Thiamine-responsive, life-threatening, pulmonary hypertensive crisis with encephalopathy in young infants: A case series.

OBJECTIVE: Thiamine (Vitamin B1) deficiency (TD), although reduced in incidence, is still seen in infants. We describe a rarely reported form of infantile TD with life-threatening pulmonary hypertensive crisis and severe encephalopathy, with dramatic response to thiamine supplementation. METHODS: Study design: Descriptive case series. Six young infants with mean age 76 days (range 1-3 months), manifesting rapidly progressive encephalopathy and cardio-pulmonary arrest were included. All infants underwent cardiac, neuroimaging and metabolic evaluations. RESULTS: All six infants had similar presentation with severe pulmonary arterial hypertension (PAH), hypotensive shock, metabolic acidosis and severe encephalopathy. All infants were exclusively breast-fed. Thiamine treatmwnt resulted in dramatic improvement in haemodynamic and neurological function in all the infants. There were no major neurological deficits on follow up. CONCLUSION: A high index of suspicion is warranted for this rarely described form of TD, as early identification helps in preventing mortality and morbidity.

The spectrum of acute leukoencephalopathy with restricted diffusion (ALERD): A case series and review of literature.

INTRODUCTION: The clinico-etiological spectrum of Acute leukoencephalopathy with restricted diffusion (ALERD) is not well known in Indian population. This is likely to vary between populations and ethnicities. METHODS: We retrospectively reviewed the clinicoetiological spectrum of ALERD at a tertiary care pediatric center, and described the clinical, imaging, etiological spectrum and short-term outcomes. RESULTS: Eleven out of 78 children with non-traumatic encephalopathy presenting to our center had a final diagnosis of ALERD. The mean age at presentation was 34.9 months (6-80 months) and 63.6% were males. The monophasic course (72.7%) and the diffuse pattern (63.6%) on neuroimaging were predominant in these children. Dengue haemorrhagic fever was the commonest underlying/triggering infection (5 of 11 children). Ten children required mechanical ventilation in view of neurogenic respiratory failure, with mean duration of ventilation of 6.4 days (Range 2-10 days). The duration of hospital stay varied from 11 to 25 days (Mean - 15.3 days). One child (9 %) died, 6 children (54.5 %) had varying degrees of cognitive impairment and 4 (36.3 %) children had a normal outcome. Children with a shorter duration of ventilation seemed to have a better outcome. CONCLUSION: Dengue haemorrhagic fever was the commonest cause, and diffuse imaging pattern with monophasic course was the commonest presentation in Indian children with ALERD. The clinical presentation and factors influencing outcome are possibly different from previously described literature.

Thiamine-Responsive Acute Pulmonary Hypertension of Early Infancy (TRAPHEI)-A Case Series and Clinical Review.

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of high pulmonary vascular resistance (PVR) commonly seen all over the world in the immediate newborn period. Several case reports from India have recently described severe pulmonary hypertension among infants in the postneonatal period. These cases typically present with respiratory distress in 1-6-month-old infants, breastfed by mothers on a polished rice-based diet. Predisposing factors include respiratory tract infection such as acute laryngotracheobronchitis with change in voice, leading to pulmonary hypertension, right atrial and ventricular dilation, pulmonary edema and hepatomegaly. Mortality is high without specific therapy. Respiratory support, pulmonary vasodilator therapy, inotropes, diuretics and thiamine infusion have improved the outcome of these infants. This review outlines four typical patients with thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) due to thiamine deficiency and discusses pathophysiology, clinical features, diagnostic criteria and therapeutic options.

A quality initiative to improve exclusive breast milk feeding in preterm neonates.

