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1/74. incidence and outcome of a 10-fold indomethacin overdose in premature infants.

    We reviewed the incidence and morbidity of a 10-fold medication error among all premature infants treated with indomethacin. We detected 4 incidents among 1059 indomethacin doses given to infants weighing less than 1000 g. None of the infants had intracranial hemorrhage, necrotizing enterocolitis, or significant deterioration of renal function. ( info)

2/74. survival of a 30-week baby with congenital myotonic dystrophy initially ventilated for 55 days.

    In previous reports, duration of initial ventilation exceeding 1 month almost always predicts non-survival of babies with congenital myotonic dystrophy. However, a baby with this condition survived beyond infancy after 55 days' ventilation. We describe this case in detail, explain why the baby survived and highlight the importance of individualized assessment, in addition to applying general prognostic terms described in the literature. ( info)

3/74. Medullary ray nodule of the kidney.

    In this short report we describe three preterm infants who died within the first 8 months of life (corrected age). All presented similar histological features within the kidney, showing medullary ray nodules composed of tubules lined by low columnar epithelium with clear cytoplasm. The immunohistochemical profile of these lesions was identical and suggested origin from distal convoluted tubule or collecting duct. The aim of this report is to draw attention to this entity and to suggest a possible origin of these lesions. ( info)

4/74. "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?" ( info)

5/74. Dependence on a respiratory ventilator due to an atrial septal defect.

    An atrial septal defect is commonly thought of as a benign cardiac lesion especially in infancy. The haemodynamic consequences for the lungs, nonetheless, can be comparable to that produced by patency of the arterial duct. In a preterm boy, this lesion led to the development of broncho-pulmonary dysplasia, and dependence on a respiratory ventilator. He could be extubated shortly after surgical closure of the septal defect. ( info)

6/74. Neonatal leukemoid reaction and early development of bronchopulmonary dysplasia in a very low-birth-weight infant.

    The factors controlling the recruitment of inflammatory cells and the activation of the cytokine cascade in low-birth-weight premature infants have been implicated in the sequence of multiorgan inflammatory diseases, including the chronic lung disease of prematurity, bronchopulmonary dysplasia. This article describes a 982-gram, 25 ( 2 days) weeks' gestation male infant, who had a leukemoid reaction throughout the first week of life, followed by early development of bronchopulmonary dysplasia. ( info)

7/74. cystic fibrosis in three children with bronchopulmonary dysplasia.

    cystic fibrosis (CF) and bronchopulmonary dysplasia (BPD) are two common causes of chronic lung disease in children. patients with BPD or CF often have recurrent respiratory symptoms, failure to thrive, and/or metabolic alkalosis during infancy and childhood. Thus, recognizing the diagnosis of CF in an infant with BPD can be difficult. We present three infants with both BPD and CF. The infants shared a history of respiratory distress and prolonged oxygen requirements. All three also had difficulty gaining weight, even after pancreatic enzyme supplementation was instituted. Metabolic alkalosis was observed in two infants. Previous studies in children with CF suggest that early diagnosis may impact both lung health and nutritional status. A high index of suspicion is necessary for clinicians to identify these children early and intervene with appropriate therapy. ( info)

8/74. Aortopulmonary collateral arteries in a child with trisomy 21.

    We describe an infant born prematurely at 30 weeks gestation with Down's syndrome who became dependent on oxygen at 3 weeks of age after an uneventful initial neonatal period. There had been no evidence of bronchopulmonary dysplasia. An isolated aortopulmonary collateral artery of moderate size was mistakenly diagnosed as persistent patency of the arterial duct on echocardiography, and subsequently successfully occluded using two coils with an excellent clinical result. To the best of our knowledge, this is the first description of a congenital aortopulmonary collateral artery in a symptomatic infant with Down's syndrome and no evidence of bronchopulmonary dysplasia. We discuss the possible etiologies of these collateral arteries. It is important to include aortopulmonary collateral arteries in the differential echocardiographic diagnosis of an arterial duct. ( info)

9/74. The use of inhaled glucocorticosteroids and recovery from adrenal suppression after systemic steroid use in a VLBW premature infant with BPD: case report and literature discussion.

    Despite development of many prevention and treatment modalities for bronchopulmonary dysplasia (BPD), a form of chronic respiratory insufficiency in premature infants recovering from respiratory distress syndrome, BPD remains a treatment challenge and a significant cause of long-term morbidity. A ventilator-dependent very low birth weight infant in our newborn special care unit was receiving multiple courses of systemic dexamethasone for severe respiratory failure. The infant demonstrated adrenal suppression manifested by a baseline cortisol concentration below reported levels in infants of similar birth weight and postnatal age. We hypothesized that he had developed adrenal insufficiency as a result of the prolonged systemic steroid administration used to treat his respiratory problems. We further hypothesized that inhaled beclomethasone therapy would aid in the infant's recovery phase during relative adrenal insufficiency--and so substituted inhaled for systemic steroids. Inhaled corticosteroid treatment improved the clinical respiratory course and postnatal growth of this premature infant with BPD without inhibiting his recovery from adrenal insufficiency. ( info)

10/74. sandhoff disease (GM2 gangliosidoses) in a premature patient with bronchopulmonary dysplasia.

    We report a female premature infant with bronchopulmonary dysplasia and Sandhoff disease. The clue for diagnosis was the fundoscopy examination. We discuss this rare disease with unusual presentation of intrauterine growth retardation, premature delivery, and bronchopulmonary dysplasia. ( info)
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