Exocrine pancreatic function in hepatocyte nuclear factor 1β-maturity-onset diabetes of the young (HNF1B-MODY) is only moderately reduced: compensatory hypersecretion from a hypoplastic pancreas
Objectives To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing. Methods Patients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin‐stimulated magnetic resonance im...
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| Published in | Diabetic medicine Vol. 30; no. 8; pp. 946 - 955 |
|---|---|
| Main Authors | , , , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
Oxford
Blackwell Publishing Ltd
01.08.2013
Blackwell |
| Subjects | |
| Online Access | Get full text |
| ISSN | 0742-3071 1464-5491 1464-5491 |
| DOI | 10.1111/dme.12190 |
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| Abstract | Objectives
To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing.
Methods
Patients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin‐stimulated magnetic resonance imaging. Seven patients and 25 controls underwent endoscopy, while eight patients and 20 controls had magnetic resonance imaging. Ductal function was assessed according to peak bicarbonate concentrations and acinar function was assessed according to peak digestive enzyme activities in secretin‐stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined.
Results
The mean increase in secretin‐stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4 ml/min; P = 0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 mEq/L; P < 0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 kU/ml; P = 0.01; median peak elastase activity: 0.056 vs 0.130 U/ml; P = 0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (r2 = 0.71, P = 0.008) in patients. The total fluid output to pancreatic gland volume ratios were higher in patients than controls (4.5 vs 1.3 ml/cm3; P = 0.03), suggesting compensatory hypersecretion in the remaining gland.
Conclusion
Carriers of the HNF1B mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of HNF1B mutation carriers is attributable to hypoplasia, not atrophy.
What's new?
The exocrine pancreatic function in HNF1B‐MODY is moderately reduced.
Both ductal and acinar pancreatic function are affected in HNF1B‐MODY.
Ductal function in HNF1B‐MODY is dependent on the pancreatic gland size.
There is a compensatory hypersecretion in HNF1B‐MODY as a response to secretin.
The small pancreas in HNF1B‐MODY is probably attributable to hypoplasia, and not atrophy. |
|---|---|
| AbstractList | Objectives
To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing.
Methods
Patients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin‐stimulated magnetic resonance imaging. Seven patients and 25 controls underwent endoscopy, while eight patients and 20 controls had magnetic resonance imaging. Ductal function was assessed according to peak bicarbonate concentrations and acinar function was assessed according to peak digestive enzyme activities in secretin‐stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined.
Results
The mean increase in secretin‐stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4 ml/min; P = 0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 mEq/L; P < 0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 kU/ml; P = 0.01; median peak elastase activity: 0.056 vs 0.130 U/ml; P = 0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (r2 = 0.71, P = 0.008) in patients. The total fluid output to pancreatic gland volume ratios were higher in patients than controls (4.5 vs 1.3 ml/cm3; P = 0.03), suggesting compensatory hypersecretion in the remaining gland.
Conclusion
Carriers of the HNF1B mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of HNF1B mutation carriers is attributable to hypoplasia, not atrophy.
What's new?
The exocrine pancreatic function in HNF1B‐MODY is moderately reduced.
Both ductal and acinar pancreatic function are affected in HNF1B‐MODY.
Ductal function in HNF1B‐MODY is dependent on the pancreatic gland size.
There is a compensatory hypersecretion in HNF1B‐MODY as a response to secretin.
