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1/19. Prolonged hyperinsulinemic hypoglycemia in a small for date preterm.

    hyperinsulinism is an important cause of hypoglycemia in early infancy. Many forms of hyperinsulinemic hypoglycemia are described: transient, prolonged, persistent. Transient forms are well recognized in infants of diabetic mother; prolonged forms are responsible for the hypoglycemia in small-for-date (SGA) infants and asphyxiated newborns. Persistent hyperinsulinemic hypoglycemia occurs in a group of congenital disorders associated with an abnormality of beta-cell regulation throughout the pancreas. Accurate diagnosis and treatment are essential in all various forms of hyperinsulinism also because newborns are at high risk of permanent brain damage. We report a case of prolonged hyperinsulinemic hypoglycemia in a SGA preterm, immediately treated with a high dose of glucose and glucocorticoid and then with diazoxide. hypoglycemia was continued until 2 months of age when it resolved spontaneously and completely.
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2/19. Extensive hepatic necrosis in a premature infant.

    A fatal case of fulminant hepatic failure that occurred in the neonatal period is reported in a premature infant born after 27 4/7-weeks' gestation. Immediately after birth the infant had severe hypoxia and hypotension resulting from birth asphyxia, hypovolemic shock, and septicemia. At autopsy, histological appearance of the liver showed virtually total hepatocellular necrosis without features of fibrosis. Although the exact cause of hepatocellular injury cannot be fully ascertained, it is assumed that hypoxia and hypotension must have been the predominant factors leading to massive hepatic necrosis.
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3/19. Symmetrical thalamic lesions in infants.

    Clinical observations and findings on imaging are reported in six newborns with symmetrical thalamic lesions (STL). In three cases the diagnosis was confirmed by postmortem examination. Characteristic observations in this series and 17 previously reported cases include no evidence of perinatal asphyxia, high incidence of polyhydramnios, absent suck and swallow, absent primitive reflexes, appreciable spasticity at or within days of birth, lack of psychomotor development, and death within days or months. Characteristic pathological findings include loss of neurons, astrogliosis, and 'incrusted' neurons particularly in the thalamus. In two thirds of cases the basal ganglia and brain stem are involved as well. A hypoxic-ischaemic event occurring two to four weeks before birth is most likely responsible for STL. Bilateral thalamic calcification can often, but not always, be demonstrated in the newborn period by computed tomography and/or cranial ultrasound. The presence of these calcifications and the observation of spasticity at birth imply that the responsible insult occurred at least two to four weeks earlier. The small number of published cases with STL suggest that it may be easily missed.
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4/19. pregnancy in renal transplant recipients: case report.

    pregnancy in a renal homograft recipient resulted in an emergency cesarean section at 32 weeks' gestation secondary to maternal small bowel obstruction with necrotic bowel and subsequent intestinal perforation. The 1814 gm female product of this pregnancy had a moderate degree of in utero asphyxia, which subsequently, based on radiologic studies, had to be differentiated from the possibility of a congenital viral infection. Several concerns arose regarding the effects on the fetus by maternal immunosuppressive therapy, the risk factors faced by both the fetus and the mother secondary to the mother's renal disease, and the general outcome of the increasing number of pregnancies in this population.
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5/19. Unilateral thalamic haemorrhage in the pre-term and full-term newborn.

    One full-term and three premature newborns with a unilateral thalamic haemorrhage are reported. The lesion was diagnosed using cranial ultrasonography and confirmed in three using magnetic resonance imaging. Severe perinatal asphyxia occurred in all infants. The neonatal clinical history, subsequent neuro-developmental outcome and neuroimaging findings are discussed. As the prognosis appears to be much better than for infants with bilateral thalamic densities, it is important that this type of lesion is recognised as a separate entity.
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6/19. Unexpected survival after conservative management of cord prolapse in two very preterm babies.

    Two very preterm infants (24 and 25 weeks) were born vaginally in spite of prolapse of the umbilical cord. The prolapses lasted 45 min and 2 h, respectively, but though both babies were at first believed to be lost, they survived, only mild to moderate asphyxia, and both were discharged well. One of the babies later developed slight neurological sequelae. Even a very preterm baby has the possibility of surviving vaginal delivery complicated by cord prolapse.
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7/19. Post-asphyxial encephalopathy in a preterm infant.

    An infant born at 31 weeks gestation had abnormalities consistent with post-asphyxial encephalopathy, including decelerated fetal heart-rate, cord-blood acidosis and depressed Apgar scores. Clinical signs included respiratory depression, hypotonia and severe seizures. When seen at six months corrected age, however, he had no abnormal neurological signs. The authors conclude that asphyxia in preterm infants may result in clinical abnormalities similar to those in mature infants, but that the abnormalities are separate from haemorrhage and ischaemia.
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keywords = asphyxia
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8/19. Fatal infantile muscle phosphorylase deficiency.

    A premature female infant born of a consanguineous union exhibited joint contractures and signs and symptoms of perinatal asphyxia. A muscle biopsy examined by light microscopic, histochemical, and electron microscopic techniques exhibited changes of muscle phosphorylase deficiency and glycogenosis, identical to those of McArdle's disease. Postmortem ultrastructural examination of liver and heart did not reveal lysosomal storage. This case and one previously reported example of fatal infantile phosphorylase deficiency suggest that the clinical spectrum of McArdle's disease may be broader than previously recognized.
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9/19. Congenital myotonic dystrophy: respiratory function at birth determines survival.

    The clinical features of 14 neonates with congenital myotonic dystrophy were retrospectively reviewed. These babies represent all the new cases of congenital myotonic dystrophy seen in this department since 1982. Twelve babies were referred because of either difficulties in diagnosis or difficulties in the management of their respiratory problems. Of the 14 babies, 13 had birth asphyxia, 11 were premature, and four had intrauterine growth retardation. Ten babies required artificial ventilation from birth. Abnormalities on chest radiography included thin ribs (n =9) and raised right hemidiaphragms (n = 5). Recurrent episodes of collapse and consolidation of the lungs secondary to poor swallowing occurred in all ventilated babies. All babies ventilated for longer than four weeks died of respiratory complications before the age of 15 months. One baby was successfully extubated after diaphragmatic plication, but he died a few months later. Duration of ventilation was the best guide to prognosis.
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10/19. Hypoxic ischaemic encephalopathy: correlation between ultrasound and computed tomography.

    Hypoxic ischaemic encephalopathy (HIE) is a major cause of motor and mental retardation. There ist no doubt that the haemorrhagic form of HIE can be detected by cerebral sonography, but it is of great interest to recognize non-haemorrhagic HIE as well. We demonstrate the follow-up of three patients with different types of non-haemorrhagic HIE: Periventricular leukomalacia in a critical ill premature infant 35 weeks gestational age, multicystic encephalopathy in a term newborn with severe perinatal asphyxia and circumscript ischaemic leukomalacia in a five month old infant with near miss event for sudden infant death. Correlation between ultrasound and computed tomography proved that non-haemorrhagic HIE produces global or circumscript high echogenicity in the first week after the hypoxic event whereas computed tomography shows pathologic hypodensity in the same areas. Cerebral sonography is a very helpful and harmless method for the difficult diagnosis HIE. In the case of periventricular leukomalacia, CT scan gives no further information and can be avoided. Concerning HIE of term newborns and small infants, CT scan remains necessary, to evaluate the extension of cerebral injury.
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