Raras
Buscar doenças, sintomas, genes...
Síndrome Pallister-Killian
ORPHA:884CID-10 · Q99.8CID-11 · LD7YOMIM 601803DOENÇA RARA

A síndrome de Pallister-Killian (PKS) é uma síndrome rara de anomalia congênita múltipla/déficit intelectual causada por tetrassomia limitada por tecido em mosaico para o cromossomo 12p.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A síndrome de Pallister-Killian (PKS) é uma síndrome rara de anomalia congênita múltipla/déficit intelectual causada por tetrassomia limitada por tecido em mosaico para o cromossomo 12p.

Publicações científicas
236 artigos
Último publicado: 2025 Jun

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
Antenatal
+ neonatal
🏥
SUS: Cobertura mínimaScore: 15%
CID-10: Q99.8
🇧🇷Dados SUS / DATASUS
PROCEDIMENTOS SIGTAP (5)
0202010503
Cariótipo — bandas G, Q ou Rgenetic_test
0202010600
Pesquisa de microdeleções/microduplicações por FISHlab_test
0202010694
Sequenciamento completo do exoma (WES)rehabilitation
0202010260
Dosagem de alfa-fetoproteína
0301070040
Atendimento em reabilitação — doenças raras
Você se identifica com essa condição?
O Raras está aqui pra te apoiar — com ou sem diagnóstico

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

😀
Face
16 sintomas
🦴
Ossos e articulações
16 sintomas
🧠
Neurológico
12 sintomas
👁️
Olhos
9 sintomas
🧬
Pele e cabelo
7 sintomas
🫃
Digestivo
7 sintomas

+ 53 sintomas em outras categorias

Características mais comuns

100%prev.
HP:0003577
Frequência: 2/2
100%prev.
Início fetal
Obrigatório (100%)
100%prev.
Mão pequena
Obrigatório (100%)
100%prev.
Fosseta sacral
Obrigatório (100%)
100%prev.
Hipoplasia labial
Obrigatório (100%)
100%prev.
Choro fraco
Obrigatório (100%)
143sintomas
Muito frequente (37)
Frequente (43)
Ocasional (18)
Sem dados (45)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 143 características clínicas mais associadas, ordenadas por frequência.

HP:0003577
Frequência: 2/2100%
Início fetalFetal onset
Obrigatório (100%)100%
Mão pequenaSmall hand
Obrigatório (100%)100%
Fosseta sacralSacral dimple
Obrigatório (100%)100%
Hipoplasia labialLabial hypoplasia
Obrigatório (100%)100%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico236PubMed
Últimos 10 anos78publicações
Pico201813 papers
Linha do tempo
2025Hoje · 2026📈 2018Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome Pallister-Killian

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

Nenhum ensaio clínico registrado para esta condição.

🧪 Está conduzindo uma pesquisa?
Divulgue para pacientes e familiares que acompanham esta doença.
Divulgar pesquisa →

Publicações mais relevantes

Timeline de publicações
77 papers (10 anos)
#1

Ocular Manifestations of Pallister-Killian Syndrome.

Ophthalmology. Retina2025 Jun
#2

Defining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.

Cell reports2024 Jun 25

Aberrant male germline development can lead to the formation of seminoma, a testicular germ cell tumor. Seminomas are biologically similar to primordial germ cells (PGCs) and many bear an isochromosome 12p [i(12p)] with two additional copies of the short arm of chromosome 12. By mapping seminoma transcriptomes and open chromatin landscape onto a normal human male germline trajectory, we find that seminoma resembles premigratory/migratory PGCs; however, it exhibits enhanced germline and pluripotency programs and upregulation of genes involved in apoptosis, angiogenesis, and MAPK/ERK pathways. Using pluripotent stem cell-derived PGCs from Pallister-Killian syndrome patients mosaic for i(12p), we model seminoma and identify gene dosage effects that may contribute to transformation. As murine seminoma models do not exist, our analyses provide critical insights into genetic, cellular, and signaling programs driving seminoma transformation, and the in vitro platform developed herein permits evaluation of additional signals required for seminoma tumorigenesis.

