGBM) from normal kidneys and from kidneys of patients with dense-deposit disease (DDD). The AGBM showed previously unrecognized three-dimensional patterns of pathologic changes. When compared to normal controls, the AGBM in DDD appeared "rigid" and thickened. Other pathologic features included coarsely granular or undulating epimembranous surfaces punctuated by single or clustered crater-like deformities. Although epimembranous crater-like deformities have been observed in other glomerulopathies, the combination of "rigid"-appearing AGBM punctuated by crater-like deformities is thus far unique to DDD.

A case of medullary type of nephrocalcinosis in hypophosphatasia is presented. Sonography revealed medullary calcification not visible on abdominal radiography. Calcification could also be demonstrated by CT scanning.

Three children presented with proteinuria, microscopic hematuria, episodic gross hematuria and normal renal function. Renal histology consisted predominantly of mesangial IgA deposition. The above clinical and biopsy findings are consistent with a diagnosis of IgA nephropathy. To date, these 3 children have maintained normal renal function. The authors hypothesize that this may be due to the absence of simultaneous complement deposition within the glomerulus.

We have recently trained two sets of parents to perform home peritoneal dialysis on their infants with chronic renal failure. Chronic dialysis was initiated before the age of one year with a body weight of less than 10 kg. The infants were maintained on dialysis for 10 and 13 months respectively awaiting cadaveric transplantation. Both infants had marked growth failure while on chronic home dialysis. Although one infant had three episodes of peritonitis, the other had none during 10 months of dialysis. Chronic home peritoneal dialysis was well tolerated in both infants.

A 5-year-old girl had hypoglycemia and was of short stature. Studies of pituitary function demonstrated combined growth hormone and adrenocorticotropic hormone (ACTH) deficiency. She was shown to have ketotic hypoglycemia. In contrast to patients previously reported with hypopituitarism and ketotic hypoglycemia, she had no deficiency of gluconeogenic substrate. Serum levels of alanine and other gluconeogenic amino acids were normal during fasting and hypoglycemia. These studies suggest that inadequate gluconeogenic precursors are not the cause of her ketotic hypoglycemia. Ketotic hypoglycemia in association with hypopituitarism may be secondary to multiple biochemical defects.

After biochemical and radiographic studies, enzyme replacement therapy in three patients with the infantile form of hypophosphatasia was attempted by weekly intravenous infusions of bone alkaline phosphatase-rich (BAP) plasma from patients with Paget bone disease. Subsequently, circulating BAP activity was substantially increased in each patient, and in one was maintained in the normal range for nearly 2 months. Despite partial or complete correction of the deficiency of circulating BAP activity, we observed no radiographic evidence for arrest of progressive osteopenia or improvement in rachitic defects in any of the patients. Failure of infants with hypophosphatasia to show significant healing of rickets on correction of circulating BAP activity supports the hypothesis that this isoenzyme functions in situ during normal skeletal mineralization.

Urinary lactic dehydrogenase (U-LDH) isoenzyme assays were performed on ch2) as well as normal controls (N = 24). Docuemntation of bladder and kidney infection was accomplished by means of the bladder washout test, culture of ureteric urine (in patients with urinary diversion), kidney function studies including the maximal urine concentration test, clinical symptomatology and radiologic appearance of the urinary tract. Total U-LDH in normal children (10.8 +/- 1 mU/ml) was lower than in patients with bladder (27.0 +/- 3.9 mU/ml) or kidney (226 +/- 67.3 mU/ml) infections (P less than 0.005). In normal children isoenzymes 1 and 2 predominated (LDH-1 migrates fastest to anode -- fast zone pattern). In patients with bladder infections, the isoenzyme patterns varied but the concentration of isoenzyme 5 (3.1 +/- 0.8 mU/ml) was lower (P less than 0.005) than in patients with kidney infections (120 +/- 39 mU/ml). In the latter, isoenzymes 4 and 5 predominated (slow zone pattern). Since overlap between kidney and bladder infections regarding isoenzyme 5 concentrations (at 3 SD) occurred in only one individual (patient 37), a correct differential diagnosis using U-LDH-5 alone would have been possible in 94% of the children with pyelonephritis or 97% of the total patient population (kidney + bladder).

