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2.
Rinsho Ketsueki ; 61(8): 857-864, 2020.
Artigo em Japonês | MEDLINE | ID: mdl-32908046

RESUMO

Nosocomial coronavirus disease 2019 (COVID-19) had occurred at our hospital. We retrospectively analyzed the differences between patients with nosocomial COVID-19 and either hematological disease (n=40) or other diseases (n=57). The analysis was completed within 60 days for surviving patients. Among the patients with hematological disease and those with other diseases, there were 21 (52.5%) and 20 (35.1%) deaths, respectively. Although the patients with hematological disease received favipiravir more frequently than patients with other diseases (21 [52.5%] vs. 15 [35.3%], respectively; P<0.05), their median overall survival was poor (29 days; P=0.078). Furthermore, the median duration from oxygen therapy initiation to death or intubation was significantly shorter in the patients with hematological disease (5 days [range, 1-17 days] vs. 10 days [1-24 days], respectively; P<0.05). Furthermore, the patients with hematological disease and nosocomial COVID-19 exhibited more marked respiratory failure and poorer outcomes leading to death in a shorter time period than the patients with other diseases and nosocomial COVID-19.


Assuntos
Infecções por Coronavirus/complicações , Infecção Hospitalar/complicações , Doenças Hematológicas/complicações , Pneumonia Viral/complicações , Betacoronavirus , Infecções por Coronavirus/fisiopatologia , Infecção Hospitalar/virologia , Doenças Hematológicas/virologia , Humanos , Pandemias , Pneumonia Viral/fisiopatologia , Estudos Retrospectivos , Taxa de Sobrevida
3.
BMC Pulm Med ; 20(1): 236, 2020 Sep 05.
Artigo em Inglês | MEDLINE | ID: mdl-32891138

RESUMO

BACKGROUND: Pneumocystis pneumonia (PCP) is an important cause of acute respiratory failure (ARF) in immunocompromised patients, yet no actual clinical tool suitably identifies patients at risk. Recently, a multivariable prediction model has been proposed for haematology patients with ARF requiring intensive care unit (ICU) admission to assess the risk of PCP (PCP score). However, it has not yet been validated externally. METHODS: To validate the PCP score, a retrospective cohort study was conducted in two large designated haematology centres in Korea. One-hundred and forty haematology patients with ARF were admitted to ICU. They underwent aetiologic evaluations between July 2016 and June 2019. The predictive ability of the score was assessed with the receiver operating characteristic (ROC) curve analysis for both the discrimination and calibration of the score. RESULTS: Among the 141 patients, 13 (9.2%) were finally diagnosed of PCP. Although the median of PCP score in PCP group was higher than in non-PCP group (3.0 [interquartile range 0.0-4.0] vs. 2.0 [0.5-4.0]), the difference was not statistically significant (P = 0.679). The area under the ROC curve of the PCP score in our cohort was 0.535 (95% CI, 0.449-0.620), indicating no discriminatory ability. When using a cut-off of 3.0 the score, the result was 38.5% (95% CI, 13.9-68.4) sensitive and 7.03% (95% CI, 61.6-78.1) specific. The negative predictive value was 58.8% and positive predictive value was 59.8% for a 10% prevalence of PCP. CONCLUSIONS: In this study, the PCP score was not useful to predict the risk of PCP in haematology patients with ARF. Further prospective validation studies are needed to validate the score's use in routine clinical practice for the early diagnosis of PCP in haematology patients.


Assuntos
Doenças Hematológicas/complicações , Pneumonia por Pneumocystis/complicações , Pneumonia por Pneumocystis/epidemiologia , Insuficiência Respiratória/etiologia , Medição de Risco , Doença Aguda , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia por Pneumocystis/diagnóstico , Estudos Retrospectivos
5.
Cancer Sci ; 111(9): 3379-3385, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32619034

