Dry lung syndrome: A distinct clinical entity.

Infants with preterm prolonged rupture of membranes can present with immediate onset of pulmonary insufficiency and need aggressive ventilatory support. Dry lung syndrome remains an under-recognised clinical entity and very few cases are reported in the literature, though a working definition has been previously proposed. The proposed underlying etiopathogenesis for this is functional hypoplasia of the lung where higher than usual ventilatory pressure for initial 1-2 days can improve oxygenation. This study presents two cases which had similar antenatal and immediate postnatal course, but had diametrically opposite outcomes. Strategies to optimise outcomes in the setting of early premature rupture of the membranes should include antenatal assessment of mothers with monitoring of the liquor volume as well as the strength of foetal breathing movements, in-utero transfer with delivery in perinatal centres and attendance by neonatal staff of appropriate experience.

Patent ductus arteriosus ligation and post-operative hemodynamic instability: Case report and framework for enhanced neonatal care.

Ligation of a patent ductus arteriosus can lead to severe cardiorespiratory compromise in preterm infants. This report reviews the postoperative course of a patient with significant cardiorespiratory instability following surgical ligation of the patent ductus arteriosus and presents a framework for enhanced cardiovascular care in this population. A preterm infant, born at 24 wk gestation underwent ligation of a large haemodynamically significant ductus arteriosus after failure of 2 courses of indomethacin. He developed systemic hypotension, which was aggressively treated with high doses of multiple cardiotropic agents. After 10 hr of refractory hypotension, the addition of hydrocortisone normalized blood pressure. This article outlines preprocedural categorization of infants according to ductal illness severity which facilitates the risk assignment for postoperative deterioration, development of clinical guidelines specific to the likely haemodynamic changes, enhanced role of functional echocardiography for guiding therapy, and interprofessional education.

Surfactant and patent ductus arteriosus.

Objective. To investigate the relationship between surfactant replacement therapy and the development of a haemodynamically significant ductus arteriosus. Methods. All premature infants at 28-32 wk gestation with a clinical diagnosis of respiratory distress syndrome were retrospectively reviewed and subdivided into two groups; intubated, mechanically ventilated and received surfactant (Group 1); and received nasal continuous positive airway pressure alone (Group 2). The relationship between groups and characteristics of the hemodynamically significant ductus arteriosus was analyzed. Results. Seventy babies were identified of whom 35 (50%) received surfactant. Babies in group I and II were comparable for gestational age, birthweight, antenatal steroids, gender and fluid intake in first week of life. Babies treated with surfactant therapy were found to be more likely to have a haemodynamically significant ductus arteriosus (p< 0.01), larger transductal diameter (p=0.01) and increased rate of therapeutic interventions to close the ductus (p<0.01). Ventilation parameters (mean airway pressure and fractional inspired oxygen) were higher in group I. Conclusion. Neonates with respiratory distress syndrome (RDS) who were treated with surfactant replacement are at increased risk of a hemodynamically significant ductus arteriosus that requires therapeutic intervention. Whether the relationship reflects their underlying lung disease or is a direct effect of surfactant requires prospective evaluation.

Developmentally supportive care and NIDCAP.

With increasing advances in field of perinatal medicine and new breakthroughs in technology, the perinatal mortality has shown a steady decline worldwide. This has given rise to a new dimension, that of developmentally oriented care of these sick neonates while they are still in intensive care. NIDCAP &developmentally supportive care is aimed at decreasing the stress levels in these babies by individualized care which also involves the family. Growth and development has been shown to get enhanced by this approach. There are only few well designed studies on this issue of extreme importance. This article presents a brief review of current literature as well as outlines relevant policies followed in the author's unit.

Experience with inhaled nitric oxide therapy in hypoxic respiratory failure of the newborn.

