RESUMO
ABSTRACT This study reports a case of a 13-year-old male with a 3-year history of severe and intermittent hypokalemia episodes of unknown origin, requiring admission to the intensive care unit (ICU) for long QT syndrome (LQTS), finally diagnosed of redistributive hypokalemia secondary to the abuse of β-adrenergic agonists in the context of a probable factitious disorder.
RESUMO O presente estudo relata o caso de um jovem de 13 anos de idade com histórico, há três anos, de episódios de hipocalemia grave intermitente de origem desconhecida, internado em unidade de terapia intensiva (UTI) por síndrome do QT longo (SQTL). O paciente foi diagnosticado com hipocalemia por redistribuição secundária ao abuso de agonistas β-adrenérgicos, em contexto de provável transtorno factício.
Assuntos
Humanos , Masculino , Adolescente , Síndrome do QT Longo/induzido quimicamente , Agonistas Adrenérgicos beta/efeitos adversos , Transtornos Autoinduzidos/diagnóstico , Hipopotassemia/induzido quimicamente , Potássio/sangue , Potássio/uso terapêutico , Recidiva , Síndrome do QT Longo/psicologia , Agonistas Adrenérgicos beta/sangue , Albuterol/sangue , Overdose de Drogas/complicações , Hipopotassemia/psicologia , Hipopotassemia/sangueRESUMO
This study reports a case of a 13-year-old male with a 3-year history of severe and intermittent hypokalemia episodes of unknown origin, requiring admission to the intensive care unit (ICU) for long QT syndrome (LQTS), finally diagnosed of redistributive hypokalemia secondary to the abuse of ß-adrenergic agonists in the context of a probable factitious disorder.
Assuntos
Agonistas Adrenérgicos beta/efeitos adversos , Transtornos Autoinduzidos/diagnóstico , Hipopotassemia/induzido quimicamente , Síndrome do QT Longo/induzido quimicamente , Adolescente , Agonistas Adrenérgicos beta/sangue , Albuterol/sangue , Overdose de Drogas/complicações , Humanos , Hipopotassemia/sangue , Hipopotassemia/psicologia , Síndrome do QT Longo/psicologia , Masculino , Potássio/sangue , Potássio/uso terapêutico , RecidivaRESUMO
INTRODUCTION: Cystinosis is a rare lysosomal systemic disease that mainly affects the kidney and the eye. Patients with cystinosis begin renal replacement therapy during the first decade of life in absence of treatment. Prognosis of cystinosis depends on early diagnosis, and prompt starting and good compliance with cysteamine treatment. Kidney disease progression, extra-renal complications and shorter life expectancy are more pronounced in those patients that do not follow treatment. The objective of this work was to elaborate recommendations for the comprehensive care of cystinosis and the facilitation of patient transition from paediatric to adult treatment, based on clinical experience. The goal is to reduce the impact of the disease, and to improve patient quality of life and prognosis. METHODS: Bibliographic research and consensus meetings among a multidisciplinary professional team of experts in the clinical practice, with cystinotic patients (T-CiS.bcn group) from 5 hospitals located in Barcelona. RESULTS: This document gathers specific recommendations for diagnosis, treatment and multidisciplinary follow-up of cystinotic patients in the following areas: nephrology, dialysis,renal transplant, ophthalmology, endocrinology, neurology, laboratory, genetic counselling,nursing and pharmacy. CONCLUSIONS: A reference document for the comprehensive care of cystinosis represents a support tool for health professionals who take care of these patients. It is based on the following main pillars: (a) a multi-disciplinary approach, (b) appropriate disease monitoring and control of intracellular cystine levels in leukocytes, (c) the importance of adherence to treatment with cysteamine, and (d) the promotion of patient self-care by means of disease education programmes. All these recommendations will lead us, in a second phase, to create a coordinated transition model between paediatric and adult care services which will contemplate the specific needs of cystinosis.