The authors have evaluated urinary adenosine 3',5'-monophosphate (cyclic AMP) excretion and renal function during Pitressin administration, hypertonic saline administration, and water deprivation in two siblings with vasopressin-resistant diabetes insipidus and in normal control subjects. After vasopressin administration normal subjects experienced a 2-fold rise in urinary cyclic AMP excretion from 3.2 +/- 0.7 to 5.6 +/- 1.3 nmol/min (P less than 0.001) whereas cyclic AMP excretion was unchanged in both patients (patient AC 4.4 +/- 0.9 to 4.3 +/- 2.1; patient TC 2.2 +/- 0.9 to 2.6 +/- 0.9 nmol/min) with nephrogenic diabetes insipidus (NDI). Urinary cyclic AMP excretion was measured during infusion of 2.5% saline, after vasopressim administration, and after water deprivation. Cyclic AMP excretion was not different from control values in the NDI patients during any of the experimental conditions. Furthermore, there was no difference in cyclic AMP excretion when periods of dilute urine excretion (patient AC 4.5 +/- 1.1; patient TC 2.1 +/- 0.8 nmol/min) were compared with periods when urine concentration was greater than that of plasma (AC 3.5 +/- 1.3; TC 1.8 +/- 0.9 nmol/min). Both subjects responded to parathyroid hormone infusion with a 2-fold increase in urinary cyclic AMP excretion. Excretion of concentrated urine was paralleled by a marked decrease in urine flow to less than 1 ml/min/m2. During periods of hypotonic urine excretion (Uosm/Posm less than 1.0) average glomerular filtration rate (GFR) in patient AC was 67.0 +/- 3.0 ml/minm2 whereas in patient TC it was 70.1 +/- 8.1 ml/min/m2. When each patient was excreting a hypertonic urine (Uosm/Posm greater than 1.0) after fluid deprivation their GFR had decreased significantly (P = 0.001) to 31.6 +/- 8.9 and 33.3 +/- 10.3 ml/min/m2, respectively. Ability of these two subjects with NDI to concentrate their urine to Uosm/Posm greater than 1.0 in the absence of an increase in urinary cyclic AMP but associated with a decrease in GFR to 50% normal indicates that urinary concentration was effected by a reduction in GFR rather than a partial response to antidiuretic hormone (ADH). Their ability to concentrate their urine during periods of modest volume depletion would protect them from progressing to more severe stages of dehydration and result in the relatively benign course of their disease. It is feasible that in patients previously reported to have had clinically "partial" NDI this mechanism may have been operative.

A boy with null-cell leukemia received a bone marrow allograft after preparation with chemotherapy and total body irradiation. Cyclosporine A was not administered following transplantation. Renal biopsy performed 6 months after transplantation because of unexplained deterioration of renal function revealed diffuse mesangiolysis and glomerular sclerosis. The significance of this finding is discussed with reference to similar, recently reported cases.

We report 3 cases of proteinuria, progressive renal insufficiency and vesicoureteral reflux with no history of urinary tract infection. Renal histology showed focal segmental glomerulosclerosis. These associations are unusual during childhood but they are common in adults. Generally, vesicoureteral reflux is discovered during radiological evaluation of children with recurrent urinary tract infections. Renal parenchymal damage in children typically results from recurrent infection or progressive hydronephrosis. Development of proteinuria with vesicoureteral reflux usually indicates an irreversible glomerular lesion. Antireflux surgery in patients with vesicoureteral reflux and decreased renal function may be beneficial if performed before the onset of proteinuria. We recommend antireflux surgery for children with persistent vesicoureteral reflux or decreased renal function.

