Esophago-gastric motility and nutritional management in a child with ATR-X syndrome.

X-linked alpha thalassemia mental retardation (ATR-X) syndrome is an X-linked recessive disorder that often involves gastrointestinal symptoms. Aspiration pneumonia related to gastroesophageal reflux has been reported as the major cause of death, but gastrointestinal function has not been well investigated. The present report describes a child with ATR-X syndrome who suffered from periodical episodes of refractory vomiting. We investigated the function of upper alimentary tract and found that esophago-gastric dysmotility and severe gastric volvulus were the major causes of gastrointestinal symptoms. This child was surgically treated with anterior gastropexy and jejunal alimentation through gastrostomy, and the symptoms were relieved with good weight gain. This report may provide insight into the gastrointestinal function and nutritional management in children with ATR-X syndrome.

Endoscopic shelf acetabuloplasty combined with labral repair, cam osteochondroplasty, and capsular plication for treating developmental hip dysplasia.

In addition to the underlying shallow acetabular deformity, a patient with hip dysplasia has a greater risk of development of a labral tear, a cam lesion, and capsular laxity. This combination of abnormalities exacerbates joint instability, ultimately leading to osteoarthritis. Unsurprisingly, only repairing the acetabular labrum remains controversial, and the outcome is unpredictable. In this technical note, with video, we demonstrate an entirely endoscopic approach for simultaneously repairing the most common mechanical abnormalities found in moderate hip dysplasia: labral repair, cam osteochondroplasty, capsular plication, and shelf acetabuloplasty using an autologous iliac bone graft.

Subtrochanteric shortening osteotomy combined with cemented total hip arthroplasty for Crowe group IV hips.

BACKGROUND: Total hip arthroplasty (THA) is a challenging surgical procedure that can be used to treat severely dislocated hips. There are few reports regarding cemented THAs involving subtrochanteric shortening osteotomy (SSO), even though cemented THAs provide great advantages because the femur is generally hypoplastic with a narrow, deformed canal. PURPOSES: We evaluated the utility of cemented THA with SSO for Crowe group IV hips, and assessed the relationship between leg lengthening and nerve injury. Our goal was to describe surgical techniques for optimizing surgical outcomes while minimizing the risk of nerve injury. METHODS: We retrospectively reviewed 34 cases of cemented THAs with transverse SSO for Crowe group IV. Prior to surgery, mean hip flexion was 93.1° (40°-130°). The mean follow-up period was 5.2 years (3-10 years). RESULTS: Bone union took an average of 7.7 months (3-24 months). Mean leg lengthening was 40.5 mm (15-70 mm) and was greater in patients without hip flexion contracture. None of the patients experienced any nerve injuries associated with leg lengthening, and radiographic evidence of loosening was not observed at the final follow-up. CONCLUSIONS: SSO combined with cemented THA is an effective treatment for severely dislocated hips. Leg lengthening is not necessarily associated with nerve injuries, and the likelihood of this surgical complication may be related to the presence of hip flexion contracture.

Analysis of perioperative clinical features and complications after orthopaedic surgery in rheumatoid arthritis patients treated with tocilizumab in a real-world setting: results from the multicentre TOcilizumab in Perioperative Period (TOPP) study.

OBJECTIVE: To evaluate perioperative changes in rheumatoid arthritis (RA) patients treated with tocilizumab. METHODS: We collected RA cases with tocilizumab and orthopaedic surgery from 1999 to 2010. Incidences of postoperative infections, delayed wound healing, and RA symptom flare-ups were extracted from the data for comparison with patients without these postoperative events. We also evaluated the changes in C-reactive protein (CRP) and body temperature in patients without postoperative complications with normal CRP before surgery, i.e., patients without postoperative events in whom the tocilizumab level was maintained, for each duration to discontinuation before surgery. RESULTS: A total of 161 cases (n = 122) were collected. The patients had mean age of 56.9 years, and mean disease duration of 12.8 years at operation. Joint replacement surgery was performed in 89 cases. Three patients had postoperative infections (two superficial and one organ/space surgical-site infection), 20 had delayed wound healing, and 36 had RA symptom flare-ups. Delayed wound healing occurred most commonly in patients who underwent spinal surgery (P = 0.0061, versus patients without delayed wound healing). CRP levels were high when tocilizumab was restarted in patients with RA symptom flare-ups (P = 0.0010, versus patients without RA symptom flare-ups). Increased postoperative CRP was observed in patients without postoperative events when the duration from final tocilizumab infusion to surgery was long. The changes in body temperature showed a similar trend to CRP. CONCLUSIONS: Although it has been demonstrated that infection rates in patients treated with tocilizumab are by no means high, incidence of delayed wound healing was significantly higher in cases with surgical interventions such as foot and spinal surgeries. Many patients treated with tocilizumab remained in a normal range of CRP even during the perioperative period. For prevention of perioperative complications, observation of postoperative conditions and surgical wounds, and subjective symptoms of patients are considered important.

