Brain Injury Patterns in Neonates With Hypernatremic Dehydration: Single Center Experience.

OBJECTIVE: To find out the incidence, spectrum, and topographical distribution of brain lesions in neonatal hypernatremic dehydration. METHODS: We prospectively enrolled 100 consecutive neonates admitted with hypernatremic dehydration. 93 neonates underwent magnetic resonance imaging brain to identify the nature and site of neurological injury. RESULTS: Neuroradiological lesions were found in 42 (45.2%) babies. Edema was the most common finding in 37 (39.8%), followed by hemorrhage in 13 (13.9%) and thrombosis in 6 (6.4%). Edema predominantly affected juxtacortical/subcortical white matter followed by periventricular white matter and centrum semiovale, posterior part of internal capsule, and basal ganglia/thalamus. Occipital horns of lateral ventricle were the main sites of hemorrhage. Thrombotic lesions predominantly involved sagittal, straight and transverse sinuses. Brain lesions were observed only in severe hypernatremia group. CONCLUSIONS: In neonatal hypernatremic dehydration, edema was the most common neurological lesion, followed by hemorrhage and thrombosis. Subcortical/juxtacortical white matter was the most commonly affected site.

Effect of Folic Acid Supplementation on Seizure Control in Epileptic Children Receiving Long Term Antiepileptic Therapy.

OBJECTIVE: To evaluate the effect of folic acid supplementation on seizure control in folate deficient children receiving long term antiepileptic therapy. METHODS: In a prospective interventional study, 140 children between age group 6 mo to 180 mo fulfilling the inclusion criteria were enrolled in study group, from October 2015 through November 2016. On the basis of serum folate, study group was divided into two subgroups by non randomization: Group A (<10 ng/ml) given folic acid supplementation for 3 mo and Group B (>10 ng/ml) was not supplemented. Response to folic acid supplementation in group A was compared with group B in terms of change in blood folate levels, frequency and duration of seizures after three months. RESULTS: Mean age of study group was 73.58 ± 46.89 mo (72.14% boys and 27.85% girls). 67.85% children were in group A and 32.14% in group B. On 3 mo follow up, children supplemented with folic acid (Group A) had significant fall in mean seizure frequency while in non-supplemented children (Group B), no significant change was seen (p value <0.05). Similar reduction in duration of seizure episode was seen in group A as compared to group B. Serum and RBC folate levels improved from baseline in group A, while in group B there was significant fall in folate levels. CONCLUSIONS: Folate deficiency is common in epileptic children on long term antiepileptic drugs (AEDs), contributes to poor seizure control and should be considered in the etiologic differentials of drug resistant epilepsy. Folate supplementation improves seizure control in these children.

Correlation of Tissue Transglutaminase with Modified Marsh Grading in Celiac Disease: A Prospective Cohort Study.

OBJECTIVE: To find out correlation between serum anti-tissue transglutaminase immunoglobulin-A (tTGA) levels and Marsh grading on duodenal histopathology in Celiac disease (CD). METHODS: In a prospective cohort study, a total of 52 symptomatic patients between age group of 2-18 y were enroled. All enroled patients were subjected to upper GI endoscopy by an experienced endoscopist. Two biopsies each from the bulb (D1) and second part (D2) of the duodenum were taken and Marsh grading was performed by a single experienced pathologist. Serum tTGA levels were also performed to find out correlation between serum tTGA levels and Marsh grading. RESULTS: The mean age of the patients was 8.21 ± 3.45 y (Range: 2-16 y). Anemia was the most common non-gastrointestinal (GI) sign and was present in 73% of the cases. However the authors could not find out any significant association between Marsh grading and hemoglobin levels (r = 0.32, p > 0.05). Serum tTGA levels were found to be positively correlated with Marsh grading (Spearmen correlation coefficient ρ = 0.74, p 0.000). Significant differences were found in tTGA levels between different Marsh gradings (ANOVA test) (p 0.000). Receiver-operator curve (ROC) analysis cut-off value of serum tTGA for predicting villous atrophy was 178.8 (nine times of cut-off value) with sensitivity of 100% and specificity of 85.7%. CONCLUSIONS: Serum tTGA levels can be used to predict villous atrophy and biopsy may be avoided in strongly suspected cases with more than 9 times of cut-offs.

Premature birth and insulin resistance in infancy: A prospective cohort study.