RESUMO

The rapid spread of coronavirus disease 2019 (COVID-19) represented the most serious issue to public health globally. Hematological patients as immunocompromised hosts are vulnerable to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. There is little information available regarding the clinical features of hematological patients concomitant with COVID-19. In this study, 9 concomitant patients were analyzed for their clinical manifestations, laboratory data, radiological findings, and immunologic features. The median age was 50 years (range, 17-68 years) and 6 patients were male. Seven patients were infected through hospital-associated transmission and other 2 through community-associated transmission. Onset of COVID-19 in all patients occurred during routine treatments for their hematological diseases. Eight patients were classified as moderate and 1 patient as critically ill COVID-19. Four patients died, 1 from leukemia progression, 2 from life-threatening secondary infection, and the other from respiratory failure caused by COVID-19. Abruptly elevated levels of cytokines were often correlated with progressive hematological disease or concurrent bacterial infections. Two patients had atypical computed tomography (CT) imaging findings of COVID-19. The median interval from the first CT scan imaging to improvement in survivors was 40 days (range, 14-51 days). Four of 5 survivors had negative serological tests 1 month after symptom onset. Positive viral load in 4 survivors lasted longer than 45 days. Our results indicated concomitant patients formed a distinct subgroup characterized by atypical clinical features, defective viral clearance, and lower level of SARS-CoV-2-specific Abs. Targeted therapies that impair host humoral immunity should be avoided. These findings will be helpful to tailor appropriate management for the concomitant patients.


Assuntos
Infecções por Coronavirus/complicações , Doenças Hematológicas/complicações , Doenças Hematológicas/terapia , Hospedeiro Imunocomprometido , Pneumonia Viral/complicações , Adolescente , Adulto , Idoso , Infecções por Coronavirus/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/imunologia , Adulto Jovem
7.
Medicine (Baltimore) ; 99(20): e19948, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32443296

RESUMO

There are limited systematic studies on hematologic disease complicated by air leak syndrome (ALS). Physicians in radiology departments and hematology departments have a limited awareness of ALS.The aim of this study was to explore the similarities and differences in clinical data between the clinical group and imaging group in patients with hematologic disease complicated by ALS.Clinical and CT data for 59 patients with hematologic disease complicated by ALS in our hospital were retrospectively reviewed. Patients were assessed by clinical grouping and image grouping. Data were compared between groups, and P < .05 was considered statistically significant.Dyspnea occurred more often in the allo-HSCT (allogeneic hematopoietic stem cell transplantation) group than that in the non-allo-HSCT group (68.8% vs 4.7%, P < .001), there were statistically significant differences in inducing factors between groups, and differences in other aspects were not statistically significant. Chest tightness and dyspnea occurred more often in the allo-HSCT with BO/BOOP (bronchiolitis Obliteran/bronchiolitis obliterans organizing pneumonia) group than those in the allo-HSCT without BO/BOOP group (80.0% vs 9.1%, P = .013), and differences in other aspects were not statistically significant. Chest pain occurred more often in the HPT (hydropneumothorax) group than that in the other 3 groups (pure pneumothorax [PT], pulmonary interstitial emphysema [PIE], complex ALS) (71.4% vs 11.1%, 0.0%, and 26.5%, P = .005); ALS thickness in the HPT group was greater than that in the other 2 groups (PIE and complex ALS) (19.7 vs 3.5 cm and 9.5 cm, P = .001); catheter drainage occurred more often in the HPT group than that in the other three groups (PT, PIE, complexALS) (64.3% vs 22.2%, 0.0%, and 2.9%, P = .001).ALS is a high risk in male patients who have a low BMI, have leukemia as a basic disease, and have basic lung diseases (eg, BO/BOOP). CT types are mainly complex ALS, HPT, and pure PT. In addition, clinical symptoms for patients in the HPT group are severe, and there is a high prevalence of catheter drainage.


Assuntos
Doenças Hematológicas/complicações , Enfisema Mediastínico/diagnóstico por imagem , Pneumotórax/diagnóstico por imagem , Enfisema Pulmonar/diagnóstico por imagem , Adolescente , Adulto , Criança , Pneumonia em Organização Criptogênica/complicações , Feminino , Doenças Hematológicas/diagnóstico por imagem , Doenças Hematológicas/terapia , Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Humanos , Masculino , Enfisema Mediastínico/etiologia , Pessoa de Meia-Idade , Pneumotórax/etiologia , Enfisema Pulmonar/etiologia , Estudos Retrospectivos , Adulto Jovem
11.
Rev Med Suisse ; 16(N° 691-2): 823-826, 2020 Apr 29.
Artigo em Francês | MEDLINE | ID: mdl-32348044

