Síndrome de Herlyn-Werner-Wünderlich: a propósito de un caso / Herlyn-Werner-Wünderlich syndrome: A case report

El síndrome de Herlyn-Werner-Wünderlich es una anomalía congénita rara del tracto urogenital que afecta a los conductos de Müller y de Wolff con la tríada útero didelfo, obstrucción de hemivagina y agenesia renal ipsilateral. Las pacientes suelen estar asintomáticas hasta la menarquía, y a partir de entonces la clínica dependerá del grado de obstrucción vaginal, apareciendo frecuentemente masa y dolor pélvico. El diagnóstico suele ser radiológico, siendo de elección la resonancia de abdomen y pelvis que nos confirmará las alteraciones genitourinarias en su totalidad, y el tratamiento es quirúrgico con resección del tabique y drenaje de la vagina obstruida

Herlyn-Werner-Wünderlich syndrome is a rare congenital anomaly of the urogenital tract affecting the Müller and Wolff ducts with the uterus didelphys triad, hemivagina obstruction and ipsilateral renal agenesis. Patients are usually asymptomatic until menarche and thereafter the clinical practice will depend on the degree of vaginal obstruction, with frequently appearing mass and pelvic pain. The diagnosis is usually radiological, with the resonance of abdomen and pelvis chosen that will confirm us the genitourinary alterations in its totality and the treatment is surgical with resection of the septum and drainage of the obstructed vagina

Paciente VIH con infección por Pneumocystis jirovecii: hallazgos en tomografía computarizada de alta resolución / HIV patient with Pneumocystis jiroveci infection: findings in high resolution tomography (HRCT)

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Efficacy of an extravascular lung water-driven negative fluid balance protocol

OBJECTIVE: To analyze the efficacy of negative fluid balance in hypoxemic patients with an elevated extravascular lung water index (EVLWI). DESIGN: A retrospective observational study was made. SETTING: Intensive Care Unit of Virgen de las Nieves Hospital (Spain). PARTICIPANTS: Forty-four patients participated in the study. INTERVENTIONS: We analyzed our database of hypoxemic patients covering a period of 11 consecutive months. We included all hemodynamically stable and hypoxemic patients with EVLWI > 9 ml/kg. The protocol dictates a negative fluid balance between 500 and 1500 ml/day. We analyzed the impact of this negative fluid balance strategy upon pulmonary, hemodynamic, and renal function. MAIN VARIABLES OF INTEREST: Demographic data, severity scores, clinical, hemodynamic, pulmonary, metabolic and renal function data. RESULTS: Thirty-three patients achieved negative fluid balance (NFB group) and 11 had a positive fluid balance (PFB group). In the former group, PaO2/FiO2 improved from 145 (IQR 106, 200) to 210 mmHg (IQR 164, 248) (p < 0.001), and EVLWI decreased from 14 (11, 18) to 10 ml/kg (8, 14) (p < 0.001). In the PFB group, EVLWI also decreased from 11 (10, 14) to 10 ml/kg (8, 14) at the end of the protocol (p = 0.004). For these patients there were no changes in oxygenation, with a PaO2/FiO2 of 216 mmHg (IQR 137, 260) at the beginning versus 205 mmHg (IQR 99,257) at the end of the study (p = 0.08). CONCLUSION: Three out of four hypoxic patients with elevated EVLWI tolerated the NFB protocol. In these subjects, the improvement of various analyzed physiological parameters was greater and faster than in those unable to complete the protocol. Patients who did not tolerate the protocol were usually in more severe condition, though a larger sample would be needed to detect specific characteristics of this group

OBJETIVO: Analizar la eficacia del balance hídrico negativo en pacientes hipoxémicos y con Agua Pulmonar Extravascular Indexada (EVLWI) elevada. Diseño: Estudio retrospectivo y observacional. ÁMBITO: Unidad de Cuidados Intensivos del Hospital Virgen de las Nieves. Participantes: 44 pacientes. Intervenciones: Se analizó la base de datos de pacientes hipoxémicos durante 11 meses consecutivos. Se incluyeron los pacientes hipoxémicos, hemodinámicamente estables y con EVLWI > 9 ml/kg. El protocolo dicta un balance hídrico negativo entre 500 y 1500 ml/día. Se analizó el impacto de esta estrategia de balance negativo en la función respiratoria, hemodinámica y renal. Variables de interés principales: Datos demográficos, escalas de gravedad y datos clínicos hemodinámicos, respiratorios, metabólicos y de función renal. RESULTADOS: 33 pacientes lograron balance hídrico negativo (Grupo BHN) y 11 tuvieron balance hídrico positivo (Grupo BHP). En el grupo BHN la PaO2/FiO2 pasó de 145 (IQR 106,200) a 210 (IQR 164, 248) mmHg (p < 0.001), el EVLWI descendió de 14 (11, 18) a 10 (8, 14) ml/kg (p < 0.001). En el grupo BHP, el EVLWI también descendió de 11(10, 14) a 10 (8, 14) ml/kg al final del protocolo (p = 0.004); en este último grupo no hubo cambios estadísticamente significativos en la oxigenación y la PaO2/FiO2 pasó de 216 (IQR 137, 260) a 205 (IQR 99, 257) mmHg (p = 0.08). CONCLUSIÓN: Tres de cada cuatro pacientes hipoxémicos y con EVLWI elevados toleraron el protocolo; en ellos, la mejora de diversos parámetros analizados fue mayor y más rápida que en los pacientes que no hicieron balance negativo. Los pacientes que no toleraron el protocolo fueron los más graves aunque se necesitaría una muestra mayor para determinar las características específicas en estos

Efficacy of an extravascular lung water-driven negative fluid balance protocol.

OBJECTIVE: To analyze the efficacy of negative fluid balance in hypoxemic patients with an elevated extravascular lung water index (EVLWI). DESIGN: A retrospective observational study was made. SETTING: Intensive Care Unit of Virgen de las Nieves Hospital (Spain). PARTICIPANTS: Forty-four patients participated in the study. INTERVENTIONS: We analyzed our database of hypoxemic patients covering a period of 11 consecutive months. We included all hemodynamically stable and hypoxemic patients with EVLWI>9ml/kg. The protocol dictates a negative fluid balance between 500 and 1500ml/day. We analyzed the impact of this negative fluid balance strategy upon pulmonary, hemodynamic, and renal function. MAIN VARIABLES OF INTEREST: Demographic data, severity scores, clinical, hemodynamic, pulmonary, metabolic and renal function data. RESULTS: Thirty-three patients achieved negative fluid balance (NFB group) and 11 had a positive fluid balance (PFB group). In the former group, PaO2/FiO2 improved from 145 (IQR 106, 200) to 210mmHg (IQR 164, 248) (p<0.001), and EVLWI decreased from 14 (11, 18) to 10ml/kg (8, 14) (p<0.001). In the PFB group, EVLWI also decreased from 11 (10, 14) to 10ml/kg (8, 14) at the end of the protocol (p=0.004). For these patients there were no changes in oxygenation, with a PaO2/FiO2 of 216mmHg (IQR 137, 260) at the beginning versus 205mmHg (IQR 99,257) at the end of the study (p=0.08). CONCLUSION: Three out of four hypoxic patients with elevated EVLWI tolerated the NFB protocol. In these subjects, the improvement of various analyzed physiological parameters was greater and faster than in those unable to complete the protocol. Patients who did not tolerate the protocol were usually in more severe condition, though a larger sample would be needed to detect specific characteristics of this group.