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Narrow palm

MedGen UID:
346628
Concept ID:
C1857632
Finding
Synonym: Narrow hands
 
HPO: HP:0004283

Definition

For children from birth to 4 years of age, the palm width is more than 2 SD below the mean; for children from 4 to 16 years of age the palm width is below the 5th centile; or, the width of the palm appears disproportionately narrow for its length. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNarrow palm

Conditions with this feature

Prader-Willi syndrome
MedGen UID:
46057
Concept ID:
C0032897
Disease or Syndrome
Prader-Willi syndrome (PWS) is characterized by severe hypotonia and feeding difficulties in early infancy, followed in later infancy or early childhood by excessive eating and gradual development of morbid obesity (unless eating is externally controlled). Motor milestones and language development are delayed. All individuals have some degree of cognitive impairment. A distinctive behavioral phenotype (with temper tantrums, stubbornness, manipulative behavior, and obsessive-compulsive characteristics) is common. Hypogonadism is present in both males and females and manifests as genital hypoplasia, incomplete pubertal development, and, in most, infertility. Short stature is common (if not treated with growth hormone); characteristic facial features, strabismus, and scoliosis are often present.
Cohen syndrome
MedGen UID:
78539
Concept ID:
C0265223
Congenital Abnormality
Cohen syndrome is characterized by failure to thrive in infancy and childhood; truncal obesity in the teen years; early-onset hypotonia and developmental delays; microcephaly developing during the first year of life; moderate to profound psychomotor retardation; progressive retinochoroidal dystrophy and high myopia; neutropenia in many with recurrent infections and aphthous ulcers in some; a cheerful disposition; joint hypermobility; and characteristic facial features.
Syndromic X-linked intellectual disability Snyder type
MedGen UID:
162918
Concept ID:
C0796160
Disease or Syndrome
Snyder-Robinson syndrome (SRS) is an X-linked intellectual disability syndrome characterized by asthenic build, facial dysmorphism with a prominent lower lip, kyphoscoliosis, osteoporosis, speech abnormalities, and seizures. Developmental delay usually presents as failure to meet early developmental milestones and then evolves to moderate to profound intellectual disability (which appears to remain stable over time) and variable motor disability. Asthenic habitus and low muscle mass usually develop during the first year, even in males who are ambulatory. During the first decade, males with SRS develop osteoporosis, resulting in fractures in the absence of trauma.
Oculocerebrofacial syndrome, Kaufman type
MedGen UID:
343403
Concept ID:
C1855663
Disease or Syndrome
Kaufman oculocerebrofacial syndrome (KOS) is characterized by developmental delay, severe intellectual disability, and distinctive craniofacial features. Most affected children have prenatal-onset microcephaly, hypotonia, and growth deficiency. Feeding issues, ocular abnormalities, hearing impairment, and respiratory tract abnormalities are common. Ocular abnormalities can include structural abnormalities (microcornea or microphthalmia, coloboma, optic nerve hypoplasia), refractive errors (myopia ± astigmatism, hyperopia), strabismus, and entropion. Both conductive and sensorineural hearing loss have been reported as well as mixed conductive-sensorineural hearing loss of variable severity. Breathing problems can lead to prolonged hospitalization after birth in more than half of individuals. Less common findings include ectodermal abnormalities, cardiac manifestations, urogenital abnormalities, seizures, and skeletal abnormalities.
Koolen-de Vries syndrome
MedGen UID:
355853
Concept ID:
C1864871
Disease or Syndrome
Koolen-de Vries syndrome (KdVS) is characterized by developmental delay / intellectual disability, neonatal/childhood hypotonia, dysmorphisms, congenital malformations, and behavioral features. Psychomotor developmental delay is noted in all individuals from an early age. The majority of individuals with KdVS function in the mild-to-moderate range of intellectual disability. Other findings include speech and language delay (100%), epilepsy (~33%), congenital heart defects (25%-50%), renal and urologic anomalies (25%-50%), and cryptorchidism (71% of males). Behavior in most is described as friendly, amiable, and cooperative.
Intellectual disability, autosomal dominant 42
MedGen UID:
934741
Concept ID:
C4310774
Mental or Behavioral Dysfunction
GNB1 encephalopathy (GNB1-E) is characterized by moderate-to-severe developmental delay / intellectual disability, structural brain abnormalities, and often infantile hypotonia and seizures. Other less common findings include dystonia, reduced vision, behavior issues, growth delay, gastrointestinal (GI) problems, genitourinary (GU) abnormalities in males, and cutaneous mastocytosis.
Ritscher-Schinzel syndrome 4
MedGen UID:
1794149
Concept ID:
C5561939
Disease or Syndrome
Ritscher-Schinzel syndrome-4 (RTSC4) is characterized by a constellation of congenital anomalies, including dysmorphic craniofacial features and structural brain anomalies, such as Dandy-Walker malformation (220200), hindbrain malformations, or agenesis of the corpus callosum, associated with global developmental delay and impaired intellectual development. Congenital cardiac defects have been reported in 1 family (summary by Ritscher et al., 1987 and Jeanne et al., 2021). For a discussion of genetic heterogeneity of Ritscher-Schinzel syndrome, see RTSC1 (220210).
Schaaf-Yang syndrome
MedGen UID:
1807366
Concept ID:
C5575066
Disease or Syndrome
Schaaf-Yang syndrome (SYS) is a rare neurodevelopmental disorder that shares multiple clinical features with the genetically related Prader-Willi syndrome. It usually manifests at birth with muscular hypotonia in all and distal joint contractures in a majority of affected individuals. Gastrointestinal/feeding problems are particularly pronounced in infancy and childhood, but can transition to hyperphagia and obesity in adulthood. Respiratory distress is present in many individuals at birth, with approximately half requiring intubation and mechanical ventilation, and approximately 20% requiring tracheostomy. Skeletal manifestations such as joint contractures, scoliosis, and decreased bone mineral density are frequently observed. All affected individuals show developmental delay, resulting in intellectual disability of variable degree, from low-normal intelligence to severe intellectual disability. Other findings may include short stature, seizures, eye anomalies, and hypogonadism.
Intellectual developmental disorder, autosomal dominant 68
MedGen UID:
1802176
Concept ID:
C5677008
Mental or Behavioral Dysfunction
Autosomal dominant intellectual developmental disorder-68 (MRD68) is characterized by developmental delay/intellectual disability, microcephaly, poor growth, feeding difficulties, and dysmorphic features. Some patients may have autism spectrum disorder or attention deficit-hyperactivity disorder (ADHD) (Cif et al., 2020).

