A síndrome de deleção 22q11.2 (SD) é uma anomalia cromossômica que causa um distúrbio de malformação congênita cujas características comuns incluem defeitos cardíacos, anomalias palatais, dismorfismo facial, atraso no desenvolvimento e deficiência imunológica.
Introdução
O que você precisa saber de cara
A síndrome da deleção 22q11.2 (que também é conhecida por vários outros nomes, listados abaixo) é um distúrbio causado pela deleção de um pequeno fragmento do cromossomo 22. A deleção ocorre próximo ao meio do cromossomo em uma localização designada q11.2.
A síndrome da deleção 22q11.2 tem muitos sinais e sintomas possíveis que podem afetar quase qualquer parte do corpo. As características desta síndrome variam amplamente, mesmo entre membros afetados da mesma família. Pessoas com a síndrome da deleção 22q11.2 costumam ter anomalias cardíacas que frequentemente estão presentes desde o nascimento, infecções recorrentes causadas por problemas no sistema imunológico e características faciais distintas. Nos indivíduos afetados, os músculos que formam o teto da boca (palato) podem não se fechar completamente, embora o tecido que os cobre o faça, resultando em uma condição chamada palato fissurado submucoso. O palato anormal costuma ser altamente arqueado e pode haver uma divisão na lâmina macia de tecido que pendura da parte posterior da boca (úvula bifida). O palato fissurado submucoso também pode interferir na fala normal ao fazer com que o ar escape pelo nariz durante a fala, levando a uma fala com som nasal. Indivíduos afetados podem ainda apresentar problemas respiratórios, anomalias renais, baixos níveis de cálcio no sangue (o que pode resultar em convulsões), diminuição das plaquetas sanguíneas (trombocitopenia), dificuldades alimentares significativas, problemas gastrointestinais e perda auditiva. Diferenças esqueléticas são possíveis, incluindo baixa estatura leve e, com menos frequência, anomalias nos ossos da coluna vertebral.
Muitas crianças com a síndrome da deleção 22q11.2 apresentam atrasos no desenvolvimento, incluindo crescimento retardado e desenvolvimento da fala atrasado, e algumas têm deficiência intelectual leve ou dificuldades de aprendizagem. Indivíduos mais velhos afetados têm dificuldade para ler, realizar tarefas que envolvem matemática e resolver problemas. Crianças com esta condição costumam precisar de ajuda para mudar e adaptar seus comportamentos ao responder a situações. Além disso, crianças afetadas têm maior probabilidade, em comparação com crianças sem a síndrome da deleção 22q11.2, de apresentar transtorno de déficit de atenção/hiperatividade (TDAH) e condições de desenvolvimento como transtorno do espectro autista, que afetam a comunicação e a interação social.
Como os sinais e sintomas da síndrome da deleção 22q11.2 são tão variados, diferentes agrupamentos de características foram, no passado, descritos como condições separadas. Os médicos nomearam essas condições como síndrome de DiGeorge, síndrome velocardiofacial (também chamada de síndrome de Shprintzen) e síndrome do rosto com anomalia conotruncal. Além disso, algumas crianças com a deleção 22q11.2 foram diagnosticadas com a forma autossômica dominante da síndrome de Opitz G/BBB e a síndrome cardiofacial de Cayler. Quando a base genética desses distúrbios foi identificada, os médicos determinaram que todas elas faziam parte de uma única síndrome com muitos sinais e sintomas possíveis. Para evitar confusão, esta condição costuma ser chamada de síndrome da deleção 22q11.2, uma descrição baseada em sua causa genética subjacente.
Fonte: MedlinePlus Genetics (NLM/NIH), traduzidoA síndrome de deleção 22q11.2 (SD) é uma anomalia cromossômica que causa um distúrbio de malformação congênita cujas características comuns incluem defeitos cardíacos, anomalias palatais, dismorfismo facial, atraso no desenvolvimento e deficiência imunológica.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 76 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 234 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
12 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant.
Leukemia, chronic myeloid
A clonal myeloproliferative disorder of a pluripotent stem cell with a specific cytogenetic abnormality, the Philadelphia chromosome (Ph), involving myeloid, erythroid, megakaryocytic, B-lymphoid, and sometimes T-lymphoid cells, but not marrow fibroblasts.
Noonan syndrome 13
A form of Noonan syndrome, a disease characterized by short stature, facial dysmorphic features such as hypertelorism, a downward eyeslant and low-set posteriorly rotated ears, and a high incidence of congenital heart defects and hypertrophic cardiomyopathy. Other features can include a short neck with webbing or redundancy of skin, deafness, motor delay, variable intellectual deficits, multiple skeletal defects, cryptorchidism, and bleeding diathesis. Individuals with Noonan syndrome are at risk of juvenile myelomonocytic leukemia, a myeloproliferative disorder characterized by excessive production of myelomonocytic cells. NS13 inheritance is autosomal dominant. There is considerable variability in severity.
Schizophrenia
A complex, multifactorial psychotic disorder or group of disorders characterized by disturbances in the form and content of thought (e.g. delusions, hallucinations), in mood (e.g. inappropriate affect), in sense of self and relationship to the external world (e.g. loss of ego boundaries, withdrawal), and in behavior (e.g bizarre or apparently purposeless behavior). Although it affects emotions, it is distinguished from mood disorders in which such disturbances are primary. Similarly, there may be mild impairment of cognitive function, and it is distinguished from the dementias in which disturbed cognitive function is considered primary. Some patients manifest schizophrenic as well as bipolar disorder symptoms and are often given the diagnosis of schizoaffective disorder.
