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11/215. Unilateral pulmonary interstitial emphysema following pneumonia in a preterm infant successfully treated with prolonged selective bronchial intubation.

    We describe a ventilated preterm infant (26 week's gestation) who developed severe right-sided pulmonary interstitial emphysema following staphylococcus aureus pneumonia. Prolonged selective bronchial intubation (10 days) resulted in a marked clinical improvement and resolution of the emphysema. Resolution of unilateral pulmonary interstitial emphysema may require a longer course of selective bronchial intubation than currently recommended.
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12/215. Fatal rupture of a sacrococcygeal teratoma during delivery.

    We report the case of premature infant born at 32 weeks' gestation with a sacrococcygeal teratoma diagnosed in utero. During delivery by cesarean section, profound bleeding due to rupture of the teratoma occurred. Despite volume expansion with saline, albumin, and whole blood, a satisfactory peripheral perfusion of the infant was only briefly achieved. Surgical intervention to stop the bleeding was unsuccessful. resuscitation of the infant was discontinued after 55 minutes. The relevant literature is discussed, and suggestions for the management of infants with sacrococcygeal teratomas are made.
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13/215. Acquired ileal atresia and spontaneous reconstitution of intestinal continuity in a premature infant with necrotizing enterocolitis.

    An 849-g (26-week gestation) premature infant had pneumoperitoneum on the 20th day of life after having normal stools and accepting partial enteric alimentation. Percutaneous penrose drainage had to be performed on 2 consecutive days at 2 different sites (right lower quadrant, left lower quadrant), at which time she stabilized. Eleven days later, she started to pass stool, and oral feeding was begun (1 to 2 mL every 4 hours). Enteral intake could not be advanced because of repeated bouts of abdominal distension, despite having regular bowel motions. Gastrointestinal contrast radiographic investigation suggested a stricture of the ileum. At laparotomy (at age 2 months) ileal atresia with a "V"-shaped defect in the mesentery was found. Surprisingly, intestinal continuity was established via an ileoileal fistula. After resection and anastomosis, she recovered fully. Mesenteric and enteric vascular ischemia (necrotizing enterocolitis) produced acquired ileal atresia-a rare occurrence. More rare is the reestablishment of intestinal continuity by fistulization.
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14/215. stenotrophomonas maltophilia pneumonia in a premature infant.

    Stenotrophomonas (xanthomonas) maltophilia is an aerobic, non-fermentative, gram-negative bacillus that is generally considered an opportunistic pathogen. Infections due to S. maltophilia have become increasingly important in the hospital environment. patients compromised by debilitating illnesses, surgical procedures or indwelling vascular catheters are most prone to S. maltophilia infections. To our knowledge, we report the first case of S. maltophilia pneumonia in a premature infant of 31 weeks gestational age. Although the therapy of choice for severe infections caused by S. maltophilia remains to be decided, this patient was successfully treated by amikacin.
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15/215. Thrombosis of the deep cerebral veins with excessive bilateral infarction in a premature infant with the thrombogenic 4G/4G genotype of the plasminogen activator inhibitor-1.

    We report on a preterm infant with deep cerebral venous thrombosis, a rare condition in this age group. This premature infant had a gestational age of 33 weeks and normal development until day 18, when he presented with tonic seizures and a tense fontanelle. Ultrasound and computed tomography revealed bilateral haemorrhagic infarction of the whole region drained by the deep cerebral veins, including the periventricular white matter, thalamus and choroid plexus. The child was homozygous for the 4G allele of the plasminogen activator inhibitor-1 (PAI-1) 4G/5G promoter polymorphism. CONCLUSION: In patients with bilateral cerebral infarction, thrombosis of the deep cerebral veins should be considered. In addition the role of prothrombotic risk factors, including PAI-1 4G/5G promoter polymorphism, in cerebral vein thrombosis should be clarified in a multicentre study.
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16/215. "What can I do to enhance the development of a premature infant with chronic lung disease?".