The small pancreas in HNF1B‐MODY is probably attributable to hypoplasia, and not atrophy. To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing. Patients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin-stimulated magnetic resonance imaging. Seven patients and 25 controls underwent endoscopy, while eight patients and 20 controls had magnetic resonance imaging. Ductal function was assessed according to peak bicarbonate concentrations and acinar function was assessed according to peak digestive enzyme activities in secretin-stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined. The mean increase in secretin-stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4 ml/min; P = 0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 mEq/L; P < 0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 kU/ml; P = 0.01; median peak elastase activity: 0.056 vs 0.130 U/ml; P = 0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (r² = 0.71, P = 0.008) in patients. The total fluid output to pancreatic gland volume ratios were higher in patients than controls (4.5 vs 1.3 ml/cm³; P = 0.03), suggesting compensatory hypersecretion in the remaining gland. Carriers of the HNF1B mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of HNF1B mutation carriers is attributable to hypoplasia, not atrophy. To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing.OBJECTIVESTo examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing.Patients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin-stimulated magnetic resonance imaging. Seven patients and 25 controls underwent endoscopy, while eight patients and 20 controls had magnetic resonance imaging. Ductal function was assessed according to peak bicarbonate concentrations and acinar function was assessed according to peak digestive enzyme activities in secretin-stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined.METHODSPatients with HNF1B mutations and control subjects were assessed using rapid endoscopic secretin tests and secretin-stimulated magnetic resonance imaging. Seven patients and 25 controls underwent endoscopy, while eight patients and 20 controls had magnetic resonance imaging. Ductal function was assessed according to peak bicarbonate concentrations and acinar function was assessed according to peak digestive enzyme activities in secretin-stimulated duodenal juice. The association of pancreatic exocrine function and diabetes status with pancreatic gland volume was examined.The mean increase in secretin-stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4 ml/min; P = 0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 mEq/L; P < 0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 kU/ml; P = 0.01; median peak elastase activity: 0.056 vs 0.130 U/ml; P = 0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (r² = 0.71, P = 0.008) in patients. The total fluid output to pancreatic gland volume ratios were higher in patients than controls (4.5 vs 1.3 ml/cm³; P = 0.03), suggesting compensatory hypersecretion in the remaining gland.RESULTSThe mean increase in secretin-stimulated duodenal fluid was smaller in patients than controls (4.0 vs 6.4 ml/min; P = 0.003). We found lower ductal function in patients than controls (median peak bicarbonate concentration: 73 vs 116 mEq/L; P < 0.001) and lower acinar function (median peak lipase activity: 6.4 vs 33.5 kU/ml; P = 0.01; median peak elastase activity: 0.056 vs 0.130 U/ml; P = 0.01). Pancreatic fluid volume outputs correlated significantly with pancreatic gland volumes (r² = 0.71, P = 0.008) in patients. The total fluid output to pancreatic gland volume ratios were higher in patients than controls (4.5 vs 1.3 ml/cm³; P = 0.03), suggesting compensatory hypersecretion in the remaining gland.Carriers of the HNF1B mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of HNF1B mutation carriers is attributable to hypoplasia, not atrophy.CONCLUSIONCarriers of the HNF1B mutation have lower exocrine pancreatic function involving both ductal and acinar cells. Compensatory hypersecretion suggests that the small pancreas of HNF1B mutation carriers is attributable to hypoplasia, not atrophy. The exocrine pancreatic function in HNF1B‐MODY is moderately reduced. Both ductal and acinar pancreatic function are affected in HNF1B‐MODY. Ductal function in HNF1B‐MODY is dependent on the pancreatic gland size. There is a compensatory hypersecretion in HNF1B‐MODY as a response to secretin. The small pancreas in HNF1B‐MODY is probably attributable to hypoplasia, and not atrophy. |
| Author | Wathle, G. Erchinger, F. Engjom, T. Haldorsen, I. S. Njølstad, P. R. Ræder, H. Molven, A. Dimcevski, G. Tjora, E. Aksnes, L. |
