Genetics of Nail-Patella Syndrome Medication

Updated: Mar 24, 2016
  • Author: Julie Hoover-Fong, MD, PhD, FACMG; Chief Editor: Maria Descartes, MD  more...
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Medication

Medication Summary

ACE inhibitors should be used to treat proteinuria, hypertension, or both in nail-patella syndrome (NPS). Consultation with a nephrologist may permit implementation of prophylactic treatment with ACE inhibitor medication prior to overt proteinuria or hypertension.

Vitamin D analogs, thiazides, and prednisone are effective in alleviating the complicating symptoms of nephrotic syndrome and end-stage renal failure (ESRF) in nail-patella syndrome as in all patients with ESRF. [12]

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Vitamin D Analog

Class Summary

Vitamin D is necessary to maintain the correct amount of calcium needed for strong bones and teeth and is needed throughout the body.

Calcitriol (Rocaltrol)

Increases calcium levels by promoting absorption of calcium in intestines and retention in kidneys. The beneficial effects of vitamin D replacement in renal osteodystrophy appear to result from correction of hypocalcemia and secondary hyperparathyroidism.

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Thiazide diuretics

Class Summary

These agents are used to treat edema caused by renal dysfunction (eg, nephrotic syndrome, chronic renal failure).

Hydrochlorothiazide (Hydro-Diuril, Microzide)

Inhibits reabsorption of sodium in distal tubules, increasing excretion of sodium, water, and potassium and hydrogen ions.

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Corticosteroids

Class Summary

These agents induce diuresis or remission of proteinuria in nephrotic syndrome.

Prednisone (Deltasone, Orasone)

Acts as an anti-inflammatory agent and immunosuppressant.

Dose depends on specific disease entity being treated; in situations of less severity, lower doses generally suffice, whereas, in selected patients, higher initial doses may be required; initial dosage should be maintained or adjusted prn.

Alternate day therapy (ADT) PO is a corticosteroid-dosing regimen in which twice the usual daily dose of corticoid is administered every other morning.

The purpose of this mode of therapy is to provide the patient who requires long-term pharmacologic dose treatment with the beneficial effects of corticoids while minimizing certain undesirable effects, including pituitary-adrenal suppression, the Cushingoid state, corticoid withdrawal symptoms, and growth suppression in children.

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Angiotensin-converting Enzyme (ace) Inhibitor

Class Summary

ACE inhibitors are preferred for treating hypertension and proteinuria associated with nail-patella syndrome.

Captopril (Capoten)

Captopril is an example of an ACE inhibitor used off-label for hypertension and proteinuria in neonates, infants, and children. Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.

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