scholarly journals Long-term follow-up of glycemic and neurological outcomes in an international series of patients with sulfonylurea-treated ABCC8 permanent neonatal diabetes

2020 ◽  
Author(s):  
Pamela Bowman ◽  
Frances Mathews ◽  
Fabrizio Barbetti ◽  
Maggie H. Shepherd ◽  
Janine Sanchez ◽  
...  
Keyword(s):  
Glycemic Control ◽  
Severe Hypoglycemia ◽  
Neonatal Diabetes ◽  
Neurological Outcomes ◽  
Long Term Follow Up ◽  
Neurological Features ◽  
High Doses ◽  
The Uk

<b>Objective </b><br><p> <i>ABCC8</i> mutations cause neonatal diabetes that can be transient (TNDM) or less commonly permanent (PNDM); ~90% individuals can be treated with oral sulfonylureas instead of insulin. Previous studies suggested that people with <i>ABCC8-</i>PNDM require lower sulfonylurea doses and have milder neurological features than those with <i>KCNJ11-</i>PNDM. However, these studies were short-term and included combinations of permanent and transient forms of <i>ABCC8</i>-NDM. We aimed to assess the long-term glycemic and neurological outcomes in sulfonylurea-treated <i>ABCC8</i>-PNDM. <b><br> Research Design and Methods </b><br> We studied all 24 individuals with <i>ABCC8-</i>PNDM diagnosed in the UK, Italy, France or USA known to transfer from insulin to sulfonylureas before May 2010. Data on glycemic control, sulfonylurea dose, adverse effects including hypoglycemia, and neurological features were analysed using non-parametric statistical methods. <b><br> Results </b><br> Long-term data were obtained for 21/24 individuals (median follow-up 10.0 (4.1-13.2) years). 18/21 remained on sulfonylureas without insulin at most recent follow-up. Glycemic control improved on sulfonylureas (pre-sulfonylurea vs 1-year post-transfer HbA1c 7.2% vs 5.7%, p=0.0004) and remained excellent long-term (1-year vs. 10-year HbA1c 5.7% vs. 6.5%, p=0.04), n=16. Relatively high doses were used (1-year vs 10-year dose 0.37 vs 0.25mg/kg/day glyburide, p=0.50), without any severe hypoglycemia. Neurological features were reported in 13/21 individuals: these improved following sulfonylurea transfer in 7/13. The commonest features were learning difficulties (52%), developmental delay (48%), and ADHD (38%).<b><br> Conclusions </b><br> Sulfonylurea treatment of <i>ABCC8</i>-PNDM results in excellent long-term glycemic control. Overt neurological features frequently occur and may improve with sulfonylureas, supporting early, rapid genetic testing to guide appropriate treatment and neurodevelopmental assessment. </p>

scholarly journals Long-term follow-up of glycemic and neurological outcomes in an international series of patients with sulfonylurea-treated ABCC8 permanent neonatal diabetes

2020 ◽  
Author(s):  
Pamela Bowman ◽  
Frances Mathews ◽  
Fabrizio Barbetti ◽  
Maggie H. Shepherd ◽  
Janine Sanchez ◽  
...  
Keyword(s):  
Glycemic Control ◽  
Severe Hypoglycemia ◽  
Neonatal Diabetes ◽  
Neurological Outcomes ◽  
Long Term Follow Up ◽  
Neurological Features ◽  
High Doses ◽  
The Uk

