Literature DB >> 7381865

Syndrome of polydactyly, cleft lip/palate or lingual lump, and psychomotor retardation in endogamic gypsies.

V Váradi, L Szabó, Z Papp.   

Abstract

Six children in an inbred isolate (a gypsy colony) were found to have a syndrome of reduplication of the big toes, supernumerary fingers on the hands, cleft lip/palate or lingual nodule, and somatic and psychomotor retardation. Other features sometimes present were absence of olfactory bulbs and tracts, cryptorchidism, inguinal hernia, and congenital heart disease. The disorder has overlapping features with several previously delineated syndromes, but is most similar to the anomalies of trisomy 13 Mand ohr's syndrome. Our patients had a normal karyotype. The mode of inheritance of this lethal genetic syndrome is probably autosomal recessive.

Entities:  

Mesh:

Year:  1980        PMID: 7381865      PMCID: PMC1048515          DOI: 10.1136/jmg.17.2.119

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  6 in total

1.  HOLOPROSENCEPHALY: A CASE REPORT WITH NO EXTRACRANIAL ABNORMALITIES AND NORMAL CHROMOSOME COUNT AND KARYOTYPE.

Authors:  K BISHOP; J M CONNOLLY; C H CARTER; D G CARPENTER
Journal:  J Pediatr       Date:  1964-09       Impact factor: 4.406

2.  Multiple congenital anomaly caused by an extra autosome.

Authors:  K PATAU; D W SMITH; E THERMAN; S L INHORN; H P WAGNER
Journal:  Lancet       Date:  1960-04-09       Impact factor: 79.321

3.  Syndrome characterized by lingual malformation, polydactyly, tachypnea, and psychomotor retardation (Mohr syndrome).

Authors:  K H Gustavson; A Kreuger; P O Petersson
Journal:  Clin Genet       Date:  1971       Impact factor: 4.438

4.  Genetic and clinical heterogeneity in the oral-facial-digital syndromes.

Authors:  D L Rimoin; M T Edgerton
Journal:  J Pediatr       Date:  1967-07       Impact factor: 4.406

5.  Autosomal trisomy syndromes: a detailed study of 27 cases of Edwards' syndrome and 27 cases of Patau's syndrome.

Authors:  A I Taylor
Journal:  J Med Genet       Date:  1968-09       Impact factor: 6.318

6.  [Severe microphthalmus with Patau's syndrome (author's transl)].

Authors:  Z Pap; I Buckó; S Szakáll; Z Papp
Journal:  Klin Monbl Augenheilkd       Date:  1978-09       Impact factor: 0.700

  6 in total
  24 in total

1.  Another case of microcephaly, facial clefting, and preaxial polydactyly.

Authors:  S L Marles; A E Chudley
Journal:  J Med Genet       Date:  1990-09       Impact factor: 6.318

Review 2.  Joubert Syndrome and related disorders.

Authors:  Francesco Brancati; Bruno Dallapiccola; Enza Maria Valente
Journal:  Orphanet J Rare Dis       Date:  2010-07-08       Impact factor: 4.123

3.  Prevalence of congenital anomaly syndromes in a Spanish gypsy population.

Authors:  M L Martínez-Frías; E Bermejo
Journal:  J Med Genet       Date:  1992-07       Impact factor: 6.318

4.  Prenatal and neonatal MR imaging findings in oral-facial-digital syndrome type VI.

Authors:  A Poretti; U Brehmer; I Scheer; V Bernet; E Boltshauser
Journal:  AJNR Am J Neuroradiol       Date:  2008-03-20       Impact factor: 3.825

5.  Orofacial manifestations and dental considerations in association with Varadi-Papp syndrome: report of a rare case.

Authors:  N Chhabra; A Chhabra; S Tandon
Journal:  Eur Arch Paediatr Dent       Date:  2015-07-10

6.  Pallister-Hall syndrome.

Authors:  L G Biesecker; J M Graham
Journal:  J Med Genet       Date:  1996-07       Impact factor: 6.318

7.  Exome sequencing identifies a homozygous C5orf42 variant in a Turkish kindred with oral-facial-digital syndrome type VI.

Authors:  Yavuz Bayram; Hatip Aydin; Tomasz Gambin; Zeynep Coban Akdemir; Mehmed M Atik; Ender Karaca; Ali Karaman; Davut Pehlivan; Shalini N Jhangiani; Richard A Gibbs; James R Lupski
Journal:  Am J Med Genet A       Date:  2015-04-06       Impact factor: 2.802

Review 8.  Genetics and signaling mechanisms of orofacial clefts.

Authors:  Kurt Reynolds; Shuwen Zhang; Bo Sun; Michael A Garland; Yu Ji; Chengji J Zhou
Journal:  Birth Defects Res       Date:  2020-07-15       Impact factor: 2.344

9.  Stringent delineation of Pallister-Hall syndrome in two long surviving patients: importance of radiological anomalies of the hands.

Authors:  A Verloes; A David; L Ngô; A Bottani
Journal:  J Med Genet       Date:  1995-08       Impact factor: 6.318

10.  Mutations in TMEM216 perturb ciliogenesis and cause Joubert, Meckel and related syndromes.

Authors:  Enza Maria Valente; Clare V Logan; Soumaya Mougou-Zerelli; Jeong Ho Lee; Jennifer L Silhavy; Francesco Brancati; Miriam Iannicelli; Lorena Travaglini; Sveva Romani; Barbara Illi; Matthew Adams; Katarzyna Szymanska; Annalisa Mazzotta; Ji Eun Lee; Jerlyn C Tolentino; Dominika Swistun; Carmelo D Salpietro; Carmelo Fede; Stacey Gabriel; Carsten Russ; Kristian Cibulskis; Carrie Sougnez; Friedhelm Hildebrandt; Edgar A Otto; Susanne Held; Bill H Diplas; Erica E Davis; Mario Mikula; Charles M Strom; Bruria Ben-Zeev; Dorit Lev; Tally Lerman Sagie; Marina Michelson; Yuval Yaron; Amanda Krause; Eugen Boltshauser; Nadia Elkhartoufi; Joelle Roume; Stavit Shalev; Arnold Munnich; Sophie Saunier; Chris Inglehearn; Ali Saad; Adila Alkindy; Sophie Thomas; Michel Vekemans; Bruno Dallapiccola; Nicholas Katsanis; Colin A Johnson; Tania Attié-Bitach; Joseph G Gleeson
Journal:  Nat Genet       Date:  2010-05-30       Impact factor: 38.330

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