Efficacy of very low-calorie ketogenic diet with the Pronokal® method in obese women with polycystic ovary syndrome: a 16-week randomized controlled trial.

Objective: The aim of this study isto assess the efficacy of a very low-calorie ketogenic diet (VLCKD) method vs a Mediterranean low-calorie diet (LCD) in obese polycystic ovary syndrome (PCOS) women of a reproductive age. Design: Randomized controlled open-label trial was performed in this study. The treatment period was 16 weeks; VLCKD for 8 weeks then LCD for 8 weeks, according to the Pronokal® method (experimental group; n = 15) vs Mediterranean LCD for 16 weeks (control group; n = 15). Ovulation monitoring was carried out at baseline and after 16 weeks, while a clinical exam, bioelectrical impedance analysis (BIA), anthropometry, and biochemical analyses were performed at baseline, at week 8, and at week 16. Results: BMI decreased significantly in both groups and to a major extent in the experimental group (-13.7% vs -5.1%, P = 0.0003). Significant differences between the experimental and the control groups were also observed in the reduction of waist circumference (-11.4% vs -2.9%), BIA-measured body fat (-24.0% vs -8.1%), and free testosterone (-30.4% vs -12.6%) after 16 weeks (P = 0.0008, P = 0.0176, and P = 0.0009, respectively). Homeostatic model assessment for insulin resistance significantly decreased only in the experimental group (P = 0.0238) but without significant differences with respect to the control group (-23% vs -13.2%, P > 0.05). At baseline, 38.5% of participants in the experimental group and 14.3% of participants in the control group had ovulation, which increased to 84.6% (P = 0.031) and 35.7% (P > 0.05) at the end of the study, respectively. Conclusion: In obese PCOS patients, 16 weeks of VLCKD protocol with the Pronokal® method was more effective than Mediterranean LCD in reducing total and visceral fat, and in ameliorating hyperandrogenism and ovulatory dysfunction. Significance statements: To the best of our knowledge, this is the first randomized controlled trial on the use of the VLCKD method in obese PCOS. It demonstrates the superiority of VLCKD with respect to Mediterranean LCD in reducing BMI with an almost selective reduction of fat mass and a unique effect of VLCKD in reducing visceral adiposity, insulin resistance, and in increasing SHBG with a consequent reduction of free testosterone. Interestingly, this study also demonstrates the superiority of the VLCKD protocol in improving ovulation, whose occurrence increased by 46.1% in the group treated by the VLCKD method against a rise of 21.4% in the group treated by Mediterranean LCD. This study extends the therapeutic approach possibilities in obese PCOS women.

Trichoscopy-Derived Hairline Recession Equivalent in Monitoring Frontal Fibrosing Alopecia.

Introduction: Frontal fibrosing alopecia (FFA) is a relatively recently described scarring hair loss condition. Frontal hair recession is observed in a vast majority of patients; other scalp areas may be included. Assessment of hair loss progression in FFA remains challenging mainly due to difficulties in unambiguous determination of the hairline. Various patterns of scarring and subtle progression rate are among factors which make naked-eye observations of limited use. Methods: Trichoscopy of the frontal hairline with hair-to-hair matching was conducted in patients with FFA patients with disease progression and clinically stable hairline. Hair loss was assessed based on analysis of trichoscopy-derived follicular maps. A relative hair density loss was calculated, and the hairline recession equivalent (HRE) was proposed as a novel hair loss progression measure. Results: Two patterns of hair loss were observed: one with significant decrease of hair density within a width of 1 mm and one with diffuse loss within a width of 10 mm. Conclusion: The hair density profile may be a useful tool to characterize different disease progression patterns. The HRE is potentially a very accurate and sensitive parameter to quantify local hair loss progression in FFA.

Efficiency of Hair Detection in Hair-to-Hair Matched Trichoscopy.