Bernard-Soulier syndrome
An autosomal recessive coagulation disorder characterized by a prolonged bleeding time, unusually large platelets, thrombocytopenia, and impaired prothrombin consumption.
Medicamentos e terapias
Mecanismo: Parathyroid hormone receptor agonist
Mecanismo: Inosine-5'-monophosphate dehydrogenase (IMPDH) inhibitor
Mecanismo: Cyclophilin A modulator
Mecanismo: FK506-binding protein 1A inhibitor
Mecanismo: Glucocorticoid receptor agonist
Mecanismo: Interleukin-2 receptor inhibitor
Mecanismo: Interleukin-2 receptor inhibitor
Variantes genéticas (ClinVar)
606 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 14 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
71 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 1.292
Interhemispheric CA1 projections to the subiculum support spatial cognition and are affected in a mouse model of the 22q11.2 deletion syndrome.
Mapping hippocampal connectivity is essential to understand the neural mechanisms of learning and memory, yet interhemispheric connections between hippocampal formations remain poorly defined. In rodents, two main commissural pathways are known: dentate gyrus hilar mossy cells project to the inner molecular layer of the contralateral dentate gyrus, and CA2/CA3 pyramidal neurons send collaterals to contralateral CA3, CA2, and CA1 regions. By contrast, commissural outputs from CA1 remain largely unexplored. Here, we show that dorsal CA1 (dCA1) pyramidal neurons located in the right hemisphere project to contralateral dorsal subiculum (dSUB) in addition to contralateral dCA1. We then assess the function of the projection from the right dCA1 to the left dSUB and find that this interhemispheric pathway supports spatial memory and spatial working memory, two cognitive functions altered in the Df16(A)+/- mouse model of 22q11.2 deletion syndrome (22q11.2DS) associated with schizophrenia. Notably, the right-to-left dCA1 interhemispheric projections are disrupted in Df16(A)+/- mice, suggesting that dysregulation of this circuit may contribute to 22q11.2DS-related cognitive deficits.
Charting Brain Structure in 22q11.2 Deletion Syndrome with Clinical Neuroimaging.
22q11.2 deletion syndrome (22q11DS) is a common microdeletion associated with widespread brain alterations and elevated risk for schizophrenia and other neuropsychiatric conditions. Prospective research studies often exclude individuals with severe cognitive impairment, medical comorbidities, or inability to tolerate research MRI without sedation, features common in 22q11DS. This limits both the generalizability of neuroimaging findings and our understanding of the full phenotypic spectrum. Moreover, while standard brain growth charts quantify deviation from typical development, they cannot identify patients who are disproportionately affected relative to their genetic peers, limiting clinical utility for risk stratification. Leveraging clinical MRI data offers a scalable approach to address these gaps. We analyzed 92 patients with 22q11DS (age 0.5-21 years, 49% female) and 252 matched clinical controls. Using normative modeling derived from 1,995 reference clinical scans, we quantified individual-level brain deviations from population norms. We validated clinical findings against the independent ENIGMA-22q research consortium, characterized rates of extreme structural deviations to assess within-syndrome heterogeneity, correlated spatial patterns of brain alterations with gene expression from the Allen Human Brain Atlas, and generated syndrome-specific growth charts to test whether deviations from syndrome-specific norms predicted cognitive and language outcomes. Patients with 22q11DS showed widespread reductions in brain volumes (max Cohen's d=-1.31) and cortical surface area (d=-0.71) with increased cortical thickness (d=0.39). These findings were highly convergent with the ENIGMA-22q research cohort (r=0.61-0.87). Forty percent of patients showed at least one global brain measure below the 2.5th percentile. Spatial patterns of cortical volume and surface area correlated with the expression of genes within the 22q11.2 locus. Critically, syndrome-specific growth charts revealed that smaller cerebellar volume relative to 22q11DS peers predicted lower language scores across two independent assessment methods (p<0.03), demonstrating potential prognostic utility. This study provides a critical proof of principle for using heterogeneous clinical imaging to robustly characterize brain structure in rare genetic disorders. Syndrome-specific growth charts provide a novel framework to quantify within-syndrome variability and demonstrate potential prognostic value by linking individual brain structure to cognitive outcomes.
Challenging Case: Complex Developmental Care and Management in a Child With a Chromosomal Deletion.
DiGeorge for the Otolaryngologist: A State-of-the-Art Review.
