Gastrointestinal manifestations of nephropathic cystinosis in children

Cystinosis is an autosomal recessive disorder which is characterized by both renal and extrarenal symptoms. Gastrointestinal dysfunction has been reported in adolescent with cystinosis, and it is rarely considered in the infants. The present case series reviewed gastrointestinal manifestations of these patients. Gastrointestinal signs and symptoms of 23 children aged 5.99 +/- 0.50 years [range, 1.0 to 12.5 years] on average with cystinosis, admitted to our department of nephrology between 1996 and 2005, were retrospectively reviewed. The inclusion criteria were the presence of the crystals of cystine in bone marrow aspiration and corneal deposition detected by slit lamp examination. Gastrointestinal signs and symptoms were as follows: vomiting in 16 patients [69.6%], hepatomegaly in 8 [34.8%], diarrhea in 6 [26.1%], splenomegaly in 5 [21.7%], constipation in 4 [17.4%], anorexia in 4 [17.4%], abdominal pain in 3 [13.0%], nausea in 2 [8.7%], and ascites in 2 [8.7%]. Height below the 3rd percentile in was seen in 16 patients [69.6%] and weight below the 3rd percentile, in 17 [73.9%]. Fifteen patients [65.2%] had both low weight and low height. Esophagogastroduodenoscopy had been performed in 6 cases and chronic inactive gastritis with H pylori infection was detected in 2 patients [8.7%]. Our study revealed a wide spectrum of gastrointestinal disturbances in young patients with cystinosis. Such findings should lead to greater awareness of the presence of gastrointestinal dysfunction in these children, encourage prompt gastrointestinal evaluation, and encourage treatment of more severely affected patients

Fungal peritonitis in Iranian children on continuous ambulatory peritoneal dialysis

Fungal peritonitis [FP], causing catheter obstruction, dialysis failure, and peritoneal dysfunction, is a rare but serious complication of peritoneal dialysis. In this study, the frequency and risk factors of FP are evaluated in children who underwent peritoneal dialysis. A retrospective multicenter study was performed at the 5 pediatric peritoneal dialysis centers in Iran from 1971 to 2006, and FP episodes among 93 children were reviewed. Risk ratios were calculated for the clinical and demographic variables to determine the risk factors of FP. Ninety-three children aged 39 months on average were included in study. Sixteen out of 155 episodes of peritonitis were fungi infections, all by Candida albicans. The risk of FP was higher in those with relapsing bacterial peritonitis [P = .009]. Also, all of the patients had received antibiotics within the 1 month prior to the development of FP. Catheters were removed in all patients after 1 to 7 days of developing FP. Six out of 12 patients had catheter obstruction and peritoneal loss after the treatment and 5 died due to infection. Fungal peritonitis, accompanied by high morbidity and mortality in children should be reduced by prevention of bacterial peritonitis. Early removal of catheter after recognition of FP should be considered

Quantification of proteinuria with urinary protein to osmolality ratios in children with and without renal insufficiency

Spot urine is recommended as an accurate method to determine proteinuria in children and adults. However, urinary excretion of creatinine may vary in newborns and spot urine may be influenced by the hydration-dehydration condition of patients. The study was done to assess the validity of the urine protein to osmolality ratio versus the urine protein to creatinine ratio in health and disease conditions. We studied the correlation of the urine protein-osmolality ratio [Uprot/Uosm] and the urine protein to creatinine ratio [Up/Ucr] and compared results with the 24-hour urinary protein excretion. Three groups were compared: children with normal renal function and without proteinuria [group 1, n=53], children with normal renal function and with proteinuria [group 2, n=52] and patients with renal insufficiency [group 3, n=45]. Early morning urine samples and 24-hour urine specimens were collected for protein, creatinine, and osmolality. The optimal cutoff value of the Uprot/Uosm ratio was determined to be 0.33 mg/L/mosm/kgH2O for abnormal proteinuria and 1.75 mg/L/mosm/kgH2O for nephrotic range proteinuria. In comparing ROC curves, we found no differences between the Uprot/Uosm and Up/Ucr ratios in detecting abnormal proteinuria or nephrotic syndrome in children with normal or decreased renal function [P>0.05]. Both the Uprot/Uosm and Up/Ucr ratios from random urine specimens are good predictors of 24-hour urinary total protein excretion in children with and without renal insufficiency