فهرست مطالب sh ansari

A fungal infection represents a growing problem in children with hematologic malignancies,during chemotherapy induced neutropenia.Fungal colonization is considered a major riskfactor for subsequent fungal infections. The aim of this retrospective study was to evaluate prevalence of fungal infection among children admitted to hospital between 2005 and 2010 in Tehran, Iran. 617 hematological patients in the age range of neoteric to 19 years old were enrolled and 87 cases with invasive fungal infections were extracted from patients'' files and documented. Diagnosis of fungal infections was based on the blood culture, urine culture and clinical examination for candidasis and galactomannan for aspergillus. the mean age of cancer diagnosis was 6.33 years old and the mean age of fungal infection was 7.95 years old. The majority of the infections was caused by candidia spp (74.7%), followed by aspergillus spp (17.2%) and zygomycetes (11.5%). Among candidiasis patients, oral infection had the highest manifestation (92.3%) whereas in 10 of 15 patients with aspergillus, the infectious site was the lung. There was a significant association between mortality and the type of fungal infection (p <0.0001). Our finding suggests that there is a high rate of fungal infections in children receiving remission therapy for onco-hematology. These results help improve the management of these patients, however Further studies are needed.

There is a risk of viral hepatitis for children with leukemia. Both hepatitis B and C virus infections cause major problems in the management of leukemic patients. In this study, we evaluated the prevalence of HBV and HCV infections in children with leukemia receiving chemotherapy and blood products. 408 patients with leukemia (mean age=5.1 years) were screened for HBV and HCV in Ali Asghar Children''s Hospital. Liver function tests, the number of transfusions, HBV and HCV serology were regularly monitored. In seropositive children, HBV-DNA and HCV-RNA were measured. Liver biopsies were performed in all patients with chronic hepatitis and the data of the study were analyzed using SPSS software. HBsAg positivity, anti-HCV, and mixed HBV and HCV infections were found in 10 cases (2.5%), 8 cases (2%), and 1 case (0.2%) of children, respectively. Of HCV infected children, 8 had positive HCV-RNA. Mixed infection developed to chronic hepatitis in one case. Children with cancer are at a high risk for hepatitis B and C infections due to immunosuppression secondry to chemotherapy and multiple transfusions of blood products during the course of their disease. We observed an increasing incidence of chronic hepatitis B and C infections. Thus no patient developed any signs or symptoms of decompensated liver disease and did not suffer from any severe liver impairment over 8 to 20 years.

Beta thalassemia major is a severe transfusion dependent anemia that causes infertility due to iron deposition to endocrine organs after overtransfusion. Very few pregnancies were reported among such patients in the past. However, after modern therapies, patients who have been receiving a prolong intensive treatment with hyper transfusion and iron chelation become spontaneously pregnants. Patients & The present study was conducted on 32 thalassemia women who were admitted to Ali Asghar children’s Hospital and the Thalassemia Clinic, and were concepted spontaneously. The objective of this cross-sectional study was to estimate the frequency of fertility (spontaneous ovulation or ovulation induction) and complications for mother and newborn including: cardiac failure, endocrinological and hepatic parameters monitored at the beginning, throughout and after pregnancy, viral infection, term and preterm deliveries and complication of pregnancy. All the files were studied and data was analyzed with SPSS software. The checklists were gathered and the findings showed that 12 babies were delivered by elective cesarean section and the others were vaginally delivered. The mean brithweight of the newborns was 2678gr. All babies were normal, 45 cases were mature, 5 of them were preterm, and 12 cases were spontaneous abortion. 27 of mothers had no cardiac problem and 5 of them had cardiac failure. Pregnancy can be safe for mothers and babies, if the women with thalassemia start early intensive treatment.

