Propionic acidemia medical therapy: Difference between revisions

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__NOTOC__
{{Propionic acidemia}}
{{CMG}}; {{AE}}
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==Overview==
==Medical Therapy==
==Medical Therapy==
===Management===
===Management===
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Liver transplant is gaining a role in the management of these patients, with small series showing improved quality of life.
Liver transplant is gaining a role in the management of these patients, with small series showing improved quality of life.


==References==
==References==
{{reflist}}
{{reflist}}
[[Category:Endocrinology]]
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{{WH}}

Latest revision as of 13:29, 25 July 2016

Propionic acidemia Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Propionic acidemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Propionic acidemia medical therapy On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Propionic acidemia medical therapy

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Propionic acidemia medical therapy

CDC on Propionic acidemia medical therapy

Propionic acidemia medical therapy in the news

Blogs on Propionic acidemia medical therapy

Directions to Hospitals Treating Cushing’s disease

Risk calculators and risk factors for Propionic acidemia medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Overview

Medical Therapy

Management

Patients with propionic acidemia should be started as early as possible on a low protein diet. In addition to a protein mixture that is devoid of methionine, threonine, valine, and isoleucine, the patient should also receive L-carnitine treatment and should be given antibiotics 10 days per month in order to remove the intestinal propiogenic flora. The patient should have diet protocols prepared for him with a “well day diet” with low protein content, a “half emergency diet” containing half of the protein requirements, and an “emergency diet” with no protein content. These patients are under the risk of severe hyperammonemia during infections that can lead to comatose states.[1]

Liver transplant is gaining a role in the management of these patients, with small series showing improved quality of life.

References

  1. Saudubray JM, Van Der Bergh G, Walter J : Inborn Metabolic Diseases Diagnosis and Treatment (2012)

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