BACKGROUND AND OBJECTIVE: Preterm babies thrive well on exclusive breastfeeding with fewer complications. In a retrospective analysis, challenges were noticed in the form of delayed first milk expression and frequency of expression, which was limited to 2-3 times a day. A quality improvement initiative was designed to increase the exclusive breast milk feeding rates in admitted preterm babies. The purpose of the present study is to evaluate the proportion of preterm neonates receiving mother's own milk by day 7 of life after such an initiative. METHODS: The quality improvement initiative was conducted at a level 3 neonatal intensive care unit from May 10, 2018, to August 10, 2018. Inborn preterm neonates <34 weeks with a minimum hospital stay of 7 days were included. Neonates of critically sick mothers and neonates having major congenital malformations were excluded. Mothers were given structured antenatal counseling regarding expressing breast milk (EBM). Postnatal visits were conducted to provide information through a video and a leaflet and formation of breastfeeding support group. RESULTS: A total of 30 babies were recruited during a period of 3 months and compared to historical controls. The median age of the first EBM improved from 2.5 days to 1.16 days, and the amount of EBM on the first day improved from 0.24 ml to 2.6 ml (p = .002). The proportion of babies receiving EBM on the first day improved from 24% to 80% (p = .0001), and at the end of 7 days, it increased to 73% from 46% (p = .02). The factors time to reach full enteral feeds, time to regain birth weight, rate of necrotizing enterocolitis, rate of sepsis, and proportion of babies on exclusive mother's milk during discharge appear comparable in both the groups. CONCLUSIONS: A simple quality improvement approach through the formation of breastfeeding support group, antenatal counseling, and postnatal support for breast milk expression has made a significant improvement in milk expression from mothers delivered prematurely. It reflects on a team approach using the available resources.

Antimicrobial Justification form for Restricting Antibiotic Use in a Pediatric Intensive Care Unit.

OBJECTIVE: To study whether introduction of an antimicrobial justification form deters clinicians from prescribing restricted antimicrobials and results in de-escalation of these antimicrobials. METHODS: Clinicians were asked to fill a justification form if prescribing an antimicrobial from the pre-identified restricted group. Antimicrobial usage pattern over next year was compared with that in the one year preceding the introduction of justification form. RESULTS: Significant overall decrease in antimicrobial usage (40.5% vs 34.6%) was noted in the post-intervention group along with a significant increase in the de-escalation of antibiotics. CONCLUSION: Introduction of a justification form before prescribing antimicrobials or at the time of deferring de-escalation can be useful in restricting usage of antimicrobials.

Papaverine for Ischemia Following Peripheral Arterial Catheterization in Neonates.

11 Extremely low birth weight neonates who developed skin discoloration after peripheral arterial catheterization were given intra-arterial papaverine before the removal of arterial line. The skin color turned normal in all these neonates and none developed residual damage. In 3 neonates who could not receive papaverine, one developed gangrene of fingers.

Safety and efficacy of intravenous colistin in children.

OBJECTIVE: To observe the safety and efficacy of Colistimethate sodium in children infected with gram-negative bacteria, susceptible only to colistimethate sodium. METHODS: This prospective observational study done over 2 years observed children who received colistin for >48 h, for renal failure as defined by p-RIFLE criteria. RESULTS: Out of 68 children, 52 (76.5%) survived. There were three children with evidence of acute kidney injury and none had neurotoxicity. Serum creatinine significantly decreased at 48 h and at end of treatment, from that at beginning of therapy (P=0.007). CONCLUSIONS: Colistimethate sodium is effective against carbapenem-resistant Gram-negative bacteria, and is safe in children.

ATP-binding cassette transporter A3 (ABCA3) mutation in a late preterm with respiratory distress syndrome.

BACKGROUND: Surfactant protein abnormalities are rare causes of respiratory distress syndrome. CASE CHARACTERISTICS: A late preterm (36 wks) who presented with respiratory distress syndrome. OBSERVATION: He was found to be a homozygous for a G to T transversion at the first base in intron 24, of ABCA3 gene which is necessary for lamellar body formation and surfactant production. OUTCOME: He died of severe respiratory failure even after multiple doses of surfactants and ventilation. MESSAGE: Surfactant deficiency with ABCA3 gene mutation needs to be suspected in late preterms who present with respiratory distress syndrome.

Respiratory flutter syndrome in a neonate.

