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Simpson-Golabi-Behmel syndrome(SGBS1)

MedGen UID:
162917
Concept ID:
C0796154
Congenital Abnormality
Synonyms: Bulldog syndrome; Dysplasia gigantism syndrome, X-linked; Golabi-Rosen syndrome; GPC3-Related Simpson-Golabi-Behmel Syndrome Type 1; GPC4-Related Simpson-Golabi-Behmel Syndrome Type 1; mental retardation-overgrowth syndrome; SGBS1; Simpson dysmorphia syndrome; Simpson dysplasia syndrome; Simpson syndrome; Simpson-Golabi-Behmel Syndrome; Simpson-Golabi-Behmel Syndrome Type 1; SIMPSON-GOLABI-BEHMEL SYNDROME, TYPE 1
Modes of inheritance:
X-linked recessive inheritance
MedGen UID:
425042
Concept ID:
CN001297
Genetic Function
A mode of inheritance that is observed for recessive traits related to a gene encoded on the X chromosome. In the context of medical genetics, X-linked recessive disorders manifest in males (who have one copy of the X chromosome and are thus hemizygotes), but generally not in female heterozygotes who have one mutant and one normal allele.
SNOMED CT: Bulldog syndrome (439143004); Simpson-Golabi-Behmel syndrome (439143004)
 
Genes: GPC3; GPC4
Cytogenetic location: Xq26.2
OMIM®: 312870
Orphanet: ORPHA373

Disease characteristics

Excerpted from the GeneReview: Simpson-Golabi-Behmel Syndrome Type 1
Simpson-Golabi-Behmel syndrome type 1 (SGBS1) is characterized by pre- and postnatal macrosomia; distinctive craniofacies (including macrocephaly, coarse facial features, macrostomia, macroglossia, palatal abnormalities); and commonly, mild to severe intellectual disability with or without structural brain anomalies. Other variable findings include supernumerary nipples, diastasis recti/umbilical hernia, congenital heart defects, diaphragmatic hernia, genitourinary defects, and GI anomalies. Skeletal anomalies can include vertebral fusion, scoliosis, rib anomalies, and congenital hip dislocation. Hand anomalies can include large hands and postaxial polydactyly. Affected individuals are at increased risk for embryonal tumors, including Wilms tumor, hepatoblastoma, adrenal neuroblastoma, gonadoblastoma, and hepatocellular carcinoma.  [from GeneReviews]
Full text of GeneReview (by section):
Summary  |  Diagnosis  |  Clinical Description  |  Differential Diagnosis  |  Management  |  Genetic Counseling  |  Resources  |  Molecular Genetics  |  References  |  Chapter Notes
Authors:
Mahin Golabi  |  Alva Leung  |  Christina Lopez   view full author information

Additional descriptions

From OMIM
Simpson-Golabi-Behmel syndrome is an X-linked condition characterized by pre- and postnatal overgrowth, coarse facies, congenital heart defects, and other congenital abnormalities (Xuan et al., 1999). It shows phenotypic similarities to Beckwith-Wiedemann syndrome (BWS; 130650), another overgrowth syndrome. See also Simpson-Golabi-Behmel syndrome type 2 (SGBS2; 300209), which has been associated with a mutation in the CXORF5 gene (300170) on chromosome Xp22.  http://www.omim.org/entry/312870
From GHR
Simpson-Golabi-Behmel syndrome is a condition that affects many parts of the body and occurs primarily in males. This condition is classified as an overgrowth syndrome, which means that affected infants are considerably larger than normal at birth (macrosomia) and continue to grow and gain weight at an unusual rate. The other signs and symptoms of Simpson-Golabi-Behmel syndrome vary widely. The most severe cases are life-threatening before birth or in infancy, whereas people with milder cases often live into adulthood. People with Simpson-Golabi-Behmel syndrome have distinctive facial features including widely spaced eyes (ocular hypertelorism), an unusually large mouth (macrostomia), a large tongue (macroglossia) that may have a deep groove or furrow down the middle, a broad nose with an upturned tip, and abnormalities affecting the roof of the mouth (the palate). The facial features are often described as "coarse" in older children and adults with this condition. Other features of Simpson-Golabi-Behmel syndrome involve the chest and abdomen. Affected infants may be born with one or more extra nipples, an abnormal opening in the muscle covering the abdomen (diastasis recti), a soft out-pouching around the belly-button (an umbilical hernia), or a hole in the diaphragm (a diaphragmatic hernia) that allows the stomach and intestines to move into the chest and crowd the developing heart and lungs. Simpson-Golabi-Behmel syndrome can also cause heart defects, malformed or abnormally large kidneys, an enlarged liver and spleen (hepatosplenomegaly), and skeletal abnormalities. Additionally, the syndrome can affect the development of the gastrointestinal system, urinary system, and genitalia. Some people with this condition have mild to severe intellectual disability, while others have normal intelligence. About 10 percent of people with Simpson-Golabi-Behmel syndrome develop cancerous or noncancerous tumors in early childhood. The most common tumors are a rare form of kidney cancer called Wilms tumor and a cancerous tumor called a neuroblastoma that arises in developing nerve cells.  http://ghr.nlm.nih.gov/condition/simpson-golabi-behmel-syndrome

