Tendinitis cálcica atraumática de tendón extensor común del dedo medio: un caso infrecuente de dolor metacarpofalángico / Atraumatic calcium tendonitis of common extensor tendon of the middle finger: a rare case of metacarpophalangeal pain

Calcific tendinitis is a pathology characterized by the deposits of periarticular hydroxyapatite. Its pathophysiology is not completely known. It is clinically characterized by important inflammatory changes with incapacitating pain. It most commonly affects the shoulder joint and it rarely affects the hand and wrist. Given the unusual nature of this localization, we present the clinical case of a woman who developed calcific tendinitis of the third metacarpophalangeal muscle. We present the clinical evolution of the case, the treatments carried out, and a review of the literature related to this unusual localization of calcific tendinitis.

Calcinosis cutis congénita tumoral asociada con encefalopatía epiléptica infantil temprana con variante patogénica en el gen FGF12 / Tumorous congenital calcinosis cutis associated with early childhood epileptic encephalopathy with a pathogenic FGF12 gene variant

Resumen Introducción: La calcinosis cutis es el depósito de sales insolubles de calcio en la piel y se clasifica, de acuerdo con su patogénesis, en distrófica, metastásica, idiopática, iatrogénica y calcifilaxis. La calcinosis idiopática se presenta en pacientes sanos y es asintomática; incluye la calcinosis escrotal, la calcinosis nodular de Winer o nódulos calcificados subepidérmicos y la calcinosis tumoral familiar. Esta última es una condición rara que se caracteriza por el depósito de calcio periarticular en pacientes normocalcémicos sin conexión al hueso. Caso clínico: Paciente de sexo masculino de 5 meses de edad, quien al séptimo día de vida fue hospitalizado por ictericia multifactorial, sepsis neonatal tardía y apnea con crisis epilépticas. La evolución fue tórpida, con ingresos hospitalarios por crisis epilépticas de difícil manejo, respuesta parcial a la difenilhidantoína y descontrol electrolítico. Mediante la secuenciación del exoma dirigido se detectó una variante patogénica de sentido equivocado en FGF12 que confirmó el diagnóstico de encefalopatía epiléptica temprana número 47. Además, el paciente presentó dermatosis congénita diseminada a las extremidades inferiores con afección en muslos, asintomática, bilateral y simétrica, constituida por hipopigmentación y fóveas duras a la palpación profunda. La biopsia mostró calcificación distrófica. Conclusiones: Se presenta el caso de un lactante con calcinosis cutis congénita profunda asociada con una variante patogénica en el gen FGF12 y con encefalopatía epiléptica, situación clínica que, a la fecha, no había sido reportada en la literatura.

Abstract Background: Calcinosis cutis is the deposit of insoluble calcium salts in the skin. It is classified according to its pathogenesis in dystrophic, metastatic, idiopathic, iatrogenic, and calciphylaxis. Idiopathic calcinosis is asymptomatic, occurs in healthy patients, and includes scrotal calcinosis, Winer's nodular calcinosis or subepidermal calcified nodules, and familial tumor calcinosis. The latter is a rare condition characterized by periarticular calcium deposition in normocalcemic patients with no bone connection. Case report: The case of a 5-month-old male patient, who on the seventh day of life was hospitalized for multifactorial jaundice, late neonatal sepsis, and apnea with epileptic seizures is described. His evolution was torpid, with hospital admissions due to epileptic seizures that were difficult to manage with partial response to the use of diphenylhydantoin and electrolyte alterations. By means of exome sequencing directed, a pathogenic variant of wrong direction in FGF12 was detected and the diagnosis of early epileptic encephalopathy number 47 was confirmed. Also, the patient showed disseminated congenital dermatosis to lower extremities affecting thighs, asymptomatic, bilateral and symmetrical, constituted by hypopigmentation and fovea hard to deep palpation. The biopsy showed dystrophic calcification Conclusions: The case of an infant with deep congenital cutis calcinosis associated with a pathogenic variant in the FGF12 gene with epileptic encephalopathy is described. To date, this clinical situation has not been previously reported in the literature.

