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Biomedical subjects

H B Robinson

Publications and source records attributed to H B Robinson.

At least 19 recordsLinked to original sources
PubMed1994

Coincident DiGeorge anomaly and renal agenesis and its relation to maternal diabetes.

We describe 2 cases of DiGeorge anomaly with bilateral renal agenesis-one, who also had hemivertebrae, in an infant of an insulin-dependent diabetic mother (IDDM). In a review we identified 2 other instances of this combination, both in IDDMs. The currently accepted notion that DiGeorge anomaly and renal agenesis are developmental field defects, coupled with their coincidence in IDDM, suggests that this combination is an association.

Abnormalities, Multiple↗
PubMed1992

Adverse outcome in pregnancy following amniotic fluid isolation of Ureaplasma urealyticum.

Infections in pregnancy with Ureaplasma urealyticum have been associated with a wide range of adverse outcomes, such as early abortion, stillbirth, prematurity, and neonatal morbidity and mortality. Causality has been difficult to demonstrate secondary to the high prevalence of asymptomatic lower genital tract (LGT) colonization and culture data from inaccessible or potentially contaminated sites. Between 1985 and 1989, 2461 second-trimester genetic amniocenteses were evaluated at the cytogenetics section of the Children's Hospital Medical Center of Akron. All were cultured for the genital mycoplasmas: Mycoplasma hominis and Ureaplasma urealyticum. A total of nine patients were positive, all for Ureaplasma urealyticum, with one patient excluded because of subsequent therapeutic abortion. In addition, complete follow-up data, such as indication for amniocentesis, serum alpha-fetoprotein levels, gestational age at parturition, and outcome of pregnancy, were available on 86 Ureaplasma-negative (U-) patients during an approximate 2-year span within the time-frame of the study. This was in part due to physician response to a questionnaire sent after amniocentesis. Of the eight positive cultures, 100 per cent were associated with an adverse outcome, defined as fetal loss or premature delivery. This was significant compared with the U- group (p less than 0.001) with a more than eight times greater risk of adverse outcome. Six (75 per cent) resulted in spontaneous miscarriage within 4 weeks of amniocentesis and at less than 21 weeks' gestation. Two (25 per cent) delivered prematurely, with one (12.5 per cent) neonatal death at 24+ weeks. Histological examination of all eight placentae and the seven fetuses revealed a 100 per cent incidence of chorioamnionitis and pneumonia, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Abortion, Spontaneous↗
PubMed1992

Standardized residuals as a means for detection of growth alteration in the pathologic human fetus.

This paper introduces and discusses the use of standardized residuals as a technique for comparing the growth of normal and pathologic human fetuses. Anthropometric measures, radiographic measures, and organ weights were regressed on known gestational age of second- and third-trimester fetuses. Standardized residuals were calculated for a group of potentially growth-impaired fetuses. Use of residuals aids in identification of patterns of growth alteration in specific pathologies. Most important, studying the response of developing organ systems to a variety of insults may elucidate mechanisms of growth regulation in the fetus. We emphasize the special quality of the multivariate measures of the core sample of fetuses from the Akron Children's Hospital collection.

Anthropometry↗
PubMed1990

Placental emboli from a fetus papyraceous.

A syndrome in monozygotic twins that consists of a macerated twin fetus (fetus papyraceous) and a live-born twin with various anatomical defects has been described. The etiology is thought to be placental transfer of emboli or thromboplastic material through vascular shunts. Thromboplastic material precipitates disseminated intravascular coagulation (DIC) in the fetus, with a resultant hypercoagulable state due to relative fetal antithrombin III deficiency. Two cases of this syndrome will be discussed. The case of a live-born twin with intestinal atresia, who developed in utero with a fetus papyraceous, is reported. Emboli were demonstrated in vascular shunts of the diamniotic-monochorionic placenta. The hypothesis of intestinal atresia as a result of a vascular accident is reviewed. Another case involving a live-born twin with congenital skin defects, who developed in utero with a fetus papyraceous, is also reported. The skin defects were a congenital disruption from fetal DIC with resultant hypercoagulable state. Several other manifestations of the placental emboli syndrome will be discussed and the vascular etiology of the disruptions explained.

Adult↗
PubMed1986

Lipoprotein disorder, cirrhosis, and olivopontocerebellar degeneration in two siblings.

Two siblings had olivopontocerebellar degeneration, failure to thrive, hepatic fatty change and cirrhosis, and a dyslipoproteinemia characterized by low cholesterol and elevated triglycerides. This condition was distinct from other cerebellar atrophies and ataxias and was not due to malabsorption or malnutrition. Cerebellar degeneration progressed rapidly during the first year of life, and both children died from intercurrent infections and surgical complications at 11 and 17 months. Stereotyped clinical and pathologic findings in the two patients suggest a previously unreported genetic metabolic disorder affecting the liver and the CNS.

