Outcomes of very low birth weight infants in a newborn tertiary center in Turkey, 1997-2000

Our purpose was to determine mortality and morbidity rates and selected outcome variables for infants weighing less than 1500 g, who were admitted to the neonatal intensive care unit of our hospital from 1997 to 2000. The ultimate goal of the study was to define a model for developing a regional database. Information on all very low birth weight (VLBW) admissions to a tertiary level neonatal intensive care unit (NICU) in Ankara between January 1997 and December 2000 was prospectively collected by three neonatologists using a standard manual of operation and definitions. The data consisted of patient information including sociodemographic characteristics; antenatal history; mode of delivery; APGAR scores; need for resuscitation; admission illness severity (Clinical Risk Index for Babies-CRIB) and therapeutic intensity (Neonatal Therapeutic Intensity Scoring System-NTISS); selected NICU parameters and procedures such as respiratory support, surfactant therapy, and postnatal corticosteroid therapy; and selected patient outcomes such as intraventricular hemorrhage, septicemia, necrotizing enterecolitis, retinopathy of prematurity, and chronic lung disease. The number of VLBW admissions to the NICU was 133, with 51 (28.6%) referrals from other maternity centers. The mean birth weight and gestational age of the infants were 1175 +/- 252 g and 30.3 +/- 2.9 weeks, respectively. One hundred and seventeen of 133 cases (88.7%) received at least one antenatal care visit. The median CRIB and NTISS scores were 4.5 and 31, respectively. Antenatal steroids had been given to 74 (55.6%) infants. Surfactant treatment and respiratory support were given to 33 (24.8%) and 73 (54.8%) infants, respectively. Among selected outcomes, chronic lung disease (CLD), threshold retinopathy of prematurity (ROP), severe intraventricular hemorrhage (IVH > or = grade III), nosocomial infection and necrotizing enterocolitis (NEC) were encountered in 14 (12.6%), 9 (8.1%), 3 (2.2%), 34 (25.5%) and 35 (26.3%) of the infants, respectively. Overall survival rate was 83.5% (111/133); most of the deceased cases were under 750 g (12/22). It was prospectively shown that 111 (100%) of the surviving infants could be regularly followed in a newborn follow-up clinic to provide health maintenance, developmental assessment and support. Compared with reports from other developing countries, VLBW infants at our center had higher survival rates. Compared to developed countries, survival rate was lower, especially for extremely very low birth weight infants. There is interaction between birth weight and survival rate. Among selected neonatal outcomes, chronic lung disease, threshold retinopathy, severe intraventricular hemorrhage (IVH > or = grade III) and nosocomial infection rates at this center were comparable with some reports from developed nations

The incidence and follow-up of isolated ventricular septal defect in newborns by echocardiographic screening

The aim of this study was to evaluate the incidence and spontaneous closure rate of ventricular septal defects in a randomly selected newborn population, using color flow Doppler echocardiographic screening. Color flow Doppler echocardiographic screening was performed in 1075 neonates within the first 72 hours of life. We also followed-up patients with ventricular septal defect for a year to detect spontaneous closure rate and its timing. The incidences of ventricular septal defect in all neonates, preterm neonates and term neonates were found as 47.4/1,000, 56/1,000 and 46.3/1,000 live births, respectively. All patients with ventricular septal defect except one were asymptomatic. Forty-six cases had a trabecular and five cases had perimembranous ventricular septal defect. With the exception of one case, all had a small ventricular septal defect (0.05). The incidence of ventricular septal defect was considerably high in neonates when routine color flow Doppler echocardiographic examination was performed. Despite increased incidence of ventricular septal defect, spontaneous closure rate was remarkably high within the first year of life. These defects may result from delayed physiologic development and have fairly good prognosis

Hypocalcemic seizure due to congenital rickets in the first day of life

Congenital rickets is considered a rare disease entity in the newborn period. Hypocalcemic seizure due to congenital rickets has been reported, but this symptom generally appears at the earliest at the end of the first month of life. A congenital rickets case presented unusually with seizure on the first day of life is reported, and the continuing occurrence of the disease with serious consequences even at the beginning of life is emphasized. Despite improved prenatal care offered today, congenital rickets still occurs. It seems that vitamin D deficiency in the neonate secondary to maternal deficiency should be a consideration for the pediatrician or neonatologist caring for newborns. Physicians should be aware of risk factors that can trigger the development of this condition and should be alert to the signs of congenital rickets in order to commence appropriate treatment and prevent complications such as seizure occurring as early as shortly after birth

Early or later prophylactic INSURE in preterm infants of less than 30 weeks' gestation

We aimed to determine whether an early prophylactic INSURE strategy combined with early nasal continuous positive airway pressure (nCPAP) treatment could decrease the subsequent need for mechanical ventilation (MV) compared to the administration of surfactant prophylaxis later, at the 15th minute after birth, combined with early nCPAP. Infants born a

Generalized periosteal reaction and tissue swelling secondary to prolonged prostaglandin E1 infusion and venous stasis: a case report

Prostaglandin E1 (PGE1) is the drug of choice for providing ductal patency in cyanotic congenital heart disease (CCHD) for a short period of time until essential surgical management. Occasionally, prolonged use of PGE1 is required when the surgical procedure is delayed due to certain clinical conditions. Prolonged use of PGE1 may lead to bone and tissue changes such as pretibial and soft tissue swelling of the extremities and reversible cortical proliferation of the bones. Venous stasis as an additional risk factor can result in generalized periosteal reaction that initially can cause the separation of the periosteum from the cortex. We report an infant with CCHD who developed severe tissue swelling and generalized periosteal reaction due to coexistence of prolonged use of PGE1 and venous stasis. To the best of our knowledge, this is the first case report with both of these risk factors

Renal tubular dysgenesis-a case presentation

Renal tubular dysgenesis (RTD) is a lethal, developmental anomaly of the fetal kidney characterized by a defect in differentiation of the proximal and distal convoluted tubules. It is usually associated with oligohydramnios in later pregnancy and Potter's syndrome. A neonate with typical features who presented with mild respiratory distress, dysmorphic appearance and anuria is described. At the age of seven days, peritoneal dialysis was started and was continued until the death of the baby at the age of three months. The diagnosis was made on the bases of clinical and ultrasonographic findings confirmed by renal biopsy. A review of the literature showed that this is the first case of RTD reported in Turkey

A novel mutation in ITGB4 gene in a newborn with epidermolysis bullosa, pyloric atresia, and aplasia cutis congenita

Background: Epidermolysis bullosa with pyloric atresia (EB-PA), also known as Carmi syndrome, is an uncommon, autosomal recessive genodermatosis that typically affects the skin and gastrointestinal tract. EB-PA is caused by homozygous or compound heterozygous mutations in the integrin alpha 6 (ITGA6) gene on chromosome 2q31.1 or in the integrin beta 4 (ITGB4) gene on 17q25.1. Case presentation: A male premature infant was born with aplasia cutis, atresia of the pylorus, and bilateral hydronephrosis. His clinical and imaging findings were compatible with EB-PA. A novel, small deletion of the last two bases in exon 6 and the first two nucleotides of intron 6 (c.565_566+2del) in ITGB4 gene was identified. Conclusion: EB-PA-aplasia cutis congenita is known to be a non-treatable condition with a poor prognosis as the reported case. The novel mutation reported in this patient may lead to the lethal form of this disease. Identification of underlying genetic abnormality is critical to give genetic counseling.</p