To provide a state-of-the-art, otolaryngology-focused review of 22q11.2 deletion syndrome (22q11.2DS), emphasizing recent developments in the understanding of airway, hearing, velopharyngeal, sleep, and perioperative manifestations that directly influence contemporary ENT practice. PubMed, Scopus, and Cochrane Library were searched for English-language publications from January 2018 to September 2025. Additional data were identified through reference lists of key guidelines and landmark cohort studies. A narrative review approach was used to synthesize recent literature addressing the epidemiology, anatomy, diagnostic advances, and management of otologic, palatal, airway, sleep, swallowing, vascular, immune, and perioperative features of 22q11.2DS. Priority was given to studies published within the past 5 years, multicenter analyses, updated clinical practice guidelines, and investigations describing evolving surgical and diagnostic paradigms. Articles focusing solely on cardiac, endocrine, or psychiatric aspects were excluded unless directly relevant to otolaryngologic care. 22q11.2DS presents a uniquely high burden of ENT disease across the lifespan, including chronic otitis media, elevated cholesteatoma risk, persistent velopharyngeal dysfunction, multilevel airway anomalies, sleep-disordered breathing, dysphagia, and perioperative vulnerability due to vascular, immunologic, and hematologic anomalies. Emerging imaging techniques, updated pediatric and adult guidelines, and expanded genotype-phenotype data have reshaped diagnostic and surgical strategies. Otolaryngologists play a central role in lifelong care of individuals with 22q11.2DS. Structured surveillance, genotype-informed imaging, proactive sleep and airway assessment, and multidisciplinary perioperative planning are essential for improving outcomes. Standardized pathways and longitudinal, ENT-specific research are urgently needed to guide evidence-based management.
Different Diagnoses, Common Ancestry: 22q11.2 Deletion Syndrome and Wiskott-Aldrich Syndrome in the Same Family.
Newborn screening program enable pre-symptomatic detection patients with severe forms of T and B cell immunodeficiency. Despite the high prevalence of 22q11.2 deletion syndrome, only 1 in 10 patients develop lymphopenia that can be detected by newborn screening program. This report presents a unique familial case of 22q11.2 deletion syndrome and Wiskott-Aldrich syndrome, highlighting the importance genetic counseling and a detailed analysis of the family history. The article presents a case of the two rare genetic diseases in patients from the same family: 22q11.2 deletion syndrome in one and Wiskott-Aldrich syndrome in the other. Both diseases belong to the group of combined immunodeficiencies with syndromic features and are potentially life-threatening conditions requiring follow-up by a wide range of specialists: immunologists, geneticists, cardiologists, endocrinologists, and medical psychologists. Our work aimed to describe the clinical manifestations, genetic characteristics, and diagnosis of these disorders. It is worth emphasizing that the timely diagnosis of not only 22q11.2 deletion syndrome but also other primary immunodeficiencies in the Russian Federation became possible due to the introduction of extended neonatal screening into widespread practice. For such patients, comprehensive early interventional treatment and follow-up by a wide range of specialists is important to improve prognosis and quality of life.
Publicações recentes
Human neuromodulatory assembloids to study serotonin signaling and disease.
Integrated multiomics profiling of amniotic fluid exosomes reveals dysregulated lipid and protein signatures in fetal 22q11.2 deletion syndrome.
HIRA, NKX2-5, and GATA4 Alterations versus Cardiac Malformations Related to 22q11.2 Deletion Syndrome.
Hippocampal transcriptome profiling in a 22q11.2 deletion syndrome mouse model: comparison with human schizophrenia.
🥉 Relato de casoMedical challenges and unmet needs of individuals with 22q11.2 deletion syndrome as perceived by caregivers: A thematic analysis and natural language processing-based thematic extraction.
📚 EuropePMC1.275 artigos no totalmostrando 199
EEG power spectral and microstate analyses in the 22q11.2 deletion syndrome: from genetic risk to schizophrenia.
Journal of neural transmission (Vienna, Austria : 1996)Interhemispheric CA1 projections to the subiculum support spatial cognition and are affected in a mouse model of the 22q11.2 deletion syndrome.
Cell reportsDiGeorge for the Otolaryngologist: A State-of-the-Art Review.
Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck SurgeryDifferent Diagnoses, Common Ancestry: 22q11.2 Deletion Syndrome and Wiskott-Aldrich Syndrome in the Same Family.
Molecular syndromologyGenetic Mapping of the 22q11.2 Deletion Syndrome (DiGeorge Syndrome) Microdeletion Types Revealed Novel Candidate Breakpoints.
GenesAdaptive and Behavioral Phenotype in Pediatric 22q11.2 Deletion Syndrome: Characterizing a High-Risk Neurogenetic Copy Number Variant.
GenesThe 22q11.2 deletion syndrome: Genetic mechanisms, clinical manifestations, and therapeutic strategies.
Clinica chimica acta; international journal of clinical chemistryFollicular Helper T Cells and B Cell Maturation in Patients with 22q11.2 Deletion Syndrome and Recurrent Infections.
Journal of clinical immunology22q11.2 Deletion Syndrome in Offspring Conceived via Assisted Reproductive Technology Versus Spontaneously.
GenesNavigating Services in the UK: The Lived Experiences of Families Affected by 22q11.2 Deletion Syndrome.
Behavioral sciences (Basel, Switzerland)Quantitative susceptibility mapping of deep brain nuclei in 22q11.2 deletion syndrome.
Frontiers in psychiatryMulticenter retrospective study on effectiveness, reported side effects, and cognitive outcomes of SSRIs in 22q11.2 deletion syndrome.
Research squareChallenging Case: Complex Developmental Care and Management in a Child With a Chromosomal Deletion.
Journal of developmental and behavioral pediatrics : JDBPEvaluating the effectiveness of routine noninvasive prenatal screening for CNVs in 22q11.2 region in a cohort of 38,495 pregnancies.
Scientific reportsAripiprazole for treatment of attenuated psychosis syndrome in an adolescent with 22q11.21 deletion syndrome: A case report.
MedicineFrom DGCR8 expression analysis to diseased pathways in 22q11.2 deletion syndrome.
