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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histologic, immunohistologic and electron microscopic findings in three children with primary immunodeficiencies are reported. Classical X-linked infantile agammaglobulinemia Bruton was present in case 1 (male, aged 16 years), selective cellular immunodeficiency with thrombopenia in case 2 (male, aged 2 1/2 years) and non-lymphopenic severe combined immunodeficiency in case 3 (male, aged 1 3/4 years). At autopsy, all three cases exhibited unusual types of pneumonia. In case 2 a generalized cytomegalovirus infection was present. Case 3 disclosed panmyelopathia and chronic liver lesions due to severe GvH-reaction subsequent to bone marrow transplantation. A detailed morphologic study of the immune system revealed distinct alterations in the thymus, spleen, and lymph nodes and the lymphatic tissues of the gastrointestinal tract characteristic of an immunodeficiency state, either humoral (case 1), cellular (case 2) or combined (case 3).
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PMID:Pathomorphology of humoral, cellular and combined primary immunodeficiencies. 19 90

A young man with X-linked chronic granulomatous disease of childhood, who is of the rare McLeod phenotype with antibodies in his serum shown to be hemolytic and reactive against all red cells with normal expressions of the Kell antigens, developed a severe Nocardia pneumonia with abscess formation and was subsequently treated successfully with granulocyte transfusions in spite of the presence of anti-KX in the patient's serum. The anti-KX did not appear to alter significantly the effectiveness of the transfused granulocytes; it did, however, cause a mild hemolytic transfusion reaction. The patient made a remarkable recovery from this episode and his condition has progressed to a state satisfactory enough for him to donate his own blood for storage and possible use in the future.
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PMID:Chronic granulomatous disease and the Mcleod phenotype. Successful treatment of infection with granulocyte transfusions resulting in subsequent hemolytic transfusion reaction. 83 62

Nocardia infections are infrequently recognized in humans. Nocardia species may cause severe life-threatening infections among immunocompromised patients and have been reported to cause actinomycotic mycetomas, primarily in tropical areas. Two severely immunocompromised patients had disseminated N. transvalensis infections. One had underlying X-linked variant chronic granulomatous disease and died of disseminated N. transvalensis infection, which was diagnosed only at postmortem examination. The second patient developed N. transvalensis pneumonia within 3 months of undergoing renal transplantation and died of disseminated mixed Pseudallescheria boydii and N. transvalensis infections. Thus, N. transvalensis may cause invasive and potentially fatal pulmonary and disseminated infections. Accordingly, clinical microbiology laboratories should become proficient in identifying this uncommon aerobic actinomycete.
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PMID:Disseminated Nocardia transvalensis infection: an unusual opportunistic pathogen in severely immunocompromised patients. 172 88

We described three patients with severe combined immunodeficiency disease (SCID) with B lymphocytes from a single family. Adenosine deaminase and purine nucleoside phosphorylase activities were normal. Two of them received bone marrow transplantation from an HLA haplotype-mismatched mother and an HLA-identical sibling, respectively, with successful immunological reconstitution. Another patient died of severe pneumonia. X-linked inheritance was suggested through the analysis of the pedigree extending four generations. This is probably the largest SCID kindred reported in Japan.
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PMID:A Japanese family pedigree of patients with severe combined immunodeficiency disease with X-linked inheritance. 192 Sep 12

An 18-year-old boy with X-linked chronic granulomatous disease (CGD) developed Aspergillus fumigatus pneumonia and multifocal osteomyelitis. Treatment with amphotericin B resulted in only moderate improvement of the lesions and was accompanied by considerable toxicity. In contrast, administration of the new triazole drug itraconazole led to complete disappearance of all signs of infection. We conclude that itraconazole may be a valuable new drug for treating invasive aspergillosis in patients with CGD, although the duration of treatment remains to be established.
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PMID:Treatment of invasive aspergillosis with itraconazole in a patient with chronic granulomatous disease. 215 38

