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Biomedical subjects

S Gross

Publications and source records attributed to S Gross.

At least 199 records · Page 11Linked to original sources
PubMed1983

Severe congenital hypoplastic anemia. Transmission from a healthy female to opposite sex step-siblings.

Congenital hypoplastic anemia (CHA) is a rare entity characterized by selective depression of erythropoiesis. The disease was first described in 1938, in four patients reported by Diamond and Blackfan, followed by a number of longitudinal studies and reviews of approximately 175 well-documented cases and/or unpublished records. The typical patient presents in infancy (median 1 month of age) with pallor. In as many as 30% of the patients there is an inconsistent pattern of congenital anomalies of the heart, integument, musculoskeletal system, and kidneys. Although its congenital nature is firmly established, the pathophysiology is still unknown, and it is likely that there are multiple biochemical or cellular defects producing the same clinical syndrome. That the disease is genetically determined is suggested by some cases accompanied by congenital anomalies, by racial differences--only a few blacks and one Asian have been described--and by multiple occurrences in some families. The number of such families is small, and the mode of inheritance appears inconsistent. The purpose of this report is to emphasize the apparently variable genetics of a disease which is undoubtedly variable in its clinical expression. We describe the unique occurrence of CHA in half-siblings of opposite sex, both with moderately severe disease, transmitted by a healthy female.

Adult↗
PubMed1982

Ischemic heart disease in systemic lupus erythematosus in the young patient: report of six cases.

To clarify the clinical spectrum of coronary arterial abnormalities in systemic lupus erythematosus, the data were reviewed on six patients who had a diagnosis of lupus at ages 15 to 29 years and who had ischemic heart disease before age 35. Two patients had coronary arteritis diagnosed on postmortem examination. In a third patient alterations in coronary arterial anatomy occurred with angiographic improvement temporally related to the initiation of steroid therapy. The other three patients had severe diffuse atherosclerotic coronary disease that was identified in two at postmortem examination. In the third patient the course of the disease strongly suggested coronary atherosclerosis, and eventually coronary bypass grafting was performed for relief of angina. In summary, clinically important extramural coronary arteritis and atherosclerosis both occur, although rarely, in young patients with lupus. Coronary artery disease may occur with or without coexisting active extracardiac lupus manifestations. Short-term steroid therapy and follow-up angiography for those with angina and in whom coronary arteritis is suspected warrant consideration. When stable coronary arterial anatomy is demonstrated on follow-up angiography, management is determined by the patient's symptoms irrespective of the prior history of lupus and, if indicated, cardiac surgery for symptomatic relief can be safely performed.

Adolescent↗
PubMed1982

Bone marrow transplantation with high-dose cytosine arabinoside.

Eleven patients received allogeneic bone marrow from all HCA-identical sibling for the treatment of acute leukemia. The preparative regiment included high-dose cytosine arabinoside (Ara-C), 3 g/m2q 12 h for 12 doses, and 1000 rad single-fraction total body irradiation (5 patients) or 1200 rad fractionated total body irradiation (6 patients). The 5 patients receiving the 1000 rad single-fraction regimen were transplanted in relapse, while the 6 patients receiving the 1200 rad fractionated program were transplanted during remission. Both regimens were well-tolerated, but the single-dose radiation regimen was associated with more severe skin problems. Bone marrow engraftment was documented in 10 of 10 evaluable patients, occurring 2-3 weeks after marrow infusion. No recurrences have been observed during the brief follow-up time.

Bone Marrow Transplantation↗
PubMed1982

Adenosine deaminase positive, E receptor negative lymphoma.

The measurement of enzymatic activity in the blast cell is a recent investigational technique which seems to complement immunologic measurement of cell membrane receptors in childhood lymphoproliferative malignancies. We have identified a patient with non-Hodgkin's lymphoma, not readily classifiable on the basis of either morphology or cell surface markers, i.e., E rosette and SIg negative, EAC positive. Analysis of enzymatic function, however, revealed markedly elevated ADA activity and essentially normal TdT activity. ADA positive lymphoma can arise from a primitive T-cell line. It is, thus, apparent that the morphological description of malignant lymphoid lines must be accompanied by surface marker and enzymatic data in order to properly classify cell lineage and identify appropriate therapeutic modalities.

Adenosine Deaminase↗
PubMed1981

Lymphocyte aromatic hydrocarbon responsiveness in acute leukemia of childhood.

Aryl hydrocarbon hydroxylase (AHH) activity and inducibility were examined in mitogen-stimulated cultured lymphocytes from children with acute leukemia in remission, with nonleukemic malignancies, and with no family or personal history of malignant disease. Neither morphological differences nor differences in mitogen responsiveness were observed among the three sources of cells studied. Levels of constitutive and dibenzanthracene-induced AHH activity were found to be similar among the three groups by analysis of variance. However, when results were analyzed in terms of inducibility ratios, it was found that cells from leukemic children were significantly less inducible (p less than 0.005) than cells from unaffected children or children with nonleukemic malignancies. The reason for this difference became apparent when statistical criteria were employed for the pheontypic separation of individuals who were highly aromatic hydrocarbon responsive and minimally responsive. A significantly larger proportion (p less than 0.001) of leukemic children than unaffected children or children with nonleukemic malignancy were found to be minimally aromatic hydrocarbon responsive. Moreover, in patients with acute lymphoblastic leukemia relapsing while on therapy, longer durations of the first remission were correlated (r = 0.63, p less than 0.05) with the highly inducible AHH phenotype.

