Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 70-year-old man presented with clonal large granular lymphocytosis of T-suppressor/cytotoxic immunophenotype, neutropenia, paraproteinemia, and proneness to infection. The patient became severely leukopenic during 14 days of chemotherapy with low-dose cyclophosphamide, and remained so after discontinuation of the drug. Clinically, he was thought to have prolonged chemotherapy-induced marrow hypoplasia. At death, 16 days after the last dose of chemotherapy, autopsy confirmed bone marrow hypoplasia and revealed that well-differentiated, polymorphous, and (immunophenotypically and genotypically) polyclonal B-lymphocytes predominated in normal hematopoietic and lymphoid organs. A similar lymphoid infiltrate was intimately associated with multiple ulcers and smooth muscle necrosis in the stomach. These terminal findings resemble B-lymphoproliferative conditions described in certain forms of immune deficiency.
 ...
PMID:Large granular lymphocytosis terminating in a polymorphous B-lymphocytic proliferation after low-dose cyclophosphamide therapy: a case report with necropsy findings. 133 79

Two unrelated patients with Hirschsprung disease and very short stature since birth are reported. Attention is drawn to the early roentgenographic signs of recessive McKusick metaphyseal chondrodysplasia syndrome or cartilage-hair hypoplasia syndrome. Some, but not all, patients with this chondrodysplasia exhibit anomalies of the hair, neutropenia and immune deficiency. Presence of congenital megacolon is apparently not exceptional. Nosologic limits are discussed.
 ...
PMID:[Autosomal recessive metaphyseal chondrodysplasia and Hirschsprung's disease]. 200 30

We reviewed 91 cases of pediatric Escherichia coli bacteremia during a 10-year period. Thirty one patients were afebrile; a significantly greater proportion of these patients were aged less than 1 month, had ampicillin-resistant E coli isolates, or had persistent bacteremia 24 hours after initiating antibiotic therapy. Infection was community acquired in 65 cases; associated urinary tract infection was six times more common in this setting than in nosocomially acquired infections. In 85 cases at least 1 underlying medical condition/focus of infection was identified at the time the positive blood culture was obtained, the most common were immune deficiency states (38 cases), urinary tract infection (29 cases), and lesions of the gastrointestinal tract (27 cases). Polymicrobial bacteremia occurred in five cases. Twelve patients died; significantly associated with death were hypotension requiring pressor therapy, presence of a central venous catheter, and neutropenia.
 ...
PMID:Escherichia coli bacteremia in children. A review of 91 cases in 10 years. 203 96

We report a case of cartilage-hair hypoplasia in a girl. The patient had short stature with short limbs and scalloped long bone metaphyses, sparse, poorly-pigmented hair, and increased susceptibility to infections. Several features of this case deserve attention: neutropenia, probably due to an autoimmune mechanism, was an initial manifestation; the immune deficiency (defective B and T cell immunity, autoimmune manifestations) was severe, and cytomegalovirus infection possibly aggravated the immune deficiency and hormone abnormalities. Whereas this patient's short stature is becoming more marked, prophylaxis of infections and administration of immune globulins have effectively prevented complications for the last two years.
 ...
PMID:[Hypoplasia of cartilage and hair with combined immune deficiency]. 275 30

Noma is an uncommon gangrenous process usually affecting malnourished children. A full-term neonate with orofacial noma, bilateral choanal atresia, and transient neutropenia with B cell deficiency is reported. This unusual appearance of noma in a well-nourished newborn might be related to the combination of choanal atresia and transient immune deficiency.
 ...
PMID:Noma in a full-term neonate. 387 41

Various combinations of immune deficiency, tumor effect, surgery, ionizing radiation, chemotherapy, and neutropenia result in infection-prone states for hospitalized cancer patients. Recognition of nosocomial infection may be difficult, and fever may be the only finding. Pathogens may be exogenously acquired from the contaminated hospital environment. Transmission by contact with hospital personnel is the usual mode of exogenous acquisition of hospital bacteria, and handwashing is the most effective means of prevention. The utility of conventional protective isolation in prevention of exogenous transmission is in question. Inattention to infection control measures by nurses and physicians may result in higher infection rates and more serious types of infections. Endogenous infection by the patient's own bacteria and fungi also occurs in the cancer ward. Autoinfection is "amplified" by the use of cannulae, catheters, and other hospital devices. Meticulous nursing care, particularly in neutropenic persons, is important in reducing the incidence of endogenous spread of microbes. Exogenous and endogenous infections in neutropenic patients are reduced using laminar air flow rooms combined with prophylactic antibiotics. It is still not clear if these expensive measures are effective in prolonging survival of patients with acute leukemia.
 ...
PMID:Prevention of infection on the oncology unit. 700 78

