2024 ICD-10-CM Diagnosis Code E71.541
Zellweger-like syndrome
- ICD-10-CM Code:
- E71.541
- ICD-10 Code for:
- Zellweger-like syndrome
- Is Billable?
- Yes - Valid for Submission
- Chronic Condition Indicator: [1]
- Chronic
- Code Navigator:
E71.541 is a billable diagnosis code used to specify a medical diagnosis of zellweger-like syndrome. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024.
Approximate Synonyms
The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:
- Contiguous ABCD1 DXS1357E deletion syndrome
- Loss of multiple peroxisomal functions
- Zellweger-like syndrome without peroxisomal anomaly
- Zellweger's-like syndrome
Clinical Classification
Clinical Category is Other specified and unspecified nutritional and metabolic disorders
- CCSR Category Code: END016
- Inpatient Default CCSR: Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.
- Outpatient Default CCSR: Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.
Clinical Information
Peroxisomal Disorders
a heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional peroxisomes. peroxisomal enzymatic abnormalities may be single or multiple. biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. diseases in this category include zellweger syndrome; infantile refsum disease; rhizomelic chondrodysplasia (chondrodysplasia punctata, rhizomelic); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and adrenoleukodystrophy (x-linked). neurologic dysfunction is a prominent feature of most peroxisomal disorders.
Index to Diseases and Injuries References
The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).
- - Disorder (of) - See Also: Disease;
- - peroxisomal - E71.50
- - Zellweger-like syndrome - E71.541
- - peroxisomal - E71.50
- - Syndrome - See Also: Disease;
- - Zellweger-like syndrome - E71.541
Convert E71.541 to ICD-9-CM
- ICD-9-CM Code: 277.86 - Peroxisomal disorders
Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.
Patient Education
Metabolic Disorders
Metabolism is the process your body uses to get or make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues, such as your liver, muscles, and body fat.
A metabolic disorder occurs when abnormal chemical reactions in your body disrupt this process. When this happens, you might have too much of some substances or too little of other ones that you need to stay healthy. There are different groups of disorders. Some affect the breakdown of amino acids, carbohydrates, or lipids. Another group, mitochondrial diseases, affects the parts of the cells that produce the energy.
You can develop a metabolic disorder when some organs, such as your liver or pancreas, become diseased or do not function normally. Diabetes is an example.
[Learn More in MedlinePlus]
D-bifunctional protein deficiency
D-bifunctional protein deficiency is a disorder that causes deterioration of nervous system functions (neurodegeneration) beginning in infancy. Newborns with D-bifunctional protein deficiency have weak muscle tone (hypotonia) and seizures. Most babies with this condition never acquire any developmental skills. Some may reach very early developmental milestones such as the ability to follow movement with their eyes or control their head movement, but they experience a gradual loss of these skills (developmental regression) within a few months. As the condition gets worse, affected children develop exaggerated reflexes (hyperreflexia), increased muscle tone (hypertonia), more severe and recurrent seizures (epilepsy), and loss of vision and hearing. Most children with D-bifunctional protein deficiency do not survive past the age of 2. A small number of individuals with this disorder are somewhat less severely affected. They may acquire additional basic skills, such as voluntary hand movements or unsupported sitting, before experiencing developmental regression, and they may survive longer into childhood than more severely affected individuals.
Individuals with D-bifunctional protein deficiency may have unusual facial features, including a high forehead, widely spaced eyes (hypertelorism), a lengthened area between the nose and mouth (philtrum), and a high arch of the hard palate at the roof of the mouth. Affected infants may also have an unusually large space between the bones of the skull (fontanelle). An enlarged liver (hepatomegaly) occurs in about half of affected individuals. Because these features are similar to those of another disorder called Zellweger syndrome (part of a group of disorders called the Zellweger spectrum), D-bifunctional protein deficiency is sometimes called pseudo-Zellweger syndrome.
[Learn More in MedlinePlus]
Code History
- FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
- FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
- FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
- FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
- FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
- FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
- FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
- FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
- FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.
Footnotes
[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:
- The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
- The condition places limitations on self-care, independent living, and social interactions.