[Biochemistry of benign-symmetrical lipomatosis (adenolipomatosis Launois-Bensaude, Madelung's disease)]. / Zur Biochemie der benignen-symmetrischen Lipomatose (Adenolipomatose Launois-Bensaude, Madelung'sche Krankheit)

1. Total lipids, total cholesterol, cholesterol esters, phospholipids, triglycerides and free fatty acids as well as the fatty acids profiles of the different lipid classes were determined in serum, lipomatous and normal adipose tissue. Triglycerides were elevated in patient L's serum. The distribution of serum lipoproteins in this patient's serum showed a type IV according to Fredrickson. All other lipid parameters were within the normal range. Palmitoleic acid was increased nearly in all lipid fractions of the patients' sera as well as in the lipids of lipomatous subcutaneous adipose tissue. 2. The lipomatous adipose tissues of the patients showing no histological abnormalities revealed higher levels of cyclic AMP than normal subcutaneous adipose tissue. 3. Serum uric acid was normal (patient E.), between the normal and pathological range (patient L.) and elevated (patient W.). Urinary uric acid excretion was increased in all three patients. 4. 14C-glycine was overincorporated into urinary uric acid in all three patients. 5. Adenine phosphoribosyltransferase activities in the hemolysates were within the normal range. A decrease of hypoxanthine-guanine phosphoribosyltransferase activities could be demonstrated in two patients' (e., w.) erythrocytes. Erythrocyte phosphoribosylpyrophate synthetase activity was slightly increased in patient L.'s and twice the normal value in patient W.'s erythrocytes.

[Polycythaemia as sole symptom of renal adenoma (author's transl)]. / Polyzythämie als einziges Symptom eines Nierenadenoms bei 2 Kindern.

In two children, a 9 year-old boy and a 10 1/2 year-old girl, who presented with polycythaemia as the only symptom, the expected renal tumour was only found after exclusion of all other causes of polycythaemia. The delay in diagnosis was caused by technically inadequate intravenous urograms, which were erroneously passed as normal. In one child low kv X-ray exposition of the kidneys led to the diagnosis of a renal tumour. In the other child high-dose urography and tomography gave the indication for selective angiography. Normalization of the red blood count postoperatively verifies the connection between preoperative erythrocytosis and the renal tumour. Histologically both cases proved to be renal adenomas, which are extremely rare in childhood.

[Congenital hepatic fibrosis and polycystic disease of the kidneys in two siblings (author's transl)]. / Congenitale Leberfibrose und Zystennieren bei zwei Geschwistern.

Report of 2 siblings, aged 12 1/2 and 9 years, with congenital hepatic fibrosis and polycystic disease of the kidneys. Hepatosplenomegaly had been noted in both children at birth. The younger child had suffered from oliguria aged 2 1/2 years. At diagnosis both children had low platelet counts, one also had leucopenia. The cystic disease of the kidneys was verified by angiography. Coeliacography and splenopartography were diagnostically irrelevant. The diagnosis only became apparent from liver biopsy which was performed during splenectomy. After splenectomy there was an increase of platelets, white blood cells and the clotting factors II, V and X. The three years follow-up showed a constancy of renal impairment and of the minor oesophageal varices observed in the one patient who did not have a spontaneous spleno-renal anastomosis. So far no bleeding has been observed. Porto caval anastomosis was omitted in both children. Pros and cons are being discussed.