Different etiologies of superficial siderosis / Diferentes etiologias de siderose superficial

Superficial Siderosis (SS) is an uncommon condition caused by hemosiderin deposition into the subarachnoid space. SS is characterized by cerebellar ataxia, progressive sensorineural hearing loss and pyramidal signs, but is often an unrecognized disorder. Magnetic Resonance Imaging (MRI) is the diagnostic procedure of choice due its high sensitivity to hemosiderin deposits in addition to being a non-invasive exam. This paper aims to describe a case of SS and to perform a literature review about SS etiologies, neuroimaging features and clinical characteristics. A 65-year-old man came to a neurology outpatient clinic with seizures and cerebellar ataxia with a history of car accident and severe traumatic brain injury 45 years ago. MRI SWAN showed a hyposignal in the cisterns of the base and on the cerebellar surface and T1-weighted images left hippocampal sclerosis.

A Siderose Superficial (SS) é uma condição rara causada por depósitos de hemossiderina no espaço subaracnóideo. SS é caracterizada por ataxia cerebelar, perda neurosensorial auditiva progressiva e sinais piramidais, mas é frequentemente uma desordem de difícil diagnóstico. A Ressonância Magnética (RM) é o exame de escolha para o diagnóstico devido a sua alta sensibilidade aos depósitos de hemossiderina, além de ser um exame não invasivo. Este artigo tem como objetivo descrever um caso de SS e realizar uma revisão da literatura sobre as etiologias da SS, suas características na neuroimagem e suas características clínicas. Um homem de 65 anos de idade procurou o ambulatório de neurologia com convulsões e ataxia cerebelar. Ele informou histórico de acidente automobilístico e lesão cerebral traumática grave há 45 anos. A RNM SWAN mostrou hipossinal nas cisternas da base e na superfície cerebelar e as imagens em T1 evidenciaram a presença de esclerose hipocampal esquerda.

Histopathologically proven siderotic cataract with disintegrated intralenticular foreign body.

Cataract formation may be an indicator of early siderosis and has been associated with intralenticular foreign bodies. We report a unique case of histopathologically proven lens siderosis in a young man with a preceding history of trauma but no signs of retained intraocular foreign body. He presented with a total white cataract with brownish deposits on anterior capsule and underwent cataract surgery for same followed by histopathological staining of anterior capsule for iron deposits. This case illustrates the importance of close monitoring of patients with history of trauma or previous penetrating injury to the eye, albeit no intraocular foreign body, as they might develop ocular siderosis at a later stage.

Short stature, body disproportion, and endocrine disorders in thalassemia major

short stature and endocrine disorders are common compications of beta thalassemia major [TM]. The aim of this study was to determine the prevalence and causes of short stature, body disproportion, delayed puberty, and skeletal changes among thalassemia patients in our area. Patients and a prospective study was done on 314 patients with TM, randomly chosen at the Thalassemic Treatment Center of Al Yarmook Teaching Hospital during the period from January 2, 2001 to April 30, 2001. Patients ranged from 2-20 years of age and were divided into 5 groups. The male to female ratio was 1.2:1. The study included measurements of standing height, ratio of upper body to lower segment, puberty staging according to Tanner staging. Estimation of the hormones for which assays were available including growth hormone, T4, TSH, LH, and FSH were performed. The liver enzymes AST and ALI, and serum calcium were measured. Radiographs of the head, long bones, vertebrae, and wrist joints were done. The abnormalities noted were correlated with the duration of the DFX chelation and serum ferritin levels. the study revealed that 28% of the patients exhibited short stature, predominantly in the age group 15-20 years, and 25% of the patients had short trunk but normal stature predominantly in the age group of 10-14. There was a significant relation to the duration of DFX chelation, p=0.0004. Delayed puberty was present in 33/63 patients [52.4%; 19 were females [57.5%] and 14 were males [42.5%]. Endocrine disorders were present in 25.9%. Hypogonadotropic hypogonadism and hypocalcemia were present in 8.4% and 12% of the patients respectively. There was a significant relation to the duration of the disease, p=0.00003 and 0.0004 respectively. Skeletal changes including platyspondylosis, maxillary overgrowth, hair-on-end appearance, rectangular metacarpals and metatarsals, and osteoporosis were found in 38.6% of the patients in the age group of 10-14 years and were significantly related to the duration of DFX chelation, p=0.0001. short stature, short trunk, skeletal changes and delayed puberty are significant problems in our thalassemic patients. The role of siderosis and DFX toxicity need further elucidation

Cuerpo extraño intraocular / Intraocular foreign body

Se realizó un seguimiento de 17 pacientes portadores de Cuerpo extraño intraocular metálico, entre los años 1991-1995, con un seguimiento mínimo de 6 meses y un máximo de 48. A todos los pacientes se les practicó un examen clínico, radiológico y ecográfico con el fin de ubicar el CEIO. El mecanismo de injuria más importante fue martillar metal contra metal, que ocurrió en el 71 por ciento de los casos. La totalidad de los pacientes fueron hombres, con una edad promedio de 35,6 años. La técnica quirúrgica más utilizada fue la vitrectomías con extracción del CE mediante micromagneto de tierras raras (59 porciento de los casos). Del total de pacientes, el 65 porciento obtuvo una agudeza visual final igual o mejor a 20/40, proporción que disminuye a 58 porciento si se consideran los cuerpos extraños localizados exclusivamente en el polo posterior. Las lesiones más frecuentes fueron la hemorragia vítrea (47 porciento) y la catarata (71 porciento). En cuanto a las complicaciones tardías, las más frecuentes fueron la proliferación vitreorretinal (35 porciento) y las siderosis bulbi (17,6 porciento). Los resultados globales del estudio son concordantes con los que se observan en la literatura mundial y comparables con otras series nacionales presentadas con anterioridad

Siderose pulmonar por óxido de ferro em trabalhadores: uma poeira inerte? / Pulmonary siderosis due to iron oxide in workers: an inert dust?

Säo descritos quatro casos de siderose pulmonar em trabalhadores de moagem e ensacamento de óxido de ferro. A partir da identificaçäo de comprometimento pulmonar em dois deles, foi conduzida uma investigaçäo ambiental epidemiológica e clínica cujos resultados demosntraram tratar-se de pneumoconiose por inalaçäo de poeiras de óxido de ferro puro. A análise ambiental revelou niveis de poeira inferiores aos padröes internacionais de segurança para poeiras de ferro, porém os achados clínicos e laboratoriais confirmaram a doença. A presença de fibrose intersticial moderada nos impede de classificar o agente causal como "benigno" ou "inerte" e somente a evoluçäo a longo prazo demonstrará tratar-se simplesmente de pneumoconiose de depósito