Estenosis mitral con calcificación severa del anillo mitral: reemplazo mitral con prótesis OnX Conform en posición supraanular / Mitral stenosis with severe annular calcification: mitral valve replacement with an OnX Conform prosthesis in a supra annular position

Abstract A 68-year-old man previously subjected to radiotherapy had a prior aortic valve replacement due de radiation induced calcification of the aortic valve. Presently the patient developed severe calcification of the mitral valve ring leading to critical mitral valve stenosis. A supra annular implantation of an On X Conform valve was successfully achieved. The clinical course was uneventful, and the echocardiographic evaluation demonstrated a normal function of the valve. Different alternatives for the surgical management of this complication are discussed.

Hipoparatiroidismo y calcificaciones cerebrales: reporte de caso / Hypoparathyroidism and brain calcifications: a case report

Introducción. El hipoparatiroidismo es una enfermedad caracterizada por la ausencia o concentraciones inadecuadamente bajas de hormona paratiroidea (PTH), que conduce a hipocalcemia, hiperfosfatemia y excreción fraccional elevada de calcio en la orina. Las calcificaciones del sistema nervioso central son un hallazgo frecuente en estos pacientes. Caso clínico. Mujer de 56 años con antecedente de hipotiroidismo, que ingresó por un cuadro de 6 días de evolución caracterizado por astenia, parestesias periorales y movimientos anormales de manos y pies. Las pruebas de laboratorio demostraron hipocalcemia, hiperfosfatemia y niveles bajos de hormona paratiroidea. Se realizó una tomografía computarizada de cráneo que mostró áreas bilaterales y simétricas de calcificaciones en hemisferios cerebelosos, ganglios basales y corona radiata. No se evidenciaron trastornos en el metabolismo del cobre y hierro. Se estableció el diagnóstico del síndrome de Fahr secundario a hipoparatiroidismo y se inició tratamiento con suplementos de calcio y vitamina D con evolución satisfactoria. Discusión. El síndrome de Fahr es un trastorno neurológico caracterizado por el depósito anormal de calcio en áreas del cerebro que controlan la actividad motora. Se asocia a varias enfermedades, especialmente, hipoparatiroidismo. La suplementación con calcio y vitamina D con el objetivo de normalizar los niveles plasmáticos de estos cationes es el tratamiento convencional. (AU)

Introduction. Hypoparathyroidism is a disease characterized by absence or inappropriately low concentrations of circulating parathyroid hormone, leading to hypocalcaemia, hyperphosphataemia and elevated fractional excretion of calcium in the urine. Central nervous system calcifications are a common finding in these patients. Case report. 56-year-old woman with a history of hypothyroidism who was admitted for a 6-day course of illness characterized by asthenia, perioral paresthesias, and abnormal movements of the hands and feet. Laboratory tests showed hypocalcemia, hyperphosphatemia, and low parathyroid hormone levels. A cranial computed tomography was performed. It showed bilateral and symmetrical areas of calcifications in the cerebellar hemispheres, basal ganglia, and radiata crown. No disorders of copper or iron metabolism were evident. The diagnosis of Fahr syndrome secondary to hypoparathyroidism was established and treatment with calcium and vitamin D supplements was started with satisfactory evolution. Discussion. Fahr's syndrome is a neurological disorder associated with abnormal calcium deposition in areas of the brain that control motor activity. It is associated with various diseases, especially hypoparathyroidism. The conventional treatment is supplementation with calcium and vitamin D, with the aim of normalizing their plasma levels. (AU)

The relationship between mitral annular calcification, metabolic syndrome and thromboembolic risk

Abstract Introduction: Metabolic syndrome (MetS) is defined as an association between diabetes, hypertension, obesity and dyslipidemia and an increased risk of cardiovascular disease. Mitral annular calcification (MAC) is associated with several cardiovascular disorders, including coronary artery disease, atrial fibrillation (AF), heart failure, ischemic stroke and increased mortality. The CHA2DS2-VASc score is used to estimate thromboembolic risk in AF. However, the association among MAC, MetS and thromboembolic risk is unknown and was evaluated in the current study. Methods: The study group consisted of 94 patients with MAC and 86 patients with MetS. Patients were divided into two groups: those with and those without MAC. Results: Patients with MAC had a higher MetS rate (P<0.001). In patients with MAC, the CHA2DS2-VASc scores and the rate of cerebrovascular accident and AF were significantly higher compared to those without MAC (P<0.001, for both parameters). The results of the multivariate regression analysis showed that history of smoking, presence of MetS and high CHA2DS2-VASc scores were associated with the development of MAC. ROC curve analyses showed that CHA2DS2-VASc scores were significant predictors for MAC (C-statistic: 0.78; 95% CI: 0.706-0.855, P<0.001). Correlation analysis indicated that MAC was positively correlated with the presence of MetS and CHA2DS2-VASc score (P=0.001, r=0.264; P<0.001, r=0.490). Conclusion: We have shown that CHA2DS2-VASc score and presence of MetS rates were significantly higher in patients with MAC compared without MAC. Presence of MAC was correlated with CHA2DS2-VASc score, presence of MetS, AF and left atrial diameter and negatively correlated with left ventricular ejection fraction.

