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PubMedDate not supplied

[Chronic diarrhea as a clinical expression of common variable hypogammaglobulinemia: apropos of 3 cases].

Sixty per cent of the patients with immuno-compromise of the humoral compartment may show chronic diarrhoea, specially seen in the common variable hypogammaglobulinemia (CVH). We presented 3 cases of late-onset CVH who referred chronic diarrhoea as the main symptom. The immunodiagnosis revealed undetectable or subnormal immunoglobulins levels (G, M, A), preserved total haemolytic complement and a negative autoimmune screening. The cell compartment analysis showed increased number of suppressor T lymphocytes (CD8) plus hyporesponsiveness of the total peripheral lymphocytes to polyclonal mitogens such PHA. The clinical approach of the CVH patients should be performed by a multispecialized team including the clinical immunologist and the gastrointestinal and infectious diseases specialists.

Adult↗
PubMed1989

[A case of hereditary angioneurotic edema associated with systemic lupus erythematosus].

A 33-year-old woman noticed recurrent and sudden attacks of subcutaneous swelling of the extremities and face since the age of 4 years. Sometimes the attacks involved colicky abdominal pain. Her mother and younger sister had episodes of recurrent swelling of the extremities as well. Complement studies revealed low CH50, C1q, C4, and C1 inhibitor levels, with normal C3 and C5 levels. Similar reductions of CH50 C4 and C1 inhibitor levels were observed in her mother, older, and younger sisters. Therefore, she was diagnosed as hereditary angioneurotic edema. In addition, she was diagnosed as having a butterfly rash at the age of 20 years and had a history of solar sensitivity. Histologically the facial lesion showed liquefaction degeneration of the basal cell layer. Direct immunofluorescent staining of the affected skin lesion showed basement membrane-zone staining of IgG and IgM. Laboratory studies revealed lymphopenia and positive ANF. On the basis of the above findings, hereditary angioneurotic edema associated with systemic lupus erythematosus was diagnosed.

Adult↗
PubMed1989

Kidney biopsy in SLE. I. A clinical-morphologic evaluation.

The relationship between renal morphology and clinical disease was analysed in 148 patients with SLE attending a lupus clinic. Patients were not selected for renal disease. Renal tissue was assessed according to the World Health Organization classification of lupus nephritis, the presence of active and chronic lesions was recorded and disease activity was measured according to a standard protocol. All sections of the classification were represented in this group of patients. Active and chronic lesions were more likely to occur among patients with Class III/IV (proliferative glomerulonephritis), than in any other category. Patients with Class III/IV biopsy were more likely to have evidence of clinical renal disease than patients in Class II (mesangial). However, almost half of the Class II patients had some evidence of renal disease, including elevated serum creatinine, as well as important non-glomerular lesions. Without biopsy they might have been thought to have proliferative lesions and been treated more aggressively. Two patients with proliferative glomerulonephritis had no clinical evidence of renal disease. Thus, at the time of biopsy results renal histological examination did not uniformly correlate with clinical renal disease.

Adolescent↗
PubMed1989

[Permanent decrease in immune function in patients with splenectomy for trauma].

The authors describe the peripheral blood analyses in patients splenectomized for trauma in consideration on the concentration of the immunoglobulins total complement CH50 levels, T and B lymphocyte populations and compare this with the control group. The levels of the IgM were significantly decreased (p0.001) in splenectomised while the levels of the IgA and IgG were significantly increased (for IgA - p0.001, for IgG p0.01). Total lymphocyte count averaged 54501999 in splenectomized, with number of T cells 2463930 and B lymphocyte 460236. The control group showed total lymphocyte count 460236. The control group showed total lymphocyte count 520235 (p0.001) with number of T cells 314147 (p0.001) and number of B cells 7434 (p0.001), what is significantly less than in splenectomized population. The level of total complement CH50 in splenectomized population was 11216 (p0.01), what is significantly less than in the control group 12515. These data demonstrate persistent abnormalities in immune function and suggest a possible explantation for the increased rizu of sepsis in this group of patients.

Adolescent↗
PubMed1995

Circulating auto-antibodies in Ménière's disease.

