Systemic lupus erythematosus and periodic peritonitis (FMF)
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Biomedical subjects
Publications and source records attributed to F Bakir.
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Short-course chemotherapy has not been previously assessed in tuberculous serositis. In this study, 18 patients with tuberculous serositis were studied. 600 mg of rifampicin, 300 mg of isoniazid and 25 mg of pyridoxine were given daily for 6 months. 14 patients completed the therapeutic regimen and were evaluated. 11 of these showed excellent results on the long-term follow-up (18-24 months) and 3 of shorter-term follow-up (9-12 months). Short-course chemotherapy appears to be efficacious in the treatment of tuberculous serositis. However, further and larger trials are recommended.
Primary lymphomas of the small intestine (PIL) show definite racial and geographic distribution. Clinical and laboratory data on 132 patients with PIL treated and followed up over a 14-year period in Baghdad, Iraq, are presented. Based on the pattern of involvement of the bowel and the histopathologic appearance of the tumor, the patients can be divided into three main groups. Ninety-seven patients had diffuse lymphoplasmacytic infiltration affecting large segments of the upper bowel. Abdominal pain, anorexia, weight loss, diarrhea, malabsorption, and clubbing of the fingers are the most common clinical findings. The barium appearance of the small intestine and peroral jejunal biopsy specimens are abnormal in nearly all cases. This clinicopathologic entity has been referred to in the literature as Mediterranean lymphoma (ML). Ten of 34 patients tested had free alpha-heavy chain in the serum. Twenty-three cases had other "Western" variants of lymphoma (18 lymphocytic and 5 plasmacytic). The lesions were localized, occurring most frequently in the lower ileum or ileocecal area. The most common presentation was intestinal obstruction. Twelve children had Burkitt's lymphoma (BL), presenting most commonly with abdominal masses and/or intestinal obstruction. These patients responded poorly to cyclophosphamide, and the disease disseminated early and extensively. None of the patients with the localized lymphomas or BL had free alpha chain in the serum.
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The incidence of chronic cor pulmonale in Iraq has been studied for the first time. Of the total 1,102 cardiac cases admitted to the Medical City Hospital in a 1-year period, 168 patients belonged to this group (15.25%). The most frequent cause is chronic obstructive airway disease. Smoking is thought to be an important factor in the occurrence of obstruction. Chronic and repeated infections during different phases of life are thought to play a major role in the pathogenesis. Occupation and dust probably have no significant effect. Schistosomiasis and tuberculosis are rare causes.
The clinical, laboratory, and radiological features of 32 patients with primary gastric lymphoma are reported. These tumours constitute about 9% of gastric neoplasms in Iraq. As a group our patients seem to present at an earlier age and with more extensive disease at the time of diagnosis than those with other gastric neoplasms. Surgical excision with chemotherapy and/or radiotherapy seems to offer the best chance of long symptom-free survival.
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An opportunity to study the effects of methylmercury poisoning in humans was provided by the large outbreak in Iraq in 1971-2. In adults, poisoning resulted from the ingestion of home-made bread prepared from methylmercury-treated seed grain and there was a highly significant correlation between the amount of bread ingested and blood mercury levels. Poisoning in infants resulted either from prior exposure in utero or from suckling or both. Blood mercury levels were higher in infants and children than in adults. There was no increased incidence of congenital defects. Symptoms and signs of poisoning and histopathological changes were mainly confined to the CNS. Symptoms developed, on average, 1-2 months after exposure. In children there was mental retardation with delayed onset of speech and impaired motor, sensory and autonomic function. Severely affected children were blind and deaf. In adults, the clinical picture could be classified as 1, mild (mainly of sensory symptoms) 2, moderate (sensory symptoms accompanied by cerebellar signs) and 3, severe (gross ataxia with marked visual and hearing loss which, in some cases, progressed to akinetic mutism followed by coma). Grades 1 and 2 carried a better prognosis thant grade 3. Interference with transmission at the myoneural junction was found in 14% of patients studied. There was no evidence of peripheral nerve involvement per se and sensory symptoms may be of central origin. The clinical differences between the Iraqi and Japanese outbreaks may be a result, in part at least, of the severe, prolonged and continuous exposure which occurred in the latter outbreak. Improvement was observed among the mild and moderate group. Treatment with chelating agents, thiol resin, haemodialysis and exchange transfusion lowered blood mercury concentrations but produced no convincing clinical benefit. To be effective, treatment may need to be instituted soon after exposure.
