Clinical relevance of macroglossia to disease progression in ventilation dependent patients with advanced ALS.

BACKGROUND: Macroglossia is one of the clinical signs that emerges in patients with amyotrophic lateral sclerosis (ALS) who are undergoing invasive ventilation. OBJECTIVE: We aimed to investigate the relationship between the progression of macroglossia and the clinical characteristics during the advanced stages of ALS. METHODS: Forty-one patients with ALS using invasive ventilation with tracheostomy were prospectively followed over a 3-year period. We evaluated the prevalence of macroglossia, motor disabilities including oral function, communication ability (communication stage I to V) as a marker of disease progression, and nutritional factors. We analyzed the potential factors affecting the progression of macroglossia using a multivariate logistic analysis. RESULTS: The number of patients with macroglossia was increased during the follow-up (from 24.4 to 53.7%), while the communication stage progressed from 2.6 in average (SD 1.7) to 3.5 (1.4). During the study, body weight increased, while energy intake decreased, suggesting progressive hypometabolism. Eight patients had newly developed macroglossia during the study and showed greater progression of communication impairment than those without macroglossia. Multivariate logistic regression analysis showed that communication impairment was a factor associated with macroglossia. CONCLUSION: Macroglossia in advanced ALS with invasive ventilation is associated with disease progression.

Manifestaciones bucales de pacientes con mucopolisacaridosis / Oral manifestations in patients with mucopolysaccharidosis

Objetivo: Describir las características bucales prevalentes de pacientes argentinos con mucopolisacaridosis (MPS) atendidos en el Servicio de Odontología del Hospital Nacional "Prof. Alejandro Posadas". Materiales y métodos: Se consideraron las historias clínicas de 19 pacientes con diagnóstico de MPS. Se registraron la edad, el sexo, el lugar de residencia, el tipo de MPS y la presencia de retraso madurativo. La muestra estuvo constituida por 13 niños (6,7±3 años) y 6 adultos (26±9 años): 2 eran mujeres (1 con MPS tipo I; 1 con MPS tipo IV A) y 17 eran hombres (15 con MPS tipo 2; 1 con MPS tipo 1; 1 con MPS tipo III); 13 de los pacientes presentaban discapacidad intelectual. Se evaluaron: tipo de dentición, oclusión, macroglosia, hipoplasias del esmalte, tipo de respiración predominante, clase molar y tratamiento realizado. Resultados: Ambos casos con MPS I presentaban mordida abierta anterior y giroversión dental, y solo uno de estos, diastemas, microdoncia, hipoplasias del esmalte, macroglosia y respiración bucal. De los 15 pacientes con MPS II, 11 presentaban mordida abierta anterior (73%), 3 mordida cruzada posterior (20%), 5 giroversión dental (33%), 11 diastemas (73%), 3 retraso en la erupción (20%), 4 hiperplasia gingival (26%), 13 macroglosia (87%), 7 hipoplasias del esmalte (47%), 2 microdoncia (13%), 9 respiración bucal (60%). Se registraron 5 pacientes con clase molar I (33%), 3 con clase molar II (20%), 3 con clase molar III (20%) y en 3 casos no se pudo evaluar (20%). En el paciente con MPS tipo III se halló mordida abierta anterior, diastemas, retraso en la erupción, macroglosia, respiración bucal y clase molar II; y en el caso de MPS tipo IV A, mordida abierta anterior, diastemas, hiperplasia gingival, macroglosia y clase molar II. El 90% de los pacientes requirió tratamiento odontológico (AU)