Lithopedion causing intestinal obstruction in a 71-year-old woman: A case report / Litopedia causando obstrução intestinal em idosa de 71 anos: relato de caso

Abstract Ectopic pregnancy is the leading cause of pregnancy-related death during the first trimester, and it occurs in 1 to 2% of pregnancies. Over 90% of ectopic pregnancies are located in the fallopian tube. Abdominal pregnancy refers to an ectopic pregnancy that has implanted in the peritoneal cavity, external to the uterine cavity and fallopian tubes. The estimated incidence is 1 per 10,000 births and 1.4%of ectopic pregnancies. Lithopedion is a rare type of ectopic pregnancy, and it occurs when the fetus from an unrecognized abdominal pregnancymay die and calcify. The resulting "stone baby" may not be detected for decades andmay cause a variety of complications. Lithopedion is a very rare event that occurs in 0.0054% of all gestations. About 1.5 to 1.8% of the abdominal babies develop into lithopedion. There are only ~ 330 known cases of lithopedion in the world. We describe a lithopedion that complicated as intestinal obstruction in a 71-year-old woman.

Resumo A gravidez ectópica é a principal causa de morte materna no primeiro trimestre, e ocorre em 1 a 2% das gestações. Mais de 90% ocorrem nas tubas uterinas. Gravidez abdominal refere-se à gravidez ectópica implantada na cavidade peritoneal, externamente ao útero e às tubas uterinas.Aincidência estimada éde 1 por 10mil nascimentos e 1,4%das gravidezes ectópicas. A litopedia é um tipo raro de gravidez ectópica, e ocorre quando o feto de uma gravidez abdominal não reconhecida morre e se calcifica. O "bebê de pedra" resultante pode não ser detectado por décadas, e pode causar complicações futuras. A litopedia é um evento muito raro que ocorre em 0,0054% de todas as gestações. Cerca de 1,5 a 1,8% dos bebês abdominais se tornam litopédios. Existem somente cerca de 330 casos conhecidos de litopedia no mundo. Descrevemos uma litopedia que se agravou, tornando-se uma obstrução intestinal, em uma idosa de 71 anos.

Microlitiasis alveolar pulmonar. Reporte de caso / Pulmonary Alveolar microlithiasis, Case Report

Antecedentes: La microlitiasis es una afección pulmonar poco frecuente, caracterizada por acumulo de fosfatos de calcio en el interior de los alveolos pulmonares, originando cuerpos nodulares conocidos como microlitos, siendo esta su principal caracterís-tica. Destacando la poca relación Clínico Radiológica, dada su escasa documentación y relativamente poco estudio, es difícil su diag-nóstico y frecuentemente es confundida con otras entidades patológicas. Caso Clínico: Femenina de 38 años, hipertensa controlada con atenolol 100 mg vía oral día, exposición al humo de leña, con 6 meses de tos intermitente, seca, disnea y sibilancias nocturnas, se le practicó una tomografía que en la ventana mediastial se aprecia corazón de tamaño normal con algunas adenomegalias de pocos milímetros para órticos y peri traqueales no sospechosos y en la ventana pulmonar hay llenado alveolar homogéneo difuso bilateral, con engrosamiento nodular centro lobular y septal con calciicaciones inas de la pleura. La biopsia trasbronquial reporta espacios al-veolares con presencia de concentraciones laminares en piel de cebolla basóilos intra alveolares compatible con microlitiasis alveolar. Discusión: Identiicar estas particularidades nos permitiría realizar un abordaje temprano para evitar complicaciones iniciales de esta enfermedad, aunque no se conoce un tratamiento curativo...(AU)