Atrophy↗
PubMed1984

Agenesis of the cloacal membrane. A probable teratogenic anomaly.

Five infants with a rare and distinct malformation complex were encountered in a single community within a 7 1/2-month period. Only seven previous reports of this condition were found in the 54-year period between 1926 and 1980. The principal findings in the previously published cases were absence of external genitalia, urinary, genital, and anal orifices, and persistence of the cloaca. This report documents the occurrence of the syndrome in a temporal and regional cluster. Detailed morphologic evaluation of each infant provides the basis for a theory of embryogenesis of the complex, and preliminary data suggest a teratogenic cause. Embryonic exposure to doxylamine succinate within the first 50 days of the pregnancy was certain in three and was probable in two of the five pregnancies.

Abnormalities, Drug-Induced↗
PubMed1981

Screening for the Tay-Sachs carrier: a compromise program.

A modified program designed to screen for the Tay-Sachs carrier is presented in which testing is limited to one or both partners, as needed, in an Ashkenazi Jewish (Jews of central and eastern Europe ancestry) mating when there is a definite commitment toward having a child, or in the early stages of the pregnancy. Testing of unmarried individuals is discouraged. The approach maximizes individualization of both the medical and laboratory aspects of the program and promotes a positive and beneficial relationship between physician and clinical chemist. There is little involvement of the lay population or clergy, and no special sources of funding are required. Whereas most mass screening programs for the Tay-Sachs carrier have attempted to educate large numbers of the lay public to bring pressure upon the medical community, we have reversed this approach and taken the much easier course of educating small numbers of physicians to better care for their patients. The program has been used successfully in a moderate-size city in which communication lines between laboratory and physician are easily established.

Communication↗
PubMed1980

Apparent normal leukocyte acid maltase activity in glycogen storage disease type II (Pompe's disease).

We present a case of glycogen storage disease type II (Pompe's disease) with the classical clinical presentation and characteristic electrocardiographic changes of this disorder. An acid maltase (EC 3.2.1.20) determination in the peripheral leukocytes revealed normal activity; however, acid maltase activity was completely absent in a pre-mortem skeletal muscle biopsy. Post-mortem studies showed acid maltase activity to be absent in all tissues examined, including cultured skin fibroblasts. Massive glycogen deposition corresponded to the localization of the enzymic deficiency, except in the brain, where glycogen content was within the normal range. The acid maltase activity in mixed peripheral leukocytes was due to an isoenzyme of acid maltase in the granulocyte series. Antenatal diagnosis was accurate in a subsequent pregnancy, but discordance between enzyme activity in different cell lines in an individual with a genetic disease is a conceivable source of error in both prenatal and postnatal diagnoses.

Female↗
PubMed1979

Internal vascular access for hemodialysis in children weighing less than fifteen kilograms.

An arteroarterial femoral graft using expanded polytetrafluoroethylene is described which has been successfully for vascular access in young children having small peripheral vessels. This graft allows high flow and favorable patency for dialysis without the complications of arteriovenous shunting or the risks associated with an external hemodialysis device. This graft has been used successfully for outpatient dialysis in children weighing as little as 9 kg and may be a useful adjunct in long-term dialysis of patients for whom more conventional means of vascular access are not acceptable.

Blood Vessel Prosthesis↗
PubMed1978

Bilateral congenital subdural cysts associated with porencephaly and CSF-subdural fistula. A report of two cases.

Tow infants had bilateral congenital subdural cysts associated with encephaloclastic porencephaly and a CSF-subdural fistula. The cysts occupied 80% to 90% of the crainal cavity. There was severe atrophy and malformation of the underlying brain, suggesting that its development had been disturbed at an early stage of gestation. The clinical and radiologic findings were indistinguishable from those of hydranencephaly.

Brain↗
PubMed1978

Discussion and management of late failures in reconstructive procedures involving the abdominal aorta.

Review of fifty-eight late failures of 326 procedures performed for revascularization of the abdominal aorta over the past six years showed a low overall operative mortality of 9 per cent. Secondary vascular procedures directed at the aorta itself or at its graft substitute proved more effective in relieving symptoms and restoring flow than did secondary procedures directed at more distal problems in the lower extremities. The aortofemoral graft in conjunction with profundaplasty proved to be the most effective means of restoring flow to the lower extermity after graft occlusion in a previous aortoiliac or aortofemoral graft.

Adult↗