1/27. 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.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
2/27. 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.- - - - - - - - - - ranking = 6keywords = cor (Clic here for more details about this article) |
3/27. Successful treatment of ARDS and severe pulmonary hypertension in a child with bordetella pertussis infection.infection with bordetella pertussis can cause severe illness with neurological and pulmonary complications in children. Pulmonary hypertension is an early sign of potentially fatal disease and can cause failure of conventional respiratory therapy in severe acute respiratory distress syndrome (ARDS). We report a 4 1/2-year-old boy with B. pertussis infection who developed severe ARDS and pulmonary hypertension. Because of severe neurological signs the patient did not qualify for extracorporal membrane oxygenation (ECMO). After conventional ventilation, surfactant and high frequency oscillation ventilation (HFOV) failed, treatment with nitric oxide (NO) improved oxygenation, allowing recovery without the need for ECMO. The patient survived with few sequelae. Thus, this treatment may be an option in high-risk children who meet the criteria for ECMO but are excluded because of poor neurological status, as in our patient.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
4/27. The perinatal autopsy: a neglected source of discovery.The perinatal autopsy is frequently viewed by pathologists as being scientifically unrewarding and contributing little to patient care. To emphasize its importance in patient management, genetic counselling and specific research, a ten-year review (1979-88) was made of the perinatal autopsy experience at Loyola University Medical Center. The 657 deaths included late fetal deaths (22%), early neonatal deaths (51%), late neonatal deaths (10%) and deaths between 29 days and one year (17%). Comparison of the principal causes of death in various groups categorized by birth weight and age revealed significant trends. There has been a steady decline in mortality from immediate complications of immaturity, while the mortality rate from long-term complications of immaturity has not increased. There were increased frequencies of congenital diaphragmatic hernia and cardiac malformations, while the frequency of renal malformations decreased; the etiological significance of these changes requires further evaluation. A correlation of clinical observations with post-mortem findings indicates that newer diagnostic procedures, such as ultrasound, echocardiography and cardiac catheterization, are of limited value for accurate diagnosis of complex anomalies, cystic renal disease and chromosomal anomalies. A substudy included cases up to 18 years of age. The frequency of childhood neoplasia was low (17 cases), and among these cases there was a predominance of haematological malignancies (11 cases). Even with these small numbers, a shift in cause of death from disseminated malignancy to overwhelming infection was apparent. This paper includes the essential elements of a protocol for perinatal autopsies, with illustrations of specific applications. The perinatal autopsy is clearly an undervalued source of information and discovery. Little or no information is available from developing countries, where autopsies could provide information on causes of paediatric mortality and permit recognition of disease patterns, which is so critical to the planning of health services.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
5/27. Effects of supplemental oxygen administration in an infant with pulmonary artery hypertension.In patients with pulmonary disease, pulmonary artery hypertension often occurs as a result of pulmonary artery vasoconstriction, primarily from hypoxia and alveolar hypotension. In this report we describe the hemodynamic effects of breathing supplemental oxygen in a child with bronchopulmonary dysplasia and pulmonary artery hypertension. These hemodynamic effects include an improvement in oxygenation, an increase in systemic vascular resistance, and a decrease in the pulmonary vascular resistance. As a direct result of these changes in vascular resistances, alterations of heart rate, cardiac index, stroke volume, aortic pressure, oxygen consumption, and pulmonary artery pressure have been shown to occur. Oxygen is widely used to treat many physiologic conditions. However, during the administration of supplemental oxygen, rarely do we recognize the hemodynamic changes associated with its use. These hemodynamic effects must be clearly understood and appreciated before oxygen administration in any clinical situation.- - - - - - - - - - ranking = 0.022440371372136keywords = heart (Clic here for more details about this article) |
6/27. Midfacial hypoplasia associated with long-term intubation for bronchopulmonary dysplasia.Six preterm infants with bronchopulmonary dysplasia were nasotracheally intubated for 68 to 243 days. gestational age at birth ranged from 24 to 35 weeks. Endotracheal tube size was changed to account for growth and varied from 2.5 to 4.0 mm. These infants developed features of midfacial hypoplasia, namely, depressed nasal bridge, small-tipped nose, long philtrum, underdeveloped malar areas, and carplike mouth. These features have not been associated with long-term intubation in premature infants. We suggest that features of prolonged nasotracheal intubation, such as direct compression by the tube and the method of tube fixation, decreased air flow through the developing nares and sinuses and reduced faciomuscular activity, resulting in the observed midfacial hypoplasia. The degree to which growth corrects these deformations is unknown.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
