Detection of Anti-Platelet Glycoprotein Antibodies Using MAIPA Method

AITP mostly occur in children accompanied by variable clinical sings including petechiae, purpura, ecchymosis and severe bleeding. This study has determined and characterized the anti-platelet glycoproteins in children with ITP. The aim of this study was to determinate anti-platelet glycoproteins (GPs) using MAIPA method. During 18 months 38 children with clinical signs of AITP were studied in Mofid children hospital. To determine anti-platelet antibodies by ELISA technique, washed O negative platelets were used as a source of platelet antigens. MAIPA method was used to detect antibodies against individual platelet membrane glycoprotein. The anti-platelet antibodies level above mean+ 3SD of control group was assumed as positive. The results indicated that the platelet count ranges was between 2×109/L and 95×109/L. 63.5 % out of 38 patients were anti-platelet antibodies positive with ELISA method. The correlation between the above patients with anti-platelet antibody positive and clinical signs was 0.4. Results for determination of antibody against platelet GPIIb/IIIa, GPIb/IX and GPIa/IIa using MAIPA method were 44%, 51% and 25% respectively. In conclusion the preference of MAIPA method is the detection of very small amount of antibody. Since MAIPA is the specific method for the detection of antibody against glycoprotein antigens, it has the advantage of differentiating immune and non-immune thrombocytopenia

Tumors of the Central Nervous System: An 18-Year Retrospective Review in a Tertiary Pediatric Referral Center

How to Cite This Article: Aghayan Golkashani H, Hatami H, Farzan A, Mohammadi HR, Nilipour Y, Khoddami M, Jadali F. Tumors of the Central Nervous System: An 18-Year Retrospective Review in a Tertiary Pediatric Referral Center. Iran J Child Neurol. Summer 2015;9(3):24-33. AbstractObjectiveFew studies exist on the demographics and trends of pediatric central nervous system (CNS) tumors in Iran. In this study, we retrospectively reviewed all cases with confirmed CNS tumors admitted to Mofid Pediatric Hospital, Tehran, Iran during the last 18 years.Materials & MethodsData on gender, age of diagnosis, pathologic classification and tumor location were extracted from the available medical records. We used the last version of International Classification of Childhood Cancer.ResultOverall, 258 (81.9%) brain tumors and 57 (18.1%) spinal tumors were identified.Our subjects comprised of 147 (46.7%) female and 168 (53.3%) male children.More male dominancy was observed in brain tumors with a male to female ratio of 1.2 compared with 1.03 of spinal tumors. Malignant CNS tumors were most common in 1-4 yr age group. The four most common brain tumors in our subjects were astrocytomas, medulloblastoma, ependymoma and craniopharyngioma.Overall, 53.1% of the brain tumors were supratentorial. Gliomas, PNET and neuroblastma were the most frequent primary spinal tumors in our study. We observed an increasing trend for both brain and spinal tumors that was more remarkable in the last 5 years.ConclusionOur results are comparable with similar single center studies on CNS tumors during childhood. The observed disparities could be attributed to the single center nature of our study and geographical, environmental and racial variations in pediatric CNS tumors. The increasing trend of both brain and spinal tumors could warrant further investigations at provincial and national levels to investigate probable contributing environmental risk factors.

Expression of Bmi-1 in Pediatric Brain Tumors as a New Independent Prognostic Marker of Patient Survival

Objectives. The B-cell-specific moloney leukemia virus insertion site 1 (the Bmi-1) gene is an important member in the family of polycomb group (PcG) genes that plays an oncogenic role in several types of cancer, but it’s expression as a prognostic marker in pediatric brain tumors has not been indicated. Materials and Methods. The Bmi-1 gene expression, clinic pathological and prognostic significance in a series of pediatric brain tumors were examined by real-time PCR method in 56 pediatric brain tumors. Results. The Bmi-1 gene expression in various types of pediatric brain tumors compared to that in normal brain tissue was 4.85-fold. The relative expression varied from 8.64-fold in ependymomas to 2.89-fold in other types. Expression level in high-grade tumors compared to that in low-grade tumors was 2.5 times. In univariate survival analysis of the pediatric brain tumors, a significant association of high expression of the Bmi-1 with patient survival was demonstrated. In multivariate analysis, the Bmi-1 high expression provided significant independent prognostic factors. Conclusion. Increased expression of the Bmi-1 in pediatric brain tumors may be important in the acquisition of an aggressive phenotype. In addition, it can be used as a strong and independent molecular marker of prognosis in pediatric brain tumors