| Author_xml | – sequence: 1 givenname: E. surname: Tjora fullname: Tjora, E. organization: KG Jebsen Center for Diabetes Research, Department of Clinical Science, University of Bergen, Bergen, Norway – sequence: 2 givenname: G. surname: Wathle fullname: Wathle, G. organization: Department of Radiology, Haukeland University Hospital, Bergen, Norway – sequence: 3 givenname: F. surname: Erchinger fullname: Erchinger, F. organization: Voss Hospital, Haukeland University Hospital, Bergen, Norway – sequence: 4 givenname: T. surname: Engjom fullname: Engjom, T. organization: Department of Medicine, Haukeland University Hospital, Bergen, Norway – sequence: 5 givenname: A. surname: Molven fullname: Molven, A. organization: KG Jebsen Center for Diabetes Research, Department of Clinical Science, University of Bergen, Bergen, Norway – sequence: 6 givenname: L. surname: Aksnes fullname: Aksnes, L. organization: KG Jebsen Center for Diabetes Research, Department of Clinical Science, University of Bergen, Bergen, Norway – sequence: 7 givenname: I. S. surname: Haldorsen fullname: Haldorsen, I. S. organization: Department of Radiology, Haukeland University Hospital, Bergen, Norway – sequence: 8 givenname: G. surname: Dimcevski fullname: Dimcevski, G. organization: Department of Medicine, Haukeland University Hospital, Bergen, Norway – sequence: 9 givenname: H. surname: Ræder fullname: Ræder, H. organization: KG Jebsen Center for Diabetes Research, Department of Clinical Science, University of Bergen, Bergen, Norway – sequence: 10 givenname: P. R. surname: Njølstad fullname: Njølstad, P. R. email: pal.njolstad@uib.no organization: KG Jebsen Center for Diabetes Research, Department of Clinical Science, University of Bergen, Bergen, Norway |
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| Cites_doi | 10.1097/MPA.0b013e3181656f8 10.1073/pnas.0405776102 10.1677/joe.1.06768 10.1111/j.1464-5491.2008.02460.x 10.1111/j.1464-5491.2006.01999.x 10.7326/0003-4819-140-7-200404060-00009 10.1111/j.1445-5994.1995.tb01526.x 10.1097/MPA.0b013e31824abb4d 10.2337/db06-0859 10.3748/wjg.14.3149 10.2214/AJR.10.5713 10.1159/000161013 10.1056/NEJM197304192881603 10.1093/hmg/ddl161 10.1111/j.1572-0241.2006.00406.x 10.1007/s00330-002-1605-x 10.1097/MPA.0b013e3182072032 10.1159/000199364 10.2337/diacare.27.5.1102 10.1111/j.1399-5448.2011.00773.x 10.1038/nrendo.2011.197 10.3748/wjg.v17.i35.3957 10.2337/diabetes.55.02.06.db05-1019 10.1111/j.1572-0241.2006.00949.x 10.1093/ndt/gfm603 10.1007/BF02774228 |
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| Copyright | 2013 The Authors. Diabetic Medicine © 2013 Diabetes UK 2014 INIST-CNRS 2013 The Authors. Diabetic Medicine © 2013 Diabetes UK. |
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| Keywords | Endocrinopathy Hypoplasia Maturity onset diabetes young Obesity Nutrition Digestive system Liver Nutrition disorder Metabolic diseases Hypersecretion Genetic disease Reduction Hepatocyte Malformation Pancreas Endocrinology Nutritional status |
| Language | English |
| License | CC BY 4.0 2013 The Authors. Diabetic Medicine © 2013 Diabetes UK. |
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| Notes | istex:E5D711AEFE287F18E9EFA17C4A86F156C92530D9 Table S1 Characteristics of control groupsTable S2 Details about enzyme activity assays used [1-3]Table S3 Imaging protocol of the pancreas and upper abdomenFigure S1 Peak activity levels of α-amylase (a) and chymotrypsin (b) in duodenal juice after rapid endoscopic secretin test in controls and HNF1B mutation carriers. Bars indicate medians. There were no significant differences between the two groups with respect to these enzymes.Figure S2 Scatterplots showing relations between pancreatic lipase (a), elastase (b), α-amylase (c), chymotrypsin (d) and body-surface-adjusted pancreas volume. There were no significant correlations between digestive enzyme and body surface adjusted pancreas volume.Figure S3 Panel (a) shows the relation between age of examination and body-surface-adjusted pancreas volume. The crosses represent age of diabetes diagnosis in patients with diabetes. HbA1c levels are given for patients with no diabetes. There was a nonsignificant trend towards earlier onset of diabetes in the patients with smaller body-surface-adjusted pancreatic gland volume (hazard ratio 0.5-1.1, P = 0.12). The same relationship in 20 healthy controls is demonstrated in panel (b). There was no significant trend in body-surface-adjusted pancreas volume correlated to age.Appendix S1 Supplementary methods. ark:/67375/WNG-FWB0PGJ5-D ArticleID:DME12190 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
