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Biomedical subjects

B Goldman

Publications and source records attributed to B Goldman.

At least 109 records · Page 6Linked to original sources
PubMed1993

X-linked recessive primary retinal dysplasia is linked to the Norrie disease locus.

X-linked primary retinal dysplasia (PRD) refers to an abnormal proliferation of retinal tissue causing either its neural elements or its glial tissue to form folds, giving rise to gliosis. A Jewish family of oriental origin was previously reported by Godel and Goodman, in which a total of five males suffer from different degrees of blindness. The authors postulated that the described findings are distinguished from Norrie disease, since in this case no clinical findings, other than those associated with the eyes, were noticed in the affected males. In addition, two of the carrier females exhibit minimal eye changes. We have performed linkage analysis of the family using the L1.28, p58-1 and m27 beta probes, and DXS426 and MAOB associated microsatellites. Our results map the gene responsible for the disorder between the MAOB and DXS426, m27 beta and p58-1 loci, on the short arm of the X chromosome at Xp11.3, which suggest the possibility that the same gene is responsible for both primary retinal dysplasia and Norrie disease.

Blindness↗
PubMed1993

Improving access to the underserved through Medicaid managed care.

New York City's Harlem community faces extraordinary health care needs that a failing economy has made more urgent. In an attempt to open lines of access to health care, the New York City Health and Hospitals Corporation has implemented a managed care program for Medicaid participants. The program is a team approach to link each Medicaid patient with a primary care physician responsible for coordinating medical services for the patient. We hope the managed care program will help us conserve our dwindling resources while better managing patients' services and improving access to care.

Adolescent↗
PubMed1993

Use and abuse of opioid analgesics in chronic pain.

Primary care physicians are frequently required to treat patients with chronic debilitating pain. Opioid analgesics can successfully manage chronic pain. To prescribe opioid analgesics effectively, physicians must identify appropriate patients. Several methods can be used to identify and distinguish appropriate patients, addicted patients, and for-profit drug seekers.

Analgesics, Opioid↗
PubMed1993

Aortic dissection in cardiac allograft recipients. A report of two cases.

Aortic dissection in cardiac allograft recipients has not been reported previously, to our knowledge. We have observed two fatal aortic dissections in heart transplant recipients: one in the immediate postoperative period and one occurring 5 years after transplantation. We discuss the clinical presentations, pathologic findings, possible pathogenetic mechanisms, and risk factors. Because the frequency of aortic dissection after heart transplantation appears to be relatively low (< 1%), such disease may be clinically unsuspected. Given the uniformly poor outcome observed in our two patients, we suggest that increased awareness of the possible development of aortic dissection after heart transplantation may result in improved survival of affected patients.

Aortic Dissection↗
PubMed1993

Prenatal and postnatal studies of a late infantile GM2 gangliosidosis in a family of Syrian origin: a possible B1 variant.

We describe late infantile Tay-Sachs disease with high residual hexosaminidase A activity in two siblings of a Syrian Druze family. The patients' leukocytes had 26% of normal hexosaminidase A activity when tested with the conventional fluorogenic substrate 4-methyl-umbelliferyl-2-acetamido-2-deoxy-beta-D-glucopyranoside (4-MUG) and only about 10% when assayed with the sulfated substrate, 4-methyl-umbelliferal- beta-N-acetyl-glucosamine-6-sulfate (4-MUGS). According to the standard procedure of the heterozygote screening program (employing 4-MUG and heat inactivation), the parents were not diagnosed as an at-risk couple since the father was classified as a noncarrier. However, both parents' levels were clearly within the carrier range on the basis of 4-MUGS. The unique catalytic characteristics of the patients' enzyme forward the assumption that the affected sibs are B1 variants. The parents' enzymatic levels, together with their known consanguinity, might indicate that these patients are homozygotes for the rare mutation and not genetic compounds as has been documented for most of the infantile B1 variants. To the best of our knowledge this is the first reported case of B1 variant in a child of that extraction.

Female↗
PubMed1992

Rhythms of enzymatic activity in maternal and umbilical cord blood.