RESUMO

The COVID-19 pandemic impacts the hematology practice. Intensive chemotherapies for high-grade lymphomas and acute leukemias, multiple myeloma treatments and most hematopoietic stem cell transplantations should be performed as usual. Low-grade lymphomas should only be treated when strictly indicated, maintenance can be postponed. Other myeloid neoplasia and their therapies cause imunosupression; dose adjustment is recommended but no brisk stopping. Sickle cell anemia patients are highly succeptible to severe COVID-19 course. Thrombocytopenia and procoagulant state are associated with severe courses of COVID-19, requiring an individualized therapy. No data indicate a risk of SARS-CoV-2 transmission through blood product transfusion.


Assuntos
Betacoronavirus , Infecções por Coronavirus , Doenças Hematológicas/complicações , Pandemias , Pneumonia Viral , Infecções por Coronavirus/complicações , Infecções por Coronavirus/epidemiologia , Doenças Hematológicas/diagnóstico , Doenças Hematológicas/terapia , Hematologia/tendências , Humanos , Pneumonia Viral/complicações , Pneumonia Viral/epidemiologia
15.
Ann Hematol ; 99(4): 839-845, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32025839

RESUMO

Hemorrhagic cystitis (HC) has been reported with increased frequency following post-transplantation cyclophosphamide (PTCy)-based haploidentical hematopoietic cell transplantation (HCT) along with a strong association with BK viruria. We prospectively evaluated the incidence of BK viruria and HC in 115 patients (median age 20 years, 2-65) undergoing PTCy-based haploidentical HCT with (n = 71) or without (n = 44) CTLA4Ig. HC prophylaxis consisted of a continuous infusion of mesna 30 min prior and 48 h post-PTCy. The overall incidence of BK viruria was 65.7%. None with BK viruria < 104 copies/ml developed clinical symptoms (n = 65). The incidence of BK viruria ≥ 104 copies/ml was 7.1% (n = 8) and 75% developed HC. The incidence of HC was 5.4% at a median of 30 days. Both BK viruria ≥ 104 copies/ml and HC were strongly associated with acute GVHD (p < 0.001). A higher NRM was observed in those with BK viruria ≥ 104 copies/ml, related to GVHD and its complications (41.7% vs 12.6%, p = 0.04). The incidences of acute GVHD, vis-à-vis, overall BK viruria, BK viruria ≥ 104 copies/ml, and HC, tended to be lower in patients receiving CTLA4Ig. Thus, extended infusional mesna, coupled with significant reduction in alloreactivity along with possible preservation of antiviral immunity associated with the use of CTLA4Ig, was probably responsible for a much lower incidence of BK viruria and resultant HC than reported previously following PTCy-based haploidentical HCT.


Assuntos
Abatacepte/uso terapêutico , Vírus BK/isolamento & purificação , Ciclofosfamida/efeitos adversos , Cistite/prevenção & controle , Transplante de Células-Tronco Hematopoéticas , Hematúria/prevenção & controle , Imunossupressores/efeitos adversos , Mesna/uso terapêutico , Infecções por Polyomavirus/urina , Transplante Haploidêntico , Infecções Tumorais por Vírus/urina , Abatacepte/administração & dosagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Ciclofosfamida/administração & dosagem , Cistite/induzido quimicamente , Cistite/urina , Cistite/virologia , Feminino , Doença Enxerto-Hospedeiro/prevenção & controle , Doenças Hematológicas/complicações , Doenças Hematológicas/terapia , Neoplasias Hematológicas/complicações , Neoplasias Hematológicas/terapia , Hematúria/induzido quimicamente , Hematúria/virologia , Humanos , Imunossupressores/administração & dosagem , Infusões Intravenosas , Estimativa de Kaplan-Meier , Masculino , Mesna/administração & dosagem , Pessoa de Meia-Idade , Infecções por Polyomavirus/complicações , Infecções por Polyomavirus/virologia , Infecções Tumorais por Vírus/complicações , Infecções Tumorais por Vírus/virologia , Urina/virologia , Adulto Jovem
16.
Infect Immun ; 88(4)2020 03 23.
Artigo em Inglês | MEDLINE | ID: mdl-31964743