Acute Disease↗
PubMed1981

Chronic ITP terminating in aplastic anemia.

A 14-year-old white male developed gastric anemia with normal chromosomes following a 6-year history of chronic ITP refractory to splenectomy. The disorder was partially responsive to oxymethalone which was discontinued because of hepatotoxicity. Antiplatelet and antigranulocyte antibodies were identified in the patient's sera. Absorbed sera suppressed normal marrow CFU-C and CFU-E. This case may represent a single-entity autoimmune mechanism characterized initially by thrombocytopenia followed by pancytopenia.

Adolescent↗
PubMed1981

Sibling groups. Interaction with siblings of oncology patients.

Siblings of pediatric oncology patients in active therapy met as a group for 5 consecutive weeks with two oncology team members. The parents met simultaneously in another location in the hospital. The goals were to provide (1) a support group for the siblings, (2) information regarding the various treatment regimens, and (3) specific knowledge regarding the particular stresses of specific therapy programs. The following problem areas were identified: (1) reiteration of resentment toward the patient due to the amount of attention focused on them by family and friends, (2) embarrassment in talking about the patient's illness, and (3) the relationship between the parent's willingness to discuss the patient's illness at home and the sibling's comprehension of the illness.

Child↗
PubMed1981

Prevention of umbilical artery catheter clots with heparinized infusates.

49 neonates requiring umbilical artery catheters (UACs) were randomly assigned to receive standard or heparin-containing infusates. 3 of 23 (13%) of the patients receiving heparin had catheters removed because they became functionally occluded compared to 15 of 26 (58%) in the control group (p less than 0.005). 4 of 13 (31%) single injection aortograms obtained in control infants demonstrated thrombi, compared to none of 7 in the heparin group. 1 patient in the heparin group had an aortic clot demonstrated at post-mortum examination. There were neither clinical coagulopathies nor abnormalities of partial thromboplastin time attributable to the administration of heparinized fluids. Heparinization of UAC infusates appears to be a safe method of reducing the risk of catheter occlusion. Heparin effect on large vessel clot risk remains unproven.

Blood Coagulation Disorders↗
PubMed1980

The altered baroreceptor response to nitroglycerin in congestive heart failure.

The use of nitroglycerin as a vasodilator in the treatment of congestive heart failure has demonstrated that the hemodynamic response is related to the degree of left ventricular dysfunction. Little emphasis has been placed on the mechanisms responsible for the variability in heart rate response to nitroglycerin. To study this problem, 33 patients with a variety of chronic cardiac disorders were given 0.4 mg of nitroglycerin sublingually. An increase in heart rate of less than 3 beats/min to sublingual nitroglycerin allowed a clear identification of a group of patients who had a left ventricular end-diastolic pressure greater than 18 mmHg or pulmonary artery mean pressure greater than 22 mmHg, and who had improvement in hemodynamic parameters following nitroglycerin. This alteration in baroreceptor sensitivity appears to be related to the level of left ventricular filling pressure, and can be used as a simple bedside means of identifying a group of patients who benefit the most from the vasodilator action of nitroglycerin.

Female↗
PubMed1980

Analysis of auditory nerve-brainstem responses (ABR) in neonates and very young infants.

Auditory nerve-brainstem responses (ABR) to click stimuli were recorded in 26 normal infants when they were neonates and again in the same infants when they were 3 months old. The cochlear microphonic potential was also recorded in several of the neonates. The following response parameters were studied: latency and amplitude of the auditory nerve response, amplitude of the brainstem response elicited from the region of the interior colliculus, the ratio of the amplitudes of these two waves and brainstem transmission time (BTT): the time interval between these two responses. It was found that the auditory nerve response is only slightly prolonged in neonates with respect to adults and shortens by a small amount from birth to the age of 3 months. The latency of the auditory nerve response in neonates is only about 0.3 ms longer than in adults. BTT is longest in neonates and shortens significantly in 3-month-old infants.

Acoustic Stimulation↗
PubMed1980

Folate compartments during gestational maturation.

Studies on 41 healthy preterm infants and 53 term infants revealed that almost 70% of the total folate is present in the bound form. Beyond the enonatal period the increase in bound folate with increasing gestational maturity reflects fetal demands placed on folate transport and affords an explanation for the loss of folate and corresponding increase in unsaturated binder during pregnancy. With the knowledge of folate biology gained from radioligand data, it is reasonable to postulate that effective folate metabolism depends upon a metabolically active transport mechanism.

Biological Transport↗
PubMed1980

The role of eosinophils in granulopoiesis. I. Eosinophilia in neutropenic patients.

The role of eosinophils in granulopoiesis is unclear. In a variety of conditions, the level of eosinophils is inversely related to the level of neutrophils. The present report describes two patients with eosinophilia and neutropenia and examines the in vitro effects of eosinophils on the colony formation of their bone marrow in semisolid culture medium. The addition of autologous eosinophils to bone marrow cultures from these patients resulted in a decrease in the number of colonies; in contrast, antieosinophilic serum increased the number of colony-forming units. Indomethacin, an inhibitor of prostaglandin, was capable of reversing the effects of added eosinophils. The findings suggest that eosinophils have an inhibitory effect on in vitro granulopoiesis. The suppressive effects may be due to the high content of prostaglandin E found in eosinophils. A schematic diagram based on the current knowledge of the mechanism of granulopoiesis is presented.

Aged↗