فهرست مطالب gholamhossein falahi

Adenosine, a signaling nucleoside, is controlled in part by the enzyme adenosine deaminase (ADA). There are rare reports on the role of adenosine levels and ADA in cystic fibrosis (CF) patients.
The aim of this study was to assess serum ADA in CF patients in order to find whether the severity of lung disease in CF is related to significant changes of ADA or not.
Patients and Venous blood serum ADA was measured in CF patients (3-15 years) and 49 healthy children (3-15 years) referred to Children’s Medical Center. Classification of respiratory and gastrointestinal disease severity in CF patients as well as Body Mass Index (BMI) was performed. The results were compared with values obtained from healthy children matched for age and gender.
This study included 49 children of both genders (20 females and 29 males) with CF (mean age: 6.36 ± 2.22 years). Mean serum ADA in CF patients group and control group was 9.38 ± 2.72 and 16.04 ± 1.27, respectively (P value = 0.001). Mean serum ADA in CF patients with normal BMI was higher than in patients with low BMI (P value = 0.002).
In this study the lower serum level of ADA was seen in CF patients compared to control group. The clinical symptoms, especially respiratory symptoms, in CF patients might be associated with reduction of serum ADA and rising serum adenosine; therefore, further studies on the use of ADA enzyme therapy in CF patients are highly recommended.

In diagnosing inflammatory bowel disease، one of diagnostic way is upper gastrointestinal endoscopy، which helps in differential diagnosis of unspecified colitis as well. The aim of this study was to investigate the necessity of upper gastrointestinal endoscopy in patients with inflammatory bowel disease. In this descriptive cross-sectional study، 30 children with definite diagnosis of Crohn''s disease and ulcerative colitis underwent upper endoscopy in Medical Center Hospital from October 2009 to March 2011 and the data were analyzed using SPSS software and T-test. Of 30 children with inflammatory bowel disease، 15 patients were diagnosed with Crohn''s disease and fifteen patients with ulcerative colitis. The present results demonstrated that esophageal، stomach، and duodenum involvements in patients were 93%، 87%، and 26%، respectively. Esophageal، stomach، and duodenum involvements with ulcerative colitis were observed in 80%، 66%، and 20% of patients، respectively. Patients’ involvements with Crohn''s disease were in esophageal (94%)، stomach (80%)، and duodenum (47%). Regarding the high percentage of upper gastrointestinal involvement in patients with inflammatory bowel disease، endoscopy and histopathological examinations seem necessary for all patients irrespective of the type and locality of involvement

Shwachman-Diamond syndrome (SDS) is a rare autosomal recessive disorder which presented with multiple organ involvement including exocrine pancreatic dysfunction and bone marrow failure. Liver involvement is less common sign and may be missed. In this study we report an infant with SDS and liver involvement. Case report: An 11 month old infant boy, with acute diarrhea, dehydration and disorders in weight gaining and fatty defection was referred to hospital when he was 4 months old. He was checked because of his big liver and increment of liver enzymes. Considering the fatty stool, osteopenia, patient's abnormal face, appearing neutropenia and being dropped the fibreosis cystic disease diagnosis, so Shwachman diamond syndrome was diagnosed Introducing this patient beside diag nosis this rare diseases which will be result in retardation of children growth, also recall the liver involvement sometimes results in initial manifestation of growth retardation.

There is a well-recognized relationship between aplastic anemia and viral hepatitis. Clinically apparent hepatitis precedes aplastic anemia by a period of weeks to months. Hepatitis is an infrequent cause of aplastic anemia and is usually severe and fatal if untreated. The clinical features and, particularly the response to immunosuppressive therapy strongly suggest that immune mechanisms mediate the marrow aplasia. The cause of the hepatitis is unknown, but it does not appear to be due to any of the known hepatitis viruses. In this study we present two cases of hepatitis associated aplastic anemia (HAAA) at the ages of 10-11 years old. They both received immunosuppressive therapy, Anti thrombocytic globulin and Cyclosporine. They achieved a persistent clinicohematological remission.