Frontiers in immunologyA Rare Case of Coexisting 22q11.2 Deletion Syndrome and Cornelia De Lange Syndrome: A Case Report and Review of the Literature.
CureusCharting Brain Structure in 22q11.2 Deletion Syndrome with Clinical Neuroimaging.
medRxiv : the preprint server for health sciencesUrinary metabolomic profiling in 22q11.2 deletion syndrome reveals microbial and mitochondrial signatures related to autism and psychosis risk.
PCN reports : psychiatry and clinical neurosciencesMovement Disorders Associated with 22q11.2 Microdeletion: A Scoping Review.
Movement disorders clinical practiceDevelopmental Selective GSK3α Inhibition Rescues Working Memory Deficits in a Mouse Model of Schizophrenia Predisposition.
Complex psychiatryOtolaryngologic disease in 22q11.2 deletion syndrome: spectrum, co-occurrence, and outcomes in a contemporary pediatric cohort.
European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck SurgeryMaternal Genotype and Dietary Vitamin A Modify Aortic Arch Phenotypes in a Mouse Model of 22q11DS.
International journal of molecular sciencesSingle cell RNA-seq analysis of hematopoietic cell types in 22q11.2 deletion syndrome reveals significant changes to non-T cells.
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and ImmunologyMexican Patients With Suspected 22q11.2 Deletion Syndrome: Clinical Characterization and Molecular Findings by Fluorescence In Situ Hybridization and Multiplex Ligation-Dependent Probe Amplification.
Molecular genetics & genomic medicineTracheal Compression on Chest X-ray Leading to the Diagnosis of Right Aortic Arch in 22q11.2 Deletion Syndrome.
CureusCognitive-Developmental Mechanisms in Hallucinations.
Schizophrenia bulletinNatural history study of scoliosis in patients with 22q11.2 deletion syndrome, starting before disease onset.
Spine deformityThe acoustic startle response in 22q11 deletion syndrome: from animal models to humans.
Frontiers in neuroscienceSuccessful use of clozapine in a patient with schizophrenia comorbid with 22q11.2 deletion syndrome and multiple periventricular nodular heterotopia: A case report.
PCN reports : psychiatry and clinical neurosciencesEvaluating the relationship between caregiver depression, social support, and children's internalizing and externalizing symptoms in families affected by 22q11.2 deletion syndrome.
Orphanet journal of rare diseasesInvestigating the relationship between sleep disturbances and psychopathology In children and adolescents with microdeletion of 22q11 chromosome: an exploratory study.
Frontiers in psychiatryParental Education Predicts Longitudinal IQ Trajectories in 22q11.2 Deletion Syndrome: A Three-Cohort European Study.
Journal of intellectual disability research : JIDRAutoantibodies in patients with 22q11.2 deletion syndrome and psychosis.
The Journal of allergy and clinical immunologyOxytocin seals the blood-brain barrier, improving 22q11.2 deletion syndrome trajectories.
Brain : a journal of neurologyDetermining the origin of genome aberrations improves the positive predictive value of NIPT for 22q11.2 deletion syndrome.
Scientific reportsCorrelation between 22q11.2 deletion syndrome phenotype and deletion location: a meta-analysis.
Archives of gynecology and obstetricsDissecting the Phenotypic Spectrum and Complexity of Movement Disorders in 22q11.2 Deletion Syndrome.
European journal of neurologyAge-Related Dysphagia Among Children with 22q11.2-Deletion Syndrome.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationPenetrance of Neurodevelopmental Copy Number Variants Is Associated With Variations in Cortical Morphology.
Biological psychiatry. Cognitive neuroscience and neuroimagingStreamlined Preoperative Evaluation of Velopharyngeal Dysfunction: A Single-Session Protocol for Speech MRI and Carotid MRA.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationPresence, Severity, and Functional Associations of Incomplete Hippocampal Inversion in 22q11.2 Deletion Syndrome.
Biological psychiatry. Cognitive neuroscience and neuroimagingSelf-Esteem and Psychopathology Differentially Relate to Real-Life and Social Functioning in People With 22q11.2 Deletion Syndrome.
International journal of developmental neuroscience : the official journal of the International Society for Developmental NeuroscienceAssessment of vascular tortuosity in 22q11.2 deletion syndrome using optical coherence tomography angiography.
Photodiagnosis and photodynamic therapyHealthcare Transition to Adulthood in Patients with 22q11.2 Deletion Syndrome: A Comprehensive Literature Review and Transition Framework.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationPrevalence of cholesteatoma in children with 22q11.2 deletion syndrome.
International journal of pediatric otorhinolaryngologyChildren With 22.Q.11.2 Deletion Syndrome: Sleep-Disordered Breathing and Management.
American journal of medical genetics. Part APhonemic-Phonological Profile of People with 22q11.2 Deletion Syndrome: A Pilot Study.
Brain sciencesInvestigation of 22q11.2 Deletion Syndrome in the first Moroccan Pediatric Patients series.
La Tunisie medicaleReview of the Pathophysiology and Clinical Manifestations of 22q11.2 Deletion and Duplication Syndromes.
Clinical reviews in allergy & immunologyPerception of psychosocial burden in mothers of children with rare pediatric neurological diseases.
Scientific reportsDriving Innovation to Support Pupils with SEND Through Co-Production in Education and Research: Participatory Action Research with 22q11.2 Deletion Syndrome Families in England.