Clinical features

Nephroblastoma (Wilms tumor)
MedGen UID:
505324
Concept ID:
CN002424
Finding
The presence of a nephroblastoma, which is a neoplasm of the kidney that primarily affects children.
Renal cyst
MedGen UID:
776573
Concept ID:
C2173677
Finding
A cyst is a fluid-filled sac. There are two types of kidney cysts. . Polycystic kidney disease (PKD) runs in families. In PKD, the cysts take the place of the normal tissue. They enlarge the kidneys and make them work poorly, leading to kidney failure. When PKD causes kidneys to fail - which usually happens after many years - people need dialysis or kidney transplantation. About half of people with the most common type of PKD end up with kidney failure. PKD also causes cysts in other parts of the body, such as the liver. . Symptoms of PKD include: -Pain in the back and lower sides. -Headaches. -Urinary tract infections. -Blood in the urine. Doctors diagnose PKD with imaging tests and family history. Treatments include medications, and, when people with PKD develop kidney failure, dialysis or kidney transplants. Acquired cystic kidney disease (ACKD) usually happens in people who are on dialysis. Unlike PKD, the kidneys are normal sized, and cysts do not form in other parts of the body. People with ACKD already have chronic kidney disease when they develop cysts. ACKD often has no symptoms. In most cases, the cysts are harmless and do not need treatment. . NIH: National Institute of Diabetes and Digestive and Kidney Diseases.
Cryptorchidism
MedGen UID:
504309
Concept ID:
CN000029
Finding
Absence of one or both testes from the scrotum owing to failure of the testis or testes to descend through the inguinal canal to the testis.
Hypospadias
MedGen UID:
504317
Concept ID:
CN000047
Finding
Displacement of the urethral opening on the ventral (inferior) surface of the penis.
Nephroblastoma (Wilms tumor)
MedGen UID:
505324
Concept ID:
CN002424
Finding
The presence of a nephroblastoma, which is a neoplasm of the kidney that primarily affects children.
Wide mouth
MedGen UID:
504368
Concept ID:
CN000150
Finding
Distance between the oral commissures more than 2 SD above the mean. Alternatively, an apparently increased width of the oral aperture (subjective).
Macroglossia
MedGen UID:
504371
Concept ID:
CN000154
Finding
Increased length and width of the tongue.
Hypertelorism
MedGen UID:
504419
Concept ID:
CN000296
Finding
Interpupillary distance more than 2 SD above the mean (alternatively, the appearance of an increased interpupillary distance or widely spaced eyes).
Wide nasal bridge
MedGen UID:
504442
Concept ID:
CN000404
Finding
Increased breadth of the nasal bridge (and with it, the nasal root).
Dental malocclusion
MedGen UID:
504556
Concept ID:
CN000647
Finding
Dental malocclusion refers to an abnormality of the occlusion, or alignment, of the teeth and the way the upper and lower teeth fit together, resulting in overcrowding of teeth or in abnormal bite patterns.
Short nose
MedGen UID:
505478
Concept ID:
CN002885
Finding
Distance from nasion to subnasale more than two standard deviations below the mean, or alternatively, an apparently decreased length from the nasal root to the nasal tip.
Depressed nasal bridge
MedGen UID:
446656
Concept ID:
CN004681
Finding
Posterior positioning of the nasal root in relation to the overall facial profile for age.
Hypertelorism
MedGen UID:
504419
Concept ID:
CN000296
Finding
Interpupillary distance more than 2 SD above the mean (alternatively, the appearance of an increased interpupillary distance or widely spaced eyes).
