Potter Syndrome Follow-up

  • Author: Sushil Gupta, MD; Chief Editor: Craig B Langman, MD   more...
 
Updated: Jun 30, 2010
 

Further Inpatient Care

Neonates with Potter syndrome should be admitted to the neonatal ICU (NICU).

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Further Outpatient Care

  • Depends on the underlying renal condition and renal function
  • Close follow-up with a pediatric nephrologist is needed
  • Involves careful monitoring of renal function
  • Requires careful monitoring of respiratory function
  • Includes careful monitoring of medications and their adverse effects.
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Inpatient & Outpatient Medications

  • Medications that are used in the treatment of hypertension include beta-blockers, calcium-channel blockers, ACE inhibitors, and diuretics.
  • Diuretics can be used in the treatment of fluid overload and related hypertension.
  • Calcium carbonate is used to treat hypocalcemia and hyperphosphatemia.
  • Vitamin D is used to treat hyperparathyroidism.
  • Erythropoietin is used in the treatment of anemia associated with renal failure.
  • Growth hormone is used in children with growth failure associated with chronic renal failure.
  • Oral or parenteral iron may be required to treat anemia associated with chronic renal failure.
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Transfer

Transfer the patient to a center where pediatric subspecialists are available for consultation.

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Deterrence/Prevention

No preventive measures are known for any causes listed above.

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Complications

  • Associated pulmonary complications include the following:
    • Spontaneous pneumothorax due to pulmonary hypoplasia
    • Neonatal respiratory distress due to pulmonary hypoplasia
  • Associated renal complications include the following:
    • Hypertension that requires antihypertensive drug therapy
    • Hyperkalemia
    • Hypercalcemia
    • Hyperphosphatemia
    • Hyponatremia
    • Acute renal failure
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Prognosis

See Mortality/Morbidity.

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Patient Education

  • Prenatal care should be provided with the help of a perinatologist.
  • Parents should be fully aware of and educated about oligohydramnios and its long-term consequences on the developing fetus.
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Contributor Information and Disclosures
Author

Sushil Gupta, MD  Fellow, Department of Pediatric Nephrology, University Of Florida College of Medicine

Sushil Gupta, MD is a member of the following medical societies: American Society of Nephrology, American Society of Pediatric Nephrology, Indian Medical Association, and National Kidney Foundation

Disclosure: Nothing to disclose.

Coauthor(s)

Carlos E Araya, MD  Clinical Assistant Professor, Division of Pediatric Nephrology, University of Florida College of Medicine; Medical Director, Pediatric Dialysis Unit, Shands Children's Hospital

Carlos E Araya, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Nephrology, and International Pediatric Transplant Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Laurence Finberg, MD  Clinical Professor, Department of Pediatrics, University of California at San Francisco and Stanford University

Laurence Finberg, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

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.

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; Genzyme Grant/research funds None; Merck Grant/research funds None; NIH Grant/research funds None; Raptor Pharmaceuticals, Inc Grant/research funds None; Alexion Pharmaceuticals, Inc. Grant/research funds None

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Sonogram obtained before second-trimester amnioinfusion. This fetus has bilaterally absent kidneys consistent with a diagnosis of Potter syndrome. The cystic structures in the renal fossae are most likely the adrenal glands.
 
 
 
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