#3

Structural brain abnormalities in Pallister-Killian syndrome: a neuroimaging study of 31 children.

Orphanet journal of rare diseases2024 Mar 08

Pallister-Killian syndrome (PKS) is a rare genetic disorder caused by mosaic tetrasomy of 12p with wide neurological involvement. Intellectual disability, developmental delay, behavioral problems, epilepsy, sleep disturbances, and brain malformations have been described in most individuals, with a broad phenotypic spectrum. This observational study, conducted through brain MRI scan analysis on a cohort of patients with genetically confirmed PKS, aims to systematically investigate the neuroradiological features of this syndrome and identify the possible existence of a typical pattern. Moreover, a literature review differentiating the different types of neuroimaging data was conducted for comparison with our population. Thirty-one individuals were enrolled (17 females/14 males; age range 0.1-17.5 years old at first MRI). An experienced pediatric neuroradiologist reviewed brain MRIs, blindly to clinical data. Brain abnormalities were observed in all but one individual (compared to the 34% frequency found in the literature review). Corpus callosum abnormalities were found in 20/30 (67%) patients: 6 had callosal hypoplasia; 8 had global hypoplasia with hypoplastic splenium; 4 had only hypoplastic splenium; and 2 had a thin corpus callosum. Cerebral hypoplasia/atrophy was found in 23/31 (74%) and ventriculomegaly in 20/31 (65%). Other frequent features were the enlargement of the cisterna magna in 15/30 (50%) and polymicrogyria in 14/29 (48%). Conversely, the frequency of the latter was found to be 4% from the literature review. Notably, in our population, polymicrogyria was in the perisylvian area in all 14 cases, and it was bilateral in 10/14. Brain abnormalities are very common in PKS and occur much more frequently than previously reported. Bilateral perisylvian polymicrogyria was a main aspect of our population. Our findings provide an additional tool for early diagnosis.Further studies to investigate the possible correlations with both genotype and phenotype may help to define the etiopathogenesis of the neurologic phenotype of this syndrome.

#4

Human Genetics of Ventricular Septal Defect.

Advances in experimental medicine and biology2024

Ventricular septal defects (VSDs) are recognized as one of the commonest congenital heart diseases (CHD), accounting for up to 40% of all cardiac malformations, and occur as isolated CHDs as well as together with other cardiac and extracardiac congenital malformations in individual patients and families. The genetic etiology of VSD is complex and extraordinarily heterogeneous. Chromosomal abnormalities such as aneuploidy and structural variations as well as rare point mutations in various genes have been reported to be associated with this cardiac defect. This includes both well-defined syndromes with known genetic cause (e.g., DiGeorge syndrome and Holt-Oram syndrome) and so far undefined syndromic forms characterized by unspecific symptoms. Mutations in genes encoding cardiac transcription factors (e.g., NKX2-5 and GATA4) and signaling molecules (e.g., CFC1) have been most frequently found in VSD cases. Moreover, new high-resolution methods such as comparative genomic hybridization enabled the discovery of a high number of different copy number variations, leading to gain or loss of chromosomal regions often containing multiple genes, in patients with VSD. In this chapter, we will describe the broad genetic heterogeneity observed in VSD patients considering recent advances in this field.

#5

Pineocytoma in a child with Pallister-Killian syndrome: a case report and review of the literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery2024 Aug

Pallister-Killian syndrome (PKS; OMIM #601803) is a rare genetic disorder typically characterized by developmental delay, seizures, sparse temporal hair, and facial dysmorphisms. PKS is most frequently caused by mosaic supernumerary isochromosome 12p. Here, we report a 27-month-old girl with a prenatal diagnosis of PKS and a histopathological diagnosis of pineocytoma.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC195 artigos no totalmostrando 74

2025

Ocular Manifestations of Pallister-Killian Syndrome.

Ophthalmology. Retina
2024

Pallister-Killian Syndrome.

NeoReviews
2024

Human Genetics of Ventricular Septal Defect.

