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In the neonatal intensive care units (NICU), epicutaneo-caval catheters (ECCs) are common alternative vascular routes. Pericardial effusion (PCE) and cardiac tamponade (CT) are rare but serious complications in infants with ECCs. It may be asymptomatic or present with a variety of significant clinical signs, including dyspnea, bradycardia, sudden asystole, and hypotension. If untreated, PCE can be fatal. This report presents, three cases of ECC-associated PCE/CT during NICU stay. All three patients were born before 30 weeks of gestation and weighed less than 1500 g. Echocardiography was used for diagnosis all patients. PCE/CT was detected incidentally in one patient and after hemodynamic deterioration in the other two. In one patient, CT was developed due to catheter malposition, and the other two patient, the catheter tip was found in the right atrium. PCE did not recur in any of the patients after pericardial fluid was drained and the catheters were removed. No PCE/CT-related deaths were observed. In all three patients, X-ray was used to evaluate the location of the catheter tips. However, after clinical deterioration, echocardiography showed that in the first two cases the tips were actually in the right atrium. Real-time ultrasound was suggested with strong evidence to evaluate the location of the catheter tip and to detect secondary malapposition. PCE/CT should be considered in the presence of unexplained and refractory respiratory distress, abnormal heart rate and blood pressure, and metabolic acidosis in a neonate with ECC. Early diagnosis and prompt pericardiocentesis are essential to reduce mortality and improve prognosis. Prospective studies with educational interventions should be designed to demonstrate that the use of point-of-care ultrasound (POCUS) can be easily acquired and may reduce complications.

Deferred (also known as delayed) cord clamping can improve survival of infants born preterm (before 37 weeks of gestation), but the optimal duration of deferral remains unclear. We conducted a systematic review and individual participant data network meta-analysis with the aim of comparing the effectiveness of umbilical cord clamping strategies with different timings of clamping or with cord milking for preterm infants.

Umbilical cord clamping strategies at preterm birth have the potential to affect important health outcomes. The aim of this study was to compare the effectiveness of deferred cord clamping, umbilical cord milking, and immediate cord clamping in reducing neonatal mortality and morbidity at preterm birth.

Background: Nonimmune hydrops fetalis (NIHF) can be caused by different types of etiologies. Some rare intrathoracic lesions are associated with NIHF. Combination of mediastinal teratoma, NIHF, and chylothorax is extremely rare. Mediastinal teratomas which are located in midline should be difficult to be detected. Thoracic imaging should be performed with unknown etiology for hydrops, and in case of chylothorax, the presence of a mass compressing the ductus thoracicus should be considered primarily. Case presentation: An infant was born with a diagnosis of NIHF. Bilateral chest tubes were inserted cause of bilateral pleural effusions. After enteral feeding, the previously clear pleural fluid became chylous. Medium-chain triglyceride infant formula and somatostatin analog octreotide were initiated. A mass was appeared on her neck with the disappearance of skin edema. Magnetic resonance imaging confirmed a large, heterogeneous mass which was suggesting immature teratoma originating from thyroid gland. Complete surgical excision of the mass was performed. Histology confirmed high-grade immature teratoma. The neonate made an uneventful recovery. Following complete cessation of pleural fluid drainage, octreotide was stopped. She was discharged home on exclusive breast milk on day 34 of life. Conclusions: Mediastinal teratomas are rare masses that cause hydrops fetalis. Although the association of NIHF and mediastinal teratoma is rare, thoracic imaging can be performed if an etiology cannot be found despite basic evaluations for hydrops. In case of chylothorax, the presence of a mass compressing the ductus thoracicus should be considered primarily, and thoracic imaging should be performed.

AbstractBackground:Recessive mutations in ABCC8/KCNJ11of β-cell KATPchannel generally cause severe medically unresponsive hyperinsulinemic hypoglycemia (HH). Rarer dominant mutations in these genes have been described that mostly cause milder, medically responsive congenital hyperinsulinism. Rarer dominant mutations in these genes have been described that mostly cause milder, medically responsive congenital hyperinsulinism. To date the phenotype of patients with dominant mutations seems to be different from those with recessive mutations as the majority of patients are responsive to diazoxide therapy. Controversy exists on whether these dominant ABCC8 or KCNJ11genes mutations predispose to diabetes mellitus in adulthood or not.Subjects:We report the clinical and genetic characteristics of five patients with neonatal HH, three had recessively inherited KATPchannel mutations and two with a dominantly acting mutation. As a result of failure to medical therapy, patients with recessive KATPchannel mutations underwent a near total pancreatectomy. Two siblings with a novel dominant mutation showed good response to medical treatment. Although the HH remitted in early infancy, they became diabetic at the prepubertal age. Their mother, maternal aunt and maternal grandfather had the same mutation without any medical history of neonatal HH.Conclusion:The clinical presentation of our two patients with a dominant ABCC8 mutation was milder than that of patients with the resessive form of the disease as they responded well to medical management.

