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The inner retina was mostly within normal limits. There was central depigmentation on near infrared fundus autofluorescence. Biallelic mutations were identified in NMNAT1 One was previously reported (c.769 G>A; pGlu257Lys), and the other one (c.245T>C; pVal82Ala) was novel. CONCLUSION NMNAT1 mutations cause a consistent phenotype characterized by early-onset, progressive, cone>rod retinawide dysfunction and predominantly central abnormalities ranging from a hypoplastic to an atrophic fovea, supporting a critical role for NMNAT1 in central retinal development and maintenance. Relatively preserved inner retina and detectable photoreceptors may become therapeutic targets.PURPOSE We report a patient with CTNNB1-associated vitreoretinopathy. We discuss imaging findings and surgical management. METHODS Case report. RESULTS An 18-month-old girl with microcephaly, failure to thrive, developmental delay, and chronic rhinitis presented with bilateral central and peripheral tractional retinal detachments and an anomalous retinal vasculature. She underwent multimodal imaging and genetic testing, and we discuss successful surgical management. CONCLUSION CTNNB1 mutations can cause a vision-threatening vitreoretinopathy. We recommend CTNNB1 to be considered as part of the workup of patients presenting with familial exudative vitreoretinopathy-like clinical findings, especially if there are systemic manifestations.PURPOSE Optical coherence tomography (OCT) cross-sections have shown limited ellipsoid zone (EZ) improvement in mild hydroxychloroquine (HCQ) retinopathy within a few years after drug cessation. However, the extent, functional significance, and stability of such changes over time remain unclear. METHODS We created en face EZ maps using automated pixel-by-pixel segmentation for four patients with early-moderate HCQ toxicity followed for 6-8 years after drug cessation. These maps were compared with OCT cross-sections, fundus autofluorescence, and automated 10-2 visual fields. RESULTS One patient had no EZ line loss; one had stable EZ loss throughout follow-up; two showed 30 to 40% reduction in the area of loss, largely in the first 2 years. This limited recovery mostly occurred in regions where the EZ line was only thinned or fragmented; other similar areas did not improve. Fundus autofluorescence hyperfluorescence and visual fields did not show consistent correlation with topography. CONCLUSION Anatomic EZ recovery, when present, was restricted to regions of mild damage and did not correlate with fundus autofluorescence or improvement in visual fields. Topographic mapping seemed no more sensitive locally than cross-sectional OCT but may aid detection and longitudinal follow-up of toxicity by showing early damage or changes in the macula that could be missed with individual cross-sections.INTRODUCTION Radiotherapy is a valid treatment option for nasopharyngeal carcinoma. However, complications can occur following irradiation of the closest anatomical structures, including brainstem radionecrosis (BRN). The rehabilitation is poorly described in patients with BRN, despite its usefulness in improving functional independence in patients with brain tumors. We aimed at testing the usefulness of intensive, robot-assisted neurorehabilitation program to improve functional independence in a 57-year-old male with BRN. PATIENT CONCERNS A 57-year-old male diagnosed with a nasopharyngeal carcinoma, received a radiation total dose of 72 Gy. Owing to the appearance of a severe symptomatology characterized by dysphagia, hearing loss, and left sided hemiparesis, the patient was hospitalized to be provided with intensive pharmacological and neurorehabilitation treatment. DIAGNOSIS Follow-up brain magnetic resonance imaging disclosed no residual cancer, but some brainstem lesions compatible with BRN areas were appreciable. INTERVENTION The patient underwent a 2-month conventional, respiratory, and speech therapy. Given that the patient only mildly improved, he was provided with intensive robot-aided upper limb and gait training and virtual reality-based cognitive rehabilitation for other 2 months. OUTCOMES The patient reported a significant improvement in functional independence, spasticity, cognitive impairment degree, and balance. CONCLUSION Our case suggests the usefulness of neurorobotic intensive rehabilitation in BRN to reduce functional disability. Future studies should investigate whether an earlier, even multidisciplinary rehabilitative treatment could lead to better functional outcome in patients with BRN.BACKGROUND The general consensus regarding a rational choice among various treatment strategies for medial meniscus posterior root tears (MMPRTs) has yet to be clearly established. selleckchem The purpose of this systematic review was to analyze patient selection criteria based on index arthrosis, as well as clinical and radiological outcomes after repair or nonrepair treatment in patients with MMPRTs. METHODS A systematic electronic search was performed with established medical databases. Data from the selected studies which were assessed using the modified Coleman methodology score were analyzed in terms of index arthrosis and degree of lower limb alignment, functional and radiologic outcomes after meniscus repair, partial meniscectomy, and conservative treatment. RESULTS In total, 17 studies and 655 patients (665 cases) were enrolled in this study, of which 42% (279 cases) underwent MMPRT repair and 58% (386 cases) were treated using a nonrepair strategy. The mean age and the mean follow-up period were 54.7 years and atic relief in some selected cases, despite the somewhat heterogenous results. Given the subgroup analysis for the functional results reported in this review, strict patient selection is important to obtain satisfactory clinical outcomes, regardless of the treatment option selected.The present study aimed to investigate the long-term