Pediatric nephrology : journal of the International Pediatric Nephrology Association
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Comparative Study
Sterile pyuria in patients with Kawasaki disease originates from both the urethra and the kidney.
To identify the origin of urinary leukocytes in Kawasaki disease (KD) patients with pyuria, we prospectively studied clinical and laboratory findings of 23 KD patients. Patients were divided into three groups: patients without pyuria, patients with pyuria in both voided urine and bladder urine obtained by transurethral catheterization (bladder pyuria) and patients with pyuria only in voided urine (urethral pyuria). Pyuria in voided urine was found in ten of 23 KD patients (43.5%), with subsequent urine cultures proving sterile. ⋯ Urinary protein levels were higher in patients with bladder pyuria and in patients with urethral pyuria than in patients without pyuria. Urinary beta2-microglobulin concentrations and serum blood urea nitrogen (BUN) and creatinine levels were higher in patients with bladder pyuria than in patients with urethral pyuria or in patients without pyuria, although the serum BUN and creatinine levels of patients with bladder pyuria were within the normal ranges. These results suggest that some patients with KD develop sterile pyuria that originates from the urethra and/or the kidney as a result of mild and subclinical renal injury.
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A 2-year-old girl presented with hypertension, anorexia and vomiting, restlessness, insomnia and acrodynia. Her blood pressure upon arrival was 145/98 mmHg. Ultrasound of the abdomen, CT scan of chest, abdomen and pelvis, and echocardiogram, were normal. ⋯ Following a 1-month course of oral treatment with dimercaptosuccinic acid (DMSA) the child's symptoms and signs resolved, and urinary mercury and catecholamines levels normalized. Mercury intoxication should be suspected in a patient with severe hypertension, personality changes and acrodynia. Normal blood levels of mercury do not exclude this diagnosis, and catecholamine levels may serve as a surrogate marker for confirmation of the diagnosis and to evaluate response to treatment.
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Case Reports
Is there a role for rituximab in the treatment of idiopathic childhood nephrotic syndrome?
We each have nephrotic patients who become steroid dependent and in whom multiple immunosuppressive agents are employed. There is a need to balance possible therapeutic benefits with drug toxicity. This case report describes such a patient, who has suffered from nephrotic syndrome for over 11 years and had become resistant to the usual therapies. He was therefore given a single dose of the anti-CD20 drug rituximab, to which he showed a prompt response, leaving him free of proteinuria for the past 10 months.
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Continuous renal replacement therapy (CRRT) has become an important supportive therapy for critically ill children with acute renal failure. In Turkey, commercially available diafiltration and replacement fluids cannot be found on the market. Instead, peritoneal dialysis fluids for dialysis and normal saline as replacement fluid are used. ⋯ Using peritoneal dialysis fluid as dialysate is not a preferable solution. Early initiation of CRRT offered survival benefits to critically ill pediatric patients. Mortality was associated with the primary disease diagnosis.
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Comparative Study
Mutational analysis of NPHS2 and WT1 in frequently relapsing and steroid-dependent nephrotic syndrome.
Idiopathic nephrotic syndrome is a common pediatric kidney disease, 80% of all cases are steroid sensitive (SSNS). A significant proportion of children with SSNS will have a frequently relapsing or steroid-dependent course (FRNS/SDNS) that is associated with significant treatment-related morbidity. Mutations in NPHS2 account for more than 28% of all cases of steroid-resistant nephrotic syndrome (SRNS) and dominant mutations in WT1 for 5%; while mutations are absent from children with uncomplicated SSNS. ⋯ Children with FRNS/SDNS were significantly younger at first presentation than those with SSNS and SRNS (median age: 3.0 years in FRNS/SDNS patients, 7.0 years in SSNS patients, and 5.0 in SRNS patients; p < 0.001). No NPHS2 or WT1 mutations were found in patients with FRNS/SDNS and uncomplicated SSNS. The hypothesis that FRNS/SDNS may be associated with heterozygous mutations in NPHS2 or WT1 was not confirmed.