[Urinary TGF-beta1 excretion in children with renal cysts]. / Wydalanie TGF-beta1 w moczu dzieci z torbielowatoscia nerek.

UNLABELLED: Autosomal dominant polycystic disease is characterised by abnormal polycystin, protein, which is a component of basement membrane and extracellular matrix. Transforming growth factor (TGF-beta1) is a cytokine, which takes part in development of renal tubule epithelium and stimulates the synthesis of extracellular matrix proteins. The aim of work was the assessment of TGF-beta1 concentration in children with renal polycystic disease. MATERIAL AND METHODS: The examined group (I) consisted of 33 children aged (median 14.7 years, range 4.0-17.9): A--11 children with solitary cyst, B--22 with polycystic renal disease. Control group (C) consisted of 20 healthy children at the same age. The concentration of urinary TGF-b1 was measured using immunoenzymatic ELISA method. The results showed that mean concentration of urinary TGF-beta 1 (121.9 +/- 168 pg/mg cr.) was lower than in group B, in which it was 207.2 +/- 361 pg/mg cr. However the difference was not statistically significant (p>0.05). In both subgroups (A and B) urinary excretion of TGF-beta1 was higher than in control group (C) (p<0.05). In 4 (36%) children from group A and 8 (36%) from group B the urinary concentration of TGF-beta 1 was below the sensitivity of the method. No correlation between TGF-beta 1 and children's age, urinary osmolality and GRF according to Schwartz was found. It was a positive correlation between urinary TGF-betal concentration and total diameter of renal cysts. CONCLUSIONS: TGF-betal takes part in renal cyst formation and increased urinary excretion of TGF-b1 in proportion to the dimension of renal cysts may be an evidence of that fact.

Assessment of serum cystatin C in children with congenital solitary kidney.

The aim of the study was to assess serum cystatin C level in children with a congenital solitary kidney, depending on their age and compensatory overgrowth of the kidney. The study group (I) consisted of 36 children, 3-21 years of age (median 10.8 years), with a congenital solitary kidney and no other urinary defects. The control group (C) contained 36 healthy children, 5-21 years old (median 10.9 years). Nephelometric methods were used to determine serum cystatin C level, the Jaffe method to assess creatinine concentration and the Schwartz formula to estimate glomerular filtration rate. Kidney length was measured with the patient in a supine position, and overgrowth was estimated (O%) in comparison with the respective kidney in the control group. Serum cystatin C level in group I was higher than that in the control group (P<0.05). Increased values, above 0.95 mg/l, were found in 16/36 (44%) children aged 12-21 years. Glomerular filtration rate (GFR, estimated by the Schwartz formula) and creatinine level in group I were similar to those of the control group (P>0.05). Increased kidney length was found (median 18.2%). Cystatin C concentration was positively correlated with O% (r=0.406, P<0.01) and kidney length to child height ratio (L/H) (r=0.376, P<0.05). We conclude that Increased serum cystatin C concentration in patients with a unilateral congenital solitary kidney occurs after 12 years of age and correlates with compensatory overgrowth of the kidney.

[Vasculitis of cerebral vessels, probable cause of neurological complications in a child with Schonlein-Henoch purpura]. / Zapalenie naczyn mózgu prawdopodobna przyczyna powiklan neurologicznych u dziecka z choroba Schönleina-Henocha.

We report 6-years old boy with Schönlein-Henoch purpura who presented neurologic manifestations: depressed state generalised convulsions, and cortical blindness. Sequential magnetic resonance imaging showed bilateral cerebral ischemic lesions in the cortex and white matter of parieto-occipital lobes caused by vasculitis.