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| References | Haumaitre C, Fabre M, Cormier S, Baumann C, Delezoide AL, Cereghini S. Severe pancreas hypoplasia and multicystic renal dysplasia in two human fetuses carrying novel HNF1beta/MODY5 mutations. Hum Mol Genet 2006; 15: 2363-2375. Raeder H, Haldorsen IS, Ersland L, Gruner R, Taxt T, Sovik O et al. Pancreatic lipomatosis is a structural marker in nondiabetic children with mutations in carboxyl-ester lipase. Diabetes 2007; 56: 444-449. Chen YZ, Gao Q, Zhao XZ, Bennett CL, Xiong XS, Mei CL et al. Systematic review of TCF2 anomalies in renal cysts and diabetes syndrome/maturity onset diabetes of the young type 5. Chin Med J (Engl) 2010; 123: 3326-3333. DiMagno EP, Go VL, Summerskill WH. Relations between pancreatic enzyme ouputs and malabsorption in severe pancreatic insufficiency. N Engl J Med 1973; 288: 813-815. Haldorsen IS, Vesterhus M, Raeder H, Jensen DK, Sovik O, Molven A et al. Lack of pancreatic body and tail in HNF1B mutation carriers. Diabet Med 2008; 25: 782-787. Haumaitre C, Barbacci E, Jenny M, Ott MO, Gradwohl G, Cereghini S. Lack of TCF2/vHNF1 in mice leads to pancreas agenesis. Proc Natl Acad Sci U S A 2005; 102: 1490-1495. Stevens T, Parsi MA. Update on endoscopic pancreatic function testing. World J Gastroenterol 2011; 17: 3957-3961. Philippe MF, Benabadji S, Barbot-Trystram L, Vadrot D, Boitard C, Larger E. Pancreatic volume and endocrine and exocrine functions in patients with diabetes. Pancreas 2011; 40: 359-363. Lankisch PG, Lembcke B, Wemken G, Creutzfeldt W. Functional reserve capacity of the exocrine pancreas. Digestion 1986; 35: 175-181. Bellanne-Chantelot C, Chauveau D, Gautier JF, Dubois-Laforgue D, Clauin S, Beaufils S et al. Clinical spectrum associated with hepatocyte nuclear factor-1beta mutations. Ann Intern Med 2004; 140: 510-517. Hahn JU, Kerner W, Maisonneuve P, Lowenfels AB, Lankisch PG. Low fecal elastase 1 levels do not indicate exocrine pancreatic insufficiency in type-1 diabetes mellitus. Pancreas 2008; 36: 274-278. Haldorsen IS, Raeder H, Vesterhus M, Molven A, Njolstad PR. The role of pancreatic imaging in monogenic diabetes mellitus. Nat Rev Endocrinol 2012; 8: 148-159. Stevens T, Conwell DL, Zuccaro G Jr, Lewis SA, Love TE. The efficiency of endoscopic pancreatic function testing is optimized using duodenal aspirates at 30 and 45 minutes after intravenous secretin. Am J Gastroenterol 2007; 102: 297-301. Jensen NM, Larsen S. A rapid, endoscopic exocrine pancreatic function test and the Lundh test: a comparative study. Pancreatology 2008; 8: 617-624. Welters HJ, Senkel S, Klein-Hitpass L, Erdmann S, Thomas H, Harries LW et al. Conditional expression of hepatocyte nuclear factor-1beta, the maturity-onset diabetes of the young-5 gene product, influences the viability and functional competence of pancreatic beta-cells. J Endocrinol 2006; 190: 171-181. Kenkel J. Analytical chemistry for technicians. 3rd edn. Boca Raton, FL: CRC Press LLC, 2003. Pearson ER, Badman MK, Lockwood CR, Clark PM, Ellard S, Bingham C et al. Contrasting diabetes phenotypes associated with hepatocyte nuclear factor-1alpha and -1beta mutations. Diabetes Care 2004; 27: 1102-1107. Gonc EN, Ozturk BB, Haldorsen IS, Molnes J, Immervoll H, Raeder H et al. HNF1B mutation in a Turkish child with renal and exocrine pancreas insufficiency, diabetes and liver disease. Pediatr Diabetes 2012; 13: e1-5. Punwani S, Gillams AR, Lees WR. Non-invasive quantification of pancreatic exocrine function using secretin-stimulated MRCP. Eur Radiol 2003; 13: 273-276. Erturk SM, Ichikawa T, Motosugi U, Sou H, Araki T. Diffusion-weighted MR imaging in the evaluation of pancreatic exocrine function before and after secretin stimulation. Am J Gastroenterol 2006; 101: 133-136. Higuchi A, Yasugi H, Yokota T, Tobe T, Mizumoto R. Changes of pancreatic exocrine function after major resection of the pancreas in dogs. Gastroenterol Jpn 1979; 14: 316-326. Brackenridge A, Pearson ER, Shojaee-Moradie F, Hattersley AT, Russell-Jones D, Umpleby AM. Contrasting insulin sensitivity of endogenous glucose production rate in subjects with hepatocyte nuclear factor-1beta and -1alpha mutations. Diabetes 2006; 55: 405-411. Edghill EL, Oram RA, Owens M, Stals KL, Harries LW, Hattersley