Aim: To identify the most prevalent oral manifestations of 19 Argentine patients with mucopolysaccharidos (MPS) attending the Dentistry Service of the National Posadas Hospital. Materials and methods: The medical records of 19 patients diagnosed with MPS were considered. Age, sex, place of residence, type of MPS, and presence of maturational delay were recorded. The sample consisted of 13 children (6.7 ± 3 years) and 6 adults (26 ± 9 years): 2 were women (1 with MPS type I; 1 with MPS type IV A) and 17 were men (15 with MPS type 2; 1 with MPS type 1; 1 with MPS type III); 13 of the patients had intellectual disabilities. The following were evaluated: type of dentition, occlusion, macroglossia, enamel hypoplasia, predominant type of respiration, molar class and treatment performed Results: Both cases with MPS I presented anterior open bite and dental gyroversion, and only one of these, diastemas, microdontia, enamel hypoplasia, macroglossia and mouth respiration. Of the 15 patients with MPS II, 11 presented anterior open bite (73%), 3 posterior crossbite (20%), 5 dental gyroversion (33%), 11 diastemas (73%), 3 delayed eruption (20%), 4 gingival hyperplasia (26%), 13 macroglossia (87%), 7 enamel hypoplasia (47%), 2 microdontia (13%), 9 mouth breathing (60%). 5 patients with molar class I (33%), 3 with molar class II (20%), 3 with molar class III (20%) and in 3 cases it could not be evaluated (20%). In the patient with type III MPS, anterior open bite, diastemas, delayed eruption, macroglossia, mouth breathing and molar class II were found; and in the case of type IV A MPS, anterior open bite, diastemas, gingival hyperplasia, macroglossia and molar class II. 90% of the patients required dental treatment. Conclusion: The most observed oral manifestations were macroglossia (84.2%) and anterior open bite (73%) (AU)

Prevalencia de lesiones bucales en tejido blando encontradas en la Clínica de Estomatología de la Facultad de Odontología de la Universidad de los Andes: periodo 2015-2018 / Prevalence of oral lesions in soft tissue found in the Clinic of Stomatology of the Faculty of Dentistry of the University of los Andes: 2015-2018 period

Introducción: La epidemiología sobre alteraciones en tejidos blandos bucales es limitada cuando se compara con caries, enfermedades periodontales y maloclusiones, por lo que su estudio representa un paso adelante en la odontología más allá de los dientes. Objetivo: Describir la prevalencia de las lesiones bucales en tejido blando encontradas en la Clínica de Estomatología de la Facultad de Odontología de la ULA, del 2015 al 2018, con la finalidad de proporcionar una fuente de datos actualizada, que oriente a una mejor prevención y oportuno diagnóstico. Material y métodos: Se realizó una investigación descriptiva, documental, retrospectiva y multivariable. Resultados: De 1,000 fichas clínicas estudiadas, las patologías más frecuentes fueron: lengua saburral (50.6%), queilitis (33.4%), traumatismo de la mucosa de los carrillos (27.6%), várices linguales (18.3%), anquiloglosia (13.7%) y agrandamiento de rugas palatinas (11.7%). La zona con mayor alteración fue la lengua (92.1%), mientras que el paladar fue la menos afectada (29%). De los hábitos predisponentes a la formación de lesiones, el mordisqueo de mucosa fue el más común (16.5%). En cuanto a los factores locales asociados, una higiene oral regular/ deficiente resultó el principal (53.8%). Conclusión: La educación del paciente sigue siendo considerada la clave para disminuir la aparición de patologías y su evolución a entidades más graves (AU)

Introduction: Epidemiological studies on soft tissue alterations in the mouth are limited when compared with caries, periodontal diseases and malocclusions, so their study represents a step forward in dentistry beyond the teeth. Objective: To describe the prevalence of soft tissue oral lesions found in the Stomatology Clinic of the ULA School of Dentistry, from 2015 to 2018, in order to provide an up-to-date data source, to guide better prevention and timely diagnosis. Material and methods: A descriptive and documentary, retrospective and multivariable research was carried out. The statistical package IBM SPSS Statistics v 23 was applied for the analysis. Results: 1,000 clinical records studied, the most frequent pathologies were: saburral tongue (50.6%), cheilitis (33.4%), traumatism of the cheek mucosa (27.6%), lingual varices (18.3%), ankyloglossia (13.7%) and enlargement of palatal rugas (11.7%). The tongue was the most affected (92.1%), while the palate was the least affected (29%). Of the predisposing habits to the formation of lesions, mucosal nipping was the most common (16.5%). Regarding the associated local factors, a regular / deficient oral hygiene was the main one (53.8%). Conclusion: Patient education is still considered the key to diminish not only the appearance of pathologies but their evolution to more serious entities (AU)

Neonatal Macroglossia: Demographics, Cost of Care, and Associated Comorbidities.

