Most Read Articles
Elvira Manzano, 4 days ago
Bisphosphonates have proven antifracture efficacy and remain to be the cornerstone of osteoporosis treatment. However, a drug holiday is of particular importance with bisphosphonates due to some signals with long-term use of the drug, including rare incidence of atypical femoral fracture (AFF) and osteonecrosis of the jaw (ONJ), says a leading endocrinologist at AFOS 2017.
Yesterday
Breast cancer patients have notably different microbiomes in the local breast tissue and urinary tract, a recent study reveals. Particularly, species in the Methylobacterium genus are reduced in the local breast tissue while the urinary tract is enriched in gram-positive bacteria.
Pearl Toh, 13 Oct 2017
Women with higher plasma tryptophan concentrations were less likely to have poor sleep quality during pregnancy, especially among those with anxiety symptoms, according to the GUSTO* study.
Tristan Manalac, Yesterday
Statin use, particularly in smokers, obese patients and nondiabetics, has a strong and dose-dependent association with decreasing risk of pancreatic ductal adenocarcinoma (PDAC), according to a new study.

Factors for Fabry disease progression during enzyme replacement therapy

16 Aug 2017

Hypertension, cardiac fibrosis, renal dysfunction and proteinuria predict faster progression of Fabry disease (FD) while on enzyme replacement therapy (ERT), according to a recent study.

Investigators classified 293 treatment-naïve FD patients (median age at initiation 45.5 years; 55.6 male) into two according to FD phenotype: classical (n=203) and nonclassical (n=90). All patients subsequently received ERT with either agalsidase alfa or agalsidase beta for 9 months.

Over a median follow-up of 6.8 (0.8 to 15.4) years, 102 (34.8 percent) clinical events in patients on ERT were recorded. The resulting rate of events was 0.66 per 10 patient years at risk. Cardiac events, observed in 53 patients, were the most common. This was followed by stroke or transient ischaemic attacks (n=24), renal events (n=16) and death (n=9).

Cox regression analysis showed that a history of clinical (hazard ratio [HR], 2.37; 95 percent CI, 1.47 to 3.85; p<0.001) and renal (HR, 1.29; 1.81 to 7.21; p<0.001) events prior to ERT initiation, and hypertension (HR, 1.87; 1.18 to 2.97; p<0.01) significantly raised the risk of new clinical events.

Additionally, the risk of clinical events significantly increased by 19 percent (p<0.001) with every 10 mL/min/1.73m2-decrease in estimated glomerular filtration rate (eGFR) at baseline. Patients with proteinuria also experienced a significant decline in eGFR (p=0.005).

An eGFR of <60 mL/min/1.73m2 was also significantly associated with a higher risk of development of clinical events during ERT (HR, 3.58; 2.12 to 6.05; p<0.001).

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Most Read Articles
Elvira Manzano, 4 days ago
Bisphosphonates have proven antifracture efficacy and remain to be the cornerstone of osteoporosis treatment. However, a drug holiday is of particular importance with bisphosphonates due to some signals with long-term use of the drug, including rare incidence of atypical femoral fracture (AFF) and osteonecrosis of the jaw (ONJ), says a leading endocrinologist at AFOS 2017.
Yesterday
Breast cancer patients have notably different microbiomes in the local breast tissue and urinary tract, a recent study reveals. Particularly, species in the Methylobacterium genus are reduced in the local breast tissue while the urinary tract is enriched in gram-positive bacteria.
Pearl Toh, 13 Oct 2017
Women with higher plasma tryptophan concentrations were less likely to have poor sleep quality during pregnancy, especially among those with anxiety symptoms, according to the GUSTO* study.
Tristan Manalac, Yesterday
Statin use, particularly in smokers, obese patients and nondiabetics, has a strong and dose-dependent association with decreasing risk of pancreatic ductal adenocarcinoma (PDAC), according to a new study.