<b>Objective </b><br><p> <i>ABCC8</i> mutations cause neonatal diabetes that can be transient (TNDM) or less commonly permanent (PNDM); ~90% individuals can be treated with oral sulfonylureas instead of insulin. Previous studies suggested that people with <i>ABCC8-</i>PNDM require lower sulfonylurea doses and have milder neurological features than those with <i>KCNJ11-</i>PNDM. However, these studies were short-term and included combinations of permanent and transient forms of <i>ABCC8</i>-NDM. We aimed to assess the long-term glycemic and neurological outcomes in sulfonylurea-treated <i>ABCC8</i>-PNDM. <b><br> Research Design and Methods </b><br> We studied all 24 individuals with <i>ABCC8-</i>PNDM diagnosed in the UK, Italy, France or USA known to transfer from insulin to sulfonylureas before May 2010. Data on glycemic control, sulfonylurea dose, adverse effects including hypoglycemia, and neurological features were analysed using non-parametric statistical methods. <b><br> Results </b><br> Long-term data were obtained for 21/24 individuals (median follow-up 10.0 (4.1-13.2) years). 18/21 remained on sulfonylureas without insulin at most recent follow-up. Glycemic control improved on sulfonylureas (pre-sulfonylurea vs 1-year post-transfer HbA1c 7.2% vs 5.7%, p=0.0004) and remained excellent long-term (1-year vs. 10-year HbA1c 5.7% vs. 6.5%, p=0.04), n=16. Relatively high doses were used (1-year vs 10-year dose 0.37 vs 0.25mg/kg/day glyburide, p=0.50), without any severe hypoglycemia. Neurological features were reported in 13/21 individuals: these improved following sulfonylurea transfer in 7/13. The commonest features were learning difficulties (52%), developmental delay (48%), and ADHD (38%).<b><br> Conclusions </b><br> Sulfonylurea treatment of <i>ABCC8</i>-PNDM results in excellent long-term glycemic control. Overt neurological features frequently occur and may improve with sulfonylureas, supporting early, rapid genetic testing to guide appropriate treatment and neurodevelopmental assessment. </p>

scholarly journals Long-term Follow-up of Glycemic and Neurological Outcomes in an International Series of Patients With Sulfonylurea-Treated ABCC8 Permanent Neonatal Diabetes

Diabetes Care ◽  
2020 ◽  
Vol 44 (1) ◽  
pp. 35-42
Author(s):  
Pamela Bowman ◽  
Frances Mathews ◽  
Fabrizio Barbetti ◽  
Maggie H. Shepherd ◽  
Janine Sanchez ◽  
...  
Keyword(s):  
Neonatal Diabetes ◽  
Permanent Neonatal Diabetes ◽  
Neurological Outcomes ◽  
Long Term Follow Up

Endoscopic thoracic microdiscectomy

2005 ◽  
Vol 3 (6) ◽  
pp. 459-464 ◽  
Author(s):  
Rod J. Oskouian ◽  
J. Patrick Johnson
Keyword(s):  
Hospital Length ◽  
Outcome Data ◽  
Hospital Length Of Stay ◽  
Content Type ◽  
Neurological Outcomes ◽  
Long Term Follow Up ◽  
Thoracic Disc ◽  
Fine Print

Object. The purpose of this clinical study was to evaluate prospectively surgical and neurological outcomes after endoscopic thoracic disc surgery. Methods. The authors assessed the following quantifiable outcome data in 46 patients: operative time, blood loss, duration of chest tube insertion, narcotic use, hospital length of stay (LOS), and long-term follow-up neurological function and pain-related symptoms. In patients who presented with myelopathy there was a postoperative improvement of two Frankel grades. Pain related to radiculopathy was improved by 75% and in one patient it worsened postoperatively. The authors also present operative data, surgical outcomes, and complications. Conclusions. Thoracoscopic discectomy can be used to achieve acceptable results. It has several distinct advantages such as reduced postoperative pain, morbidity, and LOS compared with traditional open procedures.

Long-term follow-up of survivors of childhood cancer in the UK

2004 ◽  
Vol 42 (2) ◽  
pp. 161-168 ◽  
Author(s):  
Aliki Taylor ◽  
Mike Hawkins ◽  
Annie Griffiths ◽  
Helena Davies ◽  
Carolyn Douglas ◽  
...  
Keyword(s):  
Childhood Cancer ◽  
Long Term Follow Up ◽  
The Uk ◽  
Survivors Of Childhood Cancer

scholarly journals S46 Outcome after pulmonary endarterectomy (PEA): Long term follow-up of the UK national cohort: Abstract S47 Table 1.

Thorax ◽  
2013 ◽  
Vol 68 (Suppl 3) ◽  
pp. A25.3-A26
Author(s):  
J Cannon ◽  
K Page ◽  
M Roots ◽  
A Ponnaberanam ◽  
C Tracy ◽  
...  
Keyword(s):  
Pulmonary Endarterectomy ◽  
Long Term Follow Up ◽  
National Cohort ◽  
The Uk
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