Behavioral sciences (Basel, Switzerland)Development of a TaqMan-based dosage analysis PCR assay for the molecular diagnosis of 22q11.2 deletion syndrome.
Genes & genetic systemsSleep difficulties related to psychopathology and neurocognition in people with 22q11.2 deletion syndrome.
Psychiatry researchReal-Life Functioning in 22q11.2 Deletion Syndrome in Relation to Neurocognitive Abilities and Psychotic Symptoms: A Comparison With Idiopathic Schizophrenia.
Journal of intellectual disability research : JIDRInvestigating psychiatric morbidity, hopelessness and suicide risk in patients with 22q11.2 deletion syndrome: a case-control study.
International journal of psychiatry in clinical practiceMuscle Weakness in an Adult With 22q11.2 Deletion Syndrome.
CNS neuroscience & therapeuticsSensory Processing Challenges in Children with Neurodevelopmental Disorders and Genetic Conditions: An Observational Study.
NeuroSciHearing Loss in Children with 22q11.2 Deletion Syndrome.
The LaryngoscopeEuropean Society for Immunodeficiencies guidelines for the management of patients with congenital athymia.
The Journal of allergy and clinical immunologyAn Assessment of Adverse Events in Patients with 22q11.2 Deletion Syndrome Undergoing Palatoplasty: An Analysis of the NSQIP Pediatric Database.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationPlatelet changes and bleeding symptoms in children, adolescents, and adults with 22q11.2 deletion syndrome.
Pediatric blood & cancerThe Co-Occurrence of 22q11.2 Deletion Syndrome and Epithelial Basement Membrane Dystrophy: A Case Report and Review of the Literature.
Life (Basel, Switzerland)Treatment-resistant schizophrenia with 22q11.2 deletion and additional genetic defects.
Neuropsychopharmacology reportsUnique Functional Neuroimaging Signatures of Genetic Versus Clinical High Risk for Psychosis.
Biological psychiatryAn uncommon neuroradiological finding of hippocampal malrotation in childhood onset schizophrenia and 22q11.2 Deletion Syndrome: a case report and a brief review of the literature.
European child & adolescent psychiatryThe 22q11.2 Deletion Syndrome from A Biopsychosocial Perspective: A Series of Cases with an ICF-Based Approach.
Children (Basel, Switzerland)Comparison of the Accuracy in Provisional Diagnosis of 22q11.2 Deletion and Williams Syndromes by Facial Photos in Thai Population Between De-Identified Facial Program and Clinicians.
The application of clinical geneticsComprehensive insights into health services accessibility and quality of life of families with individuals with 22q11.2 deletion syndrome in Brazil.
Orphanet journal of rare diseasesCancer in 22q11.2 deletion syndrome: A case report and literature review.
European journal of medical geneticsExtent of magnitude representation deficit and relationship with arithmetic skills in children with 22q11.2DS.
Orphanet journal of rare diseasesAerodynamic Study of Velopharyngeal Insufficiency in 22q11.2 Deletion Syndrome.
Journal of personalized medicineComputer-vision analysis of craniofacial dysmorphology in 22q11.2 deletion syndrome and psychosis spectrum disorders.
Journal of neurodevelopmental disordersHuman Genetics of Truncus Arteriosus.
Advances in experimental medicine and biologyNext-generation sequencing profiling of miRNAs in individuals with 22q11.2 deletion syndrome revealed altered expression of miR-185-5p.
Human genomicsMulticenter appraisal of comorbid TANGO2 deficiency disorder in patients with 22q11.2 deletion syndrome.
American journal of medical genetics. Part ANeuropsychological Profile of 25 Brazilian Patients with 22q11.2 Deletion Syndrome: Effects of Clinical and Socioeconomic Variables.
GenesReduced amplitude and slowed latency of the acoustic startle response in adolescents and adults with 22q11.2 deletion syndrome.
Schizophrenia research22q11.2 Deletion Syndrome: Influence of Parental Origin on Clinical Heterogeneity.
GenesGenome Sequencing in an Individual Presenting with 22q11.2 Deletion Syndrome and Juvenile Idiopathic Arthritis.
GenesNeuroanatomical Correlates of Cognitive Dysfunction in 22q11.2 Deletion Syndrome.
GenesThe impact of social-environmental factors on IQ in syndromic intellectual developmental disabilities.
Journal of clinical and translational scienceBernard-Soulier syndrome caused by a novel GP1BB variant and 22q11.2 deletion.
International journal of hematologyA regulatory variant impacting TBX1 expression contributes to basicranial morphology in Homo sapiens.
American journal of human genetics22q11.2 Deletion-Associated Blood-Brain Barrier Permeability Potentiates Systemic Capillary Leak Syndrome Neurologic Features.
Journal of clinical immunologyUnderstanding the Variability of 22q11.2 Deletion Syndrome: The Role of Epigenetic Factors.
GenesPsychosis spectrum symptoms among individuals with schizophrenia-associated copy number variants and evidence of cerebellar correlates of symptom severity.
Psychiatry researchThalamic contributions to psychosis susceptibility: Evidence from co-activation patterns accounting for intra-seed spatial variability (μCAPs).
Human brain mappingCongenital cytomegalovirus infection in a preterm infant with 22q11.2 deletion syndrome and immunological abnormalities.
Nagoya journal of medical scienceSocial skills in neurodevelopmental disorders: a study using role-plays to assess adolescents and young adults with 22q11.2 deletion syndrome and autism spectrum disorders.
