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1/29. Cerebellar defect associated with Schimke immuno-osseous dysplasia.

    We report the finding of an absent cerebellar hemisphere and partial absence of the cerebellar vermis in a child with dysmorphic features, spondyloepiphyseal dysplasia, steroid resistant nephrotic syndrome secondary to focal segmental glomerulosclerosis and T-cell lymphopenia (Schimke immuno-osseous dysplasia). These findings have not, to our knowledge, been described before and are likely to represent the consequence of a vascular event either in-utero or in early infancy. CONCLUSION: Cerebral imaging should be performed early in the course of the disease and should be repeated if further neurological events develop.
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ranking = 1
keywords = dysplasia
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2/29. decompression of multiple pneumatoceles in a premature infant by percutaneous catheter placement.

    Pneumatoceles due to acquired localized overinflation as a form of pulmonary interstitial emphysema are complications of advanced bronchopulmonary dysplasia. Different ventilation modes, selective bronchial intubation, balloon obstruction of the affected bronchus and steroids have been reported with success. Lobectomy has also been used. We present a premature infant with multiple large pneumatoceles causing respiratory compromise. In our case percutaneous decompression under fluoroscopy guidance resulted in a permanent cure.
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ranking = 1962.0773521032
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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3/29. Developmental bilateral perisylvian dysplasia.

    Acquired bilateral anterior opercular lesions result in the characteristic Foix-Chavany-Marie syndrome that features expressive dysphasia and pseudobulbar palsy. A developmental congenital variant that represents a restricted disorder of neuronal migration was recently reported. We report a newborn with autopsy-confirmed developmental bilateral perisylvian dysplasia. Polymicrogyria was found on detailed histologic study confirming the only prior pathologic study of this syndrome. The clinical heterogeneity of this disorder with neonatal and childhood modes of presentation is reviewed. Speculation regarding pathogenesis focuses on either a genetically determined selective aberration of neuronal migration or an in utero postmigration vascular accident.
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ranking = 0.83333333333333
keywords = dysplasia
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4/29. Clinico-pathological conference: a preterm infant with multiple congenital anomalies.

    A preterm female infant (28 weeks; 880 g) presented with bilateral ectrodactyly of the feet, small cleft palate, esophageal atresia and T-E fistula, multivalvular dysplasia and VSD, thrombocytopenia, and other minor anomalies. cytogenetic analysis showed trisomy 18.
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ranking = 0.16666666666667
keywords = dysplasia
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5/29. Anesthetic considerations of an infant with beckwith-wiedemann syndrome.

    The case of a 3-day-old infant with beckwith-wiedemann syndrome who required anesthetic care during closure of an abdominal wall defect is presented. beckwith-wiedemann syndrome comprises a constellation of clinical features, including macroglossia, macrosomia, omphalocele, visceromegaly, mild microcephaly, facial nevus flammeus, horizontal earlobe creases, and renal medullary dysplasia. Due to the high rate of omphalocele in this syndrome, anesthetic care is frequently required during the neonatal period. Many of these infants (greater than 50%) are born prematurely. Therefore, their anesthetic care may be further complicated by associated diseases of prematurity, such as hyaline membrane disease. Additional anesthetic implications of this syndrome relate to the occurrence and management of hypoglycemia and polycythemia. Careful intraoperative management of glucose homeostasis is particularly important, since eventual neurologic outcome and intelligence will be normal provided prolonged neonatal hypoglycemia is avoided. Preoperative evaluation of the cardiac and genitourinary system, including echocardiography and renal ultrasound, are recommended because of the frequent occurrence of associated anomalies with omphalocele.
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ranking = 0.16666666666667
keywords = dysplasia
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6/29. Supratentorial functional disturbances in two children with cerebellar cortical dysplasia.

    When evaluating children with mental retardation, subtle cerebral and cerebellar morphologic anomalies are often noted at magnetic resonance imaging (MRI). Some, such as cerebellar cortical dysplasia (CCD), have been considered as subtle markers of cerebral dysgenesis. Their functional significance and their effect on brain function, remain unknown. To study supratentorial functional disturbances related to CCD we performed Positron-Emission-tomography (PET) studies in two children with isolated CCD, in order to investigate the degree of involvement of supratentorial structures. One had developmental delay, motor disturbances and ataxia, and the other one only had mental retardation. PET studies revealed hypoperfusion and hypometabolism within the vermis, thalamus and the right striatum in one case, and hypometabolism in the basal ganglia and cerebellar deep grey nuclei in the other case. Our results could lead to a hypothesis explaining motor disturbances as well as cognitive impairment, and could suggest a pathological functional significance of CCD. Nevertheless, the relationship between these findings and mental retardation needs further investigation.
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ranking = 0.83333333333333
keywords = dysplasia
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7/29. short rib-polydactyly syndrome: lethal chondrodysplasia associated with brain malformations in a 35-week-gestation infant.

