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Nail-patella syndrome(NPS)

MedGen UID:
10257
Concept ID:
C0027341
Disease or Syndrome
Synonyms: Arthro-Onychodysplasia; Fong disease; NPS; NPS 1; Onychoosteodysplasia; Osteo-Onychodysplasia; Turner-Kieser syndrome
SNOMED CT: Nail-patella syndrome (22199006); Hereditary osteo-onychodysplasia (22199006); Turner Kieser syndrome (22199006); Onychoosteodysplasia (22199006); Nail patella syndrome (22199006)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
Concept ID:
C0443147
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
 
Gene (location): LMX1B (9q33.3)
 
Monarch Initiative: MONDO:0008061
OMIM®: 161200
Orphanet: ORPHA2614

Disease characteristics

Excerpted from the GeneReview: Nail-Patella Syndrome
Nail-patella syndrome (NPS) (previously referred to as Fong's disease), encompasses the classic clinical tetrad of changes in the nails, knees, and elbows, and the presence of iliac horns. Nail changes are the most constant feature of NPS. Nails may be absent, hypoplastic, or dystrophic; ridged longitudinally or horizontally; pitted; discolored; separated into two halves by a longitudinal cleft or ridge of skin; and thin or (less often) thickened. The patellae may be small, irregularly shaped, or absent. Elbow abnormalities may include limitation of extension, pronation, and supination; cubitus valgus; and antecubital pterygia. Iliac horns are bilateral, conical, bony processes that project posteriorly and laterally from the central part of the iliac bones of the pelvis. Renal involvement, first manifest as proteinuria with or without hematuria, occurs in 30%-50% of affected individuals; end-stage kidney disease occurs up to 15% of affected individuals. Primary open-angle glaucoma and ocular hypertension occur at increased frequency and at a younger age than in the general population. [from GeneReviews]
Authors:
Elizabeth Sweeney  |  Julie E Hoover-Fong  |  Iain McIntosh   view full author information

Additional descriptions

From OMIM
Nail-patella syndrome (NPS) is an autosomal dominant disorder characterized by developmental defects of dorsal limb structures, the kidney, and the eye, manifested by nail dysplasia, patellar abnormalities, elbow dysplasia, iliac horns, nephropathy, and glaucoma, respectively (summary by Bongers et al., 2005).  http://www.omim.org/entry/161200
From MedlinePlus Genetics
Nail-patella syndrome is characterized by abnormalities of the nails, knees, elbows, and pelvis. The features of nail-patella syndrome vary in severity between affected individuals, even among members of the same family.

Nail abnormalities are seen in almost all individuals with nail-patella syndrome. The nails may be absent or underdeveloped and discolored, split, ridged, or pitted. The fingernails are more likely to be affected than the toenails, and the thumbnails are usually the most severely affected. In many people with this condition, the areas at the base of the nails (lunulae) are triangular instead of the usual crescent shape.

Individuals with nail-patella syndrome also commonly have skeletal abnormalities involving the knees, elbows, and hips. The kneecaps (patellae) are small, irregularly shaped, or absent, and dislocation of the patella is common. Some people with this condition may not be able to fully extend their arms or turn their palms up while keeping their elbows straight. The elbows may also be angled outward (cubitus valgus) or have abnormal webbing. Many individuals with nail-patella syndrome have horn-like outgrowths of the iliac bones of the pelvis (iliac horns). These abnormal projections may be felt through the skin, but they do not cause any symptoms and are usually detected on a pelvic x-ray. Iliac horns are very common in people with nail-patella syndrome and are rarely, if ever, seen in people without this condition.

