Modern autonomic function tests can non-invasively evaluate the severity and distribution of autonomic failure. They have sufficient sensitivity to detect even subclinical dysautonomia. Standard laboratory testing evaluates cardiovagal, sudomotor and adrenergic autonomic functions. Cardiovagal function is typically evaluated by testing heart rate response to deep breathing at a defined rate and to the Valsalva maneuver. Sudomotor function can be evaluated with the quantitative sudomotor axon reflex test and the thermoregulatory sweat test. Adrenergic function is evaluated by the blood pressure and heart rate responses to the Valsalva maneuver and to head-up tilt. Tests are useful in defining the presence of autonomic failure, their natural history, and response to treatment. They can also define patterns of dysautonomia that are useful in helping the clinician diagnose certain autonomic conditions. For example, the tests are useful in the diagnosis of the autonomic neuropathies and distal small fiber neuropathy. The autonomic neuropathies (such as those due to diabetes or amyloidosis) are characterized by severe generalized autonomic failure. Distal small fiber neuropathy is characterized by an absence of autonomic failure except for distal sudomotor failure. Selective autonomic failure (which only one system is affected) can be diagnosed by autonomic testing. An example is chronic idiopathic anhidrosis, where only sudomotor function is affected. Among the synucleinopathies, autonomic function tests can distinguish Parkinson's disease (PD) from multiple system atrophy (MSA). There is a gradation of autonomic failure. PD is characterized by mild autonomic failure and a length-dependent pattern of sudomotor involvement. MSA and pure autonomic failure have severe generalized autonomic failure while DLB is intermediate.
Axons ; Blood Pressure ; Heart Rate ; Hypohidrosis ; Multiple System Atrophy ; Natural History ; Parkinson Disease ; Primary Dysautonomias ; Pure Autonomic Failure ; Reflex ; Respiration ; Sweat ; Valsalva Maneuver

PURPOSE: The objectives of this study are to describe the outcome of adolescent idiopathic scoliosis (AIS) patients treated with Video Assisted Thoracoscopic Surgery (VATS) plus supplementary minimal incision in the lumbar region for thoracic and lumbar deformity correction and fusion. MATERIALS AND METHODS: This is a case series of 13 patients treated with VATS plus lumbar mini-open surgery for AIS. A total of 13 patients requiring fusions of both the thoracic and lumbar regions were included in this study: 5 of these patients were classified as Lenke type 1A and 8 as Lenke type 5C. Fusion was performed using VATS up to T12 or L1 vertebral level. Lower levels were accessed via a small mini-incision in the lumbar area to gain access to the lumbar spine via the retroperitoneal space. All patients had a minimum follow-up of 1 year. RESULTS: The average number of fused vertebrae was 7.1 levels. A significant correction in the Cobb angle was obtained at the final follow-up (p = 0.001). The instrumented segmental angle in the sagittal plane was relatively well-maintained following surgery, albeit with a slight increase. Scoliosis Research Society-22 (SRS-22) scores were noted have significantly improved at the final follow-up (p < 0.05). CONCLUSION: Indications for the use of VATS may be extended from patients with localized thoracic scoliosis to those with thoracolumbar scoliosis. By utilizing a supplementary minimal incision in the lumbar region, a satisfactory deformity correction may be accomplished with minimal post-operative scarring.
Adolescent ; Child ; Female ; Humans ; Male ; Scoliosis/*surgery ; Thoracic Surgery, Video-Assisted/*methods ; Treatment Outcome

STUDY DESIGN: Retrospective comparative study. PURPOSE: To study and compare the surgical outcomes of muscular dystrophy (MD) and spinal muscle atrophy (SMA). OVERVIEW OF LITERATURE: There are few reports that have evaluated and compared the surgical outcomes of MD and SMA patients. METHODS: The patients (n = 35) were divided into two groups: a MD group with 24 patients and a SMA group with 11 patients. The average follow-up period was 21 months. All patients were operated for scoliosis correction using posterior instrumentation and fusion. In the immediate postoperative period, all efforts were made to reduce the pulmonary complications using non-invasive positive pressure ventilation and a coughing assist devices. The patients were evaluated by radiograph in terms of the Cobb's angle, pelvic obliquity, T1 translation, thoracic kyphosis and lumbar lordosis. The pulmonary function and self-image satisfaction were also assessed. RESULTS: There was a lower correction rate in the MD group (41.5%) than in the SMA group (48.3%), even though the curves were smaller in the MD group. The correction in the pelvic obliquity was significantly better in the SMA group (p = 0.03). The predicted vital capacity showed a 4% reduction in the MD group 1 year after surgery, while the SMA group showed a 10% reduction. The peak cough flow and end tidal PCO2 did not deteriorate and were well maintained. The average score for the improvement in self-image satisfaction postoperatively was 3.96 and 4.64 for the MD and SMA groups, respectively. The total complication rate was 45.7%; 14.3% of which were respiratory-related. CONCLUSIONS: Surgical intervention for MD and SMA may be performed safely in patients with a very low forced vital capacity (< 30%) through aggressive preoperative and postoperative rehabilitation efforts.
Animals ; Cough ; Follow-Up Studies ; Humans ; Kyphosis ; Lordosis ; Muscular Atrophy ; Muscular Atrophy, Spinal ; Muscular Dystrophies ; Positive-Pressure Respiration ; Postoperative Period ; Retrospective Studies ; Scoliosis ; Vital Capacity

