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Biomedical subjects

M A Koerper

Publications and source records attributed to M A Koerper.

36 records · Page 2Linked to original sources
PubMed1990

Relapsing post-hepatitis aplastic anemia. Immunosuppressive therapy.

A 20-month-old Hispanic male developed severe aplastic anemia after an episode of non-A, non-B hepatitis. Prompt and complete recovery of all hematopoietic cell lines occurred after treatment with antithymocyte globulin (ATG) and high-dose corticosteroids. Severe aplasia recurred two months later coincident with a mild upper respiratory infection. A second course of immunosuppressive therapy was followed by complete, sustained improvement. The authors' experience provides clinical evidence indicating that immunologic mechanisms are important in the treatment of severe post-hepatitis aplastic anemia. Children in whom aplastic anemia recurs after immunosuppressive treatment may respond to a second course of therapy.

Adrenal Cortex Hormones↗
PubMed1989

AIDS and hemophilia.

Explore the source record for details and available documents.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Management of hemophilia in otolaryngologic surgery. A contemporary protocol.

Hemophiliacs are well known to be among the high-risk groups for acquiring acquired immunodeficiency syndrome due to their frequent exposure to pooled blood products. We reviewed our recent experience involving hemophiliacs undergoing a variety of otolaryngologic surgical procedures. A protocol was developed to minimize the risks of hemorrhage through the judicious use of preoperative and post-operative coagulation replacement products. Modern hemostatic techniques, such as the use of the surgical laser, also had a role in lessening the incidence of bleeding problems. The relative risks of the various hemostatic products with regard to the transmission of communicable diseases such as acquired immunodeficiency syndrome and hepatitis were evaluated. Recent data suggest that heat treatment of factors VIII and IX concentrates eliminates the risk of acquired immunodeficiency syndrome transmission, and these heated concentrates should be used in preference to older products. Hepatitis remains a problem, but this risk may be reduced to some degree through immunization with hepatitis B vaccines that have recently been proved safe and effective.

Clinical Protocols↗
PubMed1984

Magnetic resonance imaging of transfusional hemosiderosis complicating thalassemia major.

Tissue deposits of hemosiderin, a paramagnetic iron-protein complex, resulted in marked abnormalities of magnetic resonance (MR) spin-echo signal intensity within the viscera of three children with transfusional hemosiderosis and thalassemia major. In all patients the liver and bone marrow demonstrated abnormally low spin-echo intensities and the kidneys and muscles had abnormally high intensities. These observations correlate with in vitro MR observations of ferric (Fe+3) solutions, in which concentrations of ferric salts greater than 20 mmol yielded a low MR intensity signal and ferric concentrations less than 15 mmol yielded higher intensities than did water alone. MR imaging is sensitive to the tissue deposition of hemosiderin, and MR intensity appears to provide a rough measure of the amount of iron deposited.

Adolescent↗
PubMed1983

Immunologic abnormalities in patients with hemophilia A.

To assess the immunologic status of healthy persons with hemophilia A, we performed studies of T cell immunity in 21 patients, 10 given only cryoprecipitate and 11 given factor VIII concentrate. Patients in the factor VIII group had significantly decreased helper/suppressor T cell ratios. Both groups had diminished mononuclear cell response to phytohemagglutinin and normal mixed lymphocyte culture, compared with controls. Abnormalities in T cell number or function did not correlate with the presence of antibody to cytomegalovirus, Epstein-Barr virus, or hepatitis B. Physicians caring for patients with hemophilia A should realize that asymptomatic individuals may have early evidence of immunodeficiency.

Acquired Immunodeficiency Syndrome↗
PubMed1980

PMNs in normal spinal fluid examined by the cytocentrifuge technique.

The presence in unconcentrated CSF specimens of even a single polymorphonuclear (PMN) cell has been regarded as diagnostic of central nervous system (CNS) disease. In 18 of 50 consecutive cytocentrifuged specimens obtained from patients without evidence of CNS disease, however, we found 1 to 6 PMN cells. The observation of occasional PMN cells in concentrated CSF specimens does not appear to be diagnostic of a CNS abnormality.