OBJECTIVE: This study was done to determine the role of prematurity and other variables to predict insulin sensitivity in infancy. SUBJECTS AND METHODS: In this prospective study, 36 preterm appropriate for gestational age (AGA), 11 preterm small for gestational age (SGA), and 17 term SGA included as study cohort and 36 term AGA as control cohort. Detailed anthropometry assessment was performed at birth, 3, 6, and 9 months and at 9 months, fasting plasma glucose and serum insulin was done. Insulin resistance was determined by using homeostasis model assessment version 2. RESULTS: It is found that preterm AGA (mean difference 0.617, 95% confidence interval [CI]; 0.43-0.80, P = 0.0001), preterm SGA (mean difference 0.764, 95% CI; 0.44-1.09, P = 0.0001), and term AGA (mean difference 0.725, 95% CI; 0.49-0.96, P = 0.0001) group had significantly higher insulin resistance than control. There was no significant difference in between preterm SGA and preterm AGA (mean difference 0.147 95% CI; -0.13-0.42, P = 0.927). In multiple regression models, SGA status (ß =0.505) was more significant predictor of insulin resistance index than gestational age (ß = -0.481), weight-for-length (ß =0.315), and ponderal index (ß = -0.194). CONCLUSION: Preterm birth is a risk factor for the future development of insulin resistance which may develop as early as infancy.

Clinical profile of hemophilia patients in Jodhpur Region.

BACKGROUND: Hemophilia is widely distributed all over the world, but little is known about its clinical profile in resource-limited regions. An insight into its clinical spectrum will help in the formulation of policies to improve the situation in these areas. AIMS: To study the clinical profile of hemophiliacs (age <18 years) in Jodhpur region and screen them for transfusion-transmitted infections. MATERIALS AND METHODS: A cross-sectional study conducted in the Department of Pediatrics, Umaid Hospital, Dr. S. N. Medical College, Jodhpur, over a period of 12 months. RESULT: Out of a total of 56 cases enrolled, 51 (91%) cases were diagnosed as hemophilia A while 5 (9%) were diagnosed as hemophilia B. Positive family history was found in 26 (46%) cases. According to their factor levels, 25 (44%) cases had severe disease, 20 (36%) had moderate disease, and 11 (20%) had mild disease. The mean age of onset of symptoms and diagnosis was 1.73 ± 1.43 and 3.87 ± 3.84 years, respectively. First clinical presentation was posttraumatic bleed in 20 (36%), gum bleeds in 17 (30%), epistaxis in 4 (7%), joint bleeds in 4 (7%), skin bleeds in 4 (7%), and circumcision bleed in 3 (5%) cases. Knee joint was the predominant joint affected by hemarthrosis in 38 (68%), followed by ankle in 29 (52%), elbow in 20 (36%), and hip joint in 7 (13%) cases. All patients had a negative screening test for transfusion-transmitted infections. CONCLUSION: Occurrence of posttraumatic bleeds and gum bleeds in an otherwise normal child should warn the clinician for evaluation of hemophilia.

Joint Health Status of Hemophilia Patients in Jodhpur Region.

Hemophilia refers to a group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of the blood. Susceptibility to joint hemorrhage in persons with Hemophilia suggests that the routine assessment of joint health is an important aspect of clinical management and outcome studies assessing the efficacy of treatment. This prospective study was conducted to study joint health status in Hemophilia patients and draw their joint disability score by using Hemophilia Joint Health Score (HJHS). Out of total 56 cases 51 (91.07 %) cases were diagnosed as hemophilia A while 5 cases (8.92 %) were diagnosed as hemophilia B. According to their factor level 44 % cases had severe 36 % had moderate and 20 % had mild disease. Knee joint was the predominant joint affected by hemarthrosis in 67.85 % cases followed by ankle joint (51.7 %) elbow joint (35.7 %), hip joint (12.5 %), shoulder joint (5.3 %) and proximal metacarpophalangeal joint (1.78 %).Out of total 37.5 % patients of hemophilia had developed target joint. Knee joint was the predominant target joint in 28.57 % cases and ankle joint was the target joint in 8.92 % cases. Maximum number of patients (40.47 %) had HJHS score of zero. The mean HJHS score was 6.78 ± 9.04. HJHS score showing significant positive correlation with age of patient (p < 0.0001). Most risky period and most aggravating development of hemophilic joint damage starts from 7 years of age. Therefore, treatment decisions, such as starting prophylaxis, should be tailored according to bleeding pattern and age of patients rather than based on the clotting factor activity levels.