The effect of KW-2228, a derivative of recombinant human granulocyte colony stimulating factor, on neutropenia in children was studied in 23 cases of aplastic anemia, 13 cases of chronic benign neutropenia, 6 cases of congenital neutropenia (Kostmann type), 2 cases of cyclic neutropenia, 2 cases associated with glycogenosis type Ib and 1 cases associated with immune deficiency. KW-2228 was administered at 1-8 micrograms/kg subcutaneously and at 2-16 micrograms/kg intravenously. As a principle, the administration was started at low doses and continued for 7-28 days increasing the doses in the cases who didn't respond to the treatment well. The response rate of all the cases by the physicians in charge was 81. 8% (36/44). The mean absolute neutrophil count was increased from 304 to 1,300/microliters in aplastic anemia, from 204 to 3,027/microliters in chronic benign type, from 125 to 2,193/microliters in Kostmann type, and from 360 to 2,007 microliters in others. KW-2228 did not induce any noteworthy serious side effects. These results indicated that KW-2228 is a useful drug to treat neutropenia in children.
 ...
PMID:[Clinical study of recombinant human granulocyte-colony stimulating factor (KW-2228) in pediatric field. 1. Effectiveness on neutropenia by various causes and safety]. 768 91

The clinical and immunological aspects of 16 children with the syndrome of hypogammaglobulinaemia associated with normal or increased IgM (the hyper IgM syndrome) and their responses to treatment are reviewed. Increased concentrations of IgM, neutropenia, and recurrent infections could usually be controlled by antimicrobial and intravenous immunoglobulin treatment. Together with the bacterial infections characteristic of hypogammaglobulinaemia, these patients often developed opportunistic infections, including Pneumocystis carinii pneumonia, often presenting in the first year of life. The occurrence of sclerosing cholangitis, neurological complications, and neutropenia may be a result of an underlying cell mediated immune deficiency, autoimmunity, or infection. Despite a high incidence of opportunistic infections, immunological investigations did not show any abnormality of T cell function. These findings are discussed in the light of the recent demonstration that the lack of expression of a T lymphocyte activation antigen is the molecular basis of the X linked form of the disorder.
 ...
PMID:Hypogammaglobulinaemia associated with normal or increased IgM (the hyper IgM syndrome): a case series review. 794 38

This report describes a patient with combined immune deficiency associated with congenital neutropenia (CID/CN) and reports a partial characterization of his hematopoietic abnormalities. The CID/CN syndrome described is characterized by neutropenia and by deficiencies in B-lymphoid and T-lymphoid cell number and function. Red cell and platelet counts were normal. In vitro assays indicate that the myeloid lineage was developmentally arrested at the level of the committed monocyte/granulocyte progenitor (CFU-GM), while precursors to the CFU-GM progenitor were normal. In vitro studies showed that the defect in myeloid development was not corrected with G-CSF or GM-CSF. However, combinations of cytokines present in conditioned media from the T-cell lines MO or C5MJ, or defined multiple cytokine combinations containing IL-1, IL-3, GM-CSF, kit ligand, IL-6, and IL-9, restored myelopoiesis in-vitro. In contrast, C5MJ-conditioned media did not correct deficiencies in immune function in the patient's lymphocytes and accessory cells. No abnormalities in the production of G-CSF, GM-CSF, M-CSF, or IL-1 from the patient could be identified to account for the defects in myelopoiesis orimmune function.
 ...
PMID:Dyshematopoiesis in combined immune deficiency with congenital neutropenia. 825 11

A young man developed infectious mononucleosis complicated by profound anaemia due to haemolysis. The Donath Landsteiner antibody was found in his serum. He was treated successfully by blood transfusion but subsequently developed severe neutropenia. Both complications have been reported previously but not in the same individual. No underlying immune deficiency could be identified.
 ...
PMID:Infectious mononucleosis complicated by haemolytic anaemia due to the Donath Landsteiner antibody and by severe neutropenia. 834 76


1 2 3 4 5 Next >>