Journal of neurodevelopmental disordersLymphoblast transcriptome analysis in 22q11.2 deletion syndrome individuals with schizophrenia-spectrum disorder.
The world journal of biological psychiatry : the official journal of the World Federation of Societies of Biological PsychiatryAn Infant Diagnosed With 22q11.2 Deletion Syndrome Following Frequent Apneic Attacks.
CureusUsing transcranial alternating current stimulation to enhance working memory skills in youths with 22q11.2 deletion syndrome: A randomized double-blind sham-controlled study.
Psychiatry researchUntargeted metabolomic, and proteomic analysis identifies metabolic biomarkers and pathway alterations in individuals with 22q11.2 deletion syndrome.
Metabolomics : Official journal of the Metabolomic SocietyMental health, coping, and protective factors in mothers of children with 22q11.2 deletion syndrome.
American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric GeneticsVariants in KMT2A in Three Individuals with Previous Suspicion of 22q11.2 Deletion Syndrome.
GenesThe effectiveness and tolerability of pharmacotherapy for psychosis in 22q11.2 Deletion Syndrome: A systematic review.
The Australian and New Zealand journal of psychiatryRemote assessment of the Penn computerised neurocognitive battery in individuals with 22q11.2 deletion syndrome.
Journal of intellectual disability research : JIDRNeonatal manifestation of 22q11.2 deletion syndrome - four case reports and a mini-literature review.
Annals of agricultural and environmental medicine : AAEMDgcr8 functions in the secondary heart field for outflow tract and right ventricle development in mammals.
Developmental biology22q11.2 Deletion Syndrome Diagnosed 47 Years After Surgery for Tetralogy of Fallot.
CureusHypoparathyroidism Associated with Benign Thyroid Nodules in DiGeorge-like Syndrome: A Rare Case Report and Literature Review.
Endocrine, metabolic & immune disorders drug targetsSurgical Management of Velopharyngeal Dysfunction.
Facial plastic surgery clinics of North AmericaHypocalcemia and Graves' disease associated with 22q11.2 deletion syndrome.
Pediatrics international : official journal of the Japan Pediatric SocietyExploring pathway interactions to detect molecular mechanisms of disease: 22q11.2 deletion syndrome.
Orphanet journal of rare diseasesIncidental finding at methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA): how to proceed?
Frontiers in genetics22q11.2 Deletion Syndrome in Taiwan: Clinical Presentation and Immune System Status of Patients.
International journal of medical sciencesOmenn syndrome in a 10-month-old male with athymia and VACTERL association.
The journal of allergy and clinical immunology. GlobalNeurodevelopmental dimensional assessment of young children at high genomic risk of neuropsychiatric conditions.
JCPP advancesPrenatal vs postnatal diagnosis of 22q11.2 deletion syndrome: cardiac and noncardiac outcomes through 1 year of age.
American journal of obstetrics and gynecologyDistinct neuroanatomical and neuropsychological features of Down syndrome compared to related neurodevelopmental disorders: a systematic review.
Frontiers in neurosciencePolysomnographic findings in children with 22q deletion & duplication syndrome: relationship to genetic diagnosis, parent-reported symptoms, and calcium levels.
Sleep & breathing = Schlaf & AtmungDiGeorge syndrome critical region gene 2 (DGCR2), a schizophrenia risk gene, regulates dendritic spine development through cell adhesion.
Cell & bioscience[Prenatally detected aortic arch anomalies and their consequences after birth].
Orvosi hetilapClinical Course and Outcome of Prenatally Detected 22q11.2 Deletion Syndrome-A Retrospective Analysis.
Diagnostics (Basel, Switzerland)Hardiness as a Resilience Factor for Adaptation in Families of Children With 22q11.2 Deletion Syndrome: A Mixed Methods Study of Parents' Perspectives.
Journal of intellectual disabilities : JOIDBehaviors related to autism spectrum disorder in children with developmental language disorder and children with 22q11.2 deletion syndrome.
Autism & developmental language impairmentsDistinct Neurocognitive Profiles and Clinical Phenotypes Associated with Copy Number Variation at the 22q11.2 Locus.
medRxiv : the preprint server for health sciencesNeurodevelopmental and other psychiatric disorders in 22q11.2 deletion syndrome from childhood to adult age: Prospective longitudinal study of 100 individuals.
American journal of medical genetics. Part C, Seminars in medical geneticsHistological Analysis of a Mouse Model of the 22q11.2 Microdeletion Syndrome.
BiomoleculesDevelopment of a storytelling communication facilitation tool (SCFT) to facilitate discussion of complex genetic diagnoses between parents and their children: A pilot study using 22q11.2 deletion syndrome as a model condition.
PEC innovationMultivariate patterns of disrupted sleep longitudinally predict affective vulnerability to psychosis in 22q11.2 Deletion Syndrome.
Psychiatry researchExecutive functioning in preschoolers with 22q11.2 deletion syndrome and the impact of congenital heart defects.
Journal of neurodevelopmental disordersNeurobehavioral risk factors influence prevalence and severity of hazardous substance use in youth at genetic and clinical high risk for psychosis.
Frontiers in psychiatryCongenital Heart Defects and 22q11.2 Deletion Syndrome: A 20-Year Update and New Insights to Aid Clinical Diagnosis.
