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Papers by Morton Cowan

Research paper thumbnail of Stem cell transplantation for primary immunodeficiency diseases

Current Opinion in Allergy and Clinical Immunology, Dec 1, 2014

Research paper thumbnail of A defective Vkappa A2 allele in Navajos which may play a role in increased susceptibility to haemophilus influenzae type b disease

Journal of Clinical Investigation, May 15, 1996

Research paper thumbnail of Hematopoietic Stem Cell Transplantation Activity in Pediatric Cancer between 2008 and 2014 in the United States: A Center for International Blood and Marrow Transplant Research Report

Biology of Blood and Marrow Transplantation, Aug 1, 2017

Research paper thumbnail of Restoration of in Vitro Function of Adenosine 41 Deaminase (Ada) Deficient Lymphocytes by INTER-LEUKIN-2 (IL-2): 41

Pediatric Research, Jul 1, 1985

Research paper thumbnail of Successful Use of Cryopreservation and Shipping of CD34-Selected Mismatched Related Donor Grafts for Treatment of Children

Research paper thumbnail of T Cell and B Cell Immunity can be Reconstituted with Mismatched Hematopoietic Stem Cell Transplantation Without Alkylator Therapy in Artemis-Deficient Mice Using Anti-Natural Killer Cell Antibody and Photochemically Treated Sensitized Donor T Cells

Biology of Blood and Marrow Transplantation, Feb 1, 2012

Research paper thumbnail of Lentivector cryptic splicing mediates increase in CD34+ clones expressing truncated HMGA2 in human X-linked severe combined immunodeficiency

Nature Communications, Jun 28, 2022

Research paper thumbnail of Application of a radiosensitivity flow assay in a patient with DNA ligase 4 deficiency

Blood Advances, Jul 30, 2018

Research paper thumbnail of New Insights Into Multicenter PICU Mortality Among Pediatric Hematopoietic Stem Cell Transplant Patients*

Critical Care Medicine, Sep 1, 2015

Research paper thumbnail of Elevated Erythrocyte Adenosine Deaminase (Ada) Activity in Acquired Immunodeficiency Disease (Aids): 40

Pediatric Research, Jul 1, 1985

Research paper thumbnail of 962 Restoration of in Vitro Function of Adenosine Deaminase (Ada) Deficient Lymphocytes by INTER-LEUKIN-2 (IL-2)

Pediatric Research, Apr 1, 1985

We have previously reported abnormalities in the membrane glycoproteins of the T lymphocytes and ... more We have previously reported abnormalities in the membrane glycoproteins of the T lymphocytes and platelets of WAS patients. Deficiencies in a 115,000 dalton lymphocyte glycoprotein (GPL-115) appear to be diagnostic of WAS. To determine if the membrane glycoprotein abnormalities have morphological consequences, normal and WAS peripheral blood lymphocytes (PBL) and thymocytes were fi xed in 1.2% glutaraldehyde and examined by scanning electron microscopy (SEM). Using a l to 4 grading scale based upon the character of the lymphocyte surface projections (4 • villus projections on > 75% of lymphocyte surface area; 3 • vi llus projections on < 75% of surface area; 2 • ridge projec-

Research paper thumbnail of Postnatal lung growth after repair of diaphragmatic hernia

Thorax, Jun 1, 1979

The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphr... more The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphragmatic hernia on the first day of life were examined. Lung volumes were appropriate for the size of the children, and the ratio of right lung volume to left lung volume was also normal in both patients. The lungs, however, were grossly abnormal with evidence of enlargement and destruction of respiratory tissue. The left lung was affected more than the right in both subjects. In one patient the total number of alveoli in the lungs was similar to that of normal children of the same age, but this was because the right lung had more than twice as many alveoli as the left lung. It appears that alveolar multiplication is impaired after repair of diaphragmatic hernia. The number of bronchioles was reduced in the infant with very few alveoli, and there may have been too few bronchioles in the other patient.

Research paper thumbnail of Clinical and laboratory findings in childhoodmixed connective tissue disease: Presence of antibody to ribonucleoprotein containing the small nuclear ribonucleic acid U1

The Journal of Pediatrics, 1983

Research paper thumbnail of Postnatal lung growth after repair of diaphragmatic hernia

Journal of Pediatric Surgery, 1980

The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphr... more The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphragmatic hernia on the first day of life were examined. Lung volumes were appropriate for the size of the children, and the ratio of right lung volume to left lung volume was also normal in both patients. The lungs, however, were grossly abnormal with evidence of enlargement and destruction of respiratory tissue. The left lung was affected more than the right in both subjects. In one patient the total number of alveoli in the lungs was similar to that of normal children of the same age, but this was because the right lung had more than twice as many alveoli as the left lung. It appears that alveolar multiplication is impaired after repair of diaphragmatic hernia. The number of bronchioles was reduced in the infant with very few alveoli, and there may have been too few bronchioles in the other patient.

