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Results for “Common Variable Immunodeficiency”

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PubMed1997

Prolonged poliovirus excretion in an immunodeficient person with vaccine-associated paralytic poliomyelitis.

Recently completed molecular studies of poliovirus isolates suggest that viral replication of vaccine-related polioviruses may have persisted for as long as 7 years in a patient with vaccine-associated paralytic poliomyelitis (VAPP) in whom common variable immunodeficiency syndrome (CVID) previously had been diagnosed. This report summarizes the clinical and virologic data and discusses the possible implications of these new findings for the global polio eradication initiative, which include how and when to discontinue vaccination when polio has been eradicated.

Adolescent↗

PubMed2005

Cutaneous granulomas associated with primary immunodeficiency disorders.

Cutaneous granulomas are uncommon in primary immunodeficiency disorders. We report cutaneous granulomas in a child with ataxia telangiectasia (AT) and compare the clinical course with similar lesions in an adult with common variable immunodeficiency (CVI). A 4-year-old female with AT developed cutaneous granulomas as erythematous plaques. The largest lesion appeared on her left cheek and continued to progress despite treatment with topical and intralesional steroids. Disease control was obtained initially with oral antibiotics and low-dose oral steroids. On cessation of oral steroids, significant relapse of the facial granuloma occurred. Pulsed and then oral steroids were required to stop the disease process leaving significant scarring. The second case is of cutaneous granulomas in a 66-year-old man, with CVI, who presented with an erythematous reticulate rash on the legs. We consider it useful to report this patient here as disease control was obtained in a similar way with systemic immunosuppression. In this patient a combination of oral steroids and azathioprine was used. These cutaneous granulomas are thought to be a manifestation of immune dysregulation. No infectious cause has been found so far. We recommend the use of broad-spectrum antibiotics in conjunction with systemic steroids for progressive granulomas, as these patients are immunosuppressed and infection with an unidentified organism cannot be excluded.

Aged↗

PubMedDate not supplied

[The Spanish Registry of Primary Immunodeficiencies (REDIP)].

Two thousand and fifty cases (n = 2050) of primary immunodeficiencies (PID) were registered up to February 2001. The Spanish Register for Primary Immunodeficiencies (REDIP) began in 1993. PID nomenclature and diagnostic criteria were made according to the report of the World Health Organization Scientific Group (1999). The most frequent disorders were IgA deficiency (797 registers) and common variable immunodeficiency (CVI) (389), followed by severe combined immunodeficiency and predominantly T cell defects (268), complement deficiencies (207 registers), X-linked agammaglobulinemia (87), IgG subclass deficiency (71), chronic granulomatous disease (64). Gammaglobulin replacement was the therapy in 638 patients (76%) belonging to antibody deficient group. 61 bone marrow transplants were done, 46 severe combined immunodeficiencies, 6 phagocytic disorders and 1 unclassified. Important differences in the number of cases submitted from different country areas were found.

Agammaglobulinemia↗

PubMed1997

Response to minocycline of arthritis associated with agammaglobulinemia. A case-report.

Seven to 22% of patients with agammaglobulinemia develop joint manifestations consisting of septic arthritis or aseptic arthritis of unclear pathogenesis. Intravenous gammaglobulin therapy seems effective on the latter condition but is burdensome and expensive. We report the case of a patient with common variable immunodeficiency and aseptic oligoarthritis in which minocycline therapy was effective in relieving the joint symptoms.

Agammaglobulinemia↗

PubMed1996

Home therapy with subcutaneous immunoglobulin infusions in children with congenital immunodeficiencies.

OBJECTIVES: Patients with congenital, humoral immunodeficiencies are usually treated with intravenous immunoglobulin infusions. Subcutaneous infusions have emerged as an alternative treatment modality also in children. Our institution has run a subcutaneous infusion home therapy program for 6 years, and the purpose of this report is to describe our experience with this regimen. METHODS: The subcutaneous therapy of eight patients with immunodeficiency (three with agammaglobulinemia, one with common variable immunodeficiency, one with severe combined immunodeficiency and bone marrow transplantation, and three with hyper-immunoglobulin M syndrome) was evaluated by chart review and a questionnaire answered by all the families. The infusions were given for at least 3 hours each week by a small syringe driver at home after the family had attended an intensive educational course at the hospital. RESULTS: The children were given a total of approximately 1100 infusions. They started at the age of 2 to 8 (mean, 4.5) years and received these infusions for 1.5 to 6 (mean, 3) years. By administering immunoglobulin doses from 58 to 149 (mean, 97) mg/kg per week, trough serum immunoglobulin G values from 5.2 to 9.6 (mean, 7) g/L were obtained. No serious infections occurred. Short-lasting, local side effects such as swelling and redness were frequently reported, but pain or systemic adverse reactions during or after the infusions were never encountered. CONCLUSIONS: Home therapy with subcutaneous immunoglobulin infusions in children with congenital immunodeficiencies is a feasible and safe treatment alternative.

