Early neonatal outcomes of very-low-birth-weight infants in Turkey: A prospective multicenter study of the Turkish Neonatal Society.

OBJECTIVE: To investigate the early neonatal outcomes of very-low-birth-weight (VLBW) infants discharged home from neonatal intensive care units (NICUs) in Turkey. MATERIAL AND METHODS: A prospective cohort study was performed between April 1, 2016 and April 30, 2017. The study included VLBW infants admitted to level III NICUs. Perinatal and neonatal data of all infants born with a birth weight of ≤1500 g were collected for infants who survived. RESULTS: Data from 69 NICUs were obtained. The mean birth weight and gestational age were 1137±245 g and 29±2.4 weeks, respectively. During the study period, 78% of VLBW infants survived to discharge and 48% of survived infants had no major neonatal morbidity. VLBW infants who survived were evaluated in terms of major morbidities: bronchopulmonary dysplasia was detected in 23.7% of infants, necrotizing enterocolitis in 9.1%, blood culture proven late-onset sepsis (LOS) in 21.1%, blood culture negative LOS in 21.3%, severe intraventricular hemorrhage in 5.4% and severe retinopathy of prematurity in 11.1%. Hemodynamically significant patent ductus arteriosus was diagnosed in 24.8% of infants. Antenatal steroids were administered to 42.9% of mothers. CONCLUSION: The present investigation is the first multicenter study to include epidemiological information on VLBW infants in Turkey. Morbidity rate in VLBW infants is a serious concern and higher than those in developed countries. Implementation of oxygen therapy with appropriate monitoring, better antenatal and neonatal care and control of sepsis may reduce the prevalence of neonatal morbidities. Therefore, monitoring standards of neonatal care and implementing quality improvement projects across the country are essential for improving neonatal outcomes in Turkish NICUs.

Systemic Pseudohypoaldosteronism Type 1 due to 3 Novel Mutations in SCNN1Aand SCNN1BGenes.

OBJECTIVE: The systemic form of pseudohypoaldosteronism type 1 (PHA1) is an autosomal recessive disorder characterized by defective sodium transport in multi-organ systems. Mutations in the genes encoding the amiloride-sensitive epithelial sodium channel, ENaC, account for genetic causes of systemic PHA1. We describe systemic PHA1 due to 4 novel variants detected in SCNN1A and SCNN1B in 3 cases from 3 unrelated consanguineous families. PATIENTS AND METHODS: We evaluated the clinical presentations, biochemical and hormonal characteristics, and molecular genetic analysis results of 3 patients from 3 unrelated consanguineous families and parents from whom samples were available. RESULTS: The ages at presentation were postnatal days 9, 10, and 5. The main presentation symptoms were vomiting, poor feeding, weakness, weight loss, and skin rash. All patients exhibited laboratory characteristics including severe hyponatremia, hyperkalemia, metabolic acidosis, elevated plasma renin, elevated aldosterone, and positive sweat tests, suggesting a diagnosis of systemic PHA1. Molecular genetic analysis revealed 2 novel pathogenic variants [c.87C>A(p.Tyr29*)/IVS9 + 1G>A (c.1346 + 1G>A)] in SCNN1Bin case 1, a novel homozygous pathogenic variant [p.His69Arg(c.206A>G] in SCNN1Ain case 2, and a homozygous one-base duplication, p.A200Gfs*6 (c.598dupG), in SCNN1A in case 3. CONCLUSION: PHA1 should be considered at differential diagnosis in patients presenting with hyponatremia, hyperkalemia, and metabolic acidosis. The cases in this report involving 4 novel variants will add valuable insights into the phenotype-genotype relationship and will expand the mutation database.

Combined treatment with chlorhexidine and 0·9% saline in a newborn infant with an infected surgical wound.

Newborns are more susceptible to infection; this makes proper wound care extremely important in them. Unfortunately, in spite of successful surgery, patients can die as a result of wound area infections. Herein, we report a case in which a combined therapy of chlorhexidine (a disinfectant) and saline (a cleansing agent used in wound care) was used effectively to treat the wound in a newborn infant with an antibiotic-resistant, Gram-negative, bacteria-related surgical site infection.