BACKGROUND: Respiratory diseases are the commonest cause of morbidity and mortality in newborns. Inhaled nitric oxide (iNO) has been shown to be effective in the management of persistent pulmonory hypertension of newborn (PPHN). OBJECTIVES: To retrospectively analyse data to determine the effectiveness of inhaled nitric oxide (iNO) in the management of newborns with PPHN in terms of survival and changes in oxygenation status. METHODS: Neo-natal data since inception of iNO therapy at the unit (past six years) was reviewed. Pertinent demographic and clinical information was collected from medical records of newborns that received inhaled nitric oxide therapy during their stay. Details of underlying illnesses, other therapeutic modalities, arterial blood gas, ventilatory and nitric oxide parameters were assessed and analysed to ascertain efficacy of iNO. RESULTS: A total of 36 babies (gestational age ranging from 24-41 weeks) received iNO during this period; two were excluded from final analysis. Overall survival rate was 80 percent. There was a statistically significant increase in systemic oxygenation (PaO2) from 41.1 +/- 2.1 mmHg to 128.5 +/- 13.2 mmHg and a decline in oxygenation index (OI) from 49.4 +/- 5.9 to 17.3 +/- 2.5, when assessed after four hours (P < 0.001). Mean duration of iNO therapy was 63 +/- 7.3 hours and the maximum methaemoglobin levels were noted to be 2.1 percent. CONCLUSIONS: Inhaled nitric oxide appears to be an effective rescue therapy for the management of PPHN associated with hypoxic respiratory failure. It is safe and well tolerated with no evidence of clinical or biochemical side effects.

Liquid ventilation.

Respiratory diseases are the commonest cause of morbidity and mortality in newborn babies. During the past few years several new modalities of treatment like surfactant have been introduced. One of them, and probably the most fascinating, is of liquid ventilation. Partial liquid ventilation, on which much of the existing research has concentrated, requires partial filling of lungs with perfluorocarbons (PFC's) and ventilation with gas tidal volumes using a conventional mechanical ventilators. Various physico-chemical properties of PFC's make them the ideal media. It results in a dramatic improvement in lung compliance and oxygenation and decline in mean airway pressure and oxygen requirements. It shows further promise for lung lavaging procedures, pulmonary image enhancement, pulmonary administration of drugs and as a technique to increase functional residual capacity in lung hypoplasia syndromes. There are no long-term side effect reported.

Scimitar syndrome.

Scimitar syndrome is a relatively uncommon constellation of cardio-pulmonary anomalies, its typical feature being partial anomalous pulmonary venous connection. It can present in the neonatal period as well as later in life. We present the case of a girl diagnosed in the newborn period, along with a brief review of literature.

Complex glycerol kinase deficiency: an X-linked disorder associated with adrenal hypoplasia congenita.

Complex glycerol kinase deficiency (GKD) results from the contiguous deletion on Xp21 of all or part of the gene for glycerol kinase together with that for adrenal hypoplasia congenita (AHC) and /or Duchenne muscular dystrophy (DMD). The authors present the case of a newborn whose initial issues were refractory hypoglycaemia along with hyponatremia and hyperkalemia. He also had low serum cortisol levels and raised urinary excretion of glycerol and required steroid supplementation. His creatinine phosphokinase (CPK) levels were normal. Molecular studies revealed a contiguous Xp21 deletion. Therapy in such cases must be prompt and includes correction of hypoglycaemia and dyselectrolytemia, a low fat diet and steroid replacement.

Parental awareness regarding childhood injuries.

OBJECTIVE: To assess parental awareness regarding common childhood injuries and to focus on preventive issues. METHODS: Two hundred parents of children each grouped into 2 attending OPD of government hospital and private clinic respectively were enrolled, interviewed and responses analyzed. RESULTS: The commonest potential mode of injuries identified in both groups was falls (53% vs 84%). None of the households in group 1 and less than 2/3rd in group 2 had provision of restraints at stairs. Awareness was found to be greater in the group with higher educational background and socioeconomic status. CONCLUSION: Awareness regarding common childhood injuries and their prevention was lacking. There was a felt need that the pediatricians must counsel the parents on this aspect during visits. Since the sample size is small, a larger community based study is needed.

Maternal and neonatal profile and immediate outcome in ELBW babies.

