Notes

Built-in bioinformatics analysis of the NEDD4 loved ones unveils a new prognostic worth of NEDD4L in clear-cell renal cell cancers.
Obstructive sleep apnea (OSA) is a common sleep-disordered breathing disease that often leads to many comorbidities (e.g., cognitive dysfunction), which adversely affect the quality of life for patients with OSA. Thus far, the underlying mechanisms of this dysfunction remain unclear. Many studies have focused on OSA-related characteristics, including intermittent hypoxemia and sleep fragmentation. There is increasing emphasis on neuroimaging studies to explore underlying relationships between neuropathological changes and cognitive dysfunction. This article reviews recent research progress concerning cognitive dysfunction associated with OSA to reveal potential mechanisms that contribute to this dysfunction.
Scimitar syndrome (SS) is a rare type of congenital heart disease characterized by total or partial anomalous venous drainage of the right lung to the inferior vena cava. However, the surgical repair techniques for SS vary according to patients' anatomical and pathological features.

This study was performed to analyze the mid-term results of a less invasive surgical correction technique for SS in children.

Eleven patients with SS who underwent surgical repair from January 2012 to March 2020 were retrospectively analyzed. The anomalous scimitar vein (SV) was directly reimplanted to the left atrium, and the concomitant atrial septal defect was simultaneously repaired with cardiopulmonary bypass.

Three male and eight female patients were included in the study. Their mean age was 3.1 ± 1.3 years, and their mean body weight was 12.8 ± 3.0 kg. Most patients had symptoms, such as upper respiratory tract infection, dyspnea, and recurrent pneumonia, and two patients had pulmonary hypertension. None of the 11 patients who underwent direct SV reimplantation by right thoracotomy developed bleeding, arrhythmia, heart failure, or perioperative death, and no patients required reoperation during a mean follow-up period of 36.6 ± 15.2 months. Postoperative echocardiography revealed no restenosis or obstruction of the anastomosis in any patients.

Surgical repair for SS by right thoracotomy and direct anastomosis of the SV to the posterior wall of the left atrium is safe and effective, with good long-term patency of the reimplanted SV and a low mortality rate.
Surgical repair for SS by right thoracotomy and direct anastomosis of the SV to the posterior wall of the left atrium is safe and effective, with good long-term patency of the reimplanted SV and a low mortality rate.
The use of factor VIII (FVIII) concentrates under pharmacokinetic (PK) guidance has become the main approach for treatment of hemophilia. However, limited PK research has been conducted in Chinese pediatric patients.

To investigate the PK parameters of various FVIII concentrates in Chinese pediatric patients.

Seventy-nine patients were enrolled (28 treated with Kogenate FS
, 23 treated with Advate
, and 28 treated with GreenMono™). All enrolled patients participated in single-dose PK analysis after at least a 3-day washout period. Blood samples were collected predose, as well as at 1 h, 9 h, 24 h, and 48 h after infusion; FVIII levels were measured using a one-stage clotting assay. selleck chemicals von Willebrand Factor Antigen (VWFAg) levels and blood types were also determined. PK parameters were evaluated by WAPPS-Hemo.

Mean values of terminal elimination half-life time (t
) for the Kogenate FS
, Advate
, and GreenMono™ FVIII groups were 12.24 h, 10.18 h, and 9.62 h; median clearance values were 4.16, 6.23, and 5.11 mL·kg
·h
; and median
recovery values were 1.97, 1.55, and 1.61 IU/dL per IU/kg. Longer t
, higher
recovery, and lower clearance were observed in patients with higher VWFAg level who were treated with recombinant concentrates.

Chinese pediatric patients with hemophilia had FVIII PK characteristics similar to those previously observed in non-Chinese children, including large variation among individuals. VWFAg level and FVIII brand were associated with differences in FVIII PK. Thus, PK-guided dosing should be used to optimize individualized therapy in Chinese children.
Chinese pediatric patients with hemophilia had FVIII PK characteristics similar to those previously observed in non-Chinese children, including large variation among individuals. VWFAg level and FVIII brand were associated with differences in FVIII PK. Thus, PK-guided dosing should be used to optimize individualized therapy in Chinese children.
Cleft lip and palate (CLP) is globally among the most common childhood malformations. This disorder impacts childhood development, including speech and language, and affects children worldwide.

To analyze child development skills (adaptive fine motor, gross motor, personal-social, and language) in preschool children with isolated CLP compared with children without this malformation.

The participants included an experimental group of 27 children with isolated CLP and a comparison group of 27 children without CLP aged between 48 and 59 months. The groups were evaluated using two instruments the Denver Developmental Screening Test II (DDST-II) and the Avaliação do Desenvolvimento da Linguagem (ADL-Language Development Assessment). Data were analyzed by descriptive and inductive analyses, using the Student's
-test and the Mann-Whitney test, at a significance level of

0.05.

All children in the comparison group performed within normal standards for their age range in the DDST-II and the ADL. The worst performance in the experimental group was observed in language skills, followed, in declining order, by adaptive fine motor, personal-social, and gross motor as measured by the DDST-II. Children with isolated CLP also performed poorly in receptive, expressive, and global language in the ADL. No statistically significant differences were observed in the experimental group's scores for the ADL and the DDST-II.

Developmental skill levels were below expectations for children of this age with isolated CLP.
Developmental skill levels were below expectations for children of this age with isolated CLP.
Extensive population-based studies have explored the prevalence of primary hypertension (HTN) in children and adolescents. However, there is little published data on the characteristics of different types of pediatric HTN and the causes of secondary HTN.

To investigate the characteristics of different types of pediatric HTN and the causes of secondary HTN in a hospital setting.

The study cohort comprised pediatric inpatients (<18 years of age) discharged with a diagnosis of HTN from Beijing Children's Hospital during 2015-2020. Pediatric patients with HTN were allocated to secondary and primary HTN groups on the basis of comprehensive analyses of their diagnoses, family history of HTN, and findings on physical examination, as documented in their medical records. The Mann-Whitney
test,

and Fisher's exact test were used to assess differences in characteristics of patients with different HTN types and causes of secondary HTN.

Data of 1470 inpatients with HTN from 18 clinical departments were included in the analysis.
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