Visual field defects after macular hole surgery with indocyanine green-assisted internal limiting membrane peeling

PURPOSE: To report visual field defects after macular hole surgery with indocyanine green (ICG)-assisted internal limiting membrane (ILM) peeling. DESIGN: Interventional consecutive case series. METHODS: A retrospective review of 140 eyes with macular hole after vitrectomy with ILM peeling. The group with 0.25% of ICG for ILM peeling consisted of 96 eyes. The group without ICG consisted of 44 eyes. RESULTS: Nasal visual field defects occurred in 11 eyes, temporal visual field defects in seven eyes, and concentric visual field defects in one eye. In the group with ICG, nasal visual field defects were far higher in the left eye than in the right eye. CONCLUSIONS: We speculate that the postoperative nasal visual field defects were caused by the enhanced toxicity of ICG resulting from the exposure to illumination.     

Utility of a simplified ultrasonography scoring system among patients with rheumatoid arthritis: A multicenter cohort study

We aimed to evaluate the utility of a simplified ultrasonography (US) scoring system, which is desired in daily clinical practice, among patients with rheumatoid arthritis (RA) receiving biological/targeted synthetic disease-modifying antirheumatic drugs (DMARDs).A total of 289 Japanese patients with RA who were started on tumor necrosis factor inhibitors, abatacept, tocilizumab, or Janus kinase inhibitors between June 2013 and April 2019 at one of the 15 participating rheumatology centers were reviewed. We performed US assessment of articular synovia over 22 joints among bilateral wrist and finger joints, and the 22-joint (22j)-GS and 22-joint (22j)-PD scores were evaluated as an indicator of US activity using the sum of the GS and PD scores, respectively.The top 6 most affected joints included the bilateral wrist and second/third metacarpophalangeal joints. Therefore, 6-joint (6j)-GS and -PD scores were defined as the sum of the GS and PD scores from the 6 synovial sites over the aforementioned 6 joints, respectively. Although the 22j- or 6j-US scores were significantly correlated with DAS28-ESR or -CRP scores, the correlations were weak. Conversely, 6j-US scores were significantly and strongly correlated with 22j-US scores not only at baseline but also after therapy initiation.Using a multicenter cohort data, our results indicated that a simplified US scoring system could be adequately tolerated during any disease course among patients with RA receiving biological/targeted synthetic DMARDs.     

Haemophagocytic lymphohistiocytosis, interferon-gamma-naemia and Epstein-Barr virus involvement

Summary. To clarify the correlation between Epstein-Barr virus (EBV) involvement and hypercytokinaemia in haemophagocytic lymphohistiocytosis (HLH), we analysed serum interferon-gamma levels and EBV-DNA in biological specimens obtained from 25 HLH cases (23 children and two adults). We found that HLH patients showed a wide range of serum IFN-gamma levels from 0.2 to 1300 U/ml, with a median 126U/ml for EBV-DNA-positive (n = 9) and 4.5 U/ml for EBV-DNA-negative (n = 16) groups. The latter group could be classified further into a group with hyper-IFN-gamma-naemia (> 4.5 U/ml) (n = 8) and a group without hyper-IFN-gamma-naemia (n = 8). The survival of the hyper-IFN-gamma-naemic cases was significantly poorer than non-hyper-IFN-gamma-naemic cases. We conclude that EBV is probably involved in one third of the HLH cases, all of whom show hyper-IFN-gamma-naemia, and in the half of the HLH cases with hyper-IFN-gamma-naemia who have a rapidly fatal outcome.     

Mutation Analysis of the IL36RN Gene in 14 Japanese Patients with Generalized Pustular Psoriasis

