A very rare case of congenital adrenocortical insufficiency accompanied with ventricular septal defect, is presented. Surgical treatment together with glucocorticoid replacement therapy was performed. The patient, a 4-month-old girl with congenital adrenocortical insufficiency, had been treated with a long-term adrenocortical hormones replacement. Surgical treatment for ventricular septal defect was applied in order to reduce the risk of heart failure. The administration dose of glucocorticoid was determined according to the body surface area and chronologic change of serum cortisol. Following the surgical treatment, and with adequate glucocorticoid replacement, the patient showed a good clinical outcome. In conclusion, we showed a beneficial treatment protocol with adequate glucocorticoid replacement in open heart surgery for a case of congenital adrenocortical insufficiency.

STUDY DESIGN: Retrospective cohort study. PURPOSE: To assess bone mineral density (BMD) and bone metabolism ≥27 years after surgery in female patients who underwent spinal fusion for adolescent idiopathic scoliosis (AIS) during adolescence and to determine their associations with physical performance. OVERVIEW OF LITERATURE: There are no studies investigating postsurgical BMD in middle-aged AIS patients. METHODS: This study included 23 patients who provided informed consent among 229 female patients with AIS who underwent spinal fusion from 1968 until 1988. Average age at the time of observation was 48.8 years. BMD was measured at the left femoral neck, and the levels of two bone metabolism markers–procollagen type 1 N-terminal propeptide (P1NP) and tartrate-resistant acid phosphatase 5b (TRACP-5b)–were measured from blood samples. Physical performance was measured using grip strength, sit-ups, sit-and-reach, side step, and standing long jump. RESULTS: Mean BMD was 0.784 g/cm2. According to the World Health Organization diagnostic criteria, one subject (4.3%) had osteoporosis, whereas nine subjects (39.1%) had osteopenia. In patients with osteoporosis or osteopenia, P1NP and TRACP-5b levels were high, and BMD loss was because of high metabolic turnover. All calculated standard scores for physical performance were lower in the study cohort than in healthy individuals. There was a positive correlation between BMD and the standard score for grip strength, whereas there were weak positive correlations between BMD and the standard scores for side step and standing long jump. CONCLUSIONS: In female AIS patients who underwent spinal fusion in adolescence, 4.3% and 39.1% had osteoporosis and osteopenia, respectively, ≥27 years after surgery. Exercise performance of these patients was poor compared with the national standards. In these patients, increased physical activity should be encouraged to prevent BMD loss in middle age.
Acid Phosphatase ; Adolescent* ; Bone Density* ; Bone Diseases, Metabolic ; Cohort Studies ; Female* ; Femur Neck ; Hand Strength ; Humans ; Informed Consent ; Metabolism ; Middle Aged ; Motor Activity ; Osteoporosis ; Retrospective Studies ; Scoliosis* ; Spinal Fusion ; World Health Organization

Methods: Fifty-six patients (13 males and 43 females) with an average age of 80.2±9.2 years admitted to the hospital for FFS between 2006 and 2021 were analyzed retrospectively. The following patient data were collected using medical records: pain regions, a history of trauma, initial diagnoses, and rates of fracture detection using radiography, computed tomography (CT), and magnetic resonance imaging (MRI). Results: Forty-one patients presented with low back and/or buttock pain, nine presented with groin pain, and 17 presented with thigh or leg pain. There was no history of trauma in 18 patients (32%). At the initial visit, 27 patients (48%) were diagnosed with sacral or pelvic fragility fractures. In contrast, 29 patients (52%) were initially misdiagnosed with lumbar spine disease (23 patients), hip joint diseases (three patients), and buttock bruises (three patients). Fracture detection rates for FFS were 2% using radiography, 71% using CT, and 93% using MRI. FFS was diagnosed definitively using an MRI with a coronal short tau inversion recovery (STIR) sequence. Conclusions Some patients with FFS have leg pain with no history of trauma and are initially misdiagnosed as having lumbar spine disease, hip joint disease, or simple bruises. When these clinical symptoms are reported, we recommend considering FFS as one of the differential diagnoses and performing lumbar or pelvic MRIs, particularly coronal STIR images, to rule out FFS.

OBJECTIVESThe toxicity of microglass fibers (MG), one of the man-made mineral fibers, has not been sufficiently evaluated. The aim of the current study was to evaluate the cytotoxicity of MGin vitro.

METHODSAlveolar macrophages were obtained from the bronchoalveolar lavage of male F344/N rats. The macrophages were exposed to MG at concentrations of 0, 40, 80, 160 and 320 μg/ml. The effects of MG on the macrophages were examined by cell magnetometry, LDH assay and morphological observation.

