The clinical and laboratory manifestations of Iranian patients with cystic fibrosis.

Cystic fibrosis (CF) is a hereditary disease, characterized by chronic pulmonary disease, pancreatic insufficiency and abnormal electrolytes in the sweat. In order to evaluate the clinical manifestations and laboratory findings of Iranian children with CF during a 10-year period, 243 CF patients, with a median age of 5 months, were investigated in this study. The most common manifestations were gastrointestinal disorders and respiratory manifestations. Cough was the most common symptom, followed by malnutrition, diarrhea, respiratory distress, and vomiting. The frequency of these findings after treatment was significantly decreased in comparison with the period before diagnosis. During the mean follow-up of 40.9 months, seven cases died due to severe infections. Cystic fibrosis as a common genetic disorder should be considered in any child with recurrent gastrointestinal and respiratory manifestations, since delayed diagnosis could lead to severe complications and even death in this group of patients.

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

AIM: To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. METHODS: 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. RESULTS: 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 PaO(2) 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). CONCLUSION: Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome.

Antimicrobial resistance of Helicobacter pylori isolates from Iranian adults and children.

BACKGROUND: Helicobacter pylori isolates from 84 adults and 51 children were assessed during the period of 2001 through 2004 to find out whether their susceptibilities to metronidazole, clarithromycin, amoxicillin, tetracycline, and furazolidone differ between the two groups or have changed compared with the results from our previous study done between 1997 - 2000. METHODS: Biopsies from 135 dyspeptic patients were cultured on Brucella blood agar. Pure cultures of H. pylori isolates were used for antibiotic susceptibility tests. Disk diffusion method was recruited to assess the susceptibility of H. pylori isolates to different dilutions of the above mentioned antimicrobials. Results obtained from the two groups were compared and minimum inhibitory concentrations determined. RESULTS: Thirty-five percent of H. pylori isolates from adults and 37% from children were resistant to metronidazole. Clarithromycin resistance rate was 2.4% and 5.9% in H. pylori isolates from adults and children, respectively. Amoxicillin resistance was 2.4% in isolates from adults and 5.9% in isolates from children. Tetracycline resistance rate was 0% in H. pylori isolates from adults and 2.0% in isolates from children. Resistance to furazolidone was not observed. CONCLUSION: Resistance rates of H. pylori isolates from adults and children to metronidazole, clarithromycin, amoxicillin, tetracycline, and furazolidone are similar and not significantly affected by age and time.

Low incidence of alpha-1-antitrypsin deficiency in Iranian patients with neonatal cholestasis.

BACKGROUND/AIMS: There is little data concerning the incidence of alpha-1-antitrypsin"(AAT) deficiency, the most common genetic cause of liver disease, among children with neonatal cholestasis in Iran. Thus, this study was performed to analyze AAT deficiency in this group of patients. MATERIALS AND METHODS: DNA samples from patients with neonatal cholestasis were investigated for Pi S and Pi Z alleles, using polymerase chain reaction-restriction fragment length polymorphism. RESULTS: Thirty patients with neonatal cholestasis were enrolled. Among those who underwent biopsies, the results revealed neonatal hepatitis in 19, bile duct paucity in 1, steatohepatitis in 1, bile duct proliferation in 1, cirrhosis in 2, fibrosis in 2, and extrahepatic biliary atresia in 1 patient. No mutant allele was found in any patient. CONCLUSION: The incidence of AAT deficiency is very low in Iran; therefore, screening for AAT is not recommended for patients with neonatal cholestasis in Iran.

Clinical characteristics of Iranian pediatric patients with inflammatory bowel disease.

BACKGROUND: Inflammatory bowel disease (IBD) is a group of disorders, including Crohn's disease (CD), ulcerative colitis (UC) and indeterminate colitis (IC). Small intestine and colon are primarily affected in this group of disorders. PURPOSE: This single center study was performed to define demographic features and clinical characteristics of Iranian pediatric patients with IBD. METHOD: Fifty nine patients with IBD, who have been referred to Children's Medical Center Hospital during a 10-year period, were investigated in this study. The data was gathered by interviewing the patients and their families, as well as reviewing their medical records. RESULTS: Among 59 patients with IBD, 23 cases had UC and 19 cases had CD, while the remaining 17 cases had IC. Patients with UC were significantly younger at the time of diagnosis in comparison with patients with CD. The most common symptoms were abdominal pain, diarrhea, fever and growth failure. Hepatobiliary abnormalities and arthritis were common extra intestinal manifestations. The median Pediatric Crohn's Disease Activity Index was 42.5 (range 20-60), whereas the median Pediatric Ulcerative Colitis Activity Index was 40 (range 20-70). Seven of 23 UC (30.4%) had proctatis, while 16 (69.6%) had extensive colitis. In CD, 11 of 19 (57.9%) had involvement of terminal ileum and colon, while inflammation was limited to the colon in 8 cases (42.1%). The colonoscopic findings in the IC group were heterogeneous. CONCLUSION: This study provides epidemiological data on pediatric patients with IBD, which could be useful for health care workers in prompt diagnosis and appropriate treatment of early onset IBD.