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Seckel syndrome 8(SCKL8)

MedGen UID:
856014
Concept ID:
C3891452
Disease or Syndrome
Synonym: SCKL8
 
Gene (location): DNA2 (10q21.3)
 
Monarch Initiative: MONDO:0014350
OMIM®: 615807

Definition

Seckel syndrome-8 (SCKL8) is characterized by severe microcephaly and markedly reduced height. Some patients have bird-like facies and exhibit developmental delays (Shaheen et al., 2014; Tarnauskaite et al., 2019). For a discussion of genetic heterogeneity of Seckel syndrome, see SCKL1 (210600). [from OMIM]

Clinical features

From HPO
Ectopic kidney
MedGen UID:
68661
Concept ID:
C0238207
Congenital Abnormality
A developmental defect in which a kidney is located in an abnormal anatomic position.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Spinal cord compression
MedGen UID:
11549
Concept ID:
C0037926
Disease or Syndrome
External mechanical compression of the spinal cord.
Global developmental delay
MedGen UID:
107838
Concept ID:
C0557874
Finding
A delay in the achievement of motor or mental milestones in the domains of development of a child, including motor skills, speech and language, cognitive skills, and social and emotional skills. This term should only be used to describe children younger than five years of age.
Intellectual disability
MedGen UID:
811461
Concept ID:
C3714756
Mental or Behavioral Dysfunction
Intellectual disability, previously referred to as mental retardation, is characterized by subnormal intellectual functioning that occurs during the developmental period. It is defined by an IQ score below 70.
Micrognathia
MedGen UID:
44428
Concept ID:
C0025990
Congenital Abnormality
Developmental hypoplasia of the mandible.
Kyphoscoliosis
MedGen UID:
154361
Concept ID:
C0575158
Anatomical Abnormality
An abnormal curvature of the spine in both a coronal (lateral) and sagittal (back-to-front) plane.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Convex nasal ridge
MedGen UID:
66809
Concept ID:
C0240538
Finding
Nasal ridge curving anteriorly to an imaginary line that connects the nasal root and tip. The nose appears often also prominent, and the columella low.

Professional guidelines

PubMed

Fan E, Del Sorbo L, Goligher EC, Hodgson CL, Munshi L, Walkey AJ, Adhikari NKJ, Amato MBP, Branson R, Brower RG, Ferguson ND, Gajic O, Gattinoni L, Hess D, Mancebo J, Meade MO, McAuley DF, Pesenti A, Ranieri VM, Rubenfeld GD, Rubin E, Seckel M, Slutsky AS, Talmor D, Thompson BT, Wunsch H, Uleryk E, Brozek J, Brochard LJ; American Thoracic Society, European Society of Intensive Care Medicine, and Society of Critical Care Medicine
Am J Respir Crit Care Med 2017 May 1;195(9):1253-1263. doi: 10.1164/rccm.201703-0548ST. PMID: 28459336
Faienza MF, Acquafredda A, D'Aniello M, Soldano L, Marzano F, Ventura A, Cavallo L
J Pediatr Endocrinol Metab 2013;26(7-8):771-4. doi: 10.1515/jpem-2012-0397. PMID: 23612698

