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Biomedical subjects

R Hong

Publications and source records attributed to R Hong.

At least 127 records · Page 7Linked to original sources
PubMed1982

Spontaneous recovery of selective IgA deficiency. Additional case reports and a review.

Patients with selective IgA deficiency, the most common primary immunodeficiency, occasionally may undergo spontaneous recovery. In this paper we present three additional children whose IgA levels spontaneously returned to normal. All three presented with gastrointestinal symptoms, and one child also had frequent upper respiratory infections. We review other cases of spontaneous IgA deficiency, and the role of exogenous function in its etiology, and possible mechanisms of recovery.

Child, Preschool↗
PubMed1982

Diminished reactivity to alloantigen following transplantation of cultured thymic fragments.

Transplantation of allogeneic thymus into thymic deficient individuals will restore T-cell function including ability to demonstrate alloreactivity. In allogeneically reconstituted athymic (nude) mice, alloreactivity as manifested by positive mixed leukocyte reactivity, cell mediated lympholysis and skin graft rejection is present for all alloantigens, save those of the thymus donor. Such reconstituted animals possess double tolerance, for self and for donor. Cultured thymic fragments have been used to correct the immunodeficiency of thymic deficient humans. In a patient with severe combined immunodeficiency who acquired T-killer activity after allogeneic cultured thymic fragment transplant, mixed leukocyte response to the thymus donor was nearly absent. Patients with cancer of the lung, although not profoundly T deficient, received cultured thymic fragments in a project designed to enhance their immunity. Diminished alloreactivity for donor cells was seen in 2 of 3 such patients.

Chromosome Deletion↗
PubMed1982

Transplantation of cultured thymus fragments. III. Induction of allotolerance.

Balb/c nude mice were transplanted with cultured thymic fragments from C57BL/6, hybrid (C57BL/6xC3H F1 or BALB/cxC3H F1) or 2 separate strains simultaneously. Tolerance to H-2 of the thymus donor strain(s) was assessed by skin-graft acceptance and was seen in all cases except where 2 thymuses, each of differing strains, were transplanted simultaneously. Examination of mixed leukocyte culture and cell mediated lympholysis revealed blunted reactivity against thymus donor strains when skin graft tolerance was seen. Demonstration of a suppressor cell population was unsuccessful although a decreased cytotoxic T-cell potential was suggested by decreased GVH activity. These studies showed a unique dependence upon the thymus gland for the phenomenon demonstrated and are taken to be an example of a more generalized process in which effector cell function of the thymus is controlled by intrathymic events during the early maturation of precursor effector cells.

Animals↗
PubMed1981

Biotin-responsive in vivo carboxylase deficiency in two siblings with secretory diarrhea receiving total parenteral nutrition.

Two siblings with a congenital syndrome of secretory diarrhea and seizures developed progressive skin rash, alopecia, and mucocutaneous candidiasis while receiving biotin-free total parenteral nutrition. Abnormally low urinary biotin excretion was associated with these clinical findings, but the serum concentration of biotin was within the normal range. There was also increased urinary excretion of lactic acid, 3-hydroxyisovaleric acid, 3-hydroxypropionic acid, and 3-methylcrotonylglycine. The younger of the two children subsequently died with severe metabolic acidosis. In the oder sibling, intravenous treatment with biotin (200 micrograms/day) resulted in resolution of the organic aciduria. A larger dose (10 mg/day) appeared to be required for rapid improvement in the skin lesions. These cases suggest that clinically significant biotin deficiency can occur in patients with chronic diarrhea receiving biotin-free total parenteral nutrition.

Biotin↗
PubMed1981

Recategorizing childhood acute lymphoblastic leukemia with monoclonal antibodies to human T cells.

The lymphoblasts of three patients with childhood acute lymphoblastic leukemia (ALL) were analyzed for their immunologic surface markers. Blasts from two of these patients did not form rosettes with sheep erythrocytes and the third did so marginally, suggesting these patients had non-T-cell leukemia. These blasts were also tested with monoclonal antibodies that detect thymocyte differentiation markers, and all three patients were highly reactive with at least two of these reagents. We anticipate the availability of multiple standardized monoclonal reagents will necessitate a recategorization of ALL phenotypes. Some of these leukemic phenotypes may not correspond to normal stages of lymphoid differentiation. Therefore, we suggest that it may be inappropriate to attempt to identify and categorize leukemic cells by the pathways of normal differentiation.

Adolescent↗
PubMed1981

The cerebro-hepato-renal syndrome of Zellweger: similarity to and differentiation from the DiGeorge syndrome.

A child with the cerebro-hepato-renal syndrome of Zellweger, who was originally diagnosed as having the DiGeorge syndrome, was studied and transplanted unsuccessfully with cultured thymus. The pertinent literature is reviewed and the importance of distinguishing the two disorders emphasized. Autopsy studies reveal that transplanted cultured thymic fragments can attract lymphoid aggregates as early as 2 wk after transplantation.

Abnormalities, Multiple↗
PubMed1981

Thymic dysfunction in chronic lymphocytic leukemia.

Chronic lymphocytic leukemia (CLL) is, in the great majority of cases, a neoplastic proliferation of B cells. The associated immunologic dysfunction accounts for many of the clinically associated phenomenon such as infection and second malignancies. Much of this dysfunction has been attributed to poor B-cell function. Functional impairment of the T-cell component is currently being elucidated. In the investigation of B cells. The associated immunologic dysfunction accounts for many of the clinically associated phenomenon such as infection and second malignancies. Much of this dysfunction has been attributed to poor B-cell function. Functional impairment of the T-cell component is currently being elucidated. In the investigation of B cells. The associated immunologic dysfunction accounts for many of the clinically associated phenomenon such as infection and second malignancies. Much of this dysfunction has been attributed to poor B-cell function. Functional impairment of the T-cell component is currently being elucidated. In the investigation of B cells. The associated immunologic dysfunction accounts for many of the clinically associated phenomenon such as infection and second malignancies. Much of this dysfunction has been attributed to poor B-cell function. Functional impairment of the T-cell component is currently being elucidated. In the investigation of B cells. The associated immunologic dysfunction accounts for many of the clinically associated phenomenon such as infection and second malignancies. Much of this dysfunction has been attributed to poor B-cell function. Functional impairment of the T-cell component is currently being elucidated. In the investigations reported herein, we further demonstrate the immune deficiency of CLL by measuring proliferation and immunoglobulin synthesis in response to mitogens. Stimulation of various cell fractions indicates that in certain cases there is deficient T-helper, while others T function appears intact. In 2 of 4 cases tested there was augmentation of ig synthesis when the CLL cells were cocultured with thymic epithelium. The implications of such in vitro modulation are discussed.

Aged↗