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1.
Am J Med Genet A ; 191(11): 2736-2742, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37658587

RESUMO

In this retrospective cohort study, we investigated the impact of tracheostomies on the long-term survival of children with trisomy 13 syndrome at a Japanese tertiary pediatric center. We compared survival and survival to discharge rates between patients who underwent tracheostomies during their NICU stays (T group, n = 8) and those who did not (non-T group, n = 11). A total of 19 patients enrolled. Median survival in all patients was 673 (266-1535) days. Significant differences in the 1-, 2-, and 3-year survival rates were found between the T and the non-T groups (100% vs. 46%, p = 0.018; 88% vs. 18%, p = 0.006; 63% vs. 9%, p = 0.041, respectively). The survival to discharge rate was higher in the T versus non-T group (75% vs. 45%, p = 0.352). This study highlights a significantly higher long-term survival of patients with trisomy 13 syndrome who underwent tracheostomies during their NICU stays.

2.
Pediatr Res ; 94(4): 1400-1407, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36528748

RESUMO

BACKGROUND: Elevated albumin-free or unbound bilirubin (UB) levels beyond the first week of life have been associated with the development of bilirubin encephalopathy in preterm infants. However, the mechanism(s) that induces this prolonged unbound bilirubinemia has remained unknown. We hypothesized that it may due to a sustained lower bilirubin-binding affinity of albumin in extremely premature infants. METHODS: Twenty-two very preterm infants born at 28-31 weeks' gestational age (GA) (VPT Group) and 21 extremely preterm infants born at 22-27 weeks' GA (EPT Group) were retrospectively studied. On days 14, 21, and 28, bilirubin-binding affinity of albumin was assessed by calculating of the UB/total bilirubin ratio, bilirubin-albumin molar ratio (BAMR), and binding affinity (Ka). RESULTS: On days 14, 21, and 28, significantly higher UB/total bilirubin ratios were found in the EPT than in the VPT Group. Although BAMRs were comparable, significantly lower Ka values on days 14, 21, and 28 were observed in the EPT than those in the VPT Group (56.1 vs. 70.9 L/µmol, p < 0.001; 55.2 vs. 74.7 L/µmol, p < 0.001; 53.0 vs. 86.5 L/µmol, p < 0.001, respectively). CONCLUSIONS: EPT infants have a sustained lower bilirubin-binding affinity of albumin beyond the first week of life. IMPACT: Bilirubin encephalopathy is still reported in extremely preterm (EPT) infants. EPT infants often have prolonged unbound bilirubinemia beyond the first week of life. Sustained lower bilirubin-binding affinity of albumin, regardless of the bilirubin-albumin molar ratio (BAMR), is observed in EPT infants. BAMRs should not be used as a surrogate marker of unbound bilirubinemia, especially in EPT infants at a later postnatal period.


Assuntos
Lactente Extremamente Prematuro , Kernicterus , Humanos , Bilirrubina/metabolismo , Estudos Retrospectivos , Albumina Sérica/análise , Recém-Nascido
3.
Clin Lab ; 68(2)2022 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-35142193

RESUMO

BACKGROUND: In the original glucose oxidase-peroxidase (GOD-POD) protocol, the time required to decrease the initial total bilirubin (TB) level by 20% is used for unbound bilirubin (UB) calculation. However, it needs to continuously monitor the TB levels by spectrometry. METHODS: Here, we hypothesized that the TB decrease during fixed time periods can also be used to extrapolate UB values (fixed-time protocol). Serum UB levels measured by the different protocols were compared using 10 newborn serum samples. RESULTS: Serum UB levels determined using the fixed-time protocol, especially using periods of 10 - 40 seconds, were strongly correlated with those determined using the original protocol (coefficient of determination > 0.9). The fixed-time protocol, using periods of 20 - 60 seconds, showed the high measurement precision (coefficient of variation < 5%). CONCLUSIONS: The fixed-time protocol, using periods of 20 - 40 seconds, can help determine serum UB levels as effectively as the original protocol.


