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Stuve-Wiedemann syndrome 2(STWS2)

MedGen UID:
1805977
Concept ID:
C5676919
Disease or Syndrome
Synonym: Stüve-Wiedemann syndrome 2
 
Gene (location): IL6ST (5q11.2)
 
Monarch Initiative: MONDO:0030756
OMIM®: 619751

Definition

Stuve-Wiedemann syndrome-2 (STWS2) is an autosomal recessive lethal skeletal dysplasia characterized by short stature, small chest, bowing of the long bones, and neonatal cardiopulmonary and autonomous dysfunction. Additional variable features include congenital thrombocytopenia, eczematoid dermatitis, renal anomalies, and defective acute-phase response (Chen et al., 2020). For a general phenotypic description and discussion of genetic heterogeneity of Stuve-Wiedemann syndrome, see STWS1 (601559). [from OMIM]

Clinical features

From HPO
Congestive heart failure
MedGen UID:
9169
Concept ID:
C0018802
Disease or Syndrome
The presence of an abnormality of cardiac function that is responsible for the failure of the heart to pump blood at a rate that is commensurate with the needs of the tissues or a state in which abnormally elevated filling pressures are required for the heart to do so. Heart failure is frequently related to a defect in myocardial contraction.
Pulmonary arterial hypertension
MedGen UID:
425404
Concept ID:
C2973725
Disease or Syndrome
Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.
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.
Dysphagia
MedGen UID:
41440
Concept ID:
C0011168
Disease or Syndrome
Difficulty in swallowing.
Motor delay
MedGen UID:
381392
Concept ID:
C1854301
Finding
A type of Developmental delay characterized by a delay in acquiring motor skills.
Disseminated intravascular coagulation
MedGen UID:
41620
Concept ID:
C0012739
Disease or Syndrome
Disseminated intravascular coagulation is characterized by the widespread activation of coagulation, which results in the intravascular formation of fibrin and ultimately thrombotic occlusion of small and midsize vessels.
Thrombocytopenia
MedGen UID:
52737
Concept ID:
C0040034
Disease or Syndrome
A reduction in the number of circulating thrombocytes.
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.
Camptodactyly
MedGen UID:
195780
Concept ID:
C0685409
Congenital Abnormality
The distal interphalangeal joint and/or the proximal interphalangeal joint of the fingers or toes cannot be extended to 180 degrees by either active or passive extension.
Thoracic hypoplasia
MedGen UID:
373339
Concept ID:
C1837482
Congenital Abnormality
Enlarged cisterna magna
MedGen UID:
344031
Concept ID:
C1853377
Finding
Increase in size of the cisterna magna, one of three principal openings in the subarachnoid space between the arachnoid and pia mater, located between the cerebellum and the dorsal surface of the medulla oblongata.
Short long bone
MedGen UID:
344385
Concept ID:
C1854912
Finding
One or more abnormally short long bone.
Bowing of the long bones
MedGen UID:
340849
Concept ID:
C1855340
Congenital Abnormality
A bending or abnormal curvature of a long bone.
Respiratory distress
MedGen UID:
96907
Concept ID:
C0476273
Sign or Symptom
Respiratory distress is objectively observable as the physical or emotional consequences from the experience of dyspnea. The physical presentation of respiratory distress is generally referred to as labored breathing, while the sensation of respiratory distress is called shortness of breath or dyspnea.
Eczematoid dermatitis
MedGen UID:
3968
Concept ID:
C0013595
Disease or Syndrome
Eczema is a form of dermatitis that is characterized by scaly, pruritic, erythematous lesions located on flexural surfaces.

Professional guidelines

PubMed

Injarie AM, Narang A, Idrees Z, Saggar AK, Nischal KK
Cornea 2012 Mar;31(3):269-72. doi: 10.1097/ICO.0b013e3182182089. PMID: 22316651

Recent clinical studies

Etiology

Warnier H, Barrea C, Bethlen S, Schrouff I, Harvengt J
Orphanet J Rare Dis 2022 Apr 23;17(1):174. doi: 10.1186/s13023-022-02323-8. PMID: 35461249Free PMC Article
Guran T, Guran O, Paketci C, Kipoglu O, Firat I, Turan S, Atay Z, Haliloglu B, Bereket A
Pituitary 2015 Aug;18(4):456-60. doi: 10.1007/s11102-014-0594-5. PMID: 25145448
Buonuomo PS, Macchiaiolo M, Cambiaso P, Rana I, Digilio MC, Bartuli A
Clin Dysmorphol 2014 Apr;23(2):45-46. doi: 10.1097/MCD.0000000000000023. PMID: 24477277
Injarie AM, Narang A, Idrees Z, Saggar AK, Nischal KK
Cornea 2012 Mar;31(3):269-72. doi: 10.1097/ICO.0b013e3182182089. PMID: 22316651
Superti-Furga A, Tenconi R, Clementi M, Eich G, Steinmann B, Boltshauser E, Giedion A
Am J Med Genet 1998 Jun 30;78(2):150-4. doi: 10.1002/(sici)1096-8628(19980630)78:2<150::aid-ajmg10>3.0.co;2-m. PMID: 9674906