Child↗

PubMed2001

Herpes simplex virus lymphadenitis: case report and review of the literature.

Localized or regional necrotizing lymphadenitis is an extremely uncommon manifestation of herpes simplex virus (HSV) infection. We report a case of necrotizing HSV lymphadenitis in a patient with both common variable immunodeficiency and natural killer cell deficiency and review the literature on this unusual complication of HSV infection.

Adult↗

PubMed2003

Susceptibility to fungal infections of nails in patients with primary antibody deficiency.

Primary antibody deficiencies are rare diseases, which require early treatment with intravenous immunoglobulins to prevent fatal infections. The cell mediated immunity in patients with those immunodeficiencies remains unimpaired and usually they do not develop fungal infections. The aim of the study was to determine the susceptibility to fungal infections of nails in children with X-linked agammaglobulinaemia (XLA) and common variable immunodeficiency (CVID). Nail plate fragments collected from five patients with XLA and five with CVID were experimentally infected with a Candida albicans and Trichophyton mentagrophytes strains. The same procedures were carried out with the nails from a control group of 10 healthy volunteers. The intensity of the infection was evaluated on the basis of hyphae ingrown into the nail fragments. The main finding of the study was the increased susceptibility of antibody deficient patients to experimental nail infection with C. albicans and T. mentagrophytes.

Adolescent↗

PubMed1999

[Successful treatment with splenic irradiation for idiopathic thrombocytopenic purpura associated with primary immunodeficiency syndrome].

A 50-year-old man was admitted to our hospital because of intracranial hemorrhage and thrombocytopenia (platelet count: 3,000/microliter). Low levels of IgG (76 mg/dl) and IgA (30 mg/dl) and a normal pattern of peripheral blood T and B cell subsets yielded a diagnosis of common variable immunodeficiency (CVID). The number of megakaryocytes in bone marrow was within normal limits (64/microliter), and a diagnosis of idiopathic thrombocytopenic purpura was made. Both high-dose intravenous gamma-globulin and prednisolone were ineffective. Because of the coexistence of CVID, splenic irradiation (total 15 Gy) was performed instead of splenectomy. The platelet number began to increase 5 days after the initiation of irradiation, had increased to 8.7 x 10(4)/microliter at the end of irradiation, and was 22.9 x 10(4)/microliter 2 weeks later.

Common Variable Immunodeficiency↗

PubMed1996

Prevalence of hepatitis C in patients with primary antibody deficiency.

Concern over sporadic hepatitis C virus (HCV) infection from treatment with contaminated immunoglobulin in patients with immunodeficiency prompted use to screen serum from 44 patients for HCV RNA. We found no evidence of either horizontal or iatrogenic infection in our Clinic, except in two patients known to have received contaminated batches, and in one who had had regular plasma infusions. Thus, common variable immunodeficiency (CVID) patients in England do not have a high incidence of HCV infection despite exposure to various immunoglobulin products. This contrasts with the recently reported high prevalence in similar patients in Italy. Although our data reinforce the impression that both intramuscular and current licensed i.v. preparations in the UK are safe, manufacturers should be encouraged to PCR screen all batches of immunoglobulin for HCV.

Agammaglobulinemia↗

PubMedDate not supplied

[Autoimmune disease in primary antibody deficiencies].