Recent clinical studies

Etiology

Nerakh G, Vineeth VS, Tallapaka K, Nair L, Dalal A, Aggarwal S
Am J Med Genet A 2022 Jul;188(7):2139-2146. Epub 2022 Mar 17 doi: 10.1002/ajmg.a.62725. PMID: 35298084
Monteiro A, Cortez GM, Granja MF, Agnoletto GJ, Kranich J, Padilha MVR, Aldana P, Hanel R
J Neurointerv Surg 2021 Feb;13(2):171-176. Epub 2020 Jun 10 doi: 10.1136/neurintsurg-2020-016069. PMID: 32522788
Willems M, Geneviève D, Borck G, Baumann C, Baujat G, Bieth E, Edery P, Farra C, Gerard M, Héron D, Leheup B, Le Merrer M, Lyonnet S, Martin-Coignard D, Mathieu M, Thauvin-Robinet C, Verloes A, Colleaux L, Munnich A, Cormier-Daire V
J Med Genet 2010 Dec;47(12):797-802. Epub 2009 Jul 29 doi: 10.1136/jmg.2009.067298. PMID: 19643772
Ducos B, Cabrol S, Houang M, Perin L, Holzenberger M, Le Bouc Y
J Clin Endocrinol Metab 2001 Nov;86(11):5516-24. doi: 10.1210/jcem.86.11.7985. PMID: 11701730
Hayani A, Suarez CR, Molnar Z, LeBeau M, Godwin J
J Med Genet 1994 Feb;31(2):148-9. doi: 10.1136/jmg.31.2.148. PMID: 8182723Free PMC Article

Diagnosis

Khojah O, Alamoudi S, Aldawsari N, Babgi M, Lary A
Childs Nerv Syst 2021 Dec;37(12):3847-3860. Epub 2021 Aug 3 doi: 10.1007/s00381-021-05284-8. PMID: 34345934Free PMC Article
Marakhonov AV, Konovalov FA, Makaov AK, Vasilyeva TA, Kadyshev VV, Galkina VA, Dadali EL, Kutsev SI, Zinchenko RA
BMC Med Genomics 2018 Feb 13;11(Suppl 1):8. doi: 10.1186/s12920-018-0326-1. PMID: 29504900Free PMC Article
Willems M, Geneviève D, Borck G, Baumann C, Baujat G, Bieth E, Edery P, Farra C, Gerard M, Héron D, Leheup B, Le Merrer M, Lyonnet S, Martin-Coignard D, Mathieu M, Thauvin-Robinet C, Verloes A, Colleaux L, Munnich A, Cormier-Daire V
J Med Genet 2010 Dec;47(12):797-802. Epub 2009 Jul 29 doi: 10.1136/jmg.2009.067298. PMID: 19643772
Thapa R, Mukherjee K
J Child Neurol 2010 Feb;25(2):231-3. Epub 2009 Apr 15 doi: 10.1177/0883073809332694. PMID: 19372093
Adiyaman P, Berberoğlu M, Aycan Z, Evliyaoğlu O, Ocal G
J Pediatr Endocrinol Metab 2004 Jan;17(1):105-10. doi: 10.1515/jpem.2004.17.1.105. PMID: 14960029

Therapy

Stiff T, Casar Tena T, O'Driscoll M, Jeggo PA, Philipp M
Hum Mol Genet 2016 Apr 15;25(8):1574-87. Epub 2016 Feb 11 doi: 10.1093/hmg/ddw034. PMID: 26908596Free PMC Article
Faienza MF, Acquafredda A, D'Aniello M, Soldano L, Marzano F, Ventura A, Cavallo L
J Pediatr Endocrinol Metab 2013;26(7-8):771-4. doi: 10.1515/jpem-2012-0397. PMID: 23612698
Ducos B, Cabrol S, Houang M, Perin L, Holzenberger M, Le Bouc Y
J Clin Endocrinol Metab 2001 Nov;86(11):5516-24. doi: 10.1210/jcem.86.11.7985. PMID: 11701730
Hayani A, Suarez CR, Molnar Z, LeBeau M, Godwin J
J Med Genet 1994 Feb;31(2):148-9. doi: 10.1136/jmg.31.2.148. PMID: 8182723Free PMC Article