AT et al. Hepatocyte nuclear factor-1beta gene deletions-a common cause of renal disease. Nephrol Dial Transplant 2008; 23: 627-635. Duggan SN, O'Sullivan M, Hamilton S, Feehan SM, Ridgway PF, Conlon KC. Patients with chronic pancreatitis are at increased risk for osteoporosis. Pancreas 2012; 41: 1119-1124. Sanyal R, Stevens T, Novak E, Veniero JC. Secretin-enhanced MRCP: review of technique and application with proposal for quantification of exocrine function. AJR Am J Roentgenol 2012; 198: 124-132. Lieb JG2nd, Draganov PV. Pancreatic function testing: here to stay for the 21st century. World J Gastroenterol 2008; 14: 3149-3158. Edghill EL, Bingham C, Slingerland AS, Minton JA, Noordam C, Ellard S et al. Hepatocyte nuclear factor-1 beta mutations cause neonatal diabetes and intrauterine growth retardation: support for a critical role of HNF-1beta in human pancreatic development. Diabet Med 2006; 23: 1301-1306. Pandol S. The exocrine pancreas. San Rafael, CA: Morgan & Claypool Life Sciences 2010. Holick MF. Vitamin D: importance in the prevention of cancers, type 1 diabetes, heart disease, and osteoporosis. Am J Clin Nutr 2004; 79: 362-371. 1973; 288 2007; 102 1979; 14 2004; 27 2010 2006; 55 2004; 140 1986; 35 2010; 123 2011; 40 2006; 15 2008; 14 2008; 36 2003; 13 2008 2008; 8 2003 2011; 17 2012; 13 2007; 56 2012; 198 2006; 23 2006; 190 2005; 102 2004; 79 2008; 25 2008; 23 2006; 101 2012; 8 2012; 41 Higuchi A (e_1_2_6_26_1) 1979; 14 e_1_2_6_10_1 e_1_2_6_31_1 e_1_2_6_30_1 e_1_2_6_13_1 Chen YZ (e_1_2_6_3_1) 2010; 123 e_1_2_6_14_1 e_1_2_6_11_1 e_1_2_6_12_1 e_1_2_6_17_1 e_1_2_6_18_1 Punwani S (e_1_2_6_16_1) 2003; 13 e_1_2_6_21_1 e_1_2_6_20_1 Pandol S (e_1_2_6_19_1) 2010 e_1_2_6_9_1 e_1_2_6_8_1 e_1_2_6_5_1 e_1_2_6_4_1 e_1_2_6_7_1 e_1_2_6_6_1 Søvik O (e_1_2_6_2_1) 2008 e_1_2_6_25_1 e_1_2_6_24_1 e_1_2_6_23_1 e_1_2_6_22_1 Kenkel J (e_1_2_6_15_1) 2003 e_1_2_6_29_1 e_1_2_6_28_1 e_1_2_6_27_1 |
| References_xml | – reference: Chen YZ, Gao Q, Zhao XZ, Bennett CL, Xiong XS, Mei CL et al. Systematic review of TCF2 anomalies in renal cysts and diabetes syndrome/maturity onset diabetes of the young type 5. Chin Med J (Engl) 2010; 123: 3326-3333. – reference: Haldorsen IS, Vesterhus M, Raeder H, Jensen DK, Sovik O, Molven A et al. Lack of pancreatic body and tail in HNF1B mutation carriers. Diabet Med 2008; 25: 782-787. – reference: Brackenridge A, Pearson ER, Shojaee-Moradie F, Hattersley AT, Russell-Jones D, Umpleby AM. Contrasting insulin sensitivity of endogenous glucose production rate in subjects with hepatocyte nuclear factor-1beta and -1alpha mutations. Diabetes 2006; 55: 405-411. – reference: Sanyal R, Stevens T, Novak E, Veniero JC. Secretin-enhanced MRCP: review of technique and application with proposal for quantification of exocrine function. AJR Am J Roentgenol 2012; 198: 124-132. – reference: Gonc EN, Ozturk BB, Haldorsen IS, Molnes J, Immervoll H, Raeder H et al. HNF1B mutation in a Turkish child with renal and exocrine pancreas insufficiency, diabetes and liver disease. Pediatr Diabetes 2012; 13: e1-5. – reference: Edghill EL, Bingham C, Slingerland AS, Minton JA, Noordam C, Ellard S et al. Hepatocyte nuclear factor-1 beta mutations cause neonatal diabetes and intrauterine growth retardation: support for a critical role of HNF-1beta in human pancreatic development. Diabet Med 2006; 23: 1301-1306. – reference: Lankisch PG, Lembcke B, Wemken G, Creutzfeldt W. Functional reserve capacity of the exocrine pancreas. Digestion 1986; 35: 175-181. – reference: Raeder H, Haldorsen IS, Ersland L, Gruner R, Taxt T, Sovik O et al. Pancreatic lipomatosis is a structural marker in nondiabetic children with mutations in carboxyl-ester lipase. Diabetes 2007; 56: 444-449. – reference: Punwani S, Gillams AR, Lees WR. Non-invasive quantification of pancreatic exocrine function using secretin-stimulated MRCP. Eur Radiol 2003; 13: 273-276. – reference: Pearson ER, Badman MK, Lockwood CR, Clark PM, Ellard S, Bingham C et al. Contrasting diabetes phenotypes associated with hepatocyte nuclear factor-1alpha and -1beta mutations. Diabetes Care 2004; 27: 1102-1107. – reference: Bellanne-Chantelot C, Chauveau D, Gautier JF, Dubois-Laforgue D, Clauin S, Beaufils S et al. Clinical spectrum associated with hepatocyte nuclear factor-1beta mutations. Ann Intern Med 2004; 140: 510-517. – reference: Haumaitre C, Barbacci E, Jenny M, Ott MO, Gradwohl G, Cereghini S. Lack of