Other areas of the body may also be affected in nail-patella syndrome, particularly the eyes and kidneys. Individuals with this condition are at risk of developing increased pressure within the eyes (glaucoma) at an early age. Some people develop kidney disease, which can progress to kidney failure.  https://medlineplus.gov/genetics/condition/nail-patella-syndrome

Clinical features

From HPO
Back pain
MedGen UID:
2530
Concept ID:
C0004604
Sign or Symptom
An unpleasant sensation characterized by physical discomfort (such as pricking, throbbing, or aching) localized to the back.
Hematuria
MedGen UID:
5488
Concept ID:
C0018965
Disease or Syndrome
The presence of blood in the urine. Hematuria may be gross hematuria (visible to the naked eye) or microscopic hematuria (detected by dipstick or microscopic examination of the urine).
Nephrotic syndrome
MedGen UID:
10308
Concept ID:
C0027726
Disease or Syndrome
Nephrotic syndrome is a collection of findings resulting from glomerular dysfunction with an increase in glomerular capillary wall permeability associated with pronounced proteinuria. Nephrotic syndrome refers to the constellation of clinical findings that result from severe renal loss of protein, with Proteinuria and hypoalbuminemia, edema, and hyperlipidemia.
Proteinuria
MedGen UID:
10976
Concept ID:
C0033687
Finding
Increased levels of protein in the urine.
Renal insufficiency
MedGen UID:
332529
Concept ID:
C1565489
Disease or Syndrome
A reduction in the level of performance of the kidneys in areas of function comprising the concentration of urine, removal of wastes, the maintenance of electrolyte balance, homeostasis of blood pressure, and calcium metabolism.
Clubfoot
MedGen UID:
3130
Concept ID:
C0009081
Congenital Abnormality
Clubfoot is a congenital limb deformity defined as fixation of the foot in cavus, adductus, varus, and equinus (i.e., inclined inwards, axially rotated outwards, and pointing downwards) with concomitant soft tissue abnormalities (Cardy et al., 2007). Clubfoot may occur in isolation or as part of a syndrome (e.g., diastrophic dysplasia, 222600). Clubfoot has been reported with deficiency of long bones and mirror-image polydactyly (Gurnett et al., 2008; Klopocki et al., 2012).
Pes planus
MedGen UID:
42034
Concept ID:
C0016202
Anatomical Abnormality
A foot where the longitudinal arch of the foot is in contact with the ground or floor when the individual is standing; or, in a patient lying supine, a foot where the arch is in contact with the surface of a flat board pressed against the sole of the foot by the examiner with a pressure similar to that expected from weight bearing; or, the height of the arch is reduced.
Disproportionate prominence of the femoral medial condyle
MedGen UID:
320339
Concept ID:
C1834392
Finding
Patellar hypoplasia
MedGen UID:
327021
Concept ID:
C1840068
Finding
Underdevelopment of the patella.
Clinodactyly of the 5th finger
MedGen UID:
340456
Concept ID:
C1850049
Congenital Abnormality
Clinodactyly refers to a bending or curvature of the fifth finger in the radial direction (i.e., towards the 4th finger).
Absent distal interphalangeal creases
MedGen UID:
350011
Concept ID:
C1861349
Finding
Absence of the distal interphalangeal flexion creases of the fingers.
Limited elbow extension
MedGen UID:
401158
Concept ID:
C1867103
Finding
Limited ability to straighten the arm at the elbow joint.
Patellar aplasia
MedGen UID:
401474
Concept ID:
C1868578
Congenital Abnormality
Absence of the patella.
Hypoplastic radial head
MedGen UID:
867331
Concept ID:
C4021695
Anatomical Abnormality
Elongated radius
MedGen UID:
870599
Concept ID:
C4025048
Anatomical Abnormality
Increased length of the radius.
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).
Sensorineural hearing loss disorder
MedGen UID:
9164
Concept ID:
C0018784
Disease or Syndrome
A type of hearing impairment in one or both ears related to an abnormal functionality of the cochlear nerve.
Spina bifida
MedGen UID:
38283
Concept ID:
C0080178
Congenital Abnormality
Incomplete closure of the embryonic neural tube, whereby some vertebral arches remain unfused and open. The mildest form is spina bifida occulta, followed by meningocele and meningomyelocele.
Scoliosis
MedGen UID:
11348
Concept ID:
C0036439
Disease or Syndrome
The presence of an abnormal lateral curvature of the spine.
Iliac horns
MedGen UID:
120492
Concept ID:
C0263925
Finding
Horn-like malformations of the iliac crests with symmetrical bilateral central posterior iliac processes. A characteristic finding in the Nail-Patella syndrome. Iliac horns are visible on X-ray and may be palpable, but are asymptomatic.
Patellar dislocation
MedGen UID:
253896
Concept ID:
C1135812
Injury or Poisoning
The kneecap normally is located within the groove termed trochlea on the distal femur and can slide up and down in it. Patellar dislocation occurs if the patella fully dislocates out of the groove.