7/27. In utero resolution of hydrops fetalis following the death of one twin in twin-twin transfusion.A case of massive hydramnios involving a twin-twin transfusion syndrome is presented with death of the larger twin and the development of hydrops fetalis in the surviving smaller twin. The amniotic fluid volume of the surviving twin became normal and the hydrops fetalis resolved spontaneously in utero. Concomitant with the resolution of the hydrops fetalis, the umbilical cord systolic to diastolic ratio improved. At the time of delivery, the hydrops had resolved. The liveborn infant died 11 days later secondary to pulmonary hypertension and cardiorespiratory failure. Although the fetal status improved in utero, the pulmonary hypertension postdelivery resulted in neonatal death.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
8/27. bronchopulmonary dysplasia: a review for the pediatrician.In this review we have attempted to introduce bronchopulmonary dysplasia as a new chronic lung disease of infancy and childhood. The major risk factors for this illness are preterm birth and the respiratory distress syndrome. The precise etiology of BPD is not understood but trauma from mechanical ventilation and toxicity from exposure to supplemental oxygen are thought to be important. Problems in diagnosis and diagnostic criteria have been discussed as have the details of the unfavorable pulmonary mechanics. We have mentioned some of our own practices in regard to a large and successful home oxygen therapy program. Suggestions have been made for establishing readiness for discharge and for follow-up of these children. Medical management of these patients presently suffers from a lack of prospective and controlled studies. Medical care draws heavily from experience with pediatric asthma. What is known about the long-term outcome of these children has been reviewed with an attempt to highlight controversies between published reports and underscore the need for further investigation. The greatest future success in this area would be the prevention of premature birth. Prior to this, we must await the completion of future controlled and prospective studies.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
9/27. Rhabdomyomatous dysplasia of the lung.This article deals with the presence of nontumoral striated muscle fibers in the lungs of 3 neonates. These cells were diffusely distributed in one lung (case 1) or in both (case 2), or focally localized to the lung parenchyma adjacent to the liver in a case with a large right diaphragmatic hernia (case 3). The striated muscle fibers were located in the walls of small bronchi and bronchioli or in the alveolar interstitium. Other major lung malformations found simultaneously were absence of lobation, hypoplastic lungs, and hypoplastic pulmonary vessels. The origin of striated muscle fibers in the neonatal lung has been attributed to anomalous differentiation of mesoblastic cells (as in cases 1 and 2). The presence of striated muscle cells in the lower margin of a hypoplastic lung associated with a right diaphragmatic hernia (case 3) suggests that intrapulmonary inclusion of diaphragmatic muscle fibers might be a source as well. Striated muscle fibers in the lung are commonly associated with major malformations involving heart and lungs, suggesting a much wider morphogenetic error.- - - - - - - - - - ranking = 0.022440371372136keywords = heart (Clic here for more details about this article) |
10/27. Neurologic aspects of bronchopulmonary dysplasia.Thirty seven infants were identified with bronchopulmonary dysplasia (BPD). Their hospital records were examined and survivors evaluated to determine the neurologic aspects of BPD. Five infants exhibited progressive neurologic disease; nine infants, Five infants exhibited progressive neurologic disease; nine infants, nonprogressive neurologic disease; and 19, normal neurologic outcome. In four, neurologic outcome was indeterminate because of early death. Thus, evaluation of this population of infants with BPD has established two syndromes of neurologic disease--progressive and nonprogressive. The patients with nonprogressive neurologic disease exhibited static neurologic deficits identical to those described in earlier reports of outcome in infants with BPD. The infants with apparently progressive neurologic disease had clinical courses comparable to the fatal pattern of cerebral deterioration previously described. The syndromes appear to be distinct in etiology and clinical presentation. Intraventricular hemorrhage complicated by hemorrhagic intracerebral involvement appears the factor most clearly associated with nonprogressive neurologic disease. The etiology of the striking progressive neurologic syndrome could not be so clearly established but appears to be related to the course and treatment of BPD.- - - - - - - - - - ranking = 1keywords = cor (Clic here for more details about this article) |
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