Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: UMLS:C0038002 (splenomegaly)
9,873 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

1. Laennec's lung disease lasted for at least 20 years. Its stigmata included chronic cough, sputum production and intermittent wheeze. 2. Laennec had long term stigmata commonly associated with chronic bronchiectasis, sinusitis, physical frailty, and short stature (5ft 2in). 3. Chronic diarrhoea of at least 20 years duration is not strongly associated with tuberculosis. 4. During Laennec's last illness his physicians equivocated as to whether he had respiratory disease at all. Bronchial breathing at the apex, if indeed present, could have been caused by compensatory emphysema secondary to middle lobe bronchiectasis rather than to active tuberculosis. 5. Laennec did not have haemoptysis in his final illness. 6. Laennec's last illness, a wasting illness characterised by intermittent fevers, cardiac murmur, and persistent tachycardia followed a dental manipulation. The painful "abdominal abscess" noted by Laennec's colleagues may actually have been splenomegaly. These features suggest endocarditis. The cardiac murmurs associated with pulmonary hypertension secondary to bronchiectasis are not usually audible at a remote distance from the patient. Endocarditis was a disease largely unknown to physicians of the early 19th century before Osler clarified its pathology in the 1880s.
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PMID:Rene Laennec: his brilliant life and tragic early death. 266 72

We report the clinical features and outcome of 16 patients with cryoglobulinaemia. Two patients with Type I cryoglobulinaemia both had IgG kappa monoclonal paraproteins. Nine of 10 with Type II disease had monoclonal IgM kappa and polyclonal IgG; one had monoclonal IgG kappa and polyclonal IgG in the cryoglobulin. Underlying disorders identified in 3 of the 4 Type III patients were Sjogren's syndrome, infective endocarditis, and non-A non-B hepatitis and HTLV III infection. The commonest presenting features were rash in 94 p. 100 (ulceration 25 p. 100), arthralgia in 63 p. 100 (erosive arthritis 32 p. 100), renal disease in 63 p. 100, neurological involvement in 56 p. 100, hepatomegaly in 32 p. 100 and splenomegaly in 32 p. 100. Major associated conditions were progressive bronchiectasis in one case, and severe peripheral vascular disease in another; underlying malignancy was found in 2 cases (lymphoma and malignant melanoma). Treatment was with plasma exchange (PE) and immunosuppressive drugs (ID) in 10, PE alone in 3, ID alone in 2 and antibiotics [corrected] in 1. Fourteen of 16 patients showed an initial clinical response and fall in cryoglobulin levels. Four patients have died, one each from gastro-intestinal haemorrhage, sepsis, pulmonary embolism and lymphoma. Of the remaining 12 patients, all are symptomatically controlled and 10 have persisting cryoglobulinaemia (3 on PE and ID, 2 on PE, 2 on ID and 3 on no treatment). Of the two cases in whom cryoglobulinaemia resolved, one (Type II) had received PE and ID and the other (Type III) had been treated with antibiotics and surgery for infective endocarditis.
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PMID:Cryoglobulinaemia: clinical features and response to treatment. 376 96

The computed tomography (CT) scans of 37 patients with primary hypogammaglobulinaemia were reviewed to determine the frequency of enlarged mediastinal lymph nodes and splenomegaly in this group. None of the 10 X-linked Agammaglobulinaemia (XLA) patients had enlarged nodes and only one had splenomegaly. Eleven of the 27 Common Variable Immunodeficiency (CVID) group had enlarged nodes (41%) and 13 had splenomegaly (48%). There was no significant correlation between the presence of enlarged nodes and splenic enlargement. Twenty-two patients had bronchiectasis but the presence of bronchiectasis did not correlate with the presence of either splenomegaly or lymphadenopathy. Three to 6 years follow-up is available for 36 of the 37 patients and none of this group have developed lymphoma or other malignancy. Enlarged mediastinal nodes and/or splenomegaly are frequently found in patients with CVID and are usually due to a benign, non-neoplastic, process. Mediastinal lymph node enlargement is not a feature of XLA and splenomegaly is unusual in this condition.
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PMID:Mediastinal lymph node enlargement and splenomegaly in primary hypogammaglobulinaemia. 761 97

