Thoracoplasty-Current View on Indication and Technique.

Thoracoplasty was invented for removing cavities between thoracic wall and remnant lung or mediastinum. It was initially used in cases of tuberculosis or unspecific infections, while currently it is used mainly for space problems after lobectomy/pneumonectomy.This article presents an overview of the historical and current techniques of this surgical procedure.Nowadays, thoracoplasty is rarely performed due to the low incidence of diseases for which this method is necessary. Therefore, this method has even been discredited. Furthermore, certain technical aspects of the thoracoplasty are not very well known because of the infrequent application of this procedure.Unfortunately, a look into the literature of thoracoplasty is not always usefull due to the biased views of advocates of different techniques such as Schede's thoracoplasty, Heller's Jalousie-Plastik, Alexander's extramusculoperiosteal thoracoplasty, Bjork's osteoplastic thoracoplasty, etc.Not to forget, there has always been a lack of research on the relevance and on the several techniques of thoracoplasty.The point is precise indication and correct execution of thoracoplasty as a final therapeutic option, which allows a safe and definitive solution of the space problem even in complex cases, without creating serious functional and cosmetic impairment for the patient.The main types of thoracoplasty are described in this article. Although the core principle of this operation remains unchanged, modern techniques are often cosmetically more considerable and less destructive, compared with techniques that were used in the past.

Effects of asfotase alfa in adults with pediatric-onset hypophosphatasia over 24 months of treatment.

BACKGROUND: Hypophosphatasia (HPP) is a rare, heritable metabolic disorder caused by deficient activity of tissue-nonspecific alkaline phosphatase (TNSALP). Asfotase alfa (AA) is a human recombinant TNSALP that promotes bone mineralization and is approved to treat eligible patients with HPP. METHODS: This prospective single-center observational study evaluated AA in adults with pediatric-onset HPP over 2 years of treatment (ClinicalTrials.govNCT03418389). Primary outcomes evaluated physical function; secondary outcomes assessed quality of life (QoL) and pain. RESULTS: The study included 17 females and 5 males (mean age: 48.7 years). Median distance walked in the 6-Minute Walk Test increased significantly from baseline to 12 months (P = 0.034) and results were sustained. Median Timed Up and Go test time significantly decreased from baseline at 12 (P = 0.003) and 24 months (P = 0.005), as did the median chair rise time test at 12 (P = 0.003) and 24 months (P < 0.002). The change from baseline in usual gait speed was significant at 12 (P = 0.003) and 24 months (P = 0.015). Mean dominant and nondominant hand grip strength improved at 24 months (P = 0.029 and P = 0.019, respectively). Median Short Form 36 Physical Component Summary scores significantly improved from baseline at 12 (P = 0.012) and 24 (P = 0.005) months, and median Lower Extremity Functional Scale scores improved from baseline at 12 (P = 0.001) and 24 (P = 0.002) months. No significant change was noted in pain level at these timepoints. While injection site reactions occurred in 86.4 % of the participants, there were no severe side effects or safety findings. CONCLUSIONS: Adults with pediatric-onset HPP treated with AA experienced marked improvement in functional and QoL outcomes that were observed as early as within 3 months of initial treatment and were sustained over 24 months.