Pennboysen9514
CLOVES syndrome is a novel sporadic mosaic segmental overgrowth syndrome, currently categorized under the canopy of PROS (PIK3CA-related overgrowth spectrum) disorders. All PROS disorders harbor heterozygous postzygotic activating somatic mutations involving the PIK3CA gene. As an upstream regulator of the PI3K/AKT/mTOR signal transduction pathway, activating mutations of PIK3CA gene commence in uncontrolled growth of cutaneous, vascular (capillaries, veins, and lymphatics), adipose, neural, and musculoskeletal tissues. The excessive growth is segmental, patchy, asymmetric, and confined to body parts affected by the mutation. The term 'CLOVES' is an acronym denoting congenital lipomatous overgrowth, vascular malformations, epidermal nevi and spinal (scoliosis) and/ or skeletal anomalies. The syndrome is characterized by an admixture of overgrown tissues, derived mainly from mesoderm and neuroectoderm. Among PROS disorders, CLOVES syndrome represents the extreme end of the spectrum with massive affection of almost the entire body. The syndrome might judiciously be treated with medications hampering with the PI3K/AKT/mTOR signal transduction pathway. This article aims at reviewing the cutaneous and musculoskeletal manifestations of CLOVES syndrome, as the paradigm for PROS disorders. CLOVES syndrome and other PROS disorders are still misdiagnosed, underdiagnosed, underreported, and undertreated by the dermatology community.
Port wine stain (PWS) is a congenital skin lesion involving capillary malformations. Most PWS lesions will gradually become hypertrophic and appear nodular in contour. Current research shows that rapamycin, an mTOR inhibitor, is probably a promising adjunctive therapy for PWS, which suggests that the mTOR signaling pathway may play an important role in its pathological process.
From January 2013 to January 2019, 13 samples were obtained during the surgical excision. Each sample was divided into 3 parts according to the type of lesion, namely, the flat, hypertrophic and nodular lesions. Pathologic structures of each type were observed under the microscope after HE staining. The expression of mTORC1, p70S6, p-p70S6, eIF4EBP1 and p-eIF4EBP1 was examined by immunohistochemical staining and western blotting. The location of the expression of mTORC1, p-p70S6 and p-elF4EBP1 was further detected by immunofluorescence staining.
Large amounts of dilated and malformed vessels were observed in all types of PWS lesilated syndromes by inhibiting mTOR signaling pathway.Bullous pemphigoid (BP) is the most frequent autoimmune bullous disease mainly affecting elderlies. Diagnosis usually results from clinical features, histological examination, and the quantification of circulating typical autoantibodies, due to its higher incidence in elderly patients, bullous pemphigoid treatment and management still represents a challenge due to the higher frequency of several comorbidities in this group of patients, which may also be linked to a reduced tolerance to BP treatments. Hence, an early diagnosis and a prompt correct treatment are mandatory to reach better clinical outcomes and improve as much as possible BP outcomes. Herein, we carried out a comprehensive literature review about the known clinical presentations, diagnosis, assessment and monitoring procedures used in daily clinical practice in patients with BP, to better define strategies to improve as much as possible BP clinical outcomes.
As the human body's largest organ exposed to the external environment, the skin suffers from internal and external aging factors, leading to wrinkles, loss of elasticity, sagging, and rough appearance. However, little is known of the characteristics of skin aging of different body parts in Chinese women. Here, we study the signs of extrinsic skin aging in different body parts to identify the knowledge map of manifestations of aging in Chinese women.
Wrinkle and texture phenotypes and collagen samples from the face, neck, hands, and arms of 326 Chinese women were collected. The correlations between phenotypes and ages and the differences in phenotypes by age were evaluated.
The wrinkle and texture phenotypes around the eyes and mouth and of the hands were strongly correlated with age. Ages 32 and 58 showed the largest number of differentially changed aging phenotypes. The number of aging phenotypes increased sharply between the ages of 24 and 30, suggesting that the skin was undergoing rapid aging. Eye aging was the most rapidly changing phenotype between 19 and 30 years old. Wrinkles at the corner of the eyes showed a significant difference in the older group, suggesting an early onset and long-term effects.
This is the first study to be performed on the characteristics of skin aging among Chinese women that takes account of multiple areas of the body. It was found that 24 years old was the time point at which the skin begins to age in Chinese women. This provides important clues for aging-related research and personalized skin care.
This is the first study to be performed on the characteristics of skin aging among Chinese women that takes account of multiple areas of the body. It was found that 24 years old was the time point at which the skin begins to age in Chinese women. This provides important clues for aging-related research and personalized skin care.Pilomatricoma, also known as "benign calcifying epithelioma", is an uncommon slow-growing benign adnexal skin tumor, which originates from primitive cells of the hair matrix and usually appears as a solitary, firm, and asymptomatic nodule beneath the skin. Bullous pilomatricoma is a rare form of pilomatricoma, always presenting with firm subcutaneous nodules with a bullous appearance. In this study, we report a 9-year-old Chinese presenting girl with bullous pilomatricoma after influenza vaccination.