Diagnosis

McDermott H, Simmonds J, Thyagarajan M; Genomics England Research Consortium, Islam L, Naik S, Titheradge H
Eur J Med Genet 2023 Aug;66(8):104788. Epub 2023 Jun 7 doi: 10.1016/j.ejmg.2023.104788. PMID: 37295610
Van De Maele K, Smulders C, Ecury-Goossen G, Rosina-Angelista I, Redeker E, van Haelst M
Clin Dysmorphol 2019 Apr;28(2):57-62. doi: 10.1097/MCD.0000000000000255. PMID: 30614825
Elsaid MF, Chalhoub N, Kamel H, Ehlayel M, Ibrahim N, Elsaid A, Kumar P, Khalak H, Ilyin VA, Suhre K, Abdel Aleem A
Clin Genet 2016 Feb;89(2):210-6. Epub 2015 Sep 29 doi: 10.1111/cge.12657. PMID: 26285796
Buonuomo PS, Macchiaiolo M, Cambiaso P, Rana I, Digilio MC, Bartuli A
Clin Dysmorphol 2014 Apr;23(2):45-46. doi: 10.1097/MCD.0000000000000023. PMID: 24477277
Kozlowski K, Tenconi R
Am J Med Genet 1996 May 3;63(1):17-9. doi: 10.1002/(SICI)1096-8628(19960503)63:1<17::AID-AJMG6>3.0.CO;2-P. PMID: 8723081

Therapy

Chen YH, Zastrow DB, Metcalfe RD, Gartner L, Krause F, Morton CJ, Marwaha S, Fresard L, Huang Y, Zhao C, McCormack C, Bick D, Worthey EA, Eng CM, Gold J, Undiagnosed Diseases Network, Montgomery SB, Fisher PG, Ashley EA, Wheeler MT, Parker MW, Shanmugasundaram V, Putoczki TL, Schmidt-Arras D, Laurence A, Bernstein JA, Griffin MDW, Uhlig HH
J Allergy Clin Immunol 2021 Aug;148(2):585-598. Epub 2021 Mar 23 doi: 10.1016/j.jaci.2021.02.044. PMID: 33771552
Injarie AM, Narang A, Idrees Z, Saggar AK, Nischal KK
Cornea 2012 Mar;31(3):269-72. doi: 10.1097/ICO.0b013e3182182089. PMID: 22316651
Crisponi L, Crisponi G, Meloni A, Toliat MR, Nurnberg G, Usala G, Uda M, Masala M, Hohne W, Becker C, Marongiu M, Chiappe F, Kleta R, Rauch A, Wollnik B, Strasser F, Reese T, Jakobs C, Kurlemann G, Cao A, Nurnberg P, Rutsch F
Am J Hum Genet 2007 May;80(5):971-81. Epub 2007 Mar 30 doi: 10.1086/516843. PMID: 17436252Free PMC Article

Prognosis

Warnier H, Barrea C, Bethlen S, Schrouff I, Harvengt J
Orphanet J Rare Dis 2022 Apr 23;17(1):174. doi: 10.1186/s13023-022-02323-8. PMID: 35461249Free PMC Article
Sarafidis K, Piretzi K, Agakidou E, Kohlhase J, Zafeiriou D
Pediatr Int 2015 Apr;57(2):302-4. doi: 10.1111/ped.12431. PMID: 25868946
Injarie AM, Narang A, Idrees Z, Saggar AK, Nischal KK
Cornea 2012 Mar;31(3):269-72. doi: 10.1097/ICO.0b013e3182182089. PMID: 22316651
Raas-Rothschild A, Ergaz-Schaltiel Z, Bar-Ziv J, Rein AJ
Am J Med Genet A 2003 Aug 30;121A(2):156-8. doi: 10.1002/ajmg.a.20066. PMID: 12910496
Al-Gazali LI, Ravenscroft A, Feng A, Shubbar A, Al-Saggaf A, Haas D
Clin Dysmorphol 2003 Jan;12(1):1-8. doi: 10.1097/00019605-200301000-00001. PMID: 12514358

Clinical prediction guides

Elsaid MF, Chalhoub N, Kamel H, Ehlayel M, Ibrahim N, Elsaid A, Kumar P, Khalak H, Ilyin VA, Suhre K, Abdel Aleem A
Clin Genet 2016 Feb;89(2):210-6. Epub 2015 Sep 29 doi: 10.1111/cge.12657. PMID: 26285796
Dagoneau N, Scheffer D, Huber C, Al-Gazali LI, Di Rocco M, Godard A, Martinovic J, Raas-Rothschild A, Sigaudy S, Unger S, Nicole S, Fontaine B, Taupin JL, Moreau JF, Superti-Furga A, Le Merrer M, Bonaventure J, Munnich A, Legeai-Mallet L, Cormier-Daire V
Am J Hum Genet 2004 Feb;74(2):298-305. Epub 2004 Jan 21 doi: 10.1086/381715. PMID: 14740318Free PMC Article
Raas-Rothschild A, Ergaz-Schaltiel Z, Bar-Ziv J, Rein AJ
Am J Med Genet A 2003 Aug 30;121A(2):156-8. doi: 10.1002/ajmg.a.20066. PMID: 12910496
Superti-Furga A, Tenconi R, Clementi M, Eich G, Steinmann B, Boltshauser E, Giedion A
Am J Med Genet 1998 Jun 30;78(2):150-4. doi: 10.1002/(sici)1096-8628(19980630)78:2<150::aid-ajmg10>3.0.co;2-m. PMID: 9674906

Recent systematic reviews

Warnier H, Barrea C, Bethlen S, Schrouff I, Harvengt J
Orphanet J Rare Dis 2022 Apr 23;17(1):174. doi: 10.1186/s13023-022-02323-8. PMID: 35461249Free PMC Article

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