In the last years, it has been claimed that patients suffering from Menière's disease exhibit Circulating Immune Complexes, as well as free antibodies specifically directed against type II collagen, a component of the mammal inner ear. Furthermore, by means of repeated injections of type II collagen to the guinea-pig. Menière's disease has been reproduced. Taken together, all these features strongly suggest that Menière's disease fulfills most of the postulates that Witebsky established for the classification of auto-immune diseases. However, in spite of being a very well known marker of auto-immune conditions, non organ nor species specific Circulating Auto-Antibodies (CAA) have not been reported, to our knowledge, in Meniere's disease. Based upon this fact, Anti-Nuclear, Anti-Mitochondrial, Anti-Smooth Muscle, Anti-LKM and Anti-Parietal Gastric Cells Auto-Antibodies have been investigated in 49 patients diagnosed as having Meniere's disease according to the criteria of the AAOO. All of them were asymptomatic at the time of the study. Seventy eight serum samples from Normal Healthy Subjects (NHS) constituted the control group. CAA were tested by indirect immunofluorescence on rat organs according to the method described by Coons. Additionally, the seric levels of C3, and C4 as well as the haemolytic capacity (CH-50) of the serum samples were tested. Furthermore, Circulating Immune Complexes (CIC) as well as the Complement-Mediated Mechanisms involved in the clearance of CIC (Complement-Mediated Solubilization and Inhibition of the Immune Precipitation) were investigated in parallel.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

C5 deficiency in a patient with primary Sjögren's syndrome.

We report a case of C5 deficiency in combination with Sjögren's syndrome (SS). Our patient presented with polyarthritis and complaints of oral and ocular dryness. In the serum there was a very low titer of total hemolytic complement (CH50) due to a deficiency of the fifth complement component. C5 deficiency is often associated with recurrent life threatening infections, membranous glomerulonephritis, and discoid lupus erythematosus, but it has not been described in association with primary SS.

Adult↗
PubMed1995

Serum cryoglobulin and chronic hepatitis C virus disease among Japanese patients.

OBJECTIVES: Hepatitis C virus (HCV)-associated mixed cryoglobulins appear to be detected often in hepatitis C-related chronic liver disease. The association of the two phenomenon among Japanese patients is the subject of the present study. METHODS: Serum levels of total hemolytic complement (CH50) and anti-C3d-binding immune complex, as well as the prevalence of cryoglobulins, were studied in 213 patients with chronic liver disease (hepatitis C, 155; hepatitis B, 58). Cryoprecipitates were tested for anti-HCV Ab and HCV RNA. RESULTS: CH50 activity was significantly lower in patients with hepatitis C than in those with hepatitis B except in responders to interferon who showed a sustained loss of HCV RNA. Cryoglobulins were detected in 24 (37%) of 65 patients with hepatitis C; they generally consisted of polyclonal immunoglobulins but one case. Cryoglobulins were more frequently observed in cirrhotic patients and in those with a longer duration of disease. Cryoglobulinemia-related clinical signs such as vasculitis occurred in only three cases. Patients with cryoglobulins had lower CH50 activity and higher immune complex values than those without cryoglobulins. Anti-HCV Ab and HCV RNA were detected in all cryoprecipitates tested. CONCLUSIONS: These findings suggest that HCV is a major cause of cryoglobulins and advanced liver damage. However, serum cryoglobulins with polyclonal immunoglobulins appear to be less frequent among Japanese patients than among those studied in Western countries.

Adult↗
PubMed1994

[Evaluation of serum C3 and CH50 levels as markers of disease-activity and indicators of efficacy of treatment of lupus nephritis in childhood].

Serum levels of complement components (C3 and C4) and its activity (CH50) were analysed in 20 children with lupus nephritis from view points of disease-activity and efficacy of therapy. After the initiation of prednisolone or methylprednisolone pulse therapy for children in active phases the depressed C3 and CH50 levels were rapidly recovered to normal ranges within 4 months and 2 months, respectively. On the contrary serum C4 levels were normalized later in 11 months. Increased levels of antinuclear antibody and anti-DNA antibody titers have reciprocally dropped in response to those therapy. However, in 9 of 20 children the titers of antinuclear antibody and anti-DNA antibody remained high. Proteinuria observed in active phases of lupus nephritis has disappeared by therapy in all but two exceptions: these two children were complicated with nephrotic syndrome. Histological improvement after therapy was not observed (WHO classification). These findings suggested that it is useful to monitor serum C3 and CH50 levels but not C4 levels to judge disease-activity, and to evaluate efficacy of therapy of lupus nephritis in childhood.

Adolescent↗
PubMedDate not supplied

[Humoral immunologic changes in inflammatory colonic diseases].

