The Need of Differential Diagnosis Between Vulvar Lichen Sclerosus and Autoimmune Dermatoses in Adolescent Girls.

INTRODUCTION: Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which can manifest in prepubertal or adolescent patients. The prevailing theories point to autoimmune and genetic factors. The primary symptoms of VLS typically include vulvar itching, discomfort, dysuria, and constipation. Physical examination often reveals a characteristic figure 8 pattern, involving the labia minora, clitoral hood, and perianal region. However, these symptoms and the age of onset are nonspecific and require differentiation from autoimmune dermatoses such as bullous diseases, pemphigus diseases, epidermolysis bullosa acquisita, and dermatitis herpetiformis. We performed this study to distinguish VLS from autoimmune dermatoses, and in doing so, uncover the underlying causes of chronic vulvar changes. This knowledge will enable healthcare providers to offer appropriate medical care to affected patients. METHODS: The study was conducted between July 2020 and February 2021, with a sample of 55 girls aged 2-18 years who did not have any systemic diseases. The study group was composed of 20 girls previously diagnosed with vulvar lichen sclerosus, while the control group included 35 girls without VLS. Questionnaires regarding the medical history of the children were completed by their legal guardians. Blood samples were collected and analyzed biochemically to assess human immunoglobulin A (IgA), IgG, and IgM antibodies against various substrates, including the desmosome of stratum spinosum, basement membrane zone, desmoglein 1 (DSG1), desmoglein 3 (DSG3), BP180-NC16A-4X, BP230gC, pemphigoid antigen, collagen type VII NC1, transitional epithelium, gliadin (GAF-3X), endomysium (EMA), and cellular nucleus (ANA). RESULTS: The analysis of the study group revealed that the most commonly observed signs and symptoms included: itching, soreness, burning sensations, and excoriation, as well as erythema or/and pallor of the skin and perineal mucosa. Among the assessed antibodies, only anti-GAF3x antibodies and ANA antibodies were detected. However, the results did not reach statistical significance (p > 0.5).

COVID 19 vaccination as a trigger of acute genital ulcers in an immunocompromised adolescent-case study and literature review.

Acute genital ulcers can affect females of all ages. In children, they often appear as an emergency and remain a diagnostic challenge for pediatricians, gynecologists and dermatologists. Prompt diagnosis and identification of disease- related factors help to implement appropriate treatment. Firstly, it is crucial to properly compile the past medical history of the patient. Past infectious, autoimmune, malignant or traumatic conditions, as well as vaccinations may contribute to the occurrence of acute genital ulcers. Moreover, new infectious agents, such as severe acute respiratory syndrome coronavirus 2 and vaccinations against Coronavirus disease of 2019, may play a significant role in the development of atypical clinical symptoms. Here we present a case of a 12-year-old girl with acute genital ulcers. Additional symptoms accompanying the ulcer included: abdominal pain, nausea, vomiting, dysuria, vulvar pain and fever. Blood test showed leukocytosis, especially neutrophilia and monocytosis and increased levels of c-reactive protein and procalcitonin. Serological tests for the most common infections were negative. Moreover, the patient had a history of autoimmune diseases. She had periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, and IgA vasculitis, also known as Henoch-Schönlein purpura in her past medical history. Additionally, she was vaccinated against SARS-CoV-2 shortly before the lesions appeared.

Breastfeeding and fatty liver - is there any association?

The campaign to promote the natural feeding of infants, at least for the first six months of life, conducted over recent years has deep justification from a medical point of view. Numerous gynecological and pediatric societies around the world recommend breastfeeding as the most appropriate way of feeding infants. It has been proven that the benefits of this type of nutrition go beyond nutritional aspects, proper growth and development. The list of long-term metabolic benefits, which include reducing the incidence of obesity, allergies, infections and diabetes, is constantly growing. It has been shown that the method of feeding infants using various mechanisms may influence the tendency of the liver to accumulate fatty compounds and develop fatty liver disease with its metabolic consequences leading to liver failure, cirrhosis and hepatocellular carcinoma. This is an important discovery due to the growing obesity epidemic in adults and children. Metabolic dysfunction - associated fatty liver disease (MAFLD) has become the most common cause of chronic liver disease, affecting 25% of the global population. The results of studies conducted in recent years have shown the protective effect of breastfeeding on the risk of developing MAFLD later in life in both children and breastfeeding women. New scientific reports provide the basis for qualifying breastfeeding as a modifiable risk factor for MAFLD.

