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F Daum

Publications and source records attributed to F Daum.

67 records · Page 4Linked to original sources

Proceedings: Persepectives on adolescent medicine: concepts and program design.

The concepts and goals of a program in adolescent medicine should include development of a capability to focus on current health needs of youth in a variety of settings; to plan clinical services to meet those needs with the flexibility necessary to respond to changing future requirements; and to deliver service within such a context while simulataneously creating a milieu conducive to education and investigation into the very process and definition of adolescence. The Division of Adolescent Medicine at Montefiore Hospital and Medical Center was designed 7 years ago to fulfill these goals and consequently may serve others as a functional model for health care delivery to teenagers. The Division is comprised of: (1) a 37 bed in-patient unit; (2) a hospital-based ambulatory program including general diagnostic and follow-up services, as well as a speciality service capability in the areas of gynecology and family planning, cardiology, gastroenterology and nutrition; (3) primary care health services within teenage dentention and prison facilities; (4) addictive disease diagnostic and treatment programs; (5) school health programs from intermediate school through college levels, and (6) the division also performs supportive and consultative functions for a variety of community-based agencies. Within the programatic design approximately 70,000 adolescents have been served. The cornerstone of the educational and investigative efforts has been the concept that all the above six functional units are clinical laboratories and classrooms so that training and research activities are integral parts of each of the service areas. This program design is continually undergoing revision and refinement so as to remain ever-responsive to new and emerging problems to meet additional training demands and, most importantly, to permit and encourage creativity and growth patients and staff.

Adolescent↗

Arteriohepatic dysplasia. I. Pitfalls in diagnosis and management.

Differentiating intrahepatic cholestasis from extrahepatic biliary tract obstruction may be difficult. Four patients with intraoperative cholangiographic evidence of extrahepatic ductal atresia who underwent hepatoportoenterostomy are described. All were ultimately shown to have arteriohepatic dysplasia with hypoplastic but patent extrahepatic ductal systems. The difficulty in establishing an accurate diagnosis, hazards associated with hepatoportoenterostomy, and suggestions for evaluation and management are discussed.

Bile Ducts↗

Arteriohepatic dysplasia. II. Hepatobiliary morphology.

Five children were noted to have arteriohepatic dysplasia (Alagille's syndrome) between 3 and 7 months of age. Prior to diagnosis, four underwent Kasai procedures after intraoperative cholangiograms failed to demonstrate patency of the extrahepatic bile ducts. In three patients, a focal proximal hypoplasia of the common hepatic duct was demonstrated with fibrosis and increased vascularity. Hypoplasia of the gallbladder occurred in two patients. Changes were observed in the porta hepatis. Eighty of 208 micrometers bile ducts were associated with peripherally located gland-like structures. These changes are indistinguishable from those in fibrous remnants of extrahepatic biliary atresia. Hepatic features of sequential liver biopsies obtained in the five patients were divided into early and late changes. From birth to 3 months of age, the pathology consisted of cholestasis and bile duct destruction. After 3 months of age, there was persistent cholestasis, paucity of interlobular bile ducts, and portal fibrosis. Ductular proliferation was not an intrinsic change. When present, it was related to a recent episode of cholangitis.

Bile Ducts↗

Nonsyndromatic paucity of interlobular bile ducts: light and electron microscopic evaluation of sequential liver biopsies in early childhood.

Liver biopsies and/or autopsy specimens from 17 children (ages 1 week to 5 years) with nonsyndromatic paucity of the interlobular bile ducts were studied by light and electron microscopy. Initial biopsies were obtained before 90 days of age from all patients, and two or more specimens were available from nine. No specific underlying condition was found in nine infants. The remaining cases were associated with Down's syndrome (n = 2), hypopituitarism (n = 2), cystic fibrosis (n = 1), alpha 1-antitrypsin deficiency (n = 1), cytomegalovirus (n = 1) and Ivemark syndrome (n = 1). Before 90 days of age, portal changes included duct paucity and fibrosis. Lobular changes were nonspecific, consisting of cholestasis, giant cell transformation, extramedullary hematopoiesis and perisinusoidal fibrosis. Duct paucity, portal fibrosis and perisinusoidal fibrosis persisted after 90 days. Cholestasis was mild or no longer apparent. Portal changes before 90 days of age appear to be sufficiently distinctive to microscopically distinguish nonsyndromatic from syndromatic paucity. Electron microscopic findings suggest that paucity in nonsyndromatic patients may result from a primary ductal insult: ultrastructural studies revealed bile duct destruction characterized by undulation and breaks in the basal lamina and infiltration of the epithelium by lymphocytes. Bile canalicular dilatation with blunting of microvilli and electron-dense material in the lumen, predominantly seen before 90 days, also reinforces the hypothesis of a primary ductal defect.

Age Factors↗

Cutaneous polyarteritis nodosa associated with Crohn's disease.

A 13-year-old white girl with Crohn's colitis developed recurrent erythematous tender cords and nodules in the lower and upper extremities. Histologic examination of subcutaneous nodules of the right arm revealed granulomatous panarteritis of two muscular arteries in the subcutis. The patient's resected colon showed granulomatous transmural colitis without vasculitis. The association between Crohn's disease and cutaneous polyarteritis nodosa is reviewed and emphasis placed on histologic evaluation of suspicious subcutaneous nodules for correct diagnosis.