Cell Count↗
PubMed1978

Formation of prostacyclin (PGI2) by the ductus arteriosus of fetal lambs at different stages of gestation.

Prostaglandins appear to play a role in maintaining patency of the ductus arteriosus during gestation. Prostacyclin (PGI2) is the major product of prostaglandin biosynthesis in the lamb ductus arteriosus. This factor is both a vasodilator and a potent inhibitor of human platelet aggregation. We used inhibition of platelet aggregation as a sensitive bioassay to measure PGI2 generation in rings of ductus arteriosus from fetal lambs. Mechanical manipulation accelerated the rate of PGI2 released from the tissue 10 to 50 times. Tranylcypromine, an antagonist of prostacyclin synthetase, suppressed production of PGI2 by rings of ductus arteriosus. Rings from immature animals (98-103 days gestation, term is 150 days) released significantly more PGI2 (190 +/- 28 ng/g wet weight/ 20 min, n = 9) than did those from near term animals (136-146 days; 106 +/- 23 ng/g wet weight/20 min, n = 10). The capacity of the ductus arteriosus to generate more PGI2 earlier in gestation is consistent with the observation that vessels from animals less than 110 days gestation have a significantly larger indomethacin induced contraction than do vessels near term.

Animals↗
PubMed1978

Anemia in patients with juvenile rheumatoid arthritis.

Patients with juvenile rheumatoid arthritis may have an anemia attributable to the chronic disease, to iron deficiency, or to a combination of the two. The contribution of iron deficiency is often difficult to determine by routine laboratory studies. We studied 51 patients with pauciarticular and polyarticular juvenile rheumatoid arthritis with red blood cell counts, indices, free erythrocyte protoporphyrin, and serum ferritin. Fifteen of the 18 who were anemic were restudied after a 3 to 6-month period of iron therapy. Thirteen of the 15 responded by these criteria: a rise in hemoglobin of 1.0 gm/dl or more and an increase in mean corpuscular volume of 3 fl or more; in 11 of these 13, hemoglobin values returned to the normal range for age. These findings indicate that iron deficiency can be a major component of the anemia that is commonly found in patients with active juvenile rheumatoid arthritis.

Adolescent↗
PubMed1977

Serum iron concentration and transferrin saturation in the diagnosis of iron deficiency in children: normal developmental changes.

A group of 359 healthy children and 49 adults were studied for the purpose of estimating the normal limits for serum iron concentration and transferrin saturation. The 144 children and seven adults who has any other laboratory evidence of iron deficiency (abnormal values of serum ferritin, free erythrocyte protoporphyrin, hemoglobin concentration, or mean corpuscular volume) were excluded. In evaluating the 215 children and 42 adults who met the criteria to be considered normal we found that serum iron concentration and transferrin saturation were significantly lower in children between the ages of 0.5 and 12 years than in adults. We conclude that in children between the ages of 0.5 and 12 years, a transferrin saturation of less than 16% constitutes good evidence of iron deficiency only in conjuction with anemia and low mean corpuscular volume.

Adolescent↗
PubMed1976

Developmental change in red blood cell volume: implication in screening infants and children for iron deficiency and thalassemia trait.

The mean corpuscular volumen when determined by electronic counter is an accurate tool for identification of children with microcytosis due to either iron deficiency or thalassemia trait. The purpose of this report is to describe the normal developmental changes in MCV that occur in children afler 6 months of age. In 211 healthy infants and children screened to exclude those with borderline or overt iron deficiency, thalassemia trait, or hemoglobinopathy, we found that the lower limit of normal for MCV is 70 ft between 10 and 17 months of age and that there is a gradual increase of MCV with age; the lower limit is 74 between 1 1/2 and 4 years and 76 between 4 and 7 years. All of these values are well below the minimum adult level of 80 fl.

Anemia, Hypochromic↗
PubMed1975

Ferritin turnover in plasma: an opportunistic use of blood removed during exchange transfusion.

