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Phosphorus is an essential substance in our body, and hypophosphataemia (HP) is well-described in rickets, refeeding syndrome, diabetic ketoacidosis (DKA), and in chronic alcohol-abuse. However, to our knowledge, HP among severely-malnourished children has not been studied in detail, and information on prevalence, severity, and treatment is scarce. Currently, there are only a few published case reports of HP. This case series describes three cases of HP that presented to Dhaka Hospital of the International Centre for Diarrhoeal Disease Research, Bangladesh (icddr,b). Our first case required mechanical ventilation for respiratory distress associated with severe hypokalaemia (K 1.1 mmol/L) and moderate hypophosphataemia (P 2.1 mg/dL). The second case presented with severe sepsis which was associated with symptomatic hypocalcaemia (Ca 1.68 mmol/L), hypokalaemia (K 1.82 mmol/L), and severe hypophosphataemia (P 0.9 mg/dL). The third case presented with pneumonia and sepsis which were complicated by hypokalaemia (K 2.05 mmol/L) and severe hypophosphataemia (P 1.1 mg/dL). Marked lethargy and severe hypotonia were associated with HP in all of these cases. Manifestations of HP are diverse and can occur in association with other electrolyte imbalances, especially among malnourished children. Malnutrition, combined with sepsis, is one of the major killers of children younger than 5 years of age, and both malnutrition and sepsis can cause HP. It is concluded that the underlying causes of morbidity, including HP, should be actively sought and treated to reduce the mortality of children aged below five years. [ABSTRACT FROM AUTHOR]

Yoshimatsu S, Ando Y, Terazaki H, Sakashita N, Tada S, Yamashita T, Suga M, Uchino M, Ando M (Kumamoto University School of Medicine; and Saiseikai Hospital, Kumamoto, Japan). Endoscopic and pathological manifestations of the gastrointestinal tract in familial amyloidotic polyneuropathy type I (Met30). J Intern Med 1998; 243: 65–72. ObjectivesTo evaluate the characteristic changes in the gastrointestinal tract in familial amyloidotic polyneuropathy (FAP) (Met30), both fibre gastroscopy and colonoscopy studies were performed in FAP (Met30) patients. Microscopic changes were also examined in autopsied and biopsied materials from patients with FAP, and compared with data from autopsied samples from patients with AL amyloidosis, and secondary amyloidosis patients. DesignEndoscopic and histopathological study. SettingKumamoto University Hospital, Kumamoto, Japan. SubjectsNine patients with FAP (Met30) underwent fibre gastroscopy and colonoscopy. Six autopsied and 23 biopsied gastrointestinal samples from FAP patients, four from autopsied amyloidosis (including two myeloma associated form), and two from autopsied secondary amyloidosis patients were examined for histopathological study. Main outcome measuresFibre gastroscopy and colonoscopy were employed for macroscopic study. Congo red and H-E staining were performed for histopathological study. Macroscopic changes in the gastrointestinal tract and microscopic differences in the amyloid distribution pattern were compared between the different types of amyloidosis. ResultsFibre gastroscopy and colonoscopy for nine FAP patients revealed that four showed a fine granular appearance in the duodenum, three showed lack of lustre, and two showed mucosal friability in the gastrointestinal tract; however, no macroscopic abnormality was observed in four other FAP patients. Histopathological examination of tissue from FAP patients revealed that, although a small amount of amyloid was recognized in the submucosa perivascular layer, a significant amount of amyloid was seen in and around the nerves of the gastrointestinal tract, but very little in Auerbach's nerve plexus. In total, the amount of deposited amyloid in the tissues was small compared with that in other types of systemic amyloidosis, such as AL and secondary amyloidosis. ConclusionThese results suggest that the major reason why FAP patients show such severe gastrointestinal symptoms, compared with other types of systemic amyloidosis, may be because of the deposition of a significant amount of amyloid in the nerves in the gastrointestinal tract. [ABSTRACT FROM AUTHOR]

