Adenocarcinoma of the colon presenting as polymyositis: case report.

The relationship between polymyositis and malignancy is well known. Several types of tumors can be complicated with myositis and/or more frequently dermatomyositis. It has been suggested that tumors of the large bowel are rarely complicated by myositis. We describe a patient with adenocarcinoma of the colon presenting as polymyositis and review the available literature.

Hereditary hemochromatosis: HFE mutation analysis in Greeks reveals genetic heterogeneity.

Hereditary hemochromatosis (HH) is common among Caucasians; reported disease frequencies vary from 0.3 to 0.8%. Identification of a candidate HFE gene in 1996 was soon followed by the description of two ancestral mutations, i.e., c.845G-->A (C282Y) and c.187C-->G (H63D). To these was recently added the mutation S65C, which may represent a simple polymorphism. The incidence of HH in Greece is unknown but clinical cases are rare. Also unknown is the carrier frequency of the two mutant alleles. A first estimate of the latter is given in the present report. It is based on data from the genetic analysis of 10 unrelated patients of Greek origin who were referred to our center for genotyping and 158 unselected male blood donors. The allele frequencies for the C282Y and H63D mutations were 0.003 and 0.145, respectively. The C282Y allele was detected in 50% of HH patients. This is considerably lower than the frequencies reported for HH patients in the U.S.A. (82%) and France (91 %) and closer to that reported in Italy (64%). Five patients did not carry any known HFE mutation; three may represent cases of juvenile hemochromatosis, given their early onset with iron overload, hypogonadism, and heart disease. We suggest that genetic heterogeneity is more prominent in Southern Europe. It is also possible that the penetrance of the responsible genes is different across the Mediterranean.

Chelation therapy in patients with thalassemia using the orally active iron chelator deferiprone (L1).

BACKGROUND AND OBJECTIVE: Excessive hemosiderosis is the main reason for the multi-organ failure observed in multitransfused patients. Deferiprone (1,2-dimethyl-3-hydroxy-pyridine-4-one, L1) is an orally active iron chelator mainly excreted via urine. We conducted a study in order to determine the efficacy and safety of L1 in Greek thalassemic patients. DESIGN AND METHODS: A group of 11 thalassaemic patients entered the study; L1, the Cipla formulation for deferiprone, at a daily dose of 75-100 mg/kg bw t.i.d. was used. After giving informed consent all patients were subjected to clinical examination and biological tests. RESULTS: All patients tolerated the L1 well; there were no significant side effects (except for slight gastrointestinal disturbances for the first days). The net urinary iron excretion ranged from 6.96 to 26.1 mg/24h. Serum ferritin declined within 4-6 months in most of the patients. INTERPRETATION AND CONCLUSIONS: The results suggest that L1 is a rather safe drug which decreases iron overload without causing any considerable side-effects in Greek thalassemics.

Postanginal sepsis syndrome with metastatic lung abscess in a young man.

A postanginal Sepsis Syndrome with metastatic lung abscess caused by Fusobacterium necrophorumin a 25-year-old previously healthy man is described. The incomplete and ineffective antibiotic treatment at onset of angina ended progressively in septicaemia and metastatic infections in a 3-week time period. The early parenteral use of Metronidazole based only on the clinical picture, the Gram stain findings and the strict anaerobic feature of the blood isolate in parallel with the long-term antibiotic treatment were possibly the main reasons for the good outcome of this serious infection.

HLA antigens and Adamantiades-Behçet's disease (A-BD) in Greeks.

The distribution frequency of HLA-A and B antigens was determined in 24 Greek individuals with Adamantiades-Behçet's disease (A-BD) as well as in 280 controls. The standard NIH technique was used for tissue typing. There was an increased incidence of HLA-B5 in the patients group (75% compared to controls 32%). The calculated P corrected value was Pc less than 0.02, the relative risk (RR) was 6.23 and the aetiological fraction (EF) 0.68. This study agrees with the reported association between A-BD and HLA-B5 in other populations from the Mediterranean basin, the Middle East and Far East.