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Shwachman-Diamond Syndrome Medication

  • Author: Antoinette C Spoto-Cannons, MD, FAAP; Chief Editor: Hassan M Yaish, MD  more...
 
Updated: May 06, 2016
 

Pancreatic enzymes

Class Summary

These agents are used to replace endogenous pancreatic enzymes.

Pancrelipase (Creon, Ku-Zyme, Pancrease, Viokase, Ultrase)

 

Assists in digestion of protein, carbohydrates, and fats. Dosage recommendations are only approximations for initial dosages. Actual dose depends on digestive requirement of individual patient.

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Colony-stimulating factors

Class Summary

These agents provide red cell line stimulation.

Epoetin alfa (Epogen, Procrit)

 

FDA approved for treatment of chronic anemia. Stimulates division and differentiation of committed erythroid progenitor cells; induces release of reticulocytes from bone marrow into bloodstream. SC route provides sustained serum levels compared to IV route. Reduce dose when hematocrit reaches target range of 30-36% or hematocrit increases >4 points over any 2-wk period. Hold dose if hematocrit >36%.

Filgrastim (Neupogen)

 

FDA approved for severe chronic neutropenia.

GCSF activates and stimulates production, maturation, migration, and cytotoxicity of neutrophils. If IV route is used and GCSF final concentration is < 15 mcg/mL, add 2 mg of albumin/mL to prevent drug adsorption to administration set.

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Vitamins

Class Summary

Vitamins are essential for normal DNA synthesis and cell function.

ADEK vitamins (ADEKS)

 

PO multinutrient specially formulated for use under medical supervision to provide nutritional supplementation in individuals with malabsorptive conditions. Each dose contains water-miscible forms of fat-soluble vitamins A, D, E, and K plus other nutrients, including vitamin C, B-complex vitamins, biotin, folic acid, and zinc. Available as chewable tab or pediatric drops.

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Contributor Information and Disclosures
Author

Antoinette C Spoto-Cannons, MD, FAAP Pediatric Hospitalist, Florida Pediatric Associates, LLC; Clinical Affiliate Associate Professor of Pediatrics, University of South Florida Morsani College of Medicine

Antoinette C Spoto-Cannons, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Physician Leadership, Florida Chapter of The American Academy of Pediatrics, Florida Pediatric Society

Disclosure: Nothing to disclose.

Coauthor(s)

Mudra Kumar, MD, MRCP, FAAP Professor of Pediatrics, Course Director, Course 6 MSII, Preclerkship Director, Clinical Integration, Department of Pediatrics, University of South Florida Morsani College of Medicine

Mudra Kumar, MD, MRCP, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Jessica Marie Keshishian, MD Resident Physician, Department of Pediatrics, University of South Florida

Jessica Marie Keshishian, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Student Association/Foundation, Christian Medical and Dental Associations

Disclosure: Nothing to disclose.

Sarah Syed University of South Florida College of Medicine

Sarah Syed is a member of the following medical societies: American Medical Student Association/Foundation

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Hassan M Yaish, MD Medical Director, Intermountain Hemophilia and Thrombophilia Treatment Center; Professor of Pediatrics, University of Utah School of Medicine; Director of Hematology, Pediatric Hematologist/Oncologist, Department of Pediatrics, Primary Children's Medical Center

Hassan M Yaish, MD is a member of the following medical societies: American Academy of Pediatrics, New York Academy of Sciences, American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Michigan State Medical Society

Disclosure: Nothing to disclose.

Additional Contributors

Sharada A Sarnaik, MBBS Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Associate Hematologist/Oncologist, Children's Hospital of Michigan

Sharada A Sarnaik, MBBS is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, Society for Pediatric Research, Children's Oncology Group, American Academy of Pediatrics, Midwest Society for Pediatric Research

Disclosure: Nothing to disclose.

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  99. Woloszynek JR, Rothbaum RJ, Rawls AS, et al. Mutations of the SBDS gene are present in most patients with Shwachman-Diamond syndrome. Blood. 2004 Dec 1. 104(12):3588-90. [Medline]. [Full Text].

  100. Yamaguchi M, Fujimura K, Toga H, et al. Shwachman-Diamond syndrome is not necessary for the terminal maturation of neutrophils but is important for maintaining viability of granulocyte precursors. Exp Hematol. 2007 Apr. 35(4):579-86. [Medline].

 
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