TCF2/vHNF1 in mice leads to pancreas agenesis. Proc Natl Acad Sci U S A 2005; 102: 1490-1495. – reference: Holick MF. Vitamin D: importance in the prevention of cancers, type 1 diabetes, heart disease, and osteoporosis. Am J Clin Nutr 2004; 79: 362-371. – reference: Jensen NM, Larsen S. A rapid, endoscopic exocrine pancreatic function test and the Lundh test: a comparative study. Pancreatology 2008; 8: 617-624. – reference: Lieb JG2nd, Draganov PV. Pancreatic function testing: here to stay for the 21st century. World J Gastroenterol 2008; 14: 3149-3158. – reference: Haldorsen IS, Raeder H, Vesterhus M, Molven A, Njolstad PR. The role of pancreatic imaging in monogenic diabetes mellitus. Nat Rev Endocrinol 2012; 8: 148-159. – reference: DiMagno EP, Go VL, Summerskill WH. Relations between pancreatic enzyme ouputs and malabsorption in severe pancreatic insufficiency. N Engl J Med 1973; 288: 813-815. – reference: Duggan SN, O'Sullivan M, Hamilton S, Feehan SM, Ridgway PF, Conlon KC. Patients with chronic pancreatitis are at increased risk for osteoporosis. Pancreas 2012; 41: 1119-1124. – reference: Philippe MF, Benabadji S, Barbot-Trystram L, Vadrot D, Boitard C, Larger E. Pancreatic volume and endocrine and exocrine functions in patients with diabetes. Pancreas 2011; 40: 359-363. – reference: Erturk SM, Ichikawa T, Motosugi U, Sou H, Araki T. Diffusion-weighted MR imaging in the evaluation of pancreatic exocrine function before and after secretin stimulation. Am J Gastroenterol 2006; 101: 133-136. – reference: Higuchi A, Yasugi H, Yokota T, Tobe T, Mizumoto R. Changes of pancreatic exocrine function after major resection of the pancreas in dogs. Gastroenterol Jpn 1979; 14: 316-326. – reference: Stevens T, Parsi MA. Update on endoscopic pancreatic function testing. World J Gastroenterol 2011; 17: 3957-3961. – reference: Welters HJ, Senkel S, Klein-Hitpass L, Erdmann S, Thomas H, Harries LW et al. Conditional expression of hepatocyte nuclear factor-1beta, the maturity-onset diabetes of the young-5 gene product, influences the viability and functional competence of pancreatic beta-cells. J Endocrinol 2006; 190: 171-181. – reference: Stevens T, Conwell DL, Zuccaro G Jr, Lewis SA, Love TE. The efficiency of endoscopic pancreatic function testing is optimized using duodenal aspirates at 30 and 45 minutes after intravenous secretin. Am J Gastroenterol 2007; 102: 297-301. – reference: Pandol S. The exocrine pancreas. San Rafael, CA: Morgan & Claypool Life Sciences 2010. – reference: Hahn JU, Kerner W, Maisonneuve P, Lowenfels AB, Lankisch PG. Low fecal elastase 1 levels do not indicate exocrine pancreatic insufficiency in type-1 diabetes mellitus. Pancreas 2008; 36: 274-278. – reference: Kenkel J. Analytical chemistry for technicians. 3rd edn. Boca Raton, FL: CRC Press LLC, 2003. – reference: Edghill EL, Oram RA, Owens M, Stals KL, Harries LW, Hattersley AT et al. Hepatocyte nuclear factor-1beta gene deletions-a common cause of renal disease. Nephrol Dial Transplant 2008; 23: 627-635. – reference: Haumaitre C, Fabre M, Cormier S, Baumann C, Delezoide AL, Cereghini S. Severe pancreas hypoplasia and multicystic renal dysplasia in two human fetuses carrying novel HNF1beta/MODY5 mutations. Hum Mol Genet 2006; 15: 2363-2375. – volume: 13 start-page: e1 year: 2012 end-page: 5 article-title: HNF1B mutation in a Turkish child with renal and exocrine pancreas insufficiency, diabetes and liver disease publication-title: Pediatr Diabetes – volume: 40 start-page: 359 year: 2011 end-page: 363 article-title: Pancreatic volume and endocrine and exocrine functions in patients with diabetes publication-title: Pancreas – volume: 35 start-page: 175 year: 1986 end-page: 181 article-title: Functional reserve capacity of the exocrine pancreas publication-title: Digestion – volume: 79 start-page: 362 year: 2004 end-page: 371 article-title: Vitamin D: importance in the prevention of cancers, type 1 diabetes, heart disease, and osteoporosis publication-title: Am J Clin Nutr – volume: 198 start-page: 124 year: 2012 end-page: 132 article-title: Secretin‐enhanced MRCP: review of technique and application with proposal for quantification of exocrine function publication-title: AJR Am J Roentgenol – volume: 123 start-page: 3326 year: 2010 end-page: 3333 article-title: Systematic review of TCF2 anomalies in renal cysts and diabetes syndrome/maturity onset diabetes of the young type 5 