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Biomedical subjects

D S Chudwin

Publications and source records attributed to D S Chudwin.

27 records · Page 2Linked to original sources

Response of agranulocytosis to prolonged antithymocyte globulin therapy.

A girl with a history of autoimmune disease developed life-threatening agranulocytosis. A bone marrow biopsy demonstrated selective granulocytic hypoplasia. No antineutrophil antibodies were found. In vitro bone marrow culture of granulocytic progenitor cells suggested T cell-mediated inhibition of colony formation, which was reduced by in vitro treatment of marrow cells with either hydrocortisone or an antibody directed against T-lymphocytes and complement. The patient responded to treatment with antithymocyte globulin after administration of corticosteroids and other immunosuppressants failed to increase her neutrophil count significantly. Attempts to stop ATG treatment resulted in precipitous drops in her neutrophil counts, which reversed with readministration of ATG. She then received weekly ATG infusions for over 24 months until she was able to maintain a normal neutrophil count. A trial of ATG therapy may be indicated in severe neutropenia when in vitro culture results indicate a possible autoimmune basis.

Agranulocytosis↗

Effect of antibody concentration on opsonic requirements for phagocytosis in vitro of Streptococcus pneumoniae types 7 and 19.

We investigated the effect of the concentration of type-specific antibody to pneumococcal polysaccharide (PPS) on opsonic requirements for phagocytosis in vitro of Streptococcus pneumonia types 7 and 19. We measured the uptake by human neutrophils of radiolabeled S. pneumoniae opsonized with either complement-intact, complement-depleted (by heat inactivation), or alternative complement pathway-activated (by magnesium dichloride-ethylene glycol tetraacetic acid (MgEGTA) chelation) immune sera with varying concentrations of antibody from individuals immunized with polyvalent PPS vaccine. Increased opsonization was found with increasing concentrations of type-specific antibody in the sera. Higher concentrations of antibody were required to opsonize type 7 than type 19 bacteria, both in the presence and absence of complement activity. Type 19 bacteria were more efficiently opsonized via the alternative complement pathway than type 7. For both types, antibody and the alternative complement pathway provided most of the opsonic activity in sera with lower concentrations of type-specific antibody. At high antibody concentrations, effective opsonization occurred in heat-activated sera, indicating the requirement for complement could be overcome with sufficient amounts of antibody alone.

Antibodies, Bacterial↗

Aspergillus pneumonia in chronic granulomatous disease: recurrence and long-term outcome.

Four of 13 patients followed with chronic granulomatous disease (CGD) developed biopsy-proven Aspergillus pneumonia. Two patients died of disseminated aspergillosis despite intensive treatment; delays in diagnosis appear to have been a major contributing cause. Two patients survived two episodes each of Aspergillus pneumonia, three and six years apart. At 12 and 18 years of age, both are well and active, although their chest X-rays show residual scarring and pulmonary function tests indicate obstructive lung disease. These cases demonstrate that in patients with CGD, 1) multiple episodes of Aspergillus pneumonia can occur; 2) effective immunity to the organism does not develop after infection; and 3) some survivors of Aspergillus pneumonia experience only minimal morbidity.

Aspergillosis↗

Posttransfusion syndrome. Rash, eosinophilia, and thrombocytopenia following intrauterine and exchange transfusions.

A clinical syndrome of transient maculopapular rash with associated eosinophilia, thrombocytopenia, and mild lymphopenia developed in 21 of 35 (60%) neonates receiving both intrauterine transfusions and postnatal exchange transfusions, and six of 17 (35%) neonates receiving multiple exchange transfusions for erythroblastosis fetalis. This syndrome did not develop in infants who received intrauterine transfusions without exchange transfusions or single exchange transfusions without prior intrauterine ones. Patients with this rash and associated laboratory abnormalities did not progress to acute or chronic graft-vs-host disease, and on follow-up examinations were alive and well.

Blood Transfusion, Intrauterine↗

Spectrum of Sjögren syndrome in children.

Sjögren syndrome, consisting of keratoconjunctivitis sicca and xerostomia with or without another autoimmune disease, is uncommon in children. We describe our retrospective experience with eight pediatric patients with SS. All had recurrent parotid enlargement and abnormal salivary gland biopsies, six had keratoconjunctivitis sicca, and five had other autoimmune manifestations, although only two of these had other clearly defined autoimmune disorders (mixed connective tissue disease and hypergammaglobulinemic purpura). Our patients had a higher incidence of primary SS, parotid enlargement, and hematologic abnormalities than did children previously reported with SS. Children with SS demonstrate a clinical heterogeneity comparable to that seen in adults.

Adolescent↗

Neuroblastoma presenting with hematuria.

A 3-year-old boy with neuroblastoma presented with gross hematuria, an unusual initial complaint with this malignancy. Renal findings associated with neuroblastoma are discussed.

Child, Preschool↗

Fetal hemoglobin and alpha-fetoprotein in various malignancies.

Fetal hemoglobin (HbF) concentrations were measured by a radial immunodiffusion assay in 233 patients with various malignancies. In 96 of these, alpha-fetoprotein (AFP) was also measured by radioimmunoassay. The concentration of HbF exceeded 2 SDs above the normal mean in 39 of 233 patients, most notably in patients with leukemia, lymphomas, multiple myeloma and testicular tumors. The proportion of HbF was not correlated with the total hemoglobin concentration or with serum AFP concentration.

Fetal Hemoglobin↗