The 24-h activity patterns of various enzymes were determined in human serum, red blood cells and white blood cells of maternal and umbilical cord blood. Blood was drawn from the brachial vein of mothers and from the umbilical cord within ten minutes after delivery. Corresponding blood specimens were obtained from 83 spontaneous labors, occurring at different hours over a period of 60 days. For each variable (variable = activity of a specific enzyme in one of the blood components) the results were grouped according to delivery hour, forming a 24-h pattern which was analyzed to elucidate time dependency. Five out of six corresponding maternal and fetal variables were similar with regard to pattern and peak time. The activity rhythms of glyceraldehyde-3-phosphate dehydrogenase and glucose phosphate isomerase in red blood cells of mothers and fetuses possessed a significant bimodal pattern. The activity rhythms of the latter enzyme in white blood cells and sera exhibited a significant 24-h period. Hexosaminidase activity exhibited a distinct 24-h rhythm in maternal white blood cells, but no significant rhythm could be detected in the fetal white blood cells. The activity of hexosaminidase showed, identical 24-h patterns in maternal and cord serum when analyzed by best fit cosine, and no significant time-dependency when analyzed by ANOVA.

Circadian Rhythm↗
PubMed1992

The effect of thyroxine and corticosteroids upon amniotic fluid fluorescence polarization: a randomized controlled study.

The efficacy of glucocorticoid and intra amniotic thyroxine therapy on fetal lung maturity were compared in a randomized prospective clinical trial. Eighty two patients received either intra amniotic thyroxine (n = 36), or intra muscular Dexamethasone (n = 46). The Fluorescence Polarization (FP) of amniotic fluid measured prior to and one week following treatment were similar in the two groups. The rate of decrease in FP value per unit of time (dFP/dt) was identical in the T4 and corticosteroid treated patients and the proportion of immature FP values obtained was similar in the two groups. The effect of thyroxine therapy was more pronounced in pregnancies above 33 weeks gestation. Intra amniotic thyroxine therapy appeared to be as effective as glucocorticoids for the enhancement of fetal lung maturity. This therapeutic modality may be of particular use on pregnant subjects in whom a relative contra indication for the administration of steroids exists.

Amniotic Fluid↗
PubMed1992

Ritodrine therapy in the presence of chronic abruptio placentae.

BACKGROUND: Betamimetic therapy is usually contraindicated for the treatment of premature labor associated with abruptio placentae. We report prolongation of a pregnancy for 7 weeks using ritodrine despite the presence of placental abruption. CASE: A 33-year-old primigravid woman presented at 25 weeks' gestation with irregular uterine contractions, vaginal bleeding, and sonographic evidence of abruptio placentae. Port wine-colored amniotic fluid was found during amniocentesis, and serial hematocrits decreased from 36 to 25%. A diagnosis of abruptio placentae was made, and because the maternal cardiovascular and fetal biophysical indices were normal, tocolytic therapy was started. Before the administration of ritodrine, the patient and her husband were given an extensive review of the risks, including blood transfusion, adult respiratory distress syndrome, disseminated intravascular coagulopathy, and maternal or fetal death. CONCLUSION: Although clinical suspicion of abruptio placentae remains a contraindication to betamimetic therapy, exceptions may be made if fetal and maternal well-being can be monitored and if a fully staffed operating room is always available for immediate cesarean delivery. The benefits of this management may outweigh the associated risks for carefully chosen, very preterm gestations.

Abruptio Placentae↗
PubMed1991

Concordance of auriculo-cardiovascular-skeletal malformations: a new malformation complex in an infant of a diabetic mother?

The incidence of malformations among infants of diabetic mothers (IDM) is known to be higher than in the general population. These malformations usually involve several organ systems and in the past few years there has been an attempt to group them into distinct "syndromes." The present report concerns a child with a specific constellation of findings not yet reported in the infant of a diabetic mother, and we urge our colleagues to look for other associations existing in these infants, rather than just listing series of individual malformations.

Abnormalities, Multiple↗
PubMed1991

Human chorionic gonadotrophin and trisomy 18.

Estimation of maternal serum beta-hCG is used in conjunction with alpha-fetoprotein (AFP) and estriol (E3) for estimating the risk of Down syndrome (DS) affected fetuses. However, low hCG levels have not been regarded as having clinical significance. We report on 2 patients with trisomy 18 fetuses in whom antenatal screening showed extremely low hCG levels (0.05 and 0.15 MOM). Low hCG levels might indicate increased risk for trisomy 18 despite low estimated risk for DS.

Adult↗