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Autosomal recessive infantile hypercalcemia

MedGen UID:
1380840
Concept ID:
C4329374
Disease or Syndrome
Synonym: Hypercalcemia, infantile
SNOMED CT: Autosomal recessive infantile hypercalcemia (771445001); Familial infantile hypercalcemia with suppressed intact parathyroid hormone (771445001)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0000212
OMIM® Phenotypic series: PS143880
Orphanet: ORPHA300547

Definition

Idiopathic infantile hypercalcemia is a condition characterized by high levels of calcium in the blood (hypercalcemia). Two types of idiopathic infantile hypercalcemia have been identified and are distinguished by their genetic causes: infantile hypercalcemia 1 and infantile hypercalcemia 2. In infants with either type, hypercalcemia can cause vomiting, increased urine production (polyuria), dehydration, constipation, poor feeding, weight loss, and an inability to grow and gain weight as expected (failure to thrive). As they age, affected babies usually have delayed development of mental and movement abilities (psychomotor delay). Individuals with infantile hypercalcemia 1 or 2 may also have high levels of calcium in their urine (hypercalciuria) and deposits of calcium in their kidneys (nephrocalcinosis).

With treatment, the outward symptoms of hypercalcemia, such as vomiting, dehydration, failure to thrive, and psychomotor delay, usually improve in childhood. However, affected children still tend to have higher-than-normal amounts of calcium in their blood and urine and calcium deposits in their kidneys. By adulthood, long-term hypercalcemia and hypercalciuria can lead to the formation of kidney stones (nephrolithiasis) and may damage the kidneys and impair their function. Affected adults may also develop calcium deposits in the joints or in the clear outer covering of the eye (the cornea), and some have low bone mineral density (osteoporosis).

In rare cases, affected individuals do not have symptoms of hypercalcemia in infancy, and the condition begins in later childhood or adulthood. These individuals usually develop hypercalciuria, nephrocalcinosis, and nephrolithiasis, although the features may not cause any obvious health problems.

Although most signs and symptoms are similar between the two known types of idiopathic infantile hypercalcemia, individuals with infantile hypercalcemia 2 have low levels of a mineral called phosphate in the blood (hypophosphatemia), while phosphate levels are typically normal in people with infantile hypercalcemia 1. [from MedlinePlus Genetics]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAutosomal recessive infantile hypercalcemia

Professional guidelines

PubMed

Sobacchi C, Schulz A, Coxon FP, Villa A, Helfrich MH
Nat Rev Endocrinol 2013 Sep;9(9):522-36. Epub 2013 Jul 23 doi: 10.1038/nrendo.2013.137. PMID: 23877423

Recent clinical studies

Etiology

Shadur B, Zaidman I, NaserEddin A, Lokshin E, Hussein F, Oron HC, Avni B, Grisariu S, Stepensky P
Pediatr Blood Cancer 2018 Jun;65(6):e27010. Epub 2018 Feb 22 doi: 10.1002/pbc.27010. PMID: 29469225
Schlingmann KP, Kaufmann M, Weber S, Irwin A, Goos C, John U, Misselwitz J, Klaus G, Kuwertz-Bröking E, Fehrenbach H, Wingen AM, Güran T, Hoenderop JG, Bindels RJ, Prosser DE, Jones G, Konrad M
N Engl J Med 2011 Aug 4;365(5):410-21. Epub 2011 Jun 15 doi: 10.1056/NEJMoa1103864. PMID: 21675912

Diagnosis

Lefèvre CR, Peltier L, Lokchine A, Ryckewaert A, Moreau C
Ann Biol Clin (Paris) 2022 Sep 1;80(5):460-463. doi: 10.1684/abc.2022.1747. PMID: 36453734
Granhøj J, Tougaard B, Lildballe DL, Rasmussen M
Nephron 2022;146(1):49-57. Epub 2021 Aug 30 doi: 10.1159/000518175. PMID: 34515170
Schlingmann KP, Ruminska J, Kaufmann M, Dursun I, Patti M, Kranz B, Pronicka E, Ciara E, Akcay T, Bulus D, Cornelissen EA, Gawlik A, Sikora P, Patzer L, Galiano M, Boyadzhiev V, Dumic M, Vivante A, Kleta R, Dekel B, Levtchenko E, Bindels RJ, Rust S, Forster IC, Hernando N, Jones G, Wagner CA, Konrad M
J Am Soc Nephrol 2016 Feb;27(2):604-14. Epub 2015 Jun 5 doi: 10.1681/ASN.2014101025. PMID: 26047794Free PMC Article
Sobacchi C, Schulz A, Coxon FP, Villa A, Helfrich MH
Nat Rev Endocrinol 2013 Sep;9(9):522-36. Epub 2013 Jul 23 doi: 10.1038/nrendo.2013.137. PMID: 23877423
Schlingmann KP, Kaufmann M, Weber S, Irwin A, Goos C, John U, Misselwitz J, Klaus G, Kuwertz-Bröking E, Fehrenbach H, Wingen AM, Güran T, Hoenderop JG, Bindels RJ, Prosser DE, Jones G, Konrad M
N Engl J Med 2011 Aug 4;365(5):410-21. Epub 2011 Jun 15 doi: 10.1056/NEJMoa1103864. PMID: 21675912

Therapy

Molin A, Nowoczyn M, Coudray N, Ballandone C, Abéguilé G, Mittre H, Richard N, Eckart P, Castanet M, Kottler ML
Eur J Med Genet 2019 Nov;62(11):103577. Epub 2018 Nov 10 doi: 10.1016/j.ejmg.2018.11.011. PMID: 30423445
Shadur B, Zaidman I, NaserEddin A, Lokshin E, Hussein F, Oron HC, Avni B, Grisariu S, Stepensky P
Pediatr Blood Cancer 2018 Jun;65(6):e27010. Epub 2018 Feb 22 doi: 10.1002/pbc.27010. PMID: 29469225
Schlingmann KP, Kaufmann M, Weber S, Irwin A, Goos C, John U, Misselwitz J, Klaus G, Kuwertz-Bröking E, Fehrenbach H, Wingen AM, Güran T, Hoenderop JG, Bindels RJ, Prosser DE, Jones G, Konrad M
N Engl J Med 2011 Aug 4;365(5):410-21. Epub 2011 Jun 15 doi: 10.1056/NEJMoa1103864. PMID: 21675912
McTaggart SJ, Craig J, MacMillan J, Burke JR
Pediatr Nephrol 1999 Oct;13(8):668-71. doi: 10.1007/s004670050678. PMID: 10502124

Prognosis

Lefèvre CR, Peltier L, Lokchine A, Ryckewaert A, Moreau C
Ann Biol Clin (Paris) 2022 Sep 1;80(5):460-463. doi: 10.1684/abc.2022.1747. PMID: 36453734
Shadur B, Zaidman I, NaserEddin A, Lokshin E, Hussein F, Oron HC, Avni B, Grisariu S, Stepensky P
Pediatr Blood Cancer 2018 Jun;65(6):e27010. Epub 2018 Feb 22 doi: 10.1002/pbc.27010. PMID: 29469225

Clinical prediction guides

Schlingmann KP, Ruminska J, Kaufmann M, Dursun I, Patti M, Kranz B, Pronicka E, Ciara E, Akcay T, Bulus D, Cornelissen EA, Gawlik A, Sikora P, Patzer L, Galiano M, Boyadzhiev V, Dumic M, Vivante A, Kleta R, Dekel B, Levtchenko E, Bindels RJ, Rust S, Forster IC, Hernando N, Jones G, Wagner CA, Konrad M
J Am Soc Nephrol 2016 Feb;27(2):604-14. Epub 2015 Jun 5 doi: 10.1681/ASN.2014101025. PMID: 26047794Free PMC Article

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