The present study was designed to ascertain the maternal and neonatal profile and immediate outcome of extremely low birth weight (ELBW) babies at a Level III neonatal intensive care unit (NICU). Case records of ELBW inborn babies delivered between August 2000 and August 2001 were analyzed by using a preset proforma. A total of 52 ELBW babies were admitted in NICU in the relevant period, out of which 30 (57%) survived. Maternal anemia and previous pre-term (PT) delivery were the common predisposing factors for PT delivery. Mean gestational age was 27.8 weeks and mean birth weight was 831 grams. Mortality was highest in babies less than 28 weeks gestation. Neonatal hyper-bilirubinemia (78%) and HMD/RDS (65%) were the commonest morbidity. Retinopathy of Prematurity (ROP) screening could be done in 35 babies (68%), out of which 22 were found to be normal.

Comparison of tympanic and rectal temperatures in febrile children.

The present study was designed to assess the accuracy of tympanic membrane temperature (TMT) in predicting "core" body temperature and to compare rectal temperature (RT) and TMT in febrile pediatric patients with and without meningitis. Sixty children diagnosed as having meningitis by cerebro-spinal fluid (CDF) analysis formed the cases and 60 non-meningitic febrile patients, chosen as continuous enrollment, formed the controls. Rectal and ear temperatures were assessed in both groups. Ear temperature was significantly higher in cases as compared to controls. The difference between reading of ear temperature and rectal temperature was also significantly higher in cases as compared to controls. Significant correlations were seen between ear temperature and various parameters of CSF profile.

Foreign body aspiration.

OBJECTIVE:To analyze the clinical and radiological profile of foreign body aspiration in children reaching a tertiary care center and identify areas of possible interventions for proper management of such cases. SETTING: Tertiary level teaching hospital. SUBJECTS: Case records of patients suspected to have foreign body aspiration over the past four years were analyzed. Clinico-radiological features, types and location of foreign bodies were studied. RESULTS: Of 75 children who underwent rigid bronchoscopy, 70 had tracheo-bronchial foreign bodies. History of choking was elicited in 90 percent cases. In 30 percent cases chest radiographs were non-contributory, while the commonest finding (63 percent) was distal emphysema. Over three-fourth of the cases were below the age of 2 years. Vegetative foreign bodies, mainly peanuts, were commonly present. In many cases, referral was delayed as the diagnosis was missed initially. CONCLUSION: Foreign body aspiration remains a common unintentional childhood injury due to improper exposure of young children to otherwise innocuous looking nuts and other small objects.

Comparison of tympanic and rectal temperature in febrile patients.

OBJECTIVE: To compare tympanic membrane temperature and rectal temperature in febrile pediatric patients. METHODS: Sixty febrile children were enrolled as continuous enrollment at initial triage. Two readings of ear temperature were taken in each child with Thermoscan infrared thermometer. Rectal temperature was recorded by a digital electronic thermometer. Comparison of both the techniques was done and co-relation co-efficients calculated. Parental preference for both techniques was assessed. RESULTS: It was observed that mean ear temperature was 38.9+/-0.90 C and that for rectal temperature was 38.8+/-0.80 degrees C. The correlation coefficient between the two was 0.994 (p < 0.01). Coefficients for both sites were comparable over a wide age range. The difference between readings taken from two ears was not significant. Temperature ranges over which readings were recorded were quite wide for both techniques. Parental preference for tympanic thermometry over rectal thermometry was noticed. Tympanic thermometry utilizes pyro-electric sensors, to detect infra-red rays emitted from the surface of tympanic membrane. Ear temperatures correlates well with rectal temperatures which have long been considered as "core" temperatures. Parents prefer the technique of ear thermometry which is quick (2 sec), safe and non-invasive and patient resistance for this is also less. CONCLUSION: A non-invasive, non-mucous device which is accurate over a wide range of temperature could be very useful.

Congenital diaphragmatic hernia: delayed presentation with asymptomatic spleenic herniation.

Patients with congenital diaphragmatic hernia (CDH) usually present in the immediate neonatal period with respiratory distress. Presentation beyond the neonatal period has, however, been reported. We report a case of a 3-year-old child who presented with a six months history of respiratory symptoms and had been subjected to a variety of therapies like antibiotics, antitubercular medication and two attempts at putting an intercostal tube, as radiographs were suggestive of pleural effusion. This study highlights the importance of a high index of suspicion, relevance of thorough clinical examination and non-invasive diagnostic modalities along with an uncommon occurrence of asymptomatic spleenic herniation at such a later age. Spleenic herniation is rare outside neonatal period.