Mollaret's meningitis. A variant of recurrent hereditary polyserositis, both provoked by metaraminol.

Mollaret's meningitis is a rare condition with a characteristic clinical and cerebrospinal fluid picture. In many ways it resembles recurrent hereditary polyserositis (familial Mediterranean fever) in its natural history, pattern of attacks, and response to colchicine. Association of the two conditions has been reported, so far, in two patients only. In our patient the symptoms of both conditions were induced by a metaraminol provocative infusion. We have previously introduced this as a specific diagnostic and confirmatory test for recurrent hereditary polyserositis. The possibility that the two conditions represent different manifestations of a single disease is therefore strengthened.

Helicobacter pylori in dyspeptic patients in Kuwait.

Two hundred and four patients, mainly Arabs, attending for upper gastrointestinal endoscopy at the gastroenterology clinic in Mubarak Al-Kabeer Hospital, Kuwait, were examined for evidence of infection with Helicobacter pylori and associated inflammation. Biopsy specimens of antrum, body, and duodenum; gastric juice; and antral mucosal brushings were investigated by microbiological, cytological, and histopathological methods. Clinical conditions diagnosed at endoscopy included gastritis, gastric ulcer, duodenitis and duodenal ulcer, but half the patients had endoscopically normal gastric and duodenal mucosae. H pylori was detected by one or more of the procedures in at least one specimen from 197 (96.6%) of the patients. Histological and cytological analysis showed equal sensitivity, but bacteriological culture was less reliable. The proportion of positive cases was high, compared with other reported series, which may have been accounted for by the variety of diagnostic techniques used in this study, the selected population (all with gastrointestinal symptoms) or genetic or environmental predisposing factors peculiar to the sample population.

Catecholamine metabolism in recurrent hereditary polyserositis. Pathogenesis of acute inflammation: the retention-leakage hypothesis.

Recurrent hereditary polyserositis (RHP), also known as familial Mediterranean fever, is a genetically-determined disease characterized by paroxysmal attacks of peritonitis, pleuritis, arthritis or inflammation of other serous membranes. We have previously suggested that the pathogenesis of this disease seems to be related to abnormal catecholamine metabolism. This study compares the plasma and urine catecholamine profile in patients with RHP during different clinical states to that in controls. In RHP there were lower plasma and higher urine dopamine levels in the asymptomatic state and during attacks, while norepinephrine levels remain unchanged. However, plasma epinephrine was significantly lower in the asymptomatic state but markedly higher during attacks. The urine epinephrine values in both situations were similar but significantly lower than in controls, suggesting abnormal renal excretion of epinephrine. The urine metanephrine was markedly elevated in the asymptomatic state compared to controls, but remained unchanged during the attacks, again suggesting defective renal clearance of metanephrine. Metaraminol infusion, which induces attacks in RHP patients, was associated with an increase in plasma dopamine and epinephrine (but not norepinephrine); yet the urinary levels of dopamine, epinephrine and metanephrine remained the same, confirming the dissociation between the plasma and urinary levels of these catecholamines, probably due to abnormalities in the renal clearance mechanism. We postulate that this dissociation leads to retention of these amines in the plasma which may subsequently leak through the serous membranes (the target organs) and incite an acute inflammatory process. Colchicine, the only known drug that protects against disease attacks, reduces the plasma levels of these amines, and thus may act by preventing retention that leads to leakage and subsequent inflammation.

Endoscopic features of primary small bowel lymphoma: a proposed endoscopic classification.

Primary small bowel lymphoma is relatively common in Middle Eastern countries. Of 29 such cases, proximal small bowel endoscopy was positive in 26 (89.6%) and, on the basis of endoscopy alone, the diagnosis of lymphoma was confidently made in 24 patients (82.7%). The endoscopic biopsy was diagnostic in 19 of the 26 patients with visible mucosal abnormalities (73%). Four types of lesions have been seen on the basis of which an endoscopic classification is being proposed. This classification may be of therapeutic and prognostic value.

Cigarette smoking and duodenal ulcer healing. An endoscopic study of 197 patients.

Cigarette smoking is suspected to aggravate the symptoms and to delay the healing of gastroduodenal peptic ulceration. The healing rate of duodenal ulcer was endoscopically studied in 82 heavy smokers and compared to that of a nonsmoking control group of 92 patients. The two groups received the antiulcer treatment in a double-blind fashion with one of the following regimens: cimetidine 1 g, trimipramine 50 mg, prostaglandin E1 analogue 800 or 200 micrograms, low-dose antacid or placebo. The smokers' overall healing rate was 45.1% compared to 51.1% in the nonsmokers. The difference between the two groups was not statistically significant. This finding is not in agreement with previous reports showing an adverse effect of smoking on the healing process of duodenal ulcer.