OBJECTIVES: To examine the birth prevalence of congenital macroglossia and identify demographic variables and comorbidities that may influence length of stay and cost of care. STUDY DESIGN: Retrospective cross-sectional study using the Kids' Inpatient Database 2003, 2006, 2009, and 2012. METHODS: Demographics were analyzed. Linear regression modeling and multivariate analyses were performed. RESULTS: The birth prevalence of congenital macroglossia was 4.63/100 000 births. Patients were classified as isolated (n = 349, 48.1%) or syndromic (n = 377, 51.9%). A higher incidence of isolated macroglossia was seen in females (odds ratio, OR [95% confidence interval, 95% CI]: 1.93 [1.45-2.56] and African Americans (2.02 [1.41-2.88]). Length of stay was higher for syndromic patients than for nonsyndromic patients (22.6 days [18.6-26.6] vs 7.93 days [5.95-9.90], as were inpatient costs ($3619USD [$27 628-$44 754] vs $10 168USD [$6272-$14 064]. After accounting for gender, race, location, and socioeconomic status, the presence of macroglossia alone increased length of stay by 4.07 days (0.42-7.72 days) in nonsyndromic patients and 12.02 days (3.63-20.4 days) in syndromic patients. The cost of care increased by $6207USD ($576-$11 838) among nonsyndromic newborns and $17 205USD ($374-34 035) among syndromic patients. CONCLUSION: The birth prevalence of congenital isolated macroglossia appears to vary by sex and race. Prolonged length of stay and increased costs are associated with both isolated macroglossia and syndromic macroglossia, even after controlling for other syndromic comorbidities.

Videolaringoscopio King Vision®, ¿una alternativa en la intubación del paciente despierto? / Videolaryngoscope King Vision™, an alternative in the intubation of the awake patient?

No disponible

Challenges in the diagnosis and management of acromegaly: a focus on comorbidities.

INTRODUCTION: Acromegaly is a rare, insidious disease resulting from the overproduction of growth hormone (GH) and insulin-like growth factor 1 (IGF-1), and is associated with a range of comorbidities. The extent of associated complications and mortality risk is related to length of exposure to the excess GH and IGF-1, thus early diagnosis and treatment is imperative. Unfortunately, acromegaly is often diagnosed late, when patients already have a wide range of comorbidities. The presence of comorbid conditions contributes significantly to patient morbidity/mortality and impaired quality of life. METHODS: We conducted a retrospective literature review for information relating to the diagnosis of acromegaly, and its associated comorbidities using PubMed. The main aim of this review is to highlight the issues of comorbidities in acromegaly, and to reinforce the importance of early diagnosis and treatment. FINDINGS AND CONCLUSIONS: Successful management of acromegaly goes beyond treating the disease itself, since many patients are diagnosed late in disease evolution, they present with a range of comorbid conditions, such as cardiovascular disease, diabetes, hypertension, and sleep apnea. It is important that patients are screened carefully at diagnosis (and thereafter), for common associated complications, and that biochemical control does not become the only treatment goal. Mortality and morbidities in acromegaly can be reduced successfully if patients are treated using a multimodal approach with comprehensive comorbidity management.

Oral health status of children suffering from thyroid disorders.