The effect of 30% galactose feeding on kidney function and structure was compared to the effect of streptozotocin-induced diabetes in the rat. In the galactose-fed rats there was increased urine volume (500%), creatinine clearance (40%), urinary albumin excretion (100%), urinary N-acetyl glucosaminidase (600%) and relative kidney weight (21%). These changes were similar to that observed in streptozotocin-induced diabetic animals. Galactitol in the kidney cortex of galactose-fed rats was increased 4 times similar to that observed for sorbitol in the streptozotocin-induced diabetic animals. Glycosylated hemoglobins were also increased in both galactose-fed animals and streptozotocin-treated animals. These data suggest that galactose feeding may be a useful model for investigating some aspects of diabetic nephropathy.

After using a cellular digestion technic to extract cells from the basement membranes of frozen kidney tissue, we used scanning electron microscopy to examine the acellular glomerular basement membranes (AGBM) and acellular tubular basement membranes (ATBM) from normal kidneys and from the kidneys of patients with lupus nephritis. This method revealed, in the AGBM, previously unrecognized three-dimensional patterns of pathologic changes. These patterns correlated with the World Health Organization (WHO) subclass of lupus nephritis and with the quantity of immune-complex deposition seen with two-dimensional microscopy. These pathologic changes included epimembranous, crater-like deformities with and without material resembling immune complexes; severely distorted, "moth-eaten" glomerular basement membrane; and the formation of secondary basement membrane within glomerular capillaries. We did not see similar abnormalities in the ATBM.

A male newborn presented with mild azotemia, an abdominal mass and non-visualized right kidney on excretory urography. Evaluation, including ultrasound and surgical exploration, revealed the simultaneous occurence of crossed fused renal ectopia and multicystic kidney.

In a nine-year-old girl with renal hypertension and a urinary infection, angiographic studies of the left kidney suggested an intrarenal pheochromocytoma. After nephrectomy, the kidney was found to contain only a cystic lesion characterized by multiple small, friable excrescences. Under the light microscope these appeared to be formed by granulovacuolated macrophages. Electron microscopy showed intracytoplasmic inclusion identified as Michaelis-Gutmann bodies in various stages of formation. Images suggestive of degraded bacteria were also observed within phagolysosomes.

A two-year-old child with the clinical stigmata of nail-patella syndrome, congenital urinary tract anomalies and proteinuria underwent renal biopsy. Electron microscopy revealed characteristic electron lucent areas and collagen fibril-like deposits in the glomerular basement membrane. Of special interest, electron dense deposits were seen in subendothelial areas of the capillary loops and immunofluorescent staining was striking, particularly for IgM, in a peripheral capillary loop pattern.

A four-year-old girl with growth failure and clinical and laboratory evidence of Bartter's syndrome responded to indomethacin treatment with decreased urinary prostaglandin excretion, symptomatic and chemical improvement, and accelerated growth. Large doses of aspirin produced a comparable decrease in prostaglandin excretion but no improvement in any other metabolic abnormality thus suggesting that abnormalities in prostaglandins were the result rather than the cause of the electrolyte abnormalities. Progressive renal insufficiency while on indomethacin prompted a renal biopsy, which revealed morphological changes of focal, segmental glomerular sclerosis. Subsequently, the child underwent renal transplantation with complete resolution of symptoms and abnormal metabolic findings. This observation suggests that extrarenal factors were not responsible for the development of Bartter's syndrome in this child.

Two children with hypertension had intrarenal aneurysms in the renal artery. In both children, renovascular hypertension was documented by renal vein renin assay showing elevated renin secretion from the involved kidney and suppression of renin secretion from the contralateral kidney. Transcatheter embolization of a segmental artery cured the hypertension in one case. A nephrectomy performed in the other case, because of multiple intrarenal aneurysms, was curative. Intrarenal renal artery aneurysms are an uncommon cause of renovascular hypertension in children. The aneurysms may be mycotic or congenital in origin, or secondary to diffuse vasculitis. Although spontaneous resolution of the aneurysms may occur when due to vasculitis, treatment of most aneurysms is usually necessary. Transcatheter embolization of solitary aneurysms is an attractive alternative to partial nephrectomy, since it allows maximal preservation of renal parenchyma with minimal morbidity.

