Background: β-thalassemia major is a secondary iron load state. The high serum ferritin accounts for abnormal lipid profile. This study was done to investigate the lipid profile in children with β-thalassemia major and to know their correlation with serum ferritin.Methods: Fifty-five (55) children of beta thalassemia major in the age group of 4-20 years from thalassemia unit of tertiary care hospital who were on regular blood transfusion and oral iron chelators were enrolled. Serum ferritin levels and lipid profile of these children were investigated and their correlation was assessed. Results: Out of total 55 children 31 (56.4%) children were of female sex and 48(87.3%) were Hindu by religion. The mean rate of blood transfusion was 157.02±21.33 ml/kg/year with average dose and duration of iron chelation with Desferroxamine was 34.4±26.86 mg/kg /day and 2.34±1.86 years respectively. Mean cholesterol level, high density lipoprotein (HDL), low density lipoprotein (LDL) levels were on lower side of the range with values of 124.47±19.81 mg/dl, 36.58±12.22 mg/dl, 63.94±4.57 mg/dl respectively. The mean triglyceride level (TG) was on higher side with an average of 142.93±33.7 mg/dl. The average serum ferritin levels were 2130.33±859.85 ng/ml. There was negative correlation of total cholesterol, HDL, LDL with serum ferritin with coefficient of correlation (r= -0.77, -0.55, -0.72) respectively. The serum triglyceride had positive correlation with serum ferritin with coefficient of correlation(r=+0.85).Conclusions: As the serum ferritin increases, there occurs more derangement of lipid profile in form of decreasing serum cholesterol, HDL, LDL and increasing serum TG levels.

No abstract

Background: Malnutrition is one of the most common global health problem. It produces notable morphological changes in the brains which damage the intellectual potential and leads to reduced brain size, inferred from measurements of head circumference(HC). Aim is to study the impact of malnutrition on head size and development quotient(DQ) in children suffering with malnutrition.Methods: It is a hospital based study on 120 children including 100 moderates to severely malnourished children and 20 healthy controls aged 6-60 months admitted in malnutrition treatment center. Statistical analysis of head circumference and development quotient was done with severity of malnutrition and with each other.Results: Out of 120 children, 80 were severely malnourished (SAM) and 20 were moderately malnourished (MAM) and 20 age and sex matched controls were taken. Mean age was 19.00±8.54 months. 36.25% of severely malnourished, 5% of moderately malnourished children and none in control had microcephaly (HC<-3SD). Mean DQ was 57.46±14.98, 78.35±6.60 and 94.45±3.96 in SAM, MAM and control children respectively. Statistically significant association was found for head circumference and development quotient with severity of malnutrition and with each other.Conclusions: SAM adversely affects the developing brain of children as evidenced in our study by reduced head size and low DQ scores in children suffering from malnutrition. As seen in this study, prevalence of microcephaly and lower DQ scores increases with severity of malnutrition. Therefore, the study emphasizes the importance of early and timely intervention in such children before the severity of malnutrition increases to an extent of irreversible effects on brain and development.

No abstract

Background: Cardiac complications are the major cause of mortality and morbidity in thalassemic children. Iron deposition in myocardium is the key factor leading to poor cardiac functions. Myocardial performance index (MPI) by echocardiography (ECHO) can be used for an early recognition of ventricular dysfunctions. Objectives: To assess the MPI in children with ?-thalassemia major and to establish their relationship with serum ferritin. Methods: Fifty-five children of Thalassemia major in age group of 4-20 years who were on regular blood transfusion and on oral iron chelators from thalassemia unit of tertiary hospital were enrolled.After blood transfusion, serum ferritin estimation was done. Two dimensional ECHO with color Doppler was done to estimate the cardiac functions and then MPI by various parameters was calculated. Results: Out of 55 children, most were in the age group of 4 to 8 years. Mean rate of blood transfusion in subjects was 157.01 ± 21.33 ml/kg/year and mean duration of chelation therapy was 2.34 ± 1.86 years. Mean serum ferritin of subjects was 2130 ± 859.5 ng/ml. Mean ejection fraction was 61 ± 6.2%. Mean MPI of subjects was 0.60 ± 0.14. The MPI was abnormal at all levels of more than 1000 ng/ml serum ferritin (p=0.001). There was a positive correlation between MPI and serum ferritin (Pearson’s bivariate correlation coefficient r=+0.93). Conclusion: In poorlychelated thalassemic children, MPI was abnormally high despite normal ejection fraction, which can be used as an early marker of ventricular dysfunction.

