Síndrome de Turner, também denominada 45,X ou 45,X0, é uma condição genética em mulheres caracterizada pela ausência total ou parcial de um cromossoma X. Os sinais e sintomas variam de pessoa para pessoa. Os sintomas mais comuns desde o nascimento são inchaço do dorso das mãos e dos pés, pregas redundantes na nuca, pescoço alado, tórax largo, mamilos invertidos e hipertelorismo mamário. Entre outros possíveis sintomas estão implantação baixa do cabelo na nuca, ptose, vários nevos pigmentados, quarto metacarpo e metatarso curtos e almofadas dos dedos proeminentes e com estrias. Durante o crescimento, as jovens geralmente apresentam baixa estatura.
Introdução
O que você precisa saber de cara
Síndrome do isocromossomo Y é uma condição genética rara associada à azoospermia, genitália ambígua, insuficiência gonadal primária e infertilidade masculina. Frequentemente cursa com ginecomastia, varicocele e tecido gonadal inadequado para o sexo cromossômico.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 4 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 8 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 — Isocromossomo Y
Centros de Referência SUS
24 centros habilitados pelo SUS para Isocromossomo Y
Centros para Isocromossomo Y
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
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.
Publicações mais relevantes
Spectrum and Clinical Reproductive Significance of Cytogenetic Abnormalities in Infertility and Recurrent Early Pregnancy Loss: A Five-Year Retrospective Study of 10,285 Cases.
Introduction Cytogenetic abnormalities are a major genetic cause of infertility and recurrent early pregnancy loss (REPL), conditions that affect a substantial proportion of couples of reproductive age. Both numerical and structural chromosomal abnormalities can impair gametogenesis, fertilization, implantation, or embryonic development. This study aimed to evaluate the frequency, spectrum, and clinical relevance of chromosomal abnormalities in individuals referred for infertility, single miscarriage, or recurrent pregnancy loss (RPL). Materials and methods This large retrospective cytogenetic study analyzed 10,285 individuals referred for infertility, single miscarriage, or recurrent pregnancy loss over a five-year period. Conventional G-banded karyotyping was performed in accordance with International System for Human Cytogenomic Nomenclature (ISCN) 2025 guidelines. Chromosomal abnormalities were classified as numerical or structural, with variants further categorized by specific chromosome involvement. Data on sex distribution and abnormality patterns were summarized to identify trends and clinically significant associations. Results Among the 10,285 individuals evaluated, cytogenetic abnormalities were identified in 980 cases, representing 9.5% of the study population. The cohort demonstrated a largely balanced sex distribution, with 46,XX karyotypes observed in 5,380 cases (52.3%) and 46,XY karyotypes in 4,857 cases (47.2%). Analysis of abnormal karyotypes revealed that chromosomal variants were the most frequent finding, accounting for 514 cases (52.4%), followed by variant inversions in 94 cases (9.6%). Structural chromosomal rearrangements were commonly observed and included reciprocal translocations in 120 cases (12.2%) and Robertsonian translocations in 33 cases (3.4%), involving multiple autosomes and sex chromosomes. Sex chromosome abnormalities comprised a notable subset, with Klinefelter syndrome identified in 53 cases (5.4%), Y-chromosome inversions in 36 cases (3.7%), mosaic karyotypes in 38 cases (3.9%), isochromosome X in 18 cases (1.8%), and additional X chromosomes in 12 cases (1.2%). Other abnormalities such as derivative chromosomes (24 cases, 2.4%), deletions (13 cases, 1.3%), autosomal inversions (14 cases, 1.4%), marker chromosomes (3 cases, 0.3%), and rare duplications or insertions (one case each, 0.1%) were observed less frequently, highlighting the broad spectrum of cytogenetic abnormalities associated with infertility and recurrent pregnancy loss. Conclusion This study demonstrates that chromosomal variants and structural rearrangements constitute the majority of cytogenetic abnormalities in individuals with infertility or recurrent pregnancy loss. Although clinically significant numerical and complex structural abnormalities were less frequent, they confer substantial reproductive risk through mechanisms affecting gametogenesis, chromosomal segregation, and embryonic viability.
Meta-Analysis: Liver Disease Burden and Associated Factors in Turner Syndrome.
