Tyrosinemia type 1: A case report

Tyrosinemia Type 1 is a rare inherited metabolic disorder attributable to a deficiency of enzyme fumarylacetoacetate hydrolase. It hasan autosomal recessive pattern of inheritance. The accumulation of tyrosine and its toxic metabolites succinylacetone and succinylacetoacetate in various tissues leads to the characteristic hepatic failure, renal dysfunction, and neurological crisis. Here, we present acase of a 7-month-old female infant who was brought with complaints of jaundice, dyspnea, altered level of consciousness, refusal tofeed. We highlight the need for early diagnosis, including prenatal testing and initiating treatment at the earliest, which goes a long waynot only in the survival, but also the quality of life in these patients

RAPIDIRON Trial Follow-Up Study - the RAPIDIRON-KIDS Study: Protocol of a Prospective Observational Follow-Up Study

BACKGROUND: Anemia is a worldwide problem with iron deficiency being the most common cause. When anemia occurs in pregnancy, it increases the risk of adverse maternal, fetal, and postnatal outcomes. It induces preterm births and low birth weight (LBW) deliveries, long-term neurodevelopmental sequelae, and an increased risk of earlier onset of postnatal iron deficiency. Anemia rates are among the highest in South Asia, and India\u27s National Family Health Survey (NFHS-5) for 2019-2021 indicated that over half of pregnant women, and more than 65% of children, in the country are classified as anemic (Sciences IIfP, National Family Health Survey-5, 2019-21, India Fact Sheet). In 2021, the parent RAPIDIRON Trial (Derman et al., Trials 22:649, 2021) was initiated in two states in India, with the goal of assessing whether a dose of intravenous (IV) iron given to anemic women during early pregnancy results in a greater proportion of participants with normal hemoglobin concentrations in the third trimester and a lower proportion of participants with LBW deliveries compared to oral iron. As a follow-up to the RAPIDIRON Trial, the RAPIDIRON-KIDS Study will follow the offspring of previously randomized mothers to assess, neurobehavioral, hematological, and health outcomes. METHODS: This prospective observational cohort study will follow a subset of participants previously randomized as part of the RAPIDIRON Trial and their newborns. Study visits occur at birth, 6 weeks, 4 months, 12 months, 24 months, and 36 months and include blood sample collection with both maternal and infant participants and specific neurobehavioral assessments conducted with the infants (depending on the study visit). The primary outcomes of interest are (1) infant iron status as indicated by both hemoglobin and ferritin (a) at birth and (b) at 4 months of age and (2) the developmental quotient (DQ) for the cognitive domain of the Bayley Scales of Infant Development Version IV (BSID-IV) at 24 months of age. DISCUSSION: This RAPIDIRON-KIDS Study builds upon its parent RAPIDIRON Trial by following a subset of the previously randomized participants and their offspring through the first 3 years of life to assess neurodevelopmental and neurobehavioral (infants, children), hematological, and health outcomes. TRIAL REGISTRATION: ClinicalTrials.gov NCT05504863 , Registered on 17 August 2022. Clinical Trials Registry - India CTRI/2022/05/042933 . Registered on 31 May 2022

Understanding the dielectric and electrical transport behaviors of plasma polymerized diethanolamine thin films under alternating electric field

A capacitively coupled parallel plate glow discharge reactor has been utilized to develop plasma polymerized diethanolamine (PPDEOA) thin films to look into the alternating current (AC) features of PPDEOA thin films. It is observed that the dielectric constant, epsilon' in PPDEOA thin films enhances by declining the frequency and that increases with increasing temperature. The AC electrical conductivity, sigma(ac) is frequency dependent. The frequency exponent, s values are found to be 0.32 to 1.0 and 1.0 to 1.8 in the lower (10(2)-10(3) Hz) and higher ( > 10(3) Hz) frequencies, respectively. These suggest the presence of hopping conduction in the lower frequency region and other mechanisms in the higher frequency region. The activation energies are derived to be 0.03 to 0.28 eV illustrating the less effect of temperature on them. The real part, M' and imaginary part, M '' of electric modulus vary with frequency. The frequency dependent loss tangent, tan delta is obtained. The epsilon', sigma(ac), and tan delta deviate by the virtue of film thicknesses

Clinical profile and etiology of neonatal seizures in NICU Rims, Raichur

Introduction: Seizures are more common in the neonatal period than in any other stage and affects approximately 1% of all neonates. Amongst neurological disorders, seizures are the most common in neonates. The study was conducted to determine the aetiology and clinical profile of neonatal seizures. Materials and Methodology: This study was designed to be a hospital based prospective observational study was undertaken in the in the hospital attached neonatal intensive care unit (NICU), RIMS, Raichur. A total of 500 neonates presenting with seizures or who developed seizures in hospital from 01 February 2022 to 31 May 2022 were enrolled in the study. All neonates (age 0 – 28 days) who presented to the hospital with history of seizures or who develop seizures during the course of hospital stay were taken into study. Detailed antenatal history examination and clinical details of each seizure episode reported by the mother and subsequently observed by the resident doctors on duty were recorded. Results: Among these 405 patients (81%) were from rural areas and 95 patients (19%) were from urban areas.&nbsp

To study the association and significance of hyponatremia in pneumonia in paediatric patients in Rims, Raichur