Introduction: Precise evaluation of changes in hair count is crucial for monitoring progression of hair loss and the effects of treatment. The focus of this study is the comparison of the various examination and assessment techniques in terms of the precision of hair count change observed in trichoscopy images. Methods: Controlled hair extraction of the same scalp spot was used to simulate hair loss, and the different examination techniques were performed to detect this change. The investigators who performed the counting were blinded. Results: For trichoscopy images, the average error in determining the terminal hair count change (relative to total hair count) was 9 ± 1% for automatic assessment with manual correction and 0.4 ± 0.2% for hair-to-hair matched images. For phototrichogram, the automatic measurement results were found to deviate from truth on average by 12 ± 2%. The manually corrected hair count results were much closer to the truth with average deviation at the level of 7 ± 1%. The hair-to-hair matched results corresponded to approximately 0.6 ± 0.3% average discrepancy. Conclusion: Combination of manually corrected image processing, follicular mapping, and hair-to-hair matching appears to be the most precise way of evaluating the change in hair count over time. These novel techniques should be considered valuable, especially in research and clinical trials.

Intracardiac tumor as a rare manifestation of genetic syndromes-presentation of a family with Gorlin syndrome and a literature review.

Intracardiac tumors in children are relatively rare, but their clinical consequences may include severe outflow tract obstruction, embolism, cardiac insufficiency, or rhythm disturbances. In some cases, the tumor may constitute part of a genetic condition and prompt additional investigations, as well as a modification of therapeutic management. Herein, we present a molecularly confirmed familial case of Gorlin syndrome with an early cardiac tumor as a presenting sign. We provide detailed clinical characteristics of the affected individuals and a useful review of syndromic causes of pediatric cardiac tumors in clinical practice.

Follicular Map: A Novel Approach to Quantitative Trichoscopy.

BACKGROUND: Trichoscopy (hair and scalp dermoscopy) is gaining importance as an easy-to-use diagnostic and monitoring tool in various hair and scalp conditions. OBJECTIVE: The demand for a method to identify exactly the same site on scalp skin for subsequent examinations is growing constantly. METHODS: In total, 40,000 trichoscopic images of 70-fold magnification were analyzed. Graphical pattern recognition was used to identify hair shafts in trichoscopy images and to derive the coordinates of all follicular orifices. Determination of the relative positions of hair follicle orifices formed the Follicular Map (FMap). Processing and matching of FMaps involves conceptually simple but computationally complex numerical algorithms. RESULTS: FMaps were shown to be unique for specific locations on the scalp in each human individual. The follicular patterns were not affected by natural hair cycling processes and remained unchanged over the course of time. It has been tentatively verified that FMaps remain unaffected by noncicatricial alopecia. CONCLUSIONS: The concept of FMap presents a novel approach to qualitative trichoscopy, allowing precise evaluation of treatment efficiency, clinical trial quality control, validation of diagnostic tools, and potentially other applications still to be discovered. The FMap enables the location of hair follicles to be precisely measured and recorded.

The Trichoscopy Derived Sinclair Scale: Enhancing visual assessment through quantitative trichoscopy.

INTRODUCTION: The Sinclair Scale of hair midline density is commonly used in clinical dermatology practice as a tool to evaluate the severity of female pattern hair loss (synonym androgenetic alopecia, AGA) and to monitor patient response to treatment. The Sinclair Scale involves a visual evaluation of central hair parting line width, that is performed quickly and with no optical instrumentation. Another approach used to score severity of hair loss is quantitative trichoscopy. While quantitative trichoscopy is more accurate in terms of reproducibility and objectivity, it is more time-consuming. MATERIALS AND METHODS: Patients with different stages of AGA were evaluated using both the Sinclair Scale and quantitative trichoscopy. A correlation analysis was performed between the Sinclair Scale and different parameters derived from statistical analysis of trichoscopy images. A novel parameter, cumulative hair thickness density was introduced. RESULTS: Very good correlation was observed between Sinclair Scale and the cumulative hair thickness density. The quality of correlation is sufficient to estimate Sinclair Scale from cumulative hair thickness density. A formula to calculate the 'Trichoscopy Derived Sinclair Scale' from trichoscopy statistics was derived. DISCUSSION: We propose the term 'Trichoscopy Derived Sinclair Scale' to describe the hair midline density as derived from trichoscopy and to differentiate this assessment of midline hair density from the traditional one based on visual inspection alone.

