Kıymet Çelik

Effect of unconjugated hyperbilirubinemia on neonatal autonomic functions: evaluation by heart rate variability

Abstract Background: Serum bilirubin levels beyond the physiological limits, may lead to alterations in autonomic regulation in a newborn infant. Heart rate variability (HRV), is a noninvasive and quantitative marker of the activity of the autonomic nervous system (ANS). To date, few studies have demonstrated the undesirable effects of severe unconjugated hyperbilirubinemia (UHB) on autonomic functions, and only one study has used HRV as a marker of the autonomic activity. However, the relationship between altered cardiac autonomic functions and UHB by using the HRV derived from 24-hour Holter electrocardiography (ECG) recording has not been investigated previously. Objective: We aimed to assess whether a relationship exists between severe UHB and cardiac autonomic dysfunction by evaluating HRV via 24-hour Holter ECG recording. Methods: This single-center, prospective, case-control study was conducted on 50 full-term newborn infants with severe UHB requiring phototherapy and 50 healthy infants as controls. HRV assessment was performed by using 24-hour Holter ECG recording. Results: There was no significant difference in terms of mean average heart rate, mean maximum heart rate and mean RR duration between the groups. However, mean minimum heart rate was significantly lower in the study group. When 24-hour time and frequency domain parameters were compared, time and frequency domain parameters rMSDD as well as high frequency (HF), which represent parasymphathetic activity, were significantly higher in the study group. Furthermore, low frequency to high frequency (LF/HF) ratio, that serves as an indicator of sympathovagal balance, was significantly lower in the study group. Conclusion: Severe UHB may cause cardiac autonomic dysfunction in favor of parasympathetic predominance in jaundiced neonates.

Incidence and risk factors of postnatal growth restriction in preterm infants

Abstract Aim: Preterm infants are often significantly underweight at the time of hospital discharge. Growth impairment during early infancy can have permanent detrimental effects. We aimed to evaluate the incidence and risk factors of postnatal growth restriction (PNGR) in preterm infants. Methods: This prospective study included 124 preterm infants in neonatal intensive care unit between January 2015 and June 2016. Results: Of 140 preterm infants screened, postnatal growth retardation was detected in 46 babies (37%). Low gestational age was independent risk factors for PNGR. Conclusion: Clinicians should be aware of the presence of this morbidity when caring for preterm infants and to provide optimal enteral nutrition.

Nonketotic Hyperglycinemia in the Neonatal Period: Clinical Features, Diagnosis and Treatment

Nonketotic hyperglycinemia, is a recessively inherited autosomal disorder of the amino acid metabolism caused by a deficiency in the mitochondrial glycine cleavage system. Neonatal type is the most common form. Infants are usually normal at birth and clinical manifestations such as severe hypotonia, poor feeding, seizures, and lethargy progressing rapidly to a deep coma are seen during the first few days of life. Majority of the affected infants die during the first weeks of life. Those who survive develop severe neurological sequelae. The aim of this case series is to evaluate the clinical features, treatment approaches and short term prognosis of the infants diagnosed with neonatal nonketotic hyperglycinemia during the last 5 years in our department. Data were collected retrospectively from patients’ files whose postnatal age at diagnosis varied between 2 to 14 days. All patients were admitted with failure to suck and lethargy, and all had severe hypotonia and decreased newborn reflexes on physical examination. Four patients developed resistant myoclonic seizures and deep apnea requiring mechanical ventilation support. In all patients diagnosis was made based on high plasma and cerebrospinal fluid glycine levels. Genetic study could be performed in only one patient. However enzymatic analysis could not be performed in any patient. All patients demonstrated pathological neuroimaging results, and electroencephalographic abnormalities including multifocal epileptiform abnormalities and “burst supression” patterns in four patients. All patients received low protein diet and drugs reducing plasma glycine levels. Treatment-refractory seizures could only be controlled by levetiracetam in four patients. While two patients died during follow-up, and remaining three patients survived with severe neurological sequels. Physicians should consider nonketotic hyperglycinemia in differential diagnosis in our country where consanguineous marriages are frequent, especially when a newborn healthy for a certain time period develops severe hypotonia, resistant seizures and encephalopathy as detected with routine laboratory tests performed during followup period.

A Newborn Case of “c” Subgroup Mismatch Presenting with Severe Hemolysis and Anemia

249 ©Copyright 2017 by Ege University Faculty of Medicine, Department of Pediatrics and Ege Children’s Foundation The Journal of Pediatric Research, published by Galenos Yayınevi. Ad dress for Cor res pon den ce/Ya z›fl ma Ad re si Ezgi Yangın Ergon MD, University of Health Sciences, Dr. Behçet Uz Children’s Diseases and Surgery Training and Research Hospital, Department of Neonatology, İzmir, Turkey Phone: +90 533 348 56 45 E-mail: yanginezgi@yahoo.com ORCID ID: orcid.org/0000-0003-0836-7379 Re cei ved/Ge liş ta ri hi: 02.06.2016 Ac cep ted/Ka bul ta ri hi:10.09.2016 Ağır Hemoliz ve Anemiyle Başvuran “c” Subgrup Uyuşmazlıklı Bir Yenidoğan Olgusu