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.
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.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 53 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 143 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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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
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Síndrome Pallister-Killian
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Pesquisa e ensaios clínicos
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Publicações mais relevantes
Ocular Manifestations of Pallister-Killian Syndrome.
Defining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.
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.
Structural brain abnormalities in Pallister-Killian syndrome: a neuroimaging study of 31 children.
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.
Human Genetics of Ventricular Septal Defect.
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.
Pineocytoma in a child with Pallister-Killian syndrome: a case report and review of the literature.
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
Ocular Manifestations of Pallister-Killian Syndrome.
Pallister-Killian Syndrome.
Human Genetics of Ventricular Septal Defect.
Defining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.
Pineocytoma in a child with Pallister-Killian syndrome: a case report and review of the literature.
📚 EuropePMC195 artigos no totalmostrando 74
Ocular Manifestations of Pallister-Killian Syndrome.
Ophthalmology. RetinaPallister-Killian Syndrome.
NeoReviewsHuman Genetics of Ventricular Septal Defect.
Advances in experimental medicine and biologyDefining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.
Cell reportsPineocytoma 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 NeurosurgeryA case report of Pallister-Killian syndrome with an unusual mosaic supernumerary marker chromosome 12 with interstitial 12p13.1-p12.1 duplication.
Frontiers in geneticsStructural brain abnormalities in Pallister-Killian syndrome: a neuroimaging study of 31 children.
Orphanet journal of rare diseasesTesting With Intent in Mosaic Conditions: A Case-Based Review.
CureusCo-Occurrence of Pallister-Killian Syndrome and Burkitt Lymphoma in a Patient with Near-Normal Neurocognitive Development.
Molecular syndromologyPrenatal diagnosis of a case with complete and uniform tetrasomy 12p by the utility of noninvasive prenatal testing.
Journal of assisted reproduction and geneticsSyndromic congenital diaphragmatic hernia: Current incidence and outcome. Analysis from the congenital diaphragmatic hernia study group registry.
Prenatal diagnosisCase Report: Early Neonatal EEG in Two Infants with Pallister Killian Syndrome (PKS).
HRB open researchRapid whole genome sequencing of critically ill pediatric patients from genetically underrepresented populations.
Genome medicineCongenital Diaphragmatic Hernia Repair in a Patient With Pallister-Killian Mosaic Syndrome and Left Ventricular Hypoplasia.
CureusCase Report: A Case Study on the Neurodevelopmental Profile of a Child With Pallister-Killian Syndrome and His Unaffected Twin.
Frontiers in pediatricsCognitive, Behavioral, and Sensory Profile of Pallister-Killian Syndrome: A Prospective Study of 22 Individuals.
GenesSleep in Children With Pallister Killian Syndrome: A Prospective Clinical and Videopolysomnographic Study.
Frontiers in neurologyDental Clinical and Radiographic Findings in a Patient with Pallister Killian Syndrome and 45, X/46, XY Mosaicism.
Journal of dentistry for children (Chicago, Ill.)[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 geneticsPrenatal diagnosis of Pallister-Killian syndrome and literature review.
Journal of cellular and molecular medicinePallister-Killian Syndrome versus Trisomy 12p-A Clinical Study of 5 New Cases and a Literature Review.
GenesA Novel Case of Tethered Cord in a Five-Month-Old Male With Pallister-Killian Syndrome.
CureusPolymicrogyria with calcification in Pallister-Killian syndrome detected by microarray analysis.
Brain & development[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 geneticsSeizures and Cardiomyopathy in a Patient with Pallister-Killian Syndrome due to Hexasomy 12p Mosaicism.
Molecular syndromologyClinical Variability of Pallister-Killian Syndrome in Two Egyptian Patients.
Journal of pediatric genetics[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 geneticsHypotonic infant with Pallister-Killian syndrome diagnosed by cytogenetic microarray, without pigmentary skin changes and malformations.
Journal of geneticsThe utility of genome-wide cell-free DNA screening in the prenatal diagnosis of Pallister-Killian syndrome.
Prenatal diagnosisMosaicism: Reason for Normal Phenotypes in Carriers of Small Supernumerary Marker Chromosomes With Known Adverse Outcome. A Systematic Review.
Frontiers in geneticsPrenatal diagnosis of Pallister-Killian syndrome using cord blood samples.
Molecular cytogeneticsPostnatal clinical phenotype of five patients with Pallister-Killian Syndrome (tetrasomy 12p): Interest of array CGH for diagnosis and review of the literature.
Molecular genetics & genomic medicineDuctus Venosus Agenesis as a Marker of Pallister-Killian Syndrome.
Medicina (Kaunas, Lithuania)Myoclonic epilepsy with photosensitivity in infants with Pallister-Killian Syndrome.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society[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 geneticsMosaic Trisomy 12 Associated with Overgrowth Detected in Fibroblast Cell Lines.
Cytogenetic and genome researchPrenatal detection of right aortic arch.