[Psychiatric comorbidities of psoriasis: pilot study]. / Wspólwystepowanie zaburzen psychicznych w luszczycy: doniesienie wstepne*.

AIM: There are some reports about the relatively frequent occurrence of depression in the case of psoriasis and some hypothesis about interactions between those two diseases. However there are no studies verifying those hypothesis based on reliable structured psychiatric interviews according to the current diagnostic criteria of mental disorders. The aim of the study was to compare the frequency of depression in patients suffering from psoriasis and from other chronic skin diseases with the use of a structured questionnaire for the diagnosis of the main mental disorders Mini International Neuropsychiatric Interview. METHOD: 32 consecutive outpatients (9 males and 23 females), age M = 43.9 with psoriasis were examined by a team of dermatologists, psychiatrists and a psychologist using a standard set of methods. In addition, 32 patients with other chronically occurring skin diseases, including 11 males and 21 females, age M = 31.6, were also examined and formed the control group. RESULTS; The point prevalence of mental disorders was significantly higher in the psoriatic group--20 (62.5%) versus 5 (15.62%) in the control group (p < 0.001). In all of the cases, affective disorders were diagnosed. Mild anxiety disorders were additionally found in 10 psoriatic patients (31.25%) and in 2 controls (6.25%) (p < 0.001). The level of depression was much higher in the study group than in the control group, both in the Beck Depression Inventory (M = 15.28, SD = 8.72 versus M = 8.13, SD = 6.31, p < 0.001 respectively) and in the Hamilton Rating Scale for Depression (M = 9.63, SD = 5.71 versus M = 5.09, SD = 4.26, p < 0.001 respectively). The neurotic symptoms measured by the Symptoms Check List-II by Aleksandrowicz were also significantly more intense in the psoriatic group (M = 54.37; SD = 40.99) than in the control group (M = 35.28; SD 23.96) (F = 8.55, p < 0.005). CONCLUSION: The results imply the need for the careful examination of the mental state of patients with psoriasis in order to offer and provide treatment of any concomitant psychiatric conditions.

[Current treatment of systemic sclerosis. Part I. Immunosuppressive treatment]. / Wspólczesne metody leczenia twardziny ukladowej. Czsc I. Leczenie immunosupresyjne.

Systemic sclerosis (scleroderma) has a complex pathogenesis. Thus, management of systemic sclerosis remains a challenge. Currently, treatment in systemic sclerosis is targeted at reducing 1) abnormal immune activation, 2) microvascular abnormalities and 3) fibrosis. Therapy should be adjusted individually, depending on disease subtype and activity. Immunosuppressive agents, such as cyclophosphamide or mycophenolate mofetil and occasionally methotrexate or cyclosporine A are indicated in rapidly progressing systemic sclerosis. Low or moderate doses of corticosteroids may be used in patients with musculoskeletal involvement. Reports of effective biological therapy with rituximab or infliximab are available. In cases of rapidly progressing systemic sclerosis high dose immunosuppressive or cytostatic agents with subsequent autologous bone marrow transplantation may be indicated. Novel therapies have the potential of significantly modifying disease process and improving overall clinical outcome in patients with systemic sclerosis.

[Current treatment of systemic sclerosis. Part II. Vascular and antifibrotic treatment]. / Wspólczesne metody leczenia twardziny ukladowej. Czesc II. Leki wplywajace na zaburzenia krazenia obwodowego i wlóknienie.

Microvascular abnormalities and fibrosis are important targets of therapy in systemic sclerosis (scleroderma). Calcium channel blockers, ACE inhibitors, sartans, phosphodiesterase-5 inhibitors and serotonin re-uptake blockers are used for Raynaud's phenomenon. Intravenous prostanoids (alprostadil, iloprost, epoprostenol, treprostinil) and endothelin receptor antagonists (bosentan, sitaxsentan, ambrisentan) show efficacy in treatment of pulmonary hypertension and distal ischemia. Successful treatment of digital ulcers secondary to systemic sclerosis was possible with sildenafil and bosentan. A platelet gel is currently in clinical trials for scleroderma-related digital ulcers. Several drugs, which directly reduce excessive production of collagens and other connective tissue proteins have been applied in systemic sclerosis. These include interferon gamma, d-penicillamine, kolchichicine, calcitriol, and imanitib. However, so far, strategies to control fibrosis by directly reducing excessive connective tissue production have been disappointing in controlled studies.