RESUMO

Invasive aspergillosis (IA) is a life-threatening infection that affects an increasing number of patients undergoing chemotherapy or allo-transplantation, and recent studies have shown that genetic factors contribute to disease susceptibility. In this two-stage, population-based, case-control study, we evaluated whether 7 potentially functional single nucleotide polymorphisms (SNPs) within the ARNT2 and CX3CR1 genes influence the risk of IA in high-risk hematological patients. We genotyped selected SNPs in a cohort of 500 hematological patients (103 of those had been diagnosed with proven or probable IA), and we evaluated their association with the risk of developing IA. The association of the most interesting markers of IA risk was then validated in a replication population, including 474 subjects (94 IA and 380 non-IA patients). Functional experiments were also performed to confirm the biological relevance of the most interesting markers. The meta-analysis of both populations showed that carriers of the ARNT2 rs1374213G, CX3CR1 rs7631529A, and CX3CR1 rs9823718G alleles (where the RefSeq identifier appears as a subscript) had a significantly increased risk of developing IA according to a log-additive model (P value from the meta-analysis [P Meta] = 9.8 · 10-5, P Meta = 1.5 · 10-4, and P Meta =7.9 · 10-5, respectively). Haplotype analysis also confirmed the association of the CX3CR1 haplotype with AG CGG with an increased risk of IA (P = 4.0 · 10-4). Mechanistically, we observed that monocyte-derived macrophages (MDM) from subjects carrying the ARNTR2 rs1374213G allele or the GG genotype showed a significantly impaired fungicidal activity but that MDM from carriers of the ARNT2 rs1374213G and CX3CR1 rs9823718G or CX3CR1 rs7631529A alleles had deregulated immune responses to Aspergillus conidia. These results, together with those from expression quantitative trait locus (eQTL) data browsers showing a strong correlation of the CX3CR1 rs9823718G allele with lower levels of CX3CR1 mRNA in whole peripheral blood (P = 2.46 · 10-7) and primary monocytes (P = 4.31 · 10-7), highlight the role of the ARNT2 and CX3CR1 loci in modulating and predicting IA risk and provide new insights into the host immune mechanisms involved in IA development.


Assuntos
Translocador Nuclear Receptor Aril Hidrocarboneto/genética , Aspergillus/imunologia , Fatores de Transcrição Hélice-Alça-Hélice Básicos/genética , Receptor 1 de Quimiocina CX3C/genética , Predisposição Genética para Doença , Aspergilose Pulmonar Invasiva/genética , Polimorfismo de Nucleotídeo Único , Estudos de Casos e Controles , Genótipo , Doenças Hematológicas/complicações , Humanos , Medição de Risco
17.
Acta Haematol ; 143(1): 33-39, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31216534

RESUMO

Immune-mediated processes are considered important in the pathogenesis of bone marrow failure syndromes (BFS). We previously reported that natural killer group 2D (NKG2D) ligands were expressed on pathological blood cells of patients with BFS and that NKG2D immunity may be involved in bone marrow failure. In addition to membranous NKG2D ligands on the cell surface, soluble NKG2D ligands can exist in plasma. We therefore examined the relationship between soluble NKG2D ligands and blood cell counts in 86 patients with BFS, including aplastic anemia, myelodysplastic syndrome with single lineage dysplasia, and paroxysmal nocturnal hemoglobinuria. Approximately half of the BFS patients were positive for soluble NKG2D ligands in the plasma by enzyme-linked immunosorbent assay, and soluble NKG2D ligand-positive BFS patients exhibited severe cytopenia regardless of membranous NKG2D ligand expression. In vitroanalyses demonstrated that soluble ULBP1, an NKG2D ligand, down-regulated NKG2D receptors on CD2-positive cells in peripheral blood. Moreover, soluble ULBP1 attenuated the cytotoxic effects of peripheral blood mononuclear cells on K562, which express membranous ULBP1. Our results suggest that soluble NKG2D ligands can be easy-to-measure biomarkers for the prediction of activity of immune-meditated bone marrow injury in BFS and that soluble NKG2D ligands suppress redundant immune-mediated bone marrow injury.


