Acute pancreatitis in identical twins

Henderson, J.; Ingram, David; House, Tony

doi:10.5694/j.1326-5377.1982.tb132401.x

Cited by 8 publications

(1 citation statement)

References 6 publications

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“…6 7 17-20 Four twin pairs were excluded from analysis. The twins reported by Henderson and colleagues20and Beall and colleagues18 were concordant in phenotype but were excluded because of the late age of disease onset (34 years and 31 years) in the context of excessive alcohol consumption. A pair of 13 year old twins from the J family,10 and a pair of six year old twins of the P family21 each have an affected father but were excluded from the study because of undetermined genotype and because the twins were less than 20 years of age and without phenotypic expression of pancreatitis.…”

Section: Resultsmentioning

confidence: 99%

Expression and penetrance of the hereditary pancreatitis phenotype in monozygotic twins

Amann

Gates

Aston

et al. 2001

Gut

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Background-Hereditary pancreatitis (HP) is a rare autosomal dominant disorder with variable expression and an overall lifetime penetrance of 80%. We hypothesised that (1) monozygotic twins within similar environments would develop the typical signs of HP at a similar age, and (2) if penetrance were due to modifier genes or environment, all twin pairs would be concordant for expression of HP. Aim-Identify monozygotic twins with HP and determine the penetrance, concordance, and age of onset of symptoms. Methods-Twins from HP kindreds were identified from the Midwest Multicenter Pancreatic Study group database, referrals, and literature searches. Each twin set was assessed for phenotypic expression, concordance, and diVerence in age of phenotypic onset of pancreatitis. The diVerence in onset of symptoms for symptomatic aVected non-twin sibling pairs as well as non-twin pairs that were mutation, sex, and age matched were calculated as two comparison groups. Results-Seven of 11 monozygotic pairs identified were suitable for evaluation and four were concordant for pancreatitis. Forty eight aVected sibling pairs and 33 pairs of mutation, sex, and age matched (cationic trypsinogen R122H (30 pairs) and N29I (three pairs)) subjects were identified for comparison groups. The median (quartiles Q1, Q3) diVerence in the age of phenotypic onset in the concordant twins was 1 (0, 2.4) years, 2 (1, 6) for the aVected siblings, and 7 (2, 15) years in the comparison control group. Three of the seven sets of twins (43%) were discordant for phenotypic expression of pancreatitis. The overall penetrance in the seven pairs of monozygotic twins was 78.6%. Conclusions-Genetic and/or environmental factors contribute to expression and age of onset of HP. Nuclear genes or general environmental factors alone cannot explain the 80% penetrance. Determining the mechanism of non-penetrance may help in developing a strategy to prevent the phenotypic expression of pancreatitis in individuals with an underlying genetic predisposition. (Gut 2001;48:542-547)

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Section: Resultsmentioning

confidence: 99%

Expression and penetrance of the hereditary pancreatitis phenotype in monozygotic twins

Amann

Gates

Aston

et al. 2001

Gut

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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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Hereditäre Pankreatitis

Tomsik

Wagner

1992

Ergebnisse Der Inneren Medizin Und Kinderheilkunde/Advances in Internal Medicine and Pediatrics

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Acute pancreatitis in identical twins

Cited by 8 publications

References 6 publications

Expression and penetrance of the hereditary pancreatitis phenotype in monozygotic twins

Expression and penetrance of the hereditary pancreatitis phenotype in monozygotic twins

Familial Chronic Recurrent Pancreatitis in Identical Twins

Hereditäre Pankreatitis

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