A novel X-linked combined immunodeficiency disease was found in five living males in an extended family in the United States. The age of the affected males ranged from 2.5 to 34 yr. The most prominent clinical abnormalities were a paucity of lymphoid tissue; recurrent sinusitis, otitis media, bronchitis, and pneumonia; severe varicella; and chronic papillomavirus infections. The principal immunologic features of the disorder were normal concentrations of serum immunoglobulins but restricted formation of IgG antibodies to immunogens; normal numbers of B cells and NK cells but decreased numbers of CD4+ and CD8+ T lymphocytes, particularly the CD45RA+ subpopulations; diminished proliferative responses of blood T cells to allogeneic cells, mitogens and antigens; and decreased production of IL-2 by mitogen stimulated blood lymphocytes. Thus, affected males in this family carry an abnormal gene on their X chromosome that results in a combined immunodeficiency that is distinct from previously reported disorders.
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PMID:A novel X-linked combined immunodeficiency disease. 224 35

Authors described a new case of Coffin Lowry syndrome which presents a severe form, typical of males. Slight degree of affection in the mother and sister supports hypothesis of X-linked dominant trait. Authors want to underline normal appearance of the newborn period and progressive appearance of both neurological alterations and typical face of this condition. They have observed repetitive pneumonia probably produced by alimentary aspiration.
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PMID:[Coffin Lowry syndrome associated with repeated pneumonia]. 317 63

We describe a family in which two males and seven females have brown pigmentation of the skin. In the females, the type and distribution of the pigmentation mimicked incontinentia pigmenti; in the males, the pattern was reticulate. The histological appearance was the same in both sexes with amyloid deposits in the papillary dermis, melanin in the basal layer, and slight hyperkeratosis. The females were otherwise normal. Both males had thrived poorly as infants but had survived. One had severe gastroenteritis with blood in the stools starting at the age of three weeks followed by seizures, hemiplegia, and developmental delay; the other had recurrent pneumonia throughout life, a urethral stricture, inguinal herniae, and near-blindness from amyloid deposition in the cornea. Five other males in the family had had severe illnesses. Two died of pneumonia by three months. One died at three months from colitis. Both remaining boys had colitis as infants, failed to thrive, and developed recurrent pneumonia from which one died at three years. We think all of these relatives had the same disease carried by a single gene with pleiotropic effects. The most likely form of inheritance is X-linked.
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PMID:Familial cutaneous amyloidosis with systemic manifestations in males. 679 69

Six cases of chronic granulomatous disese (CGD), three of which correspond to the X-linked genetic form and the three other to the autosomic recessive type are reported. The fact of half of the patients being females is relevant as only 24 are cited by Klebanoff and Clark in their revision in 1978. X-linked CGD: The three patients, two of them brothers, presented their first manifestations in the first year of life; in one of the BCG given at one week of life resulted in adenitis of protracted course with calcificaton. The clinical course has been very severe in two of them. At the present time the patients are 15, 11 and 9 years old. Functional studies have shown very low values in NBT tests, O2 consumption, iodination and bactericidal activity in all three. Intermediate values in the mothers and normal values in the fathers were found. Autosomal CGD: Of our three patients, two were sisters. The first manifestations appeared during the first thrimester of life. The eldest had hepatic and pulmonary granulomata at three years old. At five years, she presented an intestinal obstruction syndrome with gastric antral, duodenal and ileal stenosis caused by intramural granulomata and inflammation; she died of pneumonia shortly after. Her sister had dermatitis, hepatic abscess, pneumonia, adenitis and osteomyuelitis of the ribs; she died at six years old after a bronchopneumonia. Last patient had a sister who died at two years old affected probably gy CGD. At present our patient is 17 months old and so far had recurrent otitis, adenitis, a pneumonia and, recently, hepatic granulomata have been found. Fonctional studies in the two sisters showed similar alterations as those of the three boys. In this patient an alteration of chemotaxis of cellular origen was found as well.
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PMID:[Chronic granulomatous disease: clinical and functional studies in six cases (author's transl)]. 740 65

A 31-year-old patient was recurrently admitted because of pneumonia. Specialised leukocyte function tests revealed the diagnosis of an X-linked type of chronic granulomatous disease. Treatment with interferon-gamma successfully prevented new infections.
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PMID:Unexplained recurrent pneumonia: a post-childhood case of chronic granulomatous disease. 776 Sep 70


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