Assuntos
Biomarcadores/sangue , Transtornos da Insuficiência da Medula Óssea/diagnóstico , Peptídeos e Proteínas de Sinalização Intracelular/sangue , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anemia Aplástica/diagnóstico , Contagem de Células Sanguíneas , Transtornos da Insuficiência da Medula Óssea/complicações , Antígenos CD2/metabolismo , Regulação para Baixo , Proteínas Ligadas por GPI/sangue , Doenças Hematológicas/complicações , Doenças Hematológicas/diagnóstico , Hemoglobinúria Paroxística/diagnóstico , Humanos , Leucócitos Mononucleares/citologia , Leucócitos Mononucleares/metabolismo , Masculino , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/diagnóstico , Subfamília K de Receptores Semelhantes a Lectina de Células NK/metabolismo , Adulto Jovem
18.
Infection ; 48(2): 205-212, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31686323

RESUMO

OBJECTIVE: Stenotrophomonas maltophilia (SM) is an important nosocomial pathogen, particularly in immunocompromised patients due to their adverse antimicrobial susceptibility pattern. The objective of this article was to investigate the clinical impact of SM bacteremia on the 30-day mortality rate and identify the risk factors of the cause of mortality in patients with hematologic disorders. METHODS: We retrospectively reviewed the clinical data in patients diagnosed with hematological disorders and SM bacteremia over an 8-year period from July 2010 to July 2018 at a 248-bed hematology department. We compared patients' clinical characteristics and outcomes between the non-survivor and survivor groups. RESULTS: The overall incidence of SM bacteremia was 25.1 per 10,000 admissions. There were 59 patients (median age: 35 years; 57.6% males) included in the study with an overall SM bacteremia-related 30-day mortality of 44.1%. Multi-drug resistance was common. In vitro susceptibility is higher to ceftazidime (72.9%), ciprofloxacin (66.1%) and cefoperazone/sulbactam (59.3%). The risk factors identified in the univariate analysis were catheter re-implantation, accompanying polymicrobial infection, inadequate initial antimicrobial treatment, APACHE II score, temperature > 39 °C, septic shock, respiratory failure, and non-remission post treatment for primary diseases. Multivariate analysis further confirmed that inadequate initial antimicrobial treatment, respiratory failure, and non-remission after treatment for hematological diseases are independent risk factors associated with mortality (P = 0.001, 0.002 and 0.007, respectively). CONCLUSIONS: Our study suggests that SM bacteremia is highly associated with increased mortality in patients with hematologic diseases. Early detection, prompt comprehensive management including initiation of combined sensitive antibiotics, respiratory monitoring and support, platelet infusion, and strategies to improve patients' remission status are recommended to improve the overall survival in patients with SM bacteremia.


Assuntos
Infecções por Bactérias Gram-Negativas/complicações , Infecções por Bactérias Gram-Negativas/mortalidade , Doenças Hematológicas/complicações , Doenças Hematológicas/mortalidade , Departamentos Hospitalares/estatística & dados numéricos , Stenotrophomonas maltophilia/imunologia , Adolescente , Adulto , Antibacterianos/farmacologia , Criança , Farmacorresistência Bacteriana , Feminino , Infecções por Bactérias Gram-Negativas/epidemiologia , Doenças Hematológicas/epidemiologia , Doenças Hematológicas/microbiologia , Humanos , Incidência , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Stenotrophomonas maltophilia/efeitos dos fármacos , Stenotrophomonas maltophilia/isolamento & purificação , Adulto Jovem
19.
J Vasc Interv Radiol ; 31(2): 276-281.e1, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31883934