Generalized pustular psoriasis (GPP) is a rare, potentially life threatening, and aggressive form of psoriasis, which is characterized by sudden onset with repeated episodic skin inflammation leading to pustule formation. Familial GPP is known to be caused by recessively inherited mutations in the IL36RN gene, which encodes interleukin 36 receptor antagonist (IL‐36Ra). In this article, we performed mutation analysis of the IL36RN gene in 14 Japanese patients with GPP, and identified mutations in two of these patients analyzed. One patient was compound heterozygous for mutations c.115+6T>C and c.368C>G (p.Thr123Arg), whereas the other carried compound heterozygous mutations c.28C>T (p.Arg10*) and c.115+6T>C in the IL36RN gene. Expression studies using total RNA from the patients’ skin revealed that the mutation c.115+6T>C resulted in skipping of exon 3, leading to a frameshift and a premature termination codon (p.Arg10Argfs*1). The protein structure analysis suggested that the missense mutation p.Thr123Arg caused misfolding and instability of IL‐36Ra protein. In vitro studies in cultured cells showed impaired expression of the p.Thr123Arg mutant IL‐36Ra protein, which failed to antagonize the IL‐36 signaling pathway. Our data further underscore the critical role of IL36RN in pathogenesis of GPP.     

O2 activation by core–shell Ru13@Pt42 particles in comparison with Pt55 particles: a DFT study

The reaction of O₂ with a Ru₁₃@Pt₄₂ core–shell particle consisting of a Ru₁₃ core and a Pt₄₂ shell was theoretically investigated in comparison with Pt₅₅. The O₂ binding energy with Pt₅₅ is larger than that with Ru₁₃@Pt₄₂, and O–O bond cleavage occurs more easily with a smaller activation barrier (E_a) on Pt₅₅ than on Ru₁₃@Pt₄₂. Protonation to the Pt₄₂ surface followed by one-electron reduction leads to the formation of an H atom on the surface with considerable exothermicity. The H atom reacts with the adsorbed O₂ molecule to afford an OOH species with a larger E_a value on Pt₅₅ than on Ru₁₃@Pt₄₂. An OOH species is also formed by protonation of the adsorbed O₂ molecule, followed by one-electron reduction, with a large exothermicity in both Pt₅₅ and Ru₁₃@Pt₄₂. O–OH bond cleavage occurs with a smaller E_a on Pt₅₅ than on Ru₁₃@Pt₄₂. The lower reactivity of Ru₁₃@Pt₄₂ than that of Pt₅₅ on the O–O and O–OH bond cleavages arises from the presence of lower energy in the d-valence band-top and d-band center in Ru₁₃@Pt₄₂ than in Pt₅₅. The smaller E_a for OOH formation on Ru₁₃@Pt₄₂ than on Pt₅₅ arises from weaker Ru₁₃@Pt₄₂–O₂ and Ru₁₃@Pt₄₂–H bonds than the Pt₅₅–O₂ and Pt₅₅–H bonds, respectively. The low-energy d-valence band-top is responsible for the weak Ru₁₃@Pt₄₂–O and Ru₁₃@Pt₄₂–OH bonds. Thus, the low-energy d-valence band-top and d-band center are important properties of the Ru₁₃@Pt₄₂ particle.

In this theoretical study by DFT computations, characteristic features of the Ru₁₃@Pt₄₂ core–shell particle in O₂ activation are clearly discussed in comparison with Pt₅₅.     

Successful laparoscopic extirpation of a large omental lipoblastoma in a child

Omental lipoblastoma is extremely rare among benign tumors. We herein report the case of a child who underwent laparoscopic extirpation of a large omental lipoblastoma. A 4‐year‐old girl was diagnosed with an intra‐abdominal solid tumor. Abdominal imaging revealed a fat density mass that was well encapsulated and measured 18 × 15 × 7.5 cm in size. Considering the MRI findings and movability of the tumor, we strongly suspected that the lesion was an omental lipoblastoma. We initially decided to perform laparoscopic exploration and, if possible, extirpation of the solid tumor sequentially. A total of five trocars were used, and the tumor was found to originate from the omentum. We successfully performed complete resection of the tumor laparoscopically. A histological examination revealed lipoblastoma. For large abdominal tumors in children, the laparoscopic approach is recommended as the first procedure when the tumor is preoperatively considered to be benign and resectable.     

Arteriovenous malformation of the pancreas associated with hepatocellular carcinoma

Summary A case of pancreatic arteriovenous malformation (AVM) with hepatocellular carcinoma is reported. The patient, a 56-year-old Japanese man, was asymptomatic. The pancreatic lesion was found incidentally during an evaluation for hepatocellular carcinoma. Celiac arteriogram demonstrated tortuous feeding arteries, a racemose intrapancreatic stain, which disappeared before the venous phase, and early portal filling.