PURPOSE: The purpose of this study is to report the comparative results of thoracoscopic correction achieved via cantilever technique using a 4.5 mm thin rod and the poly-axial reduction screw technique using a 5.5 mm thick rod in Lenke type 1 adolescent idiopathic scoliosis (AIS). MATERIALS AND METHODS: Radiographic data, Scoliosis Research Society (SRS) patient-based outcome questionnaires, and operative records were reviewed for forty-nine patients undergoing surgical treatment of scoliosis. The study group was divided into a 4.5 mm thin rod group (n = 24) and a 5.5 mm thick rod group (n = 25). The radiographic parameters that were analyzed included coronal curve correction, the most caudal instrumented vertebra tilt angle correction, coronal balance, and thoracic kyphosis. RESULTS: The major curve was corrected from 49.8degrees and 47.2degrees pre-operatively to 24.5degrees and 18.8degrees at the final follow-up for the thin and thick rod groups, respectively (50.8% vs. 60.2% correction). There were no significant differences between the two groups in terms of kyphosis, coronal balance, or tilt angle at the time of the final follow-up. The mean number of levels fused was 6.2 in the thin rod group, compared with 5.9 levels in the thick rod group. There were no major intraoperative complications in either group. CONCLUSION: Significant correction loss was observed in the thin rod system at the final follow-up though both groups had comparable correction immediately post-operative. Therefore, the thick rod with poly axial screw system helps to maintain post-operative correction.
Adolescent ; Adult ; *Bone Screws ; Female ; Humans ; Male ; Retrospective Studies ; Scoliosis/radiography/*surgery ; Thoracic Surgery, Video-Assisted/*methods ; Treatment Outcome

The common data model (CDM) has found widespread application in healthcare studies, but its utilization in cancer research has been limited. This article describes the development and implementation strategy for Cancer Clinical Library Databases (CCLDs), which are standardized cancer-specific databases established under the Korea-Clinical Data Utilization Network for Research Excellence (K-CURE) project by the Korean Ministry of Health and Welfare. Fifteen leading hospitals and fourteen academic associations in Korea are engaged in constructing CCLDs for 10 primary cancer types. For each cancer type-specific CCLD, cancer data experts determine key clinical data items essential for cancer research, standardize these items across cancer types, and create a standardized schema. Comprehensive clinical records covering diagnosis, treatment, and outcomes, with annual updates, are collected for each cancer patient in the target population, and quality control is based on six-sigma standards. To protect patient privacy, CCLDs follow stringent data security guidelines by pseudonymizing personal identification information and operating within a closed analysis environment. Researchers can apply for access to CCLD data through the K-CURE portal, which is subject to Institutional Review Board and Data Review Board approval. The CCLD is considered a pioneering standardized cancer-specific database, significantly representing Korea’s cancer data. It is expected to overcome limitations of previous CDMs and provide a valuable resource for multicenter cancer research in Korea.

The common data model (CDM) has found widespread application in healthcare studies, but its utilization in cancer research has been limited. This article describes the development and implementation strategy for Cancer Clinical Library Databases (CCLDs), which are standardized cancer-specific databases established under the Korea-Clinical Data Utilization Network for Research Excellence (K-CURE) project by the Korean Ministry of Health and Welfare. Fifteen leading hospitals and fourteen academic associations in Korea are engaged in constructing CCLDs for 10 primary cancer types. For each cancer type-specific CCLD, cancer data experts determine key clinical data items essential for cancer research, standardize these items across cancer types, and create a standardized schema. Comprehensive clinical records covering diagnosis, treatment, and outcomes, with annual updates, are collected for each cancer patient in the target population, and quality control is based on six-sigma standards. To protect patient privacy, CCLDs follow stringent data security guidelines by pseudonymizing personal identification information and operating within a closed analysis environment. Researchers can apply for access to CCLD data through the K-CURE portal, which is subject to Institutional Review Board and Data Review Board approval. The CCLD is considered a pioneering standardized cancer-specific database, significantly representing Korea’s cancer data. It is expected to overcome limitations of previous CDMs and provide a valuable resource for multicenter cancer research in Korea.

The common data model (CDM) has found widespread application in healthcare studies, but its utilization in cancer research has been limited. This article describes the development and implementation strategy for Cancer Clinical Library Databases (CCLDs), which are standardized cancer-specific databases established under the Korea-Clinical Data Utilization Network for Research Excellence (K-CURE) project by the Korean Ministry of Health and Welfare. Fifteen leading hospitals and fourteen academic associations in Korea are engaged in constructing CCLDs for 10 primary cancer types. For each cancer type-specific CCLD, cancer data experts determine key clinical data items essential for cancer research, standardize these items across cancer types, and create a standardized schema. Comprehensive clinical records covering diagnosis, treatment, and outcomes, with annual updates, are collected for each cancer patient in the target population, and quality control is based on six-sigma standards. To protect patient privacy, CCLDs follow stringent data security guidelines by pseudonymizing personal identification information and operating within a closed analysis environment. Researchers can apply for access to CCLD data through the K-CURE portal, which is subject to Institutional Review Board and Data Review Board approval. The CCLD is considered a pioneering standardized cancer-specific database, significantly representing Korea’s cancer data. It is expected to overcome limitations of previous CDMs and provide a valuable resource for multicenter cancer research in Korea.