Alpha-chain disease presenting as malabsorption syndrome with exudative enteropathy (author's transl)

A 30-year-old Turk was admitted with signs of exudative enteropathy together with malabsorption. There was no improvement on a gluten-free diet. Immunological investigations demonstrated atypical IgA-immunoglobulin in the serum which did not precipitate with antisera against L-chains. Peroral ileal biopsy and surgical biopsy material showed a diffuse proliferation of plasma cells in an altered ileal mucosa and in the mesenteric lymph nodes. Skeletal X-rays showed no osteolysis and the bone marrow showed no evidence for multiple myeloma. Treatment with melphalan and steroids resulted in a three year remission. In the terminal stage an intra-abdominal malignant lymphoma developed.     

Heterologous fibrinogen for tissue adhesion. immunological and histological investigations (author's transl)

The immunological reactions of heterologous fibrin were investigated in a sensitive experimental system. In 10 rabbits autologous full-thickness skin grafts were glued with bovine fibrinogen cryoprecipitate. 5 of these animals had been immunized against this adhesive before transplantation. In a control group the graft was fixed with homologous fibrinogen cryoprecipitate. Examination of the sera of the animals for antibodies against fibrinogen was performed before immunization, 1 day prior to and 14 days after skin grafting. 14 days after skin adhesion all grafted areas were examined by histological methods. In the group without preoperative immunization antibodies were found against fibrinogen. The skin graft healed in this series in the same manner as in the sham group. In the series with preoperative immunization the glued skin graft was discharged early and the healing procedure of the defect was retarded.     

Recto-sigmoid carcinoma after uretero-sigmoidostomy. One case, with a review of the literature (author's transl)

A case of recto-sigmoid adenocarcinoma, developing 25 years after bilateral uretero-sigmo dostomy for extrophia vesicae in a 29 year old man. A study of the literature emphasises the long delay before its appearance, the gastrointestinal nature of the tumour and its grave prognosis. The pathogenesis of this lesion is unknown at present, and consideration should be given to the effects of urine on the digestive mucosa and the role of a possible inflammatory granuloma, but also the possibility of a urinary tract origin, despite the apparent histological contradiction.     

Hyperoxaluriaas a complication of intestinal diseases (author's transl)

Urinary oxalate excretion was measured in healthy persons and patients with Crohn's disease, colitis ulcerosa, sprue and other diseases accompanied with malabsorption, and patients with insufficiency of the exocrine pancreas gland. Further measurements were made in patients after resection of parts of the small intestine or the colon. We found a clear increase of urinary oxalate excretion in patients with resected parts of the small intestine, sprue or other malabsorption syndromes. In 4 patients with resected parts of small intestine or pancreas we even found urolithiasis. Urinary oxalate excretion correlated significantly with steatorrhoea and increased if larger parts of small intestine were resected. Increased resorption of oxalate from food causes increased urinary excretion. Details about the patho-mechanism of this increased excretion are not known yet; an important factor seems to be the reduced absorption of fat in the small intestine.     

Melorheostosis: study of a case (author's transl)

The Authors describe a case of melorheostosis. The diagnosis, which is only radiological, is characteized by the tipical aspect of flowing hyperostosis.     

Acute post-operative hemolysis related to a G6DP deficiency. Report of one case (author's transl)

Many drugs used during resuscitations, are mighty oxidants able to induce intravascular hemolysis in patient with G6PD deficiency. Reversible and not immediately recurrent, hemolysis should be avoided by systematic (prevention) using specific blood tests. This paper was suggested by the case of a 37-year-old (Malgache) patient, with no history sustaining an acute hemolysis with kidney failure, appearing after intra-operative phenothiazine injection.     

Rendu-Osler-Weber disease. Report of a clinical case

Rendu-Osler-Weber disease is an hereditary disorder characterized by cutaneo-mucous telangiectasis and vascular abnormalities in several organs. Bleeding, especially epistaxis, represents the most important clinical feature. Pulmonary arteriovenous fistulae can cause hypoxaemia, haemoptysis, polycythaemia and clubbing. Diagnosis is based on family and personal history, teleangiectasis, laboratory (haemochrome, fibrinogen, PT, PTT) and instrumental findings (endoscopy and/or roentgen). Therapy depends on symptoms. Embolization of pulmonary arteriovenous fistulae and laser treatment of intestinal vascular abnormalities have been successful. Danazol treatment yielded controversial results. We report the case of a patient admitted for arterial hypertension and recurrent epistaxis. Rendu-Osler-Weber disease diagnosis was made based on positivity at family and personal history, clinical examination, laboratory and instrumental findings. In conclusion we underline the pivotal role of anamnesis and clinical examination in the differential diagnosis of hereditary bleeding disorders and emphasize the importance of early diagnosis for the correct therapeutic approach.