Different clinical entities of the same mutation: a case report of three sisters with Wolfram syndrome and efficacy of dipeptidyl peptidase-4 inhibitor therapy

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Gurkan Tarcin ◽  
Hande Turan ◽  
Aydilek Dagdeviren Cakir ◽  
Yavuz Ozer ◽  
Ayca Aykut ◽  
...  
Keyword(s):  
Neurodegenerative Disorder ◽  
Wolfram Syndrome ◽  
Dipeptidyl Peptidase ◽  
Case Presentation ◽  
Dipeptidyl Peptidase 4 ◽  
First Case ◽  
Dipeptidyl Peptidase 4 Inhibitor ◽  
Remission Of Diabetes ◽  
Remission Phase ◽  
Three Sisters

Abstract Objectives Wolfram syndrome (WS) is a rarely seen autosomal recessive multisystem neurodegenerative disorder caused by mutations in the WFS1 gene. Case Presentation Three sisters with WS had diabetes mellitus (DM) at 4 years of age and optic atrophy. In addition, the first case had hearing impairment, and the second case had diabetes insipidus and urinary incontinence. Linagliptin was administered to the first case as add-on therapy to intensive insulin treatment 15 years after the onset of DM, and her insulin need showed a dramatic decrease. The third case had a remission phase one month after the onset of DM. Conclusions Even in cases with the same mutation, symptoms and findings may widely vary in WS. Remission of diabetes has rarely been reported in WS. Also, this report describes the first trial of a dipeptidyl peptidase-4 inhibitor in a patient with WS which provided a decrease in exogenous insulin need.

scholarly journals Clinical, Laboratory and Histological Features of Dipeptidyl Peptidase-4 Inhibitor Related Noninflammatory Bullous Pemphigoid

2021 ◽  
Vol 10 (9) ◽  
pp. 1916
Author(s):  
Ágnes Kinyó ◽  
Anita Hanyecz ◽  
Zsuzsanna Lengyel ◽  
Dalma Várszegi ◽  
Péter Oláh ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Clinical Laboratory ◽  
Case Series ◽  
Dipeptidyl Peptidase ◽  
Area Index ◽  
Histological Features ◽  
Dipeptidyl Peptidase 4 ◽  
First Case ◽  
Dipeptidyl Peptidase 4 Inhibitor ◽  
The Mean

Bullous pemphigoid (BP) is an autoimmune blistering disease of elderly patients that has shown increasing incidence in the last decades. Higher prevalence of BP may be due to more frequent use of provoking agents, such as antidiabetic dipeptidyl peptidase-4 inhibitor (DPP4i) drugs. Our aim was to assess DPP4i-induced bullous pemphigoid among our BP patients and characterize the clinical, laboratory and histological features of this drug-induced disease form. In our patient cohort, out of 127 BP patients (79 females (62.2%), 48 males (37.7%)), 14 (9 females and 5 males) were treated with DPP4i at the time of BP diagnosis. The Bullous Pemphigoid Disease Area Index (BPDAI) urticaria/erythema score was significantly lower, and the BPDAI damage score was significantly higher in DPP4i-BP patients compared to the nonDPP4i group. Both the mean absolute eosinophil number and the mean periblister eosinophil number was significantly lower in DPP4i-BP patients than in nonDPP4i cases (317.7 ± 0.204 vs. 894.0 ± 1.171 cells/μL, p < 0.0001; 6.75 ± 1.72 vs. 19.09 ± 3.1, p = 0.0012, respectively). Our results provide further evidence that DPP4i-associated BP differs significantly from classical BP, and presents with less distributed skin symptoms, mild erythema, normal or slightly elevated peripheral eosinophil count, and lower titers of BP180 autoantibodies. To our knowledge, this is the first case series of DPP4i-related BP with a non-inflammatory phenotype in European patients.

