Emerging trends in management of propionic acidemia / Tendências emergentes no manejo da academia propiônica

Objetivo : To evaluate the therapeutic agents used during metabolic crises and in long-term management of patients with propionic acidemia (PA).Materials and methods : The records of PA patients were retrospectively evaluated.Results : The study group consisted of 30 patients with 141 admissions. During metabolic crises, hyperammonemia was found in 130 (92%) admissions and almost all patients were managed with normal saline, ≥ 10% dextrose, and restriction of protein intake. In 56 (40%) admissions, management was done in intensive care unit, 31 (22%) with mechanical ventilation, 10 (7%) with haemodialysis, 16 (11%) with vasopressor agents, and 12 (9%) with insulin. In the rescue procedure, L-carnitine was used in 135 (96%) patients, sodium bicarbonate in 116 (82%), sodium benzoate in 76 (54%), and metronidazole in 10 (7%), biotin in about one-quarter, L-arginine in one third, and antibiotics in three-quarter of the admissions. Blood/packed RBCs were used in 28 (20%) patients, platelets in 26 (18%), fresh frozen plasma in 8 (6%), and granulocyte-colony stimulating factors in 10 (7%) admissions. All patients were managed completely/partially with medical nutrition formula plus amino acid mixture, vitamins and minerals. For long-term management 24 (80%) patients were on L-carnitine, 22 (73%) on sodium benzoate, 6 (20%) on biotin, one half on alkaline therapy and 4 (13%) on regular metronidazole use. Almost all patients were on medical formula and regular follow-up.Conclusion : Aggressive and adequate management of acute metabolic crises with restriction of protein intake, stabilization of patient, reversal of catabolism, and removal of toxic metabolites are essential steps. Concerted efforts to ensure adequate nutrition, to minimize the risk of acute decompensation and additional therapeutic advances are imperative to improve the outcome of PA patients. Arq Bras Endocrinol Metab. 2014;58(3):237-42.

Objetivo : Avaliar os agentes terapêuticos usados durante as crises metabólicas e para o manejo de longo prazo de pacientes com academia propiônica (AP).Materiais e métodos : Avaliação retrospectiva das fichas médicas de pacientes com AP.Resultados : O grupo estudado consistiu de 30 pacientes com 141 hospitalizações. Durante as crises metabólicas, a hiperamonemia foi observada em 130 (92%) pacientes hospitalizados e quase todos foram tratados com solução salina regular, ≥ 10% dextrose e restrição da ingestão de proteína. Em 56 (40%) das hospitalizações, o manejo foi feito na unidade de terapia intensiva, 31(22%) com ventilação mecânica, 10 (7%) com hemodiálise, 16 (11%) com vasopressores e 12 (9%) com insulina. Para o resgate, a L-carnitina foi usada em 135 (96%) pacientes, o bicarbonato de sódio em 116 (82%), o benzoato de sódio em 76 (54%), o metronidazole em 10 (7%), a biotina em cerca de um quarto, a L-arginina em um quarto e antibióticos em três quartos dos pacientes hospitalizados. Sangue/concentrado de hemácias foram usados em 28 (20%), plaquetas em 26 (18%), plasma fresco congelado em 8 (6%) e fatores estimulantes de colônias de granulócitos em 10 (7%) pacientes hospitalizados. Todos os pacientes foram manejados completamente/parcialmente com fórmula de nutrição hospitalar mais uma mistura de aminoácidos, vitaminas e minerais. Para o manejo de longo prazo, 24 (80%) dos pacientes foram tratados com L-carnitina, 22 (73%) com benzoato de sódio, 6 (20%) com biotina, a metade com tratamento alcalino e 4 (13%) com uso regular de metronidazole. Quase todos os pacientes foram tratados com fórmulas médicas e acompanhamento regular.Conclusão : O manejo adequado e agressivo de crises metabólicas com restrição da ingestão de proteína, ...

Ammonia levels and the severity of hepatic encephalopathy

To evaluate the correlation between ammonia levels with the severity of HE in patients coming to the tertiary care hospital with liver cirrhosis and hepatic encephalopathy [HE]. Descriptive, analytical study. Shifa International Hospital, Islamabad, from January 2011 to February 2012. A total of 135 patients with liver cirrhosis and HE had serum ammonia levels measured on admission. The diagnosis of HE was based on clinical criteria, and its severity was graded according to the West Haven Criteria for grading of mental status. Ammonia levels were correlated with the severity of HE using Spearman rank correlation. Out of 20 patients with normal ammonia levels, 13 [65%] were in HE I-II, 6 [30%] were in grade-III, while 1 [5%] patient was in grade-IV HE. Out of 45 patients with mild hyperammonemia, 27 [60%] were in grade I-II, 12 [26%] were in grade-III and 6 [13%] were in grade-IV HE. Out of 34 patients with moderate hyperammonemia, 9 [26%] were in grade I-II, 18 [53%] were in grade-III, and 7 [20%] were in grade-IV HE. Out of 36 patients with severe hyperammonemia, 31 [86%] patients were in grade-IV HE [p < 0.001]. Ammonia levels correlated with the severity of hepatic encephalopathy. Greater the ammonia level, severe is the grade of hepatic encephalopathy

Indicadores de gobernanza y protección social en salud en América Latina: fortalezas, debilidades y lecciones desde México / Health governance and social protection indicators in Latin-America: strengths, weaknesses and lessons-learned from six Mexican states

Objetivo En el contexto de reformas de los sistemas de salud es impostergable el desarrollo de proyectos de investigación evaluativa para identificar buenas prácticas. En este sentido el objetivo de este estudio fue identificar indicadores de gobernanza y protección social en salud. Métodos Investigación evaluativa con universo de estudio referido al sistema de salud para no asegurados en seis Estados de México. Para los datos primarios se realizaron entrevistas a profundidad con informantes clave de los Estados participantes; para los datos secundarios se utilizaron estadísticas oficiales y los resultados del proyecto macro sobre reformas, políticas de salud y gobernanza en México. El procesamiento y análisis de datos se realizó con dos paquetes de software: Atlas Ti y Policy Maker. Resultados Una lista de fortalezas y debilidades se presenta como evidencia de la gobernanza del sistema de salud. La rendición de cuentas en el nivel federal, aunque no está ausente, se mantiene con un carácter prescriptivo, en los Estados y municipios aún está ausente un sistema de rendición de cuentas y de transparencia en la asignación de recursos y en cuanto a las estrategias de democratización de la salud. Conclusiones En todos los Estados hay bajos niveles de gobernanza y dificultad en la conducción efectiva de programas y estrategias de reforma con falta de precisión en las reglas y los roles con que operan los diferentes actores del sistema de salud.

Objective Evaluative research projects for identifying good practice have been postponed regarding health system reform. This study was thus aimed at identifying health governance and social protection indicators. Methods This study involved evaluative research regarding the health system for the uninsured part of the population in six Mexican states. The primary data was obtained from in-depth interviews with key players from the participating states; official statistics and the results of a macro-project concerned with Mexican health and governance reform and policy was used for secondary. Atlas Ti and Policy Maker software were used for processing and analysing the data. Results A list of strengths and weaknesses was presented as evidence of health system governance. Accountability at federal level (even though not lacking) was of a prescriptive nature and a system of accountability and transparency regarding the assignment of resources and strategies for the democratisation of health in the states and municipalities was still lacking. Conclusions All six states had low levels of governance and experienced difficulty in conducting effective reform programmes and strategies involving a lack of precision regarding the rules and roles adopted by different health system actors.