BACKGROUND:Calcium sulfate has good biocompatibility and biodegradability, which is a safe and effective bone graft substitute.
OBJECTIVE:To investigate the osteogenesis ability of calcium sulfate combined with bone marrow mesenchymal stem cells.
METHODS:After L4/5 posterior lumbar discectomy, 36 rabbits were randomized into three groups:rabbits in autologous bone group were implanted with autologous iliac bone via the intervertebral space;animals in al ogenic bone group were implanted with decalcified bovine bone;rabbits in tissue-engineered bone group were implanted with calcium sulfate combined with bone marrow mesenchymal stem cells. Bone formation and molding were observed by gross observation, anteroposterior and lateral X-ray, histology and biomechanics at 4, 8 and 16 weeks. Cal us specimens were employed for histological observation of interbody fusion. Biomechanical analysis of spinal fusion site was conducted at 16 weeks.
RESULTS AND CONCLUSION:Sixteen weeks later, interbody fusion was complete in the autologous bone group, the trabecular bone bridged continuously and a large amount of woven bone was merged into pieces;in the al ogenic bone group, incomplete bony fusion was found between the intervertebral space, most of cartilage tissues differentiated into bone, but fibrous tissue was also ful of the central part;in the tissue-engineered bone group, interbody fusion was complete, and a large amount of woven bone was fused into pieces, while the artificial bone was absorbed and ossified with smal residual. Failure strength and stiffness in the autologous bone and tissue-engineered bone groups were superior to those in the al ogenic bone group. These findings indicate that the calcium sulfate/bone marrow mesenchymal stem cells tissue-engineered bone has excellent osteogenic and osteoinductive capacity that can exert a good function of promoting spinal interbody fusion.

Tumor calcinosis(TC) has different clinical and biochemical patterns. The existence of chronic injury as well as calcium and phosphorus metabolism disorder has been gradually proved to be the important link in the occurrence of TC. In this paper, the related basic diseases with the pathophysiological mechanism of calcium and phosphorus metabolism disorder were reviewed, and the pathophysiological mechanism of phosphate metabolism disorder in various diseases was summarized.In addition, the phosphate homeostasis genes including GALNT-3, FGF-23 and α-KLOTHO were described. The relevant research results have showed that mutations in any of these three genes will lead to defects in the synthesis or action of FGF-23, which will increase the reabsorption of phosphate by renal tubules, resulting in hyperphosphatemia and severe ectopic calcification of soft tissue.At present, surgical resection is still the main treatment of TC. New technologies such as cinalcet peritoneal dialysis, ultrasound-guided aspiration of TC lesions and local injection of sodium thiosulfate (STS), as well as the successful application of lanthanum carbonate and other drugs, provide alternatives to TC treatment.In this paper, the research literatures on TC at home and abroad in recent years were introduced and the genetic susceptibility genes, related pathogenic factors and the latest treatment progress of TC were reviewed.

Objective:To summarize the clinical, pathological and molecular biological characteristics of one patient of paranodal disease with anti-contactin-associated protein 1 (Caspr 1) antibodies.Methods:The patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) admitted to Qilu Hospital of Shandong University from August 2018 to December 2020 were retrospectively studied. The clinical data of one acute onset CIDP patient with anti-Caspr 1 antibodies were collected and retrospectively analyzed with literature review. Anti-nodal/paranodal IgG and their subclasses in serum and cerebrospinal fluid (CSF) were investigated by immuno?uorescence assays. Pathological characteristics were explored by sural nerve biopsy further.Results:The patient presented with tremor, ataxia and neuropathological pain besides symmetrical limb muscle weakness and hypaesthesia. The CSF protein was elevated significantly. The brachial plexus and lumbosacral plexus magnetic resonance imaging showed enlarged nerve roots. The patient was responsive well to intravenous immunoglobulin and steroids in acute phase, while the symptoms improved significantly with rituximab in chronic phase. Autoantibodies against Caspr 1 were detectable in serum and CSF, with IgG4 predominant. Sural nerve biopsy revealed segmental demyelination and myelin digestion chamber. Dispersed lamellae of myelin sheath and axonal degeneration were confirmed by electron microscopy.Conclusions:Tremor, ataxia, neuropathic pain, significantly elevated CSF protein and enlarged nerve roots are suggestive of paranodal diseases with anti-Caspr 1 antibodies. For patients with suspected Guillain-Barre syndrome/CIDP and above phenotypes, nodal/paranodal antibodies and antibody subtypes should be detected to optimize the treatment.

Objective:To report a family of Zellweger spectrum disorders (ZSD) caused by mutations in the PEX10 gene and to increase the level of awareness of the disease among clinicians. Methods:The clinical and genetic data of a brother-sister co-morbidity family with ZSD due to PEX10 gene mutation admitted to the Department of Neurology, Qilu Hospital of Shandong University in July 2022 were collected and a literature review was performed. Results:The proband was a 24-year-old female who was admitted to the Qilu Hospital of Shandong University due to unstable walking for 8 years and aggravated for 1 year with squatting effort. Cranial magnetic resonance imaging (MRI) showed cerebellar atrophy, electromyography suggested peripheral neuropathy with motor and sensory involvement, and pure tone hearing threshold measurement showed neurogenic deafness. The 15-year-old brother of the proband presented with "unsteadiness in walking and difficulty in squatting for 2 years". His cranial MRI and electromyography were similar to those of the proband, and his laboratory results suggested abnormal liver function. The whole exon sequencing results of the proband suggested a compound heterozygous mutation in the PEX10 gene with c. 992G>A(p. R331Q) and c. 988T>C(p. C330R) mutations and both were likely pathogenic mutations and respectively from her parents. And her brother also carried the above variants. A total of 9 case reports in English literature (1 of which was published by domestic scholars) were retrieved from major domestic and international databases on PEX10 gene mutations causing ZSD, with a total of 15 patients, most of whom had childhood and adolescent onset. The most common initial symptom was slowly progressed ataxia. The majority of patients showed cerebellar atrophy on cranial MRI and peripheral neuropathy was found in the most patients.The 2 patients were suggested to have phytic acid-free food, at the same time, they bagan taking L-arginine and ursodeoxycholic acid. Fortunately,both the ataxia symptom and liver function of them were dramatically alleviated 3 months later. Conclusions:For the patients with unexplained ataxia with polyneuropathy and abnormal liver function,the possibility of ZSD should be considered. Phytic acid-free food, L-arginine and ursodeoxycholic acid supplement may be beneficial for the ZSD patients.