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1.
Acta Otolaryngol ; 142(1): 106-111, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34965176

RESUMEN

BACKGROUND: The pathology of vocal fold leukoplakia (VFL) was hard to predict before surgery. OBJECTIVES: To investigate the correlation of intraepithelial papillary capillary loops (IPCLs) with the malignant transformation in VFL. MATERIALS AND METHODS: The IPCLs in 90 patients were classified according to 2019 Ni classification. Type I-III represented to non- to moderate dysplasia in pathology being viewed as 'benign leukoplasia', type IV-VI represented to severe dysplasia/cancer in situ, cancerization being viewed as 'malignant leukoplasia'. The corresponding relationship of IPCLs with pathology was calculated. RESULTS: Forty-one cases were classified as type I-III, the consistency of type I-III with pathology was 61.0%, with 100.0% of type I, 47.1% of type II and 30.0% of type III. 49 cases were classified as type IV-VI, all were 'malignant leukoplasia', the consistency of type IV-VI and pathology was 100.0%. The accuracy of type I-III, IV-VI to predict pathology were 61.0, 100.0%. The sensitivity, specificity, accuracy, positive predictive value, negative predictive value of Ni classification corresponding to 'benign/malignant leukoplasia' was 75.8, 100.0, 82.2, 100.0, 60.0%, respectively. CONCLUSIONS AND SIGNIFICANCE: IPCLs of type IV-VI in vocal cord leukoplasia had a close correlation with pathology. The degree of dysplasia was underestimated in type II and type III.


Asunto(s)
Neoplasias Laríngeas/clasificación , Neoplasias Laríngeas/patología , Leucoplasia/clasificación , Leucoplasia/patología , Pliegues Vocales/patología , Femenino , Humanos , Neoplasias Laríngeas/terapia , Leucoplasia/terapia , Masculino , Persona de Mediana Edad , Imagen de Banda Estrecha , Valor Predictivo de las Pruebas , Estudios Retrospectivos
2.
Clin Otolaryngol ; 47(1): 153-159, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34662496

RESUMEN

OBJECTIVE: To assess long-term outcomes of ALA-PDT in treating recalcitrant laryngeal leukoplakia. STUDY DESIGN: Retrospective Case-Control. METHODS: We reviewed all laryngeal leukoplakia patients treated with ALA-PDT compared with angiolytic laser treatment alone (585 nm PDL or 532 nm KTP laser) from 2000 to 2019. Patients with laryngeal cancer (or a history of laryngeal cancer), leukoplakia previously treated with radiation and no pathologic report were excluded. Patient demographics, procedure details and outcomes were examined including histopathologic diagnosis, procedures performed, ALA usage, recurrence of leukoplakia and the development of cancer. RESULTS: We identified 132 patients with laryngeal leukoplakia: 42 were treated with ALA-PDT and 90 were treated with an angiolytic laser alone (Laser group). The proportion of cases of high-grade dysplasia was 57.1% in the ALA-PDT group compared to 32.2% in the Laser group. In high-grade dysplasia cases, there was a statistically significant better recurrence-free survival (RFS) at 12 months and 60 months in those who underwent ALA-PDT 71.4% and 7.1% vs Laser 25% and 0% (p = .01). However, for overall groups, there was no difference in RFS (p = .25). Voice outcomes (patient subjective report) improved or were stable in 75% of subjects with no serious side effects reported. CONCLUSION: ALA-PDT for recalcitrant and high-grade dysplasia is highly effective with improved recurrence-free survival compared to laser alone. ALA-PDT may be an appropriate therapy in patients who have failed prior angiolytic laser alone.


Asunto(s)
Ácido Aminolevulínico/uso terapéutico , Neoplasias Laríngeas/terapia , Terapia por Láser/métodos , Leucoplasia/terapia , Fotoquimioterapia/métodos , Anciano , Estudios de Casos y Controles , Femenino , Humanos , Masculino , Persona de Mediana Edad , Fármacos Fotosensibilizantes/uso terapéutico , Estudios Retrospectivos , Calidad de la Voz
3.
Otolaryngol Head Neck Surg ; 164(6): 1153-1159, 2021 06.
Artículo en Inglés | MEDLINE | ID: mdl-33170765

