Encefalopatía por hiperamoniemia en el adolescente / Encephalopathy by hyperammonemia in the teenager

Fundamento. La hiperamoniemia origina múltiples alteraciones,principalmente en el sistema nervioso central.Si el fallo hepático no es su causa, deberán investigarseotras etiologías menos frecuentes intentandollegar a un diagnóstico definitivo.Caso clínico. Presentamos el caso de un paciente de16 años que ingresó por encefalopatía aguda e hiperamoniemia.Tras realización de analítica, TAC cerebral,ecografía y Doppler abdominal, se inició tratamientoempírico de hiperamoniemia secundaria a un errorinnato del ciclo de la urea. Se trató el edema cerebraly se tomaron medidas para eliminación del amoniacosin resultado favorable, falleciendo a los cuatro días delingreso.Conclusiones. El complejo manejo de la hiperamoniemiay la alta morbi-mortalidad que conlleva requiere unmanejo multidisciplinar. La instauración de tratamientoprecoz e identificación de la causa son claves para mejorarlos resultados(AU)

Background. Hyperammonemia causes several alterations,mainly in the central nervous system. If hepaticfailure is not its etiology, other less frequent causesmust be investigated in the search for a definitive diagnosis.Clinical case. We report the case of a 16 year old patientadmitted for acute encephalopathy and hyperammonemia.After analysis, brain CT, ultrasound and abdominalDoppler, we began empirical treatment of hyperammoniemiasecondary to disorders of the urea cycle. Wetreated the brain edema and eliminated ammonia butwe did not obtain favourable results and the patientdied four days later.Conclusions. The complex management of hyperammonemiaand the high morbidity and mortality involvedrequire a multidisciplinary approach. Only earlytreatment and identification of the hyperammonemia`setiology can avoid high morbidity and mortality in thesepatients(AU)

[Encephalopathy by hyperammonemia in teenagers]. / Encefalopatía por hiperamoniemia en el adolescente.

BACKGROUND: Hyperammonemia causes several alterations, mainly in the central nervous system. If hepatic failure is not its etiology, other less frequent causes must be investigated in the search for a definitive diagnosis. CLINICAL CASE: We report the case of a 16 year old patient admitted for acute encephalopathy and hyperammonemia. After analysis, brain CT, ultrasound and abdominal Doppler, we began empirical treatment of hyperammonemia secondary to disorders of the urea cycle. We treated the brain edema and eliminated ammonia but we did not obtain favourable results and the patient died four days later. CONCLUSIONS: The complex management of hyperammonemia and the high morbidity and mortality involved require a multidisciplinary approach. Only early treatment and identification of the hyperammonemia's etiology can avoid high morbidity and mortality in these patients.

[Prognosis of patients who recovered after an episode of sudden death]. / Pronóstico de los pacientes recuperados tras un episodio de muerte súbita.

OBJECTIVES: Sudden death constitutes a major sanitary problem with high mortality and serious neurological complications. The objective of this study was to analyze the prognosis and the characteristics of patients who initially recovered after an episode of cardiac arrest and who were admitted to the intensive care unit (ICU). METHOD: We retrospectively studied the clinical characteristics and outcome of 65 patients admitted to the Intensive Care Unit during a 3 years period with aborted sudden death. RESULTS: 65 patients, 44 (67.7%) men and 21 (32.3%) women. Middle ages 69.1 +/- 13.9. 29 (44.6%) out of hospital and 36 (55.4%) into hospital sudden death. Cardiopulmonary resuscitation was <10 minutes in 28 cases (43.1%), and > 10 minutes in 37 (56.9%). 36 (55.4%) of all sudden deaths were of cardiac origin. 37 patients (56.9%) died and 28 (43.1%) survived the episode. It was LET in 11 cases (16.9%). 29 (44.6%) of all had post- anoxic encephalopathy and most died before discharge from ICU. Of 28 survivors, 5 patients were discharged alive with post-anoxic encephalopathy (17.8%) and 23 were discharged without neurological disturbances (82.2%). This was more frequent when sudden death was into hospital (p 0.009) and cardiopulmonary resuscitation was < 10 minutes (p 0.045). CONCLUSIONS: High number of the patients admitted to a Intensive Care unit with aborted sudden death died during ICU stay. Many patients had post-anoxic encephalopathy and most of these died. So, up to 35% of the patients admitted after an episode of cardiac arrest were discharged alive and without severe neurological damage.

