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Biomedical subjects

L Dias

Publications and source records attributed to L Dias.

40 records · Page 3Linked to original sources

External tibial torsion and the effectiveness of the solid ankle-foot orthoses.

The aims of this study were to determine the influence of external tibial torsion on the effectiveness of the ankle-foot orthoses (AFO) in children with lumbosacral myelomeningocele. Forty patients with normal tibial rotation and 18 patients with excessive external tibial torsion were evaluated with three-dimensional gait analysis at their comfortable walking speed. The group with normal tibial rotation showed significantly greater knee extension and lower mean extension moment compared with the group with external tibial torsion (p < 0.05). The posteriorly and laterally deviated ground-reaction force relative to the knee-flexion axis compromises the ability of this force to facilitate knee extension. Patients with torsional magnitudes >20 degrees demand close inspection as candidates for derotation osteotomy. The AFO will continue to stabilize the ankle-foot complex, but improved knee motion, knee-extensor activity, and ultimately walking efficiency may be compromised.

Adolescent↗

[The importance of TTR met 30 in the diagnosis of familial amyloidotic polyneuropathy in the absence of a positive family history].

INTRODUCTION: Familial amyloidotic polyneuropathy type I is an hereditary autosomal dominant disease, characterized by having a mutant transthyretin (TTR met 30). For this reason there is a deposition of amyloid in several organs with their subsequent malfunction. The most characteristic clinical presentation is peripheral, sensomotor and autonomic neuropathy. The dysautonomic component is seen early and often dominates the clinical picture. Subsequently, there is cardiac, ocular or renal involvement and death generally occurs 10 to 15 years after onset of the disorder. In Portugal one thought the gene was fully penetrant and, thus, a positive family history was an important criterion for the diagnosis. However an increasing number of patients without a positive family history have been identified. CLINICAL CASES: We present the cases of three men and a woman, aged between 36 and 48 years, with clinical sensomotor and autonomic polyneuropathy. There was no known family history of the condition in any case. Nerve biopsy in three of the patients, and biopsy of abdominal fat in the other patient, showed amyloid to be present. Similarly, TTR met 30 analysis was positive in all cases, and allowed the diagnosis to be established. CONCLUSION: It is essential to do TTR met 30 analysis in all patients with peripheral polyneuropathy in whom there is obviously a dysautonomic component and no known family history.

Abdomen↗

Sacral-level myelomeningocele: long-term outcome in adults.

We reviewed long-term outcome of 46 adult patients with sacral-level myelomeningocele with an average age of 23 years (range, 18-38). Data were collected from the medical records, computerized database, and a questionnaire. Thirty patients had high sacral lesions (weak gluteus medius, gluteus maximums, and gastroc/soleus), and 16 had low sacral lesions (fair to normal-strength gluteus medius, gluteus maximus, and gastroc/soleus). Thirteen patients had signs of tethered-cord syndrome, and 12 underwent neurosurgical release. Thirty-nine patients underwent a total of 217 orthopaedic surgeries. At the final follow-up, 41 (89.13%) patients were community ambulators. Thirty-two required no external support for walking. Twenty-nine patients used some kind of orthosis. Aggressive management of tethered-cord syndrome, surgical correction of musculoskeletal deformities, and avoidance of arthrodesis at the foot level may be the main factors in accounting for these results.

Activities of Daily Living↗

Valgus knee stress in lumbosacral myelomeningocele: a gait-analysis evaluation.

Twenty-five independent community-ambulating patients with lumbosacral-level myelomeningocele (N = 50 limbs) underwent gait analysis. The limbs of these patients were divided into two groups based on thigh-foot angle (TFA): Group I (n = 20) had marked external tibial torsion, TFA > or = 20 degrees, and group II had TFA between 10 and 20 degrees. Ten limbs were excluded because of neutral or internal alignment. Twenty normal limbs with TFA = 10 degrees served as controls. An abnormal internal varus knee stress during stance was identified in all group I limbs and 12 (70%) of 20 limbs group II limbs compared with controls, which demonstrated an internal valgus stress. This internal varus moment was greater in group I limbs than in the abnormal limbs in group II (p < 0.05). Knee flexion was the only other parameter found to correlate with this stress and only in group I limbs. We conclude that (a) in this patient group, increased external tibial torsion is likely to result in an abnormal internal varus knee stress; (b) TFA > 20 degrees appears significantly to increase this stress; and (c) knee flexion is an important related parameter, but only in limbs with TFA between 10 and 20 degrees. We believe that this abnormal stress may predispose the knee to late arthrosis and that derotational osteotomies to normalize the TFA may prove to have a favorable long-term effect.

Adolescent↗