Scrotal Extramammary Paget's Disease in an Elderly Caucasian Male.

Extramammary Paget's disease (EMPD) is a rare dermatologic malignancy affecting regions with a dense population of apocrine glands within the intraepithelial tissue, including the vulva, perineum, axilla, scrotum, and penile regions. Clinical presentation varies from being asymptomatic to burning, painful, and pruritic lesions. As a result, it could be misdiagnosed for other dermatologic diseases.

An Uncommon Correlation of Rheumatoid Arthritis and Lupus Nephritis: A Case Report on the Unusual Progression of Lupus Nephritis.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by chronic, widespread inflammation and multisystem organ damage. A serious complication of SLE is damage to the kidneys, which is called lupus nephritis (LN). LN typically manifests after five years post the diagnosis of SLE.

Is It Useful to Monitor Thiopurine Metabolites in Pediatric Patients with Crohn's Disease on Combination Therapy? A Multicenter Prospective Observational Study.

Background: The additional value of azathioprine concomitant treatment on infliximab pharmacokinetics in children is not well described yet.

Aims: In the present study, we aimed to describe the relationship between thiopurine metabolite levels, infliximab trough levels, anti-IFX antibody formation, and clinical and laboratory markers of disease activity in pediatric patients with Crohn's disease, and to assess non-adherence.

Methods: Data were collected prospectively during repeated visits from pediatric patients followed for Crohn's disease in two Czech pediatric inflammatory bowel disease centers between January 2016 and June 2017.

Prediction of Thiopurine Metabolite Levels Based on Haematological and Biochemical Parameters.

Objectives: Therapeutic drug monitoring of thiopurine erythrocyte levels is not available in all centers and it usually requires quite a long time to obtain the results. The aims of this study were to build a model predicting low levels of 6-thioguanine and 6-methylmercaptopurine in pediatric inflammatory bowel disease (IBD) patients and to build a model to predict nonadherence in patients treated with azathioprine (AZA).

Methods: The study consisted of 332 observations in 88 pediatric IBD patients.

Changes in serum hepcidin levels in children with inflammatory bowel disease during anti-inflammatory treatment.

Aim: The aim of this study was to compare changes in serum hepcidin levels in paediatric patients with inflammatory bowel disease during therapy and correlate them with markers of iron metabolism, inflammation and type of treatment.

Methods: Children with newly diagnosed Crohn's disease (CD) and ulcerative colitis (UC) were included in this longitudinal study. Blood and stool samples were collected to assess levels of serum hepcidin and markers of iron metabolism parameters and inflammation.

Hepcidin in newly diagnosed inflammatory bowel disease in children.

Aim: Hepcidin is a central regulator of iron homeostasis. Its production is also influenced by systemic inflammation. The aims of this study were to compare hepcidin levels in paediatric patients newly diagnosed with Crohn's disease (CD) and ulcerative colitis (UC) and to determine the association of hepcidin levels with laboratory and clinical parameters of inflammatory bowel disease (IBD) activity.

Multifarious diagnostic possibilities of the S100 protein family: predominantly in pediatrics and neonatology.

Background: Numerous articles related to S100 proteins have been recently published. This review aims to introduce this large protein family and its importance in the diagnostics of many pathological conditions in children and adults.

Data Sources: Based on original publications found in database systems, we summarize the current knowledge about the S100 protein group and highlight the most important proteins with focus on pediatric use.

Measuring hope: Standardization of the Czech version of the Adult Dispositional Hope Scale in healthy adults.

Objectives: Hope is an important factor that influences mental state of individuals and efficacy of systematic and supportive psychotherapy. The goal of the study was to translate the Adult Dispositional Hope Scale (ADHS) to Czech, evaluate its psychometric properties and create norms to interpret the scale scores.

Methods: The scale consists of twelve items.

Bilateral position-related ulnar neuropathy at elbow in pediatric population and review of the literature.

Perioperative ulnar neuropathies attributed to inappropriate arm positioning and padding during surgical procedures are commonly found in adults. However, their extremely rare incidence in the pediatric population may cause absent awareness of the risk of nerve injury in anesthetized pediatric patients. Furthermore, young patients respond to conservative treatment of neuropathy less favorably than adults and their response also depends on the pathomechanism of the ulnar nerve injury.

Increased prevalence of bicuspid aortic valve in Turner syndrome links with karyotype: the crucial importance of detailed cardiovascular screening.

Background: Bicuspid aortic valve (BAV) represents one of the strongest risk factors for aortic dissection in Turner syndrome (TS). An exact relation between the occurrence of BAV and a particular karyotype has not been established yet. The aim of this study was to determine the association between karyotype and prevalence of BAV.

Unmet psychosocial needs in adolescents with inflammatory bowel disease.

