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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have studied a family in which three people, two child and an adult had hereditary pancreatitis. Hereditary pancreatitis is not a common disease. Abdominal pain is usually the first clinical sign in children. The knowledge of the existence of such a disease and familiarity with the family history of the child allow the pediatrician to make the diagnosis as soon as the first crisis appears. Surgery is thus avoided. Our studies show definitively that this disease is hereditary and is autosomal dominant with a strong decrease of the penetrance.
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PMID:[Hereditary chronic pancreatitis: an autosomal dominant disease (author's transl)]. 22 88

Information from 72 patients from 7 families in England and Wales confirms that hereditary pancreatitis is inherited as an autosomal dominant conditions with limited penetrance. The degree of penetrance is approximately 80%. These patients have had recurrent attacks of abdominal pain starting from childhood or young adult life. The mean age of onset in the 7 families studied was 13.6 years. There were two peaks, with maximum numbers at 5 years and 17 years. The second peak was thought to represent genetically susceptible individuals having pain brought on by alcohol rather than representing evidence of genetic heterogeneity. Five of the 7 families had members with both childhood and adult ages of onset. Only 4 patients out of 72 had life-threatening disease and in the majority of cases the attacks of pain were of nuisance value only. Hereditary pancreatitis was implicated in only 1 patient's death and this was not definite. Patients appear to get better after a period of symptoms usually as they approach middle age, or after a severe attack. In older patients alcohol, emotional upsets, and fatty food appear to precipitate attacks. Pancreatic insufficiency (5.5%), diabetes mellitus (12.5%), pseudocysts (5.5%), and haemorrhagic pleural effusion are uncommon complications. Portal vein thrombosis occurred definitely in 2 patients and was suspected in 3 others. Carcinoma of the pancreas was not found in any of 72 patients studied in detail; however, 2 members from a family not visited personally had chronic pancreatitis and malabsorption going on to carcinoma. They may have suffered from a different disease. Genetic linkage information was too slight for many definite conclusions. However, there was no suggestion of linkage with any of the markers tested.
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PMID:Hereditary pancreatitis in England and Wales. 67 83

Hereditary pancreatitis appears in many different ways and in a variety of age groups, spanning both pediatric and adult medicine. The variable expression of hereditary pancreatitis is emphasized by the difficulty in diagnosing it in a patient obviously at risk because of a severely affected father and son. The morphine prostigmine test and hypotonic duodenogram were most helpful. Aminoaciduria previously associated with this disorder is coincidental or nonspecifically related to acute pancreatic inflammation. The increased risk for pancreatic carcinoma (about 20%) is emphasized by the concern for that complication in the proband's grandfather.
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PMID:Hereditary pancreatitis. Nonspecificity of aminoaciduria and diagnosis of occult disease. 113 Sep 28

The role of operative intervention for hereditary pancreatitis, a rare form of chronic parenchymal destruction, is unclear. To determine whether surgical therapy is safe and provides prolonged symptomatic relief, the authors reviewed the management of 22 adults (11 men, 11 women) with hereditary pancreatitis treated surgically between 1950 and 1989. Hereditary pancreatitis was defined as a family history of two or more relatives with pancreatitis and clinical, biochemical, or radiologic evidence of pancreatitis. The mean ages at onset of symptoms and at operation were 15 years (range, 3 to 52 years) and 31 years (range, 18 to 54 years), respectively. Pain was the primary indication for operation in all patients. Additional symptoms included nausea, vomiting (73%), weight loss (55%), and diarrhea (41%). Ductal dilatation was present in 68%, pancreatic parenchymal calcifications in 73%, pseudocysts in 36%, and splenic vein thrombosis in 18%. Primary operations included ductal drainage in 10 patients, pancreatic resection alone in three, resection with drainage in three, cholecystectomy plus sphincteroplasty in two, cholecystectomy with or without common bile duct exploration in two, pancreatic abscess drainage in one, and pseudocyst drainage in one. There were no perioperative deaths, and the morbidity rate was 14% (intra-abdominal abscess, wound infection, and urinary tract infection). Symptoms recurred in nine patients. Severity prompted reoperation in five. Secondary operations included pancreatic resection in three, pseudocyst excision in one, and pancreaticolithotomy in one. Follow-up to date is complete and extends for a median of 85 months. Eighteen patients (82%) are clinically improved or asymptomatic. Symptoms have persisted in four patients, and two patients have died of pancreatic carcinoma. Two patients died of unrelated causes. Surgical therapy for patients with hereditary pancreatitis selected on the basis of the traditional indications for surgical treatment of chronic pancreatitis is safe and efficacious.
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PMID:The surgical spectrum of hereditary pancreatitis in adults. 173 48

