Enterokinase Intestinal Enterokinase Deficiency in Pediatrics

Authors

  • Ahmed Abdelsamie Fadl Pediatric senior registrar at Doctor Samir Abbas Hospital,Department of pediatrics, Alazhar University hospitals, Cairo. SCFHS number: 12JM0036858
  • Abdulhalim Ahmed Alabdullatif General Practitioner, King Fahd Specialist Hospital, Primary Health Care Center,Buraidah, Saudi Arabia
  • Aldebani Moluk Nabeel S Pediatric resident,Pediatric Department, Hera General Hospital, Makkah, Saudi Arabia
  • Alqahtani Mohammed Abdullah S Pediatric Resident, Pediatric Department, East Jeddah General Hospital, Jeddah, Saudi Arabia
  • Dania Ebrahim Khalil Maki Medical intern, Cairo University, Cairo, Egypt
  • Alharbi Maryam Eyadah Pediatric specialist, Makkah Healthcare Cluster, primary health care, Makkah,Saudi Arabia
  • Waleed Yahya Sufi Medical Intern, Jazan University, Jazan, Saudi Arabia
  • Abdullmajeed Saleh A Alharbi Medical Intern,Ibn sina National college, Jeddah, Saudi Arabia
  • Nada Mohammed J Alzahrani Medical Intern,Ibn sina National college, Jeddah, Saudi Arabia
  • Mohammed Ali A Alshamrani Medical Intern,Ibn sina National college, Jeddah, Saudi Arabia
  • Ahmed Abdullah Bugshan Medical Intern,Ibn sina National college, Jeddah, Saudi Arabia
  • Abdulrahman Ibrahim A Altowairqi Medical Intern,Ibn sina National college, Jeddah, Saudi Arabia
  • Jallal Mohammed A Alsameen Medical Intern,Ibn sina National college, Jeddah, Saudi Arabia
  • Waad Musayid B Alsaedi Pediatric resident,Pediatric Department, Hera General Hospital, Makkah, Saudi Arabia

DOI:

https://doi.org/10.22376/ijlpr.2023.13.1.SP1.L12-L18

Keywords:

Congenital Enteropeptidase Deficiency, Enterokinase Deficiency, Diarrhea, Pancreatic-Enzyme, Pediatrics

Abstract

Abstract: Congenital enteropeptidase deficiency (CEP), also known as enterokinase deficiency. CEP is an uncommon autosomal recessive genetic disorder mostly characterized by severe chronic diarrhoea after delivery, hypoproteinemia, and failure to grow. Enteropeptidase activity is anticipated to play a significant role in protein digestion. For growth and appropriate development in newborns with a congenital lack of the enzyme, pancreatic enzyme replacement treatment or an amino acid combination must be given. Only 13 cases of enterokinase insufficiency have been recorded since it was originally characterized in 1969. Couples should be informed that prenatal screening is an option and that EKD has a favourable prognosis. However, if an EKD patient is born, parents should be aware of the feeding issue and offer the proper pancreatic exocrine secretion medication. One research found that all patients had been diagnosed as newborns 25 years ago. Even when the pancreatic-enzyme replacement was stopped, they appeared to lead regular lives as adults, free of gastrointestinal issues and with normal body weight. This can be explained by the fact that trypsin, once liberated from its precursor, encourages additional trypsinogen activation in a positive-feedback manner. Better pharmaceutical preparations such as enteric-coated minimicrospheres and delayed-release capsules are used for better results as it maintains the enzyme and prevents its breakdown by stomach acidity. This review aims to summarise current knowledge of pathophysiology, causes, and treatment of Intestinal Enterokinase Deficiency in Pediatrics 

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Published

2022-12-31

How to Cite

Abdelsamie Fadl, A., Ahmed Alabdullatif, A., Moluk Nabeel S, A., Mohammed Abdullah S, A., Ebrahim Khalil Maki, D., Maryam Eyadah, A., Yahya Sufi, W., Saleh A Alharbi, A., Mohammed J Alzahrani, N., Ali A Alshamrani, M., Abdullah Bugshan, A., Ibrahim A Altowairqi, A., Mohammed A Alsameen, J., & Musayid B Alsaedi, W. (2022). Enterokinase Intestinal Enterokinase Deficiency in Pediatrics. International Journal of Life Science and Pharma Research, 13(SP2), L12-L18. https://doi.org/10.22376/ijlpr.2023.13.1.SP1.L12-L18

Issue

Volume 13, Special Issue 2, 2023

Section

Review Articles

Copyright (c) 2022 Ahmed Abdelsamie Fadl, Abdulhalim Ahmed Alabdullatif, Aldebani Moluk Nabeel S, Alqahtani Mohammed Abdullah S, Dania Ebrahim Khalil Maki, Alharbi Maryam Eyadah, Waleed Yahya Sufi, Abdullmajeed Saleh A Alharbi, Nada Mohammed J Alzahrani, Mohammed Ali A Alshamrani, Ahmed Abdullah Bugshan, Abdulrahman Ibrahim A Altowairqi, Jallal Mohammed A Alsameen, Waad Musayid B Alsaedi

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