[Major renal biopsy complications in 11-year-old boy--a case report]. / Ciezkie powiklania biopsji nerki u 11-letniego chlopca--opis przypadku.

Renal biopsy is an essential procedure in the diagnosis, treatment and follow-up of renal diseases. The risks associated with performing percutaneous renal biopsy have substantially decreased in the past decades. However, major complications still occur. We report and discuss a case of severe renal biopsy complications in 11-year-old with nephrotic syndrome.

[Alport syndrome in 23-year-old woman]. / Zespól Alporta u 23-letniej kobiety.

We report a case of Alport syndrome in a 23-year-old woman. Early-onset microscopic hematuria, nephrotic syndrome, arterial hypertension and the presence of extrarenal symptoms, e.g. hearing loss are risk factors of disease progression towards renal failure in the patient. This case report emphasizes the importance of electron microscopic studies in the diagnosis of hereditary nephropathies.

[The results of conservative treatment of oxalate urolithiasis in children]. / Wyniki leczenia zachowawczego kamicy szczawianowej u dzieci.

UNLABELLED: Hyperoxaluria is defined as urinary oxalate excretion exceeding 0.45 mmol/1.73 m2/day and accounts for 15% of recurrent urolithiasis. There have been only a few reports on the prevalence and treatment of oxalate urolithiasis in children. THE AIM: Of the study was to assess the efficacy and safety of the protocol of intensive and combined treatment of hyperoxaluria in children. MATERIAL AND METHODS: Seventeen children at the mean age of 11.5 +/- 4.5 years with positive history of urolithiasis and diagnosis of hyperoxaluria were studied. In this group hyperoxaluria was an isolated defect in 9 of 17 children, but in 3/17 it was accompanied by hyperuricosuria, in 5/17 by hypomagnesuria and in 1 case by hypercalciuria. During the 12-month period the children were intensively hydrated and received a low-oxalate diet and supplemental therapy with vitamin B6, magnesium, citrates and lactic acid bacteria preparations. RESULTS: In all but one child oxaluria decreased below 0.45 mmol/1.73 m2/day (decrease by 45%). No new stone formation was seen during the observation period. In all patients abdominal pain and haematuria subsided. CONCLUSIONS: We conclude that the intensive, complex, conservative treatment of hyperoxaluria in children is effective and safe. It allows to decrease hyperoxaluria and prevent the recurrence of urolithiasis.

[Beneficial effect of angiotensin converting enzyme inhibitor treatment in severe cystine urolithiasis]. / Korzystny efekt leczenia inhibitorem enzymu konwertujacego angiotensyne w ciezkiej cystynowej kamicy nerkowej.

Cystinuria is an autosomal recessive defect in transepithelial transport of dibasic amino acids (e.g. cystine) which involves the proximal canaliculi, small intestine and central nervous system. It is the least common cause of nephrolithiasis, accounting for 1 to 3% of renal calculi. The natural course of the disease, characterised by recurrent stone formation, can frequently lead to renal failure, if left untreated. Until recently, treatment of cystinuria has been limited to symptomatic management including intensive hydration and urine alkalinisation. Different drugs that react with cystine to form soluble complexes have been used but their efficacy remains questionable. We present the case of a 6-year-old boy with severe, recurrent cystine urolithiasis treated with captopril. The diagnosis of cystine urolithiasis was established after a 3-year course of clinically apparent nephrolithiasis, characterised by stone passage. At the age of 5 years he underwent lithotripsy and nephrolithotomy for removal of staghorn calculi. Since then treatment with citrate and magnesium supplementation combined with captopril was introduced. After a follow-up of 12 months the patient remained stone-free. Urinary cystine decreased from 230 to 136 mg per gram creatinine. We conclude that captopril can be useful in the treatment of cystine urolithiasis in children.

[Acute renal failure in the course of IgA nephropathy in a 16-year-old boy]. / Ostra niewydolnosc nerek w przebiegu nefropatii IgA u 16-letniego chlopca.

Acute renal failure (ARF) is a rare presentation of IgA nephropathy (IgAN). It can be associated with the episodes of macroscopic haematuria, being then usually reversible, or it develops in the course of progressive glomerulopathy with extracapillary proliferation. In the first case ARF is attributed to tubular obstruction by red blood cell casts. We present the case of a 16-year-old boy with non-oliguric ARF in the course of IgAN. He was admitted to the urology unit with 4-day history of gross haematuria, severe loin pain, fever and vomiting. A year before admission he had a short episode of macrohaematuria without any other accompanying symptoms. His family history was not relevant. As the patient was suspected to have acute renal colic in the course of nephrolithiasis, intravenous urography was performed. Since no urinary tract visualisation was obtained, laboratory investigation was carried out revealing marked renal dysfunction with serum creatinine level of 743.3 mumol/l and serum urea of 49.3 mmol/l. The patient was transferred to our department, where conservative treatment was administered (dialysis was not instituted). A rapid improvement in renal function was observed; it returned to normal within 2 weeks. The biopsy findings were consistent with IgAN.

[Denys-Drash syndrome: a case report]. / Zespól Denysa-Drasha--opis przypadku.

Nephrotic syndrome (NS) rarely develops before the age of 1 year. The case is presented of nephrotic syndrome occurring in the form of Denys-Drash syndrome. In a newborn of female sex in birth certificate, dysmorphia was found of the external urogenital organs. The karyotype was 46XY. Massive proteinuria, low total serum protein level, dysproteinaemia, hypercholesterolaemia justified the diagnosis of NS. In renal biopsy performed diffuse mesangial fibrosis was found. The progression of renal insufficiency was very rapid and within few weeks terminal renal failure developed. The parents refused consent to renal replacement treatment. The baby died at the age of 102 days. The autopsy examination confirmed renal changes in the form of diffuse fibrosis; gonads of testicular structure were found in the abdominal cavity.

[Congenital nephrotic syndrome]. / Wrodzony zespóll nerczyowy.

In strict sense, the term "congenital nephrotic syndrome" (CNS) refers to those cases of the nephrotic syndrome in which clinical symptoms, e.g. massive proteinuria, hypoalbuminemia and oedema are present at birth. However, the term is often extended to babies presenting with nephrotic syndrome before 3 months of age in whom proteinuria is likely to be present earlier, before the signs of the nephrotic syndrome become clinically manifest. The most common and probably the most severe type of CNS is the congenital nephrotic syndrome of the Finnish type (CNF), which is considered as the prototype of CNS. On the basis of this syndrome the clinical course, diagnosis and complex treatment strategy are described. A well-documented case of Denys-Drash syndrome - a rare type of congenital nephrotic syndrome is also presented.

[Shunt nephritis]. / Shunt nephritis.

The term "shunt nephritis" stands for an immune-complex-mediated glomerulonephritis which develops as a complication of chronically infected ventriculoatrial or rarely ventriculoperitoneal shunt inserted for the treatment of hydrocephalus. The renal outcome of shunt nephritis is good if early diagnosis and treatment is provided. Due to a wide clinical spectrum of this disorder, as well as indolent courses of shunt infections, the diagnosis is often delayed. We present the case of 13-year-old girl with a severe course of shunt nephritis in whom the diagnosis was both overlooked and delayed. Misinterpretation of her symptoms led to erroneous diagnosis of lupus nephritis and introduction of immunosuppressive therapy.