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1.
Am J Case Rep ; 18: 644-648, 2017 Jun 09.
Article in English | MEDLINE | ID: mdl-28596512

ABSTRACT

BACKGROUND Fabry disease is a rare and progressive X-linked inherited disorder of glycosphingolipid metabolism that is due to deficient or absent lysosomal a-galactosidase A activity. Among its other associated signs and symptoms, patients present with renal failure and proteinuria, which are markers of disease progression. Renin-angiotensin-aldosterone system (RAAS) blockers can slow the progression of chronic renal failure and proteinuria. In fact, some studies have shown the beneficial effects of paricalcitol on proteinuria. CASE REPORT We present a case of a female patient with the classic variant of Fabry disease. She was treated with a high dose of paricalcitol as an antiproteinuric agent due to unsatisfactory double-RAAS blockage, which resulted in transient worsening of cardiac and renal function. CONCLUSIONS Despite the positive effects of paricalcitol as an antiproteinuric agent, as previously shown by some authors, our case highlights the possible serious adverse effects associated with the use of high doses of this drug.


Subject(s)
Bone Density Conservation Agents/adverse effects , Clinical Deterioration , Ergocalciferols/adverse effects , Fabry Disease/drug therapy , Heart Failure/etiology , Kidney Failure, Chronic/etiology , Adult , Bone Density Conservation Agents/administration & dosage , Dose-Response Relationship, Drug , Ergocalciferols/administration & dosage , Fabry Disease/complications , Female , Heart Failure/prevention & control , Humans , Kidney Failure, Chronic/prevention & control , Proteinuria/etiology , Proteinuria/prevention & control
2.
Wien Klin Wochenschr ; 129(1-2): 16-20, 2017 Jan.
Article in English | MEDLINE | ID: mdl-27848073

ABSTRACT

BACKGROUND: In-hospital hyperglycemia is common and associated with an increased risk of in-hospital mortality and extensive length of stay but there are only few studies on real-life hyperglycemia and diabetes management. METHODS: In this cross-sectional, non-interventional, prospective study we analyzed medical charts on glycemia status at our internal medicine department for 5 consecutive months. Patients were grouped by departments and divided into subgroups by diabetes type, etiology and duration. Physicians answered a questionnaire regarding knowledge on national guidelines and personal opinions on the subject. RESULTS: A total of 7080 capillary blood glucose measurements from 308 patients were included in the study. Patients were of Caucasian origin with a mean age 72.7 ± 10.7 years and 50.3% were male. Of the measurements 63.3% were within glycemia goals, 2.7% in the range of hypoglycemia and 0.3% of hyperglycemic syndromes. The mean value was 8.88 ± 3.5 mmol/l (159.84 ± 63 mg/dl). There were no differences in mean glucose measurements but significant differences in reaching glycemia target goals and frequency of acute complications between intensive care, general ward and palliative care patients. Subgroup analysis confirmed the association between glycemia management and newly discovered diabetes, type 1 diabetes, steroid use and disrupted food intake (p < 0.05 for all). CONCLUSIONS: Our results on in-hospital glycemia management seem comparable to previously published data. Patients with newly discovered diabetes, type 1 diabetes, treated with steroids and those eating improperly should be closely monitored. Additional education on guidelines and steroid-induced hyperglycemia accompanied by updated medical charts represent possible improvements. Quality standards for in-hospital glycemia management should be established.


Subject(s)
Clinical Competence/statistics & numerical data , Guideline Adherence/statistics & numerical data , Hyperglycemia/diagnosis , Hyperglycemia/therapy , Practice Guidelines as Topic , Practice Patterns, Physicians'/statistics & numerical data , Adult , Aged , Aged, 80 and over , Blood Glucose/analysis , Clinical Competence/standards , Female , Hospitals, General , Humans , Hyperglycemia/epidemiology , Longitudinal Studies , Male , Middle Aged , Slovenia/epidemiology
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