[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.

[Pyelonephritis with massive renal tissue necrosis in child with urinary tract malformation--a case report]. / Odmiedniczkowe zapalenie nerek z rozlegla martwica miazszu nerek na podlozu wady wrodzonej ukladu moczowego--opis przypadku.

The aim of the case report is presentation of unusual and heavy clinical course of pyelonephritis with renal tissue necrosis in a child with urinary tract malformation. Nine month old girl was admitted to hospital in heavy clinical status due to pyelonephritis--urosepsis. It was complicated by acute renal insufficiency. Patient was treated by broad-spectrum antibiotics and parenteral nutrition. She was feverish for 14 days. Computed tomography done in order to exclude abdominal abscess showed massive renal tissue necrosis of on both sides. Antibiotic treatment was successful after 6 weeks. Urological evaluation revealed bilateral vesico-ureteral refluxes grade IV. Scintigraphy showed multiple scars. Patient was treated Deflux injections (twice). We noted 5 urinary tract recurrences despite antibiotic profilaxis. GFR of 75 ml/min/1.73 m2 was estimated at age of 16 m. Immunodeficiency or malignancy as background of clinical course were excluded. The case we describe presents severe clinical course of pyelonephritis due to complex urinary tract malformation that is to be considered despite based on modern publications "sparing" strategies of diagnosis and profilaxis in urinary tract malformations.

[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 spectrum of congenital malformations of the urinary tract in fetuses and newborns in autopsies in 1989-2002]. / Wystepowanie wad ukladu moczowego plodów i noworodków w materiale autopsyjnym w latach 1989-2002.

The retrospective analysis of own autopsy findings of 831 fetuses and newborns with different forms of congenital malformations were carried out. In 172 cases (20.7%) urinary tract abnormalities were revealed. Isolated malformations represented 43.6% (75 cases) and 56.4% (97 cases) constituted one component of complex multisystem anomalies. Obstructive uropathies with different renal changes represented 54.7% of the isolated malformations, renal agenesis - 28%, autosomal recessive or dominant polycystic renal diseases - 16% and renal hypoplasia 1.3%. Obstructive uropathies were also most prevalent among the multisystem abnormalities (45.4%), while agenesis represented 34% of cases, horseshoe kidney - 12.4%, autosomal polycystic kidney diseases 4.1%, and renal hypoplasia 4.1%. Congenital abnormalities of urinary tract (especially the obstructive uropathies and agenesis) were more common in male fetuses and newborns. A highly frequent coexistence of urinary system congenital abnormalities and malformations of digestive tract, circulatory system and central nervous system were observed.

[Concentration of tissue plasminogen activator and its inhibitor 1 in cyclosporine A-treated children with idiopathic nephrotic syndrome]. / Stezenie tkankowego aktywatora plazminogenu i jego inhibitora 1 u dzieci z idiopatycznym zespolem nerczycowym leczonych cyklosporyna A.

UNLABELLED: Patients with idiopathic nephrotic syndrome (INS) are at increased risk of thromboembolic events at every stage of the disease. We assessed the function of the coagulation cascade and fibrinolysis in stable remission of INS, in children treated with cyclosporine A. MATERIAL: The study group consisted of 17 children (10 M, 7 F; mean age 8.5 +/- 3.2 years, range 4-18 years) with 2 month-remission of steroid-dependent INS diagnosed according to the international criteria and 20 healthy, age-matched children, and 18 children in long-term INS remission serving as controls. The children with INS relapse were treated with cyclosporine A (to maintain the drug concentration value between 80 and 150 ng/ml) and low doses of steroids, according to a standard protocol. METHODS: Fibrinolysis was assessed by measurement of tissue plasminogen activator (t-PA) and tissue plasminogen activator inhibitor (PAI-1) concentrations, whereas activation of thrombinogenesis was detected by F1 + 2 prothrombin fragment concentration. Additionally, we measured selected coagulation (concentration of thrombin-antithrombin complexes, fibrinogen concentration, prothrombin time, activated partial thromboplastin time, platelet count) and biochemical (serum albumin, cholesterol, creatinine concentration) factors. RESULTS: In children treated with cyclosporine A increased concentrations of t-PA and PAI-1 were found (p < 0.01). The concentration of F1 + 2 prothrombin fragments was also higher when compared to controls (p < 0.05). We found also increased total cholesterol, and lower creatinine concentration in the study group. CONCLUSION: Children with remission of idiopathic nephrotic syndrome treated with cyclosporine A show increased concentration of t-PA and PAI-1 and thrombinogenesis. Despite clinical and biochemical remission they may still remain at high risk of thrombosis or endothelial injury.

[Assessment of children at the beginning of renal replacement therapy in Lodz 1990-2000]. / Ocena stanu dzieci rozpoczynajacych leczenie nerkozastepcze w ICZMP w Lodzi w latach 1990-2000.

The time of patients' referral to dialysis predicts the clinical outcome of the therapy and significantly influences the mortality rate. The aim of the study was to assess the clinical and nutritional status and selected biochemical parameters (serum creatinine, urea, bicarbonate, calcium, albumin concentration) at the beginning of renal replacement therapy. We analysed medical history of 46 children (24 boys; 22 girls) aged 1 month-18 years (mean age 13.1 +/- 5.5 years). We divided them into late-referral and early-referral groups. 56% of the children were under nephrological care before the beginning of dialysis treatment. However, in 44% of the cases renal insufficiency had not been diagnosed before. It was found that the children who received no nephrological care in the past demonstrated significantly worse clinical and biochemical status at the beginning of the renal replacement therapy.

[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.

Clinical challenges in the management of a prenatally diagnosed cloacal malformation.

Cloacal dysgenesis sequence is a severe malformation of the primitive cloaca and is characterized by a phallus-like structure, smooth perineum and the absence of genitourinary and anal orifices. It is usually accompanied by oligohydramnios, kidney dysplasia, and pulmonary hypoplasia. We present a case of a 29-year-old woman who was referred at 26 weeks of gestation due to an enlarged fetal abdominal circumference. Investigations revealed the presence of fetal ascites, intrapelvic cysts, calcified meconium, severe oligohydramnios and a 46XX karyotype. Fetal abdominal parecentesis performed on several occasions failed to reduce intra-abdominal pressure. To our knowledge this case represents the first variation of cloacal dysgenesis sequence to contain three dysmorphic structures along with the common findings of this anomaly.