Concurrent Adrenal Neuroblastoma and Kawasaki Disease: A Report of a Rare Case

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology and a leading cause of acquired heart disease. It is assumed that there is an activation of the immune system by an infectious trigger in a genetically susceptible host. Neuroblastoma is the most common extracranial solid tumor in young children. It mainly originates from primordial neural crest cells that generate the adrenal medulla and sympathetic ganglia. A diagnosis of concurrent KD and neuroblastoma in a living child has been made in only one previous report. We report the second case and review the literature

Antibiotic Susceptibility Patterns in H. Influenzae Type B Isolated from Healthy Children Oropharynx in Day Care Centers of Tehran

Objective: Haemophilus influenzae type b (Hib) is a most frequent cause of invasive diseases such as meningitis, septic arthritis and pneumonia in children under 5 years old. Asymptomatic oropharyngeal colonization is an origin of distribution of microorganism to others and probable bacteremia in the same child. The aim of this study was to determine antibiotic susceptibility of Hib in Tehran day care centers. Material & Methods: Hib was isolated from oropharynx of 1000 children visiting 25 day care centers selected randomly in different parts of Tehran city during second half of year 2005. For antibiotic susceptibility determination we used disk diffusion test. Findings: Ampicillin resistance was 32.3% and Beta lactamase production was seen in 23.6%. Cephalosporins resistance except for cefixime was between 10% to 20% and in cefixime was 58.8%. Rifampin resistance was 17.6%. Resistance to studied macrolids including azythromycin and clarythromycin was 19.6% and 35.3%. Conclusion: On the base of high antibiotic resistance to Hib in our study and other similar studies in Iran, we recommend to use optimal effective and proper antibiotics to decrease the high rate of antibiotics resistance to Hib colonization and its invasive diseases

Can We Rely on Frozen Sections of a Rectal Biopsy for One-stage Trans-anal Pull-through Operation in Hirschsprung's Disease?

Objective: Successful results after one-stage trans-anal pull-through (OSTAPT) operation for Hirschsprung's disease (HD) depend on accurate identification of the aganglionic segment in intra-operative frozen section (FS). Misinterpretation of the findings of the rectal biopsy is an anxiety-evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section (PS) rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation. Methods: Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children's hospital for open rectal biopsy to diagnose HD were included in the study. All biopsies were taken 2-3 cm above the dentate line. 210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients, examined by two well experienced pediatric pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5. Findings: Two-hundred one infants and children underwent FS rectal biopsy to exclude HD. Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples. 93.9% of positive results in FS studies were confirmed by PS studies. 6.1% of FS reports were false positive and 21.7% were false negative (P<0.001). The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value (PPV) was 93.9% and negative predictive value (NPV) was 78.3% in FS studies (P<0.001). The accuracy of FS was 80.4%. Conclusion: Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base for performing primary pull-through operation before the results of the permanent section are on hand

Pulmonary alveolar proteinosis in children: a case series

Pulmonary alveolar proteinosis, (PAP) is a rare disease of unknown etiology, characterized by accumulation of intraalveolar proteinaceous material which is rich in lipid and positive on periodic acid-Schiff stain. Two clinically different pediatric types have been defined as congenital PAP which is fulminant and fatal, and a late-onset PAP which is similar to the adult form and less severe. Eight children with late-onset PAP were hospitalized from 1998 to 2005 in Mofid Children Hospital. Characteristics of these patients and the methods of diagnosis and treatment are presented in this case series