Child nephrology and urology最新文献

We present a case of urinary bladder carcinoma in a child aged 13 that followed a benign course. This confirms the idea that epithelial bladder cancer in children pursues, a much less aggressive behaviour than in the adult, possibly on the basis of its well-differentiated nature. Despite the rarity of the lesion, it should not be overlooked in the differential diagnosis of haematuria in children.

The captopril test was performed on 49 children of whom 36 were hypertensive, and the remainder were normotensive but were at risk for developing hypertension because of scarred kidneys secondary to vesico-ureteral reflux. Blood pressure (BP) was monitored in fasting supine patients throughout the duration of the test. Blood was taken for measurement of plasma renin activity (PRA); then captopril (0.7 mg/kg of body weight) was administered orally. A second blood sample was taken for PRA at 90 min postcaptopril. The mean (SEM) PRA at 90 min was 11.90 (4.01) ng/l/s [42.84 (14.44) ng/ml/h] in 7 patients with renovascular disease. In 4 patients with essential hypertension corresponding values were 0.88 (0.38) ng/l/s [3.17 (1.37) ng/ml/h]. Patients with other renal diseases showed variable values. Some individuals had PRA values as high as those of patients with renovascular disease, but the etiology of their hypertension was usually clinically evident. Our preliminary data would suggest that the captopril test may help differentiate between patients with essential hypertension and those with renovascular disease, or may help select patients that should be followed up by more definitive diagnostic procedures.

Amino acid balance and nitrogen balance during total parenteral nutrition (TPN) and continuous arteriovenous hemofiltration (CAVH) were investigated in 11 critically ill anuric patients during the first 7 days after onset of anuria. Nitrogen intake ranged from 0.115 +/- 0.013 (SEM) g/kg/day on day 1 to 0.291 +/- 0.029 (SEM) g/kg/day on day 7. After 7 days of TPN, 9 patients had a positive cumulative protein-N balance of 287.52 +/- 68.52 (SEM) mg/kg, 2 patients had a negative balance of 781.8 and 1,103.2 mg/kg, respectively. Mean amino acid loss in ultrafiltrate was 0.159 +/- 0.008 (SEM) g/kg/day. Four patients died without recovery of renal function.

Sickle cell disease is known to cause glomerulopathy, including focal segmental glomerulosclerosis (FSGS). Patients who have sickle cell glomerulopathy with FSGS are thought to have a poorer prognosis than patients who have sickle cell glomerulopathy without this lesion. The former patients are more likely to have persistent proteinuria and eventually develop end-stage renal disease. We present a boy with sickle cell glomerulopathy and FSGS who is younger than patients with similar findings reported previously. The histopathology of his renal lesions is remarkable for segmental ultrastructural changes in the glomerular basement membranes and endothelial cells. We speculate that these changes are precursory to the pathogenesis of glomerular sclerosis in patients with sickle cell disease.

Three years following successful liver transplantation, a child developed proteinuria, hematuria and hypertension in the setting of progressive renal insufficiency. These abnormalities did not resolve with lower doses of ciclosporin. Because multiple drugs were required to control the hypertension and because no other etiology of the urinary abnormalities could be found, a renal biopsy was performed. The renal biopsy revealed findings consistent with severe IgA nephropathy, including glomerulosclerosis, segmental crescents, mesangial cell and matrix expansion, mesangial deposits, and positive immunofluorescence for IgA.

We evaluated 38 newborns with acute renal failure (plasma creatinine (Pcr) concentration greater than = 1.5 mg/dl), measured between the 2nd and 5th days. We used renal ultrasound to exclude the possibility of congenital renal anomalies, obstructive pathology or vascular disorders. We calculated the glomerular filtration rate (GFR) using Schwartz' formula and the maximal concentrating capacity using intranasal administration of desamino-cis-1-D-arginine-8-vasopressin (DDAVP test). Two newborns were treated with peritoneal dialysis and died during the first month of life. Thirty-six had a follow-up blood sample drawn: 24 preterm babies between 1 and 12 months, and 12 full-term babies between 1 and 36 months of life. From this sampling 4 babies (11.1%) showed defective maximal concentrating ability. Our data reveal the persistence of altered concentrating ability in newborns affected by renal failure and shows that this problem needs a longitudinal study and further diagnostic investigations.

Two different types of familial nephrotic syndrome were observed in two unrelated families. In the first family, 2 siblings, both boys without hearing impairment, had proteinuria which was evident after 10 years of age and were resistant to steroid and immunosuppressant therapy. Their renal biopsy findings were compatible with focal-segmental glomerulosclerosis. In the second family, an elder sister and a boy had minimal-change nephrotic syndrome which responded well to steroid and immunosuppressant therapy. All 4 patients had HLA-DR5 in common, suggesting that this gene locus may play an important role in the pathogenesis of familial nephrotic syndrome.

Between 1982 and 1986, 96 children with nonobstructive vesicoureteric reflux were treated in a prospective study. There were 134 refluxing ureters. Results are reported after a follow-up period of 3 years in 94 children with 130 refluxing ureters. Initially all children with reflux grade III or less had antibiotic treatment only. Those with reflux grade IV were randomized for antibiotic treatment alone versus surgery plus antibiotic treatment, while the primary treatment of reflux grade V was reimplantation. In 84 ureters treated by antibiotics alone, reflux disappeared in 52 cases and in 18 ureters the reflux was reduced. In 49 ureters treated by reimplantation, reflux was cured in 39 cases and no severe ureteric obstruction was seen. Conservative management of reflux grade IV was less successful than surgery. The results of conservative, nonsurgical treatment of reflux grade I to III are satisfactory, but for grades IV and V reflux surgery should be the treatment of choice, provided that detrusor instability can be excluded.

Staphylococcus saprophyticus urinary-tract infection has been reported to occur in sexually active young females and in geriatric patients with obstructive uropathy. We are discussing two young male children with S. saprophyticus urinary-tract infection. We draw attention to this bacterium which is emerging as an important pathogen in children with urinary-tract infections.

The influence of chronic renal failure (CRF) on growth hormone (GH) and insulin-like growth factor I (IGF-I) metabolisms is not well understood. Clinical studies on GH secretion in CRF have yielded conflicting results. In vitro, pituitary GH secretion has been shown to be unimpaired in moderate uremia. CRF reduces binding of GH to liver as a result of decreased number of GH receptors. CRF induces elevation of serum GH concentrations and does not modify the circulating values of IGF-I. However, the somatomedin bioactivity of uremic serum is depressed, and unsaturated low-molecular-weight IGF-I-binding proteins have been suggested to act as inhibitory factors of IGF-I action. Circulating GH and IGF-I do not necessarily reflect the state of GH and IGF-I in tissues, and further investigations on the effect of CRF on GH and IGF-I metabolisms at the growth plate level are clearly required.