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Potter Syndrome Medication

  • Author: Sushil Gupta, MD; Chief Editor: Craig B Langman, MD  more...
 
Updated: Jan 21, 2015
 

Medication Summary

Multiple medications are typically indicated in patients with acute or chronic renal failure. The treatment should address fluid and electrolyte disturbances, hypertension, anemia, calcium and phosphorus disorders, and growth failure.

Treatment regimens for hypertension are designed to reduce blood pressure and other risk factors of coronary heart disease. Diuretic agents help relieve fluid overload associated with renal failure. Additional pharmacotherapy for hypertension associated with renal failure should be individualized based on the patient's age, race, known pathophysiologic variables, and concurrent conditions. Treatment goals are not only to lower blood pressure safely and effectively but also to prevent or reverse hyperlipidemia, glucose intolerance, and left ventricular hypertrophy. For complete information, see the pediatric topics Hypertension and Neonatal Hypertension.

Erythropoietin is essential for red blood cell production and may be required because of decreased erythropoietin levels in renal failure. Vitamin D analogs are essential to provide homeostasis for calcium regulation. Growth hormone may be required because of inadequate growth in children with renal failure.

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Pituitary hormones

Class Summary

The anterior lobe of the pituitary gland is responsible for the secretion of adrenocorticotrophic hormone (corticotropin); gonadotrophic hormones (gonadotropins), including follicle-stimulating hormone and luteinizing hormone; growth hormone (somatropin); lactogenic hormone (prolactin); and thyroid-stimulating hormone (thyrotropin). The secretion of anterior pituitary hormones is regulated by a complex interaction between stimulatory and inhibitory neural and hormonal influences. Hypothalamic releasing factors stimulate the release of anterior pituitary hormones into the systemic circulation. Some pituitary hormones (eg, growth hormone) are controlled with a system of double regulation (ie, the hypothalamus secretes a release-inhibiting factor).

Human growth hormone (Genotropin, Humatrope, Nutropin, Serostim, Saizen)

 

Used to treat inadequate growth in children with chronic renal failure. Stimulates growth of linear bone, skeletal muscle, and organs. Stimulates erythropoietin, which increases red blood cell mass.

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Erythropoietin

Class Summary

Glycoprotein is normally produced in the kidneys. It is responsible for the stimulation of red blood cell production. Anemia occurs because of deficient erythropoietin production during renal failure.

Epoetin alfa (Epogen, Procrit)

 

Derived via recombinant DNA techniques. The amino acid sequence is identical to that of endogenous erythropoietin. Stimulates division and differentiation of committed erythroid progenitor cells; induces reticulocyte release from bone marrow into blood stream.

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Colony-stimulating Factor

Darbepoetin (Aranesp)

 

Darbepoetin is used in anemia associated with chronic kidney disease. It is also used for the treatment of chemotherapy-induced anemia in patients with nonmyeloid malignancies. It is an erythropoiesis-stimulating protein closely related to erythropoietin, a primary growth factor produced in kidney that stimulates the development of erythroid progenitor cells.

The mechanism of action is similar to that of endogenous erythropoietin, which interacts with stem cells to increase red blood cell production. It differs from epoetin alfa (recombinant human erythropoietin) in containing 5 N-linked oligosaccharide chains, whereas epoetin alfa contains 3. It has a longer half-life than epoetin alfa (may be administered weekly or biweekly).

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Diuretic agents

Class Summary

Diuretic agents promote the excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention results in edema or ascites. They may be used as monotherapy or in combination to treat hypertension. In renal failure, hypertension is due to fluid overload.

Furosemide (Lasix)

 

Increases excretion of water by interfering with chloride-binding cotransport system, which in turn inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule. Dose must be individualized to the patient.

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Vitamin D analogs

Class Summary

These agents regulate serum calcium via their actions on calcium and phosphorus metabolism at intestinal, renal, and skeletal sites. The kidney appears to play a central role in this system. It produces calcitriol (ie, 1,25-dihydroxyvitamin D, the primary active metabolite of vitamin D3), which acts on distal organs; at the same time, it is the target organ of PTH; calcitonin; and, possibly, calcitriol.

Calcitriol (Rocaltrol)

 

Vitamin D analog used in the treatment of vitamin D deficiency. Increases calcium levels by promoting the absorption of calcium in the intestines and its retention in kidneys

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Antacid

Calcium carbonate

 

Calcium carbonate successfully normalizes phosphate concentrations in dialysis patients. It combines with dietary phosphate to form insoluble calcium phosphate, which is excreted in feces. It is marketed in a variety of dosage forms and is relatively inexpensive.

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Contributor Information and Disclosures
Author

Sushil Gupta, MD Assistant Professor of Pediatric Nephrology, University of Louisville School of Medicine

Sushil Gupta, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, American Society of Pediatric Nephrology, Indian Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Carlos E Araya, MD, FAAP Assistant Professor of Pediatrics, University of Central Florida College of Medicine; Faculty, Florida Hospital; Faculty, Nemours Children's Hospital

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

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.

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 Association for the Advancement of Science, Eastern Society for Pediatric Research, Renal Physicians Association, American Academy of Pediatrics, American Pediatric Society, American Physiological Society, American Society of Nephrology, American Society of Pediatric Nephrology, American Society of Transplantation, Federation of American Societies for Experimental Biology, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Sigma Xi, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann and Robert H Lurie Children's Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, International Society of Nephrology

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Raptor Pharmaceuticals; Eli Lilly and Company; Dicerna<br/>Received grant/research funds from NIH for none; Received grant/research funds from Raptor Pharmaceuticals, Inc for none; Received grant/research funds from Alexion Pharmaceuticals, Inc. for none; Received consulting fee from DiCerna Pharmaceutical Inc. for none.

Additional Contributors

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

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

Disclosure: Nothing to disclose.

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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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