Lumbar hyperlordosis
MedGen UID:
263149
Concept ID:
C1184923
Finding
An abnormal accentuation of the inward curvature of the spine in the lumbar region.
Thickening of the lateral border of the scapula
MedGen UID:
322404
Concept ID:
C1834383
Finding
Glenoid fossa hypoplasia
MedGen UID:
331731
Concept ID:
C1834384
Finding
Underdevelopment of the glenoid fossa, which is the cavity in the lateral part of the scapula which articulates with the head of the humerus.
Hypoplasia of first ribs
MedGen UID:
331732
Concept ID:
C1834386
Congenital Abnormality
Absence of pectoralis minor muscle
MedGen UID:
401383
Concept ID:
C1868158
Finding
Aplasia (congenital absence) of the pectoralis minor.
Pectus excavatum
MedGen UID:
781174
Concept ID:
C2051831
Finding
A defect of the chest wall characterized by a depression of the sternum, giving the chest ("pectus") a caved-in ("excavatum") appearance.
Biceps aplasia
MedGen UID:
812094
Concept ID:
C3805764
Finding
Absence of the biceps muscle.
Quadriceps aplasia
MedGen UID:
812095
Concept ID:
C3805765
Finding
Absence of the quadriceps muscle.
Triceps aplasia
MedGen UID:
816814
Concept ID:
C3810484
Finding
Absence of the triceps muscle.
Glomerulonephritis
MedGen UID:
6616
Concept ID:
C0017658
Disease or Syndrome
Inflammation of the renal glomeruli.
Cleft upper lip
MedGen UID:
40327
Concept ID:
C0008924
Congenital Abnormality
A gap or groove in the upper lip. This is a congenital defect resulting from nonfusion of tissues of the lip during embryonal development.
Cleft palate
MedGen UID:
756015
Concept ID:
C2981150
Congenital Abnormality
Cleft palate is a developmental defect of the palate resulting from a failure of fusion of the palatine processes and manifesting as a separation of the roof of the mouth (soft and hard palate).
Concave nail
MedGen UID:
66369
Concept ID:
C0221261
Finding
The natural longitudinal (posterodistal) convex arch is not present or is inverted.
Anonychia
MedGen UID:
120563
Concept ID:
C0265998
Congenital Abnormality
Congenital anonychia is defined as the absence of fingernails and toenails. Anonychia and its milder phenotypic variant, hyponychia, usually occur as a feature of genetic syndromes, in association with significant skeletal and limb anomalies. Isolated nonsyndromic congenital anonychia/hyponychia is a rare entity that usually follows autosomal recessive inheritance with variable expression, even within a given family. The nail phenotypes observed range from no nail field to a nail field of reduced size with an absent or rudimentary nail (summary by Bruchle et al., 2008). This form of nail disorder is referred to here as nonsyndromic congenital nail disorder-4 (NDNC4). For a list of other nonsyndromic congenital nail disorders and a discussion of genetic heterogeneity, see NDNC1 (161050).
Ridged nail
MedGen UID:
140853
Concept ID:
C0423820
Finding
Longitudinal, linear prominences in the nail plate.
Antecubital pterygium syndrome
MedGen UID:
401231
Concept ID:
C1867439
Disease or Syndrome
Antecubital pterygium syndrome is an autosomal dominant disorder characterized by a fleshy web extending across the anterior aspect of the cubital fossa, absence of the long head of the triceps, limitation of full elbow extension, and missing skin creases over the terminal interphalangeal joints of the fingers (summary by Wallis et al., 1988).
Ptosis
MedGen UID:
2287
Concept ID:
C0005745
Disease or Syndrome
The upper eyelid margin is positioned 3 mm or more lower than usual and covers the superior portion of the iris (objective); or, the upper lid margin obscures at least part of the pupil (subjective).
Glaucoma
MedGen UID:
42224
Concept ID:
C0017601
Disease or Syndrome
Glaucoma refers loss of retinal ganglion cells in a characteristic pattern of optic neuropathy usually associated with increased intraocular pressure.
Keratoconus
MedGen UID:
44015
Concept ID:
C0022578
Disease or Syndrome
A cone-shaped deformity of the cornea characterized by the presence of corneal distortion secondary to thinning of the apex.
Cataract
MedGen UID:
39462
Concept ID:
C0086543
Disease or Syndrome
A cataract is an opacity or clouding that develops in the crystalline lens of the eye or in its capsule.
Microphakia
MedGen UID:
78609
Concept ID:
C0266541
Congenital Abnormality
Abnormal smallness of the lens.
Microcornea
MedGen UID:
78610
Concept ID:
C0266544
Congenital Abnormality
A congenital abnormality of the cornea in which the cornea and the anterior segment of the eye are smaller than normal. The horizontal diameter of the cornea does not reach 10 mm even in adulthood.
Lester sign
MedGen UID:
869781
Concept ID:
C4024210
Finding
A zone of darker pigmentation around the central part of the iris with a roughly cloverleaf or flower shape.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNail-patella syndrome
Follow this link to review classifications for Nail-patella syndrome in Orphanet.

