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Biomedical subjects

M J Chusid

Publications and source records attributed to M J Chusid.

At least 73 records · Page 4Linked to original sources
PubMed1980

Congenital neutropenia: studies of pathogenesis.

Congenital neutropenia (CN) was diagnosed in a five-month-old boy. A variety of studies was performed to define the pathogenesis of his disease. Opsonic antineutrophil antibodies wre present in his serum. Transfused normal granulocytes circulated poorly. Incubation of the patient's serum with normal granulocytes failed to alter their metabolic or functional activity. The patient's marrow demonstrated increased numbers of colony-forming units (CFUs) in vitro compared with control marrow. The patient's parents had low marrow CFU activity. The patient's serum and peripheral lymphocytes failed to inhibit normal marrow CFU activity. The patient's marrow did inhibit CFU growth of an HLA-identical-sibling's marrow in coculture. Histocompatibility studies demonstrated the HLA-B12 antigen in this patient, a histocompatibility marker previously associated with CN. These studies suggest some cases of CN are associated with a genetically transmitted marrow factor capable of suppressing myelopoiesis in normal marrow.

Agranulocytosis↗
PubMed1980

Correction of infantile agranulocytosis (Kostmann's syndrome) by allogeneic bone marrow transplantation.

Allogeneic bone marrow transplantation has been unsuccessful as therapy for genetically determined bone marrow disorders. In patients prepared for transplantation with drugs alone long-term hematopoietic engraftment is not achieved due to the overgrowth of the infused donor bone marrow cells by residual recipient hematopoietic stem cells. Utilizing a combination of total body irradiation and antihuman thymocyte serum, the successful eradication of the abnormal hematopoietic stem cells of patients with the Wiskott-Aldrich syndrome and now infantile agranulocytosis has been achieved. Following preparation with total body irradiation and antihuman thymocyte serum a 20 month old patient with infantile agranulocytosis has complete donor hematopoietic and lymphoid engraftment one year after a histocompatible allogeneic bone marrow transplant. Prior to transplantation, this patient had no circulating or bone marrow granulocytes; following transplantation he has normal numbers of circulating granulocytes with normal in vivo and in vitro function. This therapeutic result demonstrates that genetic disorders of myeloid function can be corrected by allogeneic bone marrow transplantation following preparation with total body irradiation and antihuman thymocyte serum, and suggests that infantile agranulocytosis is due to an intrinsic defect of the pluripotent hematopoietic stem cell and not to a micro-environmental defect.

Agranulocytosis↗
PubMed1980

Illnesses associated with extreme elevation of the erythrocyte sedimentation rate in children.

The laboratory and hospital records were reviewed for the 156 children seen at Milwaukee Children's Hospital over a thirty-one month period with erythrocyte sedimentation rates (ESR) greater than or equal to 100 mm/hr. Fifty-five per cent of the children had an underlying infectious disease, 25 per cent had a collagen or renal disease, 13 per cent had neoplasms, and 6 per cent had miscellaneous disease processes. Patients with infectious diseases and high ESRs were significantly younger, significantly more febrile, and had higher leukocyte and absolute band counts than did patients in the other groups. Patients with malignancy had significantly lower hemoglobin levels, leukocyite and absolute PMN counts. Some patients did die during the 30-month study period but not during the particular hospitalization in which the ESR was obtained.

Adolescent↗
PubMed1980

Bacterial epididymo-orchitis in children and adolescents.

Five cases of bacterial epididymo-orchitis are presented. These patients demonstrate three distinct mechanisms of bacterial infection of the intrascrotal structures: epididymo-orchitis as a presentation and a complication of urinary tract infection; epididymo-orchitis associated with sexually transmitted disease; and epididymo-orchitis associated with systemic infection. We recommend urine, urethral, and blood bacterial cultures, in addition to viral serologic studies, as an appropriate evaluation for any patient with epididymo-orchitis.

Adolescent↗
PubMed1980

Determination of posttransfusion granulocyte kinetics by chemiluminescence in chronic granulomatous disease.

A 14 year old boy with CGD of childhood received HLA-matched granulocyte transfusions in the therapy of an intramural abscess of the ileum. Donor granulocyte survival after transfusion was determined with the endotoxin-stimulated NBT test as well as an assay of chemiluminescence employing whole blood. The presence of circulating donor PMNs after transfusion was documented by both techniques. The half-life of donor cells in the first 5 hr following transfusion was approximately 1.4 hr with both techniques. The greatest number of transfused cells was detected by both methods in the patient's blood immediately after the completion of the transfusion. At this time 22.7% of the expected increment in donor PMN concentration was detected by the NBT test, and 13.8% of the expected increment was detected by chemiluminescence assay. Both techniques allow studies of posttransfusion granulocyte kinetics in CGD because of the basic underlying metabolic abnormality of recipient but not donor cells. The chemiluminescence assay may also be adaptable to use in neutropenic patients receiving PMN transfusions.

Adolescent↗
PubMed1979

Perirectal abscess in childhood. A review of 29 cases.

A total of 29 cases of perirectal abscess at Milwaukee Children's Hospital between 1965 and 1975 were retrospectively reviewed. One fourth of these children had a serious underlying chronic disease. Staphylococcus aureaus was the organism that grew most frequently from pus obtained from the abscesses, accounting for 52% of the isolates. Therapy included incision and drainage alone and incision and drainage plus parenteral and/or oral antibiotic treatment. One patient, a child with leukemia, died of this infection. Complications, primarily fistulain-ano or recurrent abscess, occurred in 42% of the survivors.

Abscess↗
PubMed1979

Experimental bacterial keratitis in neutropenic guinea pigs: polymorphonuclear leukocytes in corneal host defense.

Quantitative techniques were used to determine the relative concentrations of viable bacteria and polymorphonuclear leukocytes (PMNs) in the corneas of neutropenic and non-neutropenic guinea pigs with experimental bacterial keratitis induced with three strains of Pseudomonas aeruginosa. Neutropenia was produced by whole-body X-irradiation 1 week before infection. Significantly greater numbers of bacteria were present in the cornea of neutropenic animals 48 h after infection than were present in the corneas of non-neutropenic animals. The same was true 24 and 48 h after infecting animals with Staphylococcus aureus. Examination of histological sections showed that fewer PMNs were present in the corneas of infected neutropenic animals than in the corneas of infected non-neutropenic animals. Radiolabeling of PMNs confirmed a significant reduction in PMN concentration in the corneas of infected neutropenic animals. Tears and the corneal epithelium appear to be the most important elements protecting the cornea against local invasion by bacteria. However, once bacterial keratitis is established, PMNs play a role in limiting bacterial multiplication.

Animals↗