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Purpose: To assess the combined estimate of retinal ganglion cell (RGC) count developed by Medeiros et al. as a tool for diagnosis of glaucoma in Japanese patients. Study design: Cross-sectional study. Methods: Thirty-one eyes of 19 healthy controls and 106 eyes of 70 glaucoma patients underwent standard automated perimetry (SAP) and three types of spectral domain optical coherence tomography (SD-OCT) imaging using the Cirrus, RTVue, and 3D-OCT instruments. RGC counts derived from SAP and SD-OCT data were estimated using the Harwerth model (SAPrgc and OCTrgc, respectively), from which the combined RGC count estimates (CRGC) were calculated using the formula developed by Medeiros et al. Receiver operating characteristic curve (ROC) analyses were conducted for mean deviation (MD), retinal nerve fiber layer thickness (RNFLT), and CRGC. Results: The mean OCTrgc derived from the Cirrus, RTVue, and 3D-OCT instruments were 1150, 1245, and 1316 (× 1000 cells), respectively, for the control group and 463, 519, and 516 (× 1000 cells), respectively, for the patient group. SAPrgc of the controls' group was 1526 and the patients' group, 731 (× 1000 cells), and were consistently greater than OCTrgc in both groups (a generalized estimating equation model, p < 0.001). Partial area under the curve (pAUC) of MD was 0.178, and that of RNFLT and CRGC for the three OCT instruments were 0.185, 0.18, 0.189 and 0.196, 0.196, 0.197, respectively. CRGC had larger pAUC than MD, whereas there was no or marginal difference in pAUC between CRGC and cpRNFLT, irrespective of OCT device used or glaucoma severity. Conclusion: CRGC proved well suited to discriminate glaucoma patients from controls. However, its clinical utility did not seem to overwhelm isolated structural measures in the tested Japanese patients. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 3-year-old boy who was referred to the hospital regarding his erythroderma with severe scales and was diagnosed with suggestive of harlequin ichthyosis (HI). The patient was suggested to be treated with etretinate and tacalcitol for his whitish scales. The article also discusses the survival and mortality rates for patients with HI.

We herein report two patients with anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis who experienced rapid deterioration of weakness, particularly respiratory muscle weakness, necessitating non-invasive positive pressure ventilation (NIPPV) and were treated with efgartigimod. After treatment initiation, a rapid reduction in IgG levels and recovery from clinical symptoms were observed. NIPPV was no longer required two to three weeks after the first infusion of efgartigimod. These findings suggest that the reduction of IgG levels using efgartigimod is a good treatment option in patients with myasthenia gravis positive for anti-MuSK antibodies, even during the acute phase of the disease.

Purpose: To evaluate Icare® HOME for home monitoring of intraocular pressure (IOP) and to observe diurnal IOP patterns for a short term. Patients and methods: Twenty six eyes of 16 patients with open angle glaucoma were included. After instruction and practice in using Icare® HOME, patients were asked to measure their IOP by themselves at home or in a sick room. Patients measured their IOP four times a day with a four hour interval between each measurement for three days. Patients repeated measurements until three successful measurements were obtained at each time. Intra-rater reliability was assessed calculating intraclass correlation coefficient (ICC) of the three measurements. Diurnal IOP patterns were assessed for three days. Results: No adverse events occurred. ICC of three measurements was 0.76 (95% confidence coefficient; 0.71 to 0.81). The median of the difference between the highest and the lowest IOP during a day was 4.26 mmHg (95% CI; 4.06 to 4.67). Repeatable patterns were not found in diurnal IOP for three days. Three out of ten patients who answered the questionnaire after measurements found difficulties in handling the device. Conclusions: Although Icare® HOME is safe and could be used for home monitoring of IOP, some patients had difficulties in using the instrument. Diurnal IOP patterns did not show repeatability during a short term.

Purpose: To evaluate the effects of trabeculotomy (TLO) combined with Schlemm's canal endothelium removal (SER) and deep sclerectomy (DS). Method: This retrospective study involved 131 adults eyes, diagnosed with glaucoma that were enrolled with at least 1 year follow-up after TLO. Fifty three eyes received TLO+SER+DS and 78 eyes underwent TLO+DS without SER. SER was performed as peeling of Schlemm's canal endothelium opening under the scleral flap. Surgical success was defined by the need for additional glaucoma surgery, or intraocular pressure (IOP) ≤20 mmHg (criterion A) and ≤16 mmHg (criterion B). Results: The occurrence rate of transient ocular hypertension (≥30 mmHg) was significantly less (p<0.001) in SER (3.8%) compared with Non-SER (21.8%). SER decreased IOP at 3 years without significant efficacy in terms of lowered IOP compared with Non-SER. At 3 years, Kaplan-Meier survival analysis revealed that the success rate of SER was higher than Non-SER for criterion A (p=0.008), but comparable for criterion B (p=0.06). Conclusions: SER was effective for reducing the rate of transient ocular hypertension in TLO and controlling IOP≤20 mmHg in adult eyes.

Endo-beta-N-acetylglucosaminidases are thought to be key enzymes in the catabolism of asparagine-linked oligosaccharides. However, little is known about the enzymes of this type in basidiomycetes. We investigated endo-beta-N-acetylglucosaminidases in basidiomycetes using fluorescence-labeled glycoasparagines as substrates. Flammulina velutipes showed high activity and its enzyme was named endo-beta-N-acetylglucosaminidase FV (Endo FV). The enzyme purified from the fruiting bodies of F. velutipes was separated into two forms. Endo FV was specific for high mannose and hybrid-type oligosaccharides. The enzyme was remarkably less active against asparagine-linked oligosaccharides attached to glycoproteins. It transferred an asparagine-linked oligosaccharide to Glc, but not to Gal. cDNA of Endo FV was cloned. It was composed of a 996-bp open reading frame encoding 331 amino acid residues. A recombinant Endo FV expressed in Escherichia coli showed enzymatic activity. The Endo FV gene in the genome of F. velutipes had no introns. The gene encoding Endo FV showed little homology with genes of known endo-beta-N-acetylglucosaminidases. A chitinase active site motif existed in the deduced primary structure, indicating that Endo FV belongs to glycoside hydrolase family 18. The deduced amino acid sequence of Endo FV had regions conserved in class III chitinases from fungi though it showed little homology with the sequence of any other endo-beta-N-acetylglucosaminidases. A folding model of Endo FV indicated it to be homologous with the tertiary structure of Endo H which is quite similar in specificity for asparagine-linked oligosaccharides. This study suggests that Endo FV may become similar to Endo H in substrate specificity as a result of evolutionary convergence.