E20.1 - Pseudohypoparathyroidism

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Cataract due to pseudohypoparathyroidism
• Pseudohypoparathyroidism
• Pseudohypoparathyroidism and pseudopseudohypoparathyroidism
• Pseudohypoparathyroidism type 1C
• Pseudohypoparathyroidism type I A
• Pseudohypoparathyroidism type I B
• Pseudohypoparathyroidism type II
• Pseudopseudohypoparathyroidism

Clinical Information

• Pseudohypoparathyroidism-. a hereditary syndrome clinically similar to hypoparathyroidism. it is characterized by hypocalcemia; hyperphosphatemia; and associated skeletal development impairment and caused by failure of response to parathyroid hormone rather than deficiencies. a severe form with resistance to multiple hormones is referred to as type 1a and is associated with maternal mutant allele of the alpha chain of stimulatory g protein.
• Pseudopseudohypoparathyroidism-. a form of pseudohypoparathyroidism characterized by the same features except for the abnormal response to hormones such as parathyroid hormone. it is associated with paternally inherited mutant alleles of the alpha chain of stimulatory g protein.
• Albright Hereditary Osteodystrophy with Multiple Hormone Resistance|PHP1a|Pseudohypoparathyroidism Type 1a-. parathyroid hormone (pth) resistance caused by heterozygous inactivating mutation(s) of the maternal allele of the gnas gene encoding gs-alpha, resulting in expression of pth from only the paternal allele. clinical manifestations include albright hereditary osteodystrophy, early-onset obesity, and, in some cases, resistance to thyroid-stimulating hormone, gonadotropins, and growth hormone-releasing hormone, reflecting additional manifestations of gs-alpha deficiency.
• Albright Hereditary Osteodystrophy without Multiple Hormone Resistance|Albright Hereditary Osteodystrophy with Multiple Hormone Resistance|PPHP|Pseudopseudohypoparathyroidism-. a condition caused by inactivating mutation(s) in the paternal allele of the gnas gene, encoding gs-alpha, resulting in expression of the gs-alpha protein from only the maternal allele. affected individuals have the clinical phenotype of albright hereditary osteodystrophy without hormone resistance.
• Albright's Hereditary Osteodystrophy|Albright Hereditary Osteodystrophy|Albright's Hereditary Osteodystrophy with Multiple Hormone Resistance|PHP1A|Pseudohypoparathyroidism, Type IA-. a rare, autosomal dominant syndrome caused by mutations in the gnas gene. it is characterized by the presence of short stature, obesity, round face, brachydactyly, subcutaneous ossifications, and pseudohypoparathtyroidism.
• Parathyroid Hormone Resistance|Pseudohypoparathyroidism|Pseudoparathyroidism-. a finding indicating decreased tissue sensitivity to parathyroid hormone.
• Pseudohypoparathyroidism-. a condition characterized by the insensitivity of the tissues to respond to the activity of the parathyroid hormone. it results in increased levels of parathyroid hormone in the serum, hypocalcemia, and hyperphosphatemia.
• Renal Parathyroid Hormone Resistance|Pseudohypoparathyroidism Type 1b-. parathyroid hormone resistance caused by defects in methylation in the gnas gene that cause loss of expression of gs-alpha from the maternal allele in renal tissue, resulting in decreased phosphate excretion and increased calcium excretion. individuals with this condition may also have brachydactyly and partial resistance to thyroid-stimulating hormone.
• Secondary Parathyroid Hormone Resistance|Acquired Parathyroid Hormone Resistance|Acquired Parathyroid Hormone Resistance|Pseudohypoparathyroidism, Type 2|Pseudohypoparathyroidism, Type 2-. parathyroid hormone (pth) resistance caused by vitamin d deficiency and characterized by clinically increased pth concentrations with relative hyperphosphatemia. the diagnosis can be confirmed by finding of a normal cyclic adenosine monophosphate (camp) response to pth infusion, but deficient phosphaturic response, indicating a defect distal to camp generation in renal cells.

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC). applicable from 10/01/2022 through 09/30/2023.

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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.

Code History

• 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 (First year ICD-10-CM implemented into the HIPAA code set)