Reversal of uremic tumoral calcinosis by optimization of clinical treatment of bone and mineral metabolism disorder / Reversão da calcinose tumoral urêmica pela otimização do tratamento clínico da desordem do metabolismo ósseo e mineral

Abstract Tumoral calcinosis is an uncommon type of extraosseous calcification characterized by large rubbery or cystic masses containing calcium-phosphate deposits. The condition prevails in the periarticular tissue with preservation of osteoarticular structures. Elevated calcium-phosphorus products and severe secondary hyperparathyroidism are present in most patients with uremic tumoral calcionosis (UTC). Case report of an obese secondary to chronic glomerulonephritis, undergoing continuous ambulatory peritoneal dialysis (CAPD) reported the appearance of painless tumors in the medial surface of fifth finger and left arm. Tumoral calcinosis was confirmed by left biceps biopsy. Poor adherence to CAPD. The patient was transferred to the "tidal" modality of peritoneal dialysis and after was treated by hemodialysis, despite the persistence of severe hyperparathyroidism progressive reduction of UTC until near to its complete disappearance. Nowadays, one year after patient received deceased-donor kidney transplantation, he presents with an improvement in secondary hyperparathyroidism. UTC should be included in the elucidation of periarticular calcification of every patient on dialysis. Relevant laboratory findings such as secondary hyperparathyroidism and elevated calcium- phosphorus products in the presence of periarticular calcification should draw attention to the diagnosis of UTC.

Resumo A calcinose tumoral é um tipo raro de calcificação extraóssea caracterizada por grandes massas císticas e elásticas contendo depósitos de fosfato de cálcio. A condição é mais prevalente no tecido periarticular e preserva estruturas osteoarticulares. A elevação do produtos cálcio-fósforo e o hiperparatireoidismo secundário grave estão presentes na maioria dos pacientes com calcinose tumoral urêmica (UTC). O relato de caso em questão refere-se a um homem de 22 anos, branco, obeso, com doença renal crônica secundária à glomerulonefrite crônica, em diálise peritoneal ambulatorial contínua (CAPD), que apresentou aparecimento de tumores indolores na face medial do quinto quirodáctilio e braço esquerdo. A calcinose tumoral foi confirmada por biópsia do bíceps esquerdo. O paciente apresentava baixa adesão à CAPD. Foi transferido para a modalidade de diálise peritoneal e depois iniciou tratamento por hemodiálise. Apesar da persistência do hiperparatireoidismo grave, houve redução progressiva da UTC, com resolução próxima do seu desaparecimento completo. Há 1 ano o paciente foi submetido a transplante renal, doador falecido, e apresentou melhora do hiperparatiroidismo secundário. A UTC deve ser incluída na elucidação de calcificação periarticular de pacientes em diálise. Os achados laboratoriais relevantes, tais como hiperparatiroidismo secundário e elevação dos produtos cálcio-fósforo na presença de calcificação periarticular, devem chamar a atenção para o diagnóstico da UTC.

Prevalencia y asociación de la calcificación valvular aórtica con factores de riesgo y aterosclerosis coronaria en población mexicana / Aortic valve calcification prevalence and association with coronary risk factors and atherosclerosis in Mexican population

