A rare cause of a relatively common neonatal emergency.

Malignant infantile osteopetrosis (MIOP) is a rare cause in the list of etiological factors of neonatal hypocalcemia in several textbooks. The most severe complication of MIOP is bone marrow suppression. The abnormal expansion of bone interferes with medullary haematopoiesis. Most children with this disease die within the first decade of life of secondary consequence of bone marrow failure. Hematopoietic stem cell transplantation (HSCT) is the only curative therapy for MIOP, an otherwise fatal disease. We present a neonate with MIOP that was further complicated with vitamin D deficiency.

Reference values of serum cystatin-C for full-term and preterm neonates in Istanbul.

OBJECTIVE: To determine the level and distribution of Cystatin C values in full-term and preterm healthy neonates for the purpose of diagnosis and follow-up of renal diseases of the neonates. METHODS: Eighty-eight newborn infants, including 55 preterm and 33 term born in the authors' hospital having no urinary tract pathology, symptoms or signs during prenatal and postnatal follow-up, were studied . RESULTS: There were 25 neonates born between gestational wk of 28 and 32 (Group 1), 30 neonates born between gestational wk of 33-36 (Group 2) and 33 neonates born after gestational wk of 37 (Group 3). Average cystatin C values were determined to be 1.41 mg/l, 1.22 mg/l and 1.21 mg/l for Group 1, Group 2 and Group 3, respectively. CONCLUSIONS: Evaluation of cystatin C can be effective for follow-up of renal pathologies, because it is not affected by gender, body weight and muscle mass and has a constant production rate.

The epidemiology and economic impact of varicella-related hospitalizations in Turkey from 2008 to 2010: a nationwide survey during the pre-vaccine era (VARICOMP study).

Varicella can cause complications that are potentially serious and require hospitalization. Our current understanding of the causes and incidence of varicella-related hospitalization in Turkey is limited and sufficiently accurate epidemiological and economical information is lacking. The aim of this study was to estimate the annual incidence of varicella-related hospitalizations, describe the complications, and estimate the annual mortality and cost of varicella in children. VARICOMP is a multi-center study that was performed to provide epidemiological and economic data on hospitalization for varicella in children between 0 and 15 years of age from October 2008 to September 2010 in Turkey. According to medical records from 27 health care centers in 14 cities (representing 49.3% of the childhood population in Turkey), 824 children (73% previously healthy) were hospitalized for varicella over the 2-year period. Most cases occurred in the spring and early summer months. Most cases were in children under 5 years of age, and 29.5% were in children under 1 year of age. The estimated incidence of varicella-related hospitalization was 5.29-6.89 per 100,000 in all children between 0-15 years of age in Turkey, 21.7 to 28 per 100,000 children under 1 year of age, 9.8-13.8 per 100,000 children under 5 years of age, 3.96-6.52 per 100,000 children between 5 and 10 years of age and 0.42 to 0.71 per 100,000 children between 10 and 15 years of age. Among the 824 children, 212 (25.7%) were hospitalized because of primary varicella infection. The most common complications in children were secondary bacterial infection (23%), neurological (19.1%), and respiratory (17.5%) complications. Secondary bacterial infections (p < 0.001) and neurological complications (p < 0.001) were significantly more common in previously healthy children, whereas hematological complications (p < 0.001) were more commonly observed in children with underlying conditions. The median length of the hospital stay was 6 days, and it was longer in children with underlying conditions (<0.001). The median cost of hospitalization per patient was $338 and was significantly higher in children with underlying conditions (p < 0.001). The estimated direct annual cost (not including the loss of parental work time and school absence) of varicella-related hospitalization in children under the age of 15 years in Turkey was $856,190 to $1,407,006. According to our estimates, 882 to 1,450 children are hospitalized for varicella each year, reflecting a population-wide occurrence of 466-768 varicella cases per 100,000 children. In conclusion, this study confirms that varicella-related hospitalizations are not uncommon in children, and two thirds of these children are otherwise healthy. The annual cost of hospitalization for varicella reflects only a small part of the overall cost of this disease, as only a very few cases require hospital admission. The incidence of this disease was higher in children <1 year of age, and there are no prevention strategies for these children other than population-wide vaccination. Universal vaccination is therefore the only realistic option for the prevention of severe complications and deaths. The surveillance of varicella-associated complications is essential for monitoring of the impact of varicella immunization.