RESUMEN

OBJECTIVE: This state-of-the-art article reviews the epidemiology, diagnosis, and management of vocal fold leukoplakia, with focus on recent advances. It focuses on the clinical challenges that otolaryngologists face balancing both oncological efficacy and functional outcomes in leukoplakia and presents the current philosophies and techniques to consider when managing such patients. DATA SOURCES: PubMed/MEDLINE. REVIEW METHODS: We conducted a detailed review of publications related to vocal cord and laryngeal leukoplakia, dysplasia, hyperkeratosis, leukoplakia endoscopy, and leukoplakia management focusing specifically on oncologic outcomes, voice preservation, current and emerging diagnosis, and management techniques. CONCLUSIONS: There has been a paradigm shift away from performing "vocal cord stripping" procedures that can cause irreversible hoarseness toward voice preservation surgery while achieving comparable oncologic control. Surgical technical and instrumental developments have been designed to maximally treat superficial disease while preserving underling vibratory mucosa. Recent improvements in histopathological grading systems and advances in biomarker classification may allow for improved oncologic risk stratification. Furthermore, improvements in endoscopic imaging capabilities and contact endoscopy are currently being studied for their potential diagnostic significance. IMPLICATIONS FOR PRACTICE: To optimally manage vocal fold leukoplakia, the otolaryngologist should become familiar with the oncologic implications of the disease and the importance of obtaining pathologic diagnosis to rule out malignancy. In addition, the surgeon should maintain surgical techniques and knowledge of available instruments and lasers that can assist in surgical management while prioritizing the preservation of vibratory tissue and voice quality. Finally, the surgeon and the patient should understand the clinical importance of routine endoscopic surveillance.


Asunto(s)
Enfermedades de la Laringe , Leucoplasia , Pliegues Vocales , Humanos , Enfermedades de la Laringe/diagnóstico , Enfermedades de la Laringe/epidemiología , Enfermedades de la Laringe/terapia , Leucoplasia/diagnóstico , Leucoplasia/epidemiología , Leucoplasia/terapia
4.
Artículo en Chino | MEDLINE | ID: mdl-30121994

RESUMEN

Objective: To investigate the etiology, clinical and pathological characteristics of laryngeal leukoplakia and the predictive risk factors of recurrence and malignant transformation. Methods: Clinical data of 263 patients with laryngeal leukoplakia between January 2000 and December 2015 were analyzed retrospectively. Results: The pathological diagnoses included squamous epithelial hyperplasia (54.4%), mild dysplasia (17.9%), moderate dysplasia (12.2%), severe dysplasia and carcinoma in situ (12.5%), and invasive carcinoma (3.0%). Age and the extent of lesion were statistically different among different pathological groups (P<0.05). Gender, smoking and alcohol consumption did not show statistical differences among different pathological groups (P>0.05). Follow-up of 215 patients, excluding 6 cases of invasive carcinoma. The recurrence rate was 20.6%(43/209), and the malignant transformation rate was 5.3%(11/209). Multivariate analysis showed that pathological classification of moderate to severe dysplasia was the independent risk factor for recurrence and malignant transformation of laryngeal leukoplakia (P<0.05). In patients with severe dysplasia and carcinoma in situ, the recurrence proportion of conservative treatment, vocal cords (partial) resection and radiotherapy were 8/10, 0/10 and 2/11 respectively. Conclusions: Laryngeal leukoplakia occurs frequently in elderly men with long-term smoking history. Pathological diagnoses are different. The grade of dysplasia is the predictive risk factor for the recurrence and malignant transformation of laryngeal leukoplakia. More aggressive treatment and closer follow-up should be warranted for patients with moderate dysplasia, severe dysplasia and carcinoma in situ.


Asunto(s)
Carcinoma in Situ/patología , Enfermedades de la Laringe/patología , Leucoplasia/patología , Consumo de Bebidas Alcohólicas/efectos adversos , Carcinoma/etiología , Carcinoma/patología , Carcinoma/terapia , Carcinoma in Situ/etiología , Carcinoma in Situ/terapia , Transformación Celular Neoplásica/patología , Femenino , Humanos , Hiperplasia/patología , Hiperplasia/terapia , Enfermedades de la Laringe/etiología , Enfermedades de la Laringe/terapia , Neoplasias Laríngeas/etiología , Neoplasias Laríngeas/patología , Leucoplasia/etiología , Leucoplasia/terapia , Masculino , Recurrencia Local de Neoplasia , Recurrencia , Estudios Retrospectivos , Factores de Riesgo , Factores Sexuales , Fumar/efectos adversos , Pliegues Vocales/cirugía
5.
Artículo en Chino | MEDLINE | ID: mdl-30122006

RESUMEN

Vocal cord leukoplakia is a clinical diagnosis defined as a whitish patch or a plaque on a mucosal surface. Because of the diversity of histopathological types, the complexity and unpredictability risks for malignant transformation, there are still many controversies about its histopathological classification, diagnosis and treatment. The aim of this article is to review the epidemiology, etiology, pathological classification, diagnosis, treatment and prognosis of vocal cord leukoplakia.


