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Biomedical subjects

D W Beatty

Publications and source records attributed to D W Beatty.

At least 37 records · Page 2Linked to original sources
PubMed1991

Hyper IgM with combined immunodeficiency.

This report describes a fourteen year old girl with an unusual immunodeficiency characterized by persistent lymphadenopathy and associated with hypogammaglobulinaemia, excessive IgM production and a severe T cell defect. Total T cell and T helper cell numbers were reduced and T cell proliferative responses to mitogens were poor. Serum IgM levels showed marked fluctuations and peaks correlated with acute tender lymphadenopathy. She was treated with intravenous gammaglobulin and prophylactic antibiotics. Although defective isotype switching of B cells into IgA and IgG producing cells has been accepted as the mechanism of the hyper IgM syndrome, it is becoming increasingly evident that T cell function is not uncommonly involved and may be responsible for impaired isotype switching.

Adolescent↗
PubMed1991

Human monoclonal antibodies: analysis of two antibodies derived from lymphocytes of a patient with acute rheumatic fever.

Human monoclonal antibodies were produced by fusion of peripheral blood lymphocytes from a patient with acute rheumatic fever, with the HGPRT-non-secreting murine (Balb-c) cell line SP2/0Ag14. Heterohybridomas were selected by screening against rheumatic fever-associated group A streptococci using an ELISA, and against paraffin wax-embedded human heart sections using an immunoperoxidase technique. Two human IgM monoclonal antibodies were selected for further analysis by Western blotting and ELISA. Both antibodies demonstrated multispecificity by immunoblotting and ELISA. One of the monoclonals bound to 48 kD and 83 kD bands common to group A streptococcal and heart antigen preparations. Both human monoclonal antibodies bound to a 43 kD constituent band common to human heart and sarcolemma membrane extract. Inhibition studies performed using a competitive solid phase immunoassay confirmed shared epitopes between group A streptococci and human heart. The significance of these monoclonal antibodies to the pathogenesis of rheumatic fever is uncertain.

Acute Disease↗
PubMed1990

Childhood near-drowning--factors associated with poor outcome.

Anoxic cerebral damage is the limiting factor in recovery from near-drowning accidents. This study reports factors associated with poor outcome in 100 near-drowned patients admitted to the Red Cross War Memorial Children's Hospital from 1976 to 1987. The study was designed as a retrospective folder search. Children with fixed dilated pupils, flaccidity, decerebrate or decorticate posturing, or clinical signs of cerebral oedema are more likely to have a poor outcome. Patients with metabolic acidaemia and those requiring cardiopulmonary resuscitation in the emergency room or subsequent mechanical ventilation also do poorly. Those requiring more aggressive management can be identified and a more accurate working prognosis may be possible in the hours after the accident.

Adolescent↗
PubMed1990

Immune studies in infants with congenital syphilis.

Seventeen neonates with congenital syphilis were studied to determine the immune response of the fetus following intra-uterine infection with Treponema pallidum. The results were compared with those from healthy controls matched for gestational age, birth weight and sex. B cells, IgM, and circulating immune complexes were significantly elevated in the infected newborns. There were no differences in lymphocyte transformation to phytohaemagglutinin (PHA) and in the CD3, CD4, and CD8 lymphocytes between infants with congenital syphilis and controls. Newborns with congenital syphilis have a heightened humoral response but no quantitative abnormality in cell-mediated immunity. Speculation on the role of the circulating immune complexes is presented.

Antigen-Antibody Complex↗
PubMed1989

X-linked agammaglobulinaemia.

X-linked agammaglobulinaemia (XLA) is a rare immunodeficiency disorder affecting only male subjects. There is an absence of all serum immunoglobulins and circulating B cells. T-cell function and numbers are normal. The clinical characteristics are recurrent pyogenic infections starting in infancy, hypoplasia of lymphoid tissue and a family history in about half the cases. Ten patients with XLA have been seen over the last 14 years at Red Cross War Memorial Children's Hospital, Cape Town. Chronic infections causing significant morbidity occurred in half our patients and 2 have died. Intravenous of gammaglobulin replacement therapy is superior to the intramuscular form. The recommended dose is 200-400 mg/kg/mo., although the optimal dose and frequency of the gammaglobulin infusion should be individualised for each patient.

Agammaglobulinemia↗
PubMed1988

Breakage of the T cell receptor alpha chain locus in non malignant clones from patients with ataxia telangiectasia.