Linagliptin: A Novel Xanthine-Based Dipeptidyl Peptidase-4 Inhibitor for Treatment of Type II Diabetes Mellitus

2011 ◽  
Vol 7 (5) ◽  
pp. 325-335 ◽  
Author(s):  
Somsuvra B. Ghatak ◽  
Devang S. Patel ◽  
Neeraj Shanker ◽  
Ambrish Srivastava ◽  
Shrikalp S. Deshpande ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type Ii Diabetes ◽  
Type Ii Diabetes Mellitus ◽  
Dipeptidyl Peptidase ◽  
Type Ii ◽  
Dipeptidyl Peptidase 4 ◽  
Dipeptidyl Peptidase 4 Inhibitor

Dipeptidyl peptidase‐4 inhibitor‐related bullous pemphigoid: A comparative study of 100 patients with bullous pemphigoid and diabetes mellitus

2021 ◽  
Vol 48 (4) ◽  
pp. 486-496 ◽  
Author(s):  
Kumutnart Chanprapaph ◽  
Nathathai Pratumchart ◽  
Preeyachat Limtong ◽  
Suthinee Rutnin ◽  
Chonlaphat Sukasem ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Comparative Study ◽  
Bullous Pemphigoid ◽  
Dipeptidyl Peptidase ◽  
Dipeptidyl Peptidase 4 ◽  
Dipeptidyl Peptidase 4 Inhibitor

Physicochemical characterization of dipeptidyl peptidase-4 inhibitor alogliptin in physical mixtures with excipients

2017 ◽  
Vol 130 (3) ◽  
pp. 1575-1584 ◽  
Author(s):  
Charise Dallazem Bertol ◽  
Rafael Nicolay Pereira ◽  
Cassiana Mendes ◽  
Amarilis Scremim Paulino ◽  
Marcos Antônio Segatto Silva ◽  
...  
Keyword(s):  
Physicochemical Characterization ◽  
Dipeptidyl Peptidase ◽  
Dipeptidyl Peptidase 4 ◽  
Dipeptidyl Peptidase 4 Inhibitor ◽  
Physical Mixtures

scholarly journals Risk of morbidity and mortality in patients with type 2 diabetes treated with sodium-glucose cotransporter-2 inhibitor and/or dipeptidyl peptidase-4 inhibitor: a nationwide study

2021 ◽  
Vol 9 (1) ◽  
pp. e001765
Author(s):  
Gábor Sütő ◽  
Gergő A Molnár ◽  
Gyorgy Rokszin ◽  
Ibolya Fábián ◽  
Zoltan Kiss ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Type 2 Diabetes ◽  
Propensity Score ◽  
Propensity Score Matching ◽  
Dipeptidyl Peptidase ◽  
Dipeptidyl Peptidase 4 ◽  
Sodium Glucose Cotransporter ◽  
Dipeptidyl Peptidase 4 Inhibitor ◽  
All Cause Mortality

IntroductionMortality and disability in diabetes mellitus are determined mostly by cardiovascular complications and cancer. The impact of dipeptidyl peptidase-4 inhibitor (DPP-4i) and sodium-glucose cotransporter-2 inhibitor (SGLT2i) monotherapy or combination on long-term complications of type 2 diabetes mellitus was studied.Research design and methodsPatients with type 2 diabetes treated with DPP-4i or SGLT2i during a 3-year period were identified in the database of the National Institute of Health Insurance Fund in Hungary. All-cause mortality, acute myocardial infarction, stroke, hospitalization for heart failure (HHF), lower limb amputation (LLA) and cancer were assessed. Outcomes of add-on SGLT2i to DPP-4i treatment in comparison with switching DPP-4i therapy to SGLT2i were also evaluated. After propensity score matching, survival analysis was performed with a Cox proportional hazards model.ResultsAfter propensity score matching, both SGLT2i and DPP-4i groups included 18 583 patients. All-cause mortality (HR, 0.80; 95% CI 0.68 to 0.94; p=0.0057), HHF (HR, 0.81; 95% CI 0.71 to 0.92; p=0.0018), and risk of cancer (HR, 0.75; 95% CI 0.66 to 0.86; p<0.0001) were lower in the SGLT2i population compared with DPP-4i. Risk of LLA was higher in the SGLT2i group (HR, 1.35; 95% CI 1.03 to 1.77; p=0.0315). SGLT2i in combination with DPP-4i results in lower all-cause mortality (HR, 0.46; 95% CI 0.31 to 0.67; p=0.0001), with a lower trend in stroke, LLA, HHF and cancer, but without any statistical difference.ConclusionsSGLT2i treatment leads to a lower risk of overall mortality, HHF and cancer when compared with DPP-4i treatment. Adding SGLT2i to DPP-4i instead of switching from DPP-4i to SGLT2i further lowers the risk of all-cause mortality.

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