Asunto(s)
Investigación Biomédica , Enfermedades de la Laringe/patología , Leucoplasia , Pliegues Vocales , Femenino , Humanos , Enfermedades de la Laringe/clasificación , Enfermedades de la Laringe/diagnóstico , Enfermedades de la Laringe/terapia , Leucoplasia/clasificación , Leucoplasia/diagnóstico , Leucoplasia/patología , Leucoplasia/terapia , Masculino , Pronóstico , Pliegues Vocales/patología
6.
J Investig Clin Dent ; 9(1)2018 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-28480637

RESUMEN

AIM: The aim of the present study was to systematically review the efficacy of photodynamic therapy (PDT) in the management of oral potentially-malignant disorders (PMDS) and head and neck squamous cell carcinoma (HNSCC). METHODS: From 1985 to 2015, PubMed/Medline, Google Scholar, EMBASE, and ISI Web of Knowledge were searched using different combinations of the following key words: PDT, oral precancer, leukoplakia, erythroplakia, erythroleukoplakia, verrucous hyperplasia, oral submucous fibrosis, and HNSCC. Review articles, experimental studies, case reports, commentaries, letters to the editor, unpublished articles, and articles published in languages other than English were excluded. RESULTS: Twenty-six studies were included in the present study. The number of patients ranged from 2 to 147, with a mean age of 50-67 years. The reported numbers of PMDS and HNSCC ranged between 5 and 225. Photosensitizers used were aminolevulinic acid, meta-tetrahydroxyphenylchlorin, Foscan, hematoporphyrin derivatives, Photofrin, Photosan, and chlorine-e6. Laser wavelength, power density, irradiation duration were 585-652 nm, 50-500 mW/cm2 , and 1-143 minutes, respectively. Complete, partial, and no response to PDT was found in 22.58%-100%, 4%-66%, and 0%-38.70% of PMDS, respectively, and 16%-100% of complete response in HNSCC patients. CONCLUSION: PDT is effective in the management of PMDS and HNSCC.


Asunto(s)
Carcinoma de Células Escamosas/terapia , Eritroplasia/terapia , Neoplasias de Cabeza y Cuello/terapia , Hiperplasia/terapia , Leucoplasia Bucal/terapia , Leucoplasia/terapia , Fibrosis de la Submucosa Bucal/terapia , Fotoquimioterapia/métodos , Ácido Aminolevulínico/uso terapéutico , Clorofilidas , Bases de Datos Factuales , Éter de Dihematoporfirina/uso terapéutico , Hematoporfirinas/uso terapéutico , Humanos , Indoles/uso terapéutico , Terapia por Láser , Rayos Láser , Mesoporfirinas/uso terapéutico , Compuestos Organometálicos/uso terapéutico , Fármacos Fotosensibilizantes/uso terapéutico , Porfirinas/uso terapéutico , Carcinoma de Células Escamosas de Cabeza y Cuello , Resultado del Tratamiento
7.
Br J Oral Maxillofac Surg ; 56(1): 60-63, 2018 01.
Artículo en Inglés | MEDLINE | ID: mdl-29223636

RESUMEN

Actinic keratosis, leukoplakia, carcinoma in situ, and superficially invasive carcinomas of the lower lip are caused mainly by the cumulative effects of exposure of the vermilion of the lower lip to ultraviolet radiation. Current treatments all have limitations: cryosurgery or electrosurgery is suitable to treat only focal lesions; topical chemotherapy, which is an option for diffuse actinic damage, yields unreliable results; and laser treatment fails to rejuvenate the vermilion. However, "lip shave", which involves full-thickness excision of the damaged vermilion and reconstruction with an advancement labial mucosal flap, will produce a fresh mucosal lining to the vermilion border. We describe our experience of the technique and evaluate the functional and aesthetic outcomes in 20 patients treated between January 2011 and January 2016. The follow-up period ranged from 24 to 60 months. Three-quarters of the patients had dysplasia or superficially invasive malignancy. Resected lesions were about 63mm long, 13.7mm wide, and 3.9mm deep. No patients had recurrence or secondary lesions during follow up, functional disturbance was minimal, and the cosmetic outcome pleasing. Lip shave can efficiently reconstruct the vermilion of the lower lip with minimum deformity.


