Thalassemia carrier screening in siblings of thalassemia major patients by HbA2 estimation

Objective: Thalassemia carrier screening in siblings of thalassemia major patients by HbA2 estimation. Methods: This prospective, cross-sectional study was conducted at thalassemia welfare society of JK Lon Hospital, Kota. Siblings of thalassemia major patients registered at our hospital were investigated for chemically bonded ceramics and HbA2 estimation by high performance liquid chromatography (HPLC) method. Results: A total of 121 cases were screened for carrier by HPLC method for HbA2 estimation. Total 59 (48.76%) cases had HbA2 level ≥3.5%, and considered as carrier while 62 (51.24%) cases were non-carrier. Mean HbA2 value in carrier was 5.24% ± 1.14% and in non-carrier was 2.69% ± 0.51%. Conclusion: It was concluded that β thalassemia carriers are more prevalent in siblings of thalassemia major than the normal population

Study of clinical profile of infantile tremor syndrome and its correlation with serum Vitamin B12 level

Objective: The objective of this study was to assess the clinical profile of infantile tremor syndrome (ITS) and to correlate serum Vitamin B12 level with ITS. Materials and Methods: This prospective study was done in the Department of Pediatrics of a Medical College of Kota. A total of 40 children, presenting with clinical features of ITS, were included in the study. A detailed history was taken. Investigations including complete blood count with blood indices (mean corpuscular volume, mean corpuscular hemoglobin [MCH], and MCH concentration), peripheral smear, and serum Vitamin B12 level were done in accordance with the WHO guidelines. Vitamin B12 deficiency was defined as serum Vitamin B12 concentration <150 pmol/L or 203 pg/mL. Results: All patients with ITS had pallor (100%) and skin hyperpigmentation (100%). Psychomotor changes in the form of apathy and/or developmental regression/stasis were found in 40 (100%) cases. Regression of developmental milestones was found in 26 (65%) and 10 (25%) patients had stasis of milestones. Tremor was present in 14 (35%) cases. Hepatomegaly was present in the majority 25 (62.5%) of these children, whereas splenomegaly was present in only 8 (20%) patients. Hypopigmented hair was found in only 12 (30%) cases. Twenty-five (62.5%) children were exclusively breastfed (p<0.05). Of the 40 cases, 26 (65%) presented in prodromal phase and 14 (35%) presented in the tremor phase. Among 26 patients of prodromal phase, 22 (84.62%) had Vitamin B12 deficiency while among 14 cases of tremor phase, 9 (64.28%) had Vitamin B12 deficiency. The majority of ITS children had macrocytic anemia (65%) followed by dimorphic anemia (25%). A significant number of ITS patients (21, 80.77%), having macrocytic anemia, had Vitamin B12 deficient. Conclusion: We conclude that children of ITS presented with anemia, skin hyperpigmentation, psychomotor changes, apathetic look, and developmental abnormality. The majority of children were on faulty feeding in the form of prolonged exclusive breastfeeding. Vitamin B12 deficiency was present in a significant number of patients with IT

OUTCOME OF MANAGEMENT OF SEVERE ACUTE MALNUTRITION CASES ADMITTED AT NUTRITIONAL REHABILITATION CENTER - A PROSPECTIVE OBSERVATIONAL STUDY FROM A TEACHING HOSPITAL FROM SOUTH EASTERN RAJASTHAN: Outcome of management of SAM cases at NRC

Introduction – Based on the guidelines for the management of Severe Acute Malnutrition (SAM), issued by the WHO and IAP, there is standard protocol which is applied for the management of SAM at Nutritional Rehabilitation Center (NRC). This study was aimed to evaluate the effect of nutritional interventional measures on nutritional status of SAM children admitted in NRC in terms of increment in basic defining criteria of SAM. Method : 54 cases were included in the study. Baseline variables like age, sex duration of stay; General physical examinations including status of edema and systemic examination were conducted and the clinical signs of micronutrient deficiencies were assessed. Anthropometric parameters like weight, height/Length, Mid upper arm circumference was recorded at admission and discharge. Result : The mean weight of children at admission and discharge was 5.88 ± 1.59 kg and 6.71±1.77 kg respectively. Average weight gain was 0.84±0.42 kg (t = -0.784, p = 0.437). An average rate of weight gain was 11.22±5.43 gm/kg/day. The Mean MUAC at admission was 10.62±1.21 cm. and mean MUAC on discharge was 11.49±1.24 cm. After intervention in hospital about 65% of patients had <-2 SD of weight for height. Conclusion: We concluded that NRCs are effective in management of malnutrition

Design, development and recent experiments of the CIMPLE-PSI device

The CIMPLE-PSI laboratory in India's northeast corner has been engaged over the last decade in the development of advanced experimental systems for controlled plasma fusion-relevant plasma surface interaction (PSI) studies and material testing. The CIMPLE-PSI experimental device was commissioned recently, where a magnetized collimated helium plasma beam is produced under steady-state conditions inside a linear vacuum chamber, which can be made to interact with remotely placed material targets, under controlled experimental conditions. This paper reports on the design, development of the major sub-systems of this device and the performance of the integrated system. The peak helium ion flux and heat flux were measured as 10$^{24}$ m$^{−2}$ s$^{−1}$ and 5.1 MW m$^{−2}$, respectively; this confirms that extreme ITER divertor-like parameters are successfully reproduced by this simulation device. Steady-state operation of the electromagnet allowed prolonged operation of the plasma leading to a very high fluence of 0.3  ×  10$^{28}$ m$^{−2}$, which may be enhanced further in the future. Tungsten samples were exposed in this device under helium plasma that had led to the formation of nanometer-sized tungsten fibre foam structures on the target. The technique of grazing incidence small angle x-ray scattering was successfully utilized to measure the average size of the helium bubbles remaining buried in the exposed sample and its variation with depth from the top of the surface