Shane Roy

Hematuria preceding renal calculus formation in children with hypercalciuria

Five children were evaluated for painless hematuria; currently recommended diagnostic studies were unsuccessful in determining a diagnosis. A renal calculus subsequently was passed or detected radiographically in each child from 14 to 20 months after the initial evaluation. Hypercalciuria was then demonstrated by quantitation of 24-hour urinary calcium excretion or by measurement of urinary calcium/creatinine ratio. Calculi and hematuria have not recurred following thiazide therapy. We infer that idiopathic hypercalciuria was a probable cause of the unexplained painless hematuria.

Clinical experience with pediatric urolithiasis

Our clinical experience with 47 pediatric patients with stones is reviewed. Surgical therapy was standard with successful stone manipulation in 12 of 13 patients. In 91 per cent of our patients factors causing or predisposing to stone disease were discovered. A thorough metabolic evaluation, including an oral calcium loading test in 20 children, proved to be helpful. A new patient subgroup relating unexplained hematuria to eventual stone formation is described. Our protocol for metabolic evaluation and recommendations for treatment based on the results of such an evaluation are given. We have found the metabolic evaluation of the child with stones meaningful and particularly helpful in planning subsequent therapy for these patients.

Cystic Kidneys in a Patient With Oral-Facial-Digital Syndrome Type I

A cystic renal lesion is described in a girl with oral-facial-digital syndrome, type I. Excretory urography was normal at 1 yr of age; however, flank masses, hypertension, and renal failure were discovered at 11 yr of age. Bilateral nephrectomies were performed prior to renal transplantation. The renal cortex was replaced by large cysts. The cysts were lined by flattened nondescript epithelium and many contained glomerular tufts. Twenty renal cysts were aspirated, and the cyst fluid analyzed for sodium, potassium, creatinine, and osmolality. The concentration of solutes in the cyst fluid was comparable to plasma values. The pathologic features and pattern of cyst solute concentration appear to distinguish the cystic renal lesion in oral-facial-digital syndrome from the more common adult-type polycystic kidney disease.

Poststreptococcal crescenteric glomerulonephritis in children: Comparison of quintuple therapy versus supportive care

Crescenteric glomerulonephritis preceded by a streptococcal infection with creatinine clearance CCr of less than 30 ml/minute/1.73 m2 was treated by supportive care plus three months of quintuple therapy (prednisone, azathioprine, cyclophosphamide, dipyridamole, and heparin followed by warfarin) in five children (Group A) or by supportive care alone in five others (Group B). Of the glomeruli examined, 69.8 +/- 11.7% (mean +/- SE) in Group A and 64.4 +/- 10.6% in Group B had crescents which involved 54.0 +/- 10.8% and 60.0 +/- 10.5% of glomerular circumference, respectively. Clinical and histologic findings supported a recent streptococcal infection in every patient. Two patients from Group A had mild proteinuria and normal CCr at 12 months; one died abruptly of pulmonary hemorrhage after maintaining a normal CCr for 25 months. Following a second episode of poststreptococcal acute glomerulonephritis seven months after the first, one patient from Group B had persistent mild proteinuria for 41 months and hypertension through 56 months of follow-up. Nine surviving patients have maintained normal CCr for eight to 60 months (mean 29.5 months). The findings of this study suggest that this quintuple therapy offers no advantage over supportive care in the clinical management and outcome of children with severe crescenteric glomerulonephritis when an antecedent streptococcal infection is confirmed by serologic and histopathologic criteria.

Neonatal Hypertension from a Unilateral Multicystic, Dysplastic Kidney

Multicystic renal dysplasia is an extremely uncommon cause of hypertension in children and the few reported cases have not been of newborns. We report on a neonate in whom severe hypertension associated with elevated peripheral plasma renin resulted from a unilateral multicystic dysplastic kidney. Hypertension and plasma renin activity normalized after unilateral nephrectomy. No evidence of perfusion or excretory renal function in the dysplastic kidney was present on a radionuclide renal scan. This child demonstrates a relationship between hypertension and unilateral multicystic renal dysplasia.

