Functional Study of Exocrine Pancreas in Idiopathic Hemochromatosis, Untreated and Treated by Venesections

Simon, Michelle; Gosselin, Michel; Kerbaol, M; Delanoé, G; Trébaul, L; Bourel, M

doi:10.1159/000197347

Cited by 8 publications

(3 citation statements)

References 13 publications

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“…When both these groups were treated with secretin and cholecystokinin, a significantly low concentration of pancreatic enzymes in the duodenal aspirate was found in untreated hereditary hemochromatosis compared to those who were treated with venesections, implying a direct association of iron overload and PED. This supports the finding in our case [11].…”

Section: Discussionsupporting

confidence: 94%

“…Our patient was a 45-year-old African American female with a past medical history of pre-diabetes mellitus, hypertension, alcoholic hepatitis, cholecystectomy, and anemia requiring multiple (10)(11)(12)(13)(14)(15) blood transfusions over the span of 3-4 years. She presented with daily non-bloody, greasy diarrhea, including nocturnal episodes.…”

Section: Case Presentationmentioning

confidence: 99%

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Iron-Storage Disorder Presenting as Chronic Diarrhea

Vobugari¹,

Kim²,

Gandhi³

et al. 2021

Cureus

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The involvement of the endocrine pancreas leading to bronze diabetes is well studied. However, little is known about the pathophysiology of iron dysregulation involving the exocrine pancreas. We present a unique association between the exocrine pancreas and iron dysregulation. A 45-year-old female presented with chronic diarrhea and low fecal elastase indicative of pancreatic exocrine dysfunction. MRI of the abdomen/pelvis showed iron deposition in the pancreas, suggesting an associated iron-storage disorder without features suggesting chronic pancreatitis. Association of an iron-storage disorder with pancreatic exocrine dysfunction has been reported only in one other case report. Pancreatic exocrine dysfunction can be directly associated with an iron-storage disorder that involves the pancreas. This should be included in the differential and diagnostic work-up of chronic diarrhea of unclear etiology. Based on the literature, we have highlighted the potential pathophysiology relevant to the case.

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Section: Discussionsupporting

confidence: 94%

Section: Case Presentationmentioning

confidence: 99%

Iron-Storage Disorder Presenting as Chronic Diarrhea

Vobugari¹,

Kim²,

Gandhi³

et al. 2021

Cureus

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“…However, lesser degrees of exocrine pancreatic dysfunction may be found such as increased volume excretion (Althausen et al, 195 1;Simon et al, 1973) and less often decreased enzyme secretion (Goebell et al, 1971). The finding of overt exocrine pancreatic insufficiency in a young precirrhotic haemochromatotic patient is exceptional.…”

Section: Discussionmentioning

confidence: 99%

Exocrine pancreatic insufficiency and idiopathic haemochromatosis

Jansen

Thien

Lamers

et al. 1984

Postgraduate Medical Journal

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SummaryThe case history of a 34-year-old patient with precirrhotic idiopathic haemochromatosis and severe chronic steatorrhoea is presented. The pancreas had a normal appearance on ultrasonography and endoscopic retrograde pancreaticography. However, pancreatic function tests revealed significant abnormalities. The pancreatic output of trypsin, amylase, lipase and bicarbonate was deficient and basal and stimulated serum pancreatic polypeptide levels were subnormal. In contrast, the oral glucose tolerance test was unimpaired. The pancreatic insufficiency had started suddenly during a summer vacation and may have had a viral aetiology. The hypothesis is advanced that in haemochromatosis the iron-laden pancreatic acinar and PP-producing cells are more susceptible to damage by viruses than normal pancreatic cells.

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European guidelines for the diagnosis and treatment of pancreatic exocrine insufficiency: UEG, EPC, EDS, ESPEN, ESPGHAN, ESDO, and ESPCG evidence‐based recommendations

Dominguez‐Muñoz,

Vujasinovic,

de la Iglesia

et al. 2024

UEG Journal

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Pancreatic exocrine insufficiency (PEI) is defined as a reduction in pancreatic exocrine secretion below the level that allows the normal digestion of nutrients. Pancreatic disease and surgery are the main causes of PEI. However, other conditions and upper gastrointestinal surgery can also affect the digestive function of the pancreas. PEI can cause symptoms of nutritional malabsorption and deficiencies, which affect the quality of life and increase morbidity and mortality. These guidelines were developed following the United European Gastroenterology framework for the development of high‐quality clinical guidelines. After a systematic literature review, the evidence was evaluated according to the Oxford Center for Evidence‐Based Medicine and the Grading of Recommendations Assessment, Development, and Evaluation methodology, as appropriate. Statements and comments were developed by the working groups and voted on using the Delphi method. The diagnosis of PEI should be based on a global assessment of symptoms, nutritional status, and a pancreatic secretion test. Pancreatic enzyme replacement therapy (PERT), together with dietary advice and support, are the cornerstones of PEI therapy. PERT is indicated in patients with PEI that is secondary to pancreatic disease, pancreatic surgery, or other metabolic or gastroenterological conditions. Specific recommendations concerning the management of PEI under various clinical conditions are provided based on evidence and expert opinions. This evidence‐based guideline summarizes the prevalence, clinical impact, and general diagnostic and therapeutic approaches for PEI, as well as the specifics of PEI in different clinical conditions. Finally, the unmet needs for future research are discussed.

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Functional Study of Exocrine Pancreas in Idiopathic Hemochromatosis, Untreated and Treated by Venesections

Cited by 8 publications

References 13 publications

Iron-Storage Disorder Presenting as Chronic Diarrhea

Iron-Storage Disorder Presenting as Chronic Diarrhea

Exocrine pancreatic insufficiency and idiopathic haemochromatosis

European guidelines for the diagnosis and treatment of pancreatic exocrine insufficiency: UEG, EPC, EDS, ESPEN, ESPGHAN, ESDO, and ESPCG evidence‐based recommendations

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