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Biomedical subjects

K Schopfer

Publications and source records attributed to K Schopfer.

At least 55 records · Page 3Linked to original sources

Systemic lupus erythematosus in Staphylococcus aureus hyperimmunoglobulinaemia E syndrome.

The prevalence of autoimmune diseases, including systemic lupus erythematosus, is increased in failure of certain host defence mechanisms. Systemic lupus erythematosus, however, has not been recorded as a late complication of the Staphylococcus aureus hyperimmunoglobulinaemia E (hyper-IgE) syndrome. Such a case was investigated in a man suffering from a classic example of the syndrome. Antinuclear antibodies were analysed on a molecular basis. The emergence of immunological and clinical features of systemic lupus erythematosus in patients with defective host defence mechanisms against staphylococcal infections is unlikely to be fortuitous and may help elucidate the pathogenesis of systemic lupus erythematosus. The observations will also aid the long term management of patients with S aureus hyper-IgE syndrome.

Adult↗

Molecular characterization of ribonucleoprotein antigens bound by antinuclear antibodies. A diagnostic evaluation.

The specificity of antinuclear antibodies in sera from 54 patients with various rheumatic diseases was analyzed by immunoprecipitation of 32P- or 35S-methionine-labeled HeLa cell extracts. Of 35 sera giving a speckled and/or homogeneous immunofluorescence pattern in rat liver nuclei, 20 contained antibodies to nuclear ribonucleoproteins, which are defined by their ribonucleic acid and protein components. Four sera reacted with a nuclear antigen (Ga) which has not been described so far. Of 19 sera with antinucleolar antibodies, 18 did not react with nuclear or nucleolar ribonucleoproteins. Correlation of these molecularly defined antinuclear antibody specificities with immunofluorescence patterns and rheumatic diseases is discussed.

Adult↗

[Chronic, septic granulomatosis (author's transl)].

A case report is presented on an 18-month-old boy who suffered from recurrent infections from early infancy. He was hospitalised for osteomyelitis in the left elbow; differential diagnosis also raised the possibility of a Ewing sarcoma. We were, however, able to eliminate this possibility on the basis of clinical data. Together with the immunologist, we discovered a malfunction of the granulocytes, consisting essentially in the fact that certain bacteria, although phagocytised, cannot be killed off within the cells. This has therapeutic consequences, which are discussed in this paper together with questions of prognosis.

Diagnosis, Differential↗

BCG osteomyelitis. Case report and review.

A 6-month-old boy is described presenting with BCG osteomyelitis of the distal metaphysis of the left femur extending into the epiphysis. Epidemiological, diagnostic and therapeutical problems of BCG osteomyelitis after BCG vaccination in the newborn period are discussed reviewing 89 published records of this late onset complication of BCG vaccination.

BCG Vaccine↗

[Hereditary angioedema: description of a sibship and review].

Report on a newly recognized sibship with hereditary angioedema (HAE). The 27 persons investigated include 8 with decreased concentrations of C-1INH and C4 in the Serum. Three of these are children without symptoms of HAE. Five patients have characteristic attacks of HAE, some of which are predominantly abdominal. The effects of treatment with danazol in 4 patients are described. HAE is briefly reviewed with special reference to pathogenesis and treatment.

Adolescent↗

[Clinical findings and diagnostic problems in a case of shunt nephritis. (author's transl)].

A report is presented on a nine year old girl in whom a Spitz-Holter valve had to be implanted after operation on a meningomyelocele while a neonate. Because of a macrohematuria and a hypochromic anemia, she was admitted as an inpatient. In addition, there was a pyuria with bilateral hydronephrosis. The clinical picture was initially misinterpreted as hemorrhagic cystitis with ascending pyelonephritis in neurogenic bladder. Only the reduction of serum complement suggested the presence of a shunt nephritis. The diagnosis was verified by kidney biopsy. After removal of the infected valve system, there was a prompt normalization of all laboratory parameters. Besides description of the case history, above all the diagnostic problems of this rare syndrome, which is nevertheless of practical importance, are dealth with.

Anemia, Hypochromic↗

Immunoglobulin E antibodies against Staphylococcus aureus cell walls in the sera of patients with hyperimmunoglobulinemia E and recurrent staphylococcal infection.

