1/19. Early bone grafting in Tessier number 4 cleft: a case report.Rare craniofacial clefts have an incidence of at least 1 per 100,000 live births. A Tessier number 4 cleft is one of the most rare craniofacial clefts, with less than 50 cases being reported in the literature. Both soft and bony tissue abnormalities take place in the cleft morphology, so not only clinical examination of the maxillofacial region but a detailed radiological workup is needed to assess clearly the nature of the clefts. A patient with a Tessier number 4 cleft is presented, whose bony defect was obliterated with autogenous iliac bone graft chips and soft tissue reconstruction was performed with multiple Z-plasty flaps. Postoperative clinical and radiological results demonstrate fine healing and good cosmesis. Although controversy still exists about the treatment of facial clefts with early bone grafts, advantages of performing both bony and soft tissue reconstructions in a single session make this treatment a good alternative with satisfactory clinical and radiological results.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/19. Polyclonal B-cell lymphocytosis mimicking malignant lymphoma in a newborn.We describe a 17-day-old newborn with fever and peripheral blood lymphocytosis. The circulating lymphocytes were large with lobulated and nucleolated nuclei. Their immature and uniform appearance raised the possibility of malignant lymphoma in the leukemic phase. Immunophenotypic study, however, showed that the lymphocytes were CD19( ) B cells with expression of both kappa and lambda light chains. molecular biology study confirmed a polyclonal nature of the immunoglobulin heavy-chain gene. cytogenetic analysis showed a normal karyotype, and viral cultures and serologic studies yielded negative results. The polyclonal lymphocytosis was self-limiting and disappeared within a month.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/19. Neonatal complications at term as related to the degree of umbilical artery acidemia.The degree of umbilical arterial acidemia associated with immediate newborn morbidity has not been determined. Therefore we compared 358 term infants with umbilical artery acidemia (pH less than 7.20) with 358 term, nonacidotic matched control infants, to evaluate immediate neonatal complications in both groups. Nonacidotic was defined as an umbilical artery pH greater than or equal to 7.20. Complications included seizures, persistent hypotonia, and/or signs of end-organ damage such as renal or cardiac dysfunction. None of the 693 newborns with an umbilical artery pH greater than or equal to 7.00 had such complications. Two of 23 infants with an umbilical artery pH less than 7.00 had sequelae related to intrapartum asphyxia. In these two infants the umbilical artery pH was less than 7.00, the 1-minute and 5-minute Apgar scores were less than or equal to 3 and the acidemia was metabolic in nature.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/19. Idiopathic Heinz body hemolytic anemia in newborn infants.Heinz body hemolytic anemia developed in six full-term infants while at home during the first 2 weeks of life. The disorder first manifested as hyperbilirubinemia. However, in all cases, severe anemia (hemoglobin concentration 49-73 g/L) developed during the 4-12 days of hospitalization. The infants had not been exposed to known oxidants, and their erythrocytes were not glucose-6-phosphate dehydrogenase (G6PD) deficient and contained no unstable hemoglobin. It is hypothesized that in these newborn infants, Heinz body hemolytic anemia developed as a result of ingestion of an oxidant contained in feedings. The nature of this agent is as yet unknown.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/19. Acute disseminated aspergillosis during the neonatal period. Report of an instance in a 14-day-old infant.We describe an infant who died with extensive lesions of disseminated aspergillosis on the 18th day of postnatal life. aspergillus fumigatus was recovered from blood cultures. Initial clinical manifestations were suggestive of hepatitis, and steroids and antibiotics were used in the treatment. This therapy may have fostered the onset of an opportunistic mycosis. Advanced multisystemic aspergillotic lesions were seen at autopsy, especially prominent in lungs and gastrointestinal tract. The extent and magnitude of the lesions observed suggest inception of the disease very early in life, although no case of human aspergillosis has been known to be congenital. Neonatal aspergillosis is poorly characterized. Only four previously reported cases came to our notice in which the disease could be diagnosed in the first month of life. The ubiquitous nature of pathogenic Aspergillus, joined to aggresive treatments designed to achieve increased survivals in neonatology, could potentially result in greater numbers of cases of this and other uncommon mycoses.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/19. Idiopathic infantile arterial calcification in siblings: radiologic diagnosis and successful treatment.Idiopathic infantile arterial calcification is a disorder of unknown etiology manifested by widespread arterial calcification. This usually leads to early death from coronary artery occlusion. In 12 of the 75 cases in the literature, radiographs were taken and it was possible to make the diagnosis in them all. We present two patients, siblings, in whom the diagnosis was established radiologically. The nature of the calcium deposits was studied in one of the infants and proved to be calcium hydroxyapatite. Therapy with diphosphonate was apparently successful in the other child.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/19. Pediatric osteomyelitis and septic arthritis: the pathology of neonatal disease.The morphologic and histologic examination of over fifty-five foci of metaphyseal/epiphyseal osteomyelitis and eleven septic joints from five cases of neonatal osteomyelitis and joint sepsis are described in detail. The severity of the bone and joint involvement varied considerably, allowing a better understanding of the pathophysiologic sequence of events in the disease in the neonatal time period. Of particular importance were (1) the multifocal nature of the disease, (2) the highly variable destruction of the growth plate (physis) by several discrete mechanisms, and (3) the invasion of the chondroepiphysis through the cartilage canal systems. Two of the cases died from respiratory complications several months following presumed successful treatment of their skeletal infections. S&pecimens showed significant growth plate damage continuing beyond the neonatal period. These findings support the need for rapid diagnosis and drainage, whenever feasible, to prevent long-term skeletal growth damage. The severity of involvement also should emphasize that this disease, especially in the neonate, is not an innocuous condition, as a recent review suggested.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/19. Mesenteric lymphangioma. A case report with transmission and scanning electron microscopic studies.A mesenteric lymphangioma was found in a 2-day-old newborn, thus attesting to the congenital nature of the lesion. The ultrastructural features of the tumor included a poorly developed, discontinuous basal lamina, and anchoring fibrils distinguished it from a hemangioma. An undulating surface of the endothelium and prominent grooving between the endothelial cells were found by scanning electron microscopy and were indistinguishable from arterial and lymphatic endothelial surfaces that have been studied by others.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/19. Type I congenital dyserythropoietic anaemia with myelopoietic abnormalities and hand malformations.Type I dyserythropoietic anaemia was diagnosed in an infant, who presented with respiratory distress and hepatosplenomegaly soon after birth. Anaemia became manifest during the neonatal period. The case clearly proves the congenital nature of the disease. Abnormalities of the myelopoietic series indicate that it might be a stem cell disease and the presence of skeletal anomalies of the hands suggests a genetic relationship to some cases of Fanconi and diamond anaemia. No serum lipid or vitamin e deficiency was present as in type II congenital dyserythropoietic anaemia. Serial serum ferritin determinations indicated that iron stores are increased early in type I congenital dyserythropoietic anaemia despite no transfusion load.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/19. Ultrasound in the investigation of renal disease in infants and children.The author reports the case histories of a number of neonates and children with renal disease to illustrate the use of grey-scale ultrasound in the investigation of such patients. In the series, ultrasound proved to be an excellent diagnostic tool in the investigation of masses of suspected renal origin, both in ascertaining their site of origin and in evaluating their nature. Other applications of diagnostic ultrasound included investigation of the kidney not visualised by urography, and suspected urinary tract obstruction, and renal or peri-renal infection. The characteristic ultrasound appearances of polycystic disease, multicystic kidney, renal tract obstruction, nephroblastoma and perinephric abscess are shown.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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