Advances in experimental medicine and biology
2024

Defining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.

Cell reports
2024

Pineocytoma in a child with Pallister-Killian syndrome: a case report and review of the literature.

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
2024

A case report of Pallister-Killian syndrome with an unusual mosaic supernumerary marker chromosome 12 with interstitial 12p13.1-p12.1 duplication.

Frontiers in genetics
2024

Structural brain abnormalities in Pallister-Killian syndrome: a neuroimaging study of 31 children.

Orphanet journal of rare diseases
2023

Testing With Intent in Mosaic Conditions: A Case-Based Review.

Cureus
2023

Co-Occurrence of Pallister-Killian Syndrome and Burkitt Lymphoma in a Patient with Near-Normal Neurocognitive Development.

Molecular syndromology
2023

Prenatal diagnosis of a case with complete and uniform tetrasomy 12p by the utility of noninvasive prenatal testing.

Journal of assisted reproduction and genetics
2023

Syndromic congenital diaphragmatic hernia: Current incidence and outcome. Analysis from the congenital diaphragmatic hernia study group registry.

Prenatal diagnosis
2022

Case Report: Early Neonatal EEG in Two Infants with Pallister Killian Syndrome (PKS).

HRB open research
2022

Rapid whole genome sequencing of critically ill pediatric patients from genetically underrepresented populations.

Genome medicine
2022

Congenital Diaphragmatic Hernia Repair in a Patient With Pallister-Killian Mosaic Syndrome and Left Ventricular Hypoplasia.

Cureus
2022

Case Report: A Case Study on the Neurodevelopmental Profile of a Child With Pallister-Killian Syndrome and His Unaffected Twin.

Frontiers in pediatrics
2022

Cognitive, Behavioral, and Sensory Profile of Pallister-Killian Syndrome: A Prospective Study of 22 Individuals.

Genes
2021

Sleep in Children With Pallister Killian Syndrome: A Prospective Clinical and Videopolysomnographic Study.

Frontiers in neurology
2021

Dental Clinical and Radiographic Findings in a Patient with Pallister Killian Syndrome and 45, X/46, XY Mosaicism.

Journal of dentistry for children (Chicago, Ill.)
2021

[Non-invasive prenatal testing and genetic diagnosis of a case of Pallister-Killian syndrome].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2021

Prenatal diagnosis of Pallister-Killian syndrome and literature review.

Journal of cellular and molecular medicine
2021

Pallister-Killian Syndrome versus Trisomy 12p-A Clinical Study of 5 New Cases and a Literature Review.

Genes
2020

A Novel Case of Tethered Cord in a Five-Month-Old Male With Pallister-Killian Syndrome.

Cureus
2021

Polymicrogyria with calcification in Pallister-Killian syndrome detected by microarray analysis.

Brain &amp; development
2020

[Prenatal diagnosis of a fetus with Pallister-Killian syndrome with combined cytogenetic and molecular methods].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2020

Seizures and Cardiomyopathy in a Patient with Pallister-Killian Syndrome due to Hexasomy 12p Mosaicism.

Molecular syndromology
2020

Clinical Variability of Pallister-Killian Syndrome in Two Egyptian Patients.

Journal of pediatric genetics
2020

[Prenatal diagnosis of a case of Pallister-Killian syndrome].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2020

Hypotonic infant with Pallister-Killian syndrome diagnosed by cytogenetic microarray, without pigmentary skin changes and malformations.

Journal of genetics
2020

The utility of genome-wide cell-free DNA screening in the prenatal diagnosis of Pallister-Killian syndrome.

Prenatal diagnosis
2019

Mosaicism: Reason for Normal Phenotypes in Carriers of Small Supernumerary Marker Chromosomes With Known Adverse Outcome. A Systematic Review.

Frontiers in genetics
2019

Prenatal diagnosis of Pallister-Killian syndrome using cord blood samples.

Molecular cytogenetics
2019

Postnatal clinical phenotype of five patients with Pallister-Killian Syndrome (tetrasomy 12p): Interest of array CGH for diagnosis and review of the literature.