The prevalence, incidence, and associated factors of liver disease (LD) in Turner syndrome (TS) remain uncertain. A meta-analysis was performed to quantify LD burden in TS. Four electronic databases were searched through June 2025 for observational studies involving karyotype-confirmed individuals with TS. LD was defined by raised serum liver enzymes (RLE), International Classification of Diseases codes, imaging, or histology. Pooled prevalence, incidence, and odds ratios (OR) with 95% confidence intervals (CI) were calculated. Forty studies from 19 countries with aggregate data on 9728 young TS individuals (median age 25.5 years, IQR 16.8-30.7) were included. Prevalences of RLE, steatotic LD (SLD), and significant/advanced liver fibrosis were 26.9% (95% CI 19.7-35.6), 22.3% (9.9-42.9), and 12.2% (2.6-41.7), respectively. Incidence of RLE was 16.7 per 1000 person-years (7.6-36.8). Compared to age-matched healthy controls, TS individuals had higher odds of RLE (OR 3.96 [95% CI 1.45-10.84]), SLD (OR 4.03 [1.86-8.70]), and significant/advanced fibrosis (OR 5.74 [2.99-11.01]). Compared to X monosomy, isochromosome Xq conferred a higher risk of RLE (OR 1.55 [1.15-2.10]), while mosaicism without structural abnormalities was protective (OR 0.54 [0.32-0.89]). Ring X or Y chromosome material carried risks like X monosomy. Hormone replacement therapy was not associated with an increased risk of RLE (OR 1.10 [0.79-1.53]). Liver disease is common in individuals with TS, with a 4-6 times higher risk than age-matched healthy females. X chromosome abnormalities are more strongly associated with an increased liver disease risk than hypogonadism.
Turner Syndrome.
Turner syndrome (TS) is a sex chromosome disorder affecting phenotypic females who have one intact X chromosome and a completely or partially missing second sex chromosome. It was first described approximately a century ago by Seresevskij, Ullrich and Turner. However, the cytogenetic basis of TS was only reported by Ford in 1959 following Tjio and Levan's optimisation of chromosome visualisation. TS karyotypes include classic monosomy X (40%-50%); monosomy X mosaicism (3%-25%); isochromosome X (10%-18%); ring X (10%-16%); mosaicism for monosomy X and a normal or structurally abnormal Y chromosome (6%-12%); deletion Xp (< 5%) and unbalanced X-autosome translocation (< 2%). While parental age does not affect the complete loss of one X chromosome, the paternal X chromosome is absent in three-quarters of patients with TS. Clinically, detecting the parental origin of the remaining X chromosome is not currently useful in routine TS care. Recurrence risk is low for phenotypically normal parents with a child diagnosed with TS. Pregnancy loss is the outcome for the majority (~99%) of TS cases; however, prenatal ultrasound findings for foetuses with TS may include abnormalities like cystic hygroma and hydrops. Postnatal phenotype for patients with TS includes short stature, delayed puberty, ovarian dysgenesis, hypergonadotropic hypogonadism, infertility, cardiac defects, endocrine, metabolic and autoimmune disorders. This review aims to outline clinical indications for testing, describe test methodologies, provide genetic test result examples that highlight complex TS karyotype diagnoses, summarise clinical management options and discuss the phenomenon of 'normal' sex chromosome loss with advancing age.
Variant Klinefelter Syndrome With Xq Trisomy (47,X,i(X)(q10),Y): A Case Report and Review of the Literature.
Variant Klinefelter syndrome (VKS) presents with variable phenotypes, caused by the involvement of different genetic abnormalities leading to delays in confirming diagnosis and, therefore, delays in early management of this disorder. We describe a male with VKS and trisomy Xq resulting from an isochromosome X(i(Xq10)). He had characteristics of classical KS such as azoospermia, hypergonadotropic hypogonadism with elevated follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels, and bilateral atrophic testes. He achieved a normal intelligence level. However, we observed a normal stature appropriate for his height. There was no history of developmental delays or learning problems during childhood. His karyotype was found to be 47,X,i(X)(q10),Y, which indicates that extra copies of the long arm of Xq (trisomy Xq) have phenotypic expression. In conclusion, Trisomy Xq does not affect stature and neurological development and does not express classical phenotypes during early childhood. Hence, the cytogenetic screening of sex chromosomal abnormalities is important in cases of hypergonadotropic hypogonadism and testicle abnormality, which can help in appropriate genetic counseling.
Elevated levels of neutrophils with a pro-inflammatory profile in Turner syndrome across karyotypes.
Turner syndrome (TS) presents with multiple karyotypes, including 45,X monosomy and variants such as isochromosomes and mosaicism, and is characterized by several co-morbidities, including metabolic conditions and autoimmunity. Here, we investigated the genomic landscapes across a range of karyotypes. We show that TS have a common autosomal methylome and transcriptome, despite distinct karyotypic variations. All TS individuals lacked the X chromosome p-arm, and XIST expression from the q-arm did not affect the autosomal transcriptome or methylome, highlighting the critical role of the missing p-arm with its pseudoautosomal region 1. Furthermore, we show increased levels of neutrophils and increased neutrophil activation. The increase in neutrophils was linked to TS clinical traits and to increased expression of the X-Y homologous gene TBL1X, suggesting a genetic basis, which may lead to neutrophil-driven inflammatory stress in TS. Identifying TS individuals with increased neutrophil activation could potentially mitigate the progression towards more severe metabolic issues.
