Pediatric mastocytosis is a heterogeneous disease characterized by accumulation of mast cells in the skin and less frequently in other organs. Somatic or germline mutations in the KIT proto-oncogene are detected in most patients. Cutaneous mastocytosis is the most common form of the disease in children. In the majority of cases, skin lesions regress spontaneously around puberty. However, in few patients, mastocytosis is not a self-limiting disease, but persists into adulthood and can show signs of systemic involvement, especially when skin lesions are small-sized and monomorphic. Children with mastocytosis often suffer from mast cell mediator-related symptoms. Severe hypersensitivity reactions can also occur, mostly in patients with extensive skin lesions and blistering. In a substantial number of these cases, the triggering factor of anaphylaxis remains unidentified. Management of pediatric mastocytosis is mainly based on strict avoidance of triggers, treatment with H1 and H2 histamine receptor blockers, and equipment of patients and their families with epinephrine auto-injectors for use in severe anaphylactic reactions. Advanced systemic mastocytosis occurs occasionally. All children with mastocytosis require follow-up examinations. A bone marrow investigation is performed when advanced systemic mastocytosis is suspected and has an impact on therapy or when cutaneous disease persists into adulthood.

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Mastocytosis comprises a heterogeneous group of disorders characterized by clonal, neoplastic proliferation of mast cells accumulating in one or multiple organs. In the majority of cases skin involvement is the first clinical manifestation of the disease. Clinical work-up consists of a combination of morphological, immunohistochemical, flow cytometric immunophenotyping and molecular examination. Cutaneous mastocytosis predominates in children, whereas systemic mastocytosis is the most common form of the disease in adults. Therefore, different diagnostic algorithms have to be applied in adult patients and children with suspected mastocytosis. This comprehensive review presents currently defined variants of the disease and recommendations to facilitate diagnostic work-up in children and adults with suspected mastocytosis in daily clinical practice.

Mastocytosis is characterized by the pathological accumulation of mast cells (MC) in various organs. In these patients, MC may degranulate and thereby contribute to clinical symptoms, especially when a concomitant allergy is present. However, MC activation can not only be induced by high-affinity receptors for IgE, but also by anaphylatoxins, neuropeptides, IgG immune complexes, complement-components, drugs, products of bacteria or parasites, as well as physical factors such as heat, cold, vibration, stress, sun, or physical effort. Symptoms due to mediators released by activated MC may develop in adults suffering from systemic mastocytosis, but also evolve in children who usually have cutaneous mastocytosis (CM). Clinically, CM is otherwise characterized by typical brown, maculopapular skin lesions or mastocytoma associated with a positive Darier’s sign. Pruritus and flushing are common and blistering may also be recorded, especially in diffuse CM (DCM). Pediatric patients with mastocytosis may also have gastrointestinal, respiratory, and neurologic complaints. Although anaphylaxis is not a typical finding, pediatric patients with massive skin involvement and high tryptase levels have a relatively high risk to develop anaphylaxis. This paper reviews MC mediator-related symptoms and anaphylaxis in children with mastocytosis, with special emphasis on risk factors, triggers, and management.

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Neopterin is one of biochemical markers of immune activity, which seems to be useful in monitoring inflammatory diseases. Increased serum concentration of neopterin is observed in conditions with cell-mediated immune response involvement. In clinical practice, neopterin evaluation is applied in medical conditions such as: infectious diseases, oncology, rheumatology, transplantology, transfusiology, cardiology, neurology and many others. Neopterin can be detected in serum, urine, cerebrospinal fluid, synovial fluid, saliva, ascitic fluid, bile, pancreatic juice and gastric juice. In recently published studies, various non-specific indicators of inflammation such as neopterin, C-reactive protein, procalcitonin were compared and proved to be independently useful.

The aim of this study was to analyze the usefulness of fecal lactoferrin in the diagnosis and monitoring of inflammatory bowel disease (IBD) in children. The study included 52 children with IBD (24 with Crohn's disease and 28 with ulcerative colitis) aged between 0.92 and 18 years, and 41 IBD-free controls of similar age. Fecal concentration of lactoferrin was determined with a quantitative immunoenzymatic test. Fecal concentration of lactoferrin in children with IBD was significantly higher than in the controls. The cut-off value of fecal lactoferrin concentration optimally distinguishing between the children with IBD and the controls was identified as 13 μg/g. The sensitivity and specificity of this cut-off value equaled 80.7% and 92.7%, respectively, and its positive and negative prognostic values were 96.8% and 63.3%, respectively. Patients diagnosed with moderate Crohn's disease had significantly higher fecal concentrations of lactoferrin than children with the mild or inactive disease. Similarly, children with moderate ulcerative colitis showed significantly higher fecal concentrations of lactoferrin than individuals with the mild condition. No significant relationship was found between the fecal concentration of lactoferrin and the severity of endoscopic lesions. Patients with IBD and a positive result of fecal occult blood test were characterized by significantly higher concentrations of lactoferrin than the individuals with IBD and a negative result of this test. In conclusion, fecal concentration of lactoferrin seems to be a useful parameter for diagnosis and monitoring of IBD in children.

Background COVID-19 pandemic impacts many communities worldwide. In this study the Poles’ knowledge about COVID-19 as well as people’s behaviours, attitudes and fears during the pandemic were assessed. Changes in these between the outset of the pandemic and the imposition of the strictest lockdown measures in Poland were investigated. Methods Physicians, nurses, students of medicine-oriented faculties, non-medical professionals, students of non-medicine-oriented faculties and secondary school students were surveyed by an anonymous online questionnaire two times: at the onset of the pandemic and in the second week of the strictest lockdown. Statistical analyses were performed using non-parametric tests – Pearson Chi Square, Kruskal-Wallis tests. Results In total 2618 responses were collected. At the beginning people knew that the respiratory system was attacked (97.9%); correctly identified the major symptoms of COVID-19 (95.0%) and ways to prevent infection: hand washing (99.8%), covering mouth (85.9%) and the need to call sanitary-epidemiological services if one experienced COVID-19-like symptoms (92.1%). The biggest changes between the first and second phase of the study concerned behaviours: more people wearing facial masks (+ 37.5%) and staying at home (+ 66.1%). Respondents in the second wave of the survey were also more scared of the pandemic (+ 19.6%), economic crisis (+ 64.1%), and worried about their families (+ 26.5%). However, they were less afraid of the quarantine (lockdown) (− 18.2%). Nurses and physicians were the most worried groups. Conclusions The study showed that even at the outset of the pandemic Polish population had a good initial knowledge about symptoms, transmission, and preventive behaviours regarding COVID-19. People revealed more short-term concerns, such as the worries about coping with quarantine and isolation. After a month, the knowledge and the concerns among the respondents changed. A shift towards long-term pandemic management issues was observed. Respondents reported to experience more fears concerning the pandemic in general, as well as economic and healthcare crises. Medical professionals reported higher level of fear of the pandemic than other groups included in this study. This study uses before-and-after approach which highlights the changes in people’s knowledge and perception of the COVID-19 pandemic during the pandemic’s progression.