Clinical and immunological features of common variable immunodeficiency in Mexican patients.

BACKGROUND: Common variable immunodeficiency (CVID) is characterised by hypogammaglobulinaemia and a broad clinical spectrum, mainly showing recurrent bacterial infections accompanied sometimes by increased susceptibility to chronic lung disease, autoimmunity, and neoplastic diseases. OBJECTIVES: To evaluate the clinical and immunological characteristics of patients with CVID in Mexico. METHODS: This is a retrospective analysis of 43 patients with CVID from the Immunology Division of seven different reference centres in Mexico. Patients were diagnosed according to the diagnostic criteria of the European Society for Immunodeficiency Diseases. We collected demographics, clinical and immunological data from each patient and a statistical analysis was performed. RESULTS: There were 23 (53.5%) male and 20 (46.5%) female patients. Median age at onset of disease was 13.7 years, and median age at diagnosis was 19 years. Average delay in diagnosis was 12.5 years. The median total serum levels of IgG, IgM, and IgA at diagnosis were 175, 18, and 17.8mg/dL, respectively. The mean percentage of CD19+ B cells was 8.15%. Sinusitis (83%), pneumonia (83%), gastrointestinal infection (70%), and acute otitis media (49%) were the most common manifestations. Bronchiectasis was present in 51% of the patients, 44% manifested non-infectious chronic diarrhoea, and 70% experienced weight loss. Autoimmunity was present in 23% of the patients; haemolytic anaemia and autoimmune thrombocytopenic purpura were the most common presentations. Allergy was present in 30.2% of patients, with allergic rhinitis and asthma being the most frequent types. Two patients developed malignancy. All the patients received Intravenous immunoglobulin (IVIG) as a fundamental part of the treatment at a mean dose of 408mg/kg. CONCLUSION: This is the first cohort of CVID reported in Mexico We found that infection diseases were the most frequent presentations at onset. Moreover, patients had an average diagnosis delay of twelve years and thus a major prevalence of bronchiectasis. We suggest performing an extended analysis of patients with CVID patients in other Latin American countries.

Detection of inheritance pattern in thirty-three Mexican males with chronic granulomatous disease through 123 dihydrorhodamine assay.

BACKGROUND: There are two inheritance patterns, the X-linked recessive (XL) pattern and the autosomal recessive pattern. There is no information on the predominant inheritance pattern of male patients with chronic granulomatous disease (CGD) in Mexico. OBJECTIVE: The aim of this study was to determine the inheritance pattern in a cohort of Mexican male patients with CGD by means of the detection of an XL status carrier among their female relatives, and to describe the frequency of discoid lupus (DL) among carriers. METHODS: We detected the female relatives within the families of male patients with CGD, and carried out the 123 dihydrorhodamine (DHR) assay in all female participants. All carriers were questioned for current or past established DL diagnosis. RESULTS: We detected 33 families with one or more CGD male patients; we found an XL-CGD in 79% of the relatives from at least one female relative with a bimodal pattern. For the remaining seven relatives we were not able to confirm a carrier status by means of a DHR assay. Moreover, we detected one mother with CGD secondary to skewed X-chromosome inactivation. We also found 47 carriers, and only one carrier with DL among them. CONCLUSION: We concluded that XL-CGD is the most frequent form of CGD in a cohort of CGD male patients in Mexico. DHR assay is a fast and practical tool to determine the CGD form in the Latin-American countries. Finally, DL frequency in Mexico is lower than that reported in the literature for other regions of the world.

[Use of pentoxifylline in pediatric patients with grade IV (OMS) lupus nephropathy who have received multiple treatments]. / Uso de pentoxifilina en pacientes pediátricos con nefropatía lúpica grado IV (OMS) multitratados.

AIMS: Systemic Lupus Eritematosus is an autoimmune disease, the incidence in pediatric poblation in about 5%, and until 90% develop nephropathy. MATERIAL AND METHOD: Included patients with lupic nephropathy grade IV (OMS) ages between 0 and 16 years old, multitreated, who administrated PTX. We take samples before treatment, during, and 4 month after, evaluating renal function and hepatic function. RESULTS: For female, tow male, promedium age 14.1 years old. Poteinuria get a significative p = 0.0012; hematuria was lowering its levels, While immune circulating complex, get too a significative p = 0.0050. In creatinine inverse showed an important modification of its pending. CONCLUSIONS: This results demonstrates, that PTX in nephritis lupic patients, helps to brake the habitual deterioration in renal function. Includes more patients for a long time of treatment, we'll get better results than this.

[Hyperprolactinemia and autoimmunity]. / Hiperprolactinemia y autoinmunidad.

Prolactin is a pituitary hormone with several functions, one of them, immunoregulatory. Patients with prolactinoma develop hyperprolactinemia. In the next two cases, patients with microprolactinoma, both autoimmune disease associated. First patient, male, with multiple sclerosis; the other one patient, female, with systemic lupus erythematosus. Treatment of hyperprolactinemia with bromocriptine was associated with satisfactory clinical evolution, and a reduction of dosage of immunosuppressor treatment. The patients with multiple sclerosis had neurological functions recovery and the patients with systemic lupus erythematosus had severe relapse of disease each time she dropped bromocriptine treatment. Pituitary function must be evaluated in autoimmune disease, to search alterations like hyperprolactinemia who influenced immune function.

[Clinical polymorphism in Epstein-Barr virus infection]. / Polimorfismo clínico en infección por virus de Epstein Barr.

Several diseases was associated with Epstein Barr virus (EBV) infection. In the next three cases, the clinical course was unusual . Case I: Polyclonal hypergammaglobulinemia, female 47 years old, she had systemic lupus erythematosus and clinical data of infectious mononucleosis but she evolved to a polyclonal gammopathy with IgM predominantly against EBV. Case II: Demyelinating encephalitis, male, 32 years old with central neurological alterations, IgM antibodies against EBV and demyelinating lesion in magnetic resonance image in brain steam. Case III. Villous leukoplakia, male, 40 years old developed right tonsil tumor. He had IgM antibodies against EBV. The antiviral and immunomodulator treatment (specific for each case) done a satisfactory clinical response in the three patients.

[Vasculitis and viral infection]. / Vasculitis e infección viral.

Viruses have been implicated in vasculitis. To determine activity of viral infection associated with vasculitis. 17 patients with vasculitis had been in immunological and antiviral antibodies evaluation. Twenty five healthy controls sex and age matched with hematic biometry (BH) and AA. All subjects were negative to HIV and HBV. Viral activity was demonstrated in eight patients; vascular purpura (5), Takayasu disease (1), polyarteritis nodosa (1), erythema nodosum (1). None subject of control group had IgM activity. Antibodies response of IgG in patients were of lesser intensity than in control group. 14 abnormalities in BH were found in patients and 4 in control group. Immune response in patients, measured by lymphocyte subpopulations and circulating immune complexes was abnormal. In conclusion 47% showed viral activity, but the dominant feature was abnormal immune response in 82%.

[Multiple sclerosis]. / Esclerosis múltiple.

Multiple sclerosis, a neurological problem in which mechanisms of autoimmunity and immunodeficiency may cause damage appears with a variable range of immune response. In this paper we classify in three grate subgroups the alterations observed in our patients: Type I: specific defect of immune response; Type II: immunodeficiency with autoimmune responses; Type III: mixed responses: autoimmunity with specific defect of immune response and increased cytotoxicity. A rational explanation about the various immunological changes emerged from comprehension of these mechanisms of response and following these hypothesis we propose an immunological classification of multiple sclerosis in other to reach more effective therapeutic goals.