Monitoring the Outcome of Leprosy Using Leprosy Neuropathy Scale: A Preliminary Study

The disability and progress of leprosy patients is monitored by the WHO disability grading system which has limited sensitivity in leprous neuropathy. This study aims to report the spectrum of leprosy patients at a tertiary care neurology service and compare WHO grading, modified Rankin Scale (mRS) and Leprosy Neuropathy Scale (LNS) in monitoring the treatment outcome. The patients with leprosy diagnosed as per WHO criteria were subjected to medical history and clinical examination. Their disability was graded as per WHO grading scale, modified Rankin scale (mRS) and LNS. These parameters were repeated and compared after six months of multiple drug therapy (MDT). Thirty-eight patients with leprosy, aged 40 (`5-80) years, 33 of whom were males have been evaluated. The duration of symptoms was 24 (91-120) months. Mononeuropathy was present in 14, mononeuropathy multiplex in 24, trophic ulcer in two, claw hand in 11, wrist drop in two, foot drop in four, facial palsy in one, Charcot’s joint in one and lepra reaction in seven patients. Their disability as per WHO grade 1 and 2 was in 19 patients each. After 6 months of MDT, WHO grade improved in two patients, mRS revealed improvement in seven and LNS in nine patients. LNS- a clinical scale, seems more effective and easier to use for monitoring the progress/ outcome of neuropathy in leprosy patients and may complement the WHO grading scale

Irradiated dose to unprotected lymph node stations in the VMAT and IMRT treatment of patients with upper thoracic esophageal cancer / 中华放射医学与防护杂志

Objective To compare the irradiated dose to unprotected lymph node stations (LNS) between volume-modulated arc therapy (VMAT) and 5-field intensity-modulated radiotherapy (5F-IMRT) in the treatment of patients with upper thoracic esophageal cancer.Methods A total of 20 patients were selected for re-planning.LNS were not included in the GTV and CTV,instead,LNS were contoured as normal tissues.However,LNS were not constrained in the VMAT and 5F-IMRT inverse optimization for protection.Dosimetric parameters of conformal index (CI),homogeneity index (HI) of targets,V95,V110 of planning target volume (PTV),D V5,V20,V30 of lung,D V25 of heart,Dmax of spinal cord,MU,as well as the equivalent uniform dose (EUD) and V40 of LNS were compared between the two plans.Results 5F-IMRT was superior in PTV_ V95％ (t=-9.4,P＜0.05),but worse in terms of CI (t=-5.3,P＜0.05) compared with VMAT.5F-IMRT reduced the V5 of lung by 10.9％ (t=-7.8,P＜0.05) and the Dmax of spinal cord by 9％ (t=-10.2,P＜0.05),but increased the MU (t=-6.2,P＜0.05) compared with VMAT.The average EUD and V40 of LNS in upper thoracic were significantly increased by 4.7％ and 2.4％ in 5F-IMRT compared with VMAT,respectively.The irradiated doses to LNS were significantly associated with the volume of PTV (R =0.716-0.933,P＜0.05) expect for 106tbL.Conclusions The irradiated doses to unprotected LNS were less for IMRT plans and were highly associated with PTV volume in patients with upper thoracic esophageal cancer.

Irradiated dose to unprotected lymph node stations in the VMAT and IMRT treatment of patients with upper thoracic esophageal cancer / 中华放射医学与防护杂志

Objective@#To compare the irradiated dose to unprotected lymph node stations (LNS) between volume-modulated arc therapy (VMAT) and 5-field intensity-modulated radiotherapy (5F-IMRT) in the treatment of patients with upper thoracic esophageal cancer.@*Methods@#A total of 20 patients were selected for re-planning. LNS were not included in the GTV and CTV, instead, LNS were contoured as normal tissues. However, LNS were not constrained in the VMAT and 5F-IMRT inverse optimization for protection. Dosimetric parameters of conformal index (CI), homogeneity index (HI) of targets, V95, V110 of planning target volume (PTV), Dmean, V5, V20, V30 of lung, Dmean, V25 of heart, Dmax of spinal cord, MU, as well as the equivalent uniform dose (EUD) and V40 of LNS were compared between the two plans.@*Results@#5F-IMRT was superior in PTV_V95% (t=-9.4, P<0.05), but worse in terms of CI (t=-5.3, P<0.05) compared with VMAT. 5F-IMRT reduced the V5 of lung by 10.9% (t=-7.8, P<0.05) and the Dmax of spinal cord by 9% (t=-10.2, P<0.05), but increased the MU (t=-6.2, P<0.05) compared with VMAT. The average EUD and V40 of LNS in upper thoracic were significantly increased by 4.7% and 2.4% in 5F-IMRT compared with VMAT, respectively. The irradiated doses to LNS were significantly associated with the volume of PTV (R=0.716-0.933, P<0.05) expect for 106tbL.@*Conclusions@#The irradiated doses to unprotected LNS were less for IMRT plans and were highly associated with PTV volume in patients with upper thoracic esophageal cancer.

