Mukti Sharma

Congestive Heart Failure in Infants and Children.

Congestive heart failure (CHF) refers to a clinical state of systemic and pulmonary congestion resulting from inability of the heart to pump as much blood as required for the adequate metabolism of the body. The clinical picture of CHF results from a combination of “relatively low output” and compensatory responses to increase it. Excellent reviews on CHF in infants and children are available [1, 2]. This article provides core information on selected aspects of CHF. Broadly, heart failure results either from an excessive volume or pressure overload on normal myocardium (left to right shunts, aortic stenosis), or from primary myocardial abnormality (myocarditis, cardiomyopathy). Arrhythmias, pericardial diseases and combination of various factors can also result in CHF. The resultant decrease in cardiac output triggers a host of physiological responses aimed at restoring perfusion of the vital organs [3]. Important among these are renal retention of fluid, renin-angiotensin mediated vasoconstriction and sympathetic overactivity. Excessive fluid retention increases the cardiac output by increasing the end diastolic volume (preload), but also results in symptoms of pulmonary and systemic congestion. Vasoconstriction (increase in afterload) tends to maintain flow to vital organs, but it is disproportionately elevated in patients with CHF and increases myocardial work. Similarly, sympathetic over-activity results in increase in contractility, which also increases myocardial requirements. An understanding of the interplay of the four principal determinants of cardiac output – preload, afterload, contractility and heart rate is essential in optimising the therapy of CHF. It is clinically useful to consider CHF in different age groups separately.

Beckwith Weidemann Syndrome

Since first described in 1963, Beckwith Weidemann syndrome is increasingly being recognised as an important genetic overgrowth disorder. With the characteristic diagnostic triad of exomphalos, macroglossia and gigantism, its early recognition is necessary because of the associated risk of hypoglycaemia in the neonatal period, embryonal neoplasms in childhood and for the purpose of genetic counselling. We report one such classical case.

Effects of maternal epidural analgesia on the neonate - a prospective cohort study

BackgroundEpidural analgesia is one of the most popular modes of analgesia for child birth. There are controversies regarding adverse effects and safety of epidural analgesia. This study was conducted to study the immediate effects of the maternal epidural analgesia on the neonate during early neonatal phase.MethodsA prospective cohort study of 100 neonates born to mothers administered epidural analgesia were compared with 100 neonates born to mothers not administered epidural analgesia in terms of passage of urine, initiation of breast feeding, birth asphyxia and incidence of instrumentation.ResultsThere was significant difference among the two groups in the passage of urine (P value 0.002) and incidence of instrumentation (P value 0.010) but there was no significant difference in regards to initiation of breast feeding and birth asphyxia.ConclusionsEpidural analgesia does not have any effect on the newborns in regards to breast feeding and birth asphyxia but did have effects like delayed passage of urine and increased incidence of instrumentation.

Persistent pulmonary hypertension of the newborn: a review

Persistent pulmonary hypertension of the newborn (PPHN) is a serious medical emergency in the neonatal period which occurs because of failure of transition of the foetal circulation into the normal circulation. The condition is characterised by persistently elevated pulmonary vascular pressures and despite numerous modalities of treatment available, the condition carries with it a high rate of mortality and morbidity. Early awareness of predisposing conditions and early diagnosis leads to better outcomes in PPHN.