Laparoscopic Uterine Preservation and Vaginal Reconstruction in a 13-Year-Old Girl with Uterus Didelphys, Bilateral Vesicovaginal Fistulas, and Transverse Vaginal Septum.

BACKGROUND: Complex female genital tract malformations account for 1.2% of all female genitourinary malformations. Although exceedingly rare, they can cause severe gynecologic symptoms in young women and lead to fertility problems. CASE: We present the case of a 13-year-old girl with primary amenorrhea referred for cyclic abdominal lower pain and menouria. Detailed diagnostics revealed uterus didelphys, transverse vaginal septum, and bilateral vesicovaginal fistulas. Laparoscopic left hemi-hysterectomy and salpingectomy were performed. The vesicovaginal fistula on the right side was excised, and the proximal vagina was anastomosed with the distal dimple. Since the operation, the patient has been pain-free and menstruating regularly from the right uterus. SUMMARY AND CONCLUSION: Preservation of the uterus should be considered in any case of complex female genital tract malformation and, as successful laparoscopic treatment advocates, a minimally invasive approach is feasible.

The Potential Coexistence of Autoimmune Thyroid Diseases and Pediatric Vulvar Lichen sclerosus.

INTRODUCTION: Vulvar lichen sclerosus (VLS), a chronic inflammatory skin disorder, often coexists with autoimmune thyroid disease (AITD). VLS presents with subtle symptoms including vulvar itching and discomfort. Clinically, a "Figure 8" pattern involving the labia minora, clitoral hood, and perianal region is often observed. It is prevalent both in pre-pubertal girls and women aged 40-60, and the link between VLS and AITD remains unclear, with proposed causes including autoimmune, hormonal or genetic factors, and environmental triggers. This study addresses the lack of research on the association in children, aiming to investigate the largest group of underage girls to date. AIM: This study aimed to investigate the coexistence of thyroid autoimmune diseases in girls diagnosed with vulvar lichen sclerosus (VLS) and to assess the presence of antibodies for specific thyroid autoimmune diseases. MATERIALS AND METHODS: Our study was conducted from July 2020 to February 2021, involving a sample of 55 girls aged 2-18 years old, all free from systemic diseases. The study group comprised 20 girls previously diagnosed with vulvar lichen sclerosus (VLS), while the control group included 35 girls without VLS. Legal guardians completed questionnaires detailing the medical history of their children. Blood samples were collected from all participants and subjected to biochemical analysis. The presence of human IgG antibodies against thyroid peroxidase and IgG antibodies against thyroglobulin was assessed using the immunoenzymatic method with commercially available ELISA kits. RESULTS: In the study group, common symptoms included itching, soreness, burning sensation, excoriation, and erythema or pallor of the skin and perineal mucosa. An evaluation of anti-thyroglobulin and anti-thyroid peroxidase antibodies revealed no statistical significance between the study and control groups (anti-TG p = 0.379, anti-TPO p = 0.96). Family history of autoimmune diseases showed no significant correlation with anti-thyroid antibodies in girls. Although no significant relation between VLS occurrence and antibody levels was found, it emphasizes the need for multidisciplinary medical care. Further research with larger patient groups is necessary.

The effect of gonadoliberin analog treatment in precocious puberty on polycystic ovarian syndrome prevalence in adulthood.