Resumen: Objetivo: La prevalencia de calcificación valvular aórtica (CVA) tiene influencia importante de la etnia y se desconoce en población mexicana. Nuestro objetivo fue investigar la prevalencia de CVA y sus asociaciones con factores de riesgo cardiovascular y calcificación arterial coronaria (CAC). Método: En 1,267 sujetos (53% mujeres) sin enfermedad coronaria conocida y con edad de 35 a 75 años, la CVA y la CAC se evaluaron mediante tomografía computada multidetector, utilizando el método de Agatston. Los factores de riesgo cardiovascular se documentaron en todos los participantes. Las asociaciones de CVA con CAC y factores de riesgo se estimaron usando el análisis de regresión logística múltiple. Resultados: La prevalencia global de CVA y CAC fue del 19.89% y del 26.5%, respectivamente. Ambas condiciones aumentaron con la edad y se encontraron con mayor frecuencia en hombres (25.5 y 37.1%, respectivamente) que en mujeres (14.9 y 13%, respectivamente). La CVA se observó en únicamente el 8.5% de los sujetos sin CAC, mientras que en aquellos con CAC 1-99, 100-399 y > 400 unidades Agatston, las prevalencias fueron del 36.8, 56.8 y 84%, respectivamente. El análisis de regresión logística múltiple ajustado por edad, género, obesidad, inactividad física, hipertensión, dislipidemia y valores altos de insulina, mostró que la presencia de CAC (RM [IC95%]: 3.23 [2.26-4.60]), obesidad (1.94 [1.35-2.79]), género masculino (1.44 [1.01-2.05]) y edad (1.08 [1.03-1.10]), fueron predictores independientes y significativos de la CVA. Conclusiones: La prevalencia de CVA es alta y se asocia significativamente con factores de riesgo aterosclerótico y CAC en población mexicana.

Abstract: Objetive: The prevalence of aortic valve calcification (AVC), strongly influenced by ethnicity, is unknown in Mexican population. The aim of this study was to investigate the prevalence of AVC and its associations with cardiovascular risk factors and coronary artery calcification (CAC), in Mexican subjects. Methods: In 1,267 subjects (53% women) without known coronary heart disease, aged 35 to 75 years, AVC and CAC were assessed by multidetector-computed tomography using the Agatston score. Cardiovascular risk factors were documented in all participants. The associations of AVC with CAC and risk factors were assessed by multivariable logistic regression analyses. Results: The overall prevalence of AVC and CAC was 19.89% and 26.5%, respectively. AVC and CAC increased with age and were found more frequently in men (25.5% and 37.1%, respectively) than in women (14.9% and 13.0%, respectively). AVC was observed in only 8.5% of subjects wit-hout CAC, while those with CAC 1-99, 100-399, and > 400 Agatston units had AVC prevalences of 36.8%, 56.8%, and 84.0%, respectively. The multivariable logistic regression analyses, adjusted for age, gender, obesity, physical inactivity, hypertension, dyslipidemia and high insulin levels, showed that the presence of CAC (OR [CI95%]: 3.23 [2.26-4.60]), obesity (1.94 [1.35-2.79]), male gender (1.44 [1.01-2.05]) and age (1.08 [1.03-1.10]), were significant independent predictors of AVC. Conclusion: Prevalence of AVC is high and significantly associated with atherosclerotic risk factors and CAC in this Mexican population.

Milia-like calcinosis cutis in a girl with Down syndrome

Abstract: Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, milia-like papules, mostly found on the hands. MICC can disappear spontaneously by adulthood; therefore, its early recognition is crucial to avoiding unnecessary interventions. Herein, we present a case of MICC in a 6-year-old girl with Down syndrome.

Discopatía calcificante de la infancia: una infrecuente causa de tortícolis adquirida. Caso clínico / Pediatric intervertebral disc calcification: A rare cause of acquired torticollis. Case report

Introducción: El dolor y la contractura muscular cervical son motivos de consulta usuales en los servicios de urgencia pediátrica. El primer enfrentamiento es la anamnesis y examen físico minucioso. Ante la sospecha de etiología musculoesquelética se debe solicitar radiografía de columna cervical. El hallazgo de calcificación de los discos intervertebrales, en ausencia de otras lesiones radiológicas, nos debe hacer sospechar de una discopatía calcificante de la infancia. Objetivo: Presentar un caso de discopatía calcificante de la infancia, enfermedad infrecuente, que se debe tener en cuenta como diagnóstico diferencial de tortícolis y dolor cervical en la infancia. Caso clínico: Paciente varón de 7 años, sin antecedentes mórbidos ni historia de traumatismo o deportes bruscos. Consultó por historia de dolor y contractura cervical de 6 días de evolución. La velocidad de eritrosedimentación y proteína C reactiva estaban discretamente elevadas. La radiografía de columna cervical y la tomografía computarizada mostraron calcificación discal C5-C6 y protrusión discal anterior. Se hospitalizó para estudio y tratamiento del dolor, con buena respuesta clínica, continuando el manejo ambulatorio con antiinflamatorios no esteroidales y collar blando. Evolucionó con resolución de la sintomatología clínica y de las calcificaciones a 6 meses de seguimiento. Conclusiones: El hallazgo de calcificaciones de los discos intervertebrales es una infrecuente causa de tortícolis adquirida, de evolución benigna y autolimitada. Se recomienda manejo conservador por tiempo acotado y seguimiento clínico e imagenológico.

