PICO questions and DELPHI methodology for improving the management of patients with acute hepatic porphyria.

BACKGROUND: Acute hepatic porphyrias (AHPs) are a group of rare diseases that encompasses acute intermittent porphyria, variegate porphyria, hereditary coproporphyria, and 5-aminolaevulinic acid dehydratase deficiency porphyria. Symptoms of AHP are nonspecific which, together with its low prevalence, difficult the diagnosis and follow-up of these patients. MATERIAL AND METHODS: This project used DELPHI methodology to answer PICO questions related to management of patients with AHPs. The objective was to reach a consensus among multidisciplinary porhyria experts providing answers to those PICO questions for improving diagnosis and follow-up of patients with AHP. RESULTS: Ten PICO questions were defined and grouped in four domains: 1. Biochemical diagnosis of patients with AHP. 2. Molecular tests for patients with AHP. 3. Follow-up of patients with AHP. 4. Screening for long-term complications of patients with AHP. CONCLUSIONS: PICO questions and DELPHI methodology have provided a consensus on relevant and controversial issues for improving the management of patients with AHP.

[Vascular adrenal cyst causing difficult to control high blood pressure]. / Hipertensión arterial mal controlada secundaria a quiste vascular suprarrenal.

Hypertension is a prevalent disease in developed countries. Adrenal masses, and especially adrenal cysts, are a rare and usually asymptomatic finding, which can go unnoticed or be detected as incidental findings in imaging tests. These circumstances make the multidisciplinary approach mandatory. The case is presented on a 72 year-old woman with uncontrolled high blood pressure referred to the Urology Department due to the incidental finding of a right retroperitoneal mass. A functional and imaging study was performed, establishing a diagnosis of adrenal cyst causing hypertensive symptoms. A literature search was performed in order to assess diagnostic and therapeutic approaches. With the diagnosis of adrenal cyst causing uncontrolled high blood pressure, a right laparoscopic adrenalectomy was performed. After surgery the patient has maintained blood pressure within the normal range. A multidisciplinary approach is necessary for the management of rare diseases. The surgical approach, if possible, should be laparoscopic.

[Results of pulmonary embolism treatment in a tertiary hospital short stay unit. Is this the right place?]. / Resultados del tratamiento médico de la embolia de pulmón en una unidad de corta estancia de un hospital de tercer nivel: ¿es el lugar adecuado?

OBJECTIVES: To determine the mean stay (MS) of patients with pulmonary embolism (PE) in a thrombosis unit (TU) with a short stay unit (SSU) in a tertiary hospital. To compare the data collected with those of other hospitals in the same region, of other regions (Autonomous Communities [AACC]), and within the same hospital in the year before the SSU opened. MATERIAL AND METHODS: A descriptive retrospective observational study that included patients with a diagnosis of PE in the University Hospital Virgen de la Arrixaca (HCUVA) in 2012. These data were classified by hospital department, and used for calculating the mean stay. This was then compared with that of other hospitals in our region, with the rest of the regions, and with the data in 2007 (the last year without a TU). RESULTS: A total of 113patients with PE were included, 60 (53%) in the TU with an MS of 4.39, in Oncology, 7.45, and Internal Medicine (IM), 15.38days. There were no deaths in the TU and only 3 (5%) readmissions. Published data showed that the MS in all hospitals in our region was 8.25, 5.18 in our hospital, and higher in the rest of hospitals. The best AACC was the Basque Country with an MS of 6.85days. In 2007, there were 70patients with PE in the HCUVA, 34 (49%) in IM, with an MS of 8.50, Oncology 11 (31%) with an MS 9.64, and Chest Diseases 3 (4.3%) with an MS 19days, and with an overall mortality of 11% and a rate of readmissions in IM of 6%. CONCLUSION: The mean stay for a PE in the SSU of a TU was lower than in the rest of the hospital departments, lower than the rest hospitals of our region, lower than the rest of the regions, and lower than any department of our hospital before the SSU existed, without increasing the readmission or mortality rate.

Rhabdomyolysis after initial session of indoor cycling: analysis of 11 patients.

AIM: The aim of this paper was to highlight the special frequency of cases of rhabdomyolysis related to the practice of indoor cycling and to define the characteristics, course, and outcome of this disease. METHODS: Retrospective review of clinical histories of patients diagnosed with rhabdomyolysis after indoor cycling in our unit from January 2012 to April 2013. RESULTS: Eleven patients were analyzed. All patients, regardless of the degree of previous physical training, were diagnosed after a first session of indoor cycling. Mean age was 27.63 years (SD=5.74). Fifty-four percent were women. Creatine kinase (CK) levels gradually decreased in response to rest and intensive intravenous hydration. Only in two cases was renal failure observed, and in none were electrolyte disorders, disseminated intravascular coagulation (DIC) or compartmental syndrome detected. CONCLUSION: A first session of indoor cycling has become a common cause of rhabdomyolysis secondary to the physical exercise in recent years, which should alert those responsible for teaching this sport of the need for a gradual start under adequate hydration and environmental conditions, because although the condition has a benign course with adequate treatment and the complication rate is low, there are patients with increased susceptibility to very high CK blood levels requiring hospitalization for treatment and follow-up of possible complications.