17α-hydroxylase deficiency (17OHD) is a rare disorder of secondary hypertension caused by congenital adrenal hyperplasia. In addition, co-occurrence of an adrenal tumor with 17OHD is extremely rare and easily misdiagnosed. A 33-year-old female with sicca syndrome, persistent hypertension, hypokalemia, and a left adrenal tumor was referred for confirmation of primary aldosteronism. However, the absence of secondary sexual characteristics, persistent growth beyond puberty, and laboratory data of low plasma renin activity, high aldosterone, low cortisol, low sex hormones, elevated adrenocorticotropic hormone, elevated luteinizing hormone, elevated follicle-stimulating hormone, and most importantly, decreased 17-hydroxypregnenolone, supported a diagnosis of 17OHD. We sequenced the CYP17A1 gene of the patient and her parents, which demonstrated genetic defects (D487-S,488-F489 deletion and Y329K418X). 17OHD was diagnosed. The left adrenal tumor was assessed, and a non-functional adrenal incidentaloma was confirmed; NP-59 adrenal cortical scintigraphy and adrenal venous sampling showed no functional activity and non-lateralization. Hormone replacements with estrogen, spironolactone, and prednisolone were given. The patient became more feminized and confident, and her hypertension was controlled. Early diagnosis and treatment of 17OHD not only can prevent delay development of secondary sexual characteristics but also help the patient maintain mental health and improve their quality of life. In addition, the concomitant presence of a left adrenal tumor makes misdiagnosis of a functional adenoma more likely, possibly causing unnecessary surgery and delay inappropriate treatment.
Diabetes mellitus has become a global pandemic and a major cause of death. Five million adults died from diabetes in 2017, accounting for 9.9% of all causes of mortality globally. 1 In Taiwan, diabetes was the fifth leading cause of death from 2016 to 2018, just behind malignancy, cardiovascular disease, pneumonia and cerebrovascular disease. 2 A nationwide survey in 2014 reported life expectancies of 39.6 and 33.4 years among women and men, respectively, in whom diabetes was diagnosed at the age of 40 years, which were 2.6 and 3.2 years less than in the general population. 3 It is well known that atherosclerotic cardiovascular disease (ASCVD) is the leading cause of mortality for individuals with diabetes. 4 Patients with macrovascular diseases are at increased risk of death caused by ASCVD due to the pre-existing atherosclerotic lesions; however, it is questionable whether the patients with microvascular disease are also at high risk for mortality from ASCVD. The aim of this study was to investigate the causes of in-hospital death of patients with type 2 diabetes with
Introduction: Alzheimer’s disease (AD) is the most common form of dementia. Eradication of Helicobacter pylori (H. pylori) could affect the incidence and progression of many diseases; however, there are limited studies of the association between H. pylori eradication and AD outcome. We utilized the National Health Insurance Research Database (NHIRD) of Taiwan to determine the relationship between H. pylori eradication and AD in a diabetes mellitus (DM) population.Methods: We collected data from the NHIRD and the Diabetes Mellitus Health Database in Taiwan of patients without a prior diagnosis of AD. We specified three cohorts: patients with (1) peptic ulcer disease (PUD) but no H. pylori treatment, without DM (PUD-HPRx in GP); (2) PUD and DM, but no H. pylori eradication therapy (PUD-HPRx in DM); (3) PUD and DM, with H. pylori eradication therapy (PUD+HPRx in DM). All cohorts were matched according to age, sex, Charlson Comorbidity Index score, and comorbidities.Results: Data were collected from 2000 to 2010, and 157,231 patients were enrolled in total. We compared the effects of treatment for H. pylori infection on the incidence and mortality of AD. The patients with DM who received H. pylori eradication therapy had a higher incidence of AD than the general population (adjusted hazard ratio of incidence [aHR], 1.088). Subgroup analysis showed that the risk of AD was higher in the younger patients who received H. pylori eradication therapy as compared with those who did not (aHR for younger than 45 years, 1.071; aHR of age 45-54 years, 1.089; aHR of age 55-64 years, 1.079) However, a lower mortality rate was observed in the PUD+HPRx in DM group (aHR, 0.945, compared with PUD-HPRx in DM; P < 0.001).Conclusion: In this study, we demonstrated that DM patients who underwent treatment for eradication of H. pylori had a higher incidence of AD, especially younger patients. Nevertheless, there was a lower mortality rate in patients who received H. pylori treatment. Further study is needed to clarify the interrelated roles of AD and eradication therapy for H. pylori.
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