Pronóstico de los pacientes recuperados tras un episodio de muerte súbita / Prognosis of patients who recovered after an episode of sudden death

Objetivos: La muerte súbita constituye un grave problema con alta mortalidad y serias complicaciones neurológicas. El objetivo de este estudio es analizar el pronóstico y las características de pacientes inicialmente reanimados tras un episodio de muerte súbita y que son ingresados en Unidades de Cuidados Intensivos (UCI). Método: Estudio retrospectivo de las características clínicas y estado al alta de 65 pacientes ingresados en UCI durante un periodo de 3 años tras una muerte súbita recuperada. Resultados: 65 pacientes, 44(67,7%) hombres y 21(32,3%) mujeres. Edad media 69,1 ± 13,9. 29 (44,6%) muertes súbitas extrahospitalarias y 36 intrahospitalarias. La reanimación cardiopulmonar (RCP) fue 10 minutos en 37 (56,9%). Más de la mitad de los episodios tuvo origen cardiaco 55,4% (36pacientes). 37 pacientes (56,9%) fallecieron y 28 (43,1%) sobrevivieron. Existió limitación del esfuerzo terapeutico (LET) en 11 casos (16,9%). 29 pacientes (44,6%) desarrollaron encefalopatía postanóxica (EPA) falleciendo la mayoría antes del alta. De los 28 supervivientes, 5 fueron dados de alta vivos con encefalopatía postanóxica (17,8%) y 23 lo hicieron libres de secuelas neurológicas (82,2%). Esto fué más frecuente cuando la parada cardiorrespiratoria (PCR) ocurrió dentro del hospital (p 0,009) y la reanimación duró menos de 10 minutos (p 0,045). Conclusiones: Un elevado número de pacientes ingresados en UCI tras sufrir una PCR fallecen durante su estancia. Muchos desarrollan encefalopatía postanóxica y de ellos la mayoría muere. Pero a pesar de esto, hasta un 35% de los pacientes admitidos tras una muerte súbita recuperada son dados de alta sin secuelas neurológicas

Objectives: Sudden death constitutes a major sanitary problem with high mortality and serious neurological complications. The objective of this study was to analyze the prognosis and the characteristics of patients who initially recovered after an episode of cardiac arrest and who were admitted to the intensive care unit (ICU). Method: We retrospectively studied the clinical characteristics and outcome of 65 patients admitted to the Intensive Care Unit during a 3 years period with aborted sudden death. Results: 65 patients, 44 (67.7%) men and 21 (32.3%) women. Middle ages 69.1 ± 13.9. 29 (44.6%) out of hospital and 36 (55.4%) into hospital sudden death. Cardiopulmonary resuscitation was 10 minutes in 37 (56.9%). 36 (55.4%) of all sudden deaths were of cardiac origin. 37 patients (56.9%) died and 28 (43.1%) survived the episode. It was LET in 11 cases (16.9%). 29 (44.6%) of all had post- anoxic encephalopathy and most died before discharge from ICU. Of 28 survivors, 5 patients were discharged alive with post-anoxic encephalopathy (17.8%) and 23 were discharged without neurological disturbances (82.2%). This was more frequent when sudden death was into hospital (p 0.009) and cardiopulmonary resuscitation was < 10 minutes (p 0.045). Conclusions: High number of the patients admitted to a Intensive Care unit with aborted sudden death died during ICU stay. Many patients had post-anoxic encephalopathy and most of these died. So, up to 35% of the patients admitted after an episode of cardiac arrest were discharged alive and without severe neurological damage

Meningitis por estreptococo agalactie en adulto joven / Meningitis due to estreptococcus agalactiae in young adult

Se ha detectado un incremento de infecciones en adultos por estreptococo del grupo B o agalactie los últimos años. Pueden causar bacteriemias, celulitis, neumonía, endocarditis, artritis y meningitis. Presentamos el caso de una mujer que sufrió una meningitis complicada con distress respiratorio y septicemia cuya evolución,sin embargo, fue favorable con el tratamiento instaurado. Este estreptococo es responsable de menos del 2% de casos de meningitis en adultos, siendo de alto riesgo las embarazadas y los pacientes con enfermedades crónicas como diabetes, cirrosis,cáncer, traumatizados, enfermedades autoinmunes, infectados por VIH o postoperatorios. Su mortalidad oscila del 27 al 34%. Aunque la incidencia es superior en mayores de 60 años con enfermedades crónicas hay un aumento progresivo en pacientes adultos inmunocompetentes, por lo que este agente causal no debe obviarse ante la presentica de meningitis de rápida instauración en un adulto joven