Research paper thumbnail of Biotin‐responsive multiple carboxylase deficiency in an 8‐year‐old boy with normal serum biotinidase and fibroblast holocarboxylase‐synthetase activities

Journal of Inherited Metabolic Disease, 1988

An 8-year-old boy with late onset multiple carboxylase deficiency is described. Biotinidase defic... more An 8-year-old boy with late onset multiple carboxylase deficiency is described. Biotinidase deficiency and holocarboxylase-synthetase deficiency have been excluded. A very slow biochemical response to biotin was found. The decrease in urinary organic acid excretion followed first-order kinetics with a half-life of about 50 days. The initially low carboxylase activities in thrombocytes were increased but not normalized after 3 months of treatment.

Research paper thumbnail of Total body irradiation and bone marrow transplantation using CT-based planning

Research paper thumbnail of Hematopoietic stem cell transplantation activity in Europe 1999

Bone Marrow Transplantation, 2001

Research paper thumbnail of Autoimmune hemolytic anemia in patients with systemic lupus erythematosus

The American Journal of Medicine, 2000

Research paper thumbnail of Elevated Erythrocyte Adenosine Deaminase Activity in Down's Syndrome

Research paper thumbnail of Chronic active Epstein‐Barr virus infection in children in Japan

Acta Paediatrica, 1995

The patients with chronic active Epstein‐Barr virus infection (CAEBV) in childhood in Japan are d... more The patients with chronic active Epstein‐Barr virus infection (CAEBV) in childhood in Japan are described. Among 39 registered cases, 20 patients were males and 19 were females. Unlike the X‐linked lymphoproliferative syndrome, there was no hereditary background. The incidence of hypersensitivity to mosquito bites was high (31.3%) as a past history. Most patients exhibited hepatomegaly (92.3%), splenomegaly (87.2%) and fever (84.6%). The incidence of absent anti‐EB virus nuclear antigen titres was unexpectedly low (17.1%). Lymphoreticular disorders and cardiovascular diseases were major complications. Twenty‐four (61.5%) patients died 6 months to 8 years after the onset, mainly of hepatic failure (eight cases), cardiac failure (five cases), virus‐associated haemophagocytic syndrome (three cases) and haematological malignancies (two cases). This study reveals that CAEBV in Japan has several clinical features and should be informative for the pathogenesis of EB virus.

Research paper thumbnail of Stem cell transplantation for primary immunodeficiency diseases

Current Opinion in Allergy and Clinical Immunology, Dec 1, 2014

Research paper thumbnail of A defective Vkappa A2 allele in Navajos which may play a role in increased susceptibility to haemophilus influenzae type b disease

Journal of Clinical Investigation, May 15, 1996

Research paper thumbnail of Hematopoietic Stem Cell Transplantation Activity in Pediatric Cancer between 2008 and 2014 in the United States: A Center for International Blood and Marrow Transplant Research Report

Biology of Blood and Marrow Transplantation, Aug 1, 2017

Research paper thumbnail of Restoration of in Vitro Function of Adenosine 41 Deaminase (Ada) Deficient Lymphocytes by INTER-LEUKIN-2 (IL-2): 41

Pediatric Research, Jul 1, 1985

Research paper thumbnail of Successful Use of Cryopreservation and Shipping of CD34-Selected Mismatched Related Donor Grafts for Treatment of Children

Research paper thumbnail of T Cell and B Cell Immunity can be Reconstituted with Mismatched Hematopoietic Stem Cell Transplantation Without Alkylator Therapy in Artemis-Deficient Mice Using Anti-Natural Killer Cell Antibody and Photochemically Treated Sensitized Donor T Cells

Biology of Blood and Marrow Transplantation, Feb 1, 2012

Research paper thumbnail of Lentivector cryptic splicing mediates increase in CD34+ clones expressing truncated HMGA2 in human X-linked severe combined immunodeficiency

Nature Communications, Jun 28, 2022

Research paper thumbnail of Application of a radiosensitivity flow assay in a patient with DNA ligase 4 deficiency

Blood Advances, Jul 30, 2018

Research paper thumbnail of New Insights Into Multicenter PICU Mortality Among Pediatric Hematopoietic Stem Cell Transplant Patients*

Critical Care Medicine, Sep 1, 2015

Research paper thumbnail of Elevated Erythrocyte Adenosine Deaminase (Ada) Activity in Acquired Immunodeficiency Disease (Aids): 40

Pediatric Research, Jul 1, 1985

Research paper thumbnail of 962 Restoration of in Vitro Function of Adenosine Deaminase (Ada) Deficient Lymphocytes by INTER-LEUKIN-2 (IL-2)