Lesch-Nyhan syndrome in a Chinese family with mutation in the HGPRT gene / 中国生化药物杂志

Lesch-Nyhan syndrome(LNS) is a congenital X-linked recessive inherited disorder caused by mutations in the hypoxanthine guanine phosphoribosyl transferase (HPRT) gene.A deficiency of the HPRT enzyme is responsible for the disease.The main clinical manifestation includes hyperuricemia, juvenile-onset gouty arthritis and neurological developmental disorders.Studies have reported there are more than 400 HPRT gene mutation sites, but the incidence of LNS in the Chinese population is extremely low.Here we report a 16-year-old male patient who suffered neurological dysfunction at an early age and gouty arthritis in his youth.DNA of patient and his family members were extracted from peripheral blood lymphocytes.The coding region and the intron-exon boundaries of HPRT gene were sequenced by standard methods.We found a mutation in exon 3 of the HPRT gene of the patient and his mother (Exon3:c.143G>A), which resulted in an arginine to histidine (p.R48H) substitution in the encoded protein.No activity of the enzyme HPRT was detected in the erythrocytes.The same mutation was reported in several European families, but was found in Chinese family for the first time.Clinicians in China have poor experience in diagnosing LNS case, due to the low incidence in China.Therefore LNS screening for infants or adolescents with hyperuricemia, gouty arthritis and neurological dysfunction should be performed.

Intervention Planning Using a Laser Navigation System for CT-Guided Interventions: A Phantom and Patient Study

OBJECTIVE: To investigate the accuracy, efficiency and radiation dose of a novel laser navigation system (LNS) compared to those of free-handed punctures on computed tomography (CT). MATERIALS AND METHODS: Sixty punctures were performed using a phantom body to compare accuracy, timely effort, and radiation dose of the conventional free-handed procedure to those of the LNS-guided method. An additional 20 LNS-guided interventions were performed on another phantom to confirm accuracy. Ten patients subsequently underwent LNS-guided punctures. RESULTS: The phantom 1-LNS group showed a target point accuracy of 4.0 +/- 2.7 mm (freehand, 6.3 +/- 3.6 mm; p = 0.008), entrance point accuracy of 0.8 +/- 0.6 mm (freehand, 6.1 +/- 4.7 mm), needle angulation accuracy of 1.3 +/- 0.9degrees (freehand, 3.4 +/- 3.1degrees; p < 0.001), intervention time of 7.03 +/- 5.18 minutes (freehand, 8.38 +/- 4.09 minutes; p = 0.006), and 4.2 +/- 3.6 CT images (freehand, 7.9 +/- 5.1; p < 0.001). These results show significant improvement in 60 punctures compared to freehand. The phantom 2-LNS group showed a target point accuracy of 3.6 +/- 2.5 mm, entrance point accuracy of 1.4 +/- 2.0 mm, needle angulation accuracy of 1.0 +/- 1.2degrees, intervention time of 1.44 +/- 0.22 minutes, and 3.4 +/- 1.7 CT images. The LNS group achieved target point accuracy of 5.0 +/- 1.2 mm, entrance point accuracy of 2.0 +/- 1.5 mm, needle angulation accuracy of 1.5 +/- 0.3degrees, intervention time of 12.08 +/- 3.07 minutes, and used 5.7 +/- 1.6 CT-images for the first experience with patients. CONCLUSION: Laser navigation system improved accuracy, duration of intervention, and radiation dose of CT-guided interventions.

Reduction of lns-1 gene expression and tissue insulin levels in n5-STZ rats

Objective: The high global incidence of type 2 diabetes has challenged researchers to establish animal models that resemble the chronic stage observed in type 2 diabetes patients. One such model is induced by neonatal streptozotocin (n-STZ) administration to rat pups at 0, 2, or 5 days after birth. In this study, we assessed lns-1 gene expression and tissue insulin levels as well as serum concentration of glucose and insulin, insulin resistance, and histological changes of the islets of Langerhans in n5-STZ rats after 20-weeks post-induction. Methods: Wistar rat pups were randomly distributed into a control group and a streptozotocin-induced group. Experimental induction involved a single intraperitoneal injection of streptozotocin (150 mg/kg) into neonates at five days after birth. Results: At 20 weeks post-induction, streptozotocin-induced rats exhibited increased serum glucose levels, reduced serum insulin levels, impaired glucose metabolism and insulin resistance compared to control rats. Histologically, streptozotocin-induced rats exhibited atrophic islets, vacuolization, and significantly fewer insulin-positive cells. lns-1 gene expression was significantly decreased in n5-STZ rats in comparison to the control group. Conclusion: Our findings support that the n5-STZ model 20 weeks post-induction represents an appropriate experimental tool to study T2D and to evaluate novel therapeutic agents and targets that involve insulin gene expression and secretion, as well as complications caused by chronic diabetes.