May we use ibuprofen as doses against courses in the treatment of patent ductus arteriosus in premature infants?

OBJECTIVE: We aimed to investigate the efficacy of ibuprofen doses in closing patent ductus arteriosus (PDA) and the possibility of reducing drug-related complications by reducing dose number. METHODS: We performed a prospective study with 60 premature infants (≤33 weeks) who were treated with enteral ibuprofen for hsPDA. Echocardiographic examinations were performed before each dose. Treatment was stopped when PDA was closed and patients were followed for reopening and complications. RESULTS: Rates of closure were 28.3%, 44.1%, 54.1%, 36.3%, 42.8% and 50.0% with the 1st, 2nd, 3rd, 4th, 5th and 6th doses. No closure was observed with 7th, 8th and 9th doses. Reopening was observed only in patients whose PDA closed with the 1st (3.3%), 2nd (1.6%) and 3rd (1.6%) doses. PDA diameters were higher in patients who required >4 doses. Complications were rare (6.6%) but unrelated with dose number. CONCLUSIONS: We conclude that it is possible to minimize ibuprofen exposure and achieve high closure rates of PDA in premature infants by performing echocardiography before each dose. PDA diameter should be used to estimate the duration of treatment. This approach is not effective in reducing complication rates and must be performed in attention to reopening especially for the first three doses.

The Use of Low-Dose Recombinant Tissue Plasminogen Activator to Treat a Preterm Infant with an Intrauterine Spontaneous Arterial Thromboembolism.

Neonatal thromboembolic events are rare, and only a few cases of intrauterine spontaneous arterial thromboembolisms have been reported in the literature. Thrombolytic therapy with recombinant tissue plasminogen activator is usually the preferred treatment because it has a short half-life, fewer systemic side effects, and a strong, specific affinity for fibrin. Protocols vary from center to center, but there is still no consensus regarding the proper dosage or treatment duration. Herein, we present the case of an intrauterine spontaneous arterial thromboembolism in a preterm infant that completely resolved after being treated with low-dose recombinant tissue plasminogen activator (0.02 mg/kg/h).

Safety and Efficacy of Intravenous Colistin in Neonates With Culture Proven Sepsis.

BACKGROUND: Although it is well described among adults, intravenous colistin use and its associated toxicities in newborns are poorly understood. OBJECTIVES: We present our experience of efficacy and safety of intravenous colistin in the treatment of sepsis in term and preterm neonates. PATIENTS AND METHODS: The records of neonates who received colistin between January 2013 and February 2014 were retrospectively reviewed. All neonates with culture proven nosocomial infections due to multidrug resistant organisms and treated continuously with colistin for more than 72 hours were included in the study. RESULTS: Patients were evaluated for clinical and microbiological response to the drug and its and side effects. Twelve newborn infants with mean 31.8 ± 3.5 weeks gestational age and median 1482 (810 - 3200) gram birth weight were included. 11/12 (91.7%) patients showed microbiological clearance with intravenous colistin. One patient who had recurrent cerebrospinal fluid positive culture was treated with intraventricular colistin. The major side effects observed was hyponatremia and hypokalemia in 2 (16.6%) patients, all infants required magnesium supplementation. CONCLUSIONS: Intravenous colistin administration appears to be safe and efficacious for multidrug-resistant gram-negative infections in neonates, including preterm infants. However, we believe that large prospective controlled studies are needed to confirm its efficacy and safety in neonates.

Evaluation of Premature Infants Hospitalized in Neonatal Intensive Care Unit between 2010-2012.