RESULTSIn the cell magnetometry experiment, a significant delay of relaxation (the reduction of remanent magnetic field strength) was observed in the cells treated with 160 and 320 μg/ml of MG in a dose-dependent manner. A significant increase in LDH release was also observed in the cells with 160 and 320 μg/ml in a dose-dependent manner. Changes in the cytoskeleton were observed after exposure to MG by immunofluorescent microscopy using an α-tubulin antibody.

CONCLUSIONSThe cytotoxicity of MG on alveolar macrophages was demonstrated with cell magnetometry. The mechanism of the toxic effects of MG was related to cytoskeleton damage.

STUDY DESIGN: A retrospective cohort study. PURPOSE: This study aims to investigate postoperative shoulder imbalance (PSI) ≥5 years postoperatively in patients who underwent posterior spinal fusion using hooks at the upper instrumented vertebra (UIV) for Lenke type 1 adolescent idiopathic scoliosis (AIS). OVERVIEW OF LITERATURE: Studies have reported PSI due to excessive correction of the main thoracic curve. METHODS: We examined 56 patients with AIS who underwent a posterior spinal fusion with hooks at the UIV from 2004 to 2010. Of these, we enrolled 14 patients who underwent surgery, at least, 5 years ago. X-rays and Scoliosis Research Society-22 (SRS-22) questionnaire were administered. To evaluate the shoulder balance, T1 vertebral tilt angle (T1 tilt), clavicle angle, and radiographic shoulder height (RSH) were measured. PSI was considered as the absolute value of the postoperative RSH being ≥20 mm. Based on radiographs obtained immediately postoperatively, we divided patients into two groups as follows: the balanced group (absolute value of RSH <20 mm) and imbalanced group (absolute value of RSH ≥20 mm). RESULTS: The frequency of PSI was 28.6% immediately postoperatively, 0% 2 years postoperatively, and 7.1% at the last follow-up. In the balanced group, PSI did not occur even at 2 years postoperatively or at the last follow-up. In the imbalanced group, PSI was improved in all patients 2 years postoperatively and all patients, except one patient, at the last follow-up. No significant differences were noted in the frequency of distal adding-on at 2 years postoperatively or the last follow-up between the balanced group and the imbalanced group. We observed moderate negative correlations between the absolute value of T1 tilt and the SRS-22 pain and satisfaction at the last follow-up. CONCLUSIONS: Hooks at the UIV could adjust the shoulder balance to avoid long-term PSI in patients with AIS.

Purpose: In this multicenter retrospective observational study, we examined the early effects of romosozumab in patients with severe osteoporosis in terms of time-course changes in bone metabolism marker, improvement in bone density, and adverse effects. Materials and Methods: Patients with severe osteoporosis were included. We investigated the progress of TRACP 5b and P1NP before and 1–2 months after the administration of romosozumab. We also investigated the bone density of lumbar spine, femoral neck, and the entire femur, measured by the DXA method, before and 5–7 months after the administration of romosozumab. Results: A total of 70 patients (7 males and 63 females, age 75.0±3.6 years) participated in this study. Significant improvements in TRACP 5b and P1NP levels were observed before and 1–2 months after romosozumab administration. The average bone density of lumbar spine, femoral neck, and the entire femur were measured before and 5–7 months after romosozumab administration;and a significant increase only observed in the lumbar spine. Conclusion Consistent with the findings of previous clinical studies, romosozumab has both bone formation-enhancing and bone resorption effects (dual effect). In addition, romosozumab also demonstrated improvement in bone density from the early phase after the administration, though the result was only seen in the lumbar spine.

Purpose: In this multicenter retrospective observational study, we examined the early effects of romosozumab in patients with severe osteoporosis in terms of time-course changes in bone metabolism marker, improvement in bone density, and adverse effects. Materials and Methods: Patients with severe osteoporosis were included. We investigated the progress of TRACP 5b and P1NP before and 1–2 months after the administration of romosozumab. We also investigated the bone density of lumbar spine, femoral neck, and the entire femur, measured by the DXA method, before and 5–7 months after the administration of romosozumab. Results: A total of 70 patients (7 males and 63 females, age 75.0±3.6 years) participated in this study. Significant improvements in TRACP 5b and P1NP levels were observed before and 1–2 months after romosozumab administration. The average bone density of lumbar spine, femoral neck, and the entire femur were measured before and 5–7 months after romosozumab administration;and a significant increase only observed in the lumbar spine. Conclusion Consistent with the findings of previous clinical studies, romosozumab has both bone formation-enhancing and bone resorption effects (dual effect). In addition, romosozumab also demonstrated improvement in bone density from the early phase after the administration, though the result was only seen in the lumbar spine.