TAR syndrome (thrombocytopenia-Absent Radii) is characterised by the neonatal onset thrombocytopenia and bilateral absence or hypoplasia of the radii with normal organs. This syndrome is a rare hereditary condition with a recessive autosomal character. During the first months of life, prognosis is related to the severity of hemorrhage. This paper focuses on three infants who suffered from Thrombocytopenia-Absent Radii(TAR) syndrome. All cases revealed bilateral micromelia of upper limbs, short and radially curved fore arms and hypoplastic arms at birth. These symptoms as well as severe thrombocytopenia confirmed the clinical diagnosis of Thrombocyopenia-Absent Radii(TAR). All three cases had multiple hemorrhages in neonatal period and also in the first year of their lives, however, they are in good condition right now. One of them needed prosthesis at the age of 7 but the two others are alive and being followed up.

Fanconi anemia is the most prevalent form of inherited aplastic anemia which is characterized by progressive bone marrow failure, congenital anomalies and cancer susceptibility. Common anomalies are skeletal abnormalities, skin pigmentation disorder, short stature, head abnormalities, kidney and gonad disorders respectively. Complications of fanconi anemia include: leukemia due to defective DNA repair in approximately 10% of patients, liver tumors in 5% and cancers (except leukemia and liver tumors) in 5%. In addition to clinical and radiologic investigation of children with fanconi anemia, survival, prognostic factors and complications of disease were also determined. This retrospective analytic cross-sectional study was carried out on 36 patients with fanconi anemia who were diagnosed in Ali Asghar Children Hospital from 1985-2002. Data was analyzed by SPSS software and log rank test was used to determine survival, life table and prognostic factors. Skeletal abnormalities were the most common congenital anomalies and other anomalies were skin pigmentation disorder, short stature, kidney disorder, microcephaly and male genital disorder respectively. 5 and 10-year survival rates of patients with fanconi anemia were 80% and 71% respectively. In bivariate analysis, the age at diagnosis correlated with survival rate significantly (P=0.006) and patients with age less than 10 years at diagnosis had more survival rate than others. In our study, The prevalence of kidney disorder as a congenital anomaly is more than other investigations and the frequency of short stature, microcephaly, skin pigmentation and male genitalia disorder is less than others. One of the considerable results of this research was the absence of neoplastic complication in our patients.

Aplastic anemia is a clinical syndrome in which there is peripheral blood pancytopenia due to reduced or absent production of blood cells without evidence of another marrow disorder. The disorder may be acquired or inherited like Fanconi’s anemia. In this study, survial and prognostic factors in acquired aplastic anemia is determined. This analytic cross-sectional study was done on 224 child patients who referred to blood disease clinic of Hazrat Ali-Asghar Hospital from 1977 to 2000. Data of patients was collected retrospectively in census methods. Survival state, demographic variables, signs & symptoms, laboratory test and kind of treatment were all recorded. Data was analyzed by Kaplan-Meier survival curves and life tables. In order to determine the factors influencing survival log rank test and cox-regression analysis in SPSS software, log rank test and cox-regression analysis in SPSS software were used. 5 and 10-year survival of acquired aplastic anemia were 55%, 41.6% respectively. In bivariate analysis, absolute neutrophil count less than 100/μlit (P=0.00) and ESR more than 60 mm/h (P=0.04) were negatively associated with survival and improved survival was associated with pallor at time of diagnosis(P=0.04). In multivariate analysis just association between absolute neutrophil count (EXP(B)=5.18) and pallor (EXP(B)=0.41) with survival were proved. In this study, survival of patients with acquired aplastic anemia in comparison with other countries was in the lowest range of survivals.

Brain tumors are the second common malignancy after leukemia in patients who are under 15 years and their metastatic form is rare in children. This study was undertaken on 100 patients with brain tumor who were admitted to Hazrat Ali Asghar Children Hospital during 1990 to 2000. Through this study, information about type of pathology, treatment and patients’ five-year survival was gathered in order to have better treatment and follow-up. This study was carried out as a cross-sectional survey and the obtained data was analysed via SPSS software. In this survey a questionnaire containing information about age, incidence, pathology type, clinical signs, neurological side effects and the amount of complete recovery was filled. The findings showed that the mean age of patients was 6.22±0.83(female 47%, male 53%) and male/female ratio was 1.12. Pathologically, 47% of the tumors were medulloblastoma, 18% were ependymoma, 9% were cerebellar astrocytoma, 63% inferatentorial, and 33% supratentorial. Headache(50%), nausea and vomiting (54%) were the most frequent clinical symptoms. Focal neurological sings were: hemiparesis(9%), seizure(12%), alteration in personality(10%) and ataxia(22%). 70% of the patients were treated with surgery, radiotherapy and chemotherapy, 7% were only treated with surgery, 13% with surgery and radiotherapy and 9% with surgery and chemotherapy. Post-treatment side effects in our patients were neurological complications(52%) and five-year survival(15%). For diagnosis of brain tumor CT scan was used in 78% of cases and MRI in 3%.In general, 22% of the patients were expired and only 11% of cases were alive. No information is available about 40% of the patients because of not having any address from them and 27% of the patients have just been under treatment for about 5 years.

