Turkish J Pediatr
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Review Case Reports
Congenital generalized infantile myofibromatosis and neonatal hemochromatosis. An autopsy case report.
An autopsy case of congenital infantile myofibromatosis and neonatal hemochromatosis is reported. A thirty-six-hour-old baby girl had multiple subcutaneous nodules in addition to multiple visceral involvement of heart, lungs, pharynx, larynx, stomach, small bowel, large bowel, pancreas, kidneys, spleen, thyroid, adrenal glands, lymph nodes, peripheral nerves, meninges and soft tissues. ⋯ The histological and immunohistochemical findings of the case suggested that a close relationship may exist between infantile myofibromatosis and infantile hemangiopericytoma. In addition to infantile myofibromatosis, neonatal hemochromatosis characterized by iron deposition in parenchymatous organs such as liver, pancreas, lungs, thyroid, and adrenal glands was another important characteristic of the case.
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Congenital thoracic ectopic kidney is a very rare developmental anomaly and the rarest form of all ectopic kidneys. It is usually asymptomatic and discovered incidentally on a routine chest radiography. ⋯ We hereby discuss the features of congenital thoracic ectopic kidney and review the literature. Although it is extremely rare, thoracic ectopic kidney should be considered in differential diagnosis of a mass with a well demarcated superior margin in the lower part of the thorax, and renal scintigraphy must be performed even if CT and IVP results are normal.
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Infantile pyloric stenosis is one of the most common conditions requiring surgery during the first few weeks of life. The association of infantile pyloric stenosis with gastric volvulus in an extremely uncommon occurrence. ⋯ His current problem was recurrent pulmonary infections and he was diagnosed to have organoaxial gastric volvulus and gastroesophageal reflux. The common features of presentation, radiological findings, surgical procedures and possible mechanisms of gastric volvulus associated with infantile pyloric stenosis are discussed.
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Clinical Trial
Cyclosporin A plus prednisone treatment of steroid-sensitive frequently relapsing nephrotic syndrome in children.
Recently, there have been numerous reports on the use of cyclosporin A (CyA) in children with nephrotic syndrome (NS). In this prospective study, we wanted to evaluate the efficacy of CyA together with prednisone therapy in children with steroid-sensitive frequently relapsing NS. A total of 11 children (7 boys, 4 girls) with steroid-sensitive NS were included in this study. ⋯ After discontinuation of therapy, the relapse rate dropped to a mean of 0.0179 +/- 0.031 with a range of 0 to 0.083. In conclusion, it would appear that a combination of CyA and prednisone is effective, sustaining the remission in steroid-sensitive NS. Corticosteroids in combination with CyA may be a better approach than conventional steroid treatment in such patients.
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In the last twenty-five years more has been learned about the human brain than in the past history of mankind. Through the use of new technologies such as PET and CAT scans and functional MRI's, it is now possible to see and learn much about the human brain while it is in the process of thinking. The research of neuroscientists, such as Marian Diamond, has demonstrated that the brain changes physiologically as a result of learning and experience--for better or worse--and that plasticity can continue throughout the lifespan. ⋯ And--of critical importance if any of this is to lead to a healthy future--they are helping students to learn to use knowledge responsibly, ethically, and with integrity. Furthermore, they are involving students in experiences that develop compassion and altruism in the process of their education. Our complex world urgently needs more people who have developed their fullest potential in mind, body, and spirit.