Professional guidelines

PubMed

Sever R, Fishkin M, Hemo Y, Wientroub S, Yaniv M
J Pediatr Orthop 2019 Sep;39(8):436-440. doi: 10.1097/BPO.0000000000000973. PMID: 31393310
Richert B, André J
Am J Clin Dermatol 2011 Apr 1;12(2):101-12. doi: 10.2165/11537110-000000000-00000. PMID: 21348541
Soliman N
Nephrol Dial Transplant 2009 Sep;24(9):2951; author reply 2951-2. Epub 2009 Jun 18 doi: 10.1093/ndt/gfp266. PMID: 19541663

Recent clinical studies

Etiology

Geerts-Crabbé L, Antoine P, Brugallé E, Ghoumid J, Bellengier L, Edery P, Heron D, Manouvrier-Hanu S, Fantini-Hauwel C
J Genet Couns 2019 Oct;28(5):1011-1020. Epub 2019 Jul 16 doi: 10.1002/jgc4.1153. PMID: 31313463
Das CJ, Debnath J
Indian J Pediatr 2009 Oct;76(10):1077. doi: 10.1007/s12098-009-0197-8. PMID: 19907953
Lemley KV
Pediatr Nephrol 2009 Dec;24(12):2345-54. Epub 2008 Jun 6 doi: 10.1007/s00467-008-0836-8. PMID: 18535845Free PMC Article
Sprecher E
Clin Dermatol 2005 Jan-Feb;23(1):47-55. doi: 10.1016/j.clindermatol.2004.09.009. PMID: 15708289
Beguiristáin JL, de Rada PD, Barriga A
J Pediatr Orthop B 2003 Jan;12(1):13-6. doi: 10.1097/01.bpb.0000043727.21564.e9. PMID: 12488765

Diagnosis

Nahmani L, Fitoussi F
Hand Surg Rehabil 2024 Apr;43S:101527. doi: 10.1016/j.hansur.2023.01.011. PMID: 38879228
Kundu ZS, Siwach RC
Indian J Med Res 2018 Jun;147(6):619-620. doi: 10.4103/ijmr.IJMR_1252_16. PMID: 30168496Free PMC Article
Vanlerberghe C, Boutry N, Petit F
Clin Genet 2018 Jul;94(1):43-53. Epub 2018 Apr 11 doi: 10.1111/cge.13209. PMID: 29322497
Lo Sicco K, Sadeghpour M, Ferris L
J Drugs Dermatol 2015 Jan;14(1):85-6. PMID: 25763426
Schulz-Butulis BA, Welch MD, Norton SA
J Am Acad Dermatol 2003 Dec;49(6):1086-7. doi: 10.1016/j.jaad.2002.01.001. PMID: 14639389