Precocious puberty is diagnosed when pubertal characteristics appear before the age of 8 years in females. The most common form is gonadotropin-dependent, called axial. The primary method of treatment is administration of gonadotrophin-releasing hormone analogues (GnRHa). The aim of the study was to verify hypothesis that GnRHa therapy in the childhood may be of additive risk factor for polycystic ovary syndrome (PCOS) in adulthood. Material and Methods: The study group consists of 24 women (median age 22 88 years, median BMI 23.5) treated with GnRHa for central precocious puberty in childhood. The control group includes 40 women (median age 23 years, median BMI 25.6) diagnosed with isolated premature thelarche and not using GnRHa in the childhood. Anthropometric measurements, ultrasound examination of minor pelvis and hormonal profile were performed. PCOS diagnosis was based on Rotterdam criteria. Results: The study confirmed a higher prevalence of PCOS in the study group (50%) than in the control group (10%); p=0.0006. Significant, linear correlation between free testosterone levels and ovarian size was found in the study group (R=0.45 p= 0.03). Conclusions: GnRHa therapy during childhood may have a potential influence on incidence of PCOS in the adulthood. Therefore, in this group of patients long-term follow-up focused on screening for PCOS would seem beneficial.

Alpha-fetoprotein level in fetuses, infants, and children with ovarian masses: a literature review.

Alpha-fetoprotein (AFP) is a serum protein highly produced during the fetal period. It is also known as a biomarker of various pathologies. Commonly, tumors requiring diagnosis and monitoring through AFP determination appear during the first year of life, with poorer outcomes when presenting in fetal life. Due to advancements in imaging technology, the detectability of ovarian masses in infants is higher. However, the use of AFP as a biomarker could improve diagnosis in cases when imaging and histological examinations are not sensitive enough to detect tumors. From the outcome of our investigation, it is possible to conclude that there is evidence of an association between increased AFP levels and ovarian masses. However, previous studies have presented contradictory and unverified results, with the authors emphasizing that future research is needed. In this article, an analysis of the available literature on AFP as a biomarker of ovarian masses in children was performed. Two types of literature were reviewed: guidance and published studies (clinical trials, reviews, and systematic reviews). We searched the Embase, PubMed, ScienceDirect, and Web of Science databases to collect essential data.

Congenital malformations of the female genital organs.

Congenital malformations of the female genital organs are rare anomalies and their incidence is estimated to be up to 7% in the general population. Müllerian ducts abnormalities are one of the causes of infertility and occur in approximately 16% of women with recurrent miscarriages. Sex development disorders are diagnosed at different stages of the patient's life depending on the accompanying ailments. Alarming signs of genital malformations include primary amenorrhea or dysmenorrhea, dyspareunia, and periodic abdominal pain.

Beckwith-Wiedemann Syndrome in Newborn of Mother with HELLP Syndrome/Preeclampsia: An Analysis of Literature and Case Report with Fetal Growth Restriction and Absence of CDKN1C Typical Pathogenic Genetic Variation.

Beckwith-Wiedemann Syndrome (BWS) is an imprinting disorder, which manifests by overgrowth and predisposition to embryonal tumors. The evidence on the relationship between maternal complications such as HELLP (hemolysis, elevated liver enzymes, and low platelet count) and preeclampsia and the development of BWS in offspring is scarce. A comprehensive clinical evaluation, with genetic testing focused on screening for mutations in the CDKN1C gene, which is commonly associated with BWS, was conducted in a newborn diagnosed with BWS born to a mother with a history of preeclampsia and HELLP syndrome. The case study revealed typical clinical manifestations of BWS in the newborn, including hemihyperplasia, macroglossia, midfacial hypoplasia, omphalocele, and hypoglycemia. Surprisingly, the infant also exhibited fetal growth restriction, a finding less commonly observed in BWS cases. Genetic analysis, however, showed no mutations in the CDKN1C gene, which contrasts with the majority of BWS cases. This case report highlights the complex nature of BWS and its potential association with maternal complications such as preeclampsia and HELLP syndrome. The atypical presence of fetal growth restriction in the newborn and the absence of CDKN1C gene mutations have not been reported to date in BWS.

Does body mass index affect anti-mullerian hormone levels in girls and adolescents?

Anti-mullerian hormone (AMH) is a dimeric glycoprotein which belongs to the transforming growth factor-beta superfamily. In women, it is produced by granulosa cells in pre-antral and small antral follicles. In recent years, there has been a continuous increase in obesity among children and adolescents. There are few studies that present AMH concentrations in premenarcheal and early postmenarcheal girls. The purpose of this work is to assess whether AMH levels were associated with body mass index (BMI) in adolescent girls before and after menarche. The study was performed at the Pediatric Endocrinology Department and Outpatient Clinic at Upper Silesian Child Health Center. 82 girls were enrolled to the study. Body mass index seems not to affect the AMH levels in adolescents, however a special attention must be given when interpreting AMH levels in girls with irregular menstrual cycles and observed for PCOS.

