Bony island within the articular cartilage of the knee in a child: a rare condition for early osteoarthritis

Articular cartilage is a specific type of connective tissue composed of hydrated proteoglycans within a matrix of collagen fibrils. In the elderly population, it shows degenerative changes that may results in osteoarthritis. The more severe form of osteoarthritis occasionally demonstrates bone formation within the cartilage, which is designated as a bony protuberance, however, such lesions are rare in children. This report presents the case of a 10-year-old boy with a bony protuberance within the articular cartilage of the knee. The patient initially complained of knee pain and he subsequently developed flexion contracture. Radiological and arthroscopic examinations revealed a bony protuberance in the articular cartilage and degenerative changes of the cartilage above it. He was successfully treated by the removal of the bony protuberance and osteochondral grafting. The bony protuberance may have caused cartilage degradation since the thickness of the cartilage above it was thinner than that around the lesion. The bony protuberance within the articular cartilage formed in the younger population may be a possible cause of osteoarthritis. This case is a noteworthy with regard to the pathogenesis of osteoarthritis

Hereditary Spherocytosis Coexisting with UDP-Glucuronosyltransferase Deficiency Highly Suggestive of Crigler-Najjar Syndrome Type II

Patients with co-existing hereditary spherocytosis (HS) and UDP-glucuronosyltransferase 1A1 (UGT1A1) deficiency as Gilbert's syndrome (GS) have been reported, and previous studies have demonstrated an increased risk for developing gallstones in patients with co-inheritance of GS and HS. We experienced an interesting case of HS showing persistent jaundice after splenectomy, and upon further evaluation, the 25-year-old female patient was found to have HS combined with UGT1A1 deficiency. Sequence analysis of the UGT1A1 gene revealed that she was a compound heterozygote with p.[G71R; Y486D] + [Y486D] mutations, which suggests Crigler-Najjar syndrome type II rather than GS. Careful evaluation of inappropriately elevated bilirubin level compared with the degree of hemolysis is important, reflecting the therapeutic implication of splenectomy and cholecystectomy

Outcomes of patients who developed subsequent solid cancer after hematopoietic cell transplantation

To characterize the outcomes of patients who developed a particular subsequent solid cancer after hematopoietic cell transplantation (HCT), age at cancer diagnosis, survival, and causes of death were compared with the respective primary cancer in the general population, using data from the national HCT registry and population-based cancer registries in Japan. Among 31 867 patients who underwent a first HCT between 1990 and 2013 and had progression-free survival at 1 year, 713 patients developed subsequent solid cancer. The median age at subsequent solid cancer diagnosis was 55 years, which was significantly younger than the 67 years for primary cancer patients in the general population (P < .001). The overall survival probability was 60% at 3 years after diagnosis of subsequent solid cancer and differed according to cancer type. Development of most solid cancers was associated with an increased risk of subsequent mortality after HCT. Subsequent solid cancers accounted for 76% of causes of death. Overall survival probabilities adjusted for age, sex, and year of diagnosis were lower in the HCT population than in the general population for colon, bone/soft tissue, and central nervous system cancers and did not differ statistically for other cancers. In conclusion, most subsequent solid cancers occurred at younger ages than primary cancers, emphasizing the need for cancer screening at younger ages. Subsequent solid cancers showed similar or worse survival compared with primary cancers. Biological and genetic differences between primary and subsequent solid cancers remain to be determined

Endoscopic balloon dilations for strictures of rectum, ileocecal valve and duodenum in a patient with X-linked inhibitor of apoptosis deficiency : a case report

X-linked inhibitor of apoptosis (XIAP) deficiency is a rare primary immunodeficiency and gastrointestinal (GI) lesions in XIAP deficiency are similar to Crohn's disease. For patients with Crohn's disease, endoscopic balloon dilation (EBD) is known to be a standard procedure for intestinal strictures including upper GI tract. However, there are no articles which mention the efficacy of EBDs for the strictures in upper GI tract in patients with XIAP deficiency. Herein, we describe an 18-year-old male with XIAP deficiency in whom EBDs for the rectum, ileocecal valve (ICV), and duodenum were performed. Before hematopoietic stem cell transplantation (HSCT), GI endoscopy revealed strictures of the rectum, ICV and duodenum with active ulcers. Although these ulcers healed after HSCT, the strictures progressed. Therefore, we performed EBDs for the strictures of the rectum, ICV, and duodenum. In contrast studies, we did not find any other strictures in the small intestine. Throughout the patient's clinical course, no complications of EBD occurred. He started eating after EBDs, but abdominal symptoms did not relapse without any dietary restrictions. Our case suggests that EBD could be an effective and safe procedure for intestinal strictures including upper GI tract after HSCT in patients with XIAP deficiency

Visualization of torn anterior cruciate ligament using 3-dimensional computed tomography

Recently, a remnant-preserving anterior cruciate ligament (ACL) reconstruction technique has been developed. However, the pre-operative condition of remnant ACL is occasionally difficult to evaluate by magnetic resonance imaging. The purpose of this study is to evaluate the accuracy of pre-operative visualization of remnant ACL using three-dimensional computed tomography (3D-CT). The remnant ACL in 25 patients was examined by 3D-CT before ACL reconstruction surgery. Findings on 3D-CT images and arthroscopy were compared. The 3D-CT images were classified into 4 groups: Group A, remnant fibers attached to the posterior cruciate ligament (PCL); Group B, those located between the PCL and the lateral wall; Group C, those attached to the lateral wall; and Group D, no identifiable remnant fibers on the tibial side. These groups were made up of 4, 3, 9 and 9 patients, respectively. Findings on 3D-CT images were identical to those during arthroscopy in 20 of 25 cases (80%). Remnant ACL can be accurately evaluated using 3D-CT in 80% of cases of torn ACL. This novel method is a useful technique for pre-operative assessment of remnant ACL