BACKGROUND: Immunodeficiency and autoimmune phenomena may occur concomitantly in the same individual. Many immune deficiency syndromes, mainly humoral defects, are associated with autoimmune disorders. In the present study, we describe the frequency of autoimmune disorders in these patients. METHODS AND RESULTS: We performed a retrospective review of the medical records of a cohort of 152 patients with primary antibody deficiency in the Clinical Immunology Unit of the Gregorio Maranon University Hospital in Madrid: common variable immunodeficiency (CVID) (n = 35), IgA deficiency (n = 43), specific IgG subclass deficiency (n = 56), antibody deficiency with normal immunoglobulin (n = 14) and X-linked agammaglobulinemia (n = 4). Of the 152 patients with primary antibody deficiencies, autoimmune disorders were found in 35 (23 %). Autoimmune hematologic disease was found in 14 patients (9.2 %), autoimmune diseases of the gastrointestinal tract in eight (5.3 %), autoimmune endocrine diseases in six (3.9 %), autoimmune rheumatic diseases in six (3.9 %) and vitiligo in four. Autoimmune diseases were more frequent in CVID patients (37.1 %) than in those with selective IgA deficiency (25.5 %) or selective IgG subclass deficiency (12.5 %). Thirty-seven percent of autoimmune episodes occurred prior to the diagnosis of primary antibody deficiency. Sixteen patients (10.5 %) had autoantibodies without clinical data of an autoimmune disease. CONCLUSIONS: Primary antibody deficiencies have variable autoimmune manifestations. For early detection and appropriate treatment, autoimmune disease should be suspected in patients with immunodeficiency.

Adolescent↗

PubMedDate not supplied

["Sarcoidosis-like" granulomatous disease in patients with common variable hypogammaglobulinemia].

Particular attention has been recently dedicated to the development of a sarcoidosis-like form in patients with common variable immunodeficiency (CVI). In sarcoidosis a 5-fold increase in the number of cells in the bronchoalveolar lavage compared with normal controls has been described and the predominance of CD4+ T cells is a common feature. In contrast to patients affected by sarcoidosis or CVI, patients with co-existence of the two diseases had a more favorable prognosis, as progressive pulmonary fibrosis occurred only in a few cases even when persistent alveolar lymphocytosis was present. Moreover, patients presenting with concomitant sarcoidosis and CVI, showed a lower rate of respiratory infections and of the evolution towards bronchiectasies in comparison with patients affected by CVI alone. The clinical evolution observed in our patients was similar with that reported in other studies and confirms their clinical features. One may speculate that the increase in the number of macrophages and T lymphocytes, as suggested by the increased cellularity in the bronchoalveolar lavage of these patients, can produce a peculiar microenvironment characterized by high levels of soluble factors (cytokines). In turn, the latter could be responsible for a more efficacious effector phase of the immune response. This may explain the reduced rate of infectious diseases and consequently of bronchiectasies observed in CVI patients.

Adult↗

PubMedDate not supplied

A case of recurrent pneumonias.

A description is given of the case of a young boy who presented with repeated episodes of pneumonias since childhood. In addition, he had episodes of intermittent diarrhoeas. His investigative work-up revealed a deficiency of serum immunoglobulins, that is, hypogammaglobulinaemia. The profile was suggestive of common variable immunodeficiency. Because of the relative rarity of this disease, it is often missed leading to significant morbidity. Treatment consists of immunoglobulin replacement therapy and management of recurrent infections with appropriate drugs.

Adolescent↗

PubMed2001

Rapid subcutaneous IgG replacement therapy at home for pregnant immunodeficient women.

The use of slow or rapid, subcutaneous, IgG (SCIG) infusions in pregnant women with primary antibody deficiencies has been described earlier, though only in single-case reports. In this study, we have evaluated the effect of rapid, SCIG infusions during 11 pregnancies in nine women [six women with common variable immunodeficiency (CVID), two with IgG-subclass deficiencies, and one with combined IgA and IgG2 deficiency]. The weekly infusions were given by the women themselves at a dose of 100 mg/kg/week throughout the pregnancy. No adverse systemic reactions or pronounced, local tissue reactions were recorded during or after the more than 400 infusions. The 11 babies were healthy and were born after 38-42 weeks of uneventful gestation. The concentrations of maternal S-IgG at the time of delivery in the four women with CVID ranged from 6.0 to 8.3 g/L, with cord/maternal S-IgG ratios of 1.0-1.5. The IgG subclasses were transferred to the infants. The SCIG home-therapy regime was greatly appreciated by the women.

Adult↗

PubMed1985

Monoclonal antibody-defined B lymphocyte subpopulations in primary immunodeficiency disorders.