AbstractNeonatal diabetes mellitus (NDM) is a rare condition that can be either transient or permanent. KATPchannel (Kir6.2 or SUR1) mutation, chromosome 6 abnormalities, insulin, or glucokinase gene mutations can lead to isolated NDM. Cases caused by Kir6.2 mutation usually result in permanent NDM (PNDM) rather than transient NDM (TNDM). The majority of patients with the Kir6.2 or SUR1 mutation can be successfully managed with a sulfonylurea agent, without the need for insulin. We report a preterm male with NDM having two novel missense mutations, E322A and D352H, in the KCNJ11gene. At 2 months of age, successful transition from insulin to glibenclamide (glyburide) therapy of the patient was managed. At 5 months of age, his diabetes went in to remission.

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The ophthalmo-acromelic syndrome of Waardenburg is an autosomal recessive trait comprising eye malformations ranging from true anophthalmia to mild microphthalmia with acromelic malformations. Some 29 affected individuals have been reported since Waardenburg's first report in 1935 [Waardenburg et al., 1961]. We report on a new case with bilateral anophthalmia and typical limb malformations. The patient also was found to have interruption of the inferior vena cava with azygos continuation as an additional finding. The previous reports are reviewed to elucidate the spectrum of the syndrome. Am. J. Med. Genet. 90:150–154, 2000. © 2000 Wiley-Liss, Inc.

The rate of evaporation from the skin (g/m2/h) was measured in 12 full-term and 16 preterm infants (gestational age 25–34 wk) both during incubator care and when nursed under a radiant heater. The method for evaporation rate measurement is noninvasive and based on determination of the water vapor pressure gradient close to the skin surface. Measurements were first made with the infant nursed in an incubator with a controlled environment with respect to humidity, temperature, and air velocity. The measurements in the term infants were performed at an ambient relative humidity (RH) of 50, and in the preterm infants first at 50 and subsequently at 30–40. Evaporation rate was then measured with the infant nursed under a radiant heater. In term infants, mean evaporation rate was 3.3 g/m2/h during incubator care (RH 50) and 4.4 g/m2/h during care under the radiant heater. In preterm infants, the corresponding values were 15.5 g/m2/h in the incubator at RH 50, 16.7 g/m2/h at RH 30–40, and 17.9 g/m2/h under the radiant heater. It is concluded that the evaporative water loss from the skin depends on the ambient water vapor pressure, irrespective of whether the infant is nursed in an incubator or under a radiant heater. The higher rate of evaporation during care under a radiant heater is due to the lower ambient water vapor pressure and not to any direct effect of the nonionizing radiation on the skin.

The rate of evaporation from the skin (g/m2/h) was measured in 12 full-term and 16 preterm infants (gestational age 25–34 wk) both during incubator care and when nursed under a radiant heater. The method for evaporation rate measurement is noninvasive and based on determination of the water vapor pressure gradient close to the skin surface. Measurements were first made with the infant nursed in an incubator with a controlled environment with respect to humidity, temperature, and air velocity. The measurements in the term infants were performed at an ambient relative humidity (RH) of 50%, and in the preterm infants first at 50% and subsequently at 30–40%. Evaporation rate was then measured with the infant nursed under a radiant heater. In term infants, mean evaporation rate was 3.3 g/m2/h during incubator care (RH 50%) and 4.4 g/m2/h during care under the radiant heater. In preterm infants, the corresponding values were 15.5 g/m2/h in the incubator at RH 50%, 16.7 g/m2/h at RH 30–40%, and 17.9 g/m2/h under the radiant heater. It is concluded that the evaporative water loss from the skin depends on the ambient water vapor pressure, irrespective of whether the infant is nursed in an incubator or under a radiant heater. The higher rate of evaporation during care under a radiant heater is due to the lower ambient water vapor pressure and not to any direct effect of the nonionizing radiation on the skin.

Pregnant women with low B12levels are unable to provide the necessary amount of this vitamin to their fetuses. The mothers are usually not anemic, and failure to thrive and neurologic deficits are more common in their infants than in megaloblastic anemia. We report the case of a newborn that was born to a vegetarian mother and presented with the symptoms of megaloblastic anemia at birth; we also discuss vitamin B12metabolism during pregnancy and lactation. An interesting feature of the present case is that it is the only reported case of a newborn that was treated by supplementation of the mother.

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To investigate whether radiant heat increases insensible water loss, the evaporation rate from the skin (ER) was measured in term and preterm infants, both during incubator care (A and B) and when nursed under a radiant heater (C). Relative humidity in the incubator was 50 % (A) and for the preterm infants measurements were also made at a lower level of humidity (B).The ER was higher when ihe infants were nursed under the radiant heater than during care in an incubator with humidified air, but the increase in ER was not greater than expected at that lower level of humidity. Wc conclude that the water loss from the skin depends on ihe ambient vapour pressure, bolh when infants are nursed in incubators and under radiant heaters.