Journal of pediatric geneticsAn Endocrinological Perspective on 22q11.2 Deletion Syndrome: A Single-center Experience.
Journal of clinical research in pediatric endocrinologyPsychosis spectrum features, neurocognition and functioning in a longitudinal study of youth with 22q11.2 deletion syndrome.
Psychological medicineLanguage Profiles of School-Aged Children with 22q11.2 Copy Number Variants.
GenesAberrant right subclavian artery: embryology, prenatal diagnosis and clinical significance.
Ultrasound (Leeds, England)Positive cfDNA screening results for 22q11.2 deletion syndrome-Clinical and laboratory considerations.
Frontiers in geneticsA case study of the utilization of clozapine treatment for treatment-resistant schizophrenia associated with 22q11.2 deletion syndrome.
Neuropsychopharmacology reportsChromosome 22q11.2 Deletion (DiGeorge Syndrome): Immunologic Features, Diagnosis, and Management.
Current allergy and asthma reportsEducational challenges for 22q11.2 deletion syndrome in Japan: Findings from a mixed methods survey.
Journal of applied research in intellectual disabilities : JARID22q11 Copy Number Variations in a Brazilian Cohort of Children with Congenital Heart Disorders.
Molecular syndromologyRelationship between high trait anxiety in 22q11.2 deletion syndrome and the difficulties in medical, welfare, and educational services.
PCN reports : psychiatry and clinical neurosciencesPhenotypic heterogeneity in 22q11.2 deletion syndrome: Copy Number Variants as genetic modifiers for congenital heart disease in a Brazilian cohort.
American journal of medical genetics. Part AUpdated clinical practice recommendations for managing children with 22q11.2 deletion syndrome.
Genetics in medicine : official journal of the American College of Medical GeneticsSphincter Pharyngoplasty for Velopharyngeal Dysfunction: Impact of 22q11.2 Deletion Syndrome.
The LaryngoscopeGathering the Stakeholder's Perspective: Experiences and Opportunities in Rare Genetic Disease Research.
GenesPrenatal Screening and Diagnostic Considerations for 22q11.2 Microdeletions.
GenesImproved Outcomes in Patients with 22q11.2 Deletion Syndrome and Diagnosis of Interrupted Aortic Arch Prior to Birth Hospital Discharge, a Retrospective Study.
GenesSurgical Management of Submucous Cleft Palate by Radical Muscle Dissection Veloplasty: Speech Outcomes in Patients with 22q11.2 Deletion Syndrome.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationClinical Risk Factors for Aortic Root Dilation in Patients with 22q11.2 Deletion Syndrome: A Longitudinal Single-Center Study.
GenesTonsillectomy in Children with 22q11.2 Deletion Syndrome.
GenesThe Potential Role of Dysregulated miRNAs in Adolescent Idiopathic Scoliosis and 22q11.2 Deletion Syndrome.
Journal of personalized medicineStructural Cerebellar Abnormalities and Parkinsonism in Patients with 22q11.2 Deletion Syndrome.
Brain sciencesReproductive Outcomes in Adults with 22q11.2 Deletion Syndrome.
GenesThe 22q11.2 Low Copy Repeats.
GenesCardiac function in adolescents and young adults with 22q11.2 deletion syndrome without congenital heart disease.
European journal of medical geneticsA method of large DNA fragment enrichment for nanopore sequencing in region 22q11.2.
Frontiers in geneticsRare tandem repeat expansions associate with genes involved in synaptic and neuronal signaling functions in schizophrenia.
Molecular psychiatryThe Unique Experience of a New Multidisciplinary Program for 22q Deletion and Duplication Syndromes in a Community Hospital in Florida: A Reaffirmation That Multidisciplinary Care Is Essential for Best Outcomes in These Patients.
GenesImmune status of the murine 22q11.2 deletion syndrome model.
European journal of immunologyAirway anomalies in patients with 22q11.2 deletion syndrome: A scoping review.
International journal of pediatric otorhinolaryngologyParent-Reported Social-Communicative Skills of Children with 22q11.2 Copy Number Variants and Siblings.
GenesCOVID-19 Severity, Cardiological Outcome, and Immunogenicity of mRNA Vaccine on Adult Patients With 22q11.2 DS.
The journal of allergy and clinical immunology. In practiceAortic Arch Laterality in Chromosome 22q11.2 Deletion Syndrome: Male-Female Difference.
Clinical pediatricsThe Relationships between Dopaminergic, Glutamatergic, and Cognitive Functioning in 22q11.2 Deletion Syndrome: A Cross-Sectional, Multimodal 1H-MRS and 18F-Fallypride PET Study.
GenesA Novel Non-Allelic Homologous Recombination Event in a Parent with an 11;22 Reciprocal Translocation Leading to 22q11.2 Deletion Syndrome.
GenesCharacterization of Autoimmune Thyroid Disease in a Cohort of 73 Paediatric Patients Affected by 22q11.2 Deletion Syndrome: Longitudinal Single-Centre Study.
GenesBayesian inference of molecular kinetic parameters from astrocyte calcium imaging data.
MethodsXLaryngeal web with 22q11.2 deletion syndrome.
International journal of pediatrics & adolescent medicineSleep EEG in young people with 22q11.2 deletion syndrome: A cross-sectional study of slow-waves, spindles and correlations with memory and neurodevelopmental symptoms.
eLifeDeletion Syndrome 22q11.2: A Systematic Review.