Asunto(s)
Carcinoma in Situ/cirugía , Estética Dental , Queratosis Actínica/cirugía , Leucoplasia/cirugía , Neoplasias de los Labios/cirugía , Labio/cirugía , Procedimientos de Cirugía Plástica/métodos , Colgajos Quirúrgicos/cirugía , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma in Situ/terapia , Femenino , Estudios de Seguimiento , Humanos , Queratosis Actínica/patología , Queratosis Actínica/terapia , Leucoplasia/terapia , Labio/diagnóstico por imagen , Labio/patología , Neoplasias de los Labios/terapia , Masculino , Persona de Mediana Edad , Mucosa Bucal/patología , Mucosa Bucal/cirugía , Invasividad Neoplásica , Recurrencia Local de Neoplasia/cirugía , Recurrencia Local de Neoplasia/terapia , Neoplasias/cirugía , Neoplasias/terapia , Estudios Retrospectivos , Resultado del Tratamiento
8.
Biomed Res Int ; 2017: 6958250, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28695129

RESUMEN

OBJECTIVE: To assess the effectiveness and identify vocal fold leukoplakia types appropriate for nonsurgical treatment. METHODS: The vocal fold leukoplakia in 178 patients was divided by gross appearance into three subtypes: flat and smooth, elevated and smooth, and rough. All patients received nonsurgical treatment including smoking and drinking cessation, voice rest, omeprazole, and Chinese medication therapy. The clinical response of three subtypes was assessed after a 6-month follow-up. RESULTS: Vocal fold leukoplakia subtypes included flat and smooth (n = 66; 37.1%), elevated and smooth (n = 103; 57.9%), and rough (n = 9; 5.0%). The rate of complete response was 80.3%, 66.0%, and 0.0% for the 3 lesion types, respectively (rough versus flat and smooth, P < 0.001; rough versus elevated and smooth, P < 0.001, Fisher's exact test). The incidence of carcinoma in rough leukoplakia was significantly higher than that in smooth leukoplakia (44.4% versus 2.4%, P = 0.002, Fisher's exact test). Clinical type was the only significant factor for clinical response of nonsurgical treatment (P = 0.005, ordinal logistic regression). CONCLUSIONS: The effectiveness of nonsurgical treatment for smooth vocal fold leukoplakia is better in comparison to rough vocal fold leukoplakia. Smooth leukoplakia could be managed with nonsurgical treatment; more aggressive treatments should be considered for rough leukoplakia.


Asunto(s)
Leucoplasia/terapia , Pliegues Vocales/patología , Adulto , Anciano , Femenino , Humanos , Estimación de Kaplan-Meier , Leucoplasia/diagnóstico , Leucoplasia/cirugía , Masculino , Persona de Mediana Edad , Cuidados Posoperatorios , Resultado del Tratamiento
9.
J Voice ; 30(2): 215-20, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26001502

RESUMEN

OBJECTIVES: To quantitatively analyze the vibratory characteristics of vocal folds before and after conservative treatments to evaluate the outcomes of conservative treatments for vocal fold leukoplakia using videostrobokymography (VSK). STUDY DESIGN: This is a prospective study. METHODS: Twenty patients and 20 controls were enrolled into the study. All patients received conservative treatments for 3 weeks and received VSK examination before and 3 weeks after the treatments. All controls only received VSK examination once. Vocal fold lengths of 25%, 50%, and 75% were chosen as the line-scan positions to evaluate the vocal fold vibration. Open quotient (OQ) and asymmetry index (AI) were obtained using VSK. RESULTS: Significant improvements in the main symptoms including voice hoarseness were found. Videostroboscopic findings showed that the white lesions on the vocal folds almost completely disappeared in all patients, and the vocal fold flexibility returned to normal. All OQs and AIs at each line-scan position in patients before the treatments were larger than those in controls (P < 0.017), whereas all OQs and AIs at each line-scan position decreased 3 weeks after conservative treatments (P < 0.017). No significant differences in OQs and AIs at each line-scan position were detected between patients after the treatments and controls (P > 0.017). CONCLUSIONS: VSK could quantitatively evaluate the vibratory characteristics of vocal folds before and after the treatments, and conservative treatment could improve VSK measurements to normal control values, suggesting that VSK is a tool to assess the outcomes of the conservative treatments for vocal fold leukoplakia.