Familial Mediterranean fever (recurrent hereditary polyserositis) in Arabs--a study of 175 patients and review of the literature.

Recurrent hereditary polyserositis (RHP) or familial Mediterranean fever (FMF) is a chronic inherited illness of obscure aetiology. The disease is characterised by paroxysmal attacks of fever, peritonitis, pleuritis or arthritis, and predominantly affects Sephardic Jews, Arabs, Turks and Armenians. In this study, we report our 11-year experience of 175 Arab patients with this disease. As with other ethnic groups, the most common manifestation (93.7 per cent) was peritonitis. Arthritis (33.7 per cent) and pleurisy (32 per cent) were next in frequency. Adult patients in this series unlike those in other ethnic groups, rarely presented with arthritis. Similarly rare were amyloidosis, rashes, splenomegaly, hepatomegaly or lymphadenopathy. The aetiology of this disease is not clear but we suspect that abnormalities in catecholamine metabolism may be a factor in the pathogenesis.

Coeliac disease among children in Kuwait: difficulties in diagnosis and management.

Twenty children with coeliac disease were diagnosed over a five year period in an area with 10,000-12,000 births per year. The average annual incidence was 1:3000 births. All children presented with severe symptoms and rickets was not uncommon (25%). Mean age at onset of symptoms was 38 months (range 6-120) and 72 months at the time of diagnosis (range 13-192), with a mean delay of 34 months. No cases were diagnosed during infancy. The difficulties in the differentiation of coeliac disease from the more common causes of chronic diarrhoea, and problems with diagnosis and management are discussed.

Coeliac disease in monozygotic twin girls. Synchronous presentation.

A pair of monozygotic twin girls with coeliac disease is reported. The diagnosis was made on clinical and biochemical evidence of malabsorption, characteristic histological findings, and clinical, biochemical, and histological response to gluten elimination. Monozygosity was established on finding a single placenta at birth, exact similarity of physical appearance, similar blood group, and histocompatibility antigens, and negative reaction in mixed lymphocyte culture. This is one of six well documented cases of coeliac disease in monozygotic twins and may throw light on the importance of genetic and environmental factors in the causation and expression of the disease.

Metaraminol provocative test: a specific diagnostic test for familial Mediterranean fever.

The diagnosis of familial Mediterranean fever has been one of exclusion. In a placebo-controlled, double-blind, cross-over study a challenge with a 10 mg dose of metaraminol infusion was followed within 48 h by a typical disease-like attack in all of 21 patients with familial Mediterranean fever but in none of 21 control subjects. The induced attacks were milder and of shorter duration than the spontaneous ones. The metaraminol-induced symptoms were similar to the natural disease attacks and could be prevented with prophylactic colchicine therapy. No significant side-effects were observed.

Plasma dopamine beta-hydroxylase: rapid diagnostic test for recurrent hereditary polyserositis.

The diagnosis of recurrent hereditary polyserositis (RHP; also known as familial Mediterranean fever) remains one of exclusion since there has been no specific diagnostic laboratory test. A previous study suggested that the disorder is related to abnormal catecholamine metabolism. Plasma dopamine beta-hydroxylase (DBH) activity was assayed spectrophotometrically in 91 RHP patients and 162 controls. The activity was significantly higher in untreated symptom-free patients and in patients with acute attacks, than in controls (mean [SEM] 155.8 [14.1] vs 43.3 [1.9] mumol/min/1 p less than 0.0001). Colchicine treatment reduced DBH activity to control levels. The test showed a high diagnostic accuracy and specificity for RHP, whether the patient was symptom-free or having an acute attack. Moreover, it is easy to carry out.

Peroral endoscopic duodenal biopsy in infants and children.

Peroral endoscopic duodenal biopsy was used in the diagnosis and follow-up of forty-nine infants and children with suspected small intestine disease. Their ages ranged between one month and 12 years. Fifty-four upper gastrointestinal endoscopy procedures were performed and 139 biopsies were taken. The mean procedure time was 3.2 min with a range of 1.5-5.5 min. There were no complications. Tissue adequate for histopathologic examination was obtained in all but one of the fifty-four procedures. On the basis of this experience we think that peroral endoscopic duodenal biopsy is faster, safer and as diagnostic as conventional suction biopsy in infants and children. It is also more informative when other diseases of the upper gastrointestinal tract are suspected.