Hearing impairment
MedGen UID:
446352
Concept ID:
CN000341
Finding
A decreased magnitude of the sensory perception of sound.
Preauricular skin tag
MedGen UID:
504427
Concept ID:
CN000359
Finding
A rudimentary tag of ear tissue often containing a core of cartilage and located just in front of the auricle (outer part of the ear).
Supernumerary nipples
MedGen UID:
505270
Concept ID:
CN002323
Finding
Presence of more than two nipples.
Pancreatic islet-cell hyperplasia
MedGen UID:
786397
Concept ID:
CN004001
Finding
Hyperplasia of the islets of Langerhans, i.e., of the regions of the pancreas that contain its endocrine cells.
Pectus excavatum
MedGen UID:
504591
Concept ID:
CN000721
Finding
A defect of the chest wall characterized by a depression of the sternum, giving the chest (\
Cervical ribs
MedGen UID:
504643
Concept ID:
CN000836
Finding
A supernumerary rib developing from an abnormal enlargement of the costal element of the C7 vertebra.
Postaxial hand polydactyly
MedGen UID:
427858
Concept ID:
CN001092
Finding
Supernumerary digits located at the ulnar side of the hand (that is, on the side with the fifth finger).
Broad toe
MedGen UID:
504967
Concept ID:
CN001662
Finding
Visible increase in width of the non-hallux digit without an increase in the dorso-ventral dimension.
Scoliosis
MedGen UID:
427922
Concept ID:
CN002409
Finding
The presence of an abnormal lateral curvature of the spine.
Short palm
MedGen UID:
425240
Concept ID:
CN003783
Finding
Short palm.
Accelerated skeletal maturation
MedGen UID:
428158
Concept ID:
CN004973
Finding
An abnormally increased rate of skeletal maturation. Accelerated skeletal maturation can be diagnosed on the basis of an estimation of the bone age from radiographs of specific bones in the human body.
Short distal phalanx of finger
MedGen UID:
426588
Concept ID:
CN008720
Finding
Short distance from the end of the finger to the most distal interphalangeal crease or the distal interphalangeal joint flexion point. That is, hypoplasia of one or more of the distal phalanx of finger.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Enlargement of the spleen.
Inguinal hernia
MedGen UID:
504305
Concept ID:
CN000024
Finding
Protrusion of the contents of the abdominal cavity through the inguinal canal.
Congenital diaphragmatic hernia
MedGen UID:
500895
Concept ID:
CN000730
Finding
The presence of a hernia of the diaphragm present at birth.
Diastasis recti
MedGen UID:
504851
Concept ID:
CN001406
Finding
A separation of the rectus abdominis muscle into right and left halves (which are normally joined at the midline at the linea alba).
Polysplenia
MedGen UID:
504943
Concept ID:
CN001592
Finding
Polysplenia is a congenital disease manifested by multiple small accessory spleens.
Hepatomegaly
MedGen UID:
505165
Concept ID:
CN002031
Finding
Abnormally increased size of the liver.
Meckel diverticulum
MedGen UID:
505167
Concept ID:
CN002036
Finding
Meckel's diverticulum is a congenital diverticulum located in the distal ileum.
Pancreatic islet-cell hyperplasia
MedGen UID:
786397
Concept ID:
CN004001
Finding
Hyperplasia of the islets of Langerhans, i.e., of the regions of the pancreas that contain its endocrine cells.
Preauricular skin tag
MedGen UID:
504427
Concept ID:
CN000359
Finding
A rudimentary tag of ear tissue often containing a core of cartilage and located just in front of the auricle (outer part of the ear).
Small nail
MedGen UID:
504954
Concept ID:
CN001626
Finding