Effect of Fluid Supplementation on Serum Bilirubin Level During Phototherapy of Exclusively Breastfed Term Infants with Hyperbilirubinemia.

Abstract Background: This study compared the rates of decrease in serum bilirubin levels in severely jaundiced healthy term infants given oral or intravenous fluid supplementation during phototherapy. Methods: A randomized controlled study was carried out in the neonatal intensive care unit of Zeynep Kamil Maternity and Children Hospital (Istanbul, Turkey) over a 4-month period. Two hundred fifty healthy term infants with hyperbilirubinemia were randomized to receive either solely breastmilk (n=125) or both breastmilk and intravenous fluid (n=125) during phototherapy. Results: There were no significant differences (p>0.05) in the mean birth weight, mean gestastional age, modes of delivery, mean time of admission age, mean serum osmolality, and hematocrit and reticulocyte count between the two groups. Similarly, there was no significant difference (p>0.05) in the mean indirect serum bilirubin level at the time of admission to the neonatal intensive care unit and at 4, 8, 12, 24, and 48 hours after commencement of phototherapy between the two groups. There was no significant difference (p>0.05) in the mean duration of phototherapy or in the median duration of hospitalization between the two groups. Conclusions: Based on our results, intravenous fluid support has no effect on the rate of decrease in serum bilirubin and decrease in duration of phototherapy in healthy term newborns with no dehydratation. However, using the oral route avoided the need for intravenous cannulae and their attendant complications. Insensible fluid loss is increased during phototherapy, so protection of hydration status with oral feeding is important for newborns.

Correlation between transient tachypnea of the newborn and wheezing attack.

BACKGROUND: Transient tachypnea of the newborn (TTN) is the most common cause of respiratory distress in newborns. Although associated with some morbidity, it is generally believed that once TTN resolves, there is no further increased risk for respiratory disease. However, in limited studies frequency of wheezing attacks is found to be increased in patients who had TTN diagnosis during the newborn period, in comparison to patients who had no respiratory problem. Thus, the question arises as to whether TTN is an innocent disease. METHODS: This study was done retrospectively. We recorded the demographic characteristics of 103 infants born between 17 October 2003 and 17 October 2004 at Zeynep Kamil Hospital and hospitalized because of TTN in the neonatal intensive care unit. In the second phase, we telephoned the parents of the 103 infants and asked about wheezing attacks. A total of 103 other infants, born during the same period, with no health problems during the newborn period, were included in the study as the control group and the same procedures were applied to them. RESULTS: The rate of wheezing attack among patients with TTN diagnosis was found to be significantly higher than that in patients who had no TTN diagnosis (P < 0.01). TTN was found to be an independent risk factor for wheezing attack (OR, 2.378; 95% CI, 1.20-4.70). CONCLUSION: In conclusion, we established that TTN is an independent risk factor for wheezing. In addition we also hypothesized that genetic and environmental interactions synergistically predisposed these children for future wheezing.

Carnitine levels in neonatal hypoxia.

BACKGROUND: The aim of this study is to determine carnitine levels in hypoxic-ischemiac neonates. METHODS: Total and free carnitine levels were measured in 20 normal term neonates and 20 term neonates who were diagnosed as hypoxia-ischemia. RESULTS: Both total carnitine levels (43.7 +/- 10.0 microg/dl vice 27.8 +/- 11.8 microg/dl, p<0.0001) and free carnitine levels (28.0 +/- 8.8 vice 13.2 +/- 6.8 microg/dl, p<0.0001) were lower in the hypoxic-ischemic group). Additionally acyl/total carnitine ratio was higher in the asphyxic group (0.33 +/- 0.20 vice 0.56 +/- 0.24, p<0.0001). CONCLUSIONS: The findings of this study show that neonates with hypoxia-ischemia suffer carnitine deficiency.