Serum immunoreactive interleukin (IL-)1 alpha, IL-4, IL-6 and tumor necrosis factor (TNF) alpha were measured in 42 patients with primary hypogammaglobulinemia (25 common variable immunodeficiency (CVI), 10 congenital hypogammaglobulinemia (CH), 7 X-linked agammaglobulinemia (XLA), and in 21 healthy controls. The cytokine levels were correlated to other immunological parameters including serum levels of neopterin and soluble CD8 (sCD8) antigen. IL-6 was detectable in 48% and IL-4 in 36% of the CVI patients, but in none of the controls. Seventy-five percent of the CVI patients with elevated IL-4 levels had detectable IL-6. In contrast, no patients in the XLA group and only three CH patients had detectable IL-4 or IL-6 levels. TNF alpha and IL-1 alpha were detected in only a few serum samples with no significant differences between patients and controls. In the CVI group elevated IL-6 levels were significantly associated to reduced numbers of CD4+ and CD19+ lymphocytes, elevated levels of neopterin and sCD8 antigen, and occurrence of splenomegaly and bronchiectasis. The raised IL-6 levels were confirmed in longitudinal testing, probably reflecting a characteristic immunological dysregulation in these patients. Cytokine alterations may play a role in the pathogenesis of the immunodeficiency and for the clinical manifestations in CVI patients. Alternatively, elevated cytokine levels may be only a marker of chronic immune activation, particularly in monocytes, possibly delineating a distinct subgroup of patients within the heterogeneous CVI group.
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PMID:Elevated serum levels of interleukin-4 and interleukin-6 in patients with common variable immunodeficiency (CVI) are associated with chronic immune activation and low numbers of CD4+ lymphocytes. 790 14

Cystic fibrosis (CF) is an autosomal recessive disorder that is characterized by an abnormality of exocrine gland function. Adult patients represent a rapidly growing percentage of the CF population. Pulmonary changes are seen in nearly every case and are the most serious complication of CF. In advanced lung disease, bronchiectasis, emphysematous bullae, and subpleural blebs can frequently develop. Although pulmonary disease is the most common cause of death and morbidity among CF patients, there also can be involvement of other groups, particularly in adults, which show characteristic signs on CT and spiral CT. Pancreatic abnormalities are present in 85-90% of CF patients. The degree of pancreatic involvement varies, ranging form accumulations of mucus in the small ducts to totally plugged ducts, which can cause atrophy of the exocrine glands and progressive fibrosis. Pancreatic dysfunction on CT is demonstrated as fatty replacement and fibrosis of the pancreas. However, there may be scattered foci of pancreatic calcifications that can be detectable on plain radiographs. Hepatobiliary involvement follows the same pattern as pancreatic abnormalities. Bile canaliculi are plugged by mucinous material and when this plugging is of long duration, biliary cirrhosis with diffuse hepatic nodularity may develop. Such severe hepatic involvement is see in only about 2-5% of patients, although minor hepatic alterations, such as diffuse fatty changes, are fairly common. Hepatobiliary involvement is characterized by hepatic nodularity, compatible with cirrhosis, splenomegaly, and ascites. Complete obstruction of the ileum by meconium occurs in approximately 10% of newborns with CF. Intestinal findings on CT include obstruction, although this is more common in children. These CT signs should be evaluated carefully in adult patients since they may be suggestive of CF. Computed tomography offers unique information about organ involvement (other than pulmonary) that can alter diagnosis and patient management.
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PMID:Cystic fibrosis: spectrum of thoracic and abdominal CT findings in the adult patient. 975 98