Purpose: To analyze the influence of liver dysfunction and parenchymal pathology on the accumulation of superparamagnetic iron oxide (SPIO).Methods: We evaluated MR images of 13 patients having small hepatic neoplasms before and after administration of SPIO (10 micronol/kg). Biopsy and laboratory data confirmed the presence of severe cirrhosis in two patients, mild cirrhosis in four, chronic hepatitis in five, and normal livers in two. Degrees of liver dysfunction or liver parenchymal pathology were correlated with reductions in signal intensity of the liver and spleen after administration of SPIO. Signal intensity reduction was evaluated using a 1.5 Tesla MR unit.Results: Response to SPIO of the liver and spleen did not correlate with liver parenchymal pathology, although reductions in signal intensity of the liver were somewhat small in severely cirrhotic livers. There were slight correlations between signal intensity alterations of the liver and laboratory data such as the indocyanine green retention rate (correlation coefficient 0. 47), albumin (0.36), total bilirubin (0.36), and serum glutamic oxaloacetic transaminase (GOT) (0.46). Signal intensity reduction of spleen did not correlate with liver function tests except for serum GOT. In patients with cirrhosis, heterogeneous structures were detected in the nontumorous portions of the liver. However, these did not prevent the diagnosis of small hepatomas.Conclusion: The uptake of SPIO showed some correlation with liver function but not with chronic liver parenchymal pathology. SPIO provided sufficient contrast between tumor and surrounding liver parenchyma among patients with chronic liver disease. [ABSTRACT FROM AUTHOR]

The generation of reactive free radicals by Chattonella antiqua ('Red Tide'), which may be a causative factor in fish death, has been demonstrated by electron spin resonance spectroscopy and a microelectrode technique. Electrochemical detection indicates that superoxide anions are produced as a primary product, and undergo further reactions during diffusion in glycocalyx to produce hydrogen, hydroxy-, and carbon-centered radicals which are detected by ESR as secondary products. It is proposed that these reactive radicals are responsible for the destruction of the mucous membranes of fish on exposure to 'Red Tide'. [ABSTRACT FROM AUTHOR]

Non-human primates (NHPs) are the closest animal model to humans; thus, gene engineering technology in these species holds great promise for the elucidation of higher brain functions and human disease models. Knockin (KI) gene targeting is a versatile approach to modify gene(s) of interest; however, it generally suffers from the low efficiency of homology-directed repair (HDR) in mammalian cells, especially in non-expressed gene loci. In the current study, we generated a tyrosine hydroxylase (TH)-2A-Cre KI model of the common marmoset monkey (marmoset; Callithrix jacchus) using an HDR-biased CRISPR-Cas9 genome editing approach using Cas9-DN1S and RAD51. This model should enable labeling and modification of a specific neuronal lineage using the Cre-loxP system. Collectively, the current study paves the way for versatile gene engineering in NHPs, which may be a significant step toward further biomedical and preclinical applications., Competing Interests: Declaration of interests H.O. has been a paid scientific advisory board member of San Bio Co. Ltd., Regenerative Medicine iPS Gateway Center Co. Ltd., and K Pharma, Inc. S.Y. has been a paid associate researcher of Daiichi-Sankyo RD Novare Co. Ltd. However, there was no effect of these companies on the interpretation, writing, or publication of this study. H.O. and S.Y. declare that there are no non-financial conflicts of interest with this work. In addition, the other authors declare that there are neither financial nor non-financial conflicts of interest., (Copyright © 2023 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose: To examine the fracture strength and fracture mode of flared root canals reinforced with different post and core materials., Materials and Methods: Forty endodontically treated bovine teeth structured to mimic human mandibular premolars with flared root canals were reinforced with resin composite and glass fiber post (FRC), composite resin (RC), ceramic core (LD), and ceramic core with resin composite reinforcement (RLD), and restored with single zirconia crowns (n = 10 in each group). The fracture strength and mode of the root canals restored with zirconia crown were assessed. The fracture strength was compared with a one-way analysis of variance (ANOVA) following Tukey HSD tests. A multiple regression analysis was conducted to test the effect of the post/core materials on the fracture loads. Fisher's exact test was used in the failure mode analysis., Results: The mean fracture strength of RLD was significantly higher than RC, FRC, and LD (p < 0.05), while no significant differences were found among RC, FRC, and LD (p < 0.05). The regression analysis found that the fracture strength using the lithium disilicate was significantly lower for the post and higher for the core than that using the resin composite (p < 0.05), and there were no significant difference in the fracture strengths between the resin composite and glass fiber used for the post (p > 0.05). Most of the specimens exhibited root fractures, and no significant differences were observed among the groups (p < 0.05)., Conclusions: The results of this study suggest that reinforcement of flared root canals using a combination of resin composite for the core and lithium disilicate ceramic for the post is superior to resin composite and glass fiber in mechanical properties when restoring a single crown., (© 2022 by the American College of Prosthodontists.)

Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is an inherited cerebral small vessel disease (CSVD) caused by biallelic mutations in the high-temperature requirement serine peptidase A1 (HTRA1) gene. Even heterozygous mutations in HTRA1 are recently revealed to cause cardinal clinical features of CSVD. Here, we report the first establishment of a human induced pluripotent stem cell (hiPSC) line from a patient with heterozygous HTRA1-related CSVD. Peripheral blood mononuclear cells (PBMCs) were reprogrammed by the transfection of episomal vectors encoding human OCT3/4 (POU5F1), SOX2, KLF4, L-MYC, LIN28, and a murine dominant-negative mutant of p53 (mp53DD). The established iPSCs had normal morphology as human pluripotent stem cells and normal karyotype (46XX). Moreover, we found that the HTRA1 missense mutation (c.905G>A, p.R302Q) was heterozygous. These iPSCs expressed pluripotency-related markers and had the potential to differentiate into all three germ layers in vitro. HTRA1 and the supposed disease-associated gene NOG were differentially expressed in the patient iPSCs at mRNA levels compared to those of control lines. The iPSC line would facilitate in vitro research for understanding the cellular pathomechanisms caused by the HTRA1 mutation including its dominant-negative effect., (© 2023. The Author(s).)

Extended pluripotent stem cells (EPSCs) derived from mice and humans showed an enhanced potential for chimeric formation. By exploiting transcriptomic approaches, we assessed the differences in gene expression profile between extended EPSCs derived from mice and humans, and those newly derived from the common marmoset (marmoset; Callithrix jacchus). Although the marmoset EPSC-like cells displayed a unique colony morphology distinct from murine and human EPSCs, they displayed a pluripotent state akin to embryonic stem cells (ESCs), as confirmed by gene expression and immunocytochemical analyses of pluripotency markers and three-germ-layer differentiation assay. Importantly, the marmoset EPSC-like cells showed interspecies chimeric contribution to mouse embryos, such as E6.5 blastocysts in vitro and E6.5 epiblasts in vivo in mouse development. Also, we discovered that the perturbation of gene expression of the marmoset EPSC-like cells from the original ESCs resembled that of human EPSCs. Taken together, our multiple analyses evaluated the efficacy of the method for the derivation of marmoset EPSCs., (© 2022 Molecular Biology Society of Japan and John Wiley & Sons Australia, Ltd.)

Purpose: This study aimed to evaluate the surface strain at the cervical area of endodontically treated molars with a large pulp chamber restored using a composite resin core with three different types of core build-up systems., Methods: Reproduction models of human mandibular molars with prepared post spaces were used in this study. Roots duplicated with a composite resin were used as the experimental teeth. Three types of core build-up systems were used: composite resin core(RC), composite resin core with fiber posts (FC), and composite resin core with a prefabricated zirconia tube (ZC). Each group comprised eight specimens. Crowns made of yttria partially stabilized zirconia were cemented with dual-cure resin cement. Four strain gauges were attached to the surfaces of each specimen: the cervical area of the root and crown, on the buccal and lingual sides. The surface strain at each cervical area was measured using a static loading test and statistically analyzed., Results: In the case of static loading to the buccal cusp inner slope, ZC showed a significantly lower strain than RC in the crown on the buccal side and in the root and FC in the root. In the central fossa, ZC showed a significantly lower strain than FC in the root on the lingual side., Conclusions: The prefabricated zirconia tube reduced the surface strain at the cervical area of the buccal/lingual root in molars; however, the effect was small in the cervical area of the crown.