We present the humoral immunological findings in 18 patients with ulcerative colitis and two with Crohn's disease. All of them had the disease for 3 months to five years. Fifteen were hispanics and five from European origin. There were ten males and ten females, and an average age of 38 years. All patients had active disease from clinical and endoscopy points of view. Thirteen patients had abnormal values in one of the immunoglobulins and normal in seven patients. IgA was increased in eleven, normal in seven and decreased in two. IgG increased in two, decreased in one and normal in seventeen cases. IgM was found to be increased in five and normal in the rest. IgA secretory determination in saliva, was performed twelve patients and was positive in all of them. Antinuclear antibodies were negative in eight. Anti-smooth muscle were positive in three of fifteen. Anti-mitochondrial were present in one and negative in thirteen. Values of C3 and C4 were lower than normal in 3 and 2 cases respectively from 4 cases studied. CH50 was decreased in 2 of 11 cases studied. In the patients, no correlation was found between low complement values, the positivity of auto antibodies and the clinical parameters previously mentioned. We conclude that there is quantitative alterations of the humoral response and it is necessary the immunological study in persons with inflammatory bowel disease others studies are needed to establish the real value of these findings.

Adolescent↗
PubMed1993

Total hemolytic complement (CH50) and its fractions C3 and C4 in the sera of patients with localized juvenile periodontitis.

The total hemolytic complement activity (CH50) and its fractions C3 and C4 levels were determined in the sera of 21 subjects with localized juvenile periodontitis (LJP). Total hemolytic complement activity was expressed as number of CH50 units per ml of blood, while C3 and C4 levels were assessed by radial immunodiffusion and expressed as mg%. The values were compared with an equal number of age- and sex-matched periodontally healthy controls. The results indicate a significant increase in CH50, C3, and C4 levels in the sera of patients with LJP. The study reveals the participation of complement system in the pathogenesis of LJP, and its assessment may assist in understanding the relative importance of complement activities in the disease process.

Adolescent↗
PubMed1993

[The boundary levels of laboratory data in autoimmune diseases].

The clinical significance of rheumatoid factor, antinuclear antibody, anti-thyroid antibody, CH50 and serologic tests for syphilis (STS) in boundary levels were investigated. The boundary levels were tentatively determined as: RF 5-20 U/ml, ANA 20-40X, anti-thyroid antibody 100-400X and CH50 25-30 U/ml. In STS, cases with biological false positive reaction were examined. In ninety percent of patients with positive anti-thyroid antibody, thyroid antibody, thyroid diseases were demonstrated. However, various disorders were involved at the boundary levels of rheumatoid factor, antinuclear antibody, CH50 and serologic tests for syphilis. To assess the boundary levels of these serological data, it seems important to observe serial data and other clinical and laboratory findings.

Adult↗
PubMed1993

Requirements for neutrophil products and L-arginine in ischemia-reperfusion injury.

Ischemia followed by reperfusion in rat limb results in evidence of vascular injury in the limb as well as in the lung as measured by leakage of [125I]albumin and extravasation of [51Cr] red blood cells. Vascular injury in lung and limb was proportional to the time of limb reperfusion and was associated with accumulation of myeloperoxidase, as well as evidence of complement consumption. In this model, the rank order of protective interventions was: neutrophil depletion > catalase + superoxide dismutase = allopurinol > dimethylthiourea = dimethylsulfoxide > deferoxamine = complement depletion. These data suggest that toxic oxygen products of neutrophils are related to the development of vascular injury. There was a reasonable correlation between protective effects of interventions and reduced tissue content of myeloperoxidase. Systemic treatment with the L-arginine antagonists, NG-monomethyl-L-arginine or nitro-L-arginine methyl ester, was also protective against vascular injury, suggesting that metabolic products of L-arginine participate in events leading to injury.

Animals↗
PubMed1993

[Complement analysis tests].

Although routine analysis of the complement system is rarely demanded, it is important from a diagnostical and therapeutical point of view to include the relevant tests when clinically required. Complement analysis should be performed when hereditary angioedema (C1 inhibitor deficiency) is suspected, and as part of investigation of patients with immunodeficiency (recurrent infections in childhood). Furthermore, complement analysis may be required in cases of recurrent systemic neisserial infections at any age, and to follow up certain autoimmune diseases. Only a few tests are available for routine purposes. These are reviewed briefly, with emphasis on collection and storage of samples prior to analysis. It is important that these guidelines be followed, to avoid misinterpretation of the results.

Angioedema↗
PubMed1993

[A case of severe milk allergy. Relationship with type I and Type III allergic reactions].

