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1/12. Persistent pulmonary interstitial emphysema in an unventilated neonate.

    Persistent pulmonary interstitial emphysema (PPIE) is a chronic form of pulmonary interstitial emphysema. The disease is histologically distinguished by large cysts and giant cells. Our patient was a female twin who was born at 31 weeks of gestation with a birth weight of 1,450 g. A chest X-ray at 2 hr after delivery was normal. At 12 hr, respiratory distress developed, and nasal continuous positive airway pressure (CPAP) was initiated. A chest film revealed left-sided pneumothorax. A chest tube was inserted, and the baby continued on nasal CPAP for 5 days. Her chest X-ray on postnatal day 4 showed diffuse cystic changes in the left lung. Thoracic computed tomography revealed multiple thick-walled cysts, the largest measuring 3 cm in diameter. Our case confirms that localized PIE may occur in preterm infants who have been treated with nasal CPAP only. Since this method is being used increasingly to avoid mechanical ventilation and in the postextubation period, it is very important that clinicians be aware of its complications.
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2/12. Management of giant omphalocele in a premature low-birth-weight neonate utilizing a bedside sequential clamping technique without prosthesis.

    Management of giant omphalocele in a full-term neonate is a challenging clinical situation. Even more challenging is giant omphalocele in a premature low-birth-weight infant. The authors describe a successful staged noninvasive technique for the management of giant omphalocele in a premature, low-birth-weight neonate without the use of prosthetic material until delayed primary closure could be attempted.
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3/12. Direct inguinal hernias in the newborn.

    Direct inguinal hernias occur in newborn babies, both term and premature. Five cases are reported to illustrate three types of direct hernia. The first is a direct weakness without associated significant indirect hernial sac; the second, a sliding direct hernia. The third might be called a 'secondary' direct weakness resulting from a primarily indirect hernia which assumes such large size and develops such a wide neck at the internal ring that the posterior wall of the inguinal canal is stretched and weakened. This is most likely to occur in very low birthweight babies, who develop giant inguinoscrotal hernias. Full exploration and repair of the posterior wall of the inguinal canal should be performed in such babies with huge indirect hernial sacs and in all babies where the size of the processus vaginalis identified at the internal ring is not consistent with the hernial swelling identified clinically. Repair should be performed in conventional manner with non-absorbable sutures reinforcing the transversalis fascia. Overlying Bassini repair with or without Tanner's slide can be performed. The repair should be carried out before the baby leaves a high dependency area.
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4/12. Immunocytochemical studies on a fetal brain of tuberous sclerosis.

    A female stillborn baby was delivered at 36 weeks' gestation. There were rhabdomyomas in the heart, multi-cystic kidneys, and tubers in the brain. Neuropathologic examination of the brain tissue revealed typical giant cells in the sub-ependymal nodules, cerebral white matter, and cortex. On immunocytochemical staining, the giant cells stained positively with antisera to glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), and neuron-specific enolase (NSE). The giant cells as well as adjacent glial cells with small eccentric nuclei contained both MBP and GFAP. Both cell types combined immunocytochemical characteristics of astrocytes and oligodendrocytes. There were also intermediate-sized cells found among giant cells and germ cells in subependymal nodules. In white matter nodules, similar intermediate-sized cells were found among giant cells and glial cells with small eccentric nuclei. Therefore, giant cells as well as glial cells with small eccentric nuclei may be multi-potential and may result from abnormal patterns of maturation.
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5/12. tuberous sclerosis in the premature infant: a report of a case with immunohistochemistry on the CNS.

    Numerous hard cortical nodules, a large subependymal giant-cell astrocytoma and a massive rhabdomyoma were described in a hydropic infant of 31 weeks gestation who lived only four hours. With six other documented cases, tuberous sclerosis in premature infants was reviewed in the hope to pinpoint the early lesions in the CNS and heart. Three premature infants including the present case with tuberous sclerosis presented as hydrops fetalis, and all three had a massive rhabdomyoma. immunohistochemistry on the CNS lesions disclosed aggregates of bizarre giant cells, which concomitantly expressed epitopes for astroglial, oligodendroglial and neuronal phenotypes in the subpial, cortical, subcortical and subependymal zones. The findings strongly indicate that hamartomatous differentiation of pluripotential germinal neuroglia in the subependymal layer establishes early in the gestational stage and causes aberrant and/or arrested migration towards the cortical surfaces.
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6/12. Cervical teratomas: an analysis. literature review and proposed classification.

    Cervical teratomas are uncommon lesions usually diagnosed at birth but occasionally reported in older children and adults. During a 58-year span, nine cervical teratomas were identified at our institution (four previously reported): three stillborns with giant tumors; five live newborns; and one adult with a malignant tumor. Of the five newborns, two prematures died within one hour of birth. Of the three survivors, 2 had respiratory distress at birth. These infants were treated with early excision and are well at 7, 6, and 2 years of age. The last patient also had cystic fibrosis. The adult died of metastatic disease 8 months after resection. A literature review disclosed 212 cases in addition to the five reported here. Previous attempts at categorizing cervical teratomas have failed to address clinical patterns and have little prognostic value. We propose a classification based on birth status, age at diagnosis, and the presence or absence of respiratory distress. Group I--stillborn and moribund live newborns: number (N), 27; mortality (M), 100%. Group II--newborn with respiratory distress: N, 99; M, 43.4%. Group III--newborn without respiratory distress: N, 37; M, 2.7%. Group IV--children age 1 month to 18 years: N, 31; M, 3.2%. Group V--adults: N, 23; M, 43.5%. Twenty-six patients in group II and one in group III died without excision of the mass. Seventy-three patients in group II, 36 in group III, and 31 in group IV had extirpation of the tumor. Operative mortality was 11%, 0%, and 3.2%, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)
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7/12. measles pneumonia in a newborn.

    We report a 21-day-old preterm infant who had severe respiratory distress of 6 days' duration and whose lungs revealed a giant cell pneumonia at necropsy. measles antigen was demonstrated in mononuclear and multinucleated epithelial cells of the lung by immunoperoxidase staining. We recommend the immunostaining procedure to differentiate measles from other viruses, such as parainfluenza 2 or 3, and respiratory syncytial virus, all of which may produce giant cell pneumonias.
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8/12. Miliary fungal granulomata confined to the brain: mechanism of meningitis.

    An unusual case of multiple giant cell granulomata due to candida albicans confined to the brain of a premature neonate is reported, with evidence to suggest that this fungal meningitis is preceded by a parenchymal focus.
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9/12. Acute gallbladder dilatation in a neonate: emphasis on ultrasonography.

    A neonate with giant-cell hepatitis developed gallbladder dilatation that was identified on ultrasonography and treated by tube cholecystostomy. Previous reports of hydrops and cholecystitis in neonates are reviewed. Ultrasound identifies gallstones and dilated bile ducts, establishes a large gallbladder as the cause of a palpable mass, and is the ideal way to follow the course of gallbladder dilatation.
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10/12. tuberous sclerosis presenting as diaphragmatic hernia in a newborn.

    A case of tuberous sclerosis in a preterm newborn baby is described, in whom there were also multiple rhabdomyomas of heart, diaphragmatic hernia, giant-cell astrocytoma of lateral ventricle and malrotation of the intestine. This is the first report of an association of tuberous sclerosis with diaphragmatic hernia. The presence of multiple congenital malformations supports the theory that tuberous sclerosis represents multi-germ layer dysplasia.
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