Introduction: Pain and cervical muscle spasm are common reasons why parents bring children to the pediatric emergency department. The first steps are the gathering of medical history of the patient and a physical examination. If musculoskeletal damage is suspected, cervical spine x-rays should be obtained. An intervertebral disc calcification finding, in the absence of other radiological lesions should suggest pediatric intervertebral disc calcification. Objective: To present a case of intervertebral disc calcification, a rare condition that must be considered in the differential diagnosis of torticollis and neck pain in childhood. Case report: A seven-year-old male patient without morbid history and no history of trauma or rough sport practice. He consulted the emergency room for pain and cervical contracture for the last six days. C reactive protein and red cell sedimentatio rates were slightly elevated. Imaging studies showed calcification of the C5-C6 intrvertebral disc and anterior disc protrusion. The patient was hospitalized for evaluation and pain management, with good clinical response and continue afterwards with non-steroidal anti-inflammatory drugs and a soft collar. At the 6-month-follow up, the patient had resolved symptoms and calcifications. Conclusions: Pediatric intervertebral disc calcification is a rare cause of acquired torticollis, with a benign and self-limited outcome. Conservative management, as well as clinical and imaging follow-up is recommended.

Evaluation of holmium laser for transurethral deroofing of severe and multiloculated prostatic abscesses

PURPOSE: Our objective was to evaluate the use of a holmium laser for transurethral deroofing of a prostatic abscess in patients with severe and multiloculated prostatic abscesses. MATERIALS AND METHODS: From January 2011 to April 2014, eight patients who were diagnosed with prostatic abscesses and who underwent transurethral holmium laser deroofing at Pusan National University Hospital were retrospectively reviewed. RESULTS: Multiloculated or multifocal abscess cavities were found on the preoperative computed tomography (CT) scan in all eight patients. All patients who underwent transurethral holmium laser deroofing of a prostatic abscess had successful outcomes, without the need for secondary surgery. Of the eight patients, seven underwent holmium laser enucleation of the prostate (HoLEP) for the removal of residual adenoma. Markedly reduced multiloculated abscess cavities were found in the follow-up CT in all patients. No prostatic abscess recurrence was found. Transient stress urinary incontinence was observed in three patients. The stress urinary incontinence subsided within 3 weeks in two patients and improved with conservative management within 2 months in the remaining patient. CONCLUSIONS: Transurethral holmium laser deroofing of prostatic abscesses ensures successful drainage of the entire abscess cavity. Because we resolved the predisposing conditions of prostatic abscess, such as bladder outlet obstruction and prostatic calcification, by simultaneously conducting HoLEP, there was no recurrence of the prostatic abscesses after surgery. We recommend our method in patients requiring transurethral drainage.

Peripheral Ameloblastoma with Dystrophic Calcification: An Unusual Feature in Non-Calcifying Odontogenic Tumors

Peripheral ameloblastoma is a rare extraosseous counterpart of central ameloblastoma that occurs in soft tissues and may cause bone crest resorption. This study reports a peripheral ameloblastoma on the buccal gingiva of a 56-year-old man, which presented extensive squamous metaplasia areas, keratinization and dystrophic calcifications in the neoplastic islands. It is emphasized the need of a detailed imaging study and a long follow-up period to exclude bone involvement whenever peripheral ameloblastoma diagnosis is considered.

Ameloblastoma periférico é a contraparte rara extraóssea do ameloblastoma central, que ocorre em tecidos moles e pode causar reabsorção da crista óssea. Este estudo reporta um ameloblastoma periférico localizado na gengiva vestibular de um homem de 56 anos de idade, que apresentava extensas áreas de metaplasia escamosa, queratinização e calcificação distrófica dentro das ilhas neoplásicas. É enfatizada a necessidade de um estudo de imagem detalhado e acompanhamento prolongado para excluir envolvimento ósseo sempre que o diagnóstico de ameloblastoma periférico for considerado.

