eMedicine Specialties > Pediatrics: General Medicine > Nephrology

Medullary Cystic Disease: Follow-up

Author: Prasad Devarajan, MD, Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Contributor Information and Disclosures

Updated: Dec 15, 2008

Follow-up

Transfer

  • Transfer to a pediatric dialysis unit is required when the child with nephronophthisis (NPH)–medullary cystic kidney disease (MCKD) develops end-stage renal disease (ESRD).

Complications

  • Complications are those of progressive renal failure.

Prognosis

  • ESRD develops in all patients.
  • The disease does not recur in transplanted kidneys.

Patient Education

  • As ESRD develops, appropriate nutritional and medical education should be made available.

Miscellaneous

Medicolegal Pitfalls

  • Failure to recognize nephronophthisis (NPH)–medullary cystic kidney disease (MCKD) in a child with polyuria, anemia, growth retardation, and normal kidney function
 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Marcia Del Rio, MD, to the development and writing of this article.



More on Medullary Cystic Disease

Overview: Medullary Cystic Disease
Differential Diagnoses & Workup: Medullary Cystic Disease
Treatment & Medication: Medullary Cystic Disease
Follow-up: Medullary Cystic Disease
References
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Further Reading

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Keywords

medullary cystic disease, nephronophthisis, NPH, juvenile nephronophthisis, NPH1, infantile nephronophthisis, NPH2, adolescent nephronophthisis, NPH3, medullary cystic kidney disease, MCKD, MCKD1, MCKD2, juvenile nephronophthisis–medullary cystic kidney disease complex, juvenile nephronophthisis–medullary cystic kidney disease, nephronophthisis–medullary cystic kidney disease complex, nephronophthisis–medullary cystic kidney disease, nephronophthisis–medullary cystic disease, NPH-MCKD, ciliopathies, end-stage renal disease, ESRD, renal insufficiency, polyuria, polydipsia, Cogan syndrome, Senior-Loken syndrome, Mainzer-Saldino syndrome, Joubert syndrome, Sensenbrenner syndrome, hyperuricemia, gout, Jeune syndrome, asphyxiating thoracic dysplasia, obesity, Ellis-van Creveld syndrome, PHYNS syndrome, Laurence-Moon-Bardet-Biedl syndrome, renal failure

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Contributor Information and Disclosures

Author

Prasad Devarajan, MD, Louise M Williams Endowed Chair in Pediatrics, Professor of Pediatrics and Developmental Biology, Director of Nephrology and Hypertension, Director of Clinical Nephrology Laboratories, Chief Executive Officer of Dialysis Unit, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine
Prasad Devarajan, MD is a member of the following medical societies: American Heart Association, American Society of Nephrology, American Society of Pediatric Nephrology, National Kidney Foundation, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Deogracias Pena, MD, Medical Director of Dialysis, Department of Pediatrics, Cook Children's Medical Center; Clinical Associate Professor, Texas Tech University School of Medicine
Deogracias Pena, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, and American Society of Pediatric Nephrology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Frederick J Kaskel, MD, PhD, Director of the Division and Training Program in Pediatric Nephrology, Vice Chair, Department of Pediatrics, Montefiore Medical Center and Albert Einstein School of Medicine
Frederick J Kaskel, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Eastern Society for Pediatric Research, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Renal Physicians Association, Sigma Xi, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Howard Trachtman, MD, Program Director, Pediatrics Research, Schneider Children's Hospital, Department of Pediatrics, Division of Nephrology, Professor, Albert Einstein College of Medicine
Howard Trachtman, MD is a member of the following medical societies: American Society of Hypertension, American Society of Nephrology, American Society of Pediatric Nephrology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD, The Isaac A Abt, MD, Professor of Kidney Diseases, Feinberg School of Medicine, Northwestern University; Division Head of Kidney Diseases, Children's Memorial Hospital, Chicago
Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, and International Society of Nephrology
Disclosure: Amgen Grant/research funds None; Abbott Honoraria Speaking and teaching; Altus Pharmaceuticals Grant/research funds None; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None

 
 
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