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Prominent glabella-microcephaly-hypogenitalism syndrome

MedGen UID:
162900
Concept ID:
C0796024
Disease or Syndrome
Synonyms: Mac Dermot Winter syndrome; Prominent glabella microcephaly hypogenitalism
SNOMED CT: Prominent glabella with microcephaly and hypogenitalism syndrome (716023007); MacDermot Winter syndrome (716023007)
 
Monarch Initiative: MONDO:0009543
OMIM®: 247990
Orphanet: ORPHA2083

Definition

A very rare syndrome described in two siblings with manifestation of prenatal onset of growth deficiency, microcephaly, hypoplastic genitalia, and birth onset of convulsions. [from SNOMEDCT_US]

Clinical features

From HPO
Hydronephrosis
MedGen UID:
42531
Concept ID:
C0020295
Disease or Syndrome
Severe distention of the kidney with dilation of the renal pelvis and calices.
Hypoplastic male external genitalia
MedGen UID:
338952
Concept ID:
C1852534
Finding
Underdevelopment of part or all of the male external reproductive organs (which include the penis, the scrotum and the urethra).
Fetal growth restriction
MedGen UID:
4693
Concept ID:
C0015934
Pathologic Function
An abnormal restriction of fetal growth with fetal weight below the tenth percentile for gestational age.
Macrotia
MedGen UID:
488785
Concept ID:
C0152421
Congenital Abnormality
Median longitudinal ear length greater than two standard deviations above the mean and median ear width greater than two standard deviations above the mean (objective); or, apparent increase in length and width of the pinna (subjective).
Posteriorly rotated ears
MedGen UID:
96566
Concept ID:
C0431478
Congenital Abnormality
A type of abnormal location of the ears in which the position of the ears is characterized by posterior rotation (the superior part of the ears is rotated towards the back of the head, and the inferior part of the ears towards the front).
Seizure
MedGen UID:
20693
Concept ID:
C0036572
Sign or Symptom
A seizure is an intermittent abnormality of nervous system physiology characterized by a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain.
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.
Ventriculomegaly
MedGen UID:
480553
Concept ID:
C3278923
Finding
An increase in size of the ventricular system of the brain.
Camptodactyly of finger
MedGen UID:
98041
Concept ID:
C0409348
Finding
The distal interphalangeal joint and/or the proximal interphalangeal joint of the fingers cannot be extended to 180 degrees by either active or passive extension.
Microcephaly
MedGen UID:
1644158
Concept ID:
C4551563
Finding
Head circumference below 2 standard deviations below the mean for age and gender.
Low anterior hairline
MedGen UID:
331280
Concept ID:
C1842366
Finding
Distance between the hairline (trichion) and the glabella (the most prominent point on the frontal bone above the root of the nose), in the midline, more than two SD below the mean. Alternatively, an apparently decreased distance between the hairline and the glabella.
Prominent glabella
MedGen UID:
349761
Concept ID:
C1860247
Finding
Forward protrusion of the glabella.
Highly arched eyebrow
MedGen UID:
358357
Concept ID:
C1868571
Finding
Increased height of the central portion of the eyebrow, forming a crescent, semicircular, or inverted U shape.
Frontal upsweep of hair
MedGen UID:
452910
Concept ID:
C1185616
Finding
Upward and/or sideward growth of anterior hair.
Wide intermamillary distance
MedGen UID:
473489
Concept ID:
C1827524
Finding
A larger than usual distance between the left and right nipple.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProminent glabella-microcephaly-hypogenitalism syndrome
Follow this link to review classifications for Prominent glabella-microcephaly-hypogenitalism syndrome in Orphanet.

Professional guidelines

PubMed

Shahid R, Iahtisham-Ul-Haq, Mahnoor, Awan KA, Iqbal MJ, Munir H, Saeed I
J Food Biochem 2022 Jul;46(7):e14117. Epub 2022 Feb 24 doi: 10.1111/jfbc.14117. PMID: 35199348
Afinogenova Y, Danve A, Neogi T
Curr Opin Rheumatol 2022 Mar 1;34(2):118-124. doi: 10.1097/BOR.0000000000000861. PMID: 34907116Free PMC Article
Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU
Dtsch Arztebl Int 2016 Feb 5;113(5):61-9. doi: 10.3238/arztebl.2016.0061. PMID: 26900156Free PMC Article

Recent clinical studies

Etiology

Bastiaansen AEM, van Steenhoven RW, de Bruijn MAAM, Crijnen YS, van Sonderen A, van Coevorden-Hameete MH, Nühn MM, Verbeek MM, Schreurs MWJ, Sillevis Smitt PAE, de Vries JM, Jan de Jong F, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2021 Sep;8(5) Epub 2021 Aug 2 doi: 10.1212/NXI.0000000000001039. PMID: 34341093Free PMC Article
Marušić Z, Korša L, Calonje E
Clin Dermatol 2021 Mar-Apr;39(2):291-294. Epub 2020 Oct 16 doi: 10.1016/j.clindermatol.2020.10.008. PMID: 34272024
Dinarvand P, Davaro EP, Doan JV, Ising ME, Evans NR, Phillips NJ, Lai J, Guzman MA
Arch Pathol Lab Med 2019 Nov;143(11):1382-1398. Epub 2019 May 9 doi: 10.5858/arpa.2018-0570-RA. PMID: 31070935
Libby P, Pasterkamp G, Crea F, Jang IK
Circ Res 2019 Jan 4;124(1):150-160. doi: 10.1161/CIRCRESAHA.118.311098. PMID: 30605419Free PMC Article
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923