Adolescent↗

Origin of cardiac mucosa: ontogenic consideration.

The origin and histology of the cardiac mucosa remains controversial. The classical concept that the cardiac mucosa is of gastric origin has been challenged by those who advocate that the cardiac mucosa results from a metaplastic esophageal process. Some regard cardiac mucosa as consisting solely of pure mucous glands, whereas others accept the presence of isolated parietal cells within the mucous gland (mixed glands). In this study, we have clarified the presence and site of origin of the cardiac mucosa and its histological composition. To do so we studied the microscopic characteristics of the gastric side of the squamous-columnar junction (SCJ) of 77 autopsied fetuses of different gestational ages (prenatal group) and of infants, young children, and adolescents (postnatal group). We evaluated the presence or absence of a transitional zone, defined as the area between the squamous esophageal and oxyntic mucosa, the glandular composition of the transitional zone (i.e., pure mucous and mixed glands), and the presence or absence of inflammation. Our study revealed that a transitional zone with the microscopic characteristics of cardiac mucosa was universally present at the SCJ. The microscopic characteristics of this zone varied with age. Both pure mucous and mixed glands were observed. We conclude that the cardiac mucosa is partially if not entirely the result of normal embryonic gastric development. Both mucous and mixed glands constitute normal components of the cardiac mucosa.

Adolescent↗

Hepatic Na,K-ATPase development in the rat.

The development of the enzyme Na,K-ATPase was studied in hepatic tissue from Sprague-Dawley rats at 18-20 days of gestation, 1, 3, 5, 7, 14, 21, and 30 days, and young adulthood (45 days). A developmental pattern was demonstrated for total and membrane-associated Na,K-ATPase activity. The activity in tissue homogenate, expressed per gram tissue, increased from late fetal life, 43.4 +/- 1.3 mumol Pi g liver-1 h-1, until 21 days of age, when an adult level of 187.3 +/- 19.6 mumol Pi g liver-1 h-1 was attained. A less pronounced ontogenic pattern was observed when enzyme levels were expressed as specific activity per milligram protein. The activity profile in a crude membrane preparation was similar. The potential for stimulation of enzyme activity by glucocorticoids was studied in 11-day-old animals injected with cortisone acetate (10 mg 100 g body weight-1) for 3 days. Enzyme specific activity was inducible: Specific activity was greater in cortisone-treated animals, 1.794 +/- .043 mumol Pi mg protein-1 h-1, versus controls, 1.258 +/- 0.043 mumol Pi mg protein-1 h-1 (p less than 0.01). We postulate that this developmental pattern for hepatic Na,K-ATPase activity may be a reflection of, or a contributing factor in, the ontogeny of sodium-dependent hepatic transport, such as that for bile salts.

Aging↗

Ontogeny of renal dysplasia in Ivemark syndrome: light and immunohistochemical characterization.

Ivemark syndrome is a rare sporadic or autosomal recessive disorder characterized by pancreatic fibrosis, renal dysplasia and hepatic dysgenesis. There have been no data describing the renal changes during embryologic development in this syndrome. In this report, we document the pathological findings of the kidney in three subjects with Ivemark syndrome: 6 months, 21 weeks and 16 weeks, respectively. Kidneys of subjects and age-matched controls were examined by light microscopy and immunohistochemically for cytokeratin, AE1/AE3 and epithelial membrane antigen. Renal dysplasia in Ivemark syndrome becomes apparent at 16 weeks of gestation and progresses thereafter in severity. It is characterized by disturbance in glomerular differentiation, delay in tubular differentiation and abnormal expression of epithelial markers in glomeruli and tubules. Cytokeratin and epithelial membrane antigen expression of cysts is similar to that of the collecting ducts.

Abnormalities, Multiple↗

Arteriohepatic dysplasia (Alagille's syndrome): a common cause of conjugated hyperbilirubinemia.

Syndromatic paucity of interlobular bile ducts is a common cause of conjugated hyperbilirubinemia in children. The clinical presentation is not always obvious. Therefore, the liver biopsy may be a useful diagnostic tool in the definition of this entity. The hepatic and biliary morphology of five children with arteriohepatic dysplasia (Allagille' syndrome) is described. Prior to diagnosis, four underwent Kasai procedures after intraoperative cholangiograms failed to demonstrate patency of the extrahepatic bile ducts. In three patients, a focal proximal hypoplasia of the common hepatic duct was demonstrated. Hypoplasia of the gallbladder occurred in two patients. Hepatic features of sequential liver biopsies obtained on the five patients, were divided into early and late changes. From birth to four months of age, the pathology consistent of cholestasis, paucity of interlobular bile ducts and portal fibrosis. The etiology of arteriohepatic dysplasia is unclear. The main pathogenic mechanisms are discussed. It is felt that the syndromatic duct paucity represents an acquired primary ductal defect resulting from a genetically determined immune response to as yet undefined agent or agents.

Bile Ducts↗