Indian Journal of Maternal-Fetal & Neonatal Medicine

  Mucopolysaccharidosis (MPS) are the group of autosomal recessive disorders characterized by deficiency of lysosomal enzymes which break down the glycosaminoglycans (GAGs). Their deficiency results in widespread intra & extra cellular accumulation of GAGs. Diagnosis of MPS is difficult due to rarity of these disorders and variety of clinical manifestation of varied severity. But because of genetic mutations there are high chances of recurrence which can be suspected with the help of family pedigree analysis & diagnosed by the Pre-natal genetic diagnosis (PGD). The purpose of this case is to report the process of follow up for genetic diseases which should always be kept in mind in today’s routine obstetric practice when most of pregnancies are occurring after the age of 30 years. 

Keywords: Mucopolysaccharidosis (MPS); Prenatal Genetic Diagnosis (PGD); Lysosomal Storage Disease; Sanfilippo Disease; Haparan Sulphate. 

  Encephalocele is a broad term representing herniation of cranial contents through a congenital defect in the cranium. If only cerebrospinal fluid (CSF) and meninges herniate, it is termed as a meningocele. A menin goencephalocele is herniation of both neural elements and meninges. The incidence of encephalocele is 1 per 5000 live births. Anterior encephaloceles are more common in males while 70% of posterior or occipital encephaloceles occur in females. We present one case of a giant occipital encephalocele to highlight the problems encountered in its management. 

Keywords: Microcephaly; Occipital Encephalocele; Neurological Development.

  Bioethics is the study of ethical, social, philosophical, and other related issuesin health care. Obstetrics is a speciality dealing with two lives, mother and fetus; whose interests may not always coincide. It is essential to promote and protect interests of both the pregnant mother and her fetus. Fetus has individual status and rights. In this article, ethical aspects related to fetus are discussed. Several ethical, social, legalaspects should be considered while recommending fetal therapy. Issues regarding Fetal tissue transplantation are discussed. Abortion is one of the most controversial issue in today’s world, which is discussed in ethical context. Bioethics regarding congenitally malformed fetus are highlighted. In multiple pregnancy, ethical aspects are related to many conditions which are discussed.

 Keyword: Bioethics; Fetus; Fetal Therapy; Abortion; Multiple Pregnancy. 

  Objectives: To study the variation in platelet count in critically ill children and correlate its association with their outcomes. Methods: This was a prospective cross-sectional analysis of 150 critically ill children admitted in PICU of our tertiary care hospital over period of 1 year. Laboratory data was collected with daily platelet counts from day of admission till death or discharge whichever was earlier in all patients. The study population was grouped as thrombocytopenic (platelet count < 150 x 10 9/L) and nonthrombocytopenic. They were compared with each other with respect to laboratory parameters and risk factors. Survivor and non survivors were compared with variation in platelet count. Decline in platelet count was correlated with mortality. Chi-square test, median test, ROC curve and forward stepwise binary logistic regression was used for statistical analysis. Results: Forty eight (32%) children had thrombocytopenia. They had significantly higher mortality [14(29.16%) vs 14(13.72%)] and bleeding tendency [13(28.08%) vs 4(3.92%) than non- thrombocytopenic children. Admission thrombocytopenia was not found to be risk factor for mortality. Though survivors and non- urvivors had decline in platelet count in first four days, non-survivors had significantly higher drop. Platelet counts decline >30% at 72 hours was independent risk factor (odds ratio 4.126) for mortality with high sensitivity(91.7%) and specificity (84.4%) with area under ROC curve of 0.898 which was associated with PRISMII. Conclusion: Decline in platelet count can be used as prognostic marker of poor outcome in critically ill children. 

Keywords: Thrombocytopenia; Decline in Platelet Count; Paediatric Intensive Care Unit. 

Aims & Objectives: To compare the efficacy and safety of labetalol versus methyldopa in the management of preeclampsia Materials & Method:s Eligible women were randomly assigned, treated with Tab. Labetalol and Tab Methyldopa alternatively with matching distribution, 85 patients in each group. Results: Labetalol is very effective and has early onset of control of blood pressure control with less side effects. No fetal adverse effects were seen. Conclusion: Labetalol is safe. Inspite of cost, labetalol is very effective and early onset of control of blood pressure is seen. In patients on Labetalol requirement of adding other antihypertensive is much less than methyldopa.With effective control of blood pressure, prevention of eclampsia and the pregnancy can be prolonged to achieve fetal maturity.Labetalol is not associated with adverse fetal effects. 

Keywords: Labetalol; Alpha Methyldopa; Preeclampsia.