    CASE. Timmy was born at 32 weeks of gestation after an uncomplicated pregnancy until there was a spontaneous rupture of the membranes and preterm labor associated with chorioamnionitis. A 2-month hospitalization in the neonatal intensive care unit (NICU) was associated with pneumonia, a Grade II intraventricular hemorrhage, chronic lung disease, and a slow weight gain in the nursery. He was discharged to home with plans for ongoing care by his pediatrician. The primary care pediatrician attended a multidisciplinary conference with the NICU staff and Timmy's parents. At the time of discharge from the nursery, at 38 weeks postconceptual age, Timmy still required oral diuretics and supplemental oxygen, as well as other medications such as iron. Timmy's respiratory rates were between 40 and 60 breaths per minute at rest, with mild intercostal retractions. He was discharged with a cardiorespiratory monitor. The discharge examination revealed mild to moderate symmetrical hypotonia with intact deep tendon reflexes, shoulder girdle weakness, and a mild head lag. Timmy would regard a human face and a bright object and would follow them briefly. He became active and would thrash his extremities with minimal tactile, bright light, or auditory stimulation. Typically, he settled slowly with swaddling and a pacifier. nursing was slow to develop; he was currently receiving one half of his calories at the breast and the remainder of his calories from bottle-feeding of fortified expressed breast milk. As she prepared for the first office visit with Timmy and his parents, the pediatrician asked herself, "What can I do to enhance the developmental outcome for this child?"
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keywords = gestation, pregnancy
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17/215. Fetal-onset severe skeletal muscle glycogenosis associated with phosphorylase-b kinase deficiency.

    We report on a premature newborn girl delivered after 32 weeks of gestation by cesarean section after sparse limb movements, fetal tachycardia and late heart rate decelerations had suggested fetal distress. Following 1 day of mechanical ventilation, adequate pulmonary gas exchange was achieved by spontaneous breathing. Main symptoms were virtually complete absence of spontaneous movements, increased flexor tonus of the extremities, and hypotonia of the trunk. Inability to suck or swallow required nasogastric gavage feeding. There were no hypoglycemic episodes. echocardiography revealed normal myocardial function. creatine kinase was 237 U/I at 2 days of life, declining to normal values thereafter. Muscle biopsy revealed increased glycogen storage with subsarcolemmal glycogen deposits and low phosphorylase-a activity while total phosphorylase was normal after in vitro activation, suggestive of phosphorylase-b kinase deficiency. No mutation was detected in exon 1 of the myophosphorylase gene. No psychomotor development was observed, and the infant died of central apnea at 3 months of age.
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18/215. Transthoracic pacing in a very low birth weight infant with congenital complete atrioventricular block.

    We report our experience of pacemaker treatment in a premature infant of 830 g with congenital complete atrioventricular block due to maternal sjogren's syndrome. The infant was delivered by cesarean section at an estimated gestational age of 26 weeks because of fetal bradycardia, decreasing fetal movements, and hydrops. Immediate postnatal transesophageal ventricular pacing was not successful, whereas transthoracic pacing with self-adhesive patch electrodes adapted to body size resulted in an effective increase of the infant's heart rate until operative application of temporary epimyocardial pacing wires allowed external stimulation of the heart.
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19/215. Late-diastolic forward flow in the aorta induced by left atrial contraction.

    Late-diastolic forward flow is a well-described phenomenon detectable by Doppler echocardiography in the pulmonary trunk. It is supported by a restrictive right ventricular diastolic function and by a low end-diastolic pulmonary artery pressure. A similar phenomenon for the left ventricle and the aorta has not been described. We report a case of a preterm infant with aortic stenosis and endocardial fibroelastosis, who underwent balloon valvuloplasty. Restrictive left ventricular diastolic filling led to high left atrial pressure (27 mm Hg) and a very pathologic ratio of early-to-late peak velocities (2.6) for an infant of 29 weeks' gestation. In combination with a low diastolic aortic pressure (24 mm Hg) caused by moderate aortic regurgitation after intervention, a late-diastolic forward flow was detectable in the aorta during left atrial contraction with pulsed Doppler echocardiography.
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20/215. Early fatal pontocerebellar hypoplasia in premature twin sisters.

    We report clinical, neuroradiological and neuropathological findings of monozygotic twin sisters born at 30 weeks' gestation, with pontocerebellar hypoplasia (PCH) similar but not identical to type 2 PCH. They presented with hypertonia, jitteriness, spontaneous and provoked myoclonic jerks (hyperekplexia), apnoeic episodes, and progressive microcephaly. They died at 7 weeks of age from respiratory failure.
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