Pediatric Research, Apr 1, 1985

We have previously reported abnormalities in the membrane glycoproteins of the T lymphocytes and ... more We have previously reported abnormalities in the membrane glycoproteins of the T lymphocytes and platelets of WAS patients. Deficiencies in a 115,000 dalton lymphocyte glycoprotein (GPL-115) appear to be diagnostic of WAS. To determine if the membrane glycoprotein abnormalities have morphological consequences, normal and WAS peripheral blood lymphocytes (PBL) and thymocytes were fi xed in 1.2% glutaraldehyde and examined by scanning electron microscopy (SEM). Using a l to 4 grading scale based upon the character of the lymphocyte surface projections (4 • villus projections on > 75% of lymphocyte surface area; 3 • vi llus projections on < 75% of surface area; 2 • ridge projec-

Research paper thumbnail of Postnatal lung growth after repair of diaphragmatic hernia

Thorax, Jun 1, 1979

The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphr... more The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphragmatic hernia on the first day of life were examined. Lung volumes were appropriate for the size of the children, and the ratio of right lung volume to left lung volume was also normal in both patients. The lungs, however, were grossly abnormal with evidence of enlargement and destruction of respiratory tissue. The left lung was affected more than the right in both subjects. In one patient the total number of alveoli in the lungs was similar to that of normal children of the same age, but this was because the right lung had more than twice as many alveoli as the left lung. It appears that alveolar multiplication is impaired after repair of diaphragmatic hernia. The number of bronchioles was reduced in the infant with very few alveoli, and there may have been too few bronchioles in the other patient.

Research paper thumbnail of Clinical and laboratory findings in childhoodmixed connective tissue disease: Presence of antibody to ribonucleoprotein containing the small nuclear ribonucleic acid U1

The Journal of Pediatrics, 1983

Research paper thumbnail of Postnatal lung growth after repair of diaphragmatic hernia

Journal of Pediatric Surgery, 1980

The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphr... more The lungs of two patients who died eight months and 64 months after repair of a left-sided diaphragmatic hernia on the first day of life were examined. Lung volumes were appropriate for the size of the children, and the ratio of right lung volume to left lung volume was also normal in both patients. The lungs, however, were grossly abnormal with evidence of enlargement and destruction of respiratory tissue. The left lung was affected more than the right in both subjects. In one patient the total number of alveoli in the lungs was similar to that of normal children of the same age, but this was because the right lung had more than twice as many alveoli as the left lung. It appears that alveolar multiplication is impaired after repair of diaphragmatic hernia. The number of bronchioles was reduced in the infant with very few alveoli, and there may have been too few bronchioles in the other patient.

Research paper thumbnail of Biotin‐responsive multiple carboxylase deficiency in an 8‐year‐old boy with normal serum biotinidase and fibroblast holocarboxylase‐synthetase activities

Journal of Inherited Metabolic Disease, 1988

An 8-year-old boy with late onset multiple carboxylase deficiency is described. Biotinidase defic... more An 8-year-old boy with late onset multiple carboxylase deficiency is described. Biotinidase deficiency and holocarboxylase-synthetase deficiency have been excluded. A very slow biochemical response to biotin was found. The decrease in urinary organic acid excretion followed first-order kinetics with a half-life of about 50 days. The initially low carboxylase activities in thrombocytes were increased but not normalized after 3 months of treatment.

Research paper thumbnail of Total body irradiation and bone marrow transplantation using CT-based planning

Research paper thumbnail of Hematopoietic stem cell transplantation activity in Europe 1999

Bone Marrow Transplantation, 2001

Research paper thumbnail of Autoimmune hemolytic anemia in patients with systemic lupus erythematosus

The American Journal of Medicine, 2000

Research paper thumbnail of Elevated Erythrocyte Adenosine Deaminase Activity in Down's Syndrome

Research paper thumbnail of Chronic active Epstein‐Barr virus infection in children in Japan

Acta Paediatrica, 1995

The patients with chronic active Epstein‐Barr virus infection (CAEBV) in childhood in Japan are d... more The patients with chronic active Epstein‐Barr virus infection (CAEBV) in childhood in Japan are described. Among 39 registered cases, 20 patients were males and 19 were females. Unlike the X‐linked lymphoproliferative syndrome, there was no hereditary background. The incidence of hypersensitivity to mosquito bites was high (31.3%) as a past history. Most patients exhibited hepatomegaly (92.3%), splenomegaly (87.2%) and fever (84.6%). The incidence of absent anti‐EB virus nuclear antigen titres was unexpectedly low (17.1%). Lymphoreticular disorders and cardiovascular diseases were major complications. Twenty‐four (61.5%) patients died 6 months to 8 years after the onset, mainly of hepatic failure (eight cases), cardiac failure (five cases), virus‐associated haemophagocytic syndrome (three cases) and haematological malignancies (two cases). This study reveals that CAEBV in Japan has several clinical features and should be informative for the pathogenesis of EB virus.

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