The relationship between the level of vitamin D, leptin and FGF23 in girls and young women with polycystic ovary syndrome.

Polycystic ovary syndrome is an endocrinopathy that mainly affects adolescent girls and young women of childbearing age. In girls, the presence of clinical and biochemical symptoms of hyperandrogenism should be particularly considered. The role of vitamin D deficiency in insulin resistance, inflammation, dyslipidemia, and obesity, i.e. in diseases associated with PCOS, has been investigated, which may suggest its involvement in the pathophysiology of the syndrome. Leptin has been shown to stimulate the formation of FGF23 in bones. There is a relationship between the incidence of dyslipidemia, adipose tissue mass and the concentration of fibroblast growth factor 23. The main aim of the presented research project is to assess the concentration of vitamin D, calcium, and selected hormones as well as the concentration of adipokines (leptin) in girls diagnosed with polycystic ovary syndrome. Materials and methods: The study included a population of 85 girls and young women aged 14 to 22 years. The study group included 37 girls who were diagnosed with polycystic ovary syndrome according to the modified Rotterdam's criteria. The control group consisted of 48 completely healthy girls. In the first stage of the study participants were required to answer background questions. Next, anthropometric measurements were performed. The laboratory tests assessed: leptin, FGF23, FSH, SHGB, total testosterone, DHEA-S, 25-OH-D3, PTH, calcium, androstadiene, AMH, glucose, insulin. Results: The vitamin D level in the group with polycystic ovary syndrome was lower than in the control group, but there was no statistically significant difference. The level of anti-Müllerian hormone was significantly higher in the group of girls diagnosed with PCOS compared to the control group. Statistically significant differences between both groups were also noted in the HOMA-IR value. The concentration of calcium, parathyroid hormone, FGF23 and leptin in the study and control groups showed no statistically significant difference. Conclusions: In the studied group of girls with PCOS, no correlation between the level of vitamin D and selected parameters such as: AMH leptin, HOMA-IR and FGF23 was confirmed. On this basis, it can be assumed that additional vitamin D supplementation would not reduce the symptoms of polycystic ovary syndrome.

Anti-Mullerian hormone levels in girls and adolescents.

Anti-Mullerian hormone (AMH) is a homodimeric glycoprotein which belongs to the TGF-beta superfamily of growth and differentiation factors. There are few studies that present AMH concentrations in premenarcheal and early postmenarcheal girls. The aim of this study is to evaluate AMH levels in girls. A serum AMH increase of 1 pmol/L is related with a higher possibility of menarche occurrence. AMH levels are significantly higher in postmenarcheal girls than in prepubertal girls.

The Relationship between Postmenopausal Women's Self-Esteem and Physical Activity Level-A Survey Study from Poland.

INTRODUCTION: Physical inactivity has become one of the major public health and economic concerns in Western societies. The consequences of physical inactivity are associated with many physical problems, however, the influence of physical activity (PA) on psychological health is unclear. The aims of our study were to assess self-esteem and physical activity levels in postmenopausal women and to examine the association between physical activity levels and self-esteem in this group. MATERIAL AND METHODS: Survey research was conducted on postmenopausal women aged M = 58.81 ± 7.68 in women's health clinics in Silesia, Poland. The total number of participants was 131, and 18 were excluded. A questionnaire with socio-demographic data and other international questionnaires were used: International Physical Activity Questionnaire, Rosenberg Self-Esteem Scale and Beck Depression inventory. RESULTS: 78.76% of postmenopausal women were physically active. Mean value of MET-min/week was M = 1543.46 ± 1060.92. A total of 11.51% of women reported low self-esteem, with the mean total value of SES M = 31.79 ± 2.93. There was a lack of correlation between total IPAQ score and self-esteem (r = -0.241, p = 0.01). CONCLUSIONS: Postmenopausal women have mostly average self-esteem. They are generally active, and walking is the most common form of physical activity, however, a higher PA level does not influence self-esteem.