Its detected and increment of group B streptococcus or agalactie adults infection in recent years. Can be responsible of bacteremia, cellulites, pneumonia, endocarditis, arthritis and meningitis. We report a meningitis young female case, with serious complications like a distress respiratory syndrome and sepsis, but good evolution with antibiotics. Group B streptococcus is the responsible than near of 2% adult meningitis, and is associated to pregnancy and underlying conditions like diabetes, cirrhosis, cancer, trauma, inmunologic illness, HIV infection or surgery. The mortality is around 27,34%. The incidence is superior in elderly with debilitating diseases but there is a progressive increment in patients without diseases, so we mustn´t forget this etiology in fast instauration meningitis in a young adult

[Exoftalmos and palpebral ptosis in the immunocompromised patient]. / Exoftalmos y ptosis palpebral en paciente inmunodeprimido.

The mucormycosis is an opportunistic mycosis which has a high rate of global mortality with a bad prognosis. The present clinical case shows a patient attended in the Intensive Care Unit because of a breathing insufficiency secondary to a pneumonia. Among his antecedents there is a larynx carcinoma totally remittent after chemo- and radiotherapy. He develops an infrapalpebral injury which spreads rapidly in a bilateral way acquiring a necrotic look that it did not have initially. Having that evolution, it is implemented a treatment with liposomal amphotericin B and granulocyte colony-stimulating factors after an injury biopsy, whose posterior cultive shows growing of Mucor. This kind of pathology must be initially suspected in all the patients with a neoplasia and its treatment must be early and agressive. At the moment the diagnosis is suspected there is a great tissue destruction and for that reason the patients usually die in spite of the instauration of aggressive therapies

Exoftalmos y ptosis palpebral en paciente inmunodeprimido / Exoftalmos and palpebral ptosis in non immunocompetent patient

La mucormicosis es una micosis oportunista que posee una alta tasa de mortalidad global, con mal pronóstico. En el presente caso clínico se muestra un paciente ingresado en UCI por insuficiencia respiratoria secundaria a neumonía; entre sus antecedentes figura carcinoma de laringe en remisión completa tras tratamiento con quimio y radioterapia. Desarrolla una lesión infrapalpebral que se extiende rápidamente de forma bilateral adquiriendo aspecto necrótico que inicialmente no poseía. Con dicha evolución se realiza tratamiento con anfotericina B liposomal y estimulantes de colonias previa biopsia de lesión, cuyo cultivo posterior muestra crecimiento de Mucor. Este tipo de patologías deben sospecharse inicialmente en todo paciente con antecedentes de neoplasia, su tratamiento debe ser agresivo y precoz. En el momento que se sospecha el diagnóstico existe una gran destrucción tisular, motivo por el que los pacientes suelen fallecer pese instauración de terapias agresivas (AU)

[An epidemiological and social study of 127 patients with AIDS IN Aragon]. / Estudio epidemiológico y social de 127 pacientes con sida en Aragón.

OBJECTIVE: To evaluate the profile epidemiology and social of the patient with AIDS in our environment and the direct relation with primary care. DESIGN: A descriptive, analytic and retrospective study. SETTING: The study was carried out in the Miguel Servet Hospital in Zaragoza. PARTICIPANTS: Patients with AIDS between January 1991 and june 1993 and who fulfilled the requisite diagnostic criteria. MEASUREMENTS AND MAIN RESULTS: Out of the 127 patients studied, 74.8% were male and the age was 20 to 30 years old. The transmission mechanism was blood way in drug addicts in 63.8% and heterosexual way in 14.8%. It was socioeconomic problems in 40.94%, and 69.23% of them were drug addicts. The familiar support were proved in 29.13% and had relation with primary care only 15.74%. CONCLUSIONS: An increase of the incidence of AIDS is observed, prevailing men between 31-40 years old. The heterosexual way transmission is increasing, but the blood way in drug addicts is more frequently. The majority presents socioeconomic problems and they haven't familiar support.