OBJECTIVE: With continuing developments in the field of neonatology, survival rates of low birth weight and small for gestational age infants have increased, which in turn has brought important prematurity-related problems. The aim of this study was to evaluate retrospectively the prematurity problems that are the significant causes of morbidity and mortality. MATERIALS AND METHODS: 613 premature infants hospitalized in the neonatal intensive care unit of Ataturk University Medical Faculty Hospital between January 2010 and January 2012 were included in this study. Infants were divided into groups according to their birth weight and gestational age. RESULTS: 323 infants were male (52.6%) and 290 were female (47.4%). 63.9% of infants weighed ≥1500 grams, and 58.5% had a gestational age of ≥33 weeks. Respiratory distress syndrome (RDS) was detected in 249 (40.6%), bronchopulmonary dysplasia (BPD) in 124 (20.2%), necrotizing enterocolitis (NEC) in 41 (6.6%), retinopathy of prematurity (ROP) in 202 (32.9%), and intracranial hemorrhage (ICH) in 15 (2.4%). RDS, BPD, NEC, ROP, and ICH rates were inversely proportional to decreases in gestational age and birth weight, and were found to be statistically significant. CONCLUSION: Mortality and morbidity rates were similar to the other data published from our country, but the rates were above those reported in developed countries. We believe that our morbidity and mortality rates can reach levels comparable to those of developed countries with improved antenatal care, regular follow-up of pregnancy and increased numbers of physicians and health care personnel per patient.

Cow's Milk Allergy in Preterm Infant with Bronchopulmonary Dysplasia.

Cow's milk allergy is frequent in the first year of life. The symptoms may start during the first weeks of life, and may be cutaneous (50-60%), gastrointestinal (50-60%) or respiratory (20-30%), often involving more than one organ system. In this report, we describe a case of cow's milk allergy in a preterm infant in whom rectal bleeding and respiratory symptoms resolved with the introduction of an extensively hydrolyzed formula. Occurrence of the respiratory symptoms of this disorder in a preterm infant with bronchopulmonary dysplasia may cause re-hospitalization after discharge.

Intravenous paracetamol with a lower dose is also effective for the treatment of patent ductus arteriosus in pre-term infants.

INTRODUCTION: Haemodynamically significant patent ductus arteriosus is a significant cause of morbidity and mortality in pre-term infants. This retrospective study was conducted to investigate the usefulness of lower-dose paracetamol for the treatment of patent ductus arteriosus in pre-term infants. MATERIALS AND METHODS: A total of 13 pre-term infants who received intravenous paracetamol because of contrindications or side effects to oral ibuprofen were retrospectively enrolled. In the first patient, the dose regimen was 15 mg/kg/dose, every 6 hours. As the patient developed significant elevation in transaminase levels, the dose was decreased to 10 mg/kg/dose, every 8 hours in the following 12 patients. Echocardiographic examination was conducted daily. In case of closure, it was repeated after 2 days and when needed thereafter in terms of reopening. RESULTS: A total of 13 patients received intravenous paracetamol. Median gestational age was 29 weeks ranging from 24 to 31 weeks and birth weight was 950 g ranging from 470 to 1390 g. The median postnatal age at the first intravenous paracetamol dose was 3 days ranging from 2 to 9 days. In 10 of the 13 patients (76.9%), patent ductus arteriosus was closed at the median 2nd day of intravenous paracetamol ranging from 1 to 4 days. When the patient who developed hepatotoxicity was eliminated, the closure rate was found to be 83.3% (10/12). CONCLUSION: Intravenous paracetamol may be a useful treatment option for the treatment of patent ductus arteriosus in pre-term infants with contrindication to ibuprofen. In our experience, lower-dose paracetamol is effective in closing the patent ductus arteriosus in 83.3% of the cases.

Rare cause of absence of femoral arterial pulse: bilateral common iliac artery hypoplasia.

Congenital anomaly of the iliac arteries is rare, and, given that patients are generally asymptomatic, diagnosis in the newborn period is difficult. Herein is presented the case of a newborn with bilateral hypoplasia of the common iliac arteries, seen on multi-slice computed tomography while investigating the absence of femoral pulse. This anomaly is a rare cause of absence of femoral arterial pulse.

An unusual cause of fulminant Guillain-Barré syndrome: angel's trumpet.

A 5-year-old previously healthy boy presented with typical findings of Guillain-Barré syndrome and unilateral tonic pupil. He was placed on mechanical ventilation for 35 days for respiratory failure. Plasmapheresis and two courses of intravenous immunoglobulin therapy were given to the patient, and he experienced stepwise recovery from his illness. This case of acute motor axonal neuropathy type Guillain-Barré syndrome is novel in that the cause was established as ingestion of a toxic solanaceous plant, angel's trumpet (Brugmansia suaveolens; syn. Datura suaveolens). Understanding the signs and symptoms of angel's trumpet toxicity can allow for early diagnosis and proper case management.