The concentration of plasma ferritin was measured in serial samples of blood removed from six preterm neonates undergoing exchange transfusion for hyperbilirubinemia. The average plasma ferritin concentration in the infants was 218 ng/ml compared with 47 ng/ml in the donors. The mean concentration of ferritin decreased an average of 62 ng/ml during the exchange transfusions. The plasma ferritin half-life was computed to be 2.5 and 5.5 min in the two infants weighing 2,000 and 2,500 g compared with a half-life of 4 min in the rat. In four infants weighing between 1,000 and 1,180 g, the half-life ranged from 9.1 to 34 min. These data support the concept of a rapid plasma ferritin turnover and indicate that ferritin may transport a large amount of iron through the plasma compartment in spite of its low concentration.

Birth Weight↗
PubMedDate not supplied

Factor IX inhibitors and anaphylaxis in hemophilia B.

PURPOSE: We present clinical and laboratory data on 18 children from 12 hemophilia treatment centers in the United States, Canada, and Europe with the purpose of disseminating information regarding a recently recognized, potentially life-threatening complication of treatment in very young children with hemophilia B. PATIENTS AND METHODS: Twelve hemophilia centers from the United States, Canada, and Europe provided clinical information and laboratory data concerning 18 children who had severe allergic reactions to infused factor (F) IX in close association with the development of an inhibitor to FIX. Laboratory testing for establishment of the diagnosis of hemophilia B and inhibitor to FIX was done locally at the centers treating these patients. FIX gene analysis was performed at one of six molecular genetics institutes. RESULTS: All 18 children had severe hemophilia B, and in each an inhibitor antibody to FIX developed. The median age at the time of anaphylaxis (or anaphylactoid reaction) was 16 months, and the median number of exposure days to FIX was 11. The FIX inhibitor was detected almost simultaneously with the first occurrence of anaphylaxis in 12 of 18 patients. Maximum inhibitor titers were 4.5-600 Bethesda units (BU), with a median titer of 48 BU. FIX gene analysis, performed in 17 of 18 patients, demonstrated complete deletion of the FIX gene in 10 and major derangements in seven. Immune tolerance induction (ITI) regimens have been attempted in 12 patients, with generally poor responses. Two of the 12 experienced nephrotic syndrome while on ITI. Recombinant FVIIa has been successfully used to treat bleeding episodes in 11 of these children. CONCLUSION: Physicians treating young children with hemophilia B should be aware of the potentially life-threatening complication of anaphylaxis. Children with complete gene deletions or major derangements of the FIX gene appear to be at greater risk. Those identified by genotype as being at greater risk may need to receive their first 10-20 treatments in a medical facility equipped for handling such emergencies. Recombinant FVIIa, although not licensed for use in the United States, appears to be the most suitable treatment option for bleeding episodes in such patients.

Anaphylaxis↗
PubMedDate not supplied

One course versus two courses of antithymocyte globulin for the treatment of severe aplastic anemia in children.

PURPOSE: The aim of the therapeutic trials was to optimize the treatment of severe aplastic anemia (SAA) and moderate aplastic anemia in children who lack a suitable bone marrow donor, using immunosuppressive therapy in the most effective combination and dose. PATIENTS AND METHODS: Two sequential therapeutic trials for the treatment of severe and moderate aplastic anemia in children were conducted by 10 institutions. The treatment protocols included antithymocyte globulin (ATG), prednisone, and cyclosporine A (CSA); patients entered on the first protocol, 0190 (ATG X 2), were given two courses of ATG, and those enrolled on the second protocol, 0190B (ATG X 1), were given only one course of ATG. Ten patients were evaluable on ATG X 2. All patients had SAA; three had hepatitis-induced severe aplastic anemia (HI-SAA). Twelve patients were evaluable on ATG X 1; all had SAA, one of whom had HI-SAA. RESULTS: Seven of 10 patients on ATG X 2 responded, and eight of 12 patients treated on ATG X 1 responded. CONCLUSION: Treatment with immunosuppressive therapy using ATG, CSA, and prednisone was very well tolerated. The response rates in both protocols were similar, and results compare favorably with those of previous therapeutic trials, suggesting that a second course of ATG is not necessary.

Adolescent↗