Although idiopathic humoral immunodeficiencies are arbitrarily classified into specific antibody deficiency (SAD) or common variable immunodeficiency (CVID), this distinction does not accurately predict the risk of the bronchiectasis, one of the major long-term clinical complications in these patients. In this study, clinical complications were compared with laboratory markers of cellular and humoral immunity in fifty-five consecutive patients (27 children and 28 adults) attending regional immunology clinics in Manchester, United Kingdom. Reduced CD19(+)CD27(+)IgD(-) B cell percentage but not serum immunoglobulin levels or classification of patients into SAD and CVID was associated with a significantly higher prevalence of bronchiectasis (OR 0.4 (0.2-0.8), P = 0.001), splenomegaly (OR 0.2 (0.1-0.5), P = 0.001) and autoimmunity (OR 0.4 (0.2-0.7), P = 0.003). We conclude that in patients with idiopathic humoral immunodeficiencies assessment of B cell switching more accurately predicts clinical prognosis than either classification of patients into SAD and CVID or serum immunoglobulin concentrations.
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PMID:Memory switched B cell percentage and not serum immunoglobulin concentration is associated with clinical complications in children and adults with specific antibody deficiency and common variable immunodeficiency. 1678 7

The paper describes one case of the mixed form of mucoviscidosis, with involvement of the lung (chronic obstructive bronchitis, bronchiectasis, emphysema, pneumosclerosis), liver (cirrhosis), pancreas (lipomatosis with islet atrophy), ileum (enteritis). There were the following complications: cor pulmonale, esophageal varicose veins, ascitis, splenomegaly, secondary diffuse renal amyloidosis of the kidney, adrenals, and spleen, and brain edema. Due to modern therapy the patient reached the age of 25 years, which is a rarity.
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PMID:[Pathomorphology of the mixed form of mycoviscidosis in an adult patient]. 1683 Jun 24

Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by hypogammaglobulinemia, defective specific-antibody production resulting in recurrent bacterial infections. Delay in diagnosis and inadequate treatment result in increased irreversible complications and mortality. To determine persistent morbidities, mortality rate and survival in Iranian patients with CVID, hospital records of 72 (39 males and 33 females) diagnosed CVID patients were reviewed. Probabilities of survival after diagnosis of CVID were estimated from Kaplan-Meier life tables. Studied patients were enrolled over a 20-year period (1984-2005). The most commonly observed complication was bronchiectasis (24 cases), followed by splenomegaly, intestinal villous atrophy (11 cases), and failure to thrive (10 cases). Post-diagnosis survival was estimated as 65% for the first 6.5 years, which remains the same until 14 years after diagnosis when the survival curve drops to nearly 45%. The mortality rate among patients who had no regular visits and did not receive periodical IVIG was more remarkable when compared with those who had been followed up timely (p-value = 0.001). The most common cause of death was respiratory failure. Based on our observation, it can be highlighted that all patients with CVID, even under regular immunoglobulin replacement, need close monitoring for early detection of complications and introduction of appropriate management.
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PMID:Mortality and morbidity in common variable immunodeficiency. 1716 33

Common variable immunodeficiency (CVID) is a heterogeneous syndrome characterized by defective immunoglobulin production and high frequency of bacterial infections, autoimmunity and manifestations of chronic inflammation. Abnormalities of CD4+CD25high forkhead box P3 (FoxP3)+ regulatory T cells (Treg) have been associated with autoimmune and inflammatory disorders, and we hypothesized that CVID might be characterized by Treg abnormalities. CD3+ cells from patients and controls were analysed for the expression of FoxP3 mRNA by real time reverse transcription-polymerase chain reaction (RT-PCR). Peripheral blood mononuclear cells from CVID patients and controls were stained for Treg markers, analysed by flow cytometry and compared to clinical characteristics. The main findings were: (i) CVID patients had significantly decreased expression of FoxP3 mRNA and decreased proportions of CD4+CD25highFoxP3+ cells compared to controls; (ii) CVID patients with splenomegaly had even lower proportions of Treg compared to other patients and controls; (iii) serum levels of the inflammatory marker neopterin were correlated negatively with the proportions of Treg within the CVID population, while there was no significant association with bronchiectasis. We have demonstrated decreased proportions of Treg in CVID patients, particularly in those with signs of chronic inflammation. Decreased proportions of TReg are suggested to be pathogenetically important in autoimmunity, and our results suggest that TReg may have a similar role in CVID.
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PMID:Low numbers of regulatory T cells in common variable immunodeficiency: association with chronic inflammation in vivo. 1730 2


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