Background and Aims: Decreasing mortality in sick and ventilated neonates is an endeavor of all neonatologists. To reduce the high mortality in this group of neonates, identifi cation of risk factors is important. This study was undertaken to fi nd out the indications of ventilation and complications in ventilated neonates and also study possible predictors of outcome. Subjects: Age <1-month; mechanically ventilated; not having suspected metabolic disorders or congenital anomalies; excluding postoperative patients. Methods: Neonates consecutively put on mechanical ventilation during the study period (January 2014 to February2016) enrolled. Primary disease of the neonates along with complications present listed. Clinical and laboratory parameters analyzed to fi nd the predictors of mortality. Results: Total 300  neonates were ventilated. 52% were male. Mean age, weight, and gestational age were 21 ± 62 h, 2320 ± 846.2 g, and 35.2 ± 4.9 weeks, respectively. 130 (43%) neonates died. Respiratory distress syndrome (RDS) (31.1%), sepsis (22.7%), and birth asphyxia (18%) were the most common indications for ventilation. Mortality in ventilated patients with sepsis, pneumonia, RDS or birth asphyxia was 64.7%, 60%, 44.6%, and 33.3%, respectively. Weight <2500  g, gestation <34 weeks, initial pH <7.1, presence of sepsis, apnea, shock, pulmonary hemorrhage, hypoglycemia, neutropenia, and thrombocytopenia were signifi cantly associated with mortality (P < 0.05). Resuscitation at birth, seizures, intra ventricular hemorrhage, pneumothorax, ventilatorassociated pneumonia, PO 2 , or PCO 2 did not have a signifi cant association with mortality. On logistic regression, gestation <34 weeks, initial pH <7.1, pulmonary hemorrhage, or shock were independently signifi cant predictors of mortality. Conclusions: Weight <2500 g, gestation <34 weeks, initial arterial pH <7.1, shock, pulmonary hemorrhage, apnea, hypoglycemia, neutropenia, and thrombocytopenia were signifi cant predictors of mortality in ventilated neonates. 

Keywords: Complications of Ventilation; Neonatal Mechanical Ventilation, Predictors of Mortality. 

Background and Objectives: To compare the effects of selected recorded music with the effects of ambient noise on the physiological and behavioural state responses and weight of preterm infants in NICU. Methods: 50 non-ventilated, preterm infants, 28-36 weeks of gestation aged 1-10 days of life were exposed to music on alternate days at morning and evening for 14 days. The effect of the music was measured before, during , and after by observing the oxygen saturation, heart rate, respiratory rate and wellbeing as measured by behavioural state, and feeding as measured by weight changes; and compared to a control group of 50 infants in whom no music (only standard NICU care with ambient noise) was provided. Results: Oxygen saturations and heart rate did not change significantly in the 10 min before, during or after the music in the intervention (p=0.95 and 0.19) or control group (p=0.23 and 0.20). Respiratory rate decreased in both the groups (p <0.0001 and <0.0002). In the music group Behavioural pattern changed from 3 to 4 of the Thomas score; from before the music therapy sleep-wake transition, to active sleep, showing increased mouthing, sucking and twitching movements of the limbs (p<0.0001) vs no change in control group. Increase in the weight from the mean of 1.5 (0.28) to 1.6 (0.30) after 14 days was seen in infants who received music therapy (p<0.0001). Conclusion: Behavioral state changed significantly from sleep-awake transition to active sleep and significant weight gain occurred after 14 days in music therapy group. There were no significant differences in the heart rate and oxygen saturation over the three measurement points over a period of 40 minute session for both the groups over 14 days. Respiratory rate significantly decreased in both the groups. There was no adverse effect of music therapy. 

Keywords: Music; Premature Infants; Physiological and Behavioral Response. 

Jaundice is one of the commonest problems that can occur in a newborn. Many a times it is physiological in the newborn because liver is not mature enough to handle the bilirubin and there is an increased load of bilirubin due to a higher circulating erythrocyte volume, a shorter erythrocyte life span and a larger early labeled bilirubin peak. Early prediction will help in early discharge and prevent hospitalization of babies and mothers. Albumin is synthesized by liver and it helps in transport of unconjugated bilirubin. Forty newborns were inductedintothe study with the following inclusion criteria: sequentially born term babies (gestational age > 37 weeks) from any mode of delivery, both genders, any birth weight, APGAR score of more than 7 at first and fifth minutes of life, and without Rh incompatibility between mother and child. They were estimated for cord blood serum albumin. Wherever necessary further laboratory tests were done for bilirubin and managed accordingly. Our study showed that 82 % of neonates who had albumin levels less than 2.8 gm/dl developed hyperbilirubinemia requiring phototherapy(PT) and about 12% needed exchange transfusion. At higher levels of albumin that is 2.8 - 3.3 gm/dl, 40% needed PT and with cord blood albumin > 3.3 gm/dl did not need any intervention for hyperbilirubinemia. Hence we can conclude that cord blood albumin levels more than 3.3 gm/dl is probably safe for early discharge of baby. Umbilical cord albumin levels are useful in predicting the development of jaundice in healthy terminfants. 

Keywords: Jaundice; Umbilical cord albumin; Phototherapy. 

  Background: The management of gaped wounds is a great challenge for the surgeons. Human amnion is a readily available and cost effective dressing material for these gaped wounds. Aims: The present study was carried out to study the efficacy of human amnion in gaped wounds following various obstetric and gynecological surgeries. Settings and Design: A prospective and comparative study conducted in the department of obstetrics and gynecology, Pt BDS, PGIMS, Rohtak, Haryana. Methods and Material: Forty patients with wound gaping were randomly allocated into 2 groups, group A (n-20) had wound dressing with human amnion after taking consent and group B (n- 20) had conventional dressing with normal saline, betadene. tatistical Analysis: Chi square test and Fisher exact test. Results: Majority of patients (57.5%) had wound gaping after emergency cesarean section. Out of 20 patients in group A, epithelialization and healthy granulation tissue was observed in majority of the patients (11 patients) after 2 amnion dressings (4 days) whereas in group B, these changes were observed in majority of the patients (14 patients) after 8 days. Hence resuturing was done in  majority of the patients after 4 days and 8 days in group A and B respectively (p value <0.001). Wound depth was markedly minimized in 13 out of 20 patients in group A making resuturing very easy and none in group B (p value <0.001). Conclusion: Human amnion is a readily available, cheap and highly effective dressing material for gaped wounds.

Keywords: Gaped Wounds; Amnion.