Clinical Features and Treatment Outcomes of Primary Immune Thrombocytopenic Purpura in Hospitalized Children Under 2-Years Old.

BACKGROUND: Immune thrombocytopenic purpura (ITP) is the most prevalent cause of thrombocytopenia in children. Despite the importance of ITP in children under 2-years old, only a few publications are available in the literature.ITP usually presents itself as isolated thrombocytopenia and mucocutaneous bleeding. MATERIALS AND METHODS: This study was conducted on 187 under 2-year-old children diagnosed with ITP and treated at Dr. Sheikh Hospital from 2004 to 2011.In this retrospective study, clinical symptoms, laboratory findings, history of viral infections, vaccination history, and treatment efficacy in children under 2-years old with ITP were investigated.Patients were followed for one year after being discharged from the hospital. RESULTS: The risk of the disease developing into chronic form was higher in older children (0.001). ITP in children under 3-months old was significantly associated with vaccination (p=0.007). There was no significant differences between male and female patients in regards to newly diagnosed ITP, persistent, and chronic disease status (p = 0.21). No significant difference in bleeding symptoms was observed between patients under 3-months old and 3 to 24-months old (p=0.18). CONCLUSION: Infantile ITP respond favorably to treatment. The risk of the disease developing into chronic form is higher in 3-to-24-month-old children compared to under-three-month olds.

Molecular basis of Bombay phenotype in Mashhad, Iran: identification of a novel FUT1 deletion.

BACKGROUND AND OBJECTIVES: Bombay phenotype is characterized by the lack of H substance both on red blood cell (RBC) surface and in body secretions. Mutations of fucosyltransferase 1 (FUT1) and fucosyltransferase 2 (FUT2) genes are resulted in this rare phenotype. MATERIALS AND METHODS: Five unrelated patients were tested by hemagglutination and adsorption/elution techniques for the presence of ABH antigens. The saliva specimens were analysed by hemagglutination inhibition method. The exons 6 and 7 of ABO gene were sequenced to determine ABO genotype. The coding fragments of FUT1 and FUT2 were amplified and sequenced by specific primers. RESULTS: Serologic investigation confirmed Bombay phenotype in all individuals. FUT1 molecular analysis revealed a novel large deletion. Also two novel homozygous mutations were detected; one was a missense mutation (392T>C, L131P) and the other a three nucleotide deletion (668_670delACT, Y224del). FUT2 sequencing showed one reported null allele (428G>A, W143X) and one homozygous deletion of FUT2. CONCLUSION: Although FUT2 deletion has been reported, this is the first report of FUT1 deletion. Finding two FUT1 novel alleles in Iranian people is indicative of mutation diversity in this gene.

Prevalence of Psychiatric Symptoms in ALL Patients during Maintenance Therapy.

BACKGROUND: Cancer diagnosis may cause deep emotional and affective problems in patients and their families. Nowadays, however, despite its rising prevalence, cancer is no longer synonymous with death. Given the significance of emotional well-being in cancer patients, we decided to assess the frequency of psychological problems in seven to seventeen year-olds with acute lymphoblastic leukemia. MATERIALS AND METHODS: Our sample included 42 children and adolescents with ALL referred to pediatric hematology department of Dr. Sheikh hospital, who were put under maintenance course of the treatment. Psychiatric disorders such as anxiety, depression and behavioral disturbances were examined by using RCMAS, CDI and SDQ questionnaires respectively. RESULTS: The entire population showed depressive symptoms.59.5% of patients (25 person) suffered from anxiety and 26.2% (11 person) had behavioral problems. No significant relation was found between depressive symptoms, and age (p=0.77), sex (p=0.97), length of disease (p=0.50), and type of treatment (p=0.064). Anxiety did not show any significant relation with age (p= 0.63), sex (p= 0.32), length of disease (p= 0.16) and treatment type (p= 0.064).Similarly behavioral disturbances did not indicate any suggestive relation with age (p= 0.20), sex (p= 0.56), length of disease (p= 0.81) and type of treatment (p= 0.19). CONCLUSION: Our findings suggest a high prevalence of psychiatric disorders in children and adolescents with ALL. It is strongly recommended, therefore, that besides somatic symptoms, careful attention be paid to psychological disorders. This can prevent rapid development of the disease reduce treatment costs, and improve the quality of life for both patients and their families.

Oropharyngeal candidiasis in children with lymphohematopoietic malignancies in Mashhad, Iran.

BACKGROUND AND PURPOSE: Over the past years, the role of fungi as a cause of nosocomial infections in hospitalized patients has been accentuated. Candida species constitute an important group of fungi causing diseases in immunocompromised patients. Oropharyngeal candidiasis continues to be a prevalent infection in immunodeficient patients. In this study, we aimed to determine the incidence of oropharyngeal candidiasis in children with lymphohematopoietic malignancies. MATERIALS AND METHODS: In total, 102 patients with lymphohematopoietic malignancies and 50 healthy controls were examined in terms of Candida infections via direct sampling of the oropharyngeal cavity. Fresh smears were prepared with 10% potassium hydroxide and Gram staining was carried out. Subsequently, the obtained specimens were cultured on Sabouraud dextrose agar for further analysis. RESULTS: The most common Candida species were Candida albicans (31%), other non-C. albicans species (14.7%), C. glabrata (6.8%), and C. krusei (0.98%) in the case group, while in the control group, other non-C. albicans species (10%) and C. albicans (8%) were the most common species. CONCLUSION: In the present study, Candida species were the most common fungal pathogens in pediatric cancer patients; therefore, efforts should be made to prevent fungemia and fungal pneumonia. Also, non-C. albicans species must be considered as a new risk factor for pediatric cancer patients.

Influencing factors on the functional level of haemophilic patients assessed by FISH.

Joint destruction in early adulthood brings the patients to the orthopaedic clinics. If a haemophilic patient becomes disabled, it shows a number of factors such as timely diagnosis, availability of appropriate treatment depending on the country, access and affordability to treatments and equally importantly the responsibility of the patient in managing self care by remaining compliant by prescribed treatment regimen. We assessed the functional level by functional independence score in haemophilia (FISH). Overall, 104 patients with haemophilia A and 29 with haemophilia B were evaluated. We assessed the function of the patients by FISH. We divided the sum scores into weak (FISH score 8-16), moderate (17-24), and good (25-32). For evaluating the level of functional deficit in a 2 × 2 table, we categorized the weak and moderate levels into Disordered Group and the good level into Not-Disordered Group. The average age was 26.9 ± 14.24. Each 1 year increase in age can increase 1.07 fold the possibility of being placed in Disordered Function Group. Severe haemophilia can increase 7.34 fold, presence of inhibitor can increase 9.75 fold and home self-care increases 3.89 fold the possibility of being placed in Disordered Function Group. To decrease the burden of the cost on patient, family and the government, education plays the most important role. We suggest that we send a trained team of physician and nurses to the deprived villages and cities instead of waiting for the patient to refer to our Care Center.