Diagnosis

Peng KP, Wang SJ
Curr Pain Headache Rep 2022 Jan;26(1):79-84. Epub 2022 Jan 25 doi: 10.1007/s11916-022-01005-1. PMID: 35076874Free PMC Article
Bastiaansen AEM, van Steenhoven RW, de Bruijn MAAM, Crijnen YS, van Sonderen A, van Coevorden-Hameete MH, Nühn MM, Verbeek MM, Schreurs MWJ, Sillevis Smitt PAE, de Vries JM, Jan de Jong F, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2021 Sep;8(5) Epub 2021 Aug 2 doi: 10.1212/NXI.0000000000001039. PMID: 34341093Free PMC Article
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Schmahmann JD, Sherman JC
Brain 1998 Apr;121 ( Pt 4):561-79. doi: 10.1093/brain/121.4.561. PMID: 9577385

Therapy

Tucker B, Goonetilleke N, Patel S, Keech A
Heart 2024 Apr 15;110(9):618-625. doi: 10.1136/heartjnl-2023-323177. PMID: 38331560
Peng KP, Wang SJ
Curr Pain Headache Rep 2022 Jan;26(1):79-84. Epub 2022 Jan 25 doi: 10.1007/s11916-022-01005-1. PMID: 35076874Free PMC Article
Schroeck JL, Ford J, Conway EL, Kurtzhalts KE, Gee ME, Vollmer KA, Mergenhagen KA
Clin Ther 2016 Nov;38(11):2340-2372. Epub 2016 Oct 15 doi: 10.1016/j.clinthera.2016.09.010. PMID: 27751669
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340
Bellomo R, Warrillow SJ, Reade MC
Crit Care Med 2009 Dec;37(12):3114-9. doi: 10.1097/CCM.0b013e3181bc7bd5. PMID: 19789447

Prognosis

Bastiaansen AEM, van Steenhoven RW, de Bruijn MAAM, Crijnen YS, van Sonderen A, van Coevorden-Hameete MH, Nühn MM, Verbeek MM, Schreurs MWJ, Sillevis Smitt PAE, de Vries JM, Jan de Jong F, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2021 Sep;8(5) Epub 2021 Aug 2 doi: 10.1212/NXI.0000000000001039. PMID: 34341093Free PMC Article
Yates TM, Drucker M, Barnicoat A, Low K, Gerkes EH, Fry AE, Parker MJ, O'Driscoll M, Charles P, Cox H, Marey I, Keren B, Rinne T, McEntagart M, Ramachandran V, Drury S, Vansenne F, Sival DA, Herkert JC, Callewaert B, Tan WH, Balasubramanian M
Hum Mutat 2020 May;41(5):1042-1050. Epub 2020 Mar 5 doi: 10.1002/humu.24001. PMID: 32097528
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Asmus F, Gasser T
Eur J Neurol 2010 Jul;17 Suppl 1:37-45. doi: 10.1111/j.1468-1331.2010.03049.x. PMID: 20590807

Clinical prediction guides

Bastiaansen AEM, van Steenhoven RW, de Bruijn MAAM, Crijnen YS, van Sonderen A, van Coevorden-Hameete MH, Nühn MM, Verbeek MM, Schreurs MWJ, Sillevis Smitt PAE, de Vries JM, Jan de Jong F, Titulaer MJ
Neurol Neuroimmunol Neuroinflamm 2021 Sep;8(5) Epub 2021 Aug 2 doi: 10.1212/NXI.0000000000001039. PMID: 34341093Free PMC Article
Yates TM, Drucker M, Barnicoat A, Low K, Gerkes EH, Fry AE, Parker MJ, O'Driscoll M, Charles P, Cox H, Marey I, Keren B, Rinne T, McEntagart M, Ramachandran V, Drury S, Vansenne F, Sival DA, Herkert JC, Callewaert B, Tan WH, Balasubramanian M
Hum Mutat 2020 May;41(5):1042-1050. Epub 2020 Mar 5 doi: 10.1002/humu.24001. PMID: 32097528
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Fabre A, Martinez-Vinson C, Goulet O, Badens C
Orphanet J Rare Dis 2013 Jan 9;8:5. doi: 10.1186/1750-1172-8-5. PMID: 23302111Free PMC Article
Garavelli L, Mainardi PC
Orphanet J Rare Dis 2007 Oct 24;2:42. doi: 10.1186/1750-1172-2-42. PMID: 17958891Free PMC Article

Recent systematic reviews

Zhang T, Zhang C, Zhang J, Sun F, Duan L
Front Cell Infect Microbiol 2022;12:859967. Epub 2022 Apr 1 doi: 10.3389/fcimb.2022.859967. PMID: 35433498Free PMC Article
Kim SJ, Lee HY
J Korean Med Sci 2020 Aug 3;35(30):e245. doi: 10.3346/jkms.2020.35.e245. PMID: 32743989Free PMC Article
Krause M, Zhu Y, Huhn M, Schneider-Thoma J, Bighelli I, Nikolakopoulou A, Leucht S
Eur Arch Psychiatry Clin Neurosci 2018 Oct;268(7):625-639. Epub 2018 Jan 24 doi: 10.1007/s00406-018-0869-3. PMID: 29368205
Bartsch E, Medcalf KE, Park AL, Ray JG; High Risk of Pre-eclampsia Identification Group
BMJ 2016 Apr 19;353:i1753. doi: 10.1136/bmj.i1753. PMID: 27094586Free PMC Article
Canavan TN, Mathes EF, Frieden I, Shinkai K
J Am Acad Dermatol 2015 Feb;72(2):239-45. doi: 10.1016/j.jaad.2014.06.026. PMID: 25592340

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