A nail that is diminished in length and width, i.e., underdeveloped nail.
Cardiomyopathy
MedGen UID:
504883
Concept ID:
CN001491
Finding
A myocardial disorder in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease sufficient to cause the observed myocardial abnormality.
Pulmonic stenosis
MedGen UID:
504885
Concept ID:
CN001495
Finding
A narrowing of the right ventricular outflow tract that can occur at the pulmonary valve (valvular stenosis) or just below the pulmonary valve (infundibular stenosis).
Patent ductus arteriosus
MedGen UID:
504886
Concept ID:
CN001496
Finding
In utero, the ductus arteriosus (DA) serves to divert ventricular output away from the lungs and toward the placenta by connecting the main pulmonary artery to the descending aorta. A patent ductus arteriosus (PDA) in the first 3 days of life is a physiologic shunt in healthy term and preterm newborn infants, and normally is substantially closed within about 24 hours after bith and completely closed after about three weeks. Failure of physiologcal closure is referred to a persistent or patent ductus arteriosus (PDA). Depending on the degree of left-to-right shunting, PDA can have clinical consequences.
Transposition of the great arteries
MedGen UID:
504901
Concept ID:
CN001518
Finding
A complex congenital heart defect in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle.
Congenital diaphragmatic hernia
MedGen UID:
500895
Concept ID:
CN000730
Finding
The presence of a hernia of the diaphragm present at birth.
Splenomegaly
MedGen UID:
52469
Concept ID:
C0038002
Finding
Enlargement of the spleen.
Polysplenia
MedGen UID:
504943
Concept ID:
CN001592
Finding
Polysplenia is a congenital disease manifested by multiple small accessory spleens.
Macroglossia
MedGen UID:
504371
Concept ID:
CN000154
Finding
Increased length and width of the tongue.
Muscular hypotonia
MedGen UID:
504768
Concept ID:
CN001147
Finding
Muscular hypotonia is an abnormally low muscle tone (the amount of tension or resistance to movement in a muscle), often involving reduced muscle strength. Hypotonia is characterized by a diminished resistance to passive stretching.
Inguinal hernia
MedGen UID:
504305
Concept ID:
CN000024
Finding
Protrusion of the contents of the abdominal cavity through the inguinal canal.
Congenital diaphragmatic hernia
MedGen UID:
500895
Concept ID:
CN000730
Finding
The presence of a hernia of the diaphragm present at birth.
Postaxial hand polydactyly
MedGen UID:
427858
Concept ID:
CN001092
Finding
Supernumerary digits located at the ulnar side of the hand (that is, on the side with the fifth finger).
Broad palm
MedGen UID:
504753
Concept ID:
CN001097
Finding
For children from birth to 4 years of age the palm width is more than 2 SD above the mean; for children from 4 to 16 years of age the palm width is above the 95th centile; or, the width of the palm appears disproportionately wide for the length.
Broad toe
MedGen UID:
504967
Concept ID:
CN001662
Finding
Visible increase in width of the non-hallux digit without an increase in the dorso-ventral dimension.
Short palm
MedGen UID:
425240
Concept ID:
CN003783
Finding
Short palm.
Short distal phalanx of finger
MedGen UID:
426588
Concept ID:
CN008720
Finding
Short distance from the end of the finger to the most distal interphalangeal crease or the distal interphalangeal joint flexion point. That is, hypoplasia of one or more of the distal phalanx of finger.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews
  • CROGSimpson-Golabi-Behmel syndrome

