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Two and a half years after manifestation of treatment-refractory lipoatrophic diabetes a 16-year-old girl had blood-sugar levels of about 500 mg/dl and hypertriglyceridaemia with fasting levels of about 3000 mg/dl, while there was no increase in ketone bodies. All the clinical, histological and radiological findings were those of generalized fatty tissue atrophy. In addition, she had marked axillary and periorbital acanthosis nigricans. Main symptoms were fatigue, weakness and excessive appetite. Intravenous insulin of at first 1200 IU daily reduced blood-sugar levels to normal. A good metabolic state was maintained by intensive insulin treatment with four intramuscular injections daily. On a dosage of 600-700 IU daily the HbA1 value dropped from 16.7% to 7.8%, triglyceride concentration to 300-400 mg/dl. The symptoms also regressed with normalization of the metabolic state.

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EinleitungDer Bence-Jones Eiweißkörper (BJE) war während der letzten Jahre vielfach Gegenstand klinischer und experimenteller Untersuchungen, wobei die Häufigkeit seines Vorkommens bei paraproteinämischen Erkrankungen, insbesondere seine chemische und physikalische Beschaffenheit interessierten (1,

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