Assuntos
Glucose Oxidase , Peroxidase , Bilirrubina , Humanos , Recém-Nascido , Oxirredutases , Peroxidases
4.
Am J Med Genet A ; 188(4): 1048-1055, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-34889030

RESUMO

The effects of medical and surgical interventions on the survival of patients with trisomy 18 have been reported, leading to changes in perinatal management and decision-making. However, few studies have fully reported the recent changes in survival and treatment of trisomy 18. We examined how treatment and survival of patients with trisomy 18 have changed over a decade in a Japanese pediatric tertiary referral center. This retrospective cohort study included patients with trisomy 18 who were admitted within the first 7 days of life at the Hyogo Prefectural Kobe Children's Hospital between 2008 and 2017. The patients were divided into early period (EP) and late period (LP) groups based on the birth year of 2008-2012 and 2013-2017, respectively. Changes in treatment and survival rates were compared between the two groups. A total of 56 patients were studied (29 in the EP group and 27 in the LP group). One-year survival rates were 34.5% and 59.3% in the EP and LP groups, respectively. The survival to discharge rate significantly increased from 27.6% in the EP group to 81.5% in the LP group (p < 0.001). The proportion of patients receiving surgery, especially for congenital heart defects, significantly increased from 59% in the EP group to 96% in the LP group (p = 0.001). In our single-center study, survival and survival to discharge were significantly improved in patients with trisomy 18, probably because of increased rate of surgical interventions. These findings may facilitate better decision-making by patients' families and healthcare providers.


Assuntos
Cardiopatias Congênitas , Criança , Feminino , Cardiopatias Congênitas/diagnóstico , Cardiopatias Congênitas/genética , Humanos , Alta do Paciente , Gravidez , Estudos Retrospectivos , Taxa de Sobrevida , Trissomia/genética , Síndrome da Trissomia do Cromossomo 13/diagnóstico , Síndrome da Trissomia do Cromossomo 13/genética , Síndrome da Trissomía do Cromossomo 18/diagnóstico , Síndrome da Trissomía do Cromossomo 18/genética
6.
J Oral Rehabil ; 48(4): 411-421, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33128790

RESUMO

The deterioration of oral function to a state of oral hypofunction (OHF) is reportedly associated with malnutrition and frailty. Thus, we Investigated the association of OHF with physical characteristics and function and test the effects of a programme including comprehensive oral and physical exercises and textured lunch gatherings (COPE-TeL programme) on oral and physical function in older adults with OHF. Eighty-six community-dwelling older adults were randomly assigned into control (n = 43) or intervention (n = 43) groups. The participants were further divided into OHF and normal oral function (NOF) sub-groups based on initial oral examinations. The intervention group participated in the 12-week COPE-TeL programme, while the control group performed the physical exercise regimen only. The differences in measured variables for physical and oral function between the OHF and NOF groups were statistically tested, and changes in the proportion of participants with OHF were examined. Physical function, such as hand grip strength and walking speed, was significantly lower in the OHF group at the initial assessment. The proportion of participants with OHF was 56% in the intervention group and 67% in the control group before the trial, which became significantly reduced after completing the COPE-TeL programme in the intervention group (26%, P = .002), but not in the controls (61%, P = .549). Older adults with OHF may have diminished physical function. The COPE-TeL programme of oral and physical exercises along with textured lunch gatherings may be effective for older adults with OHF.


