A case of novel NFKB2 variant with hypertensive emergency and nephrotic syndrome leading to CKD 5D.

Nuclear factor kappa B (NF-κB) family plays a central role in the human immune system. Heterozygous variants in NFKB2 typically cause immunodeficiency with various degrees of central adrenal insufficiency, autoimmunity, and ectodermal dysplasia. No reported case has presented kidney failure as an initial symptom. Moreover, documentation of kidney involvement of this disease is limited. CASE DIAGNOSIS: A 2-year-old female who presented with dyspnea and hypertensive emergency in the setting of new-onset nephrotic syndrome with acute-on chronic kidney injury with resultant chronic kidney disease (CKD) was found to have a novel heterozygous N-terminal variant in NFKB2 (c.880del: p. Tyr294Ilefs*4) with mild hypogammaglobulinemia, but no adrenal insufficiency or ectodermal dysplasia. She became dialysis-dependent during her initial hospitalization and developed CKD stage 5D, requiring continued peritoneal dialysis. She is currently awaiting kidney transplantation. CONCLUSIONS: Whether nephrotic syndrome or kidney injury or failure is the primary symptom of this variant or secondary to some event remains unknown. Further case accumulation is warranted.

Non-destructive assessment of amylose content in rice using a quality inspection system at grain elevators.

Amylose content is an important determinant of rice quality. Its accurate, non-destructive assessment is a challenge to the industry. We examined whether the automatic quality inspection system accurately measured the amylose content. Brown and milled rice models were calibrated with 902 samples produced between 2008 and 2017 in Hokkaido, Japan, and validated individually by samples collected in 2018 (n = 33) and 2019 (n = 71) from several grain elevators within the region. Models were developed by processing the automatic system data comprising of a near-infrared spectrometer, a visible light grain segregator, combined analysis of chemometrics, and by merging low and ordinary amylose variety validation results. The lower standard error of prediction (<0.52%) and a higher ratio of performance deviation (>4.0) in both models enabled accurate non-destructive assessment of amylose content. Hence, the automatic quality inspection system is a useful tool for meeting the demands of higher quality and palatability of rice.

Synthesis and redox behavior of ruthenocene-terminated oligoenes: characteristic and stable two-electron redox system and lower potential shift of the two-electron oxidation wave with elongating conjugation.

Ruthenocene-terminated butadienes and hexatrienes were prepared by the Wittig reaction of 3-ruthenocenyl-2-propenals with ruthenocenylmethylphosphonium salts and the Mukaiyama coupling of the propenals, respectively. Cyclic voltammetry of these complexes indicated that they were involved in a stable two-electron redox process. The oxidation potentials for ruthenocene-terminated oligoenes shifted progressively to lower potential with the increasing CH==CH units as follows: Rc--Rc (0.32 V)>RcCH==CHRc (+0.09 V)>Rc(CH==CH)(2)Rc (-0.06 V)>Rc(CH==CH)(3)Rc (-0.07 V), (Rc=ruthenocene). The tendency is in remarkable contrast to that in the successive one-electron redox process. These complexes were chemically oxidized to give stable crystalline solids, whose structures were confirmed by NMR spectroscopy and X-ray analysis to be oligoene analogues of a bis(fulvene) complex, for example, [(eta(5)-C(5)Me(5))Ru[mu(2)-eta(6):eta(6)-C(5)H(4)CH(CH==CH)(n)CHC(5)H(4)]Ru(eta(5)-C(5)Me(5))](2+) (n=1 or 2). The DFT calculation of the two-electron-oxidized species reproduced well the fulvene-complex structure for the ruthenocene moieties. Since both the neutral and oxidized species are stable and chemically reversible, this redox system may be serviceable as a two-electron version of the ferrocene one-electron redox system.