    This case report describes the neuropathological findings in an autopsy case of short rib-polydactyly syndrome (SRPS). The patient was a Japanese female neonate who was born at 35 weeks of gestation and died soon after birth due to severe cardiopulmonary insufficiency. Clinical and radiological findings were most consistent with SRPS type I (Saldino-Noonan type). General autopsy findings included situs inversus, persistent truncus arteriosus and endocardial cushion defect, hypoplastic lungs and adrenal glands, and vaginal atresia. Fixed brain weight was 330 g. Three different categories of pathological changes were detected in the brain. These were as follows: (1) multiple cyst formation in the parenchyma, (2) primary malformations of the nervous and mesenchymal tissues, and (3) deposition of an unusual substance in the cerebral white matter. The multiple cysts or cavities in the parenchyma may be due to severe hypoxic-ischemic insults related to the congenital heart anomaly. The primary malformations were summarized as follows: (1) capillary telangiectasia of the pia mater and choroid plexus, (2) olfactory dysplasia with asymmetry, (3) focal cortical dysplasia in the frontal lobe and cerebellum, (4) olivary dysplasia, and (5) enlargement of the posterior part of the lateral ventricle. Dysplastic changes of the nervous tissue can be classified into the group of neuronal migration disorders. Although biochemical properties of the unknown substance were not determined, it is considered to be some product derived from an inborn error of metabolism. Morphological data of SRPS is still scarce, and pathognomonic changes have not yet been elucidated. The present data suggests that coexistence of the nervous and mesenchymal malformations may be highly characteristic of SRPS.
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ranking = 1.1666666666667
keywords = dysplasia
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8/29. Long-term complications in a premature infant with tracheoesophageal fistula.

    This is a report of the short- and long-term complications in a premature infant with tracheoesophageal fistula, including those related to central venous alimentation, seizures, chylothorax, bronchopulmonary dysplasia, dental erosions, gastroesophageal reflux, pulmonary problems, and gall stones. It offers analyses of possible alternate methods and treatments, which may have provided a better course.
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ranking = 1962.0773521032
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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9/29. Chronic respiratory disease in premature infants caused by chlamydia trachomatis.

    The relation between chronic respiratory disease and infection with chlamydia trachomatis in premature infants was investigated to ascertain the aetiological importance of intrauterine C trachomatis infection and chronic respiratory disease in premature infants. serum IgM antibodies against C trachomatis were determined by enzyme linked fluorescence assay. Sections of lung tissues obtained by biopsy and at necropsy were also tested for the presence of antigens using fluorescein conjugated monoclonal antibodies to C trachomatis. Of 16 sera from premature infants with chronic respiratory diseases clinically diagnosed as bronchopulmonary dysplasia or the Wilson-Mikity syndrome, five had IgM antibodies to C trachomatis L2 strain by enzyme linked fluorescence assay (titre greater than or equal to 1/500). Of 37 sera from premature infants with extremely low birth weights, two had IgM antibodies to C trachomatis. No specific IgM antibody was detected in 31 neonates who showed raised serum IgM concentrations but who did not have respiratory tract symptoms. C trachomatis was identified from two specimens of lung tissue obtained at necropsy from premature infants with chronic respiratory disease positive for IgM antibody. These findings indicate the aetiological importance of intrauterine C trachomatis infection in chronic respiratory disease in premature infants.
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ranking = 1962.0773521032
keywords = bronchopulmonary dysplasia, bronchopulmonary, dysplasia
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10/29. Neuronal intestinal dysplasia--clinical and histochemical findings and its association with Hirschsprung's disease.

    Neuronal intestinal dysplasia (NID) is a disturbance of the innervation of the gut. Its symptoms resemble the ones seen in Hirschsprung's Disease. Contrary to aganglionosis, however, there is hyperplasia of the ganglia. Our study revealed isolated NID (4/49 cases) to be eight times rarer than aganglionosis (33/49 patients). Combination of both diseases has been reported to occur in 20% of NID cases; we did however find it in 75% (12/16) of our patients. NID was located proximal to the aganglionic segment and reached the stomach in 1 case. Combination of both diseases resulted in superposition of symptoms, thus rendering impossible to differentiate between NID group A (with hypoplasia of sympathetic innervation and acute early onset) and group B (with normal sympathetic innervation and chronic, late onset) on clinical grounds only. Major malformations were present in 5/16 patients with NID; 2 of them had trisomy 21.
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ranking = 0.83333333333333
keywords = dysplasia
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