Osobliwości kliniczne mastocytozy u dzieci

Introduction. Mastocytosis is a group of rare diseases characterized byabnormal growth of mast cells (MC) in the skin, bone marrow, liver,spleen and lymph nodes. In children cutaneous mastocytosis (CM) isthe predominant form.Objective. Presentation of four atypical cases of childhood CM with variousclinical manifestations to demonstrate the wide spectrum of skinlesions and MC mediator-related symptoms and discuss indications toperform diagnostic procedures for systemic mastocytosis (SM) in children.Case reports. We describe the clinical presentation and diagnostic proceduresperformed in 3 girls and 1 boy aged from 9 month to 6 years.Disseminated CM with bullous lesions, large mastocytoma, plaquetype and nodular type of CM were diagnosed. Elevated serum tryptase level and severe MC mediator-related symptoms were present only ina girl with extensive skin involvement and periodic blistering. SM oranaphylactic shock in the case history were not present in our children.Conclusions. Atypical clinical manifestations in infants and small childrenmake the diagnosis of CM difficult. Initial diagnosis of SM,follow-up and prevention of anaphylaxis are recommended in paediatricmastocytosis cases

Impact of Activation of <i>EGFL7</i> within Microenvironment of High Grade Ovarian Serous Carcinoma on Infiltration of CD4+ and CD8+ Lymphocytes

Background: It has been demonstrated that Egfl7 promotes tumor cell escape from immunity by downregulating the activation of tumor blood vessels. Aim: to analyze mRNA expression of EGFL7 within the tumor microenvironment of high-grade ovarian serous carcinoma and its association with a number of intraepithelial CD4+/CD8+ lymphocytes and ICAM-1 expression. Methods: qPCR analysis of EGFL7 mRNA in cancer cells and adjacent stromal endothelium microdissected from formalin-fixed paraffin-embedded tumors of 59 high-grade ovarian serous carcinoma patients, was performed. Infiltration of intraepithelial lymphocytes (CD4+/CD8+) and expression of ICAM-1 were evaluated by immunohistochemistry and compared between tumors with different statuses of EGFL7 expression. Results: EGFL7 was expressed in cancer cells (9/59, 15.25%), endothelium (8/59, 13.56%), or both cancer cells and adjacent endothelium (4/59, 6.78%). ICAM-1 was expressed on cancer cells (47/59, 79.66%), stromal endothelium (46/59, 77.97%), or both epithelium and endothelium (40 of 59, 67.8%). EGFL7-positivity of cancer cells and endothelium was associated with lower intraepithelial inflow of CD4+ (p = 0.022 and p = 0.029, respectively) and CD8+ lymphocytes (p = 0.004 and p = 0.031, respectively) but impact neither epithelial nor endothelial ICAM-1 expression (p = 0.098 and p = 0.119, respectively). The patients’ median follow-up was 23.83 months (range 1.07–78.07). Lack of prognostic significance of EGFL7-status and ICAM-1 expression was notified. Conclusion: EGFL7 is activated in the cancer cells as frequently as in the endothelium of human high-grade ovarian serous carcinoma. Activation of EGFL7 in cancer cells and/or endothelial cells could negatively impact diapedesis regardless of localization

Lesion- and Patient-Related Variables May Provide Additional Clues during Dermoscopic Assessment of Blue Nevi—A Retrospective Cohort Study

Background: Little is known about the correlation between lesion- and patient-related variables and the dermoscopic features of blue nevi. The aim of the study was dermoscopic analysis of blue nevi in association with patient- and lesion-related variables, with a special interest in structures whose prevalence has not been previously reported. Methods: This was a double-center, retrospective study, which included the analysis of histopathologically confirmed blue nevi (n = 93). Results: There was no difference in the frequency of the observed dermoscopic features according to patients’ gender and age. Pink structureless areas were more common in patients with I/II Fitzpatrick skin phototypes as well as in the patients with photodamaged skin, while blue prominent skin markings over brownish/blue-gray background occurred exclusively in patients with phototype III. Structures of previously unreported prevalence in blue nevi were skin-colored circles (present in 32.3%), gray circles (2.2%), follicular ostia with no pigmentation (18.4%; present exclusively on the face), blue skin markings over brownish background (present in 18.2%; detected only on the limbs) and dark brown polygons (one lesion located on the lower extremity). Conclusion: Dermoscopic presentation of blue nevi may vary according to the patient’s phototype and lesion size/localization rather than gender and age

Lesion- and Patient-Related Variables May Provide Additional Clues during Dermoscopic Assessment of Blue Nevi&mdash;A Retrospective Cohort Study

Background: Little is known about the correlation between lesion- and patient-related variables and the dermoscopic features of blue nevi. The aim of the study was dermoscopic analysis of blue nevi in association with patient- and lesion-related variables, with a special interest in structures whose prevalence has not been previously reported. Methods: This was a double-center, retrospective study, which included the analysis of histopathologically confirmed blue nevi (n = 93). Results: There was no difference in the frequency of the observed dermoscopic features according to patients&rsquo; gender and age. Pink structureless areas were more common in patients with I/II Fitzpatrick skin phototypes as well as in the patients with photodamaged skin, while blue prominent skin markings over brownish/blue-gray background occurred exclusively in patients with phototype III. Structures of previously unreported prevalence in blue nevi were skin-colored circles (present in 32.3%), gray circles (2.2%), follicular ostia with no pigmentation (18.4%; present exclusively on the face), blue skin markings over brownish background (present in 18.2%; detected only on the limbs) and dark brown polygons (one lesion located on the lower extremity). Conclusion: Dermoscopic presentation of blue nevi may vary according to the patient&rsquo;s phototype and lesion size/localization rather than gender and age

Prediction Model for Bronchopulmonary Dysplasia in Preterm Newborns

OBJECTIVE: To develop a multifactorial model that allows the prediction of bronchopulmonary dysplasia (BPD) in preterm newborns. MATERIALS AND METHODS: A single-center retrospective study of infants born below 32 + 0 weeks gestational age. We created a receiver operating characteristic curve to assess the multifactorial BPD risk and calculate the BPD risk accuracy using the area under the curve (AUC). BPD risk was categorized using a multifactorial predictive model based on the weight of the evidence. RESULTS: Of the 278 analyzed preterm newborns, 127 (46%) developed BPD. The significant risk factors for BPD in the multivariate analysis were gestational age, number of red blood cell concentrate transfusions, number of surfactant administrations, and hemodynamically significant patent ductus arteriosus. The combination of these factors determined the risk of developing BPD, with an AUC value of 0.932. A multifactorial predictive model based on these factors, weighted by their odds ratios, identified four categories of newborns with mean BPD risks of 9%, 59%, 82%, and 100%. CONCLUSION: A multifactorial model based on easily available clinical factors can predict BPD risk in preterm newborns and inform potential preventive measures