췌장 외분비선 기능부전증 Pancreatic exocrine insufficiency

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췌장 외분비선 기능부전증 Pancreatic exocrine insufficiency

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그림 265. 췌장과 담즙분비.

Copyright Ⓒ 2013 John Sangwon Lee, MD., FAAP

  • 췌장의 외분비선에서 분비되는 효소 결핍증으로 지방성 설사를 할 수 있고 흡수장애가 생길 수 있는 병을 췌장 외분비선 기능부전증이라 한다. 
  • 췌장의 랑겔한스 내분비선에서 인슐린, 글루카곤, 소마토스타틴이 분비되고, 
  • 췌장외분비선에서 트립시노젠, 엘라스타제, 리파제, 아밀라제 등 소화 효소가 정상적으로 분비된다. 
  • 췌장은 내분비선에 속하고 외분비선에도 속한다. 
  • 효소가 적절히 분비되지 않으면 탄수화물 음식물도 지방성분 음식물도 소화가 되지 않는다. 
  • 만일 췌장 리파제가 분비되지 않으면 지방변 설사를 하고 지방흡수 장애가 생길 수 있다. 
  • 췌장 낭포성 섬유증, 슈와크만 다이모도 증후군(Shwachman diamond syndrome/췌장 기능부전증과 골수 발육부전증), 심한 영양실조증, 선천성 리파제 결핍증 등이 있으면 췌장 외분비선 기능부전증이 생길 수 있다. 
  • 병력, 증상 징후, 검진, 췌장액 검사, 대변 검사 등을 종합해 진단한다. 
  • 췌장 효소, 종합 비타민, 트라이글리세라이드 등으로 치료하고 원인되는 병을 치료한다. 

Pancreatic exocrine insufficiency 췌장 외분비선 기능부전증

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Figure 265. Pancreas and bile secretion. Copyright Ⓒ 2013 John Sangwon Lee, MD., FAAP

• A disease that can cause fatty diarrhea and malabsorption due to deficiency of enzymes secreted from the exocrine glands of the pancreas is called pancreatic exocrine insufficiency.

• Insulin, glucagon, and somatostatin are secreted by the Langelhans endocrine glands of the pancreas,

• Digestive enzymes such as trypsinogen, elastase, lipase, and amylase are normally secreted from the pancreatic exocrine glands.

• The pancreas belongs to the endocrine glands and also the exocrine glands.

• If enzymes are not secreted properly, neither carbohydrate foods nor fatty foods can be digested.

• If pancreatic lipase is not secreted, it may cause stool diarrhea and impaired liposuction.

• Pancreatic exocrine insufficiency can occur if there are cystic fibrosis of the pancreas, Shwachman diamond syndrome (pancreatic insufficiency and bone marrow insufficiency), severe malnutrition, and congenital lipase deficiency.

• Comprehensive diagnosis including medical history, symptom signs, examination, pancreatic fluid test, and stool test.

• Treats with pancreatic enzymes, multivitamins, triglycerides, etc. and treats the underlying disease

 

출처 및 참조 문헌 Sources and references

  • NelsonTextbook of Pediatrics 22ND Ed
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  • Neonatal Resuscitation, American Academy Pediatrics
  • www.drleepediatrics.com제7권 소아청소년 감염병
  • www.drleepediatrics.com제9권 소아청소년 소화기 질환
  •  Red book 29th-31st edition 2021
  • Nelson Text Book of Pediatrics 19th — 21st Edition
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  • Pediatric nutrition handbook, AAP p.216, edition 1993
  • Text book of Pediatrics, Nelson 
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  • Other

Copyright ⓒ 2015 John Sangwon Lee, MD., FAAP

부모도 반의사가 되어야 한다”-내용은 여러분들의 의사로부터 얻은 정보와 진료를 대신할 수 없습니다.

“The information contained in this publication should not be used as a substitute for the medical care and advice of your doctor. There may be variations in treatment that your doctor may recommend based on individual facts and circumstances. “Parental education is the best medicine.”