The specificity of antistaphylococcal antibodies of the IgE class in five patients with hyperimmunoglobulinemia E and recurrent staphylococcal infection has been investigated. Purified cell walls were prepared from various staphylococcal strains, and serum immunoglobulin E binding was measured by using a solid-phase radioimmunoassay. Immunoglobulin E binding occurred only with cell walls from Staphylococcus aureus strains, including walls from a teichoic acid-deficient mutant. Immunoglobulin E did not bind to cell wall preparations from the coagulase-negative species S. capitis, S. sciuri subsp. lentus, S. simulans, S. xylosus, staphylococcal strains RB-11 and Armour, and from a group A streptococcus strain CS44. Since the glycan backbone and the tetrapeptide (pentapeptide) subunit of the peptidoglycan of all staphylococcal strains tested are believed to be identical, it is suggested that IgE binding is related to either the peptidoglycan interpeptide bridge or an unknown antigenic structure within the cell wall of S. aureus. The pathophysiological significance of antistaphylococcal immunoglobulin E antibodies in the disorder studied is at present unknown. The formation of immunoglobulin E antibodies to S. aureus cell wall components may be a manifestation of an aberrant immunological response to S. aureus related to the undue susceptibility to staphylococcal infections in these patients.

Antibodies, Bacterial↗

Congenital cytomegalovirus infection in newborn infants of mothers infected before pregnancy.

The rate of congenital cytomegalovirus (CMV) infection was studied in newborn infants in an African population in which all adults had experienced primary CMV infection during childhood. Viruria within the first 12 hours after delivery was taken as evidence of prenatal CMV infection. 28 of 2032 newborn infants examined had viruria, giving a rate of 1.4% congenital CMV infection. The presence of meternal serum antibody therefore appears not to protect the fetus from intrauterine infection. Either reactivation of latent maternal CMV infection or recurrence of infection during pregnancy despite the presence of serum antibodies may explain these findings. Whether the long-term effects of CMV infection acquired in utero differ in cases of primary maternal infection from those due to reactivated or recurrent infection in seropositive mothers, remains undecided. Thus, the value of a live CMV vaccine to prevent prenatal CMV infection may be questioned.

Adolescent↗

Fine structural studies of peripheral blood leucocytes from children with kwashiorkor: morphological and functional properties.

This is the first report of electron microscopic observation on circulating leucocytes from children with kwashiorkor, Neutrophils showed evidence of immaturity, prominent Golgi zones, granule pleomorphism; strands of endoplasmic reticulum and Döhle bodies were frequent. Although an in vitro microbicidal defect occurs with S. aureus, E. coli and C. albicans, the postphagocytic morphological events, including vacuole formation and degranulation, were normal. In mononuclear cell pellets plasmacytoid cells were frequently observed, and rare lymphoblasts occurred. The findings suggest activation of the phagocytic system and stimulation of humoral immunity in children with kwashiorkor. These fine structural observations are comparable to those which occur in trauma or thermal injury, both conditions associated with tissue breakdown and extensive antigenic stimulation.

Child↗

Neutrophil function in children with kwashiorkor.

Peripheral blood polymorphonuclear neutrophil (PMN) function has been investigated for 46 children with kwashiorkor (without overt infection) in the Ivory Coast, West Africa. In vitro chemotactic response, candidacidal activity, and kinetic studies of metabolism during phagocytosis have been performed. Postphagocytic morphological events were evaluated by electron microscopy. The reduction of nitroblue tetrazolium (NBT), measurement of enzyme activities, activity of glycolysis, and hexose monophosphate shunt (HMS) activity were assessed. The extent of iodide incorporation into trichloracetic acid (TCA)-precipitable protein by phagocytizing PMN'S and thyroid hormone degradation were measured. Chemotactic response was reduced at early time intervals (30, 60, and 120 minutes) and reached control values after 180 minutes. Whereas PMN's of controls killed 32.13 +/- 11.10 per cent of Candida albicans after 60 minutes, PMN's from kwashiorkor patients killed 18.55 +/- 7.74 per cent (p less than 0.01). HMS activity for resting PMN's of kwashiorkor children was higher than for controls, and during particle ingestion the extent of stimulation was comparable to controls. Electron microscopic assessment of phagocytic vacuole formation and degranulation showed no difference between PMN's from kwashiorkor and and control subjects. Incorporation of 131 I into TCA-precipitable proteins by phagocytizing PMN's from kwashiorkor children was reduced in compraison to controls, with either viable or heat-killed lactobacilli. No impairment in thyroxine (T4) degradation was observed for PMN's from kwashiorkor cases. PMS's from kwashiorkor patients show toxic granules, Dohle bodies, evidence of high baseline NBT reduction, and glucose decarboxylation. Functional studies indicate impaired kinetics of chemotaxis, diminished candidacidal activity, and reduced iodination. Enzymatic activities of resting cells are normal. Lactate production, HMS activity during phagocytosis, and morphological events are not impaired. Thus, impaired in vitro microbicidal activity, increased resting metabolism, and decreases iodination by PMN's may be related to the high incidence of infection in kwashiorkor.

Candida albicans↗