Recent clinical studies

Etiology

Jedraszak G, Girard M, Mellos A, Djeddi DD, Chardot C, Vanrenterghem A, Moizard MP, Gondry J, Sevestre H, Mathieu-Dramard M, Lacaille F, Demeer B
Am J Med Genet A 2014 Mar;164A(3):774-7. Epub 2013 Dec 19 doi: 10.1002/ajmg.a.36335. [Epub ahead of print] PMID: 24357529
Cottereau E, Mortemousque I, Moizard MP, Bürglen L, Lacombe D, Gilbert-Dussardier B, Sigaudy S, Boute O, David A, Faivre L, Amiel J, Robertson R, Viana Ramos F, Bieth E, Odent S, Demeer B, Mathieu M, Gaillard D, Van Maldergem L, Baujat G, Maystadt I, Héron D, Verloes A, Philip N, Cormier-Daire V, Frouté MF, Pinson L, Blanchet P, Sarda P, Willems M, Jacquinet A, Ratbi I, Van Den Ende J, Lackmy-Port Lis M, Goldenberg A, Bonneau D, Rossignol S, Toutain A
Am J Med Genet C Semin Med Genet 2013 May;163C(2):92-105. Epub 2013 Apr 18 doi: 10.1002/ajmg.c.31360. [Epub ahead of print] PMID: 23606591
Thomas M, Enciso V, Stratton R, Shah S, Winder T, Tayeh M, Roeder E
Am J Med Genet A 2012 Oct;158A(10):2534-6. Epub 2012 Aug 14 doi: 10.1002/ajmg.a.35284. [Epub ahead of print] PMID: 22893378
Garavelli L, Gargano G, Simonte G, Rosato S, Wischmeijer A, Melli N, Braibanti S, Gelmini C, Forzano F, Pietrobono R, Pomponi MG, Andreucci E, Toutain A, Superti-Furga A, Neri G
Am J Med Genet A 2012 Sep;158A(9):2245-9. Epub 2012 Jul 17 doi: 10.1002/ajmg.a.35474. [Epub ahead of print] PMID: 22807161
Gurrieri F, Pomponi MG, Pietrobono R, Lucci-Cordisco E, Silvestri E, Storniello G, Neri G
Am J Med Genet A 2011 Jan;155A(1):145-8. Epub 2010 Dec 9 doi: 10.1002/ajmg.a.33586. [Epub ahead of print] PMID: 21204223

Diagnosis

Jedraszak G, Girard M, Mellos A, Djeddi DD, Chardot C, Vanrenterghem A, Moizard MP, Gondry J, Sevestre H, Mathieu-Dramard M, Lacaille F, Demeer B
Am J Med Genet A 2014 Mar;164A(3):774-7. Epub 2013 Dec 19 doi: 10.1002/ajmg.a.36335. [Epub ahead of print] PMID: 24357529
Neri G, Marini R, Cappa M, Borrelli P, Opitz JM
Am J Med Genet A 2013 Nov;161A(11):2697-703. doi: 10.1002/ajmg.a.36317. PMID: 24166811
Villarreal DD, Villarreal H, Paez AM, Peppas D, Lynch J, Roeder E, Powers GC
Am J Med Genet A 2013 Dec;161A(12):3121-5. Epub 2013 Oct 2 doi: 10.1002/ajmg.a.36086. [Epub ahead of print] PMID: 24115482
Cottereau E, Mortemousque I, Moizard MP, Bürglen L, Lacombe D, Gilbert-Dussardier B, Sigaudy S, Boute O, David A, Faivre L, Amiel J, Robertson R, Viana Ramos F, Bieth E, Odent S, Demeer B, Mathieu M, Gaillard D, Van Maldergem L, Baujat G, Maystadt I, Héron D, Verloes A, Philip N, Cormier-Daire V, Frouté MF, Pinson L, Blanchet P, Sarda P, Willems M, Jacquinet A, Ratbi I, Van Den Ende J, Lackmy-Port Lis M, Goldenberg A, Bonneau D, Rossignol S, Toutain A
Am J Med Genet C Semin Med Genet 2013 May;163C(2):92-105. Epub 2013 Apr 18 doi: 10.1002/ajmg.c.31360. [Epub ahead of print] PMID: 23606591
Mateos ME, Beyer K, López-Laso E, Siles JL, Pérez-Navero JL, Peña MJ, Guzmán J, Matas J
Am J Med Genet A 2013 May;161A(5):1091-5. Epub 2013 Mar 5 doi: 10.1002/ajmg.a.35738. [Epub ahead of print] PMID: 23463737

Therapy

Villarreal DD, Villarreal H, Paez AM, Peppas D, Lynch J, Roeder E, Powers GC
Am J Med Genet A 2013 Dec;161A(12):3121-5. Epub 2013 Oct 2 doi: 10.1002/ajmg.a.36086. [Epub ahead of print] PMID: 24115482
Sabin MA, Werther GA, Kiess W
Best Pract Res Clin Endocrinol Metab 2011 Feb;25(1):207-20. doi: 10.1016/j.beem.2010.09.010. PMID: 21396586
Ho M, Kim H
Eur J Cancer 2011 Feb;47(3):333-8. Epub 2010 Nov 26 doi: 10.1016/j.ejca.2010.10.024. [Epub ahead of print] PMID: 21112773Free PMC Article
Wabitsch M, Brenner RE, Melzner I, Braun M, Möller P, Heinze E, Debatin KM, Hauner H
Int J Obes Relat Metab Disord 2001 Jan;25(1):8-15. PMID: 11244452

