2026 ICD-10-CM Diagnosis Code E20.0Idiopathic hypoparathyroidism

AHRQ’s CCSR groups this code into broader clinical categories.

• Hypoparathyroidism

  a condition caused by a deficiency of parathyroid hormone (or pth). it is characterized by hypocalcemia and hyperphosphatemia. hypocalcemia leads to tetany. the acquired form is due to removal or injuries to the parathyroid glands. the congenital form is due to mutations of genes, such as tbx1; (see digeorge syndrome); casr encoding calcium-sensing receptor; or pth encoding parathyroid hormone.

• Acquired Hypoparathyroidism

  hypoparathyroidism, the cause of which is not present at birth.

• 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.

• Autosomal Dominant Hypoparathyroidism

  hypoparathyroidism associated with heterozygous mutation(s) in the pth gene, which encodes parathyroid hormone, or in the gcm2 gene, which encodes chorion-specific transcription factor gcmb.

• Autosomal Recessive Hypoparathyroidism

  hypoparathyroidism associated with homozygous mutation(s) in the pth gene, which encodes parathyroid hormone, or in the gcm2 gene, which encodes chorion-specific transcription factor gcmb.

• Barakat Syndrome|Hypoparathyroidism, Deafness, and Renal Anomalies Syndrome

  a condition characterized by hypoparathyroidism, sensorineural deafness, and renal failure. it is related to autosomal dominant inactivating mutation(s) in gata3, encoding a transcription factor important for the embryonic development of the parathyroid gland, the auditory stem, and the kidneys.

• Grade 1 Hypoparathyroidism, CTCAE|Grade 1 Hypoparathyroidism

  asymptomatic; clinical or diagnostic observations only; intervention not indicated

• Grade 2 Hypoparathyroidism, CTCAE|Grade 2 Hypoparathyroidism

  moderate symptoms; medical intervention indicated

• Grade 3 Hypoparathyroidism, CTCAE|Grade 3 Hypoparathyroidism

  severe symptoms; medical intervention or hospitalization indicated

• Grade 4 Hypoparathyroidism, CTCAE|Grade 4 Hypoparathyroidism

  life-threatening consequences; urgent intervention indicated

• Grade 5 Hypoparathyroidism, CTCAE|Grade 5 Hypoparathyroidism

  death

• Hypoparathyroidism

  an endocrine disorder characterized by decreased production of parathyroid hormone by the parathyroid glands. it is usually caused by damage of the parathyroid glands during head and neck surgery. signs and symptoms include muscle cramps, abdominal pain, dry skin, brittle nails, cataracts, tetany, and convulsions.

• Hypoparathyroidism, CTCAE|Hypoparathyroidism|Hypoparathyroidism

  a disorder characterized by a decrease in production of parathyroid hormone by the parathyroid glands.

• Hypoparathyroidism-Retardation-Dysmorphism Syndrome|HRDS|Hypoparathyroidism with Short Stature, Mental Retardation, and Seizures|Hypoparathyroidism, Congenital, Associated with Dysmorphism, Growth Retardation, and Developmental Delay|Sanjad-Sakati Syndrome

  an autosomal recessive condition caused by mutation(s) in the tbce gene, encoding tubulin-specific chaperone e. it is characterized by congenital hypoparathyroidism, mental retardation, seizures and developmental delay.

• Iatrogenic Hypoparathyroidism

  hypoparathyroidism resulting from medical treatment or intervention.

• Parathyroid Hormone Resistance|Pseudohypoparathyroidism|Pseudoparathyroidism

  a finding indicating decreased tissue sensitivity to parathyroid hormone.

• Primary Hypoparathyroidism

  abnormally low levels of parathyroid hormone due to a disorder originating within the parathyroid glands.

• 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.

• TBCE wt Allele|HRD|Hypoparathyroidism, Growth and Mental Retardation, and Dysmorphism Gene|KCS|KCS1|Kenny-Caffey Syndrome Gene|PEAMO|Tubulin Folding Cofactor E wt Allele|pac2

  human tbce wild-type allele is located in the vicinity of 1q42.3 and is approximately 85 kb in length. this allele, which encodes tubulin-specific chaperone e protein, is involved in both tubulin folding and tubulin dimer dissociation. mutations in the gene are associated with progressive encephalopathy with amyotrophy and optic atrophy, hypoparathyroidism-retardation-dysmorphism syndrome and kenny-caffey syndrome 1.

• Transient Neonatal Hypoparathyroidism

  a disorder of decreased production of parathyroid hormone by the parathyroid gland in a newborn. it is due to maternal hyperparathyroidism. it may be characterized by hypocalcemic seizures in the first weeks of life.

• X-Linked Hypoparathyroidism|X-linked Hypoparathyroidism

  hypoparathyroidism in which the inheritance is recessive and linked to the q26-q27 region of the x chromosome. the parathyroid glands are usually incompletely developed (parathyroid dysgenesis) or absent (parathyroid agenesis).

Parathyroid Disorders

Most people have four pea-sized glands, called parathyroid glands, on the thyroid gland in the neck. Though their names are similar, the thyroid and parathyroid glands are completely different. The parathyroid glands make parathyroid hormone (PTH), which helps your body keep the right balance of calcium and phosphorous.

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