Relationship of serum vitamin D with liver disease severity and bone abnormalities in cholestatic children

@#<p style="text-align: justify;"><strong>Background:</strong> Vitamin D deficiency occurs in 10% to 36% of children with cholestasis. The relationship between serum vitamin D levels, severity of liver disease and bone abnormalities in children has not been extensively investigated.</p><p style="text-align: justify;"><strong>Objective:</strong> To determine serum vitamin D levels and its association with liver disease severity and presence of radiographic rickets in children with cholestasis.</p><p style="text-align: justify;"><strong>Methods:</strong> Children aged 0-10 years with cholestasis underwent serum 25-hydroxyvitamin D levels (25-[OH]D) determination, radiographs of wrists and knees and liver function tests. Liver disease severity was evaluated using the Child-Pugh score. Radiographs were assessed using Thacher Rickets Severity Score. Data were analyzed using odds ratio and Spearman's correlation coefficient.</p><p style="text-align: justify;"><strong>Results:</strong> We included 51 children [Mean (SD) age: 5 (6) months, 63% are males], mostly with biliary atresia (51%). Forty-seven (92%) had serum 25-(OH)D deficiency and four (8%) had insufficiency. Radiologic bone abnormalities were observed in 22 (43%) cases; specifically, rickets in 16 (31%). No association was observed with vitamin D levels and liver disease severity (OR 1.27, 95% CI 0.12-13.31) nor with rickets score (OR 0.07, 95% CI 0.004-1.37).</p><p style="text-align: justify;"><strong>Conclusion:</strong> Majority of the children with cholestasis had vitamin D deficiency, with a third having radiographic findings of rickets. Serum vitamin D levels were not associated with liver disease severity or with rickets score.</p><p style="text-align: justify;"><strong>Key Words:</strong> Vitamin D, rickets, cholestasis, bone disease, bone abnormalities</p>

Asymptomatic bilateral elongated and mineralized stylohyoid complex: Report of one case / Proceso estilohioideo elongado y calcificado asintomático: Presentación de un caso

The stylohyoid process is a cylindrical bony structure surrounded by important anatomical structures including vessels and nerves. Calcification and elongation of the stylohyoid ligament complex over 30 mm may be associated with neck and facial pain, known as Eagle's syndrome. However, a bilateral ossified and elongated stylohyoid complex may be devoid of symptoms. We report a 79-year-old symptom-free female who presented on a routine conventional dental radiographic exam an 80.96 mm psendoarticulated stylohyoid complex in the left side and an 75.85 mm on the other. On CAT sean, both processes were calcified.

El proceso estilohioideo es una estructura ósea rodeada por nervios y vasos sanguíneos. La calcificación elongación del ligamento estilohioideo en más de 30 mm puede asociarse a dolor facial y del cuello, conocido como síndrome de Eagle. Sin embargo, esta alteración anatómica puede ser asintomática. Presentamos una mujer asintomática de 79 años a quien se le descubrieron procesos estilohioideo pseudo articulado de 80,96 mm a un lado y 75,85 mm al otro lado, en una radiografía dental convencional. En la tomografía axial computada, ambos procesos estaban calcificados.

Neurofibromatose tipo 1: aspectos radiológicos do tórax / Type 1 neurofibromatosis: radiological findings of the chest

OBJETIVO: Identificar alterações e frequências nas radiografias simples do tórax sugestivas de neurofibromatose tipo 1 e avaliar a possibilidade de inclusão de massa no mediastino posterior como critério de diagnóstico de neurofibromatose tipo 1. MATERIAIS E MÉTODOS: Foram realizadas radiografias com técnica padrão de tórax em póstero-anterior e em perfil de 141 pacientes com neurofibromatose tipo 1, atendidos no Serviço de Radiologia do Hospital de Base e Faculdade de Medicina de São José do Rio Preto, SP. Os resultados obtidos foram avaliados por métodos não paramétricos ao nível de 0,05 de significância (p = 0,05). RESULTADOS: No presente estudo, 141 pacientes com neurofibromatose tipo 1 realizaram radiografia de tórax, sendo as alterações mais frequentes: erosão óssea das costelas (19,8 por cento), peito escavado (12,0 por cento), cifoescoliose (3,5 por cento) e massas no mediastino posterior (7,1 por cento). Esses resultados sugerem que as massas (neurofibroma e meningocele) devem ser incluídas como critério diagnóstico para neurofibromatose tipo 1, juntamente com displasia do osso esfenoide, pseudoartrose e afinamento do córtex de ossos longos, conforme definido pelo National Institutes of Health. CONCLUSÃO: A presença das massas no mediastino posterior associada às alterações ósseas características definidas pelo National Institutes of Health indicam ser um achado consistente para se considerar como critério diagnóstico da doença.

OBJECTIVE: To identify chest radiography findings suggestive of type 1 neurofibromatosis, establishing their frequency and evaluating the possibility of including the presence of posterior mediastinal masses as a criterion for the diagnosis of type 1 neurofibromatosis. MATERIALS AND METHODS: The present study included 141 patients with type 1 neurofibromatosis assisted at the Service of Radiology of Hospital de Base and Faculdade de Medicina de São José do Rio Preto, SP, Brazil, and submitted to standard chest radiography in postero-anterior and lateral views. The results were analyzed by non-parametric methods and the level of statistical significance was set at 0.05 (p = 0.05). RESULTS: The most frequent findings were the following: ribs erosion (19.8 percent), pectus excavatum (12.0 percent), kyphoscoliosis (3.5 percent) and posterior mediastinal masses (7.1 percent). Such results suggest that posterior mediastinal masses (neurofibroma and meningocele) should be included as a diagnostic criterion of type 1 neurofibromatosis, in conjunction with dysplasia of the sphenoid wing, pseudoarthrosis and thinning of long bone cortex, as defined by the National Institutes of Health. CONCLUSION: The presence of posterior mediastinal masses in association with the typical bone changes defined by the National Institutes of Health is a consistent finding to be considered as a diagnostic criterion of the disease.