Dysfunctions of the stomatognathic system and vocal aspects in Fahr disease: case report / Disfunções do sistema estomatognático e aspectos vocais na doença de Fahr: relato de caso

The aim of this study is to report the case of a patient with Fahr's Disease in order to describe the main stomatognathic and vocal changes that can be found in individuals with this disease. In order to establish the diagnosis, an assessment of the conditions of orofacial motor system and speech production, as well the efficiency of swallowing, was realized. Based on these assessments, there were difficulties in coordinating and sustaining muscle during speech and presence of oropharyngeal dysphagia. Speech disorders found in Fahr's disease manifest themselves in complex and cover various aspects of phonological knowledge and the diseases that affect the basal ganglia have similar frames of speech-language disorders of the stomatognathic system, being able to present a picture of dysarthria.

O objetivo deste estudo consiste em relatar o caso de uma paciente com Doença de Fahr buscando descrever as principais alterações estomatognáticas e vocais que podem ser encontradas em indivíduos com essa doença. A fim de estabelecer o diagnóstico fonoaudiológico, foi realizada avaliação das condições motoras orofaciais e produção da fala, além de eficiência da deglutição. Com base nessas avaliações, observaram-se dificuldades na coordenação e na sustentação muscular durante a fala e presença de disfagia orofaríngea. Os achados fonoaudiológicos na Doença de Fahr manifestam-se de forma complexa, incluindo disfagias e disartria e as doenças que acometem os núcleos da base apresentam quadros semelhantes de alterações fonoaudiológicas do sistema estomatognático, podendo apresentar quadro de disartria.

"Milk of calcium": uma apresentação rara de calcinose / "Milk of calcium": a rare presentation of calcinosis

Algumas doenças reumáticas, como dermatomiosite juvenil (DMJ), lúpus eritematoso sistêmico juvenil (LESJ) e esclerose sistêmica (ES), podem apresentar depósitos de cálcio nos tecidos subcutâneo e muscular, lesões conhecidas como calcinoses. Extensas coleções líquidas de cálcio referidas como milk of calcium são formas raras de calcinoses presentes na DMJ. Descrevemos um paciente de 15 anos de idade, com diagnóstico de síndrome de sobreposição ou overlap (esclerodermatomiosite e LESJ), cuja ressonância magnética (RM) evidenciou coleções líquidas perimusculares em membros inferiores e que, durante procedimento cirúrgico, foi observada a presença de coleção líquida esbranquiçada sugestiva de milk of calcium.

Rheumatic diseases such as juvenile dermatomyositis (JDM), juvenile sistemic lupus erythematosus (JSLE) and sistemic sclerosis may have calcium deposits in the subcutaneous and muscle tissues known as calcinosis. Extensive calcium-laden fluid collections referred as "milk of calcium" are rare forms of calcinosis in JDM. We describe a 15-year old patient with overlap syndrome (sclerodermatomyositis and JSLE), whose magnetic resonance imaging (MRI) showed perimusculares fluid collections in the lower limbs. During surgery, we observed the presence of whitish fluid collection suggestive of "milk of calcium".

Influência do escore de cálcio na gravidade de lesões coronárias moderadas avaliadas pelo ultrassom intracoronário / Influence of calcium score on the severity of moderate coronary lesions evaluated by intravascular ultrasound