A case of acute renal failure in a neonate was observed in which there were no obvious predisposing factors. Renal biopsy showed marked glomerular immaturity with otherwise normal renal architecture. Light and ultramicroscopic abnormalities noted suggest that the glomerular immaturity caused an abnormally low glomerular ultrafiltration coefficient (Kf). An inadequate rate of glomerular filtration secondary to the low Kf could have precipitated the acute renal failure. The finding of isolated glomerular maturational arrest is a previously undescribed cause of neonatal renal failure.

Two noninvasive methods of localizing the site of urinary tract infection, urinary lactic dehydrogenase (LDH) isoenzymes and antibody coating of bacteria in the urinary sediment, have been prospectively compared with the bladder washout technique in a series of children with urinary tract infection. Fifteen children had infection localized in the upper tract. Urinary LDH isoenzymes correctly localized the infection in 14 children; however, the infection was correctly localized by the antibody coating of bacteria in only eight patients (P less than .02). Fourteen children had lower tract infection by the bladder washout technique. Urinary LDH isoenzymes localized the infection in all 14 children, whereas the antibody coating correctly localized the infection in ten children (P less than .05). This study shows the urinary LDH isoenzyme pattern to be a more accurate technique than the detection of antibody-coated bacteria for localizing the site of urinary tract infection.

Both the antibody-coating and LDH isoenzyme techniques theoretically fulfill the criteria for an ideal localization test. Both are noninvasive. They are relatively easy to perform and well within the capability of most clinical laboratories. Both can be done rapidly and the results can be in the clinician's hands in a matter of a few hours. The results of the antibody-coating technique do not appear to be valid for children. Since only one controlled study with comparison with other localization techniques has been done in adults, further evaluation needs to be done. The LDH isoenzyme pattern has not been studied in adults but appears to be accurate in children. A prospective comparison of both procedures in adults using either the Fairly bladder washout technique or the Stamey ureteral catheterization method to localize definitively the site of infection would be most helpful. Definitive noninvasive localization techniques could lead to therapy being initiated with the site of infection defined. This would facilitate earlier and more intensive antibiotic therapy in those patients at risk for renal parenchymal damage from upper tract infections.

Renal tubular permeability was studied by microinjection techniques during increased intrarenal pressure in anesthetized diuretic rats. Intrarenal pressure, as evidenced by intratubular pressure (ITP), was increased by elevation of ureteral pressure, partial renal venous constriction, or massive saline diuresis. Various combinations of radioactive inulin, creatinine, mannitol, sucrose, and iothalamate in isotonic saline were microinjected into superficial proximal and distal convolutions, and recovery of the isotopes was measured in the urine. Inulin was completely recovered in the urine from the injected kidney at both normal and elevated ITP. Creatinine, mannitol, sucrose, and iothalamate were also completely recovered at normal ITP, but recoveries were significantly lower, averaging 73, 85, 89, and 85%, respectively, after early proximal injection when proximal ITP was increased to 30+/-2 mm Hg by elevation of ureteral pressure. Since transit time is prolonged under these conditions, mannitol recovery was also studied during aortic constriction, which prolongs transit time but lowers ITP. Recovery was complete. A significant loss of mannitol was observed during massive saline diuresis, which shortens transit time but increases ITP. During renal venous constriction producing a proximal ITP of 30+/-2 mm Hg, mannitol recovery was significantly less than 100% even after microinjection into distal convolutions, but the loss was greater injection at more proximal puncture sites. Mannitol recovery was complete during elevation of ureteral pressure in the contralateral kidney. These studies demonstrate a change in the permeability characteristics of all major segments of the renal tubule during elevation of intrarenal pressure. This change is rapidly reversible and does not appear to be due to a humoral factor which gains access to the general circulation.