[Y-shaped osteotomy in the apical vertebra for treating congenital complex rigid scoliosis:at least 2-year follow-up].

Objective: To investigate the clinical outcome of the coronal Y-shaped osteotomy in the apical vertebra for treating congenital complex rigid scoliosis. Methods: A retrospective analysis was conducted on 66 cases who underwent Y-shaped osteotomy treatment for congenital complex rigid scoliosis in the uppermost vertebra at the Department of Orthopedics,the Second Hospital of Shanxi Medical University from June 2007 to August 2020. There were 19 males and 47 females,with an age of (13.1±5.3) years(range:2 to 30 years).Classification of congenital scoliosis:25 cases (37.9%) were incomplete,13 cases (19.7%) were dysarthritic,and 28 cases (42.4%) were mixed. There were 25 cases (37.9%) with thoracic or rib malformations. 45 cases (68.2%) were complicated with spinal cord malformation.The main radiological indicators included Cobb angle of the curvature,Cobb angle of the local bend,apical vertebral translation (AVT),trunk shift (TS),thoracic trunk shift (TTS),radiographic shoulder height (RSH),coronal balance and sagittal vertebral axis. The preoperative,postoperative immediate,and last follow-up radiological indicators were collected and the operation time,blood loss,hospitalization time,and operation-related complications were recorded. Data were compared by repeated measure ANOVA and paired-t test. Results: All patients underwent surgery successfully. The duration of the first surgery was (221.4±52.8) minutes,and the blood loss during the first surgery was (273.2±41.8) ml. The length of the first hospital stay was (8.8±1.7) days.Unilateral fixation was performed in 19 cases (28.8%),while bilateral fixation was performed in 47 cases (71.2%). The fused segments were 7.5±2.9,and the vertebral pedicle screw density was (68.5±20.6)%. The follow-up time for the 66 patients was (36.7±17.0) months(range:24 to 102 months).The main curve Cobb Angle was improved from (58.5±18.9)°before surgery to (21.1±11.8)°after surgery,and was (23.6±15.3) ° at the last follow-up(F=273.957,P<0.01),with a correction rate of 66.2%. Segmental curve Cobb Angle was improved from (47.9±18.0)° to (16.0±11.3)° after surgery,and was (16.8±12.8) °at the last follow-up (F=270.483,P<0.01)with a correction rate of 69.2%. The AVT,TS,TTS and RSH values improved significantly at the final follow-up (all P<0.05),while coronal balance and sagittal vertical axis were maintained without significant differences between pre-operation and post-operation(both P>0.05). A total of 5 patients underwent staged operation,all of which were residual scoliosis aggravated after the first stage of orthosis operation and had good prognosis after the second stage of operation. Conclusions: Y-shaped osteotomy for the treatment of congenital rigid scoliosis results in good clinical and radiological outcomes without serious complications. This procedure can be considered as an option for the treatment of congenital complex rigid scoliosis.

[Two families of X-linked lymphoproliferative disease type 1 characterized by agammaglobulinemia].

Objective: To investigate the clinical and immunological laboratory features, mutations in SH2D1A gene and SAP protein expression in four children of two families with X-linked lymphoproliferative disease type 1(XLP-1). Method: Four patients (Family A including Patient 1 and Patient 2, Family B including Patient 3 and Patient 4) and their maternal relatives were enrolled in this study. The clinical manifestation, EBV infection status and chest CT scan were analyzed. The absolute and relative numbers of lymphocyte subsets, T lymphocyte proliferative response, SAP protein expression were assessed by flow cytometry. Quantification of signal joint TCR rearrangementexcision circle (sjTRECs), CDR3 spectratyping of TCRvß and gene mutation of SH2D1A were detected by PCR based on genomic DNA or cDNA. Result: Four male patients from two families were diagnosed with XLP-1. The ages of disease onset were more than 1 year, more than 1 year, more than 1 month and 6 months. The ages at diagnosis were nine years and ten months, sixteen years and eight months, fourteen years and ten months, four years and nine months. All patients had recurrent infections and EBV infection. Patients 1, 2, and 3 had agammaglobulinemia and Patient 4 had hypogammaglobulinemia. Chest CT scan showed all patients had atelectasis and pneumonia, and Patient 3 had bronchiectasis. Patient 3 was diagnosised as Burkitt lymphoma. For immunological function, all patients exhibited reduced CD4/CD8 ratios, increased numbers of exhausted T lymphocyte, decreased number of NK cell. The numbers of total B lymphocyte and naïve B lymphocyte were normal, but the number of memory B lymphocyte declined in all cases. Four patients' copy numbers of sjTRECs were low and CDR3 spectratypings of TCRvß showed mildly skewed. But their T lymphocyte proliferative response was normal. SAP protein expression in four cases were measured by flow cytometry. Two patients from Family A were absent and two patients from Family B showed decreased values. SH2D1A gene sequence analysis showed that the patients of Family A harbored a nonsense mutation (c.163 C>T; p.R55X) in exon 2. Their mother and two sisters were carriers. A missense mutation of SH2D1A gene (c.278 G>A; p.G93D) in exon 3 was found in the patients of Family B. The mother was carrier. Four patients remain survived, Patient 3 gave up treatment, other three patients received IVIG therapy. Conclusion: Four patients with XLP-1 from two families characterized by agammaglobulinemia have an extreme vulnerability to Epstein-Barr virus (EBV) infection. The functions of T cell, B cell and NK cell are impaired at different stages. The detection of SAP protein and SH2D1A gene are the key methods for diagnosis of XLP-1.

Analysis of thrombin-antithrombin complex contents in plasma and hematoma fluid of hypertensive intracerebral hemorrhage patients after clot removal.

BACKGROUND AND PURPOSE: Animal experiments indicate that the cerebral thrombin is associated with secondary brain damage after intracerebral hemorrhage (ICH). This study was aimed to investigate the concentrations of thrombin-antithrombin complex (TAT) in hematoma fluid and plasma of the patients with ICH after surgery and analyze the correlation between TAT complex levels and severity of ICH. METHODS: Sixty patients with ICH were enrolled. Craniotomy for removal of intracranial blood clot was performed within 24h after ICH. Hematoma fluid and plasma were collected on postoperative days 1, 2, and 4. The plasma obtained from healthy subjects and cerebrospinal fluid from patients without cerebrovascular diseases served as controls, respectively. Enzyme-linked immunosorbent assay was used to determine the concentrations of TAT complex in the patients and controls. RESULTS: TAT complex concentrations in both postoperative plasma and hematoma fluid of patients with ICH were significantly higher than those of the controls (P<0.01). In patients with ICH, hematoma fluid had a higher TAT complex level than plasma (P<0.01). The preoperative hemorrhage volume and postoperative TAT complex levels in plasma and hematoma fluid correlated positively with National Institutes of Health stroke scale and negatively with Glasgow coma score (P<0.01). CONCLUSION: This study indicates that TAT complex levels of plasma and hematoma fluid correlate positively with the severity of ICH. Determination of the plasma TAT complex concentration is helpful for the evaluation of the severity of post-ICH brain injury.

Cloning and sequencing of complementary DNA for fatty acid binding protein from rainbow trout heart.

A cDNA encoding a rainbow trout homologue of mammalian heart fatty acid binding protein (H-FABP) was isolated. The deduced protein sequence is 75% identical to that of rat H-FABP. The structural conservation of H-FABPs and their evolutionary relationship are discussed.