Turcot syndrome: a case with spinal cord and colonic neoplasms.
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Biomedical subjects
Publications and source records attributed to V G Mikity.
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Hepatic amebic abscess is a potentially fatal infection which is difficult to diagnose in children. Sonography is an ideal non-invasive imaging tool which can prove invaluable in the diagnosis and management of hepatic amebic abscess. Sonographic findings should suggest the correct diagnosis in most instances. This allows prompt initiation of therapy, forestalling life-threatening complications. Sonography is also an extremely useful non-invasive means of following response to therapy in children with hepatic amebic abscesses.
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The gross, microscopic, and ultrastructural features of a mixed liver hamartoma occurring in a three month old infant are reported. The differentiation from two solid liver masses, focal nodular hyperplasia and liver cell adenoma is emphasized. Mesenchymal hamartomas, though usually cystic rather than solid masses, share the histologic feature of fibroductular tissue with mixed liver hamartomas and focal nodular hyperplasia. Only the mixed liver hamartoma has extremely broad fields of ductules and an embryonic type of hepatocyte at the ultrastructural level. These and other hepatic lesions show morphologic evidence of transformation of liver cells into biliary epithelial cells in association with vascular connective tissue.
In order to define the minimum effective dose of human growth hormone (GH) in growth hormone deficient children, GH was administered to three groups of patients based on their body weight. Five children who received 0.01 International Unit (IU) GH/kg three times a week (tiw) failed to respond with a significant increase in their rate of growth. A dose of 0.03 IU GH/kg tiw increased the growth rate of 12 patients from 3.5 +/- 0.4 (SE) cm/year to 6.4 +/- 0.4 (SE) cm/year (P less than 0.001) during the first 12 months of therapy. Eight children (67%) larger than or equal to 6.0 cm/year. A similar increase growth rate from 3.6 +/- 0.4 (SE) cm/year to 7.3 +/- 0.4 (SE) cm/year (P less than 0.001) was observed over the first 12 months of therapy in 16 growth hormone deficient children who were given 0.06 IU GH/kg tiw. Thirteen children (81%) grew larger than or equal to 6.0 cm/year. During a second year of treatment, children receiving either 0.03 or 0.06 UI GH/kg tiw again showed a significant increase in their rate of growth. However, the response was significantly less than that observed during the first year of treatment. Comparison of these results with those available in the literature suggests that the most efficient, although not necessarily the optimal, initial dose of GH in children with growth hormone deficiency is 0.06 IU GH/kg administered three times a week.
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Six cases of cholecystitis and cholelithiasis confirmed by x-ray examination and surgical operation were observed in a ten-year period. Due to the wide variability in signs and symptoms in children, cholecystitis and cholelithiasis can be diagnosed only with a high degree of clinical suspicion and roentgenological examination. Gallbladder disease is uncommon in childhood but should be considered in children with vague abdominal pains or bouts of unexplained jaundice. If a normal appendix is found at laparotomy in the "acute abdomen," the surgeon would be wise to palpate other specific organs within the abdomen, including the liver and gallbladder. The treatment of choice is cholecystectomy. The prognosis for recovery is excellent if there is no complicating systemic disease.
Records of 25 patients with intestinal perforation in early infancy who were treated at the Los Angeles County General Hospital in a period of 15 years were reviewed. Sixteen had roentgen evidence of pneumoperitoneum, and nine did not. The mortality rate was 94 per cent in the group with pneumoperitoneum, 78 per cent in the other, and 88 per cent overall. Multiple sites in the gastrointestinal tract were involved, and the causes of the lesions were diverse and frequently obscure. Prematurity, obstetrical and iatrogenic complications, and congenital anomalies were factors often associated with intestinal perforation. Roentgen features appeared to offer the best hope for diagnosis and appropriate treatment.