and perioperative outcomes of precise hepatic pedicle dissection in anatomical resection (precise AR) vs non-anatomical resection (NAR) for hepatocellular carcinoma (HCC) patients.Data from a total of 270 consecutive HCC patients who underwent curative hepatectomy were retrospectively collected. Propensity score matching (PSM) analysis was performed. The long-term outcomes of precise AR and NAR were analyzed using the Kaplan-Meier method and the Cox proportional hazards model.The 1-, 3-, and 5-year overall survival (OS) rates were 90.3%, 76.2%, and 65.7% in the PS-precise AR group, respectively (n = 103); and 88.3%, 70.5%, and 52.0% in the PS-NAR group, respectively (n = 103) (P = .043). The 1-, 3-, and 5-year recurrence-free survival (RFS) rates were 83.4%, 63.2%, and 46.0% in the PS-precise AR group, respectively; and 75.7%, 47.4%, and 28.3% in the PS-NAR group, respectively (P = .002). Multivariate analysis showed that ICG-R15, BCLC staging, and microvascular invasion (MVI) were independent risk factors for OS; while tumor size, types of resection, surgical margin, and MVI were independent risk factors for RFS. Subgroup analysis indicated that the RFS rate was significantly better in the PS-precise AR group than in the PS-NAR group for patients with MVI and tumor size ≤5 cm.After PSM, precise hepatic pedicle dissection in AR significantly improved the recurrence-free survival rate of solitary HCC patients compared with NAR, especially in those with MVI and tumor size ≤5 cm.The modified Blumgart method for pancreaticojejunostomy has been shown to reduce the rate of postoperative pancreatic fistula (POPF) in open surgery. We describe a modified Blumgart method using LAPRA-TY suture clips to facilitate laparoscopic pancreaticojejunostomy.We prepared a double-armed 4-0 nonabsorbable monofilament, which was ligated using the LAPRA-TY clip at the tail end, 12-cm in length. Next, the U-suture was placed through the pancreatic stump and the seromuscular layer of the jejunum. We performed duct-to-mucosa suturing with a 5-0 absorbable monofilament. After completing the duct-to-mucosa suturing, as a final step we placed the sutures through the seromuscular layer of the jejunum on the ventral side and tightly secured the thread with the LAPRA-TY clips. We performed laparoscopic Blumgart pancreaticojejunostomy during pancreaticoduodenectomy in 39 patients. We compared the surgical outcomes of 19 patients who underwent Blumgart pancreaticojejunostomy using the LAPRA-TY clips (LAPRA-TY group) with 20 patients undergoing surgery not using the LAPRA-TY clips (conventional group).The rate of clinically relevant postoperative pancreatic fistula in the LAPRA-TY group was 21.1%, which did not differ significantly from the rate of the conventional group. However, the mean time of pancreaticojejunostomy in the LAPRA-TY group was 56.2 min (range, 39-79 min), which was significantly shorter than that of the conventional group (69.7 min; range, 53-105 min, P  less then  .001).Although the modified Blumgart pancreaticojejunostomy using LAPRA-TY suture clips did not improve the pancreatic fistula rate, it allowed for shorter operative times. Thus, this procedure lends itself to positive surgical and patient outcomes.BACKGROUND Renal insufficiency is an important predictor of contrast-induced acute kidney injury (CI-AKI). We performed a meta-analysis to examine the effects of short-term statin therapy on the incidence of CI-AKI, particularly in patients with renal insufficiency. METHODS A systematic search was conducted to retrieve randomized controlled trials (RCTs) that investigated the impact of statin pretreatment before administration of contrast media on the development of CI-AKI in patients with mild to moderate renal insufficiency. The primary outcome was development of CI-AKI. The secondary outcome was the incidence ofacute kidney injury requiring hemodialysis. RESULTS Data analysis from 8 RCTs, which included a total of 2313 subjects in the statin-treated group and 2322 in the control group, showed that statin pretreatment was associated with significant reduction of the risk of CI-AKI (relative risk [RR] = 0.59; 95% confidential interval [CI] 0.44-0.79; P = .0003, I = 0%). A beneficial effect of statin on preventing CI-AKI was consistent, regardless of the dose of statin and use of N-acetylcysteine. In subgroup analysis based on baseline estimated glomerular filtration rate (eGFR), patients with baseline eGFR less then 60 mL/min/1.73 m (RR = 0.63; 95% CI 0.41-0.98; P = .04, I = 0%) and 30  less then  eGFR  less then  90 mL/min/1.73 m (RR = 0.56; 95% CI 0.39-0.82; P = .003, I = 0%) showed significant reduction of risk of CI-AKI. CONCLUSION Statin pretreatment is effective at preventing CI-AKI and should be considered in patients with preexisting renal insufficiency.BACKGROUND Some studies assessed the effect of aerobic exercise on diabetic obese patients with hepatic disease, while very limited studies compared high-intensity interval (HII) versus moderate-intensity continuous (MIC) on diabetic obese patients with non-alcoholic fatty liver disease (NAFLD). OBJECTIVES This study was designed to assess the effects of HII versus MIC on intrahepatic triglycerides (IHTG) and visceral lipids in diabetic obese patients with NAFLD. DESIGN Randomized controlled trial. METHODS Forty-seven diabetic obese individuals with NAFLD were enrolled in this study. The individuals were randomly divided into 16 in HII group, 15 in MIC group, and 16 in the controls. HII group received HII exercise, MIC group received 8-week MIC exercise while the control group did not receive any exercise intervention. IHTG and visceral lipids were assessed pre- and post-intervention. RESULTS Baseline and clinical characteristics showed nonsignificant difference among the 3 groups (P > .05). Both HII and MIC groups showed a significant reduction in hepatic fat and visceral lipids (P  .
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