Publications by authors named "Joyce Teng"
Infantile hemangioma (IH) is the most common tumor in children and a paradigm for pathological vasculogenesis, angiogenesis, and regression. Propranolol, the mainstay treatment, inhibits IH vessel formation via a β-adrenergic receptor independent off-target effect of its R(+) enantiomer on the endothelial SRY box transcription factor 18 (SOX18). Transcriptomic profiling of patient-derived hemangioma stem cells (HemSC) uncovered the mevalonate pathway (MVP) as a target of R(+) propranolol.
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- The study assessed how accurately ChatGPT translates English patient education materials into Spanish, Mandarin, and Russian.
- ChatGPT performed well with Spanish and Russian translations, indicating it can be a useful tool for those languages.
- Mandarin translations need improvements, emphasizing the importance of reviewing AI-generated translations before using them in clinical settings.
Background: Recent advancements in dermatological therapeutics have highlighted the need for treatments that enhance skin regeneration and healing. Diamond-Augmented Zinc Oxide (ND-ZnO) technology combines zinc oxide with diamond particles in a unique core-shell structure, offering a multifaceted approach to overall skin health.
Aims: This study evaluates the efficacy of ND-ZnO in promoting human dermal fibroblast migration and growth, enhancing total collagen synthesis, and improving transdermal delivery of active ingredients as a daily comprehensive skin regeneration topical therapy.
Article Synopsis
- Infantile hemangioma (IH) is the most common tumor in children, primarily treated with propranolol, which affects blood vessel formation through a specific mechanism involving a protein called SOX18.
- Recent research identified the mevalonate pathway (MVP) as a target of propranolol, highlighting its role in the formation of hemangiomas and confirming this through studies on stem cells from hemangiomas.
- The study suggests that statins, which also target the MVP, could be repurposed to treat IH, revealing a new regulatory axis (SOX18-MVP) that may influence other conditions related to abnormal blood vessel growth.
Importance: Chronic skin disorders in children frequently are visible and can cause stigmatization. However, the extent of stigmatization from chronic skin disease and association with mental health needs further study.
Objective: To examine the extent of stigma, dependence on disease visibility and severity, and association with mental health and quality of life (QOL) in chronic pediatric skin disease.
Article Synopsis
- Targeting the mTOR pathway offers a new strategy for treating basal cell carcinoma (BCC), but its activation in human BCCs has been underexplored.
- The study evaluated mTOR pathway activation in both sporadic and syndromic BCCs, revealing increased levels of specific markers associated with mTOR in affected tissues.
- A case study showed that a pediatric patient with Gorlin syndrome experienced a reduction in BCC development after using topical sirolimus, suggesting its potential effectiveness and the need for further research on mTOR inhibitors in BCC treatment.
Article Synopsis
- Pachyonychia congenita (PC) is a rare genetic disorder characterized by abnormal skin cornification and is inherited in a dominant fashion.
- It has attracted significant research attention over the past 20 years, largely due to efforts from the Pachyonychia Congenita Project, a patient support organization.
- The article discusses ongoing research in PC, potential benefits for managing other diseases, and challenges facing these initiatives.
Background: In epidermolysis bullosa simplex (EBS), epithelial structural fragility results in blisters and erosions. Diacerein 1% ointment has been shown to reduce this blistering.
Objective: To evaluate the efficacy and safety of diacerein 1% ointment in the treatment of EBS.
Article Synopsis
- The Phase 2b CONTROL study analyzed the safety and effects of a new topical isotretinoin formulation, TMB-001, on laboratory values in patients with congenital ichthyosis, specifically focusing on X-linked and autosomal recessive types over 12 weeks.
- Participants aged 9 and older were randomly assigned to receive either TMB-001 or a vehicle, with laboratory tests conducted at the beginning and end of the study to monitor for adverse effects.
- Results indicated that while some minor laboratory abnormalities were noted, none were clinically significant, suggesting that TMB-001 may be a safer alternative to traditional oral retinoids for treating congenital ichthyosis.
