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1.
Rev Clin Esp ; 209(7): 332-6, 2009.
Article in Spanish | MEDLINE | ID: mdl-19709536

ABSTRACT

C1 inhibitor disorders are a group of rare conditions in which the C1 inhibitor is deficient or defective. We present the clinical characteristics of 8 patients and a review of the literature. These are characterized by recurrent episodes of angioedema, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tract. Laryngeal involvement may cause fatal asphyxiation. These disorders may be divided into two broad categories: hereditary angioedema (HAE) and acquired C1 inhibitor disorders. Indications for screening for HAE include: recurrent angioedema, episodic abdominal pain, laryngeal, a family background of angioedema, and a low C4 level. Acquired C1 inhibitor disorders are similar, but lack a family background. Treatment is divided into short and long-term prophylaxis with androgens, antifibrinolytics and C1 inhibitor replacement. First line therapy of acute attacks is C1 inhibitor.


Subject(s)
Angioedema/diagnosis , Angioedemas, Hereditary , Complement C1 Inactivator Proteins , Adolescent , Adult , Age Factors , Aged , Angioedemas, Hereditary/complications , Angioedemas, Hereditary/diagnosis , Angioedemas, Hereditary/epidemiology , Angioedemas, Hereditary/genetics , Child , Child, Preschool , Diagnosis, Differential , Female , Humans , Laryngeal Edema/diagnosis , Male , Middle Aged , Sex Factors
2.
Rev. clín. esp. (Ed. impr.) ; 209(7): 332-336, ago. 2009. tab
Article in Spanish | IBECS | ID: ibc-73069

ABSTRACT

El angioedema (AE) es una enfermedad infrecuente. Presentamos las características clínicas y evolución de 8 pacientes y revisamos la literatura. Se caracteriza por la ausencia o mal funcionamiento del C1 inhibidor, que se traduce en episodios de angioedema (hinchazón subcutánea o mucosa) de la piel y mucosas del tracto respiratorio superior y gastrointestinal. La afectación laríngea puede ser mortal. Puede ser hereditario o adquirido. Se debe sospechar angioedema hereditario en casos de: angioedema recurrente, dolor abdominal, edema laríngeo, historia familiar y niveles bajos de C4. En el déficit adquirido de C1 no hay antecedentes familiares. El tratamiento se divide en profilaxis a corto y a largo plazo con andrógenos, antifibrinolíticos y C1 inhibidor. Este último es de elección en ataques agudos (AU)


C1 inhibitor disorders are a group of rare conditions in which the C1 inhibitor is deficient or defective. We present the clinical characteristics of 8 patients and a review of the literature. These are characterized by recurrent episodes of angioedema, which most often affect the skin or mucosal tissues of the upper respiratory and gastrointestinal tract. Laryngeal involvement may cause fatal asphyxiation. These disorders may be divided into two broad categories: hereditary angioedema (HAE) and adquired C1 inhibitor disorders. Indications for screening for HAE include: recurrent angioedema, episodic abdominal pain, laryngeal, a family background of angioedema, and a low C4 level. Acquired C1 inhibitor disorders are similar, but lack a family background. Treatment is divided into short and long-term prophylaxis with androgens, antifibrinolytics and C1 inhibitor replacement. First line therapy of acute attacks is C1 inhibitor (AU)


Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Angioedemas, Hereditary/diagnosis , Angioedemas, Hereditary/epidemiology , Complement C1 Inhibitor Protein/genetics , Complement C1 Inhibitor Protein , Retrospective Studies , Abdominal Pain/diagnosis , Abdominal Pain/etiology , Antibiotic Prophylaxis
13.
An. med. interna (Madr., 1983) ; 23(11): 519-524, nov. 2006. ilus, tab
Article in Es | IBECS | ID: ibc-051700

