Exocrine pancreatic insufficiency and idiopathic haemochromatosis

Jansen, Plm; Thien, Th.; Lamers, C. B. H. W.; Yap, Sing Hiem; Reekers, P.; Strijk, S. P.

doi:10.1136/pgmj.60.708.675

Cited by 7 publications

(7 citation statements)

References 12 publications

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“…With a prolonged iron overload state, organ damage can occur in the pancreas, skin, anterior pituitary gland and heart . The clinical findings of tissue damage can include cirrhosis, pancreatic endocrine and exocrine insufficiency, polyarthritis, skin hyperpigmentation, hypogonadism, adrenal insufficiency, hypothyroidism and heart failure . Haemochromatosis is associated with an increased risk of hepatocellular carcinoma and other sequelae of cirrhosis .…”

Section: Clinical Manifestations Of Haemochromatosismentioning

confidence: 99%

“…Iron accumulation may also cause considerable damage to the pancreas, leading to the development of diabetes mellitus and exocrine pancreatic insufficiency . The pathogenesis of diabetes in haemochromatosis is multifactorial but felt to be mediated largely by the predominance of transferrin receptors in pancreatic islets, allowing transferrin‐bound iron to enter pancreatic cells.…”

Section: Assessment Of Organ Damage From Haemochromatosismentioning

confidence: 99%

“…This is likely due to the close anatomic relationship between exocrine and endocrine cells in the pancreas. Exocrine insufficiency can lead to reduced output of amylase, lipase and trypsin, with subsequent malabsorption, steatorrhea and other gastrointestinal consequences . Though no standard screening for pancreatic exocrine insufficiency is recommended, the clinician must be aware of relevant signs and symptoms to initiate work up if appropriate.…”

Section: Assessment Of Organ Damage From Haemochromatosismentioning

confidence: 99%

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Diagnosis and management of hereditary haemochromatosis

et al. 2020

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Hereditary haemochromatosis, one of the most common genetic disorders in the United States, can produce systemic iron deposition leading to end-organ failure and death if untreated. The diagnosis of this condition can be challenging as elevated serum ferritin may be seen in a variety of conditions, including acute and chronic liver disease, a range of systemic inflammatory states, and both primary and secondary iron overload syndromes. Appropriate and timely diagnosis of haemochromatosis is paramount as simple interventions, such as phlebotomy, can prevent or reverse organ damage from iron overload. The recognition of other aetiologies of elevated ferritin is also vital to ensure that appropriate intervention is provided and phlebotomy only utilized in patients who require it. In this review, we summarize the existing data on the work up and management of hereditary haemochromatosis and present a practical algorithm for the diagnosis and management of this disease.

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Section: Clinical Manifestations Of Haemochromatosismentioning

confidence: 99%

Section: Assessment Of Organ Damage From Haemochromatosismentioning

confidence: 99%

Section: Assessment Of Organ Damage From Haemochromatosismentioning

confidence: 99%

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Diagnosis and management of hereditary haemochromatosis

et al. 2020

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“…To the best of our knowledge, there is only one other reported case of hemochromatosis causing acute PED following a viral infection by Jansen et al in the 1980s [8]. Our case is unique as we report a symptomatic PED (proven low stool pancreatic elastase) due to iron deposition in the parenchyma.…”

Section: Discussionmentioning

confidence: 60%

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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“…In later stages of GH, a and 13 cells may be diminished in number as the fibrosis increases (3). Decrements in pancreatic exocrine function have also been associated with Fe overload (10,14).…”

Section: Discussionmentioning

confidence: 99%

Genome-Linked Toxic Responses to Dietary Iron Overload

et al. 1997

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Genome-related differences to Fe overload between and within rodent species were evaluated in the present study. Male B6C3F1 mice, yellow and black C5YSF1 mice, and Fischer 344 (F344) rats were fed AIN-76A diets containing 35 (control), 1,500, 3,500, 5,000, or 10,000 micrograms carbonyl Fe/g for 12 wk. No effects on body weight gain were observed in the B6C3F1 and black C5YSF1 mice, whereas at all doses of Fe above the control, weight gain was reduced in yellow C5YSF1 mice and F344 rats. At the 10,000 micrograms Fe/g dose, 9 of 12 rats died, but there was no mortality among the mice. In all animals, there was a dose-related increase in liver nonheme Fe, and the Fe was stored in hepatocytes predominantly in the periportal region. There was significant hypertrophy of the hepatocytes in both B6C3F1 mice and F344 rats fed the 10,000 micrograms Fe/g diet. PCNA assays showed significant stimulatory effects of the high dose of Fe on hepatocyte proliferation in the F344 rats and the C5YSF1 mice but not in the B6C3F1 mice. In the rat, there was pancreatic atrophy with loss of both endocrine and exocrine tissue. Morphometric evaluation of pancreas showed fewer beta cells in B6C3F1 and yellow C5YSF1 mice but not in the black C5YSF1 mice. There were fewer islets in the yellow C5YSF1 mice, and total and mean islet areas were smaller than in the control mice. Rats in the 10,000 micrograms Fe/g dose group had markedly exacerbated dose-dependent nephropathy and changes in glomerular and tubular epithelium associated with Fe accumulation. The rats also showed degeneration of the germinal epithelium of the testis, formation of multinucleated giant cells, and lack of mature sperm.

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Exocrine pancreatic insufficiency and idiopathic haemochromatosis

Cited by 7 publications

References 12 publications

Diagnosis and management of hereditary haemochromatosis

Diagnosis and management of hereditary haemochromatosis

Iron-Storage Disorder Presenting as Chronic Diarrhea

Genome-Linked Toxic Responses to Dietary Iron Overload

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