Long-term follow-up of a child with Wolcott-Rallison syndrome

2021 ◽  
Vol 14 (5) ◽  
pp. e242376
Author(s):  
Nikhil Shah ◽  
Madhura Bharat Karguppikar ◽  
Vaman Khadilkar ◽  
Anuradha Khadilkar

Wolcott-Rallison syndrome (WRS), the most common cause of permanent neonatal diabetes mellitus (DM) in consanguineous families, has a poor prognosis, with a mean survival of 5.8 years. Majority of children with WRS succumb to the disease in the first decade of life. We present the long-term follow-up of an 8-year-2-month-old girl with genetically proven WRS who was born to a non-consanguineous parentage. She is on basal bolus regimen of insulin therapy for DM. In addition, she was noted to have features of skeletal dysplasia at 3 years and 3 months of age, which has led to her short stature. Surprisingly, she has had no episodes of hepatitis or liver dysfunction so far, which is frequently seen in children with WRS. To the best of our knowledge, she is the oldest surviving patient with WRS reported in India and South Asia.

Diabetes Care ◽  
2020 ◽  
Vol 44 (1) ◽  
pp. 35-42
Author(s):  
Pamela Bowman ◽  
Frances Mathews ◽  
Fabrizio Barbetti ◽  
Maggie H. Shepherd ◽  
Janine Sanchez ◽  
...  

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Joanna Wojtasik-Bakalarz ◽  
Zoltan Ruzsa ◽  
Tomasz Rakowski ◽  
Andreas Nyerges ◽  
Krzysztof Bartuś ◽  
...  

The most relevant comorbidities in patients with peripheral artery disease (PAD) are coronary artery disease (CAD) and diabetes mellitus (DM). However, data of long-term follow-up of patients with chronic total occlusion (CTO) are scarce. The aim of the study was to assess the impact of CAD and DM on long-term follow-up patients after superficial femoral artery (SFA) CTO retrograde recanalization. In this study, eighty-six patients with PAD with diagnosed CTO in the femoropopliteal region and at least one unsuccessful attempt of antegrade recanalization were enrolled in 2 clinical centers. Mean time of follow-up in all patients was 47.5 months (±40 months). Patients were divided into two groups depending on the presence of CAD (CAD group: n=45 vs. non-CAD group: n=41) and DM (DM group: n=50 vs. non-DM group: n=36). In long-term follow-up, major adverse peripheral events (MAPE) occurred in 66.6% of patients with CAD vs. 36.5% of patients without CAD and in 50% of patients with DM vs. 55% of non-DM subjects. There were no statistical differences in peripheral endpoints in both groups. However, there was a statistically significant difference in all-cause mortality: in the DM group, there were 6 deaths (12%) (P value = 0.038). To conclude, patients after retrograde recanalization, with coexisting CTO and DM, are at higher risk of death in long-term follow-up.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Kitae Kim ◽  
Shuichiro Kaji ◽  
Takeshi Kitai ◽  
Atsushi Kobori ◽  
Natsuhiko Ehara ◽  
...  

Introduction: Ischemic mitral regurgitation (IMR) portends a poor prognosis during long-term follow-up and has been identified as an independent predictor of heart failure (HF) and reduced long-term survival. Despite the poor prognosis with chronic IMR, few studies report the impact of IMR on long-term prognosis in patients with acute myocardial infarction (AMI) who underwent primary percutaneous coronary intervention (PCI). Methods: We studied 674 consecutive patients with AMI from 2000 to 2006 who underwent emergent coronary angiography and primary PCI, and who were assessed by transthoracic echocardiography during index hospitalization. Primary outcomes were cardiac death and the development of HF during follow-up. Results: The mean age of the study patients was 65±12 years and 534 patients (79%) were men. Sixty patients (9%) had moderate or severe MR before hospital discharge. Patients with moderate or severe MR were older, more frequently non-smoker, and more likely to have Killip class ≥2, lower ejection fraction, larger left ventricular end-diastolic volume, compared with patients with no or mild MR. During the mean follow-up period of 5.7±3.6 years, 35 cardiac deaths and 53 episodes of HF occurred. Kaplan-Meier analysis revealed that patients with moderate or severe MR had significantly increased risk for cardiac death (P<0.001), and HF (P<0.001), compared with patients with no or mild MR. Multivariate analysis revealed that moderate or severe MR was the significant predictor of the development of cardiac death (P<0.001), and the development of HF (P=0.006), independently of age, gender, history of MI, Killip class ≥2, initial TIMI flow≤1, peak CPK level, ejection fraction. Conclusions: Moderate or severe IMR detected early after AMI was independently associated with adverse cardiac events during long-term follow-up in patients with AMI after primary PCI.


Pancreatology ◽  
2020 ◽  
Vol 20 (5) ◽  
pp. 813-821
Author(s):  
Sinead N. Duggan ◽  
Donal B. O’Connor ◽  
Andrius Antanaitis ◽  
John R. Campion ◽  
Oladapo Lawal ◽  
...  

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