INTRODUÇÃO: A área luminal mínima (ALM), obtida pelo ultrassom intracoronário (USIC), é preditora de eventos em pacientes com lesões de moderada gravidade, identificadas à angiografia coronária. Por outro lado, a presença de cálcio é preditora de estabilidade das lesões. O objetivo deste estudo é avaliar qual a influência da quantidade total de cálcio coronário, obtido pela tomografia computadorizada cardíaca (TCC), na gravidade de lesões moderadas, avaliadas por meio de USIC. MÉTODOS: Realizamos USIC de 27 lesões moderadas, em uma série consecutiva de 22 pacientes com indicação para o procedimento, e que tinham TCC prévia. Foram obtidas medidas da ALM, carga de placa e porcentual de área de estenose. RESULTADOS: Os pacientes eram, em sua maioria, do sexo masculino (85%), com idade de 60 ± 9 anos e 41% eram diabéticos. Observamos correlação significativa entre ALM e escore de cálcio total (r = 0,67; P = 0,002), mas não houve correlação entre escore de cálcio total e carga de placa (r = 0,02; P = 0,93) ou porcentual de área de estenose (r = 0,3; P = 0,32). Os preditores independentes da ALM na análise multivariada foram idade (P = 0,02), índice de massa corporal (P = 0,01), diabetes (P = 0,02), dislipidemia (P = 0,02), proteína C-reativa ultrassensível (P = 0,02), glicemia (P = 0,02), HDL-colesterol (P = 0,02), triglicerídeos (P = 0,02), fração de ejeção do ventrículo esquerdo < 50% (P = 0,02) e escore de cálcio total (P = 0,03). CONCLUSÕES: O escore de cálcio total apresenta correlação positiva com a ALM em artérias coronárias, sugerindo que maior quantidade de cálcio na árvore coronária implica lesões ateroscleróticas moderadas com maiores áreas luminais ao USIC.

BACKGROUND: The minimal luminal area (MLA) measured by intravascular ultrasound (IVUS) is a predictor of cardiovascular events in patients with angiographically moderate lesions. On the other hand, the presence of calcium is a predictor of lesion stability. The objective of this study is to evaluate the influence of the total amount of coronary calcium, obtained by multislice CT scanner (MDCT), on the severity of moderate lesions as assessed by IVUS. METHODS: IVUS was performed in 27 moderate lesions in a consecutive series of 22 patients with indication for the procedure who had a previous MDCT. Measurements of the minimal luminal area (MLA), plaque burden and percentage area stenosis were obtained. RESULTS: Most patients were male (85%), with mean age of 60 ± 9 years and 41% were diabetic. A significant correlation was observed between the MLA and total calcium score (r = 0.67; P = 0.002), but there was no correlation between the total calcium score and plaque burden (r = 0.02; P = 0.93) or percentage area stenosis (r = 0.3; P = 0.32). Independent predictors of MLA in the multivariate analysis were age (P = 0.02), body mass index (P = 0.01), diabetes (P = 0.02), dyslipidemia (P = 0.02), high-sensitive Creactive protein (P = 0.02), glucose (P = 0.02), HDL-cholesterol (P = 0.02), triglycerides (P = 0.02), left ventricular ejection fraction < 50% (P = 0.02) and total calcium score (P = 0.03). CONCLUSIONS: The total calcium score correlates positively with MLA in coronary arteries, suggesting that a large calcium burden in the coronary tree is related to angiographically moderate lesions with larger luminal areas at IVUS.

Mielopatia torácica por calcificação do ligamento amarelo cursando com hiperproteinorraquia e resposta à corticoterapia: relato de caso / Thoracic myelopathy due to calcification of the ligamentum flavum with hyperproteinorachia and responsive to steroid therapy: case report

Calcificação e ossificação do ligamento amarelo ou do ligamento longitudinal posterior são causas de mielopatia compressiva, mais frequentes nos níveis torácicos inferiores e bastante raras em populações ocidentais. A descompressão cirúrgica é a única terapia proposta, mas a doença costuma ser progressiva e sua recorrência após a cirurgia não é incomum. Mediadores inflamatórios podem ter algum papel na progressão da mielopatia compressiva, mas não se tem notícia de qualquer proposta de abordagem terapêutica envolvendo agentes anti-inflamatórios. Neste contexto, relatamos um caso de mielopatia compressiva por calcificação do ligamento amarelo em que se observou hiperproteinorraquia e resposta à corticoterapia. Tais informações são inéditas e podem fornecer novas ideias para a compreensão da doença.

Calcification and ossification of the ligamentum flavum or of the posterior longitudinal ligament are causes of compressive myelopathy, more frequent in the lower thoracic levels, and extremely rare in Western populations. Surgical decompression is the only therapy, but the disease is usually progressive, and its recurrence after surgery is common. Inflammatory mediators might play a role in the progression of compressive myelopathy, but, to our knowledge, the therapeutic approach involving anti-inflammatory agents has never been tried before. We report a case of compressive myelopathy due to calcification of the ligamentum flavum, in which hyperproteinorachia and response to steroid therapy have been observed. Those data have not been published before and might provide new ideas for the disease understanding.