[Prevalence of Helicobacter pylori infection in children with chronic immune [idiopathic] thrombocytopenic purpura and evaluation of Helicobacter pylori eradication on platelet count]

Recently, the high prevalence of Helicobacter pylori infection has been reported in adult patients with chronic immune [idiopathic] thrombocytopenic purpura. Furthermore, after Helicobacter pylori eradication therapy in such patients, their platelet counts have been observed to increase, suggesting that Helicobacter pylori may be a causative agent of adults' chronic idiopathic thrombocytopenic purpura. However, there have been only a few reports of this subject in children with chronic thrombocytopenic purpura. The purpose of this study is to determine prevalence of Helicobacter pylori infection in Iranian children with chronic thrombocytopenic purpura and role of Helicobacter pylori eradication in rising platelet count of these patients. This descriptive-clinical trial study was performed in 31 children under 14 years old with chronic thrombocytopenic purpura who attended hematology ward of Mofid paediatric hospital. After determining platelet count, and filling the results patients referred to gastrointestinal ward of the hospital to perform urea breath test for evaluation of Helicobacter pylori infection, then Helicobacter pylori-infected patients who were diagnosed by this test, received eradication therapy using triple therapy regimen [containing Omeperasole, Amoxicillin and Clarithromycin] for 2 weeks and their platelet counts were recorded during the follow up period. Mean age of the patients was 8.9 +/- 3.2 years old ranging from 3.5 to 14 years old. They were 17 [54.9%] girls and 14 [45.1%] boys. Mean platelet count of the patients was 51.4 +/- 34.3x10[9] / L ranging form 125x10[9]/ L to 8x10[9] / L. Mean duration of disease in the patients was 27.7 +/- 20.2 months ranging from 7 to 96 months. Helicobacter pylori infection was found in only 4 children [12.9%] and Helicobacter pylori eradication therapy was not effective in rising platelet count to achieve complete or partial remission. Comparing Helicobacter pylori-positive and negative patients, there were no significant differences regarding their age, platelet count and duration of disease. This study shows that prevalence of Helicobacter pylori infection in children with chronic immune thrombocytopenic purpura is less than that is in adults. Furthermore, we have found that platelet count in Helicobacter pylori-positive children have not been risen after eradication therapy. We suggest that more studies in different gender groups and different zones in the world with more number of samples should be performed, especially in children in order to determine both the exact role of Helicobacter pylori's pathogenesis in developing the chronic idiopathic thrombocytopenic purpura and the effectiveness of eradication therapy in rising platelet count in these patients

Griscelli Syndrome; A case report and review of the literature

Griscelli syndrome [GS] is a rare disease first described in 1978. It is inherited in autosomal recessive pattern. This disease is characterized by partial albinism, pigmentation dilution, cellular immunodeficiency, neurological involvement and uncontrolled phases of macrophage and lymphocyte activation. We report a 5 months Old Iranian girl presenting with silver-gray hair, eyelashes and eyebrows, hepatosplenomegaly, pancytopenia, hemophagocytosis and progressive neurologic deterioration. Griscelli syndrome can be suggested according to her symptoms. The chemotherapy was not effective for her and she died due to multi organ failure

[Renal tubular function in children suffering from iron deficiency anemia]

Background: iron deficiency anemia is the most prevalent blood disease during infancy and childhood and iron deficiency even in the absence anemia could negatively impress the growth and development. Iron deficiency anemia causes the destruction of proximal tubules and extension of peritubular space, especially in the cortex of kidneys. Hypoxia is assumed as the main cause of these changes. According to some studies, treatment with supplemental iron has led to renal function reach it's normal level. We planned our study to to investigate the tubular function in children suffering from iron deficiency anemia in Mofid Children's Hospital

Material and Methods: we performed our descriptive cross sectional study on the children admitted in Mofid Children's Hospital for any reason and the same time suffering from iron deficiency anemia. Via continuous visits and census, 60 children aging between 3-60 month who met inclusion criteria, entered the study and investigated for probable defects in tubular reabsorption of Na,K,Ca, Phos and proteins. Collected data were analyzed by SPSS version 11

Results: 52[87%] patients who entered the study had at least one indicator of tubular dysfunction. While the most common tubulopathy was protein losing [65%], the most uncommon was impaired reabsorption of k+ [p<0.05].Among criterias for iron deficiency anemia, total iron binding capacity[TIBC] was higher in all patients with at least one tubular dysfunction in comparison with patients without such an impairment. The difference between groups was statically significant for defects in reabsorption of Na+, K+ and Phos. ions. Tubular protein losing was more prevalent in younger patients and defects in reabsorption of K+ ion, was more common in patients with lower weight

Conclusions: according to high prevalence rate of tubular dysfunctions among those children suffering from iron deficiency anemia and lower age and weight of these patients; to avoid renal dysfunctions, it is reasonable to start the treatment as soon as possible