Asunto(s)
Quimografía , Enfermedades de la Laringe/diagnóstico por imagen , Leucoplasia/diagnóstico por imagen , Fonación , Estroboscopía , Grabación en Video , Pliegues Vocales/diagnóstico por imagen , Trastornos de la Voz/diagnóstico por imagen , Adulto , Fenómenos Biomecánicos , Estudios de Casos y Controles , Femenino , Humanos , Enfermedades de la Laringe/fisiopatología , Enfermedades de la Laringe/terapia , Leucoplasia/fisiopatología , Leucoplasia/terapia , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Estudios Prospectivos , Recuperación de la Función , Factores de Tiempo , Resultado del Tratamiento , Vibración , Pliegues Vocales/fisiopatología , Trastornos de la Voz/fisiopatología , Trastornos de la Voz/terapia , Calidad de la Voz
10.
Artículo en Chino | MEDLINE | ID: mdl-29798071

RESUMEN

Vocal fold leukoplakia is a descriptive diagnosis, and can be divided into simple hyperplasia, precancerous lesions(with atypical hyperplasia) and invasive carcinoma according to pathological types. Because of a close relationship with the laryngeal carcinoma, knowing the risk factors and treatment methods of vocal fold leukoplakia has grate significance in preventing the malignant change and recurrence. This paper summarizes the recent progress in clinical diagnosis and treatment of vocal cord leukoplakia, mainly including risk factors, means of examination, treatment and prognosis.


Asunto(s)
Leucoplasia , Disfunción de los Pliegues Vocales , Humanos , Neoplasias Laríngeas , Leucoplasia/diagnóstico , Leucoplasia/terapia , Recurrencia Local de Neoplasia , Lesiones Precancerosas , Disfunción de los Pliegues Vocales/diagnóstico , Disfunción de los Pliegues Vocales/terapia , Pliegues Vocales
13.
Pediatr Dermatol ; 28(4): 429-32, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21554383

RESUMEN

Pachyonychia congenita is a rare genodermatosis that can affect the larynx. Laryngeal obstruction is very unusual with only a few cases reported. A 2-year-old girl presented with typical clinical features of pachyonychia congenita shortly after birth. At age 9 months, following an upper respiratory infection, she developed stridor and hoarseness and was found to have severe laryngeal obstruction, which was felt to be secondary to pachyonychia congenita based on direct laryngoscopy and laryngeal biopsy. Leukokeratosis of her larynx was treated with CO(2) laser on three occasions, with improvement in her respiratory distress after each treatment. This report is the first case of pachyonychia congenita with laryngeal obstruction in which the gene mutation has been established (a deletional mutation in K6a), confirming that laryngeal obstruction can occur in PC-1.


Asunto(s)
Obstrucción de las Vías Aéreas/genética , Queratina-6/genética , Enfermedades de la Laringe/genética , Leucoplasia/genética , Paquioniquia Congénita/genética , Obstrucción de las Vías Aéreas/etiología , Obstrucción de las Vías Aéreas/cirugía , Secuencia de Bases , Preescolar , Femenino , Humanos , Enfermedades de la Laringe/etiología , Enfermedades de la Laringe/cirugía , Terapia por Láser , Leucoplasia/terapia , Paquioniquia Congénita/complicaciones , Paquioniquia Congénita/cirugía , Eliminación de Secuencia , Índice de Severidad de la Enfermedad
14.
Eur Arch Otorhinolaryngol ; 263(9): 843-52, 2006 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-16823559

RESUMEN

Laryngeal dysplasia is a common clinical concern. Despite major advancements in otolaryngology, a significant number of patients with the condition progress to invasive carcinoma. In the recent years, new diagnostic techniques such as autofluorescence and contact endoscopy have been applied to increase the accuracy of the laryngeal biopsies. Moreover, significant efforts have been made to correlate the histopathological appearance of the dysplastic samples to their clinical course. So far, a wide range of therapeutic strategies for different grades of dysplasia has been suggested by authors; however, there is a lack of outcome reviews. In the current review, the outcome of different therapeutic strategies for severe dysplasia and carcinoma in situ has been compared with statistical analysis. The paper also summarises the current knowledge of the alternative management methods as well as current areas of research in the chemoprevention of the condition. (1) Significant efforts have been made to develop new techniques to improve the accuracy of microlaryngoscopic assessment of laryngeal premalignancy. Comparative studies are required using microlaryngoscopy against the new techniques to evaluate their clinical utility. (2) The meta-analysis suggests a better local control rate with radiotherapy compared to other standard methods of management of dysplasia; however, functional impairment and complications associated with each of the standard treatments should be further evaluated.