Cardiac symptoms and premature death from cardiac causes are still major problem with beta thalassemia despite chelation therapy. Heart complications are the leading causes of mortality in the absence of effective iron chelation therapy. Many patients develop evidence of iron-induced myocardial damage with cardiac failure, cardiac arrhythmia, sudden death or death from progressive congestive failure. The characteristic lesion in the heart is caused by iron deposition in the myofibrils with myofibrillar fragmentation and diminished mitochondrial volume in myocyte. The basic cardiological assessment including 12-lead electrocardiogram, chest X-ray and echocardiogram is done from 7 years of age annually or more if it is needed. This study was undertaken on beta thalassemia patients who were under treatment in Hazrat Ali Asghar Children Hospital in the past ten years. In total of 1069 cases, 16 patients(1.5%) died from severe cardiac impairment(CHF) and 19 patients with severe cardiac problems, who were treated with monitoring cardiac function and intensive chelation therapy, are alive and relatively well. 106(9.9%) cases are asyptomatic with moderate cardiac impairment and 985(92.1%) cases of thalassemia are asymptomatic patients with normal heart condition.

Blood hypereosinophilia is a common finding in medical practice requiring further investigation. There are a wide range of potential causes including atopic disorder, drug allergy, parasitic infection, certain forms of immundeficiency, inflamatory process and malignant diseases. If hyper eosinophilia last a long time, and will not associated with parasitic infection and other disease could be suspected in idiopatic hyper eosinophilia syndrome that is rare disorder in children. Most cases are reported in women aged between 20-50 years and unknown etiology. We have reported a 4 years old girl with compliants of anemia, continuous fever, artheralgy, hepatomegaly and hypereosinophilia for two month, was admited in Children Hospital. In the CBC had leukocytosis, increased Eos(45%), and after role out of other causes of HES, she was treated and now she is alive and well.

Hodgkin’s disease is one of the most common childhood malignancies that with correct diagnosis and effective treatment is curable and without treatment is leading to death.The survival of patients with Hodgkin’s disease has dramatically improved over the past 30 years because of advances in treatment.However, concern for the risk of long tern complications has resulted in a number of trials to evaluate reduction of therapy. Causes of second malignancy are related to therapies (radiotherapy-chemotherapy-radiotherapy with chemotherapy), age of patients in the first treatment, sex and immunological deficits associated with Hodgkin’s disease. Two third of second malignancies are solid tumors and 1/3 are hematologic and lymphatic system cancer.In patients that treated first with radiotherapy, solid tumors appears higher and in patients treated first with chemotherapy leukemia appears higher. With this reuslts in childhood malignancies radiotherapy sholud be reduced and when patients treated with chemotherapy the cycles of treatment should be reduced too, and all patients should have follow up for second malignancy for several years. In 228 patients with Hodgkin’s disease in ten years in Hazrat Ali-Asghar Hospital, 4 second malignancies were occurred.Three cases presented with solid tumor and one case with leukemia (AML). Three cases that first treated with radiotherapy after 8, 18 and 19 years later (respectively) presented with solid tumor, one case died from brain tumor and two cases presented with thyroid cancer, had thyroidectomy and are alive. One case after 6 years of repeated chemotherapy for recurrent disease developed leukemia and finally died. Because in this center, radiotherapy is restricted, the incidence of second malignancy is less than other centers.