A case of cow's milk allergy related to type I and type III allergic reactions is reported. The patient is a one-month-old girl. She had watery diarrhea and abdominal distention soon after commencement of milk feeding two weeks after birth. She was cyanotic and floppy on admission. Intravenous complete hyperalimentation was tried to combat her diarrhea with good clinical effect. Then feeding with 605Z milk (enzyme digested cow's milk antigen, Meiji Milk Products Co., Ltd.) was started, and the diarrhea improved. Laboratory findings showed positive milk PK test, elevated IgE score, and positive milk specific RAST score, decreased serum complement, elevated levels of each immunoglobulin class of milk specific antibodies, especially milk specific IgG1 antibody and increased complement absorption test. She was diagnosed as having milk allergy related to both type I and type III allergies from the above-mentioned results. Specific antibodies were diminished after feeding with 605Z. Although her mother had not taken cow's milk over the past 10 years, she took a lot of milk during her pregnancy. But her serum milk specific antibodies were not elevated. It was suggested that the baby's antibodies were not derived from her mother but that the baby produced them by herself.

Animals↗
PubMed1995

[The use of measuring serum soluble IL-2 receptor levels in cyclosporine-A treated lupus nephritis].

Twenty-one patients with Lupus Nephritis (LN) were treated with cyclosporine-A (CsA) for three months. Serum soluble interleukin-2 receptors (SIL-2R) were measured before and after treatment and correlated prospectively with serologic findings of LN activity. The patients had significantly higher IL-2R level before treatment (during LN flare) than after treatment (disease remission). A significant positive correlation was present between serum SIL-2R and proteinuria, and ANA as well, whereas the SIL-2R levels in LN patients were inversely correlated with serum levels of C3 and CH50. These observations suggest that serum SIL-2R level is an useful marker of disease activity in LN and may serve as a helpful adjunct in the management of this disorder.

Adult↗
PubMed1996

Clinical use of centrifugal pumps and the roller pump in open heart surgery: a comparative evaluation.

Centrifugal pumps have been used widely as the main pump in open heart surgery to reduce damage to blood elements and to reduce the activation of the coagulation system. The purpose of this study was the evaluation and comparison of the effects of two types of centrifugal pumps and of one type of roller pump on blood elements, the coagulation system, complements, and immunoglobulins. Two types of centrifugal pumps (Lifestream; St. Jude Medical, Chelmsford, Massachusetts; and BP-80: Medtronic, BioMedicus, Inc., Eden Prairie, Minnesota, U.S.A.) and one roller pump (Mera Co.) were used separately as the main pump for cardiopulmonary bypass (CPB) in 29 patients. Platelet counts, lactate dehydrogenase, antithrombin III, thrombin-antithrombin complex (TAT), complements (C3, C4, and CH50) and immunoglobulins G, A, and M values were measured before and after CPB and compared. Values, except those for TAT, showed no significant difference among the three groups. The TAT values increased less in each of the centrifugal pump groups than in the roller pump group. This finding suggests that thrombin synthesis might be suppressed by the use of a centrifugal pump.

Adult↗
PubMed1996

[Clinical evaluation of immuno-serological laboratory data].

Clinical evaluations of various laboratory data from immuno-serological tests such as rheumatoid factor, anti-nuclear antibody, and other auto antibodies were reviewed. Rheumatoid factors (RF) were discussed in relation to positivity in various diseases, immunoglobulin class of RF, and correlation between titers of RF and circulating immune complex (IC). As a result, higher frequency and higher titers of IgA-RF were found in Sjögren syndrome patients. Titers of RF did not show disease activity of RA, but those of ESR and CRP did. Anti-nuclear antibodies (ANA) were discussed in relation to positivity in healthy subjects, specific antibodies and corresponding specific disease, correlation among titers of anti-dsDNA antibody, CH50 and circulating immune complex. As a result, an ANA frequency of 40% was found in healthy young women. Values of CH50 were much better than ANA titers for evaluating clinical activity in SLE patients. Findings of anti-cardiolipin antibody in thrombosis patients with connective tissue vascular disease (CVD), anti-centromere antibody in various CVD patients as well as CREST patients and primary biliary cirrhosis patients and anti-neutrophil cytoplasmic antibodies in various vascular diseases along with inflammatory bowel disease patients were presented. Finally, useful laboratory data at different clinical steps such as diagnosis, evaluation of disease activity and estimation of prognosis were demonstrated in CVD.

Adult↗