Patients with ataxia telangiectasia (A-T) develop specific chromosome translocations, which may confer a proliferative advantage, resulting in the appearance of large clones in the peripheral blood lymphocytes. These lymphocytes are not malignant. Using in situ hybridisation techniques we have investigated a consistent 14q11 translocation breakpoint observed in a t(X;14)(q28;q11) translocation clone from each of two different patients and a t(14;14)(q11;q32) clone from a third patient. In all cases the chromosome translocation involved breakage within the alpha chain locus of the T cell receptor (TCR alpha), between the variable and constant regions, at 14q11. Chromosome rearrangement involving breakage within TCR alpha can therefore precede the development of malignancy. Further chromosomal rearrangement may be required in these patients, for progression to the leukaemic state.

Ataxia Telangiectasia↗
PubMed1988

Immunological abnormalities in children with acute rheumatic carditis and acute post-streptococcal glomerulonephritis.

Immunological functions were investigated in 10 children with acute rheumatic fever and 11 children with acute nephritis to try and elucidate the cause of heart damage in acute rheumatic fever. Children with acute rheumatic fever and carditis showed an increase in serum IgG, IgA and antistreptococcal antibodies during the acute stage. Lymphocyte transformation responses to phytohaemagglutinin and streptococcal antigens were reduced but this was due to a serum suppressor effect. After recovering from acute rheumatic fever a lymphocytosis and an increased lymphocyte blastogenic response to streptococcal antigen were found. T-cells, T-helper cells and T-suppressor cells showed some changes in acute rheumatic fever but these were not statistically significant in our study. None of the changes in immunological responses that were seen in acute rheumatic fever were found in acute nephritis. These results support the hypothesis that an abnormal immune response to streptococcal products is involved in the development of carditis and the other phenomena observed in acute rheumatic fever.

Adolescent↗
PubMed1987

Immunological studies in haemophilic children.

A majority of haemophiliacs who have received large-pool plasma products within the past 5 years have been exposed to the putative agent of the acquired immunodeficiency syndrome (AIDS)--HIV. It is not known what the risk of infection is among patients in South Africa. A study was made of 39 children with congenital coagulation disorders attending the Red Cross War Memorial Children's Hospital Haemophilia Clinic. All but 3 had been treated exclusively with small-pool lyophilised cryoprecipitate or a factor IX concentrate prepared by local blood transfusion services. Three patients had also received imported non-heat-treated commercial products FEIBA (Immuno), Autoplex, Proplex (Hyland) or Factorate (Armour). Absolute lymphocyte counts were normal in all patients but the OKT4/OKT8 ratio was reduced below 1.0 in 9 children including 2 of the 3 who had received commercial plasma concentrates. A high titre of HIV antibody was present in 2 of the 38 patients tested. Both of these children had received imported plasma concentrates and 1 shows some features of the AIDS-related complex. These results suggest that haemophiliacs who receive non-heat-treated commercial concentrates may be at greater risk of HIV infection than patients treated with locally produced plasma products.

Adolescent↗
PubMed1987

Group B meningococcal infection in children during an epidemic in Cape Town, South Africa.

One hundred and thirteen children with meningococcal infection were studied during an epidemic caused by N. meningitidis group B. Fifteen per cent presented with only meningeal symptoms, the remainder showed signs of septicaemia or combined septicaemia and meningitis. Sixteen per cent of the children were in shock and 18% required admission to the Intensive Care Unit (ICU). The mortality was 4.4%. More than half the children were younger than 2 years old. There was no statistical association between the age or nutritional state of the children and any of our measures of severity. A short history of symptoms was more common in children who presented with septicaemia and severe illness, who needed admission to the ICU, or who died. Diagnosis was confirmed by routine bacteriological methods and counter-immuno-electrophoresis (CIE) in 104 children. Eighty-six per cent of the isolates were group B type 2. A history of recent antibiotic treatment was associated with fewer positive cultures, but detection of meningococcal antigen by CIE was not affected by this. CIE antigen detection was not reliable because of the high incidence of false-negative results.

Blood↗
PubMed1986

Ataxia-telangiectasia. X,14 translocation, progressive deterioration of lymphocyte numbers and function, and abnormal in vitro immunoglobulin production.

Three children with ataxia-telangiectasia have been followed up since their early childhood. Sequential immunological, biochemical and chromosome studies have been performed over the last 7 years. All the children showed progressive cerebellar ataxia and inexorable neurological deterioration. Further evidence for the progressive nature of this condition is the fall in lymphocyte counts, deterioration of lymphocyte transformation responses to mitogens, and an increase in chromosomal translocations and breakage. Elevated serum alpha-fetoprotein levels are a highly characteristic and useful diagnostic finding in this condition. Two of the patients had an X,14 translocation. In vitro studies of immunoglobulin synthesis suggest an intrinsic defect in B-cell synthesis as well as decreased helper T-cell activity. In spite of moderately severe and progressive abnormalities in the immune system, sinopulmonary infections have not been prominent in our patients.

