[Systemic mastocytosis with portal hypertension and hepatocellular failure]. / Mastocitosi sistemica con ipertensione portale e insufficienza epatocellulare.

Systemic mastocytosis is a rare disease of mast cell proliferation with cutaneous and multi-visceral involvement. Portal hypertension and ascites are rare manifestations of systemic mastocytosis. We report a case of systemic mastocytosis presenting with extensive nodular cutaneous lesions and hepatic dysfunction, manifested by portal hypertension (ascites, splenomegaly) and derangement of metabolic function (hyperammonemia, hypoalbuminemia, hypocholesterolemia), a picture resembling that of a common cirrhotic form. The correct diagnosis was established only after tissue sections were appropriately stained for mast cells. On the basis of our and other observations we suggest that systemic mastocytosis be added to the list of infiltrative diseases of the liver with potential evolution to portal hypertension and compromise of biochemical functions.

[Idiopathic retroperitoneal fibrosis: effectiveness of surgery combined with corticosteroid therapy]. / Fibrosi retroperitoneale idiopatica: efficacia della terapia associata chirurgica e corticosteroidea.

Idiopathic retroperitoneal fibrosis (ERF) is a rare disease characterized by non-neoplastic fibroblastic and inflammatory proliferation. We describe a case of a 58-year-old man presenting with abdominal pain, weight loss and abdominal mass on clinical examination. The patient was evaluated by abdominal CT scan and magnetic resonance imaging which disclosed a periaortic soft-tissue mass with marked longitudinal extension below renal arteries origin. The diagnosis of ERF was histologically confirmed after surgical exploration and biopsies followed by extensive ablation of fibrous tissue. After 6 months of low-doses of postoperative steroid therapy retroperitoneal fibrous tissue had almost disappeared on CT examination.

Plasma myoglobin in the early diagnosis of acute myocardial infarction.

Serum and plasma myoglobin and creatine kinase-MB catalytic activity were analysed in 157 patients admitted within 2 hours of the onset of chest pain (58 were retrospectively recognized as acute myocardial infarction). Serum and plasma values were highly correlated for both myoglobin and creatine kinase-MB. Plasma myoglobin appeared to be more sensitive than creatine kinase-MB for the early diagnosis of acute myocardial infarction; using a cut-off value of 100 micrograms/l, 90% of acute myocardial infarction cases were correctly recognized by plasma myoglobin 6 hours after the onset of chest pain, with a diagnostic specificity of 100% for non-acute myocardial infarction chest pain subjects. Plasma creatine kinase-MB showed a diagnostic sensitivity of 62% and a diagnostic specificity of 95% in the same group of patients. We suggest the inclusion of the plasma myoglobin immunonephelometric assay together with plasma creatine kinase-MB activity analysis in protocols for the early diagnosis of acute myocardial infarction.