Prognosis

Marakhonov AV, Konovalov FA, Makaov AK, Vasilyeva TA, Kadyshev VV, Galkina VA, Dadali EL, Kutsev SI, Zinchenko RA
BMC Med Genomics 2018 Feb 13;11(Suppl 1):8. doi: 10.1186/s12920-018-0326-1. PMID: 29504900Free PMC Article
Fan E, Del Sorbo L, Goligher EC, Hodgson CL, Munshi L, Walkey AJ, Adhikari NKJ, Amato MBP, Branson R, Brower RG, Ferguson ND, Gajic O, Gattinoni L, Hess D, Mancebo J, Meade MO, McAuley DF, Pesenti A, Ranieri VM, Rubenfeld GD, Rubin E, Seckel M, Slutsky AS, Talmor D, Thompson BT, Wunsch H, Uleryk E, Brozek J, Brochard LJ; American Thoracic Society, European Society of Intensive Care Medicine, and Society of Critical Care Medicine
Am J Respir Crit Care Med 2017 May 1;195(9):1253-1263. doi: 10.1164/rccm.201703-0548ST. PMID: 28459336
Willems M, Geneviève D, Borck G, Baumann C, Baujat G, Bieth E, Edery P, Farra C, Gerard M, Héron D, Leheup B, Le Merrer M, Lyonnet S, Martin-Coignard D, Mathieu M, Thauvin-Robinet C, Verloes A, Colleaux L, Munnich A, Cormier-Daire V
J Med Genet 2010 Dec;47(12):797-802. Epub 2009 Jul 29 doi: 10.1136/jmg.2009.067298. PMID: 19643772

Clinical prediction guides

Yadav N, Kirola L, Geetha TS, Mittal K, Kadandale J, Yogev Y, Birk OS, Gupta N, Balakrishnan P, Jana M, Gupta M, Kabra M, Thelma BK
Ann Hum Genet 2022 Sep;86(5):245-256. Epub 2022 Apr 22 doi: 10.1111/ahg.12469. PMID: 35451063
Fan E, Del Sorbo L, Goligher EC, Hodgson CL, Munshi L, Walkey AJ, Adhikari NKJ, Amato MBP, Branson R, Brower RG, Ferguson ND, Gajic O, Gattinoni L, Hess D, Mancebo J, Meade MO, McAuley DF, Pesenti A, Ranieri VM, Rubenfeld GD, Rubin E, Seckel M, Slutsky AS, Talmor D, Thompson BT, Wunsch H, Uleryk E, Brozek J, Brochard LJ; American Thoracic Society, European Society of Intensive Care Medicine, and Society of Critical Care Medicine
Am J Respir Crit Care Med 2017 May 1;195(9):1253-1263. doi: 10.1164/rccm.201703-0548ST. PMID: 28459336
Ogi T, Walker S, Stiff T, Hobson E, Limsirichaikul S, Carpenter G, Prescott K, Suri M, Byrd PJ, Matsuse M, Mitsutake N, Nakazawa Y, Vasudevan P, Barrow M, Stewart GS, Taylor AM, O'Driscoll M, Jeggo PA
PLoS Genet 2012;8(11):e1002945. Epub 2012 Nov 8 doi: 10.1371/journal.pgen.1002945. PMID: 23144622Free PMC Article
Alderton GK, Galbiati L, Griffith E, Surinya KH, Neitzel H, Jackson AP, Jeggo PA, O'Driscoll M
Nat Cell Biol 2006 Jul;8(7):725-33. Epub 2006 Jun 18 doi: 10.1038/ncb1431. PMID: 16783362
Kjaer I, Hansen N, Becktor KB, Birkebaek N, Balslev T
Cleft Palate Craniofac J 2001 Nov;38(6):645-51. doi: 10.1597/1545-1569_2001_038_0645_cmdasm_2.0.co_2. PMID: 11681999

Recent systematic reviews

Khojah O, Alamoudi S, Aldawsari N, Babgi M, Lary A
Childs Nerv Syst 2021 Dec;37(12):3847-3860. Epub 2021 Aug 3 doi: 10.1007/s00381-021-05284-8. PMID: 34345934Free PMC Article
Monteiro A, Cortez GM, Granja MF, Agnoletto GJ, Kranich J, Padilha MVR, Aldana P, Hanel R
J Neurointerv Surg 2021 Feb;13(2):171-176. Epub 2020 Jun 10 doi: 10.1136/neurintsurg-2020-016069. PMID: 32522788

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