Duchenne’s muscular dystrophy is the most common hereditary neuromuscular disease, which affects all races. Its classical characteristic clinical features being progressive muscular weakness, intellectual impairment and hypertrophy of the calves with proliferation of connective tissue and progressive fibrosis in muscles. As the disease is inherited as an X-linked recessive trait, thus females not manifesting the disease and acting as carriers only, as second X chromosome prevents the manifestation of disease. We report a case of classical Duchenne muscular dystrophy in 10 year old female with no intellectual deficit and no family history of similar type of muscular dystrophy.

Background: Objective of the current study was to assess biochemical profile and neuroimaging findings in children diagnosed with neurotuberculosis in a tertiary care centre in Southern Rajasthan, India.Methods: A prospective randomized controlled trial was conducted in Department of Pediatrics, tertiary care centre in Southern Rajasthan, India from July 2017 to June 2018. Total 110 children of age group of 6 months to 18 years with the diagnosis of tubercular meningoencephalitis (TBME) on the basis of clinical evaluation, cerebrospinal fluid (CSF) examination and neuroimaging were included in the study.Results: Among 110 children included in the study, CSF lymphocytic pleocytosis was seen in all. Majority of children (56.36%) cell counts were in the range of 101-500 cells/µl and mean CSF cell count was 198.09±177.86 per µl. CSF protein ranges from 100 to 400 mg/dl in 68.2% children and 19 children had CSF protein >400 mg/dl with mean of 230.98±167.73 mg/dl. In majority of patients (40%) CSF glucose level was in range of 20-40 mg/dl and in 31.82% children CSF glucose was <20 mg/dl. Mean CSF sugar level was 33.86±18.22 mg/dl. None of them demonstrated acid fast bacillus (AFB) on Ziehl-Neelsen staining of CSF sample. Chest radiographic abnormality was found in 41.82% cases. Mantoux test was positive in 16.36% (18) children admitted with TBME. Common abnormalities noted on neuroimaging were: Communicating hydrocephalus (77.27%), meningeal enhancement (40%), infarction (27.27%), cerebral oedema (11.82%) and 9.09% has tuberculoma on neuroimaging. CSF and gastric aspirate were examined by cartridge based nuleic acid amplification test (CBNAAT) for Mycobacterium Tuberculosis (MTB), among them 5 (4.55%) children had positive in CSF and 16 (14.55%) had gastric aspirate positive for MTB by CBNAAT.Conclusions: Clinical, biochemical and radiological parameters is sufficient enough to diagnose TBME in children.

Background: Fever is the most common complain of a child presenting to a health care setting. A prolonged fever however, is an intriguing challenge for clinicians and remains overly medicated and inappropriately worked up, provided a systemic approach is not followed. Our study aims to find the etiologic diagnosis in these cases of prolonged fever. Methods: The present study was conducted in a tertiary care hospital in southern Rajasthan over a period of 12 months from November 2021 to October 2022. Our descriptive, observational study enrolled 150 children who were prospectively followed during their stay in hospital. Each patient was subjected to a set of mandatory investigations followed by other investigations as per the diagnostic clues. Patients were monitored closely during their course of stay until discharge, at which the final diagnosis was charted. Results: A diagnosis could be reached for 138 patients (92%) and in 12 patients (8%), the cause of fever remained undiagnosed until discharge/death. Out of the 138 patients diagnosed, 118 patients (85.5%, 78.6% overall) had an infection as the cause of fever and 20 cases (14.49%,13.3% overall) had a non-infective etiology. Conclusions: Infections are the most common cause of prolonged fever in pediatric population - tuberculosis being the most frequent infection. Hematological malignancies were found as an emerging cause of prolonged fever in our study.