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Simple Summary: The transition period (3 wks before to 3 wks after calving) is an important period in the lactation cycle of dairy cows. During this period, dairy cows undergo a series of physiological and metabolic changes due to the demands of pregnancy, parturition and postpartum lactation, which can easily lead to a negative energy balance (NEB). Therefore, the energy balance and nutritional regulation mechanism of dairy cows during the transition period are the focus of dairy cow nutrition and physiology research. However, crossbred cows have received less attention to date. Our study showed that feeding a prenatal concentrate mix at a rate of 0.6% of the body weight of Montbéliarde–Holstein crossbred cows has no negative effect on the performance and rumen fermentation of postpartum dairy cows and can satisfy the energy needs of dairy cows in the prepartum period. Furthermore, our data provide a theoretical basis for further revisions to the feeding standard and to alleviate the NEB of Montbéliarde–Holstein crossbred cows in China. This study was conducted to investigate the effect of three rates of prepartum dietary concentrate feeding on the milk performance, energy balance, and rumen fermentation of Montbéliarde–Holstein crossbred cows. Eighteen transition Montbéliarde–Holstein crossbred cows with similar days of gestation (258 ± 12 day) and body weights (622 ± 44 kg) were selected and randomly divided into three groups. In the prepartum period, the addition of concentrates accounted for 0.3% (low concentrate, LC), 0.6% (medium concentrate, MC), and 0.9% (high concentrate, HC) of the cow's body weight. The forage was corn stover, which was fed to the cows ad libitum with free access to water. Postpartum, all of the cows were fed a common lactation total mixed ration. The experimental period lasted from 21 days prepartum to 28 days postpartum. The energy balance (EB), net energy intake (NEI), and dry matter intake (DMI) of the HC group were greater than those of the other groups (p < 0.05). Likewise, the non-esterified fatty acids (NEFA), β-hydroxybutyric acid (BHBA), and total bilirubin (TBIL) in the blood of the LC group had significantly higher concentrations than they did in the other groups (p < 0.05). Moreover, the increase in the level of dietary concentrate had no significant effect on the rumen fermentation parameters (p > 0.05), and the total intestinal digestibility of dry matter (DM), crude protein (CP), and ether extract (EE) in the HC group was significantly higher (p < 0.05) than it was in the other groups during the prepartum period. In conclusion, the administration of the MC diet in the prepartum period had no negative effect on the performance and rumen fermentation of postpartum dairy cows and can satisfy the energy needs of prepartum dairy cows. Therefore, under our experimental conditions, the 0.6% prenatal concentrate feeding amount was the most appropriate for Montbéliarde–Holstein crossbred cows. [ABSTRACT FROM AUTHOR]

Background: Beta-ketothiolase deficiency (BKTD) is an autosomal recessive disorder caused by biallelic mutation of ACAT1 that affects both isoleucine catabolism and ketolysis. There is little information available regarding the incidence, newborn screening (NBS), and mutational spectrum of BKTD in China.Results: We collected NBS, biochemical, clinical, and ACAT1 mutation data from 18 provinces or municipalities in China between January 2009 and May 2020, and systematically assessed all available published data from Chinese BKTD patients. A total of 16,088,190 newborns were screened and 14 patients were identified through NBS, with an estimated incidence of 1 per 1 million newborns in China. In total, twenty-nine patients were genetically diagnosed with BKTD, 12 of which were newly identified. Most patients exhibited typical blood acylcarnitine and urinary organic acid profiles. Interestingly, almost all patients (15/16, 94%) showed elevated 3-hydroxybutyrylcarnitine (C4OH) levels. Eighteen patients presented with acute metabolic decompensations and displayed variable clinical symptoms. The acute episodes of nine patients were triggered by infections, diarrhea, or an inflammatory response to vaccination. Approximately two-thirds of patients had favorable outcomes, one showed a developmental delay and three died. Twenty-seven distinct variants were identified in ACAT1, among which five were found to be novel.Conclusion: This study presented the largest series of BKTD cohorts in China. Our results indicated that C4OH is a useful marker for the detection of BKTD. The performance of BKTD NBS could be improved by the addition of C4OH to the current panel of 3-hydroxyisovalerylcarnitine and tiglylcarnitine markers in NBS. The mutational spectrum and molecular profiles of ACAT1 in the Chinese population were expanded with five newly identified variants. [ABSTRACT FROM AUTHOR]