ABSTRACT

Fundamento y objetivo: Con el aumento de la esperanza de vida en pacientes con infección por el virus de la inmunodeficiencia humana (VIH) tras el tratamiento antirretroviral de gran actividad (TARGA), las causas de hospitalización han cambiado. Predominan ahora comorbilidades como la hepatopatía crónica, la patología cardiovascular o los efectos secundarios de los tratamientos. Nuestro objetivo es determinar las características clinicoepidemiológicas actuales de estos pacientes. Material y métodos: Estudio descriptivo y retrospectivo. Revisamos las historias clínicas de los ingresos de pacientes con infección por el VIH hospitalizados en los servicios médicos de nuestro hospital en el transcurso de un año. Resultados: Hubo 125 ingresos de 82 pacientes, un 77% varones y un 71% con infección por el virus de la hepatitis C (VHC). La edad media fue de 42 años. En el 64% la vía de transmisión fue la drogadicción parenteral. Un 36% recibían TARGA y un 51% reunían criterios sida. Los motivos de ingreso más frecuentes fueron la patologia respiratoria (32%) y las enfermedades definitorias de sida (EDS) con un 22%, de las que la tuberculosis (TBC) fue la más frecuente. Un 10% fueron ingresados en la Unidad de Cuidados Intensivos (UCI). La mortalidad fue del 11% pacientes/año. Conclusiones: La mayoría de los pacientes fueron varones, con coinfección por el VHC y usuarios de drogas vía parenteral, siendo las causas de ingreso más frecuentes las patologías respiratorias y las enfermedades oportunistas. La TBC permanece siendo la EDS más frecuente


Background and objetive: With the increasing life expectancy in human immunodeficiency virus (HIV) infected patients since the highly active antiretroviral therapy (HAART), other underlying comorbilities such as chronic liver patology, cardiovascular diseases or side effects of treatment may lead to hospitalization. The purpose of this study is to determine the clinical and epidemiological characteristics of these patients. Material and methods: A descriptive-retrospective study. We reviewed the clinical records of patients with HIV infection admitted in our medical departments through a year. Results: There were 125 hospitalizations of 82 patients. 77% were males and 71% had hepatitis C virus (HCV) infection. The median age was 42 years. 64% HIV infection had been acquired by sharing material for intravenous drug use. A 36% were receiving HAART and 51% had AIDS. The respiratory tract patology (32%) and AIDS-defining illnesses (22%) were the most frequent causes of hospitalization, and the main opportunistic disease was tuberculosis (TBC). Ten per cent were admitted in the Intensive Care Unit. Mortality rate was 11% patients/year. Conclusions: Most of the patients were male, drug users and HCV positive, and the admissions to hospital were mostly due to respiratory patology and opportunistic diseases. TBC remains like the most frequent AIDS-defining illness


Subject(s)
Male , Female , Adult , Humans , Morbidity Surveys , Liver Diseases/complications , HIV Infections/epidemiology , HIV Infections/mortality , Comorbidity , Opportunistic Infections/complications , Opportunistic Infections/epidemiology , Liver Diseases/epidemiology , Liver Diseases/mortality , Retrospective Studies , Respiratory Tract Diseases/complications , Respiratory Tract Diseases/epidemiology , Respiratory Tract Diseases/mortality , Acquired Immunodeficiency Syndrome/epidemiology , Acquired Immunodeficiency Syndrome/mortality , Myocardial Ischemia/complications , Myocardial Ischemia/epidemiology
16.
An Med Interna ; 23(11): 519-24, 2006 Nov.
Article in Spanish | MEDLINE | ID: mdl-17222066

ABSTRACT

BACKGROUND AND OBJECTIVE: With the increasing life expectancy in human immunodeficiency virus (HIV) infected patients since the highly active antiretroviral therapy (HAART), other underlying comorbilities such as chronic liver pathology, cardiovascular diseases or side effects of treatment may lead to hospitalization. The purpose of this study is to determine the clinical and epidemiological characteristics of these patients. MATERIAL AND METHODS: A descriptive-retrospective study. We reviewed the clinical records of patients with HIV infection admitted in our medical departments through a year. RESULTS: There were 125 hospitalizations of 82 patients. 77% were males and 71% had hepatitis C virus (HCV) infection. The median age was 42 years. 64% HIV infection had been acquired by sharing material for intravenous drug use. A 36% were receiving HAART and 51% had AIDS. The respiratory tract pathology (32%) and AIDS-defining illnesses (22%) were the most frequent causes of hospitalization, and the main opportunistic disease was tuberculosis (TBC). Ten per cent were admitted in the Intensive Care Unit. Mortality rate was 11% patients/year. CONCLUSIONS: Most of the patients were male, drug users and HCV positive, and the admissions to hospital were mostly due to respiratory patology and opportunistic diseases. TBC remains like the most frequent AIDS-defining illness.


Subject(s)
AIDS-Related Opportunistic Infections/epidemiology , HIV Infections/epidemiology , HIV-1 , Adult , Anti-Retroviral Agents/therapeutic use , Cause of Death , Female , HIV Infections/drug therapy , Hospital Mortality , Humans , Inpatients/statistics & numerical data , Male , Morbidity , Retrospective Studies , Spain/epidemiology
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