Background: ß-thalassemia is an autosomal recessive single gene disorder. Physical growth failure is one of the most important complications of thalassemia. Very few data regarding growth pattern of thalassemic children is available from India especially North East part. Keeping this in mind, present study was undertaken. To study the growth pattern of transfusion dependent thalassaemic children and to compare growth pattern between regularly and irregularly transfused children.Methods: A cross-sectional observational study was done on 38 thalassaemic patients (aged 2 years-12 years) who attended Department of Pediatrics, Assam Medical College Dibrugarh. History, physical examination and investigations were done and filled in predesigned proforma.  Anthropometric measurements like weight and height were taken from all patients. Sexual maturity rating was done in girls ≥10 years and boys ≥11 years. Lab parameters included pre-transfusion hemoglobin (Hb), serum ferritin, LFT, RFT, Thyroid profile. Percentile for weight, height and body mass index were calculated using WHO (2007) reference data. Collected data were compared with age and sex matched normal children.Results: About 34.21% transfusion dependent children had under-nutrition and 50% had stunting. 42% had thinness. Stunting was more in irregularly transfused children (81.25%) as compared to regularly transfused children which was highly significant (p<0.001). Under-nutrition among irregularly transfused children was more (40%) compared to regularly transfused children (28.57%). Pubertal spurt was delayed in 66% children. Those who had Hb <5 gm/dl had 100% stunting and under-nutrition.Conclusions: Regular blood transfusion with growth monitoring and appropriate iron chelation (Sr. Ferritin >1000 ng/ml) is of utmost importance in transfusion dependent thalassaemic children.

INTRODUCTIONNeonates constitute the foundation of human life. As Neonates are unique compared to other age groups, so are their health issues and problems. This is due to structural and functional immaturity of various body organs depending upon their gestational age and birth weight.Neonatal period (first 28 days of life) is considered the most vulnerable phase of life. This period accounts for ABSTRACT Background: In developing countries like India, the culture facilities are non-existent in most of the district hospitals, so the burden of identification of sepsis lies on hematological investigations like platelet count and white blood cells and very few indian studies have been done to show the association. Objectives: 1) To know the incidence of thrombocytopenia in babies with proven bacterial sepsis. 2) To find, if any, species specific differences in severity and incidence of thrombocytopenia. 3) Clinical outcomes in thrombocytopenic and nonthrombocytopenic septic babies. Methods: It is a prospective observational hospital based study. All the neonates admitted to our NICU with probable sepsis were screened for sepsis and neonates with birth weight of 1000 grams and above with blood and/or Cerebrospinal Fluid (CSF) culture positivity for bacterial growth were recruited during the study period from November 2011 to October 2012. A total of 100 neonates with blood and/or CSF culture positivity for bacterial growth were considered convenient for the study. Results: During the present study period of 1 year we had a total of 960 Neonates admitted to our NICU. 475 neonates were screened for sepsis. A total of 100 neonates with blood and/or CSF culture positivity for bacterial growth were considered for the study. Klebsiella pneumoniae sepsis was the highest contributor to the severe thrombocytopenia category (15 out of 29cases). Out of the 100 cases, 41 had normal platelet count and 59 had thrombocytopenia. Klebsiella positivity was more significantly associated with severe thrombocytopenia than MRCONS positivity or rest of the cultures combined together (p value <0.01). Although there was no significant association between thrombocytopenia and mortality (p=0.176), the proportion of children with severe thrombocytopenia was significantly higher in babies who expired (25% vs 9%) when compared to babies who survived. Conclusions: Our study shows that in our setting bacterial sepsis is significantly complicated by thrombocytopenia. Severe thrombocytopenia in a suspected case of bacterial sepsis might predict Klebsiella species sepsis and hence it may be prudent to start empirical antibiotics covering the Klebsiella species.

Background: Dengue is the commonest mosquito born viral infection. The numbers of dengue cases are increasing in recent days. Though the manifestations of dengue are complex, management is made simple by WHO-2009 guidelines. Many of clinicians are unaware or not sensitized about WHO-2009 guidelines in classification and management of dengue cases.Methods: 143 suspected dengue cases were selected based on presumptive diagnostic criteria and were classified as group A, B and C, and all the patients were treated according to WHO guidelines. 108 cases with positive dengue IgM/IgG were chosen for analysis and rest of the cases were excluded from the study. The ELISA for IgM & IgG was sent on 5th-6th day of fever.Results: 108 dengue IgM/IgG positive cases were selected for the study. They were grouped as Group A (20), Gr B (60), Gr C (28) according to guidelines. Most common associated symptoms were vomiting (60%), pain abdomen (61%), and puffiness of face (58%). Rashes were present in small number of patients (7.4%) and bleeding in 2.7% children. 5% in group A, 6.6% in group B and 17.8% in group C had platelet counts of <20000/mm3. 70% children of Group -A, 75% of Group -B and 85.7% of Group -C had PCV of >35, with maximum of 53.2 belonging to Group C. None of the patient received platelet transfusion. All children recovered well.Conclusions: In the present study we managed all the dengue cases as per guidelines of WHO-2009.  The treatment is cost effective and has very good outcome with less complications. The mortality, morbidity and duration of hospital stay were reduced. The need for iv fluids, blood and blood products were brought down significantly.

Neonatal diabetes mellitus with congenital hypothyroidism (NDH) syndrome (OMIM: 610199) caused by GLIS3 gene mutations is a multisystem disorder. To date, only 23 cases of NDH syndrome have been reported worldwide. We report a child diagnosed on day 24 of life with neonatal diabetes, congenital hypothyroidism, congenital glaucoma, and dysmorphic facial features. Genetic analysis revealed a homozygous pathogenic frameshift variant (p.Gln361Profs*193) in the GLIS3 gene, located on chromosome 9p24.2. Our report confirms that infants with neonatal diabetes and congenital hypothyroidism along with facial dysmorphism should be screened for GLIS3 mutations.