Characteristics of patients with cystic fibrosis: experience in a large referral children's hospital in Tehran, Iran

Cystic fibrosis [CF] is an autosomal recessive disease caused by a CF trans-membrane regulator [CFTR] defect. Its prevalence is 1:2500 in Caucasians, 1:15300 among African Americans and is rare in Southeast Asia. The present study aims to review demographic data, clinical manifestations and laboratory findings of Iranian children diagnosed with CF who referred to a Children's Hospital Medical Center in Tehran, Iran during a ten-year period. In a retrospective study from 1991-2000, all hospitalized patients with documented CF were reviewed. Diagnosis was based on clinical findings and sweat chloride levels above 60 mEq/L. A total of 233 patients [females: 91 [39.1%], males: 142 [60.9%]] were enrolled. The onset of symptoms was before the first month of life in 12.1%, between 1-6 months of age in 75.1%, and between 6-12 months of age in 6.9% of patients. Consanguinity of parents was present in 42.5% of patients. Respiratory [81.5%] and gastrointestinal [73.4%] symptoms, in addition to growth retardation were the most common presentations Eighty-eight percent of patients weighted below the fifth percentile. Of the 207 chest radiographs performed, the most frequent finding was hyper-aeration associated with pneumonia. Among 138 patients in whom barium swallows were performed, 102 [74%] had gastroesophageal reflux. A total of 27 patients expired, mostly from respiratory failure [96.3%]. CF is not a rare disease in Iran. We suggest early diagnosis and appropriate maintenance therapy for improving morbidity and mortality amongst CF patients

Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome

To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome [confirmed by contrast echocardiography] at the dosage of 1 g/1.73 m[2] per day. Patients were evaluated clinically and by arterial blood gas every four weeks. The garlic capsule was administered to 15 patients with hepatopulmonary syndrome. There were 10 boys and 5 girls with a mean age of 9.4 +/- 3.9 years. The underlying problems were biliary tract atresia [4 patients], autoimmune hepatitis [4 patients], cryptogenic cirrhosis [4 patients] and presinusoidal portal hypertension [3 patients]. Eight patients [53.3%] showed an increase of 10 mmHg in their mean arterial oxygen pressure. The baseline PaO2 was 65.6 +/- 12.1 mmHg in the responder group and 47.1 +/- 11.2 mmHg in non-responder group. At the end of treatment the mean PaO[2] in responders and non-responders was 92.2 +/- 7.75 mmHg and 47.5 +/- 11.87 mmHg, respectively [P < 0.01]. Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome