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Microcephaly-glomerulonephritis-marfanoid habitus syndrome

MedGen UID:
343327
Concept ID:
C1855348
Disease or Syndrome
Synonym: Marfanoid habitus with microcephaly and glomerulonephritis
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:0009565
OMIM®: 248760
Orphanet: ORPHA2172

Definition

A rare intellectual disability syndrome characterized by intellectual deficit, marfanoid habitus, microcephaly, and glomerulonephritis. There have been no further reports since 1992. [from ORDO]

Clinical features

From HPO
Renal insufficiency
MedGen UID:
332529
Concept ID:
C1565489
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
Arachnodactyly
MedGen UID:
2047
Concept ID:
C0003706
Congenital Abnormality
Abnormally long and slender fingers ("spider fingers").
Disproportionate tall stature
MedGen UID:
323048
Concept ID:
C1836996
Finding
A tall and slim body build with increased arm span to height ratio (>1.05) and a reduced upper-to-lower segment ratio (<0.85), i.e., unusually long arms and legs. The extremities as well as the hands and feet are unusually slim.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Subnormal intellectual functioning which originates during the developmental period. Intellectual disability, previously referred to as mental retardation, has been defined as an IQ score below 70.
Kyphosis
MedGen UID:
44042
Concept ID:
C0022821
Anatomical Abnormality
Exaggerated anterior convexity of the thoracic vertebral column.
Joint laxity
MedGen UID:
39439
Concept ID:
C0086437
Finding
Lack of stability of a joint.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Glomerulonephritis
MedGen UID:
6616
Concept ID:
C0017658
Disease or Syndrome
Inflammation of the renal glomeruli.
High palate
MedGen UID:
66814
Concept ID:
C0240635
Congenital Abnormality
Height of the palate more than 2 SD above the mean (objective) or palatal height at the level of the first permanent molar more than twice the height of the teeth (subjective).
Mandibular prognathia
MedGen UID:
98316
Concept ID:
C0399526
Finding
Abnormal prominence of the chin related to increased length of the mandible.
High, narrow palate
MedGen UID:
324787
Concept ID:
C1837404
Finding
The presence of a high and narrow palate.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMicrocephaly-glomerulonephritis-marfanoid habitus syndrome
Follow this link to review classifications for Microcephaly-glomerulonephritis-marfanoid habitus syndrome in Orphanet.

Professional guidelines

PubMed

Reed AR
J Med Humanit 2018 Dec;39(4):515-533. doi: 10.1007/s10912-016-9413-5. PMID: 27761686
Akpunonu BE, Mulrow PJ, Hoffman EA
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Recent clinical studies

Etiology

Rodríguez-Palmero A, Boerrigter MM, Gómez-Andrés D, Aldinger KA, Marcos-Alcalde Í, Popp B, Everman DB, Lovgren AK, Arpin S, Bahrambeigi V, Beunders G, Bisgaard AM, Bjerregaard VA, Bruel AL, Challman TD, Cogné B, Coubes C, de Man SA, Denommé-Pichon AS, Dye TJ, Elmslie F, Feuk L, García-Miñaúr S, Gertler T, Giorgio E, Gruchy N, Haack TB, Haldeman-Englert CR, Haukanes BI, Hoyer J, Hurst ACE, Isidor B, Soller MJ, Kushary S, Kvarnung M, Landau YE, Leppig KA, Lindstrand A, Kleinendorst L, MacKenzie A, Mandrile G, Mendelsohn BA, Moghadasi S, Morton JE, Moutton S, Müller AJ, O'Leary M, Pacio-Míguez M, Palomares-Bralo M, Parikh S, Pfundt R, Pode-Shakked B, Rauch A, Repnikova E, Revah-Politi A, Ross MJ, Ruivenkamp CAL, Sarrazin E, Savatt JM, Schlüter A, Schönewolf-Greulich B, Shad Z, Shaw-Smith C, Shieh JT, Shohat M, Spranger S, Thiese H, Mau-Them FT, van Bon B, van de Burgt I, van de Laar IMBH, van Drie E, van Haelst MM, van Ravenswaaij-Arts CM, Verdura E, Vitobello A, Waldmüller S, Whiting S, Zweier C, Prada CE, de Vries BBA, Dobyns WB, Reiter SF, Gómez-Puertas P, Pujol A, Tümer Z
Genet Med 2021 May;23(5):888-899. Epub 2021 Feb 17 doi: 10.1038/s41436-020-01075-9. PMID: 33597769
Kelly C, Geraghty RM, Somani BK
Curr Urol Rep 2019 May 18;20(7):36. doi: 10.1007/s11934-019-0898-0. PMID: 31104149
Coţofană M, Muşat F, Păduraru DN, Andronic O, Bolocan A, Ion D
Chirurgia (Bucur) 2019 Jan-Feb;114(1):12-17. doi: 10.21614/chirurgia.114.1.12. PMID: 30830840
Al-Salameh A, Baudry C, Cohen R
Presse Med 2018 Sep;47(9):722-731. Epub 2018 Jun 13 doi: 10.1016/j.lpm.2018.03.005. PMID: 29909163
Thakker RV
Mol Cell Endocrinol 2014 Apr 5;386(1-2):2-15. Epub 2013 Aug 8 doi: 10.1016/j.mce.2013.08.002. PMID: 23933118Free PMC Article

Diagnosis

Dumesic DA, Padmanabhan V, Chazenbalk GD, Abbott DH
Reprod Biol Endocrinol 2022 Jan 10;20(1):12. doi: 10.1186/s12958-021-00878-y. PMID: 35012577Free PMC Article
Rodríguez-Palmero A, Boerrigter MM, Gómez-Andrés D, Aldinger KA, Marcos-Alcalde Í, Popp B, Everman DB, Lovgren AK, Arpin S, Bahrambeigi V, Beunders G, Bisgaard AM, Bjerregaard VA, Bruel AL, Challman TD, Cogné B, Coubes C, de Man SA, Denommé-Pichon AS, Dye TJ, Elmslie F, Feuk L, García-Miñaúr S, Gertler T, Giorgio E, Gruchy N, Haack TB, Haldeman-Englert CR, Haukanes BI, Hoyer J, Hurst ACE, Isidor B, Soller MJ, Kushary S, Kvarnung M, Landau YE, Leppig KA, Lindstrand A, Kleinendorst L, MacKenzie A, Mandrile G, Mendelsohn BA, Moghadasi S, Morton JE, Moutton S, Müller AJ, O'Leary M, Pacio-Míguez M, Palomares-Bralo M, Parikh S, Pfundt R, Pode-Shakked B, Rauch A, Repnikova E, Revah-Politi A, Ross MJ, Ruivenkamp CAL, Sarrazin E, Savatt JM, Schlüter A, Schönewolf-Greulich B, Shad Z, Shaw-Smith C, Shieh JT, Shohat M, Spranger S, Thiese H, Mau-Them FT, van Bon B, van de Burgt I, van de Laar IMBH, van Drie E, van Haelst MM, van Ravenswaaij-Arts CM, Verdura E, Vitobello A, Waldmüller S, Whiting S, Zweier C, Prada CE, de Vries BBA, Dobyns WB, Reiter SF, Gómez-Puertas P, Pujol A, Tümer Z
Genet Med 2021 May;23(5):888-899. Epub 2021 Feb 17 doi: 10.1038/s41436-020-01075-9. PMID: 33597769
Santi M, Graf S, Zeino M, Cools M, Van De Vijver K, Trippel M, Aliu N, Flück CE
J Clin Endocrinol Metab 2021 Apr 23;106(5):1530-1539. doi: 10.1210/clinem/dgaa948. PMID: 33367768Free PMC Article
Al-Salameh A, Baudry C, Cohen R
Presse Med 2018 Sep;47(9):722-731. Epub 2018 Jun 13 doi: 10.1016/j.lpm.2018.03.005. PMID: 29909163
Thakker RV
Mol Cell Endocrinol 2014 Apr 5;386(1-2):2-15. Epub 2013 Aug 8 doi: 10.1016/j.mce.2013.08.002. PMID: 23933118Free PMC Article

Therapy

Lindberg E, Bonsignore MR, Polo-Kantola P
Sleep Med Rev 2020 Feb;49:101225. Epub 2019 Oct 31 doi: 10.1016/j.smrv.2019.101225. PMID: 31739179
Coţofană M, Muşat F, Păduraru DN, Andronic O, Bolocan A, Ion D
Chirurgia (Bucur) 2019 Jan-Feb;114(1):12-17. doi: 10.21614/chirurgia.114.1.12. PMID: 30830840
Stevens SM, O'Connell BP, Meyer TA
Curr Opin Otolaryngol Head Neck Surg 2015 Oct;23(5):341-7. doi: 10.1097/MOO.0000000000000194. PMID: 26339964
Choi SM, Kam SC
Korean J Urol 2015 Jan;56(1):12-8. Epub 2015 Jan 6 doi: 10.4111/kju.2015.56.1.12. PMID: 25598932Free PMC Article
Schwartz A
Orthop Nurs 2007 Jan-Feb;26(1):19-24; quiz 25-6. doi: 10.1097/00006416-200701000-00007. PMID: 17273103

Prognosis

Rodríguez-Palmero A, Boerrigter MM, Gómez-Andrés D, Aldinger KA, Marcos-Alcalde Í, Popp B, Everman DB, Lovgren AK, Arpin S, Bahrambeigi V, Beunders G, Bisgaard AM, Bjerregaard VA, Bruel AL, Challman TD, Cogné B, Coubes C, de Man SA, Denommé-Pichon AS, Dye TJ, Elmslie F, Feuk L, García-Miñaúr S, Gertler T, Giorgio E, Gruchy N, Haack TB, Haldeman-Englert CR, Haukanes BI, Hoyer J, Hurst ACE, Isidor B, Soller MJ, Kushary S, Kvarnung M, Landau YE, Leppig KA, Lindstrand A, Kleinendorst L, MacKenzie A, Mandrile G, Mendelsohn BA, Moghadasi S, Morton JE, Moutton S, Müller AJ, O'Leary M, Pacio-Míguez M, Palomares-Bralo M, Parikh S, Pfundt R, Pode-Shakked B, Rauch A, Repnikova E, Revah-Politi A, Ross MJ, Ruivenkamp CAL, Sarrazin E, Savatt JM, Schlüter A, Schönewolf-Greulich B, Shad Z, Shaw-Smith C, Shieh JT, Shohat M, Spranger S, Thiese H, Mau-Them FT, van Bon B, van de Burgt I, van de Laar IMBH, van Drie E, van Haelst MM, van Ravenswaaij-Arts CM, Verdura E, Vitobello A, Waldmüller S, Whiting S, Zweier C, Prada CE, de Vries BBA, Dobyns WB, Reiter SF, Gómez-Puertas P, Pujol A, Tümer Z
Genet Med 2021 May;23(5):888-899. Epub 2021 Feb 17 doi: 10.1038/s41436-020-01075-9. PMID: 33597769
Coţofană M, Muşat F, Păduraru DN, Andronic O, Bolocan A, Ion D
Chirurgia (Bucur) 2019 Jan-Feb;114(1):12-17. doi: 10.21614/chirurgia.114.1.12. PMID: 30830840
Al-Salameh A, Baudry C, Cohen R
Presse Med 2018 Sep;47(9):722-731. Epub 2018 Jun 13 doi: 10.1016/j.lpm.2018.03.005. PMID: 29909163
Recio Rodríguez M, Martínez de Vega V, Cano Alonso R, Carrascoso Arranz J, Martínez Ten P, Pérez Pedregosa J
Radiographics 2012 Nov-Dec;32(7):E305-21. doi: 10.1148/rg.327125053. PMID: 23150864
Van Buggenhout G, Fryns JP
Orphanet J Rare Dis 2006 Jul 10;1:26. doi: 10.1186/1750-1172-1-26. PMID: 16831221Free PMC Article

Clinical prediction guides

Wiel LC, Bruno I, Barbi E, Sirchia F
Ital J Pediatr 2022 May 12;48(1):72. doi: 10.1186/s13052-022-01267-w. PMID: 35550183Free PMC Article
Rodríguez-Palmero A, Boerrigter MM, Gómez-Andrés D, Aldinger KA, Marcos-Alcalde Í, Popp B, Everman DB, Lovgren AK, Arpin S, Bahrambeigi V, Beunders G, Bisgaard AM, Bjerregaard VA, Bruel AL, Challman TD, Cogné B, Coubes C, de Man SA, Denommé-Pichon AS, Dye TJ, Elmslie F, Feuk L, García-Miñaúr S, Gertler T, Giorgio E, Gruchy N, Haack TB, Haldeman-Englert CR, Haukanes BI, Hoyer J, Hurst ACE, Isidor B, Soller MJ, Kushary S, Kvarnung M, Landau YE, Leppig KA, Lindstrand A, Kleinendorst L, MacKenzie A, Mandrile G, Mendelsohn BA, Moghadasi S, Morton JE, Moutton S, Müller AJ, O'Leary M, Pacio-Míguez M, Palomares-Bralo M, Parikh S, Pfundt R, Pode-Shakked B, Rauch A, Repnikova E, Revah-Politi A, Ross MJ, Ruivenkamp CAL, Sarrazin E, Savatt JM, Schlüter A, Schönewolf-Greulich B, Shad Z, Shaw-Smith C, Shieh JT, Shohat M, Spranger S, Thiese H, Mau-Them FT, van Bon B, van de Burgt I, van de Laar IMBH, van Drie E, van Haelst MM, van Ravenswaaij-Arts CM, Verdura E, Vitobello A, Waldmüller S, Whiting S, Zweier C, Prada CE, de Vries BBA, Dobyns WB, Reiter SF, Gómez-Puertas P, Pujol A, Tümer Z
Genet Med 2021 May;23(5):888-899. Epub 2021 Feb 17 doi: 10.1038/s41436-020-01075-9. PMID: 33597769
Santi M, Graf S, Zeino M, Cools M, Van De Vijver K, Trippel M, Aliu N, Flück CE
J Clin Endocrinol Metab 2021 Apr 23;106(5):1530-1539. doi: 10.1210/clinem/dgaa948. PMID: 33367768Free PMC Article
Coţofană M, Muşat F, Păduraru DN, Andronic O, Bolocan A, Ion D
Chirurgia (Bucur) 2019 Jan-Feb;114(1):12-17. doi: 10.21614/chirurgia.114.1.12. PMID: 30830840
Al-Salameh A, Baudry C, Cohen R
Presse Med 2018 Sep;47(9):722-731. Epub 2018 Jun 13 doi: 10.1016/j.lpm.2018.03.005. PMID: 29909163

Supplemental Content

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