Molecular genetics &amp; genomic medicine
2019

Ductus Venosus Agenesis as a Marker of Pallister-Killian Syndrome.

Medicina (Kaunas, Lithuania)
2019

Myoclonic epilepsy with photosensitivity in infants with Pallister-Killian Syndrome.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2019

[Combined chromosomal microarray analysis and fluorescence in situ hybridization for prenatal diagnosis of two cases with Pallister-Killian syndrome].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2019

Mosaic Trisomy 12 Associated with Overgrowth Detected in Fibroblast Cell Lines.

Cytogenetic and genome research
2019

Prenatal detection of right aortic arch.

Archives of gynecology and obstetrics
2019

Retrospectively investigating the 12-year experience of prenatal diagnosis of small supernumerary marker chromosomes through array comparative genomic hybridization.

Taiwanese journal of obstetrics &amp; gynecology
2019

Pallister-Killian Syndrome: The Diagnosis is in the Detail.

Klinische Padiatrie
2019

Advantages of Array Comparative Genomic Hybridization Using Buccal Swab DNA for Detecting Pallister-Killian Syndrome.

Annals of laboratory medicine
2018

For Debate: The Significance of Etiologic Diagnosis in Neonates with Overgrowth Syndromes. Lesson Learned from the Simpson-Golabi-Behmel Syndrome.

Pediatric endocrinology reviews : PER
2018

Prenatal diagnosis of Pallister-Killian syndrome in pregnancy with normal CVS result and abnormal ultrasound findings in the second trimester.

Taiwanese journal of obstetrics &amp; gynecology
2018

Prenatal profile of Pallister-Killian syndrome: Retrospective analysis of 114 pregnancies, literature review and approach to prenatal diagnosis.

American journal of medical genetics. Part A
2018

Prenatal diagnosis of Pallister-Killian syndrome in one twin.

Clinical case reports
2018

Pallister-Killian syndrome: clinical, cytogenetic and molecular findings in 15 cases.

Molecular cytogenetics
2019

Prenatal diagnosis of Pallister-Killian syndrome.

Ultrasound in obstetrics &amp; gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
2019

Pallister-Killian syndrome: Review of fetal phenotype.

Clinical genetics
2018

[Prenatally diagnosed case of Pallister‒Killian syndrome].

Orvosi hetilap
2018

[Application of single nucleotide polymorphism microarray and fluorescence in situ hybridization analysis for the prenatal diagnosis of a case with Pallister-Killian syndrome].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2018

[Pallister-Killian syndrome in a Mexican mestizo patient. Case report].

Archivos argentinos de pediatria
2018

Neuroimaging findings in Pallister-Killian syndrome.

The neuroradiology journal
2017

Fetoplacental cytogenetic discrepancy in a pregnancy with fetal mosaic tetrasomy 12p and Pallister-Killian syndrome detected by amniocentesis.

Taiwanese journal of obstetrics &amp; gynecology
2018

A review of structural brain abnormalities in Pallister-Killian syndrome.

Molecular genetics &amp; genomic medicine
2018

Progressive subglottic stenosis in a child with Pallister-Killian syndrome.

Congenital anomalies
2018

Next generation phenotyping in Emanuel and Pallister-Killian syndrome using computer-aided facial dysmorphology analysis of 2D photos.

Clinical genetics
2017

Pallister-Killian syndrome in a two-year-old boy.

Clinical case reports
2017

Unclassifiable pattern of hypopigmentation in a patient with mosaic partial 12p tetrasomy without Pallister-Killian syndrome.

American journal of medical genetics. Part A
2017

[Clinical analysis of 21 cases with short fetal femur in the third trimester].

Zhonghua fu chan ke za zhi
2017

Targeted prenatal diagnosis of Pallister-Killian syndrome.

Prenatal diagnosis
2016

Pallister-Killian syndrome: Cytogenetics and molecular investigations of mosaic tetrasomy 12p in prenatal chorionic villus and in amniocytes. Strategy of prenatal diagnosis.