Respiratory flutter or diaphragmatic flutter is a rare disorder characterized by involuntary, high frequency contractions of the diaphragm. Only 7 cases are reported in infants till date. The present case presented with life threatening respiratory distress immediately after birth. In view of high respiratory rate of 120-154 per minute, clinical and fluoroscopic evidence of diaphragmatic contraction and absence of any obvious CNS, cardiovascular and respiratory pathology, respiratory flutter was diagnosed. It was also associated with dysphagia and laryngomalacia. The patient was managed with prolonged continuous positive airway pressures (CPAP) with partial success, but symptoms improved with use of chlorpromazine.

Charcoal hemoperfusion for phenytoin intoxication.

Accidental ingestion of phenytoin can lead to severe neurological sequelae. Charcoal hemoperfusion decreases phenytoin levels but has never been reported before in children. We present a child with accidental ingestion of phenytoin who responded to charcoal hemoperfusion.

Rescue high frequency oscillation in neonates with acute respiratory failure.

The aim was to study the efficacy of rescue High Frequency Oscillatory Ventilation (HFOV) in improving the oxygenation and ventilation in neonates with acute respiratory failure after failing Conventional Mechanical Ventilation (CMV). Primary outcome was short term oxygenation, lung recruitment, and ventilation and secondary outcome studied was survival. 675 babies were ventilated and 97 of them received HFOV. HFOV significantly improved oxygenation index, alveolar-arterial oxygen gradient, pH, PCO2, PO2 and caused better lung recruitment within 2 hours. Fifty seven babies (58.77%) survived and the mortality was more in <28 weeks, babies with pulmonary hemorrhage, sepsis and CDH.

Transported neonates by a specialist team - how STABLE are they.

The study was designed to compare the biochemical characteristics of neonates transported by qualified transport team, before and after stabilization, interventions required and setbacks during these transports. Prospective cohort study of all neonatal transports during a period of 13 months from various maternity and pediatric centers was conducted; 151 babies who fulfilled the criteria were included in the study. 58% of babies < 32 wks were hypothermic, 21% of the total babies were hypotensive and a total of 25% of the babies were hypoxic at the referral hospital. Intubation was done by the transport team in 23% of babies and 25 % of the babies were transported on a ventilator. There was a significant improvement in the clinical condition of all these babies in relation to temperature control, sugar control, oxygenation and blood pressure after stabilization.

Prolonged neonatal interhospital transport on road: relevance for developing countries.

OBJECTIVE: To compare prolonged inter hospital long distance transports on road undertaken by a qualified transport team vs those done by the same team from shorter distances and time. METHODS: Retrospective descriptive comparative study of the neonatal transports done during a period of 48 mo. All neonates transferred on road to a tertiary level pediatric hospital from various maternity and pediatric centers. The biochemical characteristics, adverse effects during transport and 24 hr survival after the transport in both the groups were compared. RESULTS: The babies were comparable in their gestational age and ventilatory requirements (46% vs 39%). The biochemical and metabolic characteristics and 24 hr mortality rates for babies who were transported for longer times and distances were comparable (p value =0.75) to those transported for shorter times. CONCLUSION: Long distance neonatal transport on road is feasible and with a qualified team results can be comparable to those transported from shorter distances.

Myocardial dysfunction in severe falciparum malaria.

Severe malaria causes multiorgan dysfunction, which is the predominant reason for mortality in these children. Cardiac enzymes have been elevated and cardiac involvement has been suspected in some of these children, however, clear cut echocardiographic evidence for it was not possible. There were isolated reports of myocardial dysfunction in malaria in adults but none in children. We present two such children with cardiac involvement and myocardial dysfunction.

Long distance neonatal transport--the need of the hour.

This study compares the inter hospital long distance transports undertaken by a qualified transport team versus those done by other means. This was a retrospective descriptive comparative study of the neonatal transports done during a period of 33 months from various maternity and pediatric centers to a pediatric tertiary referral centre. We found that biochemical and temperature disturbances are more common in babies transported on their own and a specialized neonatal transport service could improve the survival of these babies. Survival was 96.2% (154/160) and 89% (114/128) in the two groups, respectively (P=0.03).