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

[Endoscopic sclerotherapy on variceal bleeding in children]

Since variceal bleeding due to portal hypertension is associated with a high mortality rate, effective treatment leads to improved survival. Endoscopic sclerotherapy is an effective treatment for bleeding esophageal varices, but it is associated with significant complications. To determine the efficacy and safety of endoscopic sclerotherapy, we evaluated the patients with variceal bleeding who underwent sclerotherapy in our center. This study was a case-series study in which, we evaluated 79 patients who referred for esophageal variceal bleeding and underwent sclerotherapy and completed the questionnaire. From79 patients, 52 [65.8%] were male, and 27 [34.2%] were female. The rate of success [arrest of acute bleeding by endoscopic sclerotherapy] was 100%, but it was 55.7%for long-terms clerotherapy; 53.3%of patients with liver disease, and 64.7%of patients with extraheaptic portal hypertension had suitable response; 58.3%of patients with Child A, 83.3%of those with Child B and 14.3%of Child C cirrhotic patients demonstrated appropriate response. In fundal varices, 15.2% were improved, 21.5% were accelerated and 43.3% showed no significant changes. During treatment, 31.6%had one of the complications of sclerotherapy. The rates of rebleeding and mortality were 54.4% and 8.9%, respectively. Although sclerotherapy reduced the mortality attributable to acute variceal bleeding, but the long-term effectiveness of sclerotherapy was not very high

[Frequency, clinical findings and outcome of foreign bodies in the upper gastrointestinal tract]

Foreign body ingestion is a potentially serious problem in children. Most of ingested foreign bodies pass through the gastrointestinal tract spontaneously, but some of them [20%] need endoscopic or surgical removal. Sharp foreign object may cause serious complications and need an emergency intervention. The aim of this study was to characterize the clinical features associated with foreign bodies in the upper gastrointestinal tract and to determine the clinical factors for their successful removal by endoscopic techniques. We analyzed retrospectively 120 cases of foreign bodies in the upper gastrointestinal tract at Children's Medical Center, Tehran. Depending on the type of the swallowed foreign body and its site obtained by routine neck and chest x-ray as well as existing symptoms, we decided to fulfill an emergency endoscopy or to wait. Of 120 patients with a mean age of 4 years, 75 [62.5%] were males and 45 [37.5%] females. There was a history of parents' presence as a witness in 93.3% of cases. Common sites of foreign bodies were stomach [66.6%], esophagus [25%], and duodenum [2.5%]; a coin was the most common foreign body [30%]. Other foreign bodies were disc battery [12.5%], and sharp foreign bodies [10%]. Most of patients were brought in the first 24 hours after ingestion of the foreign body. In only 8 patients, complications occurred. Foreign body ingestion in children is a serious problem which may need an emergency procedure. Still the best way is its prevention; children should not be allowed to play with coins, metallic objects, safety pins etc

Prospective evaluation of endo venous laser therapy for varicose vein; early efficacy and complications. The first report from Iran

In recent years, the endovenous laser therapy [EVLT] has been proposed to treat the incompetent greater saphenous veins [GSV] to increase patient comfort, and to reduce cost as well as risk. EVLT causes vein wall thickening, luminal contraction and vein fibrosis. The purpose of this article, as the first report from Iran, is to review our experience and outline the early results and complication of EVLT. 22 patients [13 females and 9 males, mean age: 40.6 +/- 11 years, range: 25-64] underwent EVLT of incompetent GSV segments with 810-nm diode laser with an average energy of 89.2 J/cm [range, 50-123 J/cm]. Success rate was defined as absence of reflux throughout the entire treated segment on follow-up doppler ultrasound [DUS] and clinical resolution of symptoms. Short-term results in the EVLT of 22 GSV indicate a 100% rate of closure. Self-limiting complication were occurred in 18% of patients and included moderate ecchymosis and paresthesias in 3 [13.5%], and 1 [4.5%] patients, respectively. No major complications such as deep vein thrombosis and pulmonary embolism were occurred. DUS demonstrated 21 [95%] and 19 [90.9%] occluded GSVs at 12 weeks, and 24 weeks respectively. The early results of our experiences are excellent. EVLT of the incompetent GSV with an 810 nm diode laser appears to be an extremely safe technique. EVLT is a very effective and safe with best cosmetic results and rare side effects