ABSTRACT
INTRODUCTION: Atypical hemolytic uremic syndrome (aHUS) is a rare and genetically mediated systemic disease most often caused by uncontrolled and chronic complement activation that leads to systemic thrombotic microangiopathy, renal and extra-renal damage. MATERIALS AND METHODS: This is descriptive, retrospective and multicenter study, which reports demographic, clinical, laboratory, and genetic characteristics, as well as their treatment response and outcome of 20 aHUS patients diagnosed between 2014 and 2018. RESULTS: Most patients were female adults (75%) and 30% were associated to pregnancy/postpartum, 15% to autoimmune disease, and 65% to infections. Gastrointestinal involvement (75%) was the most frequent extra-renal organ damage. Antenatal mortality and mortality rate were 5% and 10%, respectively. 25% of the patients progressed to end-stage renal disease. In 4/8 of patients treated within 1 week of presentation, eculizumab treatment restored multi-organ function after 4 weeks of treatment. CFH (37%) and CFI (25%) mutations were the most frequent. CONCLUSION: This is the first series of aHUS cases of Colombian Caribbean region which reports the clinical and epidemiological characteristics of this condition in this region.
Subject(s)
Atypical Hemolytic Uremic Syndrome , Thrombotic Microangiopathies , Adult , Atypical Hemolytic Uremic Syndrome/epidemiology , Atypical Hemolytic Uremic Syndrome/genetics , Atypical Hemolytic Uremic Syndrome/therapy , Colombia/epidemiology , Complement Activation , Female , Humans , Male , Pregnancy , Retrospective Studies , Thrombotic Microangiopathies/complications , Thrombotic Microangiopathies/diagnosis , Thrombotic Microangiopathies/drug therapyABSTRACT
Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiological entity characterized by variable neurological manifestations, primarily caused by pathophysiological changes related to cerebral autoregulation that result in radiologically evident vasogenic edema. It is usually associated with hypertensive states, but it is not exclusively related to those. A healthy 18-year-old primigravid woman with no proteinuria or hypertension on admission presented with normotensive PRES. She had an intense diffuse headache that preceded a generalized tonic-clonic seizure. Her neurological status deteriorated, and hypertension was detected afterward. Brain imaging revealed bilateral vasogenic edema in the occipital region. Magnesium sulfate and antihypertensive medications were administered. A cesarean section was performed, and her neurological symptoms subsequently improved, leading to discharge with no complications. This case highlights the importance of suspecting PRES in pregnant patients even in the absence of preeclampsia.
ABSTRACT
Paciente femenina en el puerperio quirúrgico mediato, a quien en su estancia en unidad de cuidados intensivos obstétrica se le inició manejo para eclampsia, con hallazgo incidental en resonancia magnética cerebral de neurocisticercosis, siendo tratada con Albendazol, Dexametasona y Acido Valproico, y se logró evolución satisfactoria después de 4 días. Entre las entidades con las que se establece un diagnóstico diferencial con la eclampsia no es frecuente relacionarlo con la neuroinfección; sin embargo, en áreas endémicas para neurocisticercosis permite que se convierta en una posibilidad diagnóstica que se debe tener en cuenta en áreas de alta incidencia y de gran flujo de inmigración poblacional desde las áreas de influencia.
Wepresent the case of a female patient in puerperal period, who in her stay in intensive care unit management for Eclampsia began as incidental finding on brain Magnetic resonance imaging with neurocysticercosis discovers, being treated with albendazole, dexamethasone and Valproic Acid achieving satisfactory progress after 4 days. Among the entities in which a differential diagnosis with eclampsia is established, it is not often relate with neuroinfection , however, in areas endemic for neurocysticercosis , can it become a possibility diagnoses to be considered in areas of high incidence and large flow of population migration from areas of influence.
