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

Liver transplant, in emergency 0 (UNOS Status 1).

INTRODUCTION: The emergency 0 (UNOS status 1) liver transplant (OLT) constitutes a challenge to the transplant team. The precarious condition of the patient and the use, sometimes forced by the instability of the patients, of marginal or different blood type grafts leads to worse results than in nonemergency OLT. Herein we have presented our experience with emergency 0 OLT in the first 5 years of our program. PATIENTS AND METHODS: Among the 167 OLTs performed in the first 5 years of our program, 10 were emergency 0 OLTs. The patients were transplanted within 72 hours of inclusion on the waiting list, seven within 24 hours. The indications for emergency OLT were fulminant liver failure (FLF) in six and graft failure in the first week post-OLT in four. RESULTS: All OLTs were performed with preservation of the vena cava (piggyback) and without venovenous bypass. There was 100% patient survival of those who required an emergency 0 OLT (follow-up period of 3 to 7 years). The graft survival in FLF was 50%. Emergency retransplant was necessary because of acute rejection due to ABO incompatibility in two patients, and due to arterial ischemia in another patient. The emergency retransplants were all successful. CONCLUSION: In our experience the emergency 0 OLT is a formidable challenge for the team, but we achieved a patient survival comparable to or even better than that of OLT for chronic liver disease.

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