Therapy

Hoang N, Harper H, Syscos S, Rickstrew J, Kaplan D
Dermatol Online J 2020 Jul 15;26(7) PMID: 32898401
Dhadwal G, Kirchhof MG
J Cutan Med Surg 2018 Mar/Apr;22(2):194-199. Epub 2017 Oct 23 doi: 10.1177/1203475417738971. PMID: 29056081
Proesmans W, Van Dyck M, Devriendt K
Nephrol Dial Transplant 2009 Apr;24(4):1335-8. Epub 2009 Jan 15 doi: 10.1093/ndt/gfn725. PMID: 19147669
Hennessey TA, Backman SB, Meterissian SH, Schricker T
Can J Anaesth 2007 Oct;54(10):835-9. doi: 10.1007/BF03021712. PMID: 17934166
Nandedkar-Thomas MA, Scher RK
J Am Acad Dermatol 2005 May;52(5):877-87. doi: 10.1016/j.jaad.2004.11.055. PMID: 15858481

Prognosis

Harita Y, Urae S, Akashio R, Isojima T, Miura K, Yamada T, Yamamoto K, Miyasaka Y, Furuyama M, Takemura T, Gotoh Y, Takizawa H, Tamagaki K, Ozawa A, Ashida A, Hattori M, Oka A, Kitanaka S
Eur J Hum Genet 2020 Oct;28(10):1414-1421. Epub 2020 May 26 doi: 10.1038/s41431-020-0655-3. PMID: 32457516Free PMC Article
Witzgall R
Pflugers Arch 2017 Aug;469(7-8):927-936. Epub 2017 Jul 5 doi: 10.1007/s00424-017-2013-z. PMID: 28681095
Harita Y, Kitanaka S, Isojima T, Ashida A, Hattori M
Pediatr Nephrol 2017 Oct;32(10):1845-1850. Epub 2016 Jul 23 doi: 10.1007/s00467-016-3462-x. PMID: 27450397
Witzgall R
Pediatr Nephrol 2008 Jul;23(7):1017-20. Epub 2008 Feb 6 doi: 10.1007/s00467-007-0714-9. PMID: 18253764Free PMC Article
Saborio P, Scheinman J
Curr Opin Pediatr 1998 Apr;10(2):174-83. doi: 10.1097/00008480-199804000-00011. PMID: 9608896

Clinical prediction guides

Bech S, Løkkegaard A, Nielsen TT, Nørremølle A, Grønborg S, Hasholt L, Steffensen GK, Graehn G, Olesen JH, Tommerup N, Mang Y, Bak M, Nielsen JE, Eiberg H, Hjermind LE
Mov Disord 2020 Dec;35(12):2343-2347. Epub 2020 Sep 19 doi: 10.1002/mds.28244. PMID: 32949189Free PMC Article
Geerts-Crabbé L, Antoine P, Brugallé E, Ghoumid J, Bellengier L, Edery P, Heron D, Manouvrier-Hanu S, Fantini-Hauwel C
J Genet Couns 2019 Oct;28(5):1011-1020. Epub 2019 Jul 16 doi: 10.1002/jgc4.1153. PMID: 31313463
Vanlerberghe C, Boutry N, Petit F
Clin Genet 2018 Jul;94(1):43-53. Epub 2018 Apr 11 doi: 10.1111/cge.13209. PMID: 29322497
Tigchelaar S, Lenting A, Bongers EM, van Kampen A
Orthop Traumatol Surg Res 2015 Dec;101(8):959-62. Epub 2015 Nov 17 doi: 10.1016/j.otsr.2015.09.033. PMID: 26596417
Sabnis SG, Antonovych TT, Argy WP, Rakowski TA, Gandy DR, Salcedo JR
Clin Nephrol 1980 Sep;14(3):148-53. PMID: 7418282

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