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PURPOSE The accuracy of the tibial crest (TC) in guiding the mechanical alignment of the tibial component in total knee arthroplasty (TKA) has been investigated in a few studies on Asian patients. No study analyzed the anatomical variants of the TC. We analyzed the morphological types of the TC in cadaveric tibiae of Caucasian subjects and assessed whether the TC may be considered an accurate guide for the mechanical alignment of the tibial component in TKA. METHODS The TC and mechanical axis (MA) were identified in 86 dried cadaveric tibiae by placing metal landmarks along the TC course and a guidewire overlapping the MA. Coronal view radiographs were taken in different positions of tibial axial rotation, and the relationships between the TC and MA were analyzed. RESULTS The TC showed three different patterns, comprising a curved, mixed and straight course in 47 (54.6%), 21 (24.4%) and 18 (20.9%) tibiae, respectively. selleck chemicals When a curved course was found, the TC intersected the MA at proximal and distal points located, on average 22.4% and 63.3% along the tibial length, respectively. When a straight course was found, the mean angle between the TC and MA was 2.9°. In 35% of the cases, the two axes differed by more than 3°. CONCLUSION The TC of Caucasian subjects exhibits a marked variability in its course and relationship with the MA. Unlike the TC in Asian subjects, the TC of Caucasians cannot be considered an accurate anatomical reference to guide the coronal alignment of the tibial component in TKA.Secretory breast carcinoma constitutes the majority of breast cancers in children and young people less than 20 years of age. Noninvasive examination is particularly necessary for the diagnosis of breast carcinoma in children. Herein, we report a case of secretory breast carcinoma in a 6-year-old girl with psychomotor retardation. She was referred to our outpatient clinic for evaluation of a palpable mass in her left breast. A hard mass, rather than the increase in size typical of premature thelarche, was palpated. An excision biopsy was performed. Pathological findings revealed an invasive secretory breast carcinoma. We performed a retrospective review of the preoperative findings of this case, and compared it to the pathological diagnosis. Elastography, which can be performed without deep sedation or general anesthesia and without causing pain, resulted in a stiffness score of 4; however, the distinction between benign and malignant tumors on elastography, which is important to decide the intra-operative procedures, was not sufficient according to the Japanese breast cancer society clinical guidelines. This is the first report of secretory breast carcinoma in a child with a stiffness score determined by tissue elasticity imaging. A breast mass in a child with a high stiffness score of more than 4 on elastography should be referred for invasive diagnostic procedures, such as fine needle aspiration or excisional biopsy. According to our experience, an accurate preoperative diagnosis could be possible for malignant breast tumors in children. Such parameters as stiffness score on elastography are practical, noninvasive, and objective diagnostic tools for the accurate preoperative diagnosis of breast tumors in children.The original version of this article unfortunately contained error in Figure 4a to where some of the text was overlapping.Mutations in the CDKL5 gene, which encodes a serine/threonine kinase, causes a rare encephalopathy, characterized by early-onset epilepsy and severe intellectual disability, named CDKL5 deficiency disorder (CDD). In vitro and in vivo studies in mouse models of Cdkl5 deficiency have highlighted the role of CDKL5 in brain development and, in particular, in the morphogenesis and synaptic connectivity of hippocampal and cortical neurons. Interestingly, Cdkl5 deficiency in mice increases vulnerability to excitotoxic stress in hippocampal neurons. However, the mechanism by which CDKL5 controls neuronal survival is far from being understood. To investigate further the function of CDKL5 and dissect the molecular mechanisms underlying neuronal survival, we generated a human neuronal model of CDKL5 deficiency, using CRISPR/Cas9-mediated genome editing. We demonstrated that CDKL5 deletion in human neuroblastoma SH-SY5Y cells not only impairs neuronal maturation but also reduces cell proliferation and survival, with alterations in the AKT and ERK signaling pathways and an increase in the proapoptotic BAX protein and in DNA damage-associated biomarkers (i.e., γH2AX, RAD50, and PARP1). Furthermore, CDKL5-deficient cells were hypersensitive to DNA damage-associated stress, accumulated more DNA damage foci (γH2AX positive) and were more prone to cell death than the controls. Importantly, increased kainic acid-induced cell death of hippocampal neurons of Cdkl5 KO mice correlated with an increased γH2AX immunostaining. The results suggest a previously unknown role for CDKL5 in DNA damage response that could underlie the pro-survival function of CDKL5.PURPOSE To address a critical gap in the existing Health-Related Quality of Life (HRQOL) literature by quantifying and describing HRQOL among transgender and gender expansive (TG/GE) youth at a rural gender clinic and comparing the HRQOL of these youth to age-stratified United States (U.S.) population standards. METHODS This cross-sectional study includes results of the baseline HRQOL assessment of 141 TG/GE patients enrolled in the Gender Wellness Center (GWC) Pediatric Patient Registry. HRQOL was assessed using the Child Health Questionnaire-Child Form 87 (CHQ-CF87; ages  less then  18) and the Short Form-36 (SF-36v2; ages 18-21). Mean subscale scores were compared to the most current U.S. population standards available. RESULTS On all but one of the CHQ-CF87 subscales, TG/GE youth scored significantly lower than 2015-2016 U.S. general population youth and youth with two chronic conditions. On the SF-36v2, TG/GE youth scored significantly higher than 2009 U.S. standards on all physical health domains but lower on all but one of the mental health domains. CONCLUSIONS Cross-sectional HRQOL data from a registry of TG/GE youth indicate significantly poorer mental health measures as compared with the U.S. general population. Longitudinal assessments are needed to evaluate whether HRQOL improves with gender-affirming care. Future studies should aim to identify sociocultural factors at the intersection of rurality and health that contribute to diminished HRQOL among rural TG/GE youth.

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