ABSTRACT
Severity meaning the risk of adverse outcomes, death or disability, varies along disease's characteristics and patient related factors. Its evaluation is complex and besides subjective perception several, more objective, clinical tools exist along with laboratory data that independently or used in combination, allows the clinician to approach severity of a given patient's condition or a particular disease. We will review several aspects of the evaluation of severity, clinical and laboratory based in addition of some scoring systems, emphasizing perfusion and organ dysfunction evaluation as key elements to assess severity particularly in critical situations. (AU)
Subject(s)
Humans , Male , Female , Severity of Illness Index , Patient Acuity , Perfusion/methods , Critical Care , Organ Dysfunction ScoresABSTRACT
The clinical course of patients with portal hypertension or liver disease may be complicated by two low prevalence entities with high morbimortality: the hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHT). Each one is a consequence ofan impaired hepatic clearance of several vascular mediators, triggering vasodilation of the pulmonary vascular territory in HPS and vasoconstriction with vessel remodelation in PPHT. Both disorders have some similar clinical findings, but useful findings for differential diagnosis are the presence of platypnoea and orthodeoxia in HPS, and echocardiographic extracardiac and intrapulmonary shunt in HPS or pulmonary hypertension in PPHT. Currently, liver transplantation is the only effective treatment for both entities provided that indication and timing must be accurately evaluated. We present a review and three cases of both entities.
El curso clínico de los pacientes con cirrosis y/o hipertensión portal puede verse complicado por dos entidades de baja prevalencia pero de elevada morbimortalidad, que corresponden al síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HPP). Ambas se presentan a consecuencia de un déficit en la depuración hepática de diversos mediadores vasculares, provocando en el territorio pulmonar una vasodilatación en el SHP y una vasoconstricción con remodelación vascular en la HPP. Si bien estas entidades comparten algunos aspectos clínicos, resulta útil en su diferenciación la presencia de platipnea y ortodeoxia y el hallazgo ecocardiográfico de un shunt extracardíaco e intrapulmonar en el SHP, o de hipertensión pulmonar en HPP. Hasta el momento la única terapia efectiva para ambas entidades es el trasplante hepático, cuya indicación exige una evaluación rigurosa y oportuna. Se presenta una revisión y tres casos clínicos de ambas entidades.
Subject(s)
Humans , Adolescent , Female , Middle Aged , Hypertension, Portal/diagnosis , Hypertension, Pulmonary/diagnosis , Hepatopulmonary Syndrome/diagnosis , Liver Cirrhosis/complications , Diagnosis, Differential , Hypertension, Portal/etiology , Hypertension, Portal/therapy , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Hepatic Insufficiency/complications , Prognosis , Hepatopulmonary Syndrome/etiology , Hepatopulmonary Syndrome/physiopathology , Hepatopulmonary Syndrome/therapyABSTRACT
El síndrome POEMS se caracteriza por la presencia de Polineuropatía, Organomegalia, Endocrinopatía, Gammapatía Monoclonal y Cambios en la Piel. Muchas otras manifestaciones clínicas del síndrome no están incluidas en este acrónimo, como las lesiones óseas osteoescleróticas, edema de papila, sobrecarga de volumen extravascular, trombocitosis, policitemia, acropaquia, hipertensión pulmonar, baja de peso, fatiga, diarrea, compromiso renal y artralgias. Es una patología infrecuente, de mayor prevalencia en varones, con una relación 2:1, preferentemente entre la quinta y sexta década de la vida. Es una enfermedad de curso crónico y tiene sobrevida promedio de 13,8 años en algunas series. La patogenia es desconocida y no existe en la actualidad consenso respecto al tratamiento ni estudios randomizados y controlados que comparen los resultados de diferentes terapias en esta enfermedad.
POEMS syndrome is characterized by the presence of Polyneuropathy, Organomegaly, Endocrinopathies, Monoclonal Gammopathy and Skin changes. Many other clinical manifestations of the syndrome are not included in this acronym, as the osteosclerotic lesions, papillary edema, extravascular volume overload, thrombocytosis, polycythemia, acropaquia, pulmonary hypertension, weight loss, fatigue, diarrhea, arthralgia, and renal involvement. It is an infrequent disease, more prevalent in males with a 2:1 ratio, mostly between the fifth and sixth decades of life. It has a chronic course with an average survival of 13.8 years in some series. The pathogenesis is unknown and currently there is no consensus regarding the treatment or randomized controlled studies comparing the outcomes of different therapies in this disease.