RESUMO

PURPOSE: To assess the utility of routine preprocedural bloodwork during elective removal of central venous access devices (CVADs) with respect to bleeding complications. MATERIALS AND METHODS: Patients who underwent removal of a CVAD (tunneled central venous catheter [CVC] or port) by the interventional radiology service between January 2009 and December 2013 were retrospectively reviewed. Removals for infection or malfunction, without preprocedural bloodwork, with another concurrent procedure at the time of CVAD removal, or in patients with a bleeding dyscrasia were excluded. Peripherally inserted central catheter removals and temporary CVAD removals were also excluded. Routine preprocedural bloodwork included hemoglobin, platelet count, partial thromboplastin time, and International Normalized Ratio. Postprocedural complications were classified according to the Society of Interventional Radiology clinical practice guidelines. RESULTS: There were 802 CVAD removals in 777 patients (351 female, 426 male). Average patient age was 8.6 years (range, 5 wk to 19 y). In total, 246 permanent CVCs and 556 ports were removed. A total of 802 cases had preprocedural bloodwork. Of the 49 patients who had a bleeding complication after the procedure (6.1%; 49 of 802), 44 had normal findings on preprocedural bloodwork and 5 had abnormal findings. There was no statistically significant difference in bleeding complications between those with normal and abnormal bloodwork results (P = .7740). CONCLUSIONS: Routine bloodwork is not necessary before elective CVAD removal in children without a bleeding dyscrasia. Most children have normal findings on preprocedural bloodwork, and the incidence of postprocedural bleeding is low and not determined by bloodwork results.


Assuntos
Cateterismo Venoso Central/instrumentação , Cateteres de Demora , Cateteres Venosos Centrais , Remoção de Dispositivo , Doenças Hematológicas/diagnóstico , Testes Hematológicos , Hemorragia Pós-Operatória/etiologia , Adolescente , Fatores Etários , Criança , Pré-Escolar , Remoção de Dispositivo/efeitos adversos , Feminino , Doenças Hematológicas/sangue , Doenças Hematológicas/complicações , Humanos , Lactente , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento , Procedimentos Desnecessários , Adulto Jovem
20.
Clin Orthop Surg ; 11(4): 474-481, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31788172

RESUMO

Background: Kabuki syndrome is a rare genetic disorder characterized by distinct dysmorphic facial features, growth deficiency, intellectual disabilities, unusual dermatoglyphic patterns, and skeletal abnormalities. The incidence of hip dislocation in Kabuki syndrome ranges from 18% to 62%. We reviewed the outcomes of management of hip dislocations in patients with Kabuki syndrome with special attention to the diagnostic processes for hip dislocation and Kabuki syndrome. Methods: Among 30 patients with mutation-confirmed Kabuki syndrome, we selected six patients who had hip dislocations and reviewed their medical records and plain radiographs. The modes of presentation and diagnostic processes for both hip dislocations and Kabuki syndrome were investigated. The management and treatment outcomes of hip dislocations in patients with Kabuki syndrome were evaluated. Results: The average age of patients at the time of diagnosis of hip dislocation was 7.7 months (range, 1 week to 22 months). None of the patients were diagnosed as having Kabuki syndrome at that time. Two patients were treated with a Pavlik harness; one, with closed reduction; two, with open reduction and later pelvic and/or femoral osteotomies; and one, with open reduction combined with pelvic osteotomy. The patients were followed up for 5.8 years on average (range, 2.0 to 10.5 years). The radiologic outcome was graded as Severin IA or IB for three patients who were older than 6 years at the latest follow-up (mean age, 9.9 years; range, 7.8 to 12.4 years). In the remaining three patients younger than 6 years (mean age, 3.8 years; range, 2.7 to 5.3 years), the lateral center edge angle was more than 15°. The clinical diagnosis of Kabuki syndrome was made during follow-up after hip dislocation treatment and confirmed by mutational analysis at a mean age of 4.7 years. The mean interval between the diagnosis of hip dislocation and Kabuki syndrome was 4.0 years. Conclusions: The management of hip dislocation by conservative or surgical method showed successful results. Awareness of Kabuki syndrome could lead to an early diagnosis of this rare disease in patients with hip dislocation and allow for early detection of other underlying conditions and multidisciplinary management.


Assuntos
Face/anormalidades , Doenças Hematológicas/complicações , Luxação do Quadril/diagnóstico por imagem , Luxação do Quadril/etiologia , Luxação do Quadril/terapia , Doenças Vestibulares/complicações , Anormalidades Múltiplas , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Radiografia , Estudos Retrospectivos
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