Publicações recentes
Clinical and molecular cytogenetic findings and pregnancy outcomes of fetuses with isochromosome Y.
Rare case of monocentric isochromosome Y with inversion duplication of p arm in patient diagnosed with azoospermia.
Localization of the SRY Gene on Chromosome 3 in a Patient with Azoospermia and a Complex Karyotype 45,X/46,X,i(Y)(q10)/46,XX/ 47,XX,i(Y)(q10).
Dynamic mosaicism manifesting as loss, gain and rearrangement of an isodicentric Y chromosome in a male child with growth retardation and abnormal external genitalia.
Isochromosome Y in an infertile heifer.
📚 EuropePMCmostrando 17
Spectrum and Clinical Reproductive Significance of Cytogenetic Abnormalities in Infertility and Recurrent Early Pregnancy Loss: A Five-Year Retrospective Study of 10,285 Cases.
CureusMeta-Analysis: Liver Disease Burden and Associated Factors in Turner Syndrome.
Alimentary pharmacology & therapeuticsVariant Klinefelter Syndrome With Xq Trisomy (47,X,i(X)(q10),Y): A Case Report and Review of the Literature.
CureusElevated levels of neutrophils with a pro-inflammatory profile in Turner syndrome across karyotypes.
NPJ genomic medicineReproductive health in Turner's syndrome: from puberty to pregnancy.
Frontiers in endocrinologyClinical profile and cytogenetic correlations in females with primary amenorrhea.
Clinical and experimental reproductive medicineCardiovascular Manifestations of Turner Syndrome: Phenotypic Differences Between Karyotype Subtypes.
Pediatric cardiologyTurner Syndrome Mosaicism 45,X/46,XY with Genital Ambiguity and Duchenne Muscular Dystrophy: Translational Approach of a Rare Italian Case.
International journal of molecular sciencesTurner syndrome: French National Diagnosis and Care Protocol (NDCP; National Diagnosis and Care Protocol).
Orphanet journal of rare diseases45,X/46,X,i(Yp): Importance of Assessment and Support during Puberty and Adolescence.
Sexual development : genetics, molecular biology, evolution, endocrinology, embryology, and pathology of sex determination and differentiationKaryotype - Phenotype Associations in Patients with Turner Syndrome.
Pediatric endocrinology reviews : PERA rare case in literature: Isochromosome Xq in Klinefelter syndrome.
AndrologiaA case of a rare variant of Klinefelter syndrome, 47,XY,i(X)(q10).
AndrologiaOtologic disorders in Turner syndrome.
European annals of otorhinolaryngology, head and neck diseasesWhole-Exome Sequencing for Diagnosis of Turner Syndrome: Toward Next-Generation Sequencing and Newborn Screening.
The Journal of clinical endocrinology and metabolismRare cytogenetic abnormalities in myelodysplastic syndromes.
Mediterranean journal of hematology and infectious diseases[Mosaic isochromosome Xq and microduplication 17p13.3p13.2 in a patient with Turner syndrome and congenital cataract].
Archivos argentinos de pediatriaAssociações
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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.
- Spectrum and Clinical Reproductive Significance of Cytogenetic Abnormalities in Infertility and Recurrent Early Pregnancy Loss: A Five-Year Retrospective Study of 10,285 Cases.
- Meta-Analysis: Liver Disease Burden and Associated Factors in Turner Syndrome.
- Turner Syndrome.
- Variant Klinefelter Syndrome With Xq Trisomy (47,X,i(X)(q10),Y): A Case Report and Review of the Literature.
- Elevated levels of neutrophils with a pro-inflammatory profile in Turner syndrome across karyotypes.
- Clinical and molecular cytogenetic findings and pregnancy outcomes of fetuses with isochromosome Y.
- Rare case of monocentric isochromosome Y with inversion duplication of p arm in patient diagnosed with azoospermia.
- Localization of the SRY Gene on Chromosome 3 in a Patient with Azoospermia and a Complex Karyotype 45,X/46,X,i(Y)(q10)/46,XX/ 47,XX,i(Y)(q10).
- Dynamic mosaicism manifesting as loss, gain and rearrangement of an isodicentric Y chromosome in a male child with growth retardation and abnormal external genitalia.
- Isochromosome Y in an infertile heifer.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:96325(Orphanet)
- MONDO:0019935(MONDO)
- GARD:19349(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55789005(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