Profile of Live-born Infants of In-vitro Fertilisation

INTRODUCTION The Army Hospital (R&R) is the only service hospital providing in-vitro fertilisation (IVF) facility. Neonatal characteristics of live-born infants at this centre over a two-year period are analyzed in this study. METHODS Data on 504 consecutive live-born IVF infants over a two-year period (01 Feb 2007 to 31 Jan 2009) were analysed. RESULT Of the 504 neonates, 190 (37.7%) were born by vaginal delivery, 156 (30.9%) by elective lower segment cesarean section (LSCS) and 127 (25.19%) following emergency LSCS. Maternal illness posing specific risk to the neonate was present in 165 out of 504 (32.7%). There were 239 (47.4%) preterm neonates. Males formed 51.8% of the cohort. Singletons accounted for 51.2%, while the rest (48.8%) were products of twin pregnancies. Small for gestational age neonates formed 22.6% (n = 114). A total of 20 (3.9%) infants had congenital malformations. There were 242 (48.1%) low birth weight neonates. A total of 128 (25.4%) neonates needed neonatal intensive care. Of the 504, there were 474 (94.1%) survivors while 30 (5.9%) did not survive. Twenty-nine (6.1%) neonates required readmission during the neonatal period. CONCLUSION In our setting, neonates born following IVF appeared to be at increased risk of prematurity, multiple births and low birth weight. Proper obstetric and neonatal management can result in good neonatal outcomes.

PROSTAGLANDINS IN CONGENITAL HEART DISEASE

Prior to the use of prostaglandins (PGE1) many infants with ductus arteriosus dependent congenital heart disease (CHD) born outside tertiary care centres did not survive the period from diagnosis to inter-hospital transportation for surgery. Development in the area of neonatal cardiac surgery has increased the importance of rapid diagnosis and stabilisation of infants with CHD. Survival after cardiac defect repair has increased in the past decade. Perhaps an important factor in this increase appears to be the early use of PGE1 to provide a more clinically stable patient for surgery [1]. Therapy with PGE1 can effectively maintain an infants cardiovascular function until palliative or corrective procedure can be performed. At present many hospitals do not maintain a supply of PGE1 mainly because of its expense and limited use due to the various side effects. Pharmacology of the Ductus Arteriosus To appreciate the importance of PGE1 therapy it is necessary to briefly review the physiology of the ductus and its relevance to infants with CHD. In the foetus ductus arteriosus connects pulmonary artery to descending aorta. Endogenous prostaglandins produced during gestation keep the ductus patent, as a result, majority of the blood passes from pulmonary artery through the ductus by passing the lungs and going directly to the aorta where it is transported to placenta for oxygenation. At birth, an increase in arterial oxygen saturation and a decrease in amount of endogenous prostaglandins, both stimulate an alteration of vascular integrity promoting ductal closure. Exogenous prostaglandins like PGE1 can be used to maintain the patency of the ductus in neonates where bypassing a defective vessel or continued mixing of oxygenated and unoxygenated blood is needed to provide adequate systemic circulation [2]. The constricted ductus arteriosus is unusually sensitive to PGE1 responding with dilation at dosage rates below those required for altering systemic or pulmonary pressure or resistance. Ductal preservation by infusion of PGE1 can be life saving in neonates with heart disease characterised by severe restriction of pulmonary blood flow, poor arterial venous admixture and severe systemic hypoperfusion.

Improving medical stores management through automation and effective communication

BACKGROUND Medical stores management in hospitals is a tedious and time consuming chore with limited resources tasked for the purpose and poor penetration of Information Technology. The process of automation is slow paced due to various inherent factors and is being challenged by the increasing inventory loads and escalating budgets for procurement of drugs. METHODS We carried out an indepth case study at the Medical Stores of a tertiary care health care facility. An iterative six step Quality Improvement (QI) process was implemented based on the Plan-Do-Study-Act (PDSA) cycle. The QI process was modified as per requirement to fit the medical stores management model. The results were evaluated after six months. RESULTS After the implementation of QI process, 55 drugs of the medical store inventory which had expired since 2009 onwards were replaced with fresh stock by the suppliers as a result of effective communication through upgraded database management. Various pending audit objections were dropped due to the streamlined documentation and processes. Inventory management improved drastically due to automation, with disposal orders being initiated four months prior to the expiry of drugs and correct demands being generated two months prior to depletion of stocks. The monthly expense summary of drugs was now being done within ten days of the closing month. CONCLUSION Improving communication systems within the hospital with vendor database management and reaching out to clinicians is important. Automation of inventory management requires to be simple and user-friendly, utilizing existing hardware. Physical stores monitoring is indispensable, especially due to the scattered nature of stores. Staff training and standardized documentation protocols are the other keystones for optimal medical store management.