BACKGROUND: Thyroid dysfunction is the second most common disorder of the endocrine system that can affect any system of the body. The oral cavity can be adversely affected by either an excess or deficiency of these hormones. AIM OF THE STUDY: To assess and compare the oral health status of children suffering from thyroid disorders and healthy children. MATERIALS AND METHODS: A total of 200 children aged between 2 years and 16 years were allocated into two groups. The study group consisted of 100 children with thyroid dysfunction (hypothyroidism/hyperthyroidism), while the control group consisted of 100 healthy children. Gingival index, plaque index, DMFT (Decayed missing filled teeth Index for permanent teeth) & Dmft index (Decayed missing filled teeth Index for primary teeth) and modified developmental defects of enamel (DDE) index were recorded and data were analyzed statistically. RESULTS: Plaque and gingival scores were significantly higher in the thyroid group compared to the control group. DMFT and dmft scores were higher in the thyroid group than the control group but the difference in score was not statistically significant. Statistically significant DDE score was found in the thyroid group. Apart from increased susceptibility to caries and poor periodontal health condition, children with thyroid disorders were also found to have other oral manifestations such as macroglossia, open bite, and change in eruption pattern. CONCLUSION: Thyroid dysfunction (both hypothyroidism and hyperthyroidism) has impact on the oral health status. Children with thyroid disorders showed high prevalence of dental caries and periodontal disease compared to the control group.

Macroglossia in advanced amyotrophic lateral sclerosis.

INTRODUCTION: An enlarged tongue (macroglossia) has been reported in advanced-stage patients with amyotrophic lateral sclerosis (ALS). METHODS: In this study we examined the prevalence of macroglossia and analyzed clinical correlations in 65 ALS patients on tracheostomy-invasive ventilation (TIV). RESULTS: Macroglossia was found in 22 patients (33.8%). Compared with those without macroglossia, patients with macroglossia had a younger age of onset, longer duration of disease and TIV use, lower ALS Functional Rating Scale score, higher body mass index, lower energy intake, more severe communication impairment, and lower oral function. Logistic multivariate analysis showed that body mass index (BMI; P = 0.007) and communication impairment (P = 0.029) were significantly correlated with macroglossia. The duration of TIV use was at the cut-off level of significance (P = 0.05). CONCLUSIONS: Macroglossia may be the result of overfeeding and replacement by fat during long-term TIV use in patients with advanced ALS. Muscle Nerve, 2016 Muscle Nerve 54: 386-390, 2016.

Pediatric otorhinolaryngologic manifestations of endocrinological pathologies.

OBJECTIVE: Aim of the present review is to offer a concise but complete discussion about pathologies with pediatric otorhinolaryngological interest recognizing: (i) growth disorders, (ii) chromosomal disorders, and (iii) tumors. METHODS: Pathologies characterized by a growth anomaly have been grouped, underlining the characteristics of otorhinolaryngological interest. Numerous syndromes typical of the pediatric age have been reported and classified according to the ailments of the orofacial district. Tumoral pathologies, for which the otorhinolaryngological operation is fundamental, have been descripted. We reviewed recent literature and older papers. We did not find recent literature regarding otorhinolaryngological features related to some of the pathologies described. RESULTS/CONCLUSIONS: Otorhinolaryngologists should be familiar with pediatric disorders, since many of them have head and neck manifestations. This knowledge allows to treat them with a correct surgical approach, in order to implore children's quality of life.

Macroglosia y el síndrome de Down / No disponible

No disponible

El SAHS en la edad pediátrica. Clínica, diagnóstico y tratamiento / No disponible

No disponible

Agravos constitucionais da boca em pré-escolares da Rede Municipal de Ensino da Cidade de Säo Paulo / Offense constitutionalists of mouth in children of Rede Municipal de Ensino da cidade de Säo Paulo

Ao mesmo tempo em que interferem apenas discretamente no desenvolvimento intelectual, os agravos constitucionais da boca constituem importante fator na vida de correlaçäo de crianças pré-escolares. Na presente comunicaçäo, apresentam-se e discutem-se características detectadas em tais lesöes, em epidemiológico exploratório realizado em amostra de 2.549 crianças representativas da populaçäo das unidade de educaçäo pré-escolar da rede municipal de ensino da cidade de Säo Paulo. Foi constatada a ocorrência de 25 agravos constitucionais da boca, sendo seis fendas labiais e seis fendas palatinas. A discussäo de tais resultados é travada tanto a partir do interesse para com as variáveis epidemiológicas que encerram (como prevalência e distribuiçäo por sexo e faixa etária) como pelos aspectos nosográficos envolvidos, particularmente no que se refere a associaçöes com agravos constitucionais em outros segmentos corporais