Gamma globulin for antibody-mediated pure red-cell aplasia.
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Antibodies, Anti-Idiotypic↗
Biomedical subjects
A Benderly
Publications and source records attributed to A Benderly.
Immunoglobulin administration into cerebral subarachnoid space in a child with hypogammaglobulinemia and aseptic meningoencephalitis.
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Agammaglobulinemia↗
Psychosexual outcome of assigned females and males with 46,XX virilizing congenital adrenal hyperplasia.
Seven genotypic female (46,XX) patients with congenital adrenal hyperplasia, four reared as girls and three reared as boys were evaluated at 16-26 years of age with psychological interviews and testing. A small penis in the three young men produced a practical and psychological barrier that severely injured their personality. In spite of some degree of pre-adolescent tomboyism and a somewhat injured body image, the girls adjusted to their feminine gender identity. It is concluded that in cases of both early and late diagnosis, the genotypic female sex should be preferred.
Adaptation, Psychological↗
Defective humoral and cellular immune functions associated with veno-occlusive disease of the liver.
We describe veno-occlusive disease of the liver associated with humoral and cellular immune defects in two siblings. Another child, with aplastic anemia, died before the age of 1 year. No consanguinity was found in the family. Both infants had lymphopenia and hypogammaglobulinemia; the surviving infant has defective in vitro immunoglobulin production after stimulation of lymphocytes with pokeweed mitogen, increased proportions of OKT8 positive cells, defective proliferative responses to phytomitogens, and decreased help for immunoglobulin production. A therapeutic trial with cimetidine, an H2 receptor antagonist, has not changed the immunologic status of the surviving child.
Agammaglobulinemia↗
Immunological studies in children with type I diabetes mellitus and their healthy relatives: in vitro antigen stimulated IgM production, T-cell subsets and islet-cell antibodies.
Several defects in immunoregulation have been described in patients with insulin dependent diabetes mellitus (IDDM). We have looked at immunoregulatory T-cell subsets autoantibodies and in vitro IgM production in IDDM patients and their healthy first degree relatives. The proportions of total T-cells (OKT11+), helper/inducer (OKT4+), suppressor/cytotoxic T-cells (OKT8+) and OKT4/OKT8 ratio were normal in 8 patients as well as in their 26 family members and in 40 age-matched controls. Anti islet-cell antibodies (ICA) were found in sera of 6 out of 17 patients (35%) in 1 out of 62 (1.6%) family members and in none of the age-matched healthy controls. A significant increase of in vitro IgM production was found in 8 patients (130 +/- 10 PFC/10(6) cultured cells, mean +/- S.E.) as compared to 21 healthy controls (57 +/- 5.0) (p less than 0.01). In vitro IgM production by B-cells of 22 family members was also increased compared to controls (83 +/- 8.0) (p greater than 0.05). This increased antibody production is characteristic for clinical states associated with impaired immunoregulation, and reflects perturbation of immunoregulatory mechanisms in IDDM.
Adolescent↗
Complete recovery of pure red cell aplasia by intramuscular gammaglobulin therapy in a child with hypoparathyroidism.
A 10-year-old girl with multiple endocrinopathies, hypoparathyroidism, and pernicious anemia subsequently developed pure red cell aplasia, which responded only temporarily to cytotoxic therapy. Because of transfusion dependency, resistance to steroids, plasmapheresis, and Cytoxan, she was given injections of intramuscular gammaglobulin. The injections caused a prompt and sustained rise in the child's erythropoiesis.
Child↗
Requirement of mineralocorticoid in congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency.
Marginal salt loss occurs in patients with congenital adrenal hyperplasia due to 11 beta-hydroxylase (11-OHase) deficiency treated with dexamethasone and is accompanied by increased PRA. The present study was undertaken to evaluate the effect of the stimulated renin-angiotensin system on pituitary-adrenal suppression. Seven patients with 11-OHase deficiency were subjected to a series of treatments with dexamethasone, cortisol, and combined cortisol and 9 alpha-fluorohydrocortisone. The latter combination suppressed PRA and sodium excretion, and produced better control of the pituitary-adrenal axis, as measured by plasma ACTA and serum 11-deoxycortisol. We conclude that in children with 11-OHase deficiency, PRA needs to be monitored, and when it is elevated, mineralocorticoid replacement is indicated.
Adolescent↗
Transient hypogammaglobulinemia of infancy with severe bacterial infections and persistent IgA deficiency.
A 1-year-old boy who had had recurrent episodes of sepsis was found to have transient hypogammaglobulinemia of infancy and was treated with gammaglobulin supplements. He subsequently remained IgA deficient and a regulatory T cell imbalance was found.
Agammaglobulinemia↗
Transient immunoregulatory perturbation during the acute phase of rheumatic fever.
An abnormal immune response directed at streptococcal antigen is currently thought to be involved in the development of autoimmune phenomena in rheumatic fever (RF). We studied T-cell subsets using the OKT monoclonal antibodies in 13 patients with newly-diagnosed RF. No difference was found in the total number of T cells or in the proportion of helper T cells (OKT4+) at the acute phase (56.6 +/- 6.9) as compared to the recovery phase (55.2 +/- 9.2). However, a significantly decreased proportion of suppressor T cells (OKT8+) was observed during the acute phase (17.2 +/- 6) as compared to the recovery phase (26.4 +/- 5.3) (p less than 0.001). The T4/T8 ratio was significantly higher during the initial phase (3.65 +/- 1.04) and returned to normal later (2.12 +/- 0.39) (p less than 0.002). The results of our study indicate that in the acute phase of RF many patients had low levels of suppressor T cells which return to normal after recovery. Thus, reduced proportion of suppressor T cells might be an indicator for the active phase of RF. Decreased suppressor cell activity may also be involved in the development of autoimmune phenomena in RF.