Thirty-three patients with periodic peritonitis were treated with colchicine for 863 patient-months of observation (average, 27 months). There is a personal optimum dose, and the daily requirement varies at 1 or 1.5 mg. All patients responded to treatment. Episodes were precipitated in all patients who stopped their medications. No side effects were noticed. Four normal full-term infants were born to patients taking colchicine during the study.
The clinical and pathological features of 18 new patients with alpha heavy chain disease seen at two referral centres in Baghdad, Iraq, are described. The series included 14 males and four females ranging in age from 14 to 47 years. Almost all patients presented because of long-standing abdominal pain and diarrhoea. The tissue diagnosis and extent of the disease were established at laparotomy in most patients. Peroral jejunal biospy was used in a number of patients, mainly for follow-up. The serological abnormality was confirmed by immunoselection technique. Most of the patients had extensive thickening of the bowel wall and/or tumour masses of the small intestine and mesenteric nodes. Histopathological sections showed muscularis. Preliminary results of the treatment, including two long remissions, are reported. In general, our observations agree with those made by other authors, mostly from the Middle East and Africa. We believe that a high index of clinical suspicion, routine use of the immunoselection, and recognition of the early pathological changes may hopefully lead to the detection of more cases before the frank neoplastic phase of the disease.
Fifty cases of lymphocytic exudate of the pleura and peritoneum were studied to determine the cause. Though tuberculosis was suspected provisionally in the majority, isolation of M. tuberculosis was rare and other investigations proved more helpful. Histological examination of material obtained by laparoscopy was the most reliable method followed by pleural biopsy, especially if it was an open one. This procedure has the invaluable advantage of a rapid diagnosis without the need to await the results of culture. The specific gravity and the cell count were of help to determine the exudative nature of the fluid. The protein content, the lactic dehydrogenase value of the fluid and its ratio to the blood value were more useful in determining a possible aetiology. A definite diagnosis of tuberculosis was made in 21 cases. A further 13 cases (3 with highly suggestive histological appearances) were judged to be tuberculosis in view of their suggestive history and excellent response to specific antituberculosis chemotherapy. Eleven cases proved to be due to malignant disease.
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Secondary sources of intoxication with methylmercury poisoning other than home-made bread were explored. Mercury levels in various food items were below 0.05 mg/kg.Over 30 000 specimens of meat and internal organs of sheep were analysed because of the strong suspicion that the animals had been fed treated barley. It was found that 6-12% of the specimens analysed contained more than 0.5 mg/kg of mercury. Consumption of meat did not appear to constitute a danger of intoxication during the outbreak.
Twenty-six patients suffering from methylmercury poisoning were treated by different therapeutic regimes. Seven received penicillamine or N-acetyl-d-L-penicillamine, 10 were treated with dimercaprol sulfonate and 9, who could not be treated, were given a placebo and were used as controls. Penicillamine, N-acetyl-d-L-penicillamine, and dimercaprol sulfonate reduced the blood level of mercury and increased its urinary excretion. No appreciable clinical improvement was noticed among the severe or very severe cases, while mild or moderate cases improved clinically irrespective of the treatment given.
Fifty-one autopsy specimens of liver were analysed for total mercury. Thirteen specimens contained less than 10 mg/kg of mercury, with a minimum value of 1.4 mg/kg, indicating that death in suspected cases was not always due to lethal exposure to methylmercury. The methylmercury concentration in 28 livers was 10-30 mg/kg. Limited additional estimations have shown that 71% of the liver mercury was organic and that the level of mercury in the liver of a 7-month-old fetus was only 25% of that in the liver of the mother. In a patient who died in hospital with a blood mercury level of 4.1 mug/ml, the liver contained 16.5 mg/kg of mercury.Differences between these results and those found in the outbreak of methylmercury poisoning in Japan are discussed. Any extrapolation of tissue mercury levels in relation to the toxic effects of methylmercury must take account of the intensity and duration of exposure.
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