The diagnosis of hereditary pancreatitis by pancreatography

Rohrmann, Charles A.; Surawicz, Christina M.; Hutchinson, Douglas T.; Silverstein, Fred E.; White, Thomas Taylor; Marchioro, Thomas L.

doi:10.1016/s0016-5107(81)73184-5

Cited by 19 publications

(5 citation statements)

References 15 publications

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“…Sarles [22] suggests, on the basis of the composition of pancreatic stones, that two forms of HP exist. This hypothesis is problematic because calcifications are unusual [8] and are a late finding in many patients with HP [23,24]. Although Giorgi et al [25] reported that levels of pancreatic stone protein messenger RNA were three times lower in patients with chronic calcifying pancreatitis than in controls and suggested that pancreatic stone protein gene expression is specifically reduced in patients with chronic calcific pancreatitis, this finding has been shown to be nonspecific [26].…”

Section: Proposed Markers For Hereditary Pancreatitismentioning

confidence: 99%

New insights into hereditary pancreatitis

Whitcomb

1999

Curr Gastroenterol Rep

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The recent discovery that mutations in the trypsinogen gene are responsible for acute and chronic pancreatitis, and that patients with hereditary pancreatitis are at great risk for pancreatic cancer, has opened the door to understanding many aspects of pancreatic disease. This review focuses on the clinical presentation of hereditary pancreatitis, the mechanism of disease, and implications of this disease on understanding acute and chronic pancreatitis.

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Section: Proposed Markers For Hereditary Pancreatitismentioning

confidence: 99%

New insights into hereditary pancreatitis

Whitcomb

1999

Curr Gastroenterol Rep

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“…Computed tomography (CT) is the most commonly used modality in patients with a clinical suspicion or known diagnosis of pancreatic disease [14][15][16] in view of its high sensitivity in detecting duct stones, which in some cases of GMAP present with a noncalcified central core [17][18][19][20]. However, very few published reports have described CT features of GMAP [21] owing to the rarity of this condition [1][2][3].…”

Section: Introductionmentioning

confidence: 99%

“…La tomografia computerizzata (TC) è la tecnica più comunemente utilizzata nei pazienti con sospetto o diagnosi di patologia pancreatica [14][15][16], in particolare per la sua elevata sensibilità ad identificare i calcoli duttali, che in alcuni casi di GMAP presentano un core centrale non calcifico [17][18][19][20]. I lavori che in letteratura descrivono tuttavia le caratteristiche TC delle GMAP sono scarsi [21], per la rarità della patologia [1][2][3].…”

Section: Introductionunclassified

“…3a, b). Rohrmann et al were the first to report the presence of stones with a dense peripheral margin and radiolucent central core at plain abdominal radiography in three patients with chronic pancreatitis; these stones had a diameter >23 mm [17]. Hoshina et al described three generations of patients with chronic pancreatitis who at endoscopic retrograde cholangiopancreatography showed large stones distributed along the entire gland and with bull's-eye appearance due to the presence of a central radiolucent core [18].…”

mentioning

confidence: 99%

“…Rohrmann et al [17], nel 1981, hanno riportato, per la prima volta in 3 pazienti affetti da pancreatite cronica, la presenza sul radiogramma diretto dell'addome di calcoli con un margine periferico denso e centro radiotrasparente; questi calcoli avevano un diametro maggiore di 23 mm. Hoshina et al [18], nel 1999, hanno descritto 3 generazioni di pazienti, studiati con colangio-pancreatografia endoscopica retrograda, tutti con pancreatite cronica che presentavano calcoli di grandi dimensioni, distribuiti lungo tutta la ghiandola, con aspetto a bull's eye per la presenza di un centro radiotrasparente.…”

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Role of multislice computed tomography in the diagnosis of gene-mutation-associated pancreatitis (GMAP)

et al. 2010

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On qualitative image assessment, in group A patients, pancreatic duct stones were detected in 2/13 with GMAP. All stones were calcified and homogenous. Enhancement of the pancreatic parenchyma was hypovascular in 7/13 patients. In group B patients, pancreatic duct stones were detected in 12/12 with GMAP. Stones were calcified in 10/12 cases and noncalcified (protein plugs) in 2/12; in 5/10 cases, the calcified stones were heterogeneous with noncalcified central core (bull's-eye appearance). Enhancement of the pancreatic parenchyma was hypovascular in 12/12 patients. On quantitative image assessment, in group A patients, the mean diameter of duct stones was 0.6 mm (range 0-5 mm). Mean diameter of the main duct in the pancreatic head and body/tail was 4.8 mm and 4.9 mm, respectively. In group B patients, the mean diameter of duct stones was 21.9 mm (range 2-50 mm). Mean diameter of the main duct in the pancreatic head and body/tail was 18.8 mm and 13.9 mm, respectively. CONCLUSIONS|: In patients with GMAP and time interval between symptom onset and first CT scan ≤24 months (group A), CT identified normal or slightly increased parenchymal thickness and a main pancreatic duct of normal calibre and without duct stones. In contrast, in patients with GMAP and time interval between symptom onset and first CT scan >25 months (group B), it identified large-calibre duct stones with bull's-eye appearance.

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Familial Chronic Recurrent Pancreatitis in Identical Twins

Freud

Barak²,

Ziv³

et al. 1992

Arch Surg

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Familial presentation of chronic recurrent pancreatitis in childhood is rare. The etiology of this illness is obscure, and its hereditary properties are not well defined. Simultaneous occurrence of chronic recurrent pancreatitis in identical twins with the same clinical presentation and similar typical pancreatographic abnormalities is exceptional. Twin sisters, aged 9 years, were admitted to the hospital because of recurrent attacks of pancreatitis. Ultrasound examination revealed an enlarged irregular pancreatic duct in both girls, and endoscopic retrograde cholangiopancreatography showed a distorted duct with multiple strictures and dilatations similar to a "chain of lakes" pattern. Both patients underwent longitudinal pancreatojejunostomy within a month. The therapeutic regimen and preoperative and surgical treatment of such patients are discussed, as is the optimal timing of intervention.

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The diagnosis of hereditary pancreatitis by pancreatography

Cited by 19 publications

References 15 publications

New insights into hereditary pancreatitis

New insights into hereditary pancreatitis

Role of multislice computed tomography in the diagnosis of gene-mutation-associated pancreatitis (GMAP)

Familial Chronic Recurrent Pancreatitis in Identical Twins

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