Acute Disease↗
Bacterial meningitis in infants two to six weeks old.
We reviewed our experience with bacterial meningitis in older neonates (2 to 6 weeks of age) during a five-year period. Seventeen patients with bacterial meningitis were diagnosed and treated. Bacteria recovered from the cerebral spinal fluid (CSF) included pneumococci (29%), E. coli and meningococci (23% each), group B streptococci (12%), Enterobacter and H. influenzae (6% each). There were no cases of Listeria monocytogenes. The mean duration of symptoms before admission was 3.1 days. The mortality rate was high (30%), and 36% of the patients had significant neurologic residua. Our study shows that this specific age group is different from newborns or older infants. Therefore, the initial selection of antibiotics for the treatment of meningitis in this age group should include antibiotics that are effective across this spectrum of potential pathogens.
Age Factors↗
[The defence mechanism against bacteria in the urinary tract].
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Bacterial Infections↗
Growth and pubertal development in patients with congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency.
To evaluate growth and pubertal development in patients with congenital adrenal hyperplasia due to 11-beta-hydroxylase deficiency, data were collected retrospectively from the charts of 24 patients, 15 girls and nine boys. Growth before diagnosis was characterized by a rapid acceleration in the second half of the first year of life, with rapid advancement of bone age. Final height was severely compromised in all the patients, regardless of age at diagnosis and quality of therapeutic control. Onset of puberty was precocious in male patients and normal in female patients. In this respect, treatment with hydrocortisone acetate proved to be superior to cortisone acetate or prednisone.
Adolescent↗
Hypocalcaemia induced by glucocorticoids in a child with hypoparathyroidism treated with 1-alpha-hydroxyvitamin D3.
Glucocorticoids tend to lower the intestinal absorption of calcium, leading to a negative calcium balance. A 7-year-old girl with hypoparathyroidism was maintained as normocalcaemic on 1-alpha-hydroxyvitamin D3 (1 alpha D3) and calcium gluconate lactate. During an episode of aplastic anaemia she was treated with prednisolone, with a subsequent dramatic fall of serum calcium despite 1 alpha D3 treatment and serum 1,25-dihydroxyvitamin D3 (1,25(OH)2D3) in the high-normal range. Glucocorticoids seem to have interfered directly with cellular events responsible for intestinal absorption of calcium.
Anemia, Aplastic↗
Role of the immune system in the etiology of rheumatic fever.
In order to summarize all the data regarding the immunological findings in RF we have elaborated the following theory about the pathogenesis of RF (fig. 1). The disease will develop only after repeated beta-hemolytic group A streptococcal throat infection in susceptible individuals. In these people, who have a specific immune response to repeated streptococcal infection due to their DR region, a decrease in suppressor activity will develop. This will lead to an increased specific humoral and cellular immune response to streptococcal antigen which cross-reacts with specific human tissue as the heart or the brain. Alternatively or concomitantly specific autoantibodies against these tissues are made. These antibodies together with the cellular response will cause the clinical manifestation seen in RF. This is only a proposed theory and still much more work has to be done to solve the enigma regarding the pathogenesis of RF.
Antibody Formation↗
Comparison of the effects of amphetamine and a fluorinated analogue on locomotion and exploratory activity in the mouse.
(+)-Amphetamine (AM) and its fluorinated analogue (+)-2-amino-3-fluoro-1-phenylpropane (fluoroamphetamine, FAM) were compared with regard to their effects on locomotor and exploratory activity in mice. Both drugs caused a reduction in spontaneous exploration, but this effect was more marked with FAM than with AM at 1 h after injection. Both compounds increased locomotor activity 10 min after injection, but FAM had sedative effects after 1 h, while AM continued to be stimulatory.
Amphetamine↗
Hyperbaric oxygenation in peripheral ischaemic lesions in infants.
Acute peripheral arterial occlusion may lead to gangrene, with loss of parts of arms and legs. Three infants with disseminated intravascular clotting developed dark red discoloration of the tips of fingers and toes which progressed proximally. Repeated hyperbaric oxygenation treatments caused regression of the demarcation line and further progression of necrosis stopped.
Arm↗
Calcitriol-resistant rickets with alopecia.
Four children of two kindred had alopecia associated with severe rickets, resistant to treatment, and caused by defective cytoplasmic and nuclear receptors for the active vitamin D metabolite calcitriol (1,25-dihydroxyvitamin D3). Scalp biopsy specimen revealed a normal number and light microscopic features of the hair and hair follicles. Calcitriol-resistant rickets should be added to the list of inherited disorders of hair growth, and the association of rickets with alopecia needs to be considered in the differential diagnosis of hair loss.
Alopecia↗
Blood level of C3 complement component as an aid in the management of acute phase glomerulonephritis.
Ninety-five patients with acute glomerulonephritis, based upon clearly defined pathological criteria, were studied and scored. The scoring was correlated to their levels of blood complement components. Patients with low C3 serum levels had a severe course during the acute phase of their disease, as compared with those with normal C3 serum levels. No such correlation was established with regard to C4 serum levels. It is suggested that C3 serum level may serve as an indicator of the expected severity of the inflammatory process and thus as an aid in the management of patients with acute glomerulonephritis in the initial phase of the disease.
Acute Disease↗