Mycoplasma pneumoniae-induced pneumonia with Stevens-Johnson syndrome of acute atypical course.

We present a case of Stevens-Johnson syndrome of acute clinical course with massive occupation of the mucus membranes of the respiratory system, oral cavity, genitals and conjunctiva in a patient with pneumonia. A probable etiological factor was infection with Mycoplasma pneumoniae, however clarithromycin could be another potential inducing factor.

Comma hairs: a dermatoscopic marker for tinea capitis: a rapid diagnostic method.

BACKGROUND: Dermatoscopy and videodermatoscopy have been used for several years in the diagnosis of skin disorders. OBJECTIVE: We sought to determine whether tinea capitis (TC) shows characteristic videodermatoscopy features that may facilitate its differentiation from alopecia areata (AA). METHODS: Two patients with TC caused by Microsporum canis, confirmed by mycological culture and fluorescence under Wood lamp, were examined with videodermatoscopy and results were compared with videodermatoscopy results of 12 patients with AA. RESULTS: The distinctive and most prominent feature of TC was presence of commalike structures (comma hairs). These were accompanied by broken and dystrophic hairs. Videodermatoscopy features of AA included exclamation mark hairs, vellus and dystrophic/cadaverized hairs, and yellow dots corresponding to hyperkeratotic hair follicle plugs. LIMITATIONS: This study was conducted on two patients, both with M canis infection. CONCLUSIONS: Comma hairs were observed as a distinctive videodermatoscopy feature of M canis-induced TC. This finding was not observed in AA, typified generally by exclamation mark hairs.

Cylindroma transforming into basal cell carcinoma in a patient with Brooke-Spiegler syndrome.

BACKGROUND: Brooke-Spiegler syndrome is a rare condition with a predisposition to develop cutaneous adnexal neoplasms, especially cylindromas, trichoepitheliomas and spiradenomas. Malignant transformation of cylindromas is rare. In such cases usually cylindrocarcinomas develop within these lesions. We present an unusual case of basal cell carcinoma developing within a preexisting cylindroma. MAIN OBSERVATIONS: 58-year-old woman with a 30-year history of multiple dermal cylindromas extensively involving her scalp was referred for dermatological treatment. The patient reported that one of the long-lasting lesions, 5.5 cm in size, ulcerated within the foregoing few weeks. Histopathology confirmed cylindromas and basal cell carcinoma within the ulcerating tumor. Surgical excision of largest cylindroma tumors led to cosmetic and functional improvement. Magnetic resonance and computed tomography showed tumor infiltration into the skull lamina externa. Metastases were excluded by chest radiography and abdominal ultrasound examination. CONCLUSION: Patients with Brooke-Spiegler syndrome should be followed-up for malignant transformation of skin tumors to prevent deep penetration and possible metastases.

Prolactin synthesis by lymphocytes from patients with systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs. It has been demonstrated that serum prolactin levels are increased in patients with various connective tissue diseases. The aim of this study was to investigate the possible source of excessive prolactin synthesis in SSc and its effects on immune cells. The study group consisted of 52 patients with diffuse SSc (44 women and eight men) and 52 age and sex matched healthy controls. The methods used were: ELISA and indirect immunofluorescence. Our results show significantly elevated level of prolactin in male and female patients with SSc and increased prolactin production by SSc lymphocytes as compared to healthy donors X lymphocytes (25.4+/-11.0 vs. 13.4+/-5.0 absorbance units). Patients X and healthy controls X lymphocytes, showed equal presence of prolactin receptors. Soluble interleukin 2 receptor (CD25) concentration, was significantly higher in supernatants of prolactin stimulated lymphocytes, as compared to non-stimulated lymphocytes. We conclude that lymphocytes might contribute to elevated prolactin levels in patients with SSc and that these cells themselves may be sensitive to prolactin stimulation. Therefore, a pharmacologic attempt to lower prolactin levels in patients with SSc could proof beneficial.