[Whipple disease. Formerly dim prognosis--often treatable today].
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Biomedical subjects
Publications and source records attributed to J Riederer.
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HISTORY AND FINDINGS: A 21-year-old woman developed increasing jaundice with severe pruritus and weight loss after a bout of dyspepsia. She had been taking contraceptives for 4 years (ciproteronacetate 2 mg, ethinylestradiol 0.035 mg). INVESTIGATIONS: Laboratory tests at first suggested cholestatic hepatitis (serum bilirubin > 5 mg/dl, predominantly indirect bilirubin, SGOT 77 U/l, SGPT 154 U/l, gamma-GT 138 U/l, alcaline phosphatase 630 U/l). Ultrasonography showed a gall bladder filled with biliary sludge and dilatation of the common bile duct and the smaller biliary passages. A tumour-like space-occupying lesion was noted near the papilla: it was not fixed and had no vascular supply. Endoscopic retrograde cholangiopancreatography (ERCP) also demonstrated a mobile space-occupying lesion in the common bile duct near the papilla and markedly dilated biliary passages. DIAGNOSIS, TREATMENT AND COURSE: The tumour-like obstruction was removed by balloon catheter after papillotomy. It consisted of several jelly-like viscous streaky bile without calculi. The procedure was complicated by subsequent pancreatitis which, however, soon subsided. Within a few days the jaundice disappeared and the pruritus ceased. At the same time the liver functions returned to normal. Ultrasonography no longer showed obstructed biliary passages and sludge. CONCLUSIONS: Obstructive jaundice may be due not only to strictures gall stones and benign or malignant tumours but also to thickened sludge in the biliary tract in women on oral contraceptives. The obstruction can be demonstrated by ERCP and removed by interventional procedures (i.e. endoscopic papillotomy).
HISTORY: At the age of 32 a "benign" monoclonal gammopathy of lightchain kappa with Bence Jones protein is diagnosed in a man born 1934. In addition a Noonan-syndrome is found. COURSE: Twenty-four years later he gradually develops a chronic lymphatic leukaemia (B-CLL) which up to now does not need treatment (October 1996). The neoplastic B-cells exprime monoclonal lightchain lambda on the cellmembrane and in the cytoplasma undetectable by immunefixation in the serum. Irrespective of that the known monoclonal gammopathy exprimes IgG-kappa without an increase in the number of plasmacells in the bonemarrow. CONCLUSION: There are hints that the congenital Noonan-syndrome can be associated with B-cell disorders.
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This is a report on a 51-year-old man with all the symptoms of a non-tropical pyomyositis, namely a prodromal stage with lassitude, associated with a high fever and diffuse joint and muscle pain followed by the gradual development of a full-blown invasive stage. In the suppurative stage, the extensive pus is caused by staphylococcus aureus located in the extensor muscle of the right thigh.
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This report describes a variant form of lipase found in a patient with cryptogenic liver cirrhosis. Serum lipase in this patient showed persistently increased activity with simultaneously normal activity of amylase. Results of exclusion chromatography demonstrate that the lipase activity in the serum of this patient eluted as a macromolecule. Since macromolecular complexes were not fixed by protein A, it seems unlikely that lipase is attached to IgG. Tests of the sera from 20 patients with raised serum lipase activity in acute pancreatitis or an acute episode of chronic pancreatitis revealed, in two patients, that a small but reproducible proportion of the total lipase activity eluted in the region of the macrolipase. In addition, 10% and 18% of the total lipase activity was found in the elution region of the macrolipase in two commercial pooled sera used for quality control. The results show that, in rare cases, macrolipasemia must be considered a possible cause of raised serum lipase activity.
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