Assuntos
Fragilidade , Almoço , Idoso , Exercício Físico , Terapia por Exercício , Força da Mão , Humanos
7.
J Bacteriol ; 202(7)2020 03 11.
Artigo em Inglês | MEDLINE | ID: mdl-31964699

RESUMO

Bacterial promoters consist of core sequence motifs termed -35 and -10 boxes. The consensus motifs are TTGACA and TATAAT, respectively, which were identified from leading investigations on Escherichia coli However, the consensus sequences are not likely to fit genetically divergent bacteria. The sigma factor of the genus Bifidobacterium has a characteristic polar domain in the N terminus, suggesting the possibility of specific promoter recognition. We reevaluated the structure of Bifidobacterium longum NCC2705 promoters and compared them to other bacteria. Transcriptional start sites (TSSs) of the B. longum NCC2705 strain were identified using transcriptome sequencing (RNA-Seq) analysis to extract promoter regions. Conserved motifs of a bifidobacterial promoter were determined using regions upstream of TSSs and a hidden Markov model. As a result, consensus motifs of the -35 and -10 boxes were TTGTGC and TACAAT, respectively. To assess each base of both motifs, we constructed 37 plasmids based on pKO403-TPCTcon, including the hup promoter connected with a chloramphenicol acetyltransferase as a reporter gene. This reporter assay showed two optimal motifs of the -35 and -10 boxes, namely, TTGNNN and TANNNT, respectively. We further analyzed spacer lengths between the -35 and -10 boxes via a bioinformatics approach. The spacer lengths predominant in bacteria have been generally reported to be approximately 17 bp. In contrast, the predominant spacer lengths in the genus Bifidobacterium and related species were 11 bp, in addition to 17 bp. A reporter assay to assess the spacer lengths indicated that the 11-bp spacer length produced unusually high activity.IMPORTANCE The structures of sigma factors vary among bacterial strains, indicating that recognition rules may also vary. Therefore, we investigated the promoter structure of Bifidobacterium longum NCC2705 using a bioinformatics approach and wet analyses. The most frequent and optimal motifs were similar to other bacterial consensus motifs. The optimal spacer length between the two boxes was reported to be 17 bp. It is widely applied to a bioinformatics approach for other bacteria. Unexpectedly, conserved spacer lengths were 11 bp as well as 17 bp in the genus Bifidobacterium Moreover, the sigma factor of the genus Bifidobacterium has a characteristic domain in the N terminus which may contribute to the additional functions. Hence, it would be valuable to reevaluate the promoter in other organisms.


Assuntos
Bifidobacterium longum/genética , Motivos de Nucleotídeos , Regiões Promotoras Genéticas , Biologia Computacional/métodos , Regulação Bacteriana da Expressão Gênica , Matrizes de Pontuação de Posição Específica , Análise de Sequência de DNA , Sítio de Iniciação de Transcrição
8.
J Oral Rehabil ; 47(2): 180-186, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31696953

RESUMO

BACKGROUND: Preserving sufficient oral function and maintaining proper nutrition are essential to prevent frailty. Thus, we have developed "munchy" foods that contain harder textures and are rich in protein. OBJECTIVES: This study aimed to test the effects of masticating textured foods on masticatory muscle activity in young and older adults. METHODS: Twenty young and 32 community-dwelling older individuals participated in this study. After measuring oral function, we subdivided the older participants into normal and oral hypofunction (OHF) groups. Two test foods (meatloaf and chicken ball) were prepared to have a harder texture using specific ingredients (munchy) or not (control). The participants ate 10 g of the test foods in random order while being measured for masseter muscle activity with a surface electromyogram (EMG). We calculated the number of chewing cycles and integrated muscle activity of the masseter muscle from the EMG data and tested for differences by food texture or age group. RESULTS: The number of chewing cycles, mean EMG amplitude and integrated EMG activity was significantly higher for the munchy foods than for the controls for all groups. The integrated masseter muscle EMG activity was significantly increased in the normal older group than in the young group for both food types, but not significantly different between in OHF and young groups. CONCLUSIONS: Our findings suggest that eating textured foods will lead to increased masticatory load and therefore increased muscle activity, especially in older adults. Application of textured food may change dietary habits in older adults.


Assuntos
Músculo Masseter , Músculos da Mastigação , Idoso , Eletromiografia , Alimentos , Humanos , Mastigação
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