Prognosis

Villarreal DD, Villarreal H, Paez AM, Peppas D, Lynch J, Roeder E, Powers GC
Am J Med Genet A 2013 Dec;161A(12):3121-5. Epub 2013 Oct 2 doi: 10.1002/ajmg.a.36086. [Epub ahead of print] PMID: 24115482
Cottereau E, Mortemousque I, Moizard MP, Bürglen L, Lacombe D, Gilbert-Dussardier B, Sigaudy S, Boute O, David A, Faivre L, Amiel J, Robertson R, Viana Ramos F, Bieth E, Odent S, Demeer B, Mathieu M, Gaillard D, Van Maldergem L, Baujat G, Maystadt I, Héron D, Verloes A, Philip N, Cormier-Daire V, Frouté MF, Pinson L, Blanchet P, Sarda P, Willems M, Jacquinet A, Ratbi I, Van Den Ende J, Lackmy-Port Lis M, Goldenberg A, Bonneau D, Rossignol S, Toutain A
Am J Med Genet C Semin Med Genet 2013 May;163C(2):92-105. Epub 2013 Apr 18 doi: 10.1002/ajmg.c.31360. [Epub ahead of print] PMID: 23606591
Zhao J, Deliard S, Aziz AR, Grant SF
BMC Med Genet 2012 Sep 24;13:89. doi: 10.1186/1471-2350-13-89. [Epub ahead of print] PMID: 22998375Free PMC Article
Hoggard N, Cruickshank M, Moar KM, Bashir S, Mayer CD
Obesity (Silver Spring) 2012 Jun;20(6):1158-67. Epub 2012 Jan 28 doi: 10.1038/oby.2012.14. [Epub ahead of print] PMID: 22286531
Glamuzina E, Aftimos S, Keesing M, Mahadevan M
Int J Pediatr Otorhinolaryngol 2009 Oct;73(10):1464-6. Epub 2009 Jul 24 doi: 10.1016/j.ijporl.2009.06.010. [Epub ahead of print] PMID: 19631996

Clinical prediction guides

McInnes KJ, Andersson TC, Simonytė K, Söderström I, Mattsson C, Seckl JR, Olsson T
Menopause 2012 Dec;19(12):1347-52. doi: 10.1097/gme.0b013e318258aad7. PMID: 23190557Free PMC Article
Zhao J, Deliard S, Aziz AR, Grant SF
BMC Med Genet 2012 Sep 24;13:89. doi: 10.1186/1471-2350-13-89. [Epub ahead of print] PMID: 22998375Free PMC Article
Hoggard N, Cruickshank M, Moar KM, Bashir S, Mayer CD
Obesity (Silver Spring) 2012 Jun;20(6):1158-67. Epub 2012 Jan 28 doi: 10.1038/oby.2012.14. [Epub ahead of print] PMID: 22286531
Capurro MI, Li F, Filmus J
EMBO Rep 2009 Aug;10(8):901-7. Epub 2009 Jul 10 doi: 10.1038/embor.2009.98. [Epub ahead of print] PMID: 19590577Free PMC Article
Buonuomo PS, Ruggiero A, Vasta I, Attinà G, Riccardi R, Zampino G
Pediatr Hematol Oncol 2005 Oct-Nov;22(7):623-8. doi: 10.1080/08880010500198988. PMID: 16166055

Recent systematic reviews

Scott RH, Walker L, Olsen ØE, Levitt G, Kenney I, Maher E, Owens CM, Pritchard-Jones K, Craft A, Rahman N
Arch Dis Child 2006 Dec;91(12):995-9. Epub 2006 Jul 20 doi: 10.1136/adc.2006.101295. [Epub ahead of print] PMID: 16857697Free PMC Article

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