Asunto(s)
Carcinoma in Situ/terapia , Neoplasias Laríngeas/terapia , Laringe/patología , Lesiones Precancerosas/terapia , Biopsia , Carcinoma in Situ/diagnóstico , Carcinoma in Situ/patología , Quimioterapia Adyuvante/métodos , Progresión de la Enfermedad , Humanos , Hiperplasia , Neoplasias Laríngeas/diagnóstico , Neoplasias Laríngeas/patología , Laringoscopía , Leucoplasia/diagnóstico , Leucoplasia/patología , Leucoplasia/terapia , Fotoquimioterapia/métodos , Lesiones Precancerosas/diagnóstico , Lesiones Precancerosas/patología , Radioterapia Adyuvante/métodos , Espectrometría de Fluorescencia
16.
Lik Sprava ; (3): 134-6, 1999.
Artículo en Ucraniano | MEDLINE | ID: mdl-10474961

RESUMEN

Methodological approaches have been developed toward raising the informative value of cytological diagnosis. There have been identified pathogenetic variants of background and precancerous affections of the vulva, their leading factors being viral infection, function disorders of the hypothalamic centres, hypoestrogenemia, hepatic and thyroidal abnormalities. The effectiveness of the treatment scheme developed with taking account of the above-stated items is 97.3%.


Asunto(s)
Leucoplasia/diagnóstico , Liquen Escleroso Vulvar/diagnóstico , Neoplasias de la Vulva/diagnóstico , Anciano , Biopsia , Terapia Combinada , Estrógenos/deficiencia , Femenino , Humanos , Leucoplasia/etiología , Leucoplasia/terapia , Persona de Mediana Edad , Factores de Tiempo , Vulva/patología , Liquen Escleroso Vulvar/etiología , Liquen Escleroso Vulvar/terapia , Neoplasias de la Vulva/etiología , Neoplasias de la Vulva/terapia
17.
Bone Marrow Transplant ; 19(4): 389-92, 1997 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-9051251

RESUMEN

Chronic restrictive lung disease in a 9-year-old boy with dyskeratosis congenita (DC) 7 years after allogeneic bone marrow transplantation (BMT) is described. When he was 1 year and 10 months old, severe aplastic anemia developed. He received a marrow transplant from his HLA serologically identical, but HLA-DP mismatched brother. He developed grade II acute graft-versus-host disease (GVHD) and thereafter chronic GVHD of progressive type, and was treated with both prednisolone and azathioprine resulting in clinical improvement. Thereafter he complained of dyspnea, and bilateral noncircumscribed interstitial shadows on chest CT scan were present. His pulmonary function showed restrictive changes. Prednisolone was not effective and he died of respiratory failure. Post-mortem examination confirmed interstitial fibrosis, lymphocytic infiltration of the bronchioles and alveoli with luminal fibrosis. There was no evidence of chronic GVHD in the skin and the liver. These findings raise the possibility that this pulmonary complication was associated with DC itself.


Asunto(s)
Anemia Aplásica/terapia , Trasplante de Médula Ósea/efectos adversos , Enfermedades Pulmonares Intersticiales/etiología , Anemia Aplásica/complicaciones , Resultado Fatal , Humanos , Hiperpigmentación/congénito , Hiperpigmentación/terapia , Lactante , Leucoplasia/congénito , Leucoplasia/terapia , Masculino , Enfermedades de la Uña/congénito , Enfermedades de la Uña/terapia , Trasplante Homólogo
20.
Dermatol Clin ; 13(1): 33-9, 1995 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-7712648

RESUMEN

Dyskeratosis congenita is a rare genodermatosis. Malignant deterioration and hematologic complications are well-described features of this syndrome. Correct recognition is essential for proper management. A review of diagnostic considerations and treatment guidelines is presented.


Asunto(s)
Leucoplasia/congénito , Enfermedades de la Uña/congénito , Lesiones Precancerosas/patología , Síndrome Rothmund-Thomson/genética , Adolescente , Adulto , Anemia Aplásica/patología , Niño , Femenino , Humanos , Leucoplasia/diagnóstico , Leucoplasia/patología , Leucoplasia/terapia , Masculino , Enfermedades de la Uña/diagnóstico , Enfermedades de la Uña/patología , Enfermedades de la Uña/terapia , Síndrome Rothmund-Thomson/diagnóstico , Síndrome Rothmund-Thomson/patología , Síndrome Rothmund-Thomson/terapia
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