PubMed Health⌕ Search

Biomedical subjects

D S Huff

Publications and source records attributed to D S Huff.

At least 37 records · Page 2Linked to original sources
PubMed1989

Postnatal testicular maldevelopment in unilateral cryptorchidism.

Histomorphometric analysis of semi-thin sections was performed on testicular biopsies of 232 unilaterally cryptorchid testes and 195 of their contralateral descended partners. The results demonstrated a decreased number of germ cells detectable from the first year of life. There was delayed and defective transformation of gonocytes to Ad spermatogonia, which normally is complete at age 6 months, delayed or failed transformation of Ad spermatogonia to primary spermatocytes, which normally commences at age 3 years and decreased numbers of Leydig cells. These abnormalities were present in the unilaterally cryptorchid testes and their contralateral descended partners but they were more severe, of earlier onset and more progressive in the cryptorchid testes. These findings are compatible with the hypothesis that hypogonadotropic hypogonadism is the cause of the increased incidence of infertility seen in unilateral cryptorchidism.

Biopsy↗
PubMed1989

Cryptorchidism, orchiopexy and infertility: a critical long-term retrospective analysis.

We assessed the fertility of 40 patients who underwent orchiopexy between 1950 and 1960. Testicular biopsies also had been performed at operation, a practice exceptional for the era. Of 23 patients with unilateral undescended testes who attempted to have children 20 (87 per cent) were successful. In contrast, only 3 (33 per cent) of 9 patients with bilateral undescended testes fathered children. Over-all sperm counts in 16 patients were low but they were not predictive of paternity. Testicular biopsy specimens were reviewed and the fertility index was determined. In most cases a good correlation was found between histological status and paternity status.

Adult↗
PubMed1987

Germ cell counts in semithin sections of biopsies of 115 unilaterally cryptorchid testes. The experience from the Children's Hospital of Philadelphia.

One hundred and fifteen biopsies from unilateral cryptorchid prepubertal patients were embedded in Epon. Semithin sections were examined by light microscope and the germ cell count per tubule was calculated. The mean number of germ cells was normal from birth to 12 months of age and dropped below the lower limits of normal between 1 and 2 years. Germ cell counts rose considerably during the 18-months observation period with the highest counts occurring in the last 6 months. Variability in the clinical diagnosis of cryptorchidism or inherent heterogeneity in the testicular histology of cryptorchidism are possible explanations.

Biopsy↗
PubMed1987

Neuronal lipidosis and neuroaxonal dystrophy in cerebro-hepato-renal (Zellweger) syndrome.

Neuropathological examination of three males with cerebro-hepato-renal (Zellweger) syndrome (CHRS) revealed selective neuronal lipidosis and neuroaxonal dystrophy of the dorsal nucleus of Clarke and lateral cuneate nucleus. This lipidotic alteration was visualized as perikaryal or axonal enlargements with cytoplasmic striations. With the light microscope, the striated material was birefringent and resistant to traditional lipid stains; ultrastructurally, it was composed of lipid clefts, lamellae and lamellar-lipid profiles; biochemically, the affected region contained large amounts of cholesterol esterified to very long-chain fatty acids, both saturated and monounsaturated. This metabolic lesion, though localized to specific sensory neurons, suggests that a more generalized defect in neuronal fatty acid metabolism may be operative in CHRS.

Adrenoleukodystrophy↗
PubMed1987

Oncocytic cardiomyopathy of infancy with Wolff-Parkinson-White syndrome and ectopic foci causing tachydysrhythmias in children.

Two female infants, ages 6 months and 13 months, were first seen in the newborn period with supraventricular tachycardia associated with Wolff-Parkinson-White syndrome. One infant had echocardiographic and angiographic evidence of diffuse cardiomyopathy and died suddenly at home. The other infant was seen initially at 13 months of age with refractory ventricular tachycardia and died following surgical resection of arrhythmogenic foci on the left and right ventricles. Autopsy showed diffuse patchy oncocytic cardiomyopathy in both instances. Serial histologic sections of the cardiac conduction system showed oncocytic involvement of the atrioventricular (AV) node, His bundle, and bundle branches. Both infants had interruption of the anulus fibrosus by oncocytic cells at several sites, resulting in multiple accessory AV and nodoventricular connections. Additionally, patient No. 1 had an accessory AV connection by oncocytic cells in the fatty fibrous tissue of the left AV sulcus. To our knowledge, this is the first report of multiple accessory AV connections of oncocytic cells seen during histologic study. In addition, both infants had oncocytic involvement of the exocrine and endocrine glands. This report discusses the clinicopathologic correlations in these two patients, the literature on oncocytic cardiomyopathy, and the types of dysrhythmias found in these patients and their management.

Cardiomyopathies↗
PubMed1986

MURCS association with additional congenital anomalies.

The postmortem findings in a patient with the MURCS association (müllerian duct aplasia/hypoplasia, renal agenesis or ectopy, and cervicothoracic somite dysplasia) are reported. This is the first autopsy study since the syndrome was recognized. The autopsy revealed abnormalities of the venous, pulmonary, and central nervous systems that had not been reported previously in patients with this syndrome. A review of the literature suggested that although the MURCS association usually occurs sporadically, as in this case, a familial association is occasionally present. In some cases the MURCS association may be a genetically determined pleiotropic condition.

Abnormalities, Multiple↗
PubMed1985

Diffuse, multicentric neurogenic tumors in two macerated fetuses: a possible intrauterine form of neurofibromatosis.