Introduction: Hyponatraemia is reported to be the most frequently occurring electrolyte imbalance which is most commonly seen in critically ill children. The aim and objective of this study was to find out the frequency of hyponatremia in pneumonia and to find the association and significance of hyponatremia in patients affected with Pneumonia. Materials and Methodology: This study has been designed as an in-patient hospital based prospective study will be conducted to evaluate the association and significance of hyponatremia in Paediatric patients affected Pneumonia being treated in RIMS, Raichur. 120 patients will be selected as the study participants for the study after obtaining informed consent from the parents as well. Statistical analysis should be carried out by using appropriate statistical tests. Both the groups have to be compared by the student t – test for continuous variables and Chi-square/ Fischer’s exact test for all the categorical variables. The p value of less than 0.05 will be taken as significant. The data will be analysed by statistical software SPSS latest version. Results: . The patient’s serum sodium level ranged from 124 - 145 mEq/L (both found at 0 hour) and none were observed to have hypernatraemia.&nbsp

To Study the Association and Significance of Hyponatremia in Pneumonia in Paediatric Patients in Rims, Raichur

Introduction: Hyponatraemia is reported to be the most frequently occurring electrolyte imbalance which is most commonly seen in critically ill children. The aim and objective of this study was to find out the frequency of hyponatremia in pneumonia and to find the association and significance of hyponatremia in patients affected with Pneumonia. Materials and Methodology: This study has been designed as an in-patient hospital based prospective study will be conducted to evaluate the association and significance of hyponatremia in Paediatric patients affected Pneumonia being treated in RIMS, Raichur. 120 patients will be selected as the study participants for the study after obtaining informed consent from the parents as well. Statistical analysis should be carried out by using appropriate statistical tests. Both the groups have to be compared by the student t – test for continuous variables and Chi-square/ Fischer's exact test for all the categorical variables. The p value of less than 0.05 will be taken as significant. The data will be analysed by statistical software SPSS latest version. Results: . The patient's serum sodium level ranged from 124 - 145 mEq/L (both found at 0 hour) and none were observed to have hypernatraemia.&nbsp

CAESAREAN DELIVERY: INCREASES SUBSEQUENT RISK OF PLACENTA PRAEVIA

Medical College Hospital, in the period from April 07 to March 08. A total of 232 multiparous pregnant women were included in this study; among them 51 were cases (with placenta praevia) and 181 were similar matched control. Past mode of delivery were explored in both the groups and analyzed statistically. Out of 232 subjects, prior caesarean delivery had 25.5 % (n=13) in cases and 44.2 % (n=80) in controls. After statistical analysis, caesarean section was considered as a risk factor for placenta praevia in subsequent pregnancy. The odds ratio = 0.43 (CI 0.22 – 0.86) and chi square test = 5.80 (df =1; p = 0.016). Key words: Placenta praevia; caesarean section. (Bangladesh J Physiol Pharmacol 2009; 25(1&2) : 13-17

RAPIDIRON Trial follow-up study — the RAPIDIRON-KIDS Study: protocol of a prospective observational follow-up study

Abstract Background Anemia is a worldwide problem with iron deficiency being the most common cause. When anemia occurs in pregnancy, it increases the risk of adverse maternal, fetal, and postnatal outcomes. It induces preterm births and low birth weight (LBW) deliveries, long-term neurodevelopmental sequelae, and an increased risk of earlier onset of postnatal iron deficiency. Anemia rates are among the highest in South Asia, and India’s National Family Health Survey (NFHS-5) for 2019–2021 indicated that over half of pregnant women, and more than 65% of children, in the country are classified as anemic (Sciences IIfP, National Family Health Survey-5, 2019–21, India Fact Sheet). In 2021, the parent RAPIDIRON Trial (Derman et al., Trials 22:649, 2021) was initiated in two states in India, with the goal of assessing whether a dose of intravenous (IV) iron given to anemic women during early pregnancy results in a greater proportion of participants with normal hemoglobin concentrations in the third trimester and a lower proportion of participants with LBW deliveries compared to oral iron. As a follow-up to the RAPIDIRON Trial, the RAPIDIRON-KIDS Study will follow the offspring of previously randomized mothers to assess, neurobehavioral, hematological, and health outcomes. Methods This prospective observational cohort study will follow a subset of participants previously randomized as part of the RAPIDIRON Trial and their newborns. Study visits occur at birth, 6 weeks, 4 months, 12 months, 24 months, and 36 months and include blood sample collection with both maternal and infant participants and specific neurobehavioral assessments conducted with the infants (depending on the study visit). The primary outcomes of interest are (1) infant iron status as indicated by both hemoglobin and ferritin (a) at birth and (b) at 4 months of age and (2) the developmental quotient (DQ) for the cognitive domain of the Bayley Scales of Infant Development Version IV (BSID-IV) at 24 months of age. Discussion This RAPIDIRON-KIDS Study builds upon its parent RAPIDIRON Trial by following a subset of the previously randomized participants and their offspring through the first 3 years of life to assess neurodevelopmental and neurobehavioral (infants, children), hematological, and health outcomes. Trial registration ClinicalTrials.gov NCT05504863 , Registered on 17 August 2022. Clinical Trials Registry – India CTRI/2022/05/042933 . Registered on 31 May 2022