Gaucher's disease: rare presentation of a rare disease.

Gaucher’s disease is a rare lysosymal storage disorder characterized by deposition of glucocerebroside in cells of the macrophage monocyte system. Gaucher’s disease has 3 types—non-neuronopathic (type I), acute neuronopathic (type II), and chronic neuronopathic (type III). It generally presents with delayed milestones, seizures, bony deformities, or massive organomegaly. The acute neuronoapthic variety is the rarer type that predominantly presents with neurological features. The authors present a case of the acute neuronopathic variety of Gaucher’s disease where the child presented with only abnormal head position.

Perinatal outcomes among children born by assisted reproductive techniques—a hospital-based case control study

BACKGROUND Widespread use of assisted reproductive techniques (ART) has raised major concern about the outcome of resulting pregnancies, as well as about the health of the newborn children. The ART conceived pregnancies have an increased risk for prematurity and low birth weight (LBW). The risk of congenital malformations among children conceived via ART is contemplated to be high. The present case control study was conducted with an aim of analysing perinatal outcomes of children born by ART in the Indian context. METHODS The case control study was conducted in the Army Hospital (Research and Referral), New Delhi. It included 82 cases and 164 controls. The data was collected on maternal and newborn characteristics among cases and controls. Perinatal outcomes were compared between ART conceived and spontaneously pregnant women in terms of birth weight, preterm, multiple pregnancies, and neonatal complications. RESULTS Significant difference was observed in terms of the preterm delivery, birth weight, neonatal complications between the cases and the controls. The adjusted odds ratio (OR) was 21.6 (95% confidence interval [CI] 4.3, 112.9) for preterm delivery; 6.0 (95% CI 1.1, 43.8) for multiple pregnancy and 3.2 (95% CI 1.5, 7.0) for caesarean section. The risk of LBW and neonatal complications was heavily confounded by preterm delivery and multiple pregnancies, the adjusted OR being 0.9 and 1.0, respectively. CONCLUSION Increased risk of preterm delivery, multiple pregnancies and caesarean section was associated with ART. The LBW was confounded by preterm delivery and multiple pregnancies.

Original ArticleProfile of Live-born Infants of In-vitro Fertilisation

INTRODUCTION The Army Hospital (R&R) is the only service hospital providing in-vitro fertilisation (IVF) facility. Neonatal characteristics of live-born infants at this centre over a two-year period are analyzed in this study. METHODS Data on 504 consecutive live-born IVF infants over a two-year period (01 Feb 2007 to 31 Jan 2009) were analysed. RESULT Of the 504 neonates, 190 (37.7%) were born by vaginal delivery, 156 (30.9%) by elective lower segment cesarean section (LSCS) and 127 (25.19%) following emergency LSCS. Maternal illness posing specific risk to the neonate was present in 165 out of 504 (32.7%). There were 239 (47.4%) preterm neonates. Males formed 51.8% of the cohort. Singletons accounted for 51.2%, while the rest (48.8%) were products of twin pregnancies. Small for gestational age neonates formed 22.6% (n = 114). A total of 20 (3.9%) infants had congenital malformations. There were 242 (48.1%) low birth weight neonates. A total of 128 (25.4%) neonates needed neonatal intensive care. Of the 504, there were 474 (94.1%) survivors while 30 (5.9%) did not survive. Twenty-nine (6.1%) neonates required readmission during the neonatal period. CONCLUSION In our setting, neonates born following IVF appeared to be at increased risk of prematurity, multiple births and low birth weight. Proper obstetric and neonatal management can result in good neonatal outcomes.