Archives of gynecology and obstetricsRetrospectively investigating the 12-year experience of prenatal diagnosis of small supernumerary marker chromosomes through array comparative genomic hybridization.
Taiwanese journal of obstetrics & gynecologyPallister-Killian Syndrome: The Diagnosis is in the Detail.
Klinische PadiatrieAdvantages of Array Comparative Genomic Hybridization Using Buccal Swab DNA for Detecting Pallister-Killian Syndrome.
Annals of laboratory medicineFor Debate: The Significance of Etiologic Diagnosis in Neonates with Overgrowth Syndromes. Lesson Learned from the Simpson-Golabi-Behmel Syndrome.
Pediatric endocrinology reviews : PERPrenatal diagnosis of Pallister-Killian syndrome in pregnancy with normal CVS result and abnormal ultrasound findings in the second trimester.
Taiwanese journal of obstetrics & gynecologyPrenatal profile of Pallister-Killian syndrome: Retrospective analysis of 114 pregnancies, literature review and approach to prenatal diagnosis.
American journal of medical genetics. Part APrenatal diagnosis of Pallister-Killian syndrome in one twin.
Clinical case reportsPallister-Killian syndrome: clinical, cytogenetic and molecular findings in 15 cases.
Molecular cytogeneticsPrenatal diagnosis of Pallister-Killian syndrome.
Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and GynecologyPallister-Killian syndrome: Review of fetal phenotype.
Clinical genetics[Prenatally diagnosed case of Pallister‒Killian syndrome].
Orvosi hetilap[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[Pallister-Killian syndrome in a Mexican mestizo patient. Case report].
Archivos argentinos de pediatriaNeuroimaging findings in Pallister-Killian syndrome.
The neuroradiology journalFetoplacental cytogenetic discrepancy in a pregnancy with fetal mosaic tetrasomy 12p and Pallister-Killian syndrome detected by amniocentesis.
Taiwanese journal of obstetrics & gynecologyA review of structural brain abnormalities in Pallister-Killian syndrome.
Molecular genetics & genomic medicineProgressive subglottic stenosis in a child with Pallister-Killian syndrome.
Congenital anomaliesNext generation phenotyping in Emanuel and Pallister-Killian syndrome using computer-aided facial dysmorphology analysis of 2D photos.
Clinical geneticsPallister-Killian syndrome in a two-year-old boy.
Clinical case reportsUnclassifiable pattern of hypopigmentation in a patient with mosaic partial 12p tetrasomy without Pallister-Killian syndrome.
American journal of medical genetics. Part A[Clinical analysis of 21 cases with short fetal femur in the third trimester].
Zhonghua fu chan ke za zhiTargeted prenatal diagnosis of Pallister-Killian syndrome.
Prenatal diagnosisPallister-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 & gynecologyUsing Array-Based Comparative Genomic Hybridization to Diagnose Pallister-Killian Syndrome.
Annals of laboratory medicine[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 geneticsEpidermal Nevus Presenting in a Pediatric Patient With Pallister-Killian Syndrome.
SkinmedOro-dental features of Pallister-Killian syndrome: Evaluation of 21 European probands.
American journal of medical genetics. Part AMosaic ratio quantification of isochromosome 12p in Pallister-Killian syndrome using droplet digital PCR.
Molecular genetics & genomic medicine[Prenatal and clinicopathological study of 6 cases of Pallister-Killian syndrome and review].
Gynecologie, obstetrique & fertiliteDental Treatment of a Child with Pallister-Killian Syndrome.
Case reports in dentistryMethylation and expression analyses of Pallister-Killian syndrome reveal partial dosage compensation of tetrasomy 12p and hypomethylation of gene-poor regions on 12p.
EpigeneticsUltrasonographic and Cytogenetic Issues in Prenatal Diagnosis of Pallister Killian Syndrome.
Genetic counseling (Geneva, Switzerland)Prenatally identified Pallister-Killian syndrome: Ultrasound pattern and diagnostic considerations.
Journal of obstetrics and gynaecology : the journal of the Institute of Obstetrics and GynaecologyCytogenomic delineation and clinical follow-up of 10 Brazilian patients with Pallister-Killian syndrome.
Molecular cytogeneticsCongenital diaphragmatic hernia in a case of patau syndrome: a rare association.
Journal of neonatal surgeryElevation 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 APallister-Killian syndrome: a study of 22 British patients.
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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.
- Ocular Manifestations of Pallister-Killian Syndrome.
- Defining the cellular origin of seminoma by transcriptional and epigenetic mapping to the normal human germline.
- Structural brain abnormalities in Pallister-Killian syndrome: a neuroimaging study of 31 children.
- Human Genetics of Ventricular Septal Defect.
- 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
- Pallister-Killian Syndrome.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:884(Orphanet)
- OMIM OMIM:601803(OMIM)
- MONDO:0011146(MONDO)
- GARD:8421(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
