Dimerized, Not Monomeric, Translationally Controlled Tumor Protein Induces Basophil Activation and Mast Cell Degranulation in Chronic Urticaria

Translationally controlled tumor protein (TCTP) is also known as histamine releasing factor as it has the ability to activate mast cells. To investigate the role of TCTP in the pathogenesis of chronic spontaneous urticaria (CSU), we evaluated serum level of TCTP and effect of TCTP on basophil and mast cell degranulation. TCTP levels in the sera from 116 CSU patients and 70 normal healthy controls (NCs) were measured by ELISA. CD203c expression on basophils from CSU patients and β-hexosaminidase release from Laboratory of Allergic Disease 2 mast cells were measured upon stimulation monomeric and dimeric TCTP. Non-reducing Western blot analysis was used for detecting dimeric TCTP. No difference was observed in serum TCTP levels between CSU patients and NCs (p=0.676). However, dimeric TCTP intensity on Western blot was stronger in CSU patients than in NCs. TCTP levels were higher in patients with severe CSU (p=0.049) and with IgG positivity to FcɛRIα (p=0.038). A significant positive correlation was observed between TCTP and eosinophil cationic protein levels (Spearman's rho=0.341; p=0.001). Both basophil and mast cell degranulation were significantly increased after stimulation with dimeric TCTP, but not with monomic TCTP. The ability of TCTP to activate basophil and mast cells is dependent on dimerization, suggesting that the inhibition of TCTP dimerization can be a therapeutic option for CSU. Association between TCTP levels and the presence of IgG to high affinity Fc epsilon receptor I alpha subunit in CSU patients indicates that autoimmune mechanisms may be involved in the dimerization of TCTP.

The Role of Regular Physical Therapy on Spasticity in Children With Cerebral Palsy

OBJECTIVE: To investigate the effect of physical therapy (PT) intervention on spasticity in patients with cerebral palsy (CP), and to assess the degree of deterioration of spasticity when regular PT is interrupted in those patients. METHODS: We recruited 35 children with spastic CP who visited our hospital for PT, and whose Modified Tardieu Scale (MTS) scores were serially recorded including before and after a 10-day public holiday time frame period. The outcome measures were the angle of range of motion (ROM) of dorsiflexion of the ankle joint (R1 and R2) in the knee flexion and extension positions as assessed using the MTS. RESULTS: The range of dorsiflexion of the ankle joint (R1 and R2) after the holiday period was significantly decreased as compared with that measured ROM noted before the holiday period, regardless of the knee position, age, or gross motor function. The dynamic component of the MTS (R2–R1) showed a slight decrease in the knee flexion position. CONCLUSION: Interruption of regular PT aggravated spasticity and decreased ankle joint ROM in children with spastic CP. Our findings suggest that regular PT in the care continuum for children with CP is crucial for the maintenance of ROM in the spastic ankle joints.

Cervical Spine Malformations Associated With a 5q34-5q35.2 Micro-interstitial Deletion: A Case Report

We report a female proband carrying a de novo 5q34-q35.2 deletion breakpoint, and review the unique skeletal phenotype and possible genotype related to this mutation. The patient presented with a persistent head tilt and limited head rotation. Non-contrast-enhanced three-dimensional computed tomography of the cervical spine revealed several malformations including a bone cleft in the right pars interarticularis, a bone defect in both C5 lamina and the transverse foramen at C2–C3, agenesis of the right articular process of C5, bony fusion of C4–C5, and subluxation of the craniocervical joints. Several deformities of the cervical spine seen in this patient have not been associated with the 5q deletion. A review of 5q-related mutations suggests that abnormalities associated with MSX2 gene might cause cervical spine abnormalities.