Adolescent↗
PubMed1986

Complement abnormalities during an epidemic of group B meningococcal infection in children.

During an epidemic of group B meningococcal infection mean values obtained in 96 consecutively affected children showed a reduction in classical pathway function (CH50), normal alternate pathway function (AP50), C4 and factor B levels, and raised C3 levels. CH50, C3 and Factor B were however significantly lower in those children who had a rapid onset of illness, were in shock, had signs of septicaemia, had extensive skin purpura, or who died. The presence of detectable meningococcal antigen by Counter Immuno Electrophoresis (CIE) and laboratory evidence of Disseminated Intravascular Coagulation (DIC) also correlated with lower complement levels. The significant reduction in CH50 and Factor B in the more severely affected patients suggests that activation of both classical and alternate pathways occurs in group B meningococcal infection.

Child↗
PubMed1985

Analbuminaemia. Clinical and laboratory features in a South African patient.

A 10-year-old white boy suffering from primary analbuminaemia presented with a 6-month history of ankle oedema. From infancy he had suffered from asthma and respiratory tract infections. His somatic growth was retarded but his mental and motor development was normal. The plasma albumin level was markedly reduced (81 mg/l) but a number of other plasma protein levels showed compensatory increases above the normal range. A study of the binding of unconjugated bilirubin added to the patient's plasma in vitro revealed an accumulation within the high-density lipoprotein fraction. It is suggested that the relative absence of severe illness in analbuminaemia reflects similar compensatory mechanisms afforded by the increase in non-albumin plasma protein levels and by other metabolic adjustments.

Ankle↗
PubMed1985

Serum zinc and copper levels in children with meningococcal disease.

Mean serum zinc and copper levels were depressed in 94 children aged 1 month to 9 years who presented with meningococcal disease. The mean serum zinc level was 44 micrograms/dl (reference value 78 micrograms/dl, SD 18) and the mean serum copper level 157 micrograms/dl (reference value 159 micrograms/dl, SD 27). Nineteen patients had serum zinc levels less than 25 micrograms/dl and ten patients had serum copper levels less than 101 micrograms/dl. Serum zinc levels were significantly lower in patients who were septicaemic or in whom manifestations of severe disease such as shock, more than 20 petechiae, ecchymoses and evidence of disseminated intravascular coagulation occurred compared to those without these features. Serum copper concentrations were higher than reference levels in patients with meningitis and in less severely ill patients. Copper levels were significantly lower in patients with septicaemia, severe disease, shock, more than 20 petechiae, ecchymoses, disseminated intravascular coagulation, leucopenia and patients who died compared with patients without these features.

Animals↗
PubMed1985

Plasma cell infiltration of the small bowel: lack of evidence for a non-secretory form of alpha-heavy chain disease.

Eight patients with diffuse plasma cell infiltration of the small bowel who had the clinical features of immunoproliferative small intestinal disease (IPSID), but whose serum was negative for free alpha-heavy chains, were investigated for evidence of a non-secretory form of alpha-chain disease (alpha-CD). Molecular sieving and immunoblotting of serum, immunoperoxidase staining of biopsy specimens, and in vitro protein synthesis studies utilising an immunoprecipitation technique and polyacrylamide gel electrophoresis, failed to detect any new cases of alpha-CD. Four of the eight cases were found to have diffuse intestinal lymphoma. The remaining four patients, who were unsuccessfully investigated for evidence of a significant abnormality in cellular immunity, have not developed detectable alpha-CD protein or lymphoma over a mean of 143 months. Despite continuing exposure to possible environmental stimuli, it is concluded that not all cases of IPSID elaborate detectable alpha-CD protein or evolve to lymphoma.

Adolescent↗
PubMed1984

A controlled trial of treatment of acquired immunodeficiency in severe measles with thymic humoral factor.

A randomized controlled trial of treatment with thymic humoral factor (THF) in 20 children with severe complicated acute measles infection, resulted in objective benefit as evidenced by improvement in the ESR and a fall in C-reactive protein, fewer complications and a reduced incidence of secondary herpes infection. An increased ratio of helper to suppressor T cells (OKT4/OKT8 ratio) and a greater lymphocyte transformation response to phytohaemagglutin was seen in those children receiving THF. We conclude that THF treatment helps to prevent the development of complications particularly secondary viral infections possibly by enhancing cell-mediated immune responses.

Child, Preschool↗