Patients seeking treatment for the midface are individuals with different objectives and characteristics of skin and soft tissue. In this study, patients were treated in the midface with one of three products with different properties; HA
, HA
, or HA
, using a treatment algorithm based on primary need for treatment (volumizing, lifting or contouring) and tissue coverage (thin or thick tissue). The aim was to optimize the treatment outcome and to provide an individualized treatment approach that distinguishes different types of patients seeking midfacial treatment.
Subjects were treated in the midface at baseline to achieve optimal aesthetic results (≤2 mL per side of the face), and touch-up was allowed after 4 weeks (≤1 mL per side of the face). Study visits were scheduled at Weeks 8, 16 and 24. Assessments included aesthetic improvement of the midface, evaluation of midface fullness, and subject satisfaction. Safety evaluations included local tolerability symptoms collected at 4 weeks after treatmenteeks after treatment.Linear scleroderma en coup de sabre (LSCS) is a variant of localized scleroderma associated with band-like fibrotic lesions in the frontoparietal area. We report a case of LSCS in a woman who presented with progressive mild hyperchromia on the right side of her forehead, with dermal atrophy and hair and eyebrow loss. After the failure of conservative treatments, the patient responded dramatically to injection of autologous localized concentrated growth factor. After three treatments, the atrophy, stiffness, and angiotelectasis on the affected area had improved. No recurrence was detected 24 months after the last treatment. This is the first study describing the use of autologous concentrated growth factor injection to alleviate clinical symptoms of LSCS. This suggests that concentrated growth factor may be a treatment for LSCS in the clinic.
To explore the relationship between non-alcoholic fatty liver disease (NAFLD) and chronic kidney disease (CKD) in patients with type 2 diabetes mellitus (T2DM).
A total of 1168 patients with T2DM were divided into the non-CKD and CKD groups, and the difference in the prevalence of NAFLD was compared. The differences in serum creatinine (SCr) and urine albumin-to-creatinine ratio (UACR) levels were compared between the non-NAFLD and NAFLD groups. Patients with T2DM were divided into three groups according to their UACR levels (UACR < 30 mg/g [U1 group]; UACR ≤ 30 mg/g to < 300 mg/g [U2 group]; and UACR ≥ 300 mg/g [U3 group]) or estimated glomerular filtration rate (eGFR) levels (≥ 90 mL/min [G1 group]; eGFR ≤ 60 mL/min to < 90 mL/min [G2 group]; and eGFR < 60 mL/min (G3 group]). The difference in the prevalence and risks of NAFLD in the different UACR or eGFR level groups was analyzed.
The prevalence of NAFLD in the CKD group was higher than that in the non-CKD group (63.5% vs 50.5%,
< 0.001). The SCr and UACR levels in the NAFLD group were higher than those in the non-NAFLD group (both
<0.05). The prevalence of NAFLD in the U3 group (75.6%) was higher than that in the U1 (50.5%,
< 0.05) and U2 (60.1%,
< 0.05) groups, and the prevalence of NAFLD in the U2 group (60.1%) was higher than that in the U1 group (50.5%,
< 0.05). The risk of NAFLD in the U3 group was higher than that in the U2 group (odds ratio [OR] = 3.032 and 1.473). Despite adjusting the parameters further, the NAFLD risk in the U3 group remained higher than that in the U2 group (OR = 1.660 and 2.342).
The risk of NAFLD in patients with T2DM is closely related to CKD.
The risk of NAFLD in patients with T2DM is closely related to CKD.
To study the role of miR-138 on the repair of diabetic foot ulcer (DFU) and further to explore its possible mechanism.
miR-138 inhibitor, IGF-1, LY294002 were used in DFU rat mode, and the mRNA expression of miR-138 was detected. HE staining was used to observe the histological changes of skin ulcer in rats. The level of inflammation, wound healing, and blood vessel formation-related factors were detected by ELISA and immunohistochemical. The expression of VEGF and PI3K/AKT pathway-related proteins were detected by Western blot. To further determine the underlying mechanism of miR-138 in the repair of DFU, telomerase inhibitor BIBR-1232 was used in HUVECs. Dual-luciferase assay was used to determine the target relationship between miR-138 and hTERT. CCK-8, transwell, and tube formation assays were conducted to observe the biological behavior of HUVECs. Inflammatory cytokines and PI3K/AKT pathway-related proteins were also measured by ELISA and Western blot.
The mRNA expression of miR-138 in DFU rat was increased and ulcer of diabetic foot rats was improved after silencing miR-138. selleck products The results of cellular bioactivity in vitro experiment were consistent with that in vivo. Meanwhile, after silencing miR-138, the level of inflammatory cytokines was decreased, while the level of anti-inflammatory and healing factors was increased in vivo and vitro. Moreover, the ratios of p-PI3K/PI3K and p-AKT/AKT were upregulated after treated with miR-138 inhibitor and miR-138 was negatively regulated the expression of hTERT. However, the inhibitory effect on inflammatory response and the promotion effect on wound healing of miR-138 inhibitor were reversed by LY294002 and BIBR-1232.
Down-regulation of miR-138 could alleviate inflammatory response and promote wound healing in DFU rats by activating PI3K/AKT pathway and hTERT.
Down-regulation of miR-138 could alleviate inflammatory response and promote wound healing in DFU rats by activating PI3K/AKT pathway and hTERT.