Second attacks of acute glomerulonephritis

The cases of 12 Negro children with two clinically and histologically similar attacks of acute glomerulonephritis are reported. The absence of clinical and histologic evidences of chronic glomerulonephritis during a period of 6 to 108 months of follow-up study is emphasized. Lobular mesangial hyperplasia constituted the major persistent histologic abnormality; focal thickening of Bowmans capsule was less frequently observed.

Urolithiasis in Pre-Term Neonates Associated with Furosemide Therapy

The administration of furosemide to pre-term neonates has been associated with urolithiasis. We report 1 of 2 such cases that we have managed. The etiology of these stones appears to be related to furosemide-induced hypercalciuria. Nonsurgical management with thiazide diuretics can be successful in this high risk group of patients.

Long versus standard initial steroid therapy for children with the nephrotic syndrome

Abstract.A retrospective cohort study was conducted by the Southwest Pediatric Nephrology Study Group (SPNSG) to address whether a longer initial course of corticosteroids in patients with idiopathic nephrotic syndrome (INS) provides superior protection against relapse without increased adverse effects. In order to be included in the evaluation, patients with INS must have responded to an initial steroid course, either standard or long regimen as defined here, and completed at least 1xa0year of follow-up. The standard regimen consisted of prednisone 2.0±0.3xa0mg/kg per day or 60±10xa0mg/m2 per day for 28±4xa0days, followed by alternate-day prednisone for 4–12xa0weeks. The long regimen consisted of daily prednisone 2.0±0.3xa0mg/kg per day or 60±10xa0mg/m2 per day for 42±6xa0days, followed by alternate-day prednisone for 6–14xa0weeks. The primary outcome measure was relapse of NS within 12xa0months of discontinuing the initial course of prednisone. There were 151 children who met the criteria for the study; 82 received the standard regimen and 69 the long regimen. The two groups did not differ in age, race, blood pressure, serum albumin, or serum cholesterol prior to the initial steroid course. The cumulative prednisone dose was 49% higher in the long regimen group than in the standard regimen group. Relapse within 12xa0months was reported in 72.5% of patients who received the long regimen versus 84.1% of those who received the standard regimen. The odds ratio for relapse within 12xa0months was 0.496 (95% confidence interval 0.22, 1.088), long versus standard regimen. This did not reach statistical significance (χ2=3.058, P=0.08). The odds ratio of experiencing at least one side effect was 3.76, long relative to standard regimen (n=133, P<0.001). Our data suggest that prolongation of the steroid treatment for the initial episode of steroid-sensitive NS may have a beneficial effect, but at the cost of increased side effects. However, definitive conclusions are limited by the retrospective design of the study and the number of patients. This may have caused failure to achieve statistical significance on the basis of a type II error.

Urolithiasis in Childhood Acute Leukemia and Nonhodgkin’s Lymphoma

Ureteral calculi were found in 5 children who were receiving or had recently completed remission induction therapy for acute leukemia or lymphoma. All 5 patients had abdominal or back pain and 3 had gross hematuria. The diagnosis of urolithiasis was suggested by excretory urograms that showed obstructive uropathy (4 patients) and by computerized tomography scans that demonstrated ureterovesical obstruction (1 patient with acute renal failure and anuria). With a single exception the calculi were not associated with urinary tract infections. Chemical analyses in the 2 patients tested indicated that the stones were composed of calcium, in contrast to the uric acid and xanthine compositions of stones in earlier studies of patients with leukemia or lymphoma. Factors that might have predisposed our patients to calculi formation include corticosteroid therapy, immobilization owing to bed rest and urinary alkalization. Other possible contributing factors were urinary stasis (2 patients) and a familial tendency for renal calculi to develop. There was no evidence of idiopathic hypercalciuria in either patient tested. Prompt detection of urolithiasis in children undergoing induction chemotherapy for a malignant disease may avoid potentially serious consequences from urinary tract obstruction.

Potential Surgical Implications of Unexplained Hematuria in Children

Hematuria in children can be secondary to hypercalciuria and may occur well before a clinical stone episode. We report on 9 patients with initially unexplained hematuria who proved subsequently to exhibit stone formation secondary to hypercalciuria. The evaluation of otherwise unexplained hematuria in children should include calcium excretion studies. Long-term followup of patients with hematuria secondary to hypercalciuria is necessary because of the potential development of surgically significant stone disease.