The authors report one case of calcifying, chronic pancreatitis in an 18-year-old patient, discovered during a diabetic coma. The patient had been suffering from steatorrhoea for several years, and his father had died from acute pancreatitis. Hereditary pancreatitis and nutritionally provoked, juvenile, tropical pancreatitis are discussed. The patient's brothers and sisters have not been examined yet.
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PMID:[Chronic calcifying pancreatitis in a young patient in a tropical environment. Report of a case]. 249 71

We report a case of an adolescent girl who presented with painless massive ascites secondary to chronic pancreatitis and a ductal fistula. The diagnosis was delayed and an unnecessary laparotomy was performed, as initial evaluation of ascites did not include measurement of serum and ascitic amylase. Evidence of pancreatic abnormalities in asymptomatic relatives suggested an underlying hereditary pancreatitis. Hereditary pancreatitis presenting as pancreatic ascites, to our knowledge, has not been described previously.
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PMID:Hereditary pancreatitis presenting with ascites. 380 82

Hereditary pancreatitis was diagnosed in dystrophic boy at the age of 2 1/2 years after recurring episodes of severe abdominal pain. Extensive left-sided pleural effusions caused surgical exploration. Pancreatography showed typical dilation of the pancreatic ductal system. After hemipancreatectomy by the boy recovered very fast. Characteristic features in diagnosing of hereditary pancreatitis are the onset of recurring attacks of abdominal pain in early childhood and anatomical defects in the pancreatic ductal system. The disease shows variability between family members.
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PMID:[Hereditary pancreatitis in early childhood (author's transl)]. 610 43

Hereditary pancreatitis is a rare form of chronic pancreatitis inherited as an autosomal dominant. Pancreas divisum results from a developmental failure of fusion of the dorsal and ventral parts of the pancreas and is associated with an increased incidence of pancreatitis. Co-existence of the two conditions in one patient is very rare. We report a case.
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PMID:A case of hereditary pancreatitis and pancreas divisum. 672 79

Hereditary pancreatitis is an autosomal dominant disease with no other known cause. It usually begins in childhood and is characterized by recurrent attacks of abdominal pain of variable intensity and duration, followed by symptom-free periods. The diagnosis is usually made in early adult life, when pancreatic insufficiency and calcifications appear. Complications are less frequent than in nonhereditary chronic pancreatitis. There are also differences between the two forms of chronic pancreatitis in sex incidence, etiologic factors and life expectancy. In a Canadian kindred three generations are affected with hereditary pancreatitis; there are four definite and four suspected cases. More than 40 affected kindreds, including 195 proven cases and 190 suspected cases, have now been reported in the literature. Thus, hereditary pancreatitis should be considered in the differential diagnosis of chronic relapsing pancreatitis of unknown cause as well as recurrent abdominal pain in childhood.
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PMID:Hereditary pancreatitis: report of an affected Canadian kindred and review of the disease. 702 15

Hereditary pancreatitis (HP) is a rare cause of chronic pancreatitis. Recurrent abdominal pain is the most common clinical manifestation, with onset in childhood or adolescence. PURPOSE--Report of a case of HP with atypical presentation and a review of the literature. METHODS--A non-alcoholic patient, without history of abdominal pain, with steatorrhea and malnutrition was investigated. The diagnostic evaluation revealed severe chronic pancreatitis. Two close relatives with early onset calcifying pancreatitis were detected. Epidemiologic and clinical features of HP were reviewed. CONCLUSION--Although uncommon, HP should be regarded in the differential diagnosis of chronic pancreatitis. Familial screening of suspected cases should be routinely performed.
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PMID:[Hereditary pancreatitis]. 763 5


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