publication-title: Chin Med J (Engl) – year: 2003 – volume: 8 start-page: 148 year: 2012 end-page: 159 article-title: The role of pancreatic imaging in monogenic diabetes mellitus publication-title: Nat Rev Endocrinol – volume: 23 start-page: 1301 year: 2006 end-page: 1306 article-title: Hepatocyte nuclear factor‐1 beta mutations cause neonatal diabetes and intrauterine growth retardation: support for a critical role of HNF‐1beta in human pancreatic development publication-title: Diabet Med – volume: 102 start-page: 1490 year: 2005 end-page: 1495 article-title: Lack of TCF2/vHNF1 in mice leads to pancreas agenesis publication-title: Proc Natl Acad Sci U S A – start-page: 1096 year: 2008 end-page: 1102 – volume: 15 start-page: 2363 year: 2006 end-page: 2375 article-title: Severe pancreas hypoplasia and multicystic renal dysplasia in two human fetuses carrying novel HNF1beta/MODY5 mutations publication-title: Hum Mol Genet – volume: 25 start-page: 782 year: 2008 end-page: 787 article-title: Lack of pancreatic body and tail in HNF1B mutation carriers publication-title: Diabet Med – volume: 8 start-page: 617 year: 2008 end-page: 624 article-title: A rapid, endoscopic exocrine pancreatic function test and the Lundh test: a comparative study publication-title: Pancreatology – volume: 102 start-page: 297 year: 2007 end-page: 301 article-title: The efficiency of endoscopic pancreatic function testing is optimized using duodenal aspirates at 30 and 45 minutes after intravenous secretin publication-title: Am J Gastroenterol – year: 2010 – volume: 13 start-page: 273 year: 2003 end-page: 276 article-title: Non‐invasive quantification of pancreatic exocrine function using secretin‐stimulated MRCP publication-title: Eur Radiol – volume: 190 start-page: 171 year: 2006 end-page: 181 article-title: Conditional expression of hepatocyte nuclear factor‐1beta, the maturity‐onset diabetes of the young‐5 gene product, influences the viability and functional competence of pancreatic beta‐cells publication-title: J Endocrinol – volume: 140 start-page: 510 year: 2004 end-page: 517 article-title: Clinical spectrum associated with hepatocyte nuclear factor‐1beta mutations publication-title: Ann Intern Med – volume: 288 start-page: 813 year: 1973 end-page: 815 article-title: Relations between pancreatic enzyme ouputs and malabsorption in severe pancreatic insufficiency publication-title: N Engl J Med – volume: 23 start-page: 627 year: 2008 end-page: 635 article-title: Hepatocyte nuclear factor‐1beta gene deletions–a common cause of renal disease publication-title: Nephrol Dial Transplant – volume: 14 start-page: 316 year: 1979 end-page: 326 article-title: Changes of pancreatic exocrine function after major resection of the pancreas in dogs publication-title: Gastroenterol Jpn – volume: 55 start-page: 405 year: 2006 end-page: 411 article-title: Contrasting insulin sensitivity of endogenous glucose production rate in subjects with hepatocyte nuclear factor‐1beta and ‐1alpha mutations publication-title: Diabetes – volume: 14 start-page: 3149 year: 2008 end-page: 3158 article-title: Pancreatic function testing: here to stay for the 21st century publication-title: World J Gastroenterol – volume: 56 start-page: 444 year: 2007 end-page: 449 article-title: Pancreatic lipomatosis is a structural marker in nondiabetic children with mutations in carboxyl‐ester lipase publication-title: Diabetes – volume: 17 start-page: 3957 year: 2011 end-page: 3961 article-title: Update on endoscopic pancreatic function testing publication-title: World J Gastroenterol – volume: 101 start-page: 133 year: 2006 end-page: 136 article-title: Diffusion‐weighted MR imaging in the evaluation of pancreatic exocrine function before and after secretin stimulation publication-title: Am J Gastroenterol – volume: 41 start-page: 1119 year: 2012 end-page: 1124 article-title: Patients with chronic pancreatitis are at increased risk for osteoporosis publication-title: Pancreas – volume: 27 start-page: 1102 year: 2004 end-page: 1107 article-title: Contrasting diabetes phenotypes associated with hepatocyte nuclear factor‐1alpha and ‐1beta mutations publication-title: Diabetes Care – volume: 36 start-page: 274 year: 2008 end-page: 278 article-title: Low fecal elastase 1 levels do not indicate exocrine pancreatic insufficiency in type‐1 diabetes mellitus publication-title: Pancreas – ident: e_1_2_6_14_1 doi: 