Hereditary pancreatitis overview

Jump to navigation Jump to search

Hereditary pancreatitis Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Hereditary pancreatitis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Guidelines for Management

Case Studies

Case #1

Hereditary pancreatitis overview On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Hereditary pancreatitis overview

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Hereditary pancreatitis overview

CDC on Hereditary pancreatitis overview

Hereditary pancreatitis overview in the news

Blogs on Hereditary pancreatitis overview

Directions to Hospitals Treating Psoriasis

Risk calculators and risk factors for Hereditary pancreatitis overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]

Overview

Historical Perspective

In 1642, Johannes Wirsung of Padua first described the pancreatic duct and the concept of the pancreas as a secretory organ. In 1952, Comfort and Steinberg , were the first one to identify a genetic background associated with hereditary pancreatitis and they found hereditary pancreatitis in six family members spanning 3 generations. In 1996, a gene for hereditary chronic pancreatitis was mapped to chromosome 7.

Classification

Hereditary pancreatitis may be classified on the basis of mode of inheritance into autosomal dominant, autosomal recessive and hereditary pancreatitis with complex genetics.

Pathophysiology

Causes

Hereditary pancreatitis may be caused by mutation in any one of the following genes PRSS1, SPINK1, CFTR and CTRC gene.

Differentiating Hereditary pancreatitis from Other Diseases

Hereditary pancreatitis needs to be differentiated from other diseases presenting with similar complaints such as abdominal pain, diarrhea and weight loss.

Epidemiology and Demographics

The incidence of hereditary pancreatitis is approximately 3.5–10 per 100,000 individuals in Europe and the USA. In Western countries, the prevalence was found to be 0.3/100 000. Hereditary pancreatitis commonly affects younger age group. The median age at which first diagnosis is made is 19 years. The median age at which the symptoms develop is 10 years.

Risk Factors

Common risk factors in the development of Hereditary pancreatitis include smoking, alcohol consumption, lack of antioxidants, genetic mutations such as increased PRSS1 gene expression and CTRC gene mutations.

Screening

Patients with hereditary pancreatitis should be screened for pancreatic cancer as they are at markedly increased risk of pancreatic cancer.

Natural History, Complications, and Prognosis

The symptoms of hereditary pancreatitis usually develop in the first or second decade of life. Patients with hereditary pancreatitis usually present with recurrent episodes of acute pancreatitis and may develop exocrine and endocrine insufficiency. If left untreated, patients with hereditary pancreatitis may progress to develop pancreatitis, biliary or pancreatic ductal dilatation, jaundice, biliary obstruction, pancreatic duct stone or stricture, pancreatic pseudocysts, and pancreatic cancer. Mortality rate is found to be increased in patients who develop pancreatic cancer.

Diagnosis

Diagnostic Criteria

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References


Template:WikiDoc Sources