Two tiny macerated fetuses with a remarkably similar pattern of multicentric neurogenic neoplasms of both paravertebral autonomic structures and peripheral nerves are described. Maceration precluded further histologic classification of the neoplasms in either fetus. The first fetus had a Meckel's diverticulum, short attachment of the small bowel mesentery, pulmonary hypoplasia, and intrauterine growth retardation. The second had the sympus bipus variant of sirenomelia sequence. The multicentric neoplasms in these two fetuses are very similar to those previously described in a few neonates and one stillborn with well-documented or suspected neurofibromatosis. It is reasonable to hypothesize that these two fetuses may represent an early intrauterine expression of neurofibromatosis characterized by multicentric neurogenic neoplasms of autonomic structures and peripheral nerves. Detailed examination of early abortuses, especially those from families with neurofibromatosis, may help to confirm or disprove the hypothesis.

Autonomic Nervous System Diseases↗
PubMed1984

Quantitative assessment of growth and function of the cardiac chambers in the normal human fetus: a prospective longitudinal echocardiographic study.

We assessed the changes in cardiac chamber size, architecture and function in the normal fetus in a prospective, longitudinal, two-dimensional, and two-dimensionally directed M mode echocardiographic study. Serial echocardiograms were recorded in 16 normal fetuses at 4 week intervals from 20 weeks gestation to parturition. Fetal gestational age was assessed by biparietal diameter. Left ventricular, right ventricular, and left atrial chamber sizes and aortic diameter all increased linearly with age. The ratios of right and left ventricular diameter, left atrial to aortic diameters, and relative left ventricular wall thickness that we used as an index of short-axis left ventricular architecture remained constant. Fractional right ventricular and left ventricular wall thicknesses were similar both on echocardiograms and in postmortem hearts over the same range of gestational ages. In addition, postmortem right ventricular and left ventricular free wall weights were indistinguishable and contributed the same proportion to total heart weight throughout gestation. Left ventricular echocardiographic mass increased linearly from a mean of 0.86 +/- 0.09 to 7.47 +/- 2.43 g at term and corresponded closely with postmortem left ventricular weight. We conclude that (1) fetal cardiac chamber dimensions, wall thicknesses, and left ventricular mass increased with gestational age, (2) cardiac architecture in terms of the ratios of right ventricular/left ventricular diameters, left atrial/aortic diameters, and relative wall thickness remained constant, (3) right and left ventricular fractional shortening did not change with age, (4) left ventricular mass assessed echocardiographically corresponded closely with postmortem left ventricular weights in fetal hearts of similar gestational ages, and (5) the similarities between right and left ventricular sizes, wall thicknesses, and free wall weights in this study do not support the theory of right ventricular dominance in the human fetus.

Echocardiography↗
PubMed1984

Quantitative assessment of right and left ventricular growth in the human fetal heart: a pathoanatomic study.

We quantitated the growth patterns of the normal fetal heart and the right and left ventricles from postmortem hearts obtained from 55 spontaneously aborted human fetuses from the completion of cardiogenesis to term. Fetal gestational age was assessed by menstrual history of the mother, crown-rump length, head circumference, and body weight and ranged from 8 to 40 weeks. Each heart was perfused and fixed at constant pressure and dissected to obtain right and left ventricular free wall, left ventricular, and total heart weights. Right and left ventricular free wall thicknesses were measured and the respective surface areas were calculated. The changes in each of these parameters with gestational age were examined by regression analysis. Total heart and right and left ventricular wall weights increased linearly with body weight, but exponentially with head circumference, crown-rump length, and menstrual history. Right and left ventricular free wall weights were similar throughout gestation and the percent that each contributed to total heart weight were constant at 29 +/- 2% and 30 +/- 2%, respectively. Right and left ventricular wall thicknesses did not differ significantly, increasing linearly with menstrual age, crown-rump length, head circumference, and body weight from 8 to 40 weeks. The surface areas of the right and left ventricular free walls that we used as an index of changing ventricular architecture were indistinguishable throughout the period of gestation studied.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1984

Case 4. The first fatal Baylisascaris infection in humans: an infant with eosinophilic meningoencephalitis.

Baylisascaris procyonis, the ascarid of raccoons, causes a characteristic, rapidly fatal eosinophilic meningoencephalitis with ocular involvement in many naturally and experimentally infected aberrant hosts, including monkeys. Warnings that humans are potentially susceptible to the devastating infection have been issued, but an instance in humans has not been recognized. This report describes a boy who died from an eosinophilic meningoencephalitis, which mimicked B. procyonis infection in monkeys. The causative agent was not identified during life. Autopsy showed a systemic larval ascarid infection with massive involvement of the brain. The size and anatomy of the larvae in histologic sections were identical to those recorded for B. procyonis. The larvae were indistinguishable from the B. procyonis larvae observed in histologic sections of experimentally infected monkeys. An indirect immunofluorescence test was positive for B. procyonis. Exposure to raccoon feces was highly likely. The evidence suggests that this is the first recognized B. procyonis infection in humans. Prudent avoidance of exposure to raccoon feces is indicated.

Animals↗
PubMed1983

Veno-occlusive disease of the liver in children following chemotherapy for acute myelocytic leukemia.

Three children developed acute veno-occlusive disease of the liver following combination chemotherapy for acute myelocytic leukemia. The clinical presentation was similar in all three, with acute onset of hepatomegaly and thrombocytopenia in the absence of significant transaminasemia or icterus. In all three patients, radionuclide imaging with technetium-99m sulfur colloid showed hepatosplenomegaly, decreased liver uptake, and increased splenic activity. The results of liver biopsy established the diagnosis, revealing marked centrilobular congestion with hemorrhage into the spaces of Disse, atrophy of central hepatic cords, and edema of the walls of the central and sublobular veins. Each patient showed marked improvement following temporary cessation of chemotherapy. The diagnosis of veno-occlusive disease is suggested by the triad of: (1) clinical signs and symptoms; (2) scintigraphic findings; and (3) temporal relationship to chemotherapy.

Adolescent↗