Clinical and radiographic results from a 2-year comparison of once-weekly versus twice-weekly administration of etanercept in biologics-naive patients with rheumatoid arthritis.

OBJECTIVES: The twice-weekly administration of 25 mg of etanercept (TW) has been shown to be effective in patients with rheumatoid arthritis (RA). However, the once-weekly administration of 25 mg of etanercept (OW) was tried in order to address the economic burden of anti-rheumatic biologics. We evaluated the clinical and radiographic results from a 2-year follow-up study of patients receiving OW or TW. METHODS: Sixty-three biologics-naive patients with RA were randomly assigned to receive either OW (n = 42) or TW (n = 21). RESULTS: From baseline to year 2, rates of clinical remission,according to the Disease Activity Score of 28 joints(DAS-28) (based on C-reactive protein; CRP)­with clinical remission being regarded as a DAS-28 (CRP) score of\2.3­were significantly improved in the OW group (from 1.6 to 39.0%) and in the TW group (from 9.5 to 47.6%),but no significant between-group difference was observed at year 2. Radiographic joint damage, quantified with the modified Sharp score, was significantly progressive in the OW group in contrast to findings in the TW group. Thus,among patients receiving TW therapy, the progression of joint damage may have been inhibited or may have shown remission. CONCLUSIONS: These results suggest that, in terms of DAS-28 remission, OW therapy can efficiently substitute for TW therapy in biologics-naive patients with RA. However, TW therapy was indispensable in preventing the worsening of joint damage.

A case of rheumatoid pericarditis associated with a high IL-6 titer in the pericardial fluid and tocilizumab treatment.

We report a 60-year-old woman with rheumatoid arthritis complicated by pericarditis. Treatment with tocilizumab improved her polyarthritis, but the pericardial effusion increased so rapidly as to cause cardiac tamponade before the treatment could prove its efficacy. Pericardial effusion disappeared after pericardiocentesis. The pericardial fluid contained a remarkably high concentration of interleukin-6 (IL-6; 351,000 pg/mL), which tocilizumab appeared to have made yet higher compared to the reported IL-6 levels in rheumatoid pericarditis. No further exacerbation of pericarditis was observed after retreatment with tocilizumab. This case has important implications in that it suggests that the prominently elevated IL-6 level in pericardial fluid during tocilizumab treatment may be an indicator of its efficacy for pericarditis.

Regenerative effects of transplanting autologous mesenchymal stem cells on corticosteroid-induced osteonecrosis in rabbits.

Osteonecrosis (ON) of the femoral head is one of the most serious complications associated with steroid administration. Here, we treated corticosteroid-induced ON in the rabbit by transplanting mesenchymal cells (MCs). Rabbits were injected once with 20 mg/kg of methylprednisolone (MPSL) and divided into three groups as follows: (1) MPSL alone (no further treatment); (2) MPSL+MCs (7 days after MPSL, MCs [1 x 10(7)/2 ml] were injected into the bone marrow cavity of the femurs); (3) MPSL+saline (7 days after MPSL, saline [2 ml] was injected into the bone marrow cavity of the femurs). Subsequently, the incidence of ON in the femurs 4 weeks after MPSL alone and MPSL+saline was 80 and 68.4%, respectively. In contrast, no ON was recorded in rabbits treated with MPSL+MCs. Vascular endothelial growth factor (VEGF) staining and terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate (dUTP) nick end-labeling (TUNEL) staining was more marked in the MPSL alone and MPSL+saline groups than in the MPSL+MCs rabbits. The percentages of cells in the G1 phase in the MPSL+MCs group were significantly lower than in the other two groups. These findings suggest that the injection of autologous MCs into the femur could prevent corticosteroid-induced ON in patients treated with high-dose short-term steroid medication.