Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0017536 (giardiasis)
 1,714 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fifty patients with late-onset idiopathic immunoglobulin deficiency were studied and the frequency of various clinical associations and complications was observed. Men and women were equally affected, although the age at onset in men peaked in the third decade whereas it was more uniformly distributed in women. Sinobronchopulmonary infections were common and were caused by Haemophilus influenzae. Diplococcus pneumoniae, Streptococcus pyogenes and Staphylococcus aureus: bronchiectasis occurred in 28 per cent. Thirty patients (60 per cent) had diarrhea, which was often associated with steatorrhea, giardiasis, achlorhydria, abnormal Schilling tests and morphologic abnormalities on small bowel biopsy specimens, including nodular lymphoid hyperplasia; three patients had pernicious anemia. In the 20 patients without diarrhea these abnormalities were not observed except for giardiasis in one patient and achlorhydria in two patients. Cholelithiasis occurred in both groups in about a third of the patients tested. A high degree of susceptibility to neoplasia was noted. Thyroid abnormalities, including primary hypothyroidism and Graves' disease, were observed in six patients. Additional occasional findings were vitiligo, keratoconjunctivitis sicca and arthritis. Splenomegaly occurred in 14 (28 per cent) patients. The percentage of B lymphocytes in the blood was determined in 10 patients; it was normal or slightly decreased in eight patients and low in two patients.
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PMID:Idiopathic late-onset immunoglobulin deficiency. Clinical observations in 50 patients. 78 41

Agammaglobulinaemia is the most common of the primary immunodeficiencies. Three major types can be distinguished: X-linked agammaglobulinaemia, early-onset agammaglobulinaemia and late-onset agammaglobulinaemia. In X-linked agammaglobulinaemia, the molecular defect has been elucidated, and genetic counseling, prenatal diagnosis and carrier detection have become important issues. The pathogenesis of early- and late-onset agammaglobulinaemia is heterogeneous and usually not within the B-cell lineage. Patients with agammaglobulinaemia mainly suffer from infections caused by pneumococci or encapsulated Haemophilus influenzae located in the respiratory tract, paranasal sinuses, ears and meninges. Other prominent infections are Campylobacter jejuni bacteraemia and Giardia lamblia infection of the intestine. Among the more rare infections are those caused by Ureaplasma and Mycoplasma hominis. There is quite a number of non-infectious abnormalities which bother agammaglobulinaemic patients, especially those with late-onset agammaglobulinaemia. Of these, gastric carcinoma and intestinal lymphoma in late-onset agammaglobulinaemia and colorectal cancer in X-linked agammaglobulinaemia are the most dramatic. Life-threatening bacterial infections can largely be prevented by immunoglobulin substitution, even at relatively low dosages. However, insufficient immunoglobulin substitution is associated with recurrent airway infection and cumulative damage to the respiratory tract. for adequate substitution, efficacieous and safe intravenous immunoglobulin preparations are available. For selected patients (children, adults with poor venous access, and those experiencing side-effects on intravenous immunoglobulin), 16% immunoglobulin can be given by the subcutaneous route. With optimal substitution and--in the case of infection--adequate antimicrobial treatment, these patients have a good prognosis.
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PMID:Agammaglobulinaemia. 783 40

In this paper, clinical data of 49 adult patients with agammaglobulinaemia (syn. hypogammaglobulinaemia), 15 cases of X-linked agammaglobulinaemia (XLA) and 34 of common variable immunodeficiency (CVID) are reviewed. Although immunoglobulin substitution largely abolished life-threatening respiratory tract infections, considerable infectious and non-infectious morbidity was still encountered in these patients. Almost all patients suffered from chronic or recurrent upper and lower airway infections, mainly caused by Haemophilus influenzae and pneumococci. The lower respiratory tract infections led to cumulative damage to the respiratory tract, especially in XLA patients. Also the incidence of infections outside the respiratory tract (giardiasis, Campylobacter jejuni infections) was more common in XLA patients than in CVID patients. Nodular lymphoid hyperplasia was only found in CVID. A variety of other non-infectious complications were seen especially in CVID. Neoplastic complications occurred in nine patients (two cases of thymoma, two colorectal cancer, one gastric carcinoma, two haematological malignancies, two cases of skin cancer). Six patients died (five XLA patients and one CVID patient, from infectious and non-infectious causes).
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PMID:Hypogammaglobulinaemia: cumulative experience in 49 patients in a tertiary care institution. 1216 71