this is very old….. more and more archives are making it to the net as various companies and journals get them added…..interesting, nonetheless.
Steatorrhea and pancreatic insufficiency in Shwachman syndrome.
Gastroenterology. 1982; 83(1 Pt 1):22-7 (ISSN: 0016-5085)
Hill RE; Durie PR; Gaskin KJ; Davidson GP; Forstner GG
Fat absorption was assessed on two or more occasions in 12 of 14 patients with Shwachman syndrome. Of 11 children who initially had steatorrhea, 6 were subsequently found to have normal fat excretion. In 10 of these 11 patients the last estimate was smaller than the initial estimate after an interval of 0.3-12.7 yr. The most dramatic improvement occurred under 4 yr of age but improvement appeared to continue over a more extended period where sufficient follow-up was available. All patients had pronounced pancreatic insufficiency as tested by duodenal drainage corrected by nonabsorbable marker under stimulation with cholecystokinin and secretin. Pancreatic lipase secretion was assessed in 3 patients with steatorrhea and 5 without steatorrhea (73% of the surviving patients) using a sensitive assay which used maximum stimulation with colipase. Pancreatic lipase secretion was less than 2% of normal mean secretion in the steatorrheic patients and ranged from 3.7% to 13.6% in the patients without steatorrhea. These results indicate that fat absorption improves in the majority of patients with Shwachman syndrome and appears to be associated with marginal improvement in pancreatic lipase secretion. Due to the occult nature of bowel complaints in older patients, the diagnosis may be overlooked unless careful screening for pancreatic function takes place.
Showing posts with label Pancreas. Show all posts
Showing posts with label Pancreas. Show all posts
Saturday, May 10, 2008
Pancreatic Exocrine Function Tests
Pancreatic Exocrine Function Tests
There is a brief overview of the topic....with a subscription, you can read the entire thing. Excellent info on the testing.
There is a brief overview of the topic....with a subscription, you can read the entire thing. Excellent info on the testing.
Sunday, March 9, 2008
Is pancreatic diabetes (type 3c diabetes) underdiagnosed and misdiagnosed?
I have the full-text PDF of this article. VERY interesting!
Is pancreatic diabetes (type 3c diabetes) underdiagnosed and misdiagnosed?
Exocrine pancreatic insufficiency is frequently associated with diabetes, with high prevalence in both insulin-dependent or insulin-independent patients. Exocrine pancreatic failure has often been perceived as a complication of diabetes. In contrast, recent clinical observations lead to the notion that nonendocrine pancreatic disease is a critical factor for development rather than a sequel to diabetes. The incidence of diabetes caused by exocrine pancreatic disease appears to be underestimated and may comprise 8% or more of the general diabetic patient population. Nonendocrine pancreas disease can cause diabetes by multiple mechanisms. Genetic defects have been characterized, resulting in a syndrome of both exocrine and endocrine failure. Regulation of beta-cell mass and physiological incretin secretion are directly dependent on normal exocrine function. Algorithms for diagnosis and therapy of diabetes should therefore address both endocrine and exocrine pancreatic function.
Is pancreatic diabetes (type 3c diabetes) underdiagnosed and misdiagnosed?
Exocrine pancreatic insufficiency is frequently associated with diabetes, with high prevalence in both insulin-dependent or insulin-independent patients. Exocrine pancreatic failure has often been perceived as a complication of diabetes. In contrast, recent clinical observations lead to the notion that nonendocrine pancreatic disease is a critical factor for development rather than a sequel to diabetes. The incidence of diabetes caused by exocrine pancreatic disease appears to be underestimated and may comprise 8% or more of the general diabetic patient population. Nonendocrine pancreas disease can cause diabetes by multiple mechanisms. Genetic defects have been characterized, resulting in a syndrome of both exocrine and endocrine failure. Regulation of beta-cell mass and physiological incretin secretion are directly dependent on normal exocrine function. Algorithms for diagnosis and therapy of diabetes should therefore address both endocrine and exocrine pancreatic function.
Saturday, February 23, 2008
Article: MRI Findings of the Pancreas in patients with SDS and mutations in the SBDS gene
Magnetic resonance imaging findings of the pancreas in patients with Shwachman-Diamond syndrome and mutations in the SBDS gene.
Medical Imaging Center, Helsinki University Hospital, Helsinki, Finland.
Pancreatic MRI was evaluated in 14 patients with a clinical diagnosis of Shwachman-Diamond syndrome, and the findings were correlated with Shwachman-Bodian-Diamond gene (SBDS) genotype. The findings suggest that patients with mutations in the SBDS gene have a characteristic magnetic resonance imaging pattern of fat-replaced pancreas and that SBDS mutations are unlikely in patients without this pattern.
This article contains charts on how each of the 14 patients pancreatic function was assessed. It lists all 14 patients and how each one’s pancreatic function was assessed and then lists the criteria for their SDS diagnosis. It also lists their neutrophil count (range) and height Z-score. In another chart, it lists how long they were on enzymes--- i.e. lists the age at which the person became pancreatic sufficient. One child was 4 years old when she came off of enzymes (i.e. she was on enzymes until age 4) and another one was 5 years old. Two came off of enzymes at 11 years and one at 22 years, one at 29 years, 3 never took enzymes and the rest are on on-going enzyme replacement.
There were 14 patients. 10 males and 4 females. 13 of them were diagnosed at a median age of 18 months….but one was referred to them at age 13 for suspected SDS. One of the patients was diagnosed with type I diabetes at age 15. With all of our diabetes talk recently, I thought that was an interesting note. The article says hat all patients with SBDS mutations showed fatty pancreas on MRI—the patients without the SBDS mutations and proven PI had NORMAL pancreatic MRIs.
Medical Imaging Center, Helsinki University Hospital, Helsinki, Finland.
Pancreatic MRI was evaluated in 14 patients with a clinical diagnosis of Shwachman-Diamond syndrome, and the findings were correlated with Shwachman-Bodian-Diamond gene (SBDS) genotype. The findings suggest that patients with mutations in the SBDS gene have a characteristic magnetic resonance imaging pattern of fat-replaced pancreas and that SBDS mutations are unlikely in patients without this pattern.
This article contains charts on how each of the 14 patients pancreatic function was assessed. It lists all 14 patients and how each one’s pancreatic function was assessed and then lists the criteria for their SDS diagnosis. It also lists their neutrophil count (range) and height Z-score. In another chart, it lists how long they were on enzymes--- i.e. lists the age at which the person became pancreatic sufficient. One child was 4 years old when she came off of enzymes (i.e. she was on enzymes until age 4) and another one was 5 years old. Two came off of enzymes at 11 years and one at 22 years, one at 29 years, 3 never took enzymes and the rest are on on-going enzyme replacement.
There were 14 patients. 10 males and 4 females. 13 of them were diagnosed at a median age of 18 months….but one was referred to them at age 13 for suspected SDS. One of the patients was diagnosed with type I diabetes at age 15. With all of our diabetes talk recently, I thought that was an interesting note. The article says hat all patients with SBDS mutations showed fatty pancreas on MRI—the patients without the SBDS mutations and proven PI had NORMAL pancreatic MRIs.
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