The Significance of a Hypoplastic Bony Canal for the Cochlear Nerve in Patients with Sensorineural Hearing Loss: CT and MRI Findings

PURPOSE: The purpose of this study is to evaluate the significance of the hypoplastic canal for the cochlear nerve in patients with sensorineural hearing loss (SNHL) and the relationship between the hypoplastic bony canal and aplasia or hypoplasia of the cochlear nerve. MATERIALS AND METHODS: A retrospective review of high resolution temporal CT(HRCT) and MRI findings was conducted. The narrow bony canal of the cochlear nerve and the relative size of the internal auditory canal were correlated with the cochlear nerve deficiency on MRI. The comparative size of the component nerves (facial, cochlear, superior vestibular, inferior vestibular nerve), and the relative size of the internal auditory canal and the bony canal of the cochlear nerve were measured. The clinical history and the results of the clinical examination were reviewed for each patient. RESULTS: High resolution MRI showed aplasia of the common vestibulocochlear nerve in one patient and a deficiency of the cochlear nerve in 9 patients. These abnormalities occurred in association with a prominent narrowing of the canal for the cochlear nerve and a stenosis of the internal auditory canal, which was observed on temporal bone CT in 9 patients with congenital SNHL. Three patients had normal IAC, despite the presence of a hypoplastic cochlear nerve on the side on which they had SNHL. In one patient, the narrowing of the canal for the cochlear nerve and internal auditory canal were not found to be associated with acquired SNHL. CONCLUSION: The hypoplastic bony canal for the cochlear nerve might be more highly indicative of congenital cochlear nerve deficiency than that of the narrow internal auditory canal, and the position of the crista falciformis should also be carefully.

Congenital External Auditory Canal Atresia and Stenosis: Temporal Bone CT Findings

PURPOSE: To determine the computed tomographic (CT) findings of atresia and stenosis of the external auditory canal (EAC), and to describe associated abnormalities in surrounding structures. MATERIALS AND METHODS: We retrospectively reviewed the axial and coronal CT images of the temporal bone in 15 patients (M:F=8:7; mean age, 15.8 years) with 16 cases of EAC atresia (unilateral n=11, bilateral n=1) and EAC stenosis (unilateral n=3). Associated abnormalities of the EAC, tympanic cavity, ossicles, mastoid air cells, eustachian tube, facial nerve course, mandibular condyle and condylar fossa, sigmoid sinus and jugular bulb, and the base of the middle cranial fossa were evaluated. RESULTS: Thirteen cases of bony EAC atresia (one bilateral), with an atretic bony plate, were noted, and one case of unilateral membranous atresia, in which a soft tissue the EAC. A unilateral lesion occurred more frequently on the right temporal bone (n=8, 73%). Associated abnormalities included a small tympanic cavity (n=8, 62%), decreased mastoid pneumatization (n=8, 62%), displacement of the mandibular condyle and the posterior wall of the condylar fossa (n=7, 54%), dilatation of the Eustachian tube (n=7, 54%), and inferior displacement of the temporal fossa base (n=8, 62%). Abnormalities of ossicles were noted in the malleolus (n=12, 92%), incus (n=10, 77%) and stapes (n=6, 46%). The course of the facial nerve was abnormal in four cases, and abnormality of the auditory canal was noted in one. Among three cases of EAC stenosis, ossicular aplasia was observed in one, and in another the location of the mandibular condyle and condylar fossa was abnormal. In the remaining case there was no associated abnormality. CONCLUSION: Atresia of the EAC is frequently accompanied by abnormalities of the middle ear cavity, ossicles, and adjacent structures other than the inner ear. For patients with atresia and stenosis of this canal, CT of the temporal bone is essentially helpful in evaluating these associated abnormalities.

The Usefulness of MR Imaging of the Temporal Bone in the Evaluation of Patients with Facial and Audiovestibular Dysfunction

OBJECTIVE: To evaluate the clinical utility of MR imaging of the temporal bone in patients with facial and audiovestibular dysfunction with particular emphasis on the importance of contrast enhancement. MATERIALS AND METHODS: We retrospectively reviewed the MR images of 179 patients [72 men, 107 women; average age, 44 (range, 1-77) years] who presented with peripheral facial palsy (n=15), audiometrically proven sensorineural hearing loss (n=104), vertigo (n=109), or tinnitus (n=92). Positive MR imaging findings possibly responsible for the patients' clinical manifestations were categorized according to the anatomic sites and presumed etiologies of the lesions. We also assessed the utility of contrast-enhanced MR imaging by analyzing its contribution to the demonstration of lesions which would otherwise not have been apparent. All MR images were interpreted by two neuroradiologists, who reached their conclusions by consensus. RESULTS: MR images demonstrated positive findings, thought to account for the presenting symptoms, in 78 (44%) of 179 patients, including 15 (100%) of 15 with peripheral facial palsy, 43 (41%) of 104 with sensorineural hearing loss, 40 (37%) of 109 with vertigo, and 39 (42%) of 92 with tinnitus. Thirty (38%) of those 78 patients had lesions that could be confidently recognized only at contrastenhanced MR imaging. CONCLUSION: Even though its use led to positive findings in less than half of these patients, MR imaging of the temporal bone is a useful diagnostic procedure in the evaluation of those with facial and audiovestibular dysfunction. Because it was only at contrast-enhanced MR imaging that a significant number of patients showed positive imaging findings which explained their clinical manifestations, the use of contrast material is highly recommended.