10.1097/MPA.0b013e3181656f8 – ident: e_1_2_6_4_1 doi: 10.1073/pnas.0405776102 – ident: e_1_2_6_9_1 doi: 10.1677/joe.1.06768 – start-page: 1096 volume-title: Inborn Errors of Development year: 2008 ident: e_1_2_6_2_1 – ident: e_1_2_6_6_1 doi: 10.1111/j.1464-5491.2008.02460.x – ident: e_1_2_6_12_1 doi: 10.1111/j.1464-5491.2006.01999.x – ident: e_1_2_6_10_1 doi: 10.7326/0003-4819-140-7-200404060-00009 – volume: 123 start-page: 3326 year: 2010 ident: e_1_2_6_3_1 article-title: Systematic review of TCF2 anomalies in renal cysts and diabetes syndrome/maturity onset diabetes of the young type 5 publication-title: Chin Med J (Engl) – volume-title: The exocrine pancreas year: 2010 ident: e_1_2_6_19_1 – ident: e_1_2_6_28_1 doi: 10.1111/j.1445-5994.1995.tb01526.x – ident: e_1_2_6_29_1 doi: 10.1097/MPA.0b013e31824abb4d – ident: e_1_2_6_17_1 doi: 10.2337/db06-0859 – ident: e_1_2_6_13_1 doi: 10.3748/wjg.14.3149 – ident: e_1_2_6_20_1 doi: 10.2214/AJR.10.5713 – ident: e_1_2_6_22_1 doi: 10.1159/000161013 – ident: e_1_2_6_30_1 doi: 10.1056/NEJM197304192881603 – ident: e_1_2_6_5_1 doi: 10.1093/hmg/ddl161 – volume-title: Analytical chemistry for technicians. 3rd edn year: 2003 ident: e_1_2_6_15_1 – ident: e_1_2_6_27_1 doi: 10.1111/j.1572-0241.2006.00406.x – volume: 13 start-page: 273 year: 2003 ident: e_1_2_6_16_1 article-title: Non‐invasive quantification of pancreatic exocrine function using secretin‐stimulated MRCP publication-title: Eur Radiol doi: 10.1007/s00330-002-1605-x – ident: e_1_2_6_25_1 doi: 10.1097/MPA.0b013e3182072032 – ident: e_1_2_6_31_1 doi: 10.1159/000199364 – ident: e_1_2_6_7_1 doi: 10.2337/diacare.27.5.1102 – ident: e_1_2_6_11_1 doi: 10.1111/j.1399-5448.2011.00773.x – ident: e_1_2_6_24_1 doi: 10.1038/nrendo.2011.197 – ident: e_1_2_6_23_1 doi: 10.3748/wjg.v17.i35.3957 – ident: e_1_2_6_8_1 doi: 10.2337/diabetes.55.02.06.db05-1019 – ident: e_1_2_6_21_1 doi: 10.1111/j.1572-0241.2006.00949.x – ident: e_1_2_6_18_1 doi: 10.1093/ndt/gfm603 – volume: 14 start-page: 316 year: 1979 ident: e_1_2_6_26_1 article-title: Changes of pancreatic exocrine function after major resection of the pancreas in dogs publication-title: Gastroenterol Jpn doi: 10.1007/BF02774228 |
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| Snippet | Objectives
To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing.
Methods... The exocrine pancreatic function in HNF1B‐MODY is moderately reduced. Both ductal and acinar pancreatic function are affected in HNF1B‐MODY. Ductal function in... To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing. Patients with HNF1B... To examine the exocrine pancreatic function in carriers of the hepatocyte nuclear factor 1β gene (HNF1B) mutation by direct testing.OBJECTIVESTo examine the... |
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| SubjectTerms | Acinar Cells - pathology Acinar Cells - secretion Adolescent Adult Aged Biological and medical sciences Central Nervous System Diseases - genetics Central Nervous System Diseases - pathology Central Nervous System Diseases - physiopathology Child Dental Enamel - abnormalities Dental Enamel - pathology Dental Enamel - physiopathology Diabetes Mellitus, Type 2 - genetics Diabetes Mellitus, Type 2 - pathology Diabetes Mellitus, Type 2 - physiopathology Diabetes. Impaired glucose tolerance Endocrine pancreas. Apud cells (diseases) Endocrinopathies Etiopathogenesis. Screening. Investigations. Target tissue resistance Exocrine Pancreatic Insufficiency - etiology Feeding. Feeding behavior Female Fundamental and applied biological sciences. Psychology Hepatocyte Nuclear Factor 1-beta - genetics Humans Kidney Diseases, Cystic - genetics Kidney Diseases, Cystic - pathology Kidney Diseases, Cystic - physiopathology Male Medical sciences Middle Aged Mutation Organ Size Pancreas, Exocrine - pathology Pancreas, Exocrine - physiopathology Pancreas, Exocrine - secretion Pancreatic Ducts - pathology Pancreatic Ducts - physiopathology Pancreatic Ducts - secretion Pancreatic Juice - chemistry Pancreatic Juice - secretion Pedigree Secretin Up-Regulation Vertebrates: anatomy and physiology, studies on body, several organs or systems Vertebrates: endocrinology |
| Title | Exocrine pancreatic function in hepatocyte nuclear factor 1β-maturity-onset diabetes of the young (HNF1B-MODY) is only moderately reduced: compensatory hypersecretion from a hypoplastic pancreas |
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