Peripheral blood from 23 patients with common variable immunodeficiency (CVI) and 5 with selective IgA deficiency (SIAD) was examined for the proportions of B lymphocytes defined with monoclonal antibodies FMC 1 and FMC 7. In healthy controls, FMC 1 monoclonal antibody reacts with antigen present on almost all surface Ig+ B lymphocytes; FMC 7 monoclonal antibody reacts with antigen present only a subset of surface Ig+ and FMC 1+ B cells. In 13 of 23 patients with CVI and all patients with SIAD, a relative increase in FMC 7+ B cells, resulting in proportions of FMC 7+ B cell comparable to surface Ig+ or FMC 1+ B cells was observed. These data suggest abnormality of the proportion of B cell subsets in patients with CVI and SIAD that could suggest an intrinsic defect of B cells in these disorders.

Adolescent↗

PubMed2001

Impact of IgG replacement therapy and antibiotic treatment on the colonization of non-encapsulated Haemophilus influenzae in the nasopharynx in patients with hypogammaglobulinaemia.

Non-typable Haemophilus influenzae (NTHI) and Streptococcus pneumoniae are regarded as the main pathogens in patients with humoral immunodeficiency. These patients have been given IgG replacement therapy since the 1950s. However, a number of individuals still suffer from recurrent episodes of respiratory tract infections. Nasopharyngeal cultures were obtained on a regular basis over a 3-6-month period from 11 patients with common variable immunodeficiency disease in 1989 and 1998. The proportion of cultures positive for NTHI decreased from 56% in 1989-90 to 16% in 1998-99 (p < 0.003). After 9 y of IgG therapy, 7 of the 11 patients were free from NTHI in the nasopharynx. Specific NTHI strains were analysed by pulsed-field gel electrophoresis and compared, with regard to genetic relatedness, on an intra-individual basis. In 1 patient the same NTHI strain was found in both 1989 and 1999. The apparent absence of NTHI from the nasopharynx in most of the patients was assumed to be due to subcutaneous IgG treatment, as well as adequate antibiotic treatment.

Adult↗

PubMed2002

Angiocentric lymphomatoid granulomatosis and severe hypogammaglobulinaemia.

Angiocentric lymphomatoid granulomatosis is a rare lymphoproliferative disease, mainly associated with pulmonary manifestation. Its origin is unknown, but Epstein-Barr virus may be one of the etiological factors. A 51-year-old male had an abdominal laparotomy in 1994 and a large granulomatous mass was removed from behind the cecum. No specific therapy was administered. In February 1998 multiple pulmonary lesions were found by X-ray and thoracoscopic biopsy was made. The histopathological diagnosis was angiocentric lymphomatoid granulomatosis. The patient received 6 cycles of CHOP chemotherapy, with which a complete remission was achieved. A consistent severe hypogammaglobulinaemia was detected, so the diagnosis of common variable immunodeficiency (CVID) was established. The diagnosed CVID was the probable causative factor of the angiocentric lymphomatoid granulomatosis. After the CHOP treatment, the patient is on intravenous immunoglobulin substitution and is well up to today.

Agammaglobulinemia↗

PubMed1994

Cytomegalovirus reactivation and tumour necrosis factor.

Tumour necrosis factor-alpha (TNF) stimulates cytomegalovirus (CMV) activity in a transfected human monocytic cell line. We assessed whether this finding is relevant in vivo by evaluating the frequency of active CMV infection in patients with diseases that enhance plasma TNF. In septic disease, peripheral blood mononuclear cells of almost all patients studied were positive for CMV. Furthermore, CMV antigenaemia and enhanced plasma TNF occurred in many patients with liver cirrhosis, common variable immunodeficiency, and B-cell chronic lymphocytic leukaemia. Thus, TNF may have a central role in CMV reactivation.

Adolescent↗

PubMed2001

Mycobacterium avium intracellulare otitis media.

Atypical mycobacterial infections of the middle ear are extremely rare. To our knowledge, only eight cases have been reported in the literature, five of which involve Mycobacterium avium intracellulare. We present a case of culture-proven, M. avium intracellulare otomastoiditis in an 8-year-old boy with common variable immunodeficiency syndrome. The patient clinically presented with pain and otorrhea. The histopathology was marked by acid-fast bacilli-laden histiocytes. Consideration of this entity in the differential diagnosis of chronic, recalcitrant otorrhea can lead to timely diagnosis, treatment, an decreased morbidity.

Child↗