Background: Emollients and keratolytics are frequently used to manage symptoms of congenital ichthyosis (CI). Systemic retinoid treatment is complicated by teratogenicity and dose-limiting adverse effects.
Objectives: This analysis from the randomized Phase IIb CONTROL study investigated the characteristics of participants who responded to treatment with TMB-001, a novel topical isotretinoin ointment formulation.
Background: In two severe congenital ichthyosis subtypes, autosomal recessive lamellar ichthyosis (ARCI-LI) and X-linked recessive ichthyosis (XLRI), cutaneous manifestations include widespread scaling. Approved topical treatment options are limited to emollients and keratolytics.
Aim: This analysis from the randomized phase IIb CONTROL study assessed whether the efficacy and safety of TMB-001, a novel topical isotretinoin ointment formulation, differed between ARCI-LI and XLRI subtypes.
Ichthyosis, cataracts, and motor delay in an infant: A case of Chanarin-Dorfman syndrome.
Pediatr Dermatol
November 2023
Chanarin-Dorfman syndrome (CDS) is a rare, autosomal recessive disorder of impaired triacylglycerol catabolism leading to cytoplasmic deposition of triglycerides in various cell types. We describe the case of an 8-month-old boy with cataracts, strabismus, motor delays, and an ichthyosiform rash since birth. Genetic testing revealed a pathogenic variant of the ABHD5 gene, suggestive of CDS, and further workup demonstrated hepatic steatosis and myopathy.
View Article and Find Full Text PDFBackground: Vascular anomalies that exhibit a slow velocity of blood flow, specifically venous malformations (VM), are associated with hypercoagulability. There is limited literature on the utilization of hormonal contraceptives (HCs) and the development of clotting events in female individuals diagnosed with VM.
Objective: We aimed to characterize HC utilization and associated odds of hypercoagulopathy in patients with VM of child-bearing age.
Article Synopsis
- Laser-assisted drug delivery (LADD) is a technique used for medical and cosmetic purposes, but there's a need for better guidelines to help clinicians use it safely and effectively.
- A comprehensive review of existing studies identified 54 relevant publications that informed new recommendations, which were developed through consensus among a panel of experts.
- Key findings include that LADD is generally safe for use on various skin types, effective for treating certain skin conditions, and that drug delivery can be optimized through specific techniques, although prophylactic antibiotics are only necessary in certain cases.
Genetic alterations in lymphatic development can lead to microcystic lymphatic malformations (micro LMs). LMs can have both microcystic and macrocytic components or be exclusively one or the other. LMs can result in serious, sometimes life-threatening, sequelae.
View Article and Find Full Text PDFBackground: Epidermolysis bullosa simplex (EBS) comprises a group of rare, blistering genodermatoses. Prior work has been limited by small sample sizes, and much remains unexplored about the disease burden and health-related quality of life (QOL) of patients with EBS. The aim of this study was to characterize the most common patient-reported clinical manifestations and the health-related impact of QOL in EBS, and to examine differences in disease burden by age.
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- Gorlin syndrome (GS) is a rare genetic condition that leads to an increased lifetime risk of basal cell carcinomas (BCCs), skeletal anomalies (SAs), and other tumors; understanding patient characteristics can help predict disease severity.
- Data from 248 GS patients was analyzed to investigate the relationship between clinical features and disease burden, revealing significant correlations between SAs and increased occurrences of BCCs and other neoplasms.
- Results indicate that patients with SAs face a higher risk of severe BCCs and other tumors, suggesting the need for more intensive and continuous medical monitoring for these individuals.
Importance: A comprehensive, user-friendly system to assess global ichthyosis disease burden is imperative to improving the care of patients with ichthyosis, identifying appropriate participants for clinical trials, and quantifying treatment outcomes. To our knowledge, there is currently no validated scale to objectively and systematically measure ichthyosis severity across the entire body.
Objective: To create and evaluate a comprehensive and user-friendly instrument to measure total body ichthyosis severity in adults and children.