Muralidhar H. Premkumar

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Nitric oxide (NO) is crucial in diverse physiological and pathological processes. We show that a hypomorphic mouse model of argininosuccinate lyase (encoded by Asl) deficiency has a distinct phenotype of multiorgan dysfunction and NO deficiency. Loss of Asl in both humans and mice leads to reduced NO synthesis, owing to both decreased endogenous arginine(More)
Necrotizing enterocolitis (NEC) is the most common gastrointestinal complication in human neonates, yet the pathogenesis of this disease remains poorly understood. A fundamental approach to understanding the etiology and underlying biology of NEC is the use of in vivo experimental animal models, primarly neonatal rodents and pigs. The rodent models using(More)
Bronchopulmonary dysplasia (BPD), which has long-term adverse outcomes, is common following extremely premature birth. BPD has a multifactorial etiology, including a high level or prolonged use of mechanical ventilation. Numerous research studies, therefore, have attempted to identify ventilatory techniques which reduce the likelihood of baro/volutrauma and(More)
Argininosuccinate lyase (ASL) is required for the synthesis and channeling of L-arginine to nitric oxide synthase (NOS) for nitric oxide (NO) production. Congenital ASL deficiency causes argininosuccinic aciduria (ASA), the second most common urea-cycle disorder, and leads to deficiency of both ureagenesis and NO production. Subjects with ASA have been(More)
Scrotoschisis is a rare congenital defect of the scrotal sac associated with extrusion of one or both testicles. Only 9 cases have been reported in the literature. The exact mechanism causing this rare congenital defect is unknown. We describe a case of unilateral scrotoschisis in a 35-week preterm infant delivered by cesarean section. The defect was(More)
Soybean oil-based intravenous fat emulsions (IVFEs) have been the predominant parenteral nutrition IVFE used in the United States for neonates over the past 45 years. Even though this emulsion has proven useful in supplying infants with energy for growth and essential fatty acids, there have been concerns over its composition in the development of several(More)
Background:Necrotizing enterocolitis (NEC) is an inflammatory bowel necrosis of premature infants. Based on our recent findings of increased Smad7 expression in surgically resected bowel affected by NEC, we hypothesized that NEC macrophages undergo inflammatory activation because increased Smad7 expression renders these cells resistant to normal,(More)
BACKGROUND Early use of nasal continuous positive airway pressure (nCPAP) may reduce lung damage, but it is not clear how many extremely preterm infants can be cared for without mechanical ventilation on the first days after delivery. OBJECTIVES To describe our experience of nCPAP in infants born at <27 weeks' gestation and to determine the chance of(More)
We describe an infant with popliteal pterygia, syngnathia, cleft lip and palate, and retrognathia diagnosed with popliteal pterygium syndrome (PPS). The neonatal course was complicated by severe obstructive apnea necessitating tracheostomy. Conclusion: This report illustrates the potential for airway compromise in PPS patients and the need for thorough(More)
OBJECTIVE To determine factors leading to resolution of cholestasis in patients with parenteral nutrition-associated liver disease treated with fish oil-based lipid emulsion (FOLE). STUDY DESIGN Prospective observational study of 57 infants <6 months of age with parenteral nutrition-associated liver disease who received parenteral FOLE as monotherapy. (More)