Professional guidelines

PubMed

Franco JV, Trivisonno L, Sgarbossa NJ, Alvez GA, Fieiras C, Escobar Liquitay CM, Jung JH
Cochrane Database Syst Rev 2023 Jun 22;6(6):CD001423. doi: 10.1002/14651858.CD001423.pub4. PMID: 37345871Free PMC Article

Recent clinical studies

Etiology

Franco JV, Trivisonno L, Sgarbossa NJ, Alvez GA, Fieiras C, Escobar Liquitay CM, Jung JH
Cochrane Database Syst Rev 2023 Jun 22;6(6):CD001423. doi: 10.1002/14651858.CD001423.pub4. PMID: 37345871Free PMC Article
Wikström F, Olsson C, Palm B, Roxhed N, Backlund L, Schalling M, Beck O
J Pharm Biomed Anal 2023 Apr 1;227:115269. Epub 2023 Jan 29 doi: 10.1016/j.jpba.2023.115269. PMID: 36724686
Chahine EB, Sucher AJ, Mantei K
Consult Pharm 2014 Sep;29(9):614-24. doi: 10.4140/TCP.n.2014.614.. PMID: 25203410
Arslan Tas D, Erken E, Sakalli H, Yucel AE
Rheumatol Int 2012 Nov;32(11):3581-6. Epub 2011 Nov 17 doi: 10.1007/s00296-011-2205-3. PMID: 22090005
Tyler T
J Oncol Pharm Pract 2011 Sep;17(3):236-45. Epub 2010 Sep 22 doi: 10.1177/1078155210379167. PMID: 20861179

Diagnosis

Lee YK
Medicine (Baltimore) 2023 Aug 4;102(31):e34566. doi: 10.1097/MD.0000000000034566. PMID: 37543774Free PMC Article
Franco JV, Trivisonno L, Sgarbossa NJ, Alvez GA, Fieiras C, Escobar Liquitay CM, Jung JH
Cochrane Database Syst Rev 2023 Jun 22;6(6):CD001423. doi: 10.1002/14651858.CD001423.pub4. PMID: 37345871Free PMC Article
Filingeri D, Zhang H, Arens EA
J Neurophysiol 2017 Apr 1;117(4):1797-1806. Epub 2017 Feb 1 doi: 10.1152/jn.00845.2016. PMID: 28148644Free PMC Article
Elwan NM, Eltatawy RA, Elfar NN, Elsakka OM
Int J Dermatol 2016 Feb;55(2):187-92. Epub 2015 Sep 4 doi: 10.1111/ijd.12882. PMID: 26341359
Furlanetto RL, De Moraes CG, Teng CC, Liebmann JM, Greenfield DS, Gardiner SK, Ritch R, Krupin T; Low-Pressure Glaucoma Treatment Study Group
Am J Ophthalmol 2014 May;157(5):945-52. Epub 2014 Feb 7 doi: 10.1016/j.ajo.2014.02.009. PMID: 24513094

