[Schönlein-Henoch purpura. A clinical study of 119 patients with special reference to unusual complications]. / Purpura Schönlein-Henoch. Eine klinische Studie an 119 Patienten unter besonderer Berücksichtigung ungewöhnlicher Komplikationen.

119 patients with Schönlein-Henoch purpura from 1 to 17 years were investigated. All patients had a rash, 76% joint affections, 76% gastrointestinal symptoms, 54% renal involvement, 17% of the boys orchitis, 2% intramuscular bleeding and 2% coagulation disorder. 1 boy showed perforation of the nasal septum, to our knowledge not described in Schönlein-Henoch purpura up to now. The mean age was 6.4 years. The seasonal peak was in October, 63% of our patients had a preceding infection, mostly of the upper respiratory tract, pointing to various infectious agents as a trigger of this disease. Patients with abdominal pain had a 4-fold, patients with bloody stools a 7.5-fold increased risk of renal involvement. 13/38 Patients had a serum albumin level below 3.5 g/dl that may have been caused either by renal or gastrointestinal losses.

Adolescent cystinosis, presenting with proteinuria.

Isolated proteinuria preceding tubular dysfunction for years was the presenting laboratory sign in a patient with adolescent cystinosis, followed up to end-stage renal failure in the second decade of life. The renal biopsy showed the early characteristic features, multinucleated giant podocytes. The diagnosis was documented by slit lamp examination of the eyes and cystine determination in fibroblasts.

Survival time in cystinosis. A collaborative study.

In a retrospective study the overall survival time of 205 cystinotic patients of six countries was determined. The median survival time was 8.5 years. The median time for 'renal death' (age at death due to uraemia or age at starting renal replacement therapy) was 9.2 years. The youngest patient dying of renal death was 5.2 years. No sex difference in survival time was noticed. Furthermore no difference in survival time was noted between the different countries. The analysis of the overall survival curve indicates no clear differences between the infantile and adolescent types of cystinosis.

[Haematuria in children. III. Treatment of glomerular diseases (author's transl)]. / Das nierenkranke Kind. III. Therapie der Glomerulopathien.

The treatment of acute Glomerulonephritis is outlined, that of the Nephrotic Syndrome discussed.

Juvenile nephronophthisis and medullary cystic disease.

Juvenile nephronophthisis and medullary cystic disease are morphologically indistinguishable hereditary renal disorders. These diseases have been described independently but very likely are a single disease entity and occur as a juvenile-onset, autosomal recessive form and as an adult-onset, autosomal dominant form. We agree with this hypothesis and present here the clinical, laboratory, and pathologic findings of six cases of the juvenile-onset, autosomal recessive form, along with an analysis of the mode of transmission of these and other published cases of the disorder.