Taiwanese journal of obstetrics &amp; gynecology
2017

Using Array-Based Comparative Genomic Hybridization to Diagnose Pallister-Killian Syndrome.

Annals of laboratory medicine
2016

[Prenatal diagnosis of a Pallister-Killian syndrome case through analysis of a supernumerary chromosome using single nucleotide polymorphism array].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2016

Epidermal Nevus Presenting in a Pediatric Patient With Pallister-Killian Syndrome.

Skinmed
2016

Oro-dental features of Pallister-Killian syndrome: Evaluation of 21 European probands.

American journal of medical genetics. Part A
2016

Mosaic ratio quantification of isochromosome 12p in Pallister-Killian syndrome using droplet digital PCR.

Molecular genetics &amp; genomic medicine
2016

[Prenatal and clinicopathological study of 6 cases of Pallister-Killian syndrome and review].

Gynecologie, obstetrique &amp; fertilite
2016

Dental Treatment of a Child with Pallister-Killian Syndrome.

Case reports in dentistry
2016

Methylation and expression analyses of Pallister-Killian syndrome reveal partial dosage compensation of tetrasomy 12p and hypomethylation of gene-poor regions on 12p.

Epigenetics
2016

Ultrasonographic and Cytogenetic Issues in Prenatal Diagnosis of Pallister Killian Syndrome.

Genetic counseling (Geneva, Switzerland)
2016

Prenatally identified Pallister-Killian syndrome: Ultrasound pattern and diagnostic considerations.

Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and Gynaecology
2015

Cytogenomic delineation and clinical follow-up of 10 Brazilian patients with Pallister-Killian syndrome.

Molecular cytogenetics
2015

Congenital diaphragmatic hernia in a case of patau syndrome: a rare association.

Journal of neonatal surgery
2015

Elevation of insulin-like growth factor binding protein-2 level in Pallister-Killian syndrome: implications for the postnatal growth retardation phenotype.

American journal of medical genetics. Part A
2015

Pallister-Killian syndrome: a study of 22 British patients.

Journal of medical genetics
Ver todos os 195 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Síndrome Pallister-Killian.

É de uma associação que acompanha esta doença? Fale com a gente →

Comunidades

Grupos ativos de quem convive com esta doença aqui no Raras

Ainda não existe comunidade no Raras para Síndrome Pallister-Killian

Pacientes, familiares e cuidadores se organizam em comunidades pra compartilhar experiências, fazer perguntas e se apoiar. Você pode ser o primeiro.

Tire suas dúvidas

Perguntas, dicas e experiências compartilhadas aqui na página

Participe da discussão

Faça login para postar dúvidas, compartilhar experiências e interagir com especialistas.

Fazer login

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Ocular Manifestations of Pallister-Killian Syndrome.
    Ophthalmology. Retina· 2025· PMID 39580727mais citado
  2. Defining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.
    Cell reports· 2024· PMID 38861385mais citado
  3. Structural brain abnormalities in Pallister-Killian syndrome: a neuroimaging study of 31 children.
    Orphanet journal of rare diseases· 2024· PMID 38459574mais citado
  4. Human Genetics of Ventricular Septal Defect.
    Advances in experimental medicine and biology· 2024· PMID 38884729mais citado
  5. Pineocytoma in a child with Pallister-Killian syndrome: a case report and review of the literature.
    Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery· 2024· PMID 38689102mais citado
  6. Pallister-Killian Syndrome.
    Neoreviews· 2024· PMID 39482245recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:884(Orphanet)
  2. OMIM OMIM:601803(OMIM)
  3. MONDO:0011146(MONDO)
  4. GARD:8421(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q1425018(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome Pallister-Killian
Compêndio · Raras BR

Síndrome Pallister-Killian

ORPHA:884 · MONDO:0011146
Prevalência
Unknown
Herança
Not applicable, Unknown
CID-10
Q99.8 · Outras anomalias cromossômicas especificadas
CID-11
Início
Antenatal, Neonatal
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0265449
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

Nenhuma novidade ainda. O agente esta monitorando.

0membros
0novidades