Therapy

Ahn S, Maarsingh H, Walker JK, Liu S, Hegde A, Sumajit HC, Kahsai AW, Lefkowitz RJ
J Clin Invest 2023 Sep 15;133(18) doi: 10.1172/JCI167337. PMID: 37432742Free PMC Article
Franco JV, Trivisonno L, Sgarbossa NJ, Alvez GA, Fieiras C, Escobar Liquitay CM, Jung JH
Cochrane Database Syst Rev 2023 Jun 22;6(6):CD001423. doi: 10.1002/14651858.CD001423.pub4. PMID: 37345871Free PMC Article
Chahine EB, Sucher AJ, Mantei K
Consult Pharm 2014 Sep;29(9):614-24. doi: 10.4140/TCP.n.2014.614.. PMID: 25203410
Tyler T
J Oncol Pharm Pract 2011 Sep;17(3):236-45. Epub 2010 Sep 22 doi: 10.1177/1078155210379167. PMID: 20861179
Sosnowska J, Balslev H
J Ethnobiol Ethnomed 2009 Dec 24;5:43. doi: 10.1186/1746-4269-5-43. PMID: 20034398Free PMC Article

Prognosis

Fischer M, Küstner T, Pappa S, Niendorf T, Pischon T, Kröncke T, Bette S, Schramm S, Schmidt B, Haubold J, Nensa F, Nonnenmacher T, Palm V, Bamberg F, Kiefer L, Schick F, Yang B
BMC Med Imaging 2023 Aug 8;23(1):104. doi: 10.1186/s12880-023-01056-9. PMID: 37553619Free PMC Article
Wikström F, Olsson C, Palm B, Roxhed N, Backlund L, Schalling M, Beck O
J Pharm Biomed Anal 2023 Apr 1;227:115269. Epub 2023 Jan 29 doi: 10.1016/j.jpba.2023.115269. PMID: 36724686
Chari A, Romanus D, Palumbo A, Blazer M, Farrelly E, Raju A, Huang H, Richardson P
Clin Lymphoma Myeloma Leuk 2020 Jan;20(1):8-17.e16. Epub 2019 Oct 10 doi: 10.1016/j.clml.2019.09.625. PMID: 31722839
Furlanetto RL, De Moraes CG, Teng CC, Liebmann JM, Greenfield DS, Gardiner SK, Ritch R, Krupin T; Low-Pressure Glaucoma Treatment Study Group
Am J Ophthalmol 2014 May;157(5):945-52. Epub 2014 Feb 7 doi: 10.1016/j.ajo.2014.02.009. PMID: 24513094
Godfrey KM, Barker DJ, Peace J, Cloke J, Osmond C
BMJ 1993 Aug 14;307(6901):405-9. doi: 10.1136/bmj.307.6901.405. PMID: 8374451Free PMC Article

Clinical prediction guides

Lee YK
Medicine (Baltimore) 2023 Aug 4;102(31):e34566. doi: 10.1097/MD.0000000000034566. PMID: 37543774Free PMC Article
Franco JV, Trivisonno L, Sgarbossa NJ, Alvez GA, Fieiras C, Escobar Liquitay CM, Jung JH
Cochrane Database Syst Rev 2023 Jun 22;6(6):CD001423. doi: 10.1002/14651858.CD001423.pub4. PMID: 37345871Free PMC Article
Wikström F, Olsson C, Palm B, Roxhed N, Backlund L, Schalling M, Beck O
J Pharm Biomed Anal 2023 Apr 1;227:115269. Epub 2023 Jan 29 doi: 10.1016/j.jpba.2023.115269. PMID: 36724686
Chari A, Romanus D, Palumbo A, Blazer M, Farrelly E, Raju A, Huang H, Richardson P
Clin Lymphoma Myeloma Leuk 2020 Jan;20(1):8-17.e16. Epub 2019 Oct 10 doi: 10.1016/j.clml.2019.09.625. PMID: 31722839
Zhou F, Xue Y, Yao X, Xu Y
Bioinformatics 2006 Apr 1;22(7):894-6. Epub 2006 Jan 24 doi: 10.1093/bioinformatics/btl013. PMID: 16434441

Recent systematic reviews

Franco JV, Trivisonno L, Sgarbossa NJ, Alvez GA, Fieiras C, Escobar Liquitay CM, Jung JH
Cochrane Database Syst Rev 2023 Jun 22;6(6):CD001423. doi: 10.1002/14651858.CD001423.pub4. PMID: 37345871Free PMC Article

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