Intact survival from severe cardiogenic shock caused by the first attack of atrial tachycardia treated with extracorporeal membrane oxygenation and surgical left atrium appendage resection: a case report

Background Atrial tachycardia (AT) is rare in children and can usually be reversed to sinus rhythm with pharmacotherapy and cardioversion. We report a rare case of severe left-sided heart failure due to refractory AT. Case presentation A 12-year-old boy had AT with a heart rate of 180 beats/minute, which was refractory to any medication and defibrillation despite the first attack. Due to rapid cardiorespiratory collapse shortly after arriving at our hospital, central extracorporeal membrane oxygenation (ECMO) with left arterial venting was started immediately. Although AT persisted after that, it stopped on the 3rd day after admission following surgical resection of the left atrial appendage thought to be the source of AT. He was weaned off ECMO on the 7th day and ventilator on the 14th day. Conclusions The appropriate timing of central ECMO and surgical ablation were effective in saving this child from a life-threatening situation caused by refractory AT

An endogenous retrovirus presumed to have been endogenized or relocated recently in a marsupial, the red-necked wallaby

白いワラビーの遺伝的原因を初めて解明 --世界各地の動物園で相次いで誕生--. 京都大学プレスリリース. 2022-01-18.An albino infant wallaby was born to a mother with the wild-type body color. PCR and sequencing analyses of TYR (encoding tyrosinase, which is essential for melanin biosynthesis) of this albino wallaby revealed a 7.1-kb-long DNA fragment inserted in the first exon. Because the fragment carried long terminal repeats, we assumed it to be a copy of an endogenous retrovirus, which we named walb. We cloned other walb copies residing in the genomes of this species and another wallaby species. The copies exhibited length variation, and the longest copy (>8.0 kb) contained open reading frames whose deduced amino acid sequences were well aligned with those of gag, pol, and env of retroviruses. It is not known through which of the following likely processes the walb copy was inserted into TYR: endogenization (infection of a germline cell by an exogenous virus), reinfection (infection by a virus produced from a previously endogenized provirus), or retrotransposition (intracellular relocation of a provirus). In any case, the insertion into TYR is considered to have been a recent event on an evolutionary timescale because albino mutant alleles generally do not persist for long because of their deleterious effects in wild circumstances

The peak height ratio of S-sulfonated transthyretin and other oxidized isoforms as a marker for molybdenum cofactor deficiency, measured by electrospray ionization mass spectrometry

AbstractMolybdenum cofactor deficiency is a fatal neurological disorder, which follows an autosomal-recessive trait and is characterized by combined deficiency of the enzyme, sulfite oxidase, xanthine dehydrogenase and aldehyde oxidase. Early detection of molybdenum cofactor-deficient patients is essential for their proper care and genetic counseling of families at risk. We demonstrate the use of S-sulfonated transthyretin (TTR) as a marker for molybdenum cofactor deficiency. Plasma or sera obtained from 4 patients with molybdenum cofactor deficiency and 57 controls were studied by electrospray ionization mass spectrometry (ESIMS) following selective enrichment of TTR by immunoprecipitation using protein G/A agarose. The data obtained from molybdenum cofactor deficiency samples indicated a strong increase in the peak height of S-sulfonated TTR. A more significant difference was revealed if the peak height ratio of S-sulfonated TTR and the sum of the other oxidized TTR were determined. By accurate determination of the ratio, the samples of molybdenum cofactor deficiency patients could clearly be distinguished from controls without molybdenum cofactor deficiency

No predialysis treatment of blood primes in pediatric continuous kidney replacement therapy

Abstract Background Pediatric continuous kidney replacement therapy (CKRT) uses blood as the priming fluid in the CKRT circuit to prevent hemodilution and hypotension and is dialyzed using a dialysate before the start of CKRT. This study aimed to investigate the safety of CKRT using a protocol of no predialysis after blood priming in underweight infants, based on hemodynamic and laboratory data changes. Methods This single-center retrospective cohort study included children weighing  95% of the levels before extracorporeal circulation. Moreover, potassium levels, which were not significantly different between extracorporeal circulation and dialysis initiation (p = 1.000), were significantly decreased after dialysis (p = 0.046). Lactate and hematocrit did not significantly change either before dialysis (p = 0.131 and 0.071, respectively) or after dialysis compared to the time of extracorporeal circulation (p = 1.000 and 0.591, respectively). Conclusions CKRT, using our protocol, could be safely performed without predialysis treatment of blood primes in children weighing < 5 kg