Children (Basel, Switzerland)Infectious Complications of DiGeorge Syndrome in the Setting of Malignancy.
CureusIdentification of 22q11.2 deletion in a patient with schizophrenia and clinically diagnosed Rubinstein-Taybi syndrome.
PCN reports : psychiatry and clinical neurosciencesSequential inhibitory plasticities in hippocampal area CA2 and social memory formation.
NeuronPost-traumatic stress in adults with 22q11.2 deletion syndrome.
BJPsych openAltered developmental trajectories of verbal learning skills in 22q11.2DS: associations with hippocampal development and psychosis.
Psychological medicineCongenital portosystemic venous shunt associated with 22q11.2 deletion syndrome: a case report.
BMC pediatrics22q11.2 Deletion Syndrome: Impact of Genetics in the Treatment of Conotruncal Heart Defects.
Children (Basel, Switzerland)Experimental and computational analyses of calcium dynamics in 22q11.2 deletion model astrocytes.
Neuroscience lettersCraniofacial Phenotypes and Genetics of DiGeorge Syndrome.
Journal of developmental biologyAssessment of the Velopharyngeal Mechanism at Rest and During Speech in Children With 22q11.2DS: A Cross-Sectional Study.
The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial AssociationGenome-wide tandem repeat expansions contribute to schizophrenia risk.
Molecular psychiatryCrossed pulmonary arteries: An underestimated cardiovascular variant with a strong association with genetic syndromes-A report of 74 cases with systematic review of the literature.
American journal of medical genetics. Part ANeural Networks for Classification and Image Generation of Aging in Genetic Syndromes.
Frontiers in geneticsEndocrine and Growth Disorders in Taiwanese Children With 22q11.2 Deletion Syndrome.
Frontiers in endocrinologyTalking with children and young people with 22q11DS about their mental health, behaviour, learning and communication.
Child: care, health and developmentRecognition of facial emotion expressions and perceptual processes in 22q11.2 deletion syndrome.
Early intervention in psychiatryBiallelic PAN2 variants in individuals with a syndromic neurodevelopmental disorder and multiple congenital anomalies.
European journal of human genetics : EJHGIncreased Central Auditory Gain and Decreased Parvalbumin-Positive Cortical Interneuron Density in the Df1/+ Mouse Model of Schizophrenia Correlate With Hearing Impairment.
Biological psychiatry global open scienceCrossed pulmonary arteries and DiGeorge syndrome: case reports and literature review.
Cardiology in the youngThe COVID-19 pandemic's impact on worry and medical disruptions reported by individuals with chromosome 22q11.2 copy number variants and their caregivers.
Journal of intellectual disability research : JIDRAn overview of the trajectory of Brazilian individuals with 22q11.2 deletion syndrome until diagnosis.
Orphanet journal of rare diseasesSpectrum of Genetic T-Cell Disorders from 22q11.2DS to CHARGE.
Clinical reviews in allergy & immunologyLong-Term Follow-Up of Newborns with 22q11 Deletion Syndrome and Low TRECs.
Journal of clinical immunologyDownregulation of miR-185 is a common pathogenic event in 22q11.2 deletion syndrome-related and idiopathic schizophrenia.
Metabolic brain diseaseA Regional Burden of Sequence-Level Variation in the 22q11.2 Region Influences Schizophrenia Risk and Educational Attainment.
Biological psychiatryNeurological manifestation of 22q11.2 deletion syndrome.
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical NeurophysiologyCell-free DNA screening for prenatal detection of 22q11.2 deletion syndrome.
American journal of obstetrics and gynecologyCharacterizing Daily-Life Social Interactions in Adolescents and Young Adults with Neurodevelopmental Disorders: A Comparison Between Individuals with Autism Spectrum Disorders and 22q11.2 Deletion Syndrome.
Journal of autism and developmental disordersA Comprehensive Analysis of Cerebellar Volumes in the 22q11.2 Deletion Syndrome.
Biological psychiatry. Cognitive neuroscience and neuroimagingImpact of Equine-Assisted Interventions on Heart Rate Variability in Two Participants with 22q11.2 Deletion Syndrome: A Pilot Study.
Children (Basel, Switzerland)Parents' perspectives, experiences, and need for support when communicating with their children about the psychiatric manifestations of 22q11.2 deletion syndrome (22q11DS).
Journal of community geneticsClinical Manifestations of 22q11.2 Deletion Syndrome.
Heart failure clinicsOcular findings in 22q11.2 deletion syndrome: A systematic literature review and results of a Dutch multicenter study.
American journal of medical genetics. Part A22q11.2 Deletion Syndrome as a Human Model for Idiopathic Scoliosis.
Journal of clinical medicineSocial Cognition Impairments in 22q11.2DS Individuals With and Without Psychosis: A Comparison Study With a Large Population of Patients With Schizophrenia.
Schizophrenia bulletin openAssociações
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Síndrome de deleção 22q11.2 e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma anomalia genética congênita caracterizada pela perda de material no cromossomo 22. Ela se manifesta tipicamente por defeitos no coração, alterações no palato, imunodeficiência e atraso no desenvolvimento.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 7 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Síndrome de deleção 22q11.2. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/sindrome-de-delecao-22q112
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Síndrome de deleção 22q11.2
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Moléculas estudadas na doença
- fonte: OpenTargets
- Ensaios clínicos
- fonte: ClinicalTrials.gov