The present study reports clinical and laboratory data of patients with Bartter syndrome at diagnosis and follow-up with emphasis on the long-term benefits and side effects of the pharmacological therapy, which includes indomethacin and potassium supplementation. We followed 12 children, 6 boys, with a median age at diagnosis of 24.5 months (range 7-137 months) and at the end of the study 157.5 months (range 26.0-224.0 months). All children presented with polyuria and polydipsia, dehydration, and metabolic and electrolyte disturbances with failure to thrive. However, at study entry 5 of 12 patients also had hypophosphatemia, which disappeared after a mean time of 50+/-22.4 months, 3 of 12 had nephrocalcinosis, and 2 of 12 had typical renal cysts. Despite treatment, hypokalemia was persistent in some patients. During long-term follow-up we observed recovery of growth velocity and adequate metabolic and electrolyte balance. However, we noticed renal and gastrointestinal complications: 2 patients had a perforated gastric ulcer, 1 had a gastric ulcer, and gastritis was detected in 3 children. A decreased glomerular filtration rate was observed in 2 patients during follow-up. Our data emphasize the need for regular surveillance of renal function and gastrointestinal endoscopy in these patients. As an alternative to indomethacin, we present our satisfactory preliminary results with rofecoxib.

The statural growth of 85 patients with steroid-responsive idiopathic nephrotic syndrome, attending the Pediatric Nephrology Unit, Children's Institute, Hospital das Clínicas School of Medicine, University of São Paulo, with a minimum follow-up of 3 years, was evaluated. Analysis of the patient population as a group did not show any significant alterations in the height Z score and the mean height percentile between the first (-0.59 and 33.9, respectively) and last consultation (-0.57 and 34.8, respectively). Analysis of each individual patient allowed the definition of two subgroups. Subgroup A, which achieved growth improvement, was composed of 47 children-initial Z score and mean initial height percentile of -0.91 and 24.0, respectively; final Z score and mean height percentile of -0.30 and 40.7, respectively ( P=0.00). Subgroup B, which showed growth retardation, was composed of 38 children-initial Z score and mean initial height percentile of -0.19 and 46.2, respectively; final Z score and mean height percentile of -0,9 and 27.5, respectively ( P=0.00). The following factors were significantly different when both subgroups were compared: (1) total duration of prednisone therapy and total prednisone dose were greater in subgroup B; (2) the final chronological age of patients using prednisone was higher in subgroup B; (3) the pubertal growth spurt in subgroup B showed attenuation and retardation.

Patients with idiopathic nephrotic syndrome present alterations in their cellular and humoral immune reactions that predispose them to the development of infectious processes.PURPOSE: To characterize the infectious processes in patients with idiopathic nephrotic syndrome. PATIENTS AND METHODS: Ninety-two children and adolescents with idiopathic nephrotic syndrome were assessed retrospectively. The types of infection were grouped as follows: upper respiratory tract infections; pneumonia; skin infections; peritonitis; diarrhea; urinary tract infection ; herpes virus; and others. The patients were divided into 2 groups: Group I (steroid-responsive) n = 75, with 4 subgroups-IA (single episode) n = 10, IB (infrequent relapsers) n = 5, IC (frequent relapsers) n = 14, and ID (steroid-dependent) n = 46; and Group II (steroid-resistant) n = 17. The incidence-density of infection among the patients was assessed throughout the follow-up period. Comparisons for each group and subgroup were done during the periods of negative and nephrotic proteinuria.RESULTS: The analysis revealed a greater incidence-density of infections during the period of nephrotic proteinuria in all the groups and subgroups, with the exception of subgroup IA. During the period of nephrotic proteinuria, subgroups IC, ID, and Group II presented a greater incidence-density of infections as compared to subgroup IA. For the period of negative proteinuria, there was no difference in the incidence-density of infections between the groups and subgroups. Upper respiratory tract infections were the most frequent infectious processes.CONCLUSION: The nephrotic condition, whether as part of a course of frequent relapses, steroid dependence, or steroid resistance, conferred greater susceptibility to infection among the patients with idiopathic nephrotic syndrome. The results of this study suggest that the best preventive action against infection in this disease is to control the nephrotic state.

ResumoObjetivo: Avaliar a freqüência diagnóstica dos vários distúr-bios causadores de hematúria na criança em um serviço universitá-rio de Nefrologia Pediátrica.Métodos: Foram analisados retrospectivamente os prontuários de 128 crianças, 78 do sexo masculino e 50 do sexo feminino, que apresentavam como queixa / achado principal de hematúria macroscópica persistente e/ou recorrente ou hematúria microscópica persistente, no período de 1978 a 1995. A análise foi realizada com especial atenção a dados de história, exame físico e antecedentes pessoais e familiares. A idade média à 1ª avaliação foi de 8,2 anos (5 meses a 16 anos), o período médio de seguimento de 3,2 anos (1 mês a 15 anos). Foram excluídos casos que abandonaram o seguimento antes da investigação etiológica.Resultados: Hematúria macroscópica ocorreu em 104 pacientes e microscópica persistente em 24 crianças. Entre os diagnósticos etiológicos firmados predominaram os distúrbios metabólicos e a litíase das vias urinárias, isoladas ou em associação (total de 65,5% dos casos). Hipercalciúria foi o distúrbio metabólico predominante (90,1%), isoladamente (73,2%) ou em associação com hiperuricosúria (16,9%). Antecedentes familiares positivos para litíase foram encontrados em 32,1% dos casos diagnosticados como portadores de litíase e/ ou distúrbios metabólicos urinários. Glomerulopatias foram diagnosticadas em 25% dos casos, com predomínio de glomerulonefrite aguda em 11 (34,5%). Em seis crianças (4,7% dos casos), apesar de intensa e exaustiva investigação incluindo biópsia renal, a causa da hematúria não foi elucidada.Conclusão: Os autores apresentam algoritmo para o diagnósti-co de hematúria na criança. E sugerem iniciar a investigação, pelos distúrbios metabólicos, quando não houver dados de anamnese que possam direcionar a pesquisa. J. pediatr. (Rio J.). 1998; 74(2):119-124: hematúria, criança. AbstractObjective: To evaluate the diagnostic frequency of the various diseases associated with the development of hematuria in children, in a pediatric nephrology unit pertaining to a university hospital.Methods: The clinical records of 128 children (70 male, 50 female) who presented intermittent/persistent macroscopic hematuria or persistent microscopic hematuria as the chief clinical complaint/finding, in the period of 1978-1995, were retrospectively analyzed. This evaluation was performed with special attention to the patient's clinical history, physical examination, personal and family morbid history information. Patients whose investigation was not complete were not considered for analysis. The mean age on presentation was 8.2 years (5 months -16 years) and the mean period of observation was 3.2 years (1 month-15 years).Results: Macroscopic hematuria occurred in 104 patients and persistent microscopic hematuria was present in 24 patients. Urinary metabolic disturbances and urinary lithiasis, alone or in association, were diagnosed in the majority of the patients (65.5%). Hypercalciuria was the urinary metabolic disturbance (90.1%) mostly detected, either alon...