Background: Inflammatory bowel diseases (IBD) are severe medical conditions with adverse impact on the quality of life of both children and their caregivers. IBD are associated with many limitations in personal and interpersonal functioning, and it also restricts the patients' ability to use the full potential (extent) of their capabilities. With the progress and humanization in society, the issue of patients' needs became an important topic; however, the psychosocial functioning and quality of life of adolescents suffering from IBD and their caregivers have been understudied.

Prospective study of hypothalamo-hypophyseal dysfunction in children and adolescents following traumatic brain injury.

Background And Aims: Retrospective studies of TBI have found a neuroendocrine dysfunction following traumatic brain injury in 23 to 60% of adults and 15 to 21% of children. Our aims were to determine the prevalence of hypothalamo-hypophyseal dysfunction in children following brain injury, assess its relationship to the type of injury and the course of the acute post-traumatic phase.

Patients And Methods: Body development (growth, pubertal development, and skeletal maturity) were evaluated in 58 patients (21 girls) after a brain injury rated 3 to 12 on the Glasgow Coma Scale (GCS).

Chromosomal aberrations in childhood acute lymphoblastic leukemia: 15-year single center experience.

Genetic analysis of leukemic cells significantly impacts prognosis and treatment stratification in childhood acute lymphoblastic leukemia (ALL). Our retrospective single center study of 86 children with ALL enrolled into three consecutive treatment protocols (ALL-BFM 90, ALL-BFM 95 and ALL IC-BFM 2002) between 1991 and 2007 demonstrates the importance of conventional cytogenetics and fluorescence in situ hybridization (FISH). Cytogenetic and FISH examinations were performed successfully in 82/86 (95.

Bone marrow metastasis of malignant melanoma in childhood arising within a congenital melanocytic nevus.

Background: Malignant melanoma in childhood is infrequent and can arise within congenital melanocytic nevi. Spread of malignant melanoma to the bone marrow, especially in children, is extremely rare.

Methods And Results: Reported is a case of a 5-year-old boy with a congenital large melanocytic nevus of the head and neck who presented with a short history of low back and leg pain, fever and cervical lymphadenopathy.

Quality of life and parental styles assessed by adolescents suffering from inflammatory bowel diseases and their parents.

Background: Inflammatory bowel diseases (IBDs) in adolescents are chronic medical conditions with a substantial influence on the quality of life (QoL) of the families.

Methods: A total of 27 adolescents suffering from IBD, 39 healthy adolescents, and their parents were included in the cross-sectional study. The adolescents completed the questionnaires ADOR (parenting styles), KidScreen-10 (QoL), SAD (The Scale of Anxiety in Children), and CDI (Children's Depression Inventory).

Treatment and prognosis of childhood acute lymphoblastic leukemia on protocols ALL-BFM 90, 95 and ALL IC-BFM 2002: a retrospective single-center study from Olomouc, Czech Republic.

Great progress has been made in the diagnostics and treatment of childhood acute lymphoblastic leukemia (ALL) over the past decades. The vast majority of children are cured, however, there is need for further improvement, especially in specific patient subgroups. Our aim was to retrospectively evaluate disease characteristics and treatment outcomes of children with ALL enrolled in a single center into consecutive treatment protocols (ALL-BFM 90, ALL-BFM 95 and ALL IC-BFM 2002) between years 1990 and 2007 and comprehensively summarize diagnostic and therapeutic advances between protocols.

Quality of life in adolescents with inflammatory bowel disease and their parents--comparison with healthy controls.

Objective: Inflammatory bowel diseases (IBD) are chronic diseases with a significant impact on quality of life (QoL). The aim of the study was to examine the QoL in children with IBD and their families, depression and anxiety for both the children and their parents.

Methods: Participants were 29 adolescents with IBD (19 individuals suffered from the Crohn disease, another ten had ulcerative colitis) and 40 healthy controls of the same age (13-16 years).

Characterization of iron metabolism and erythropoiesis in erythrocyte membrane defects and thalassemia traits.

Background And Aims: Erythropoiesis is closely related to iron metabolism in a balanced homeostasis. Analyses of diverse erythroid and iron metabolism disorders have shown that disrupted erythropoiesis negatively affects iron homeostasis and vice versa. The aim of this study was to characterize the relationship between erythropoietic activity and iron homeostasis in pediatric patients with erythrocyte membrane defects and thalassemia traits.

[The prospects for children with acute lymphoblastic leukemia of being cured has increased in the Czech Republic in the 21st century to 90% - outcome of the ALL-IC BFM 2002 trial].

Background: Acute lymphoblastic leukemia (ALL) is the most frequent childhood malignancy. Treatment has been unified in the middle of 1980 in the Czech Republic. In 2002-2007 children and adolescents with acute lymphoblastic leukemia were treated in an international randomized trial ALL-IC BFM 2002 in the Czech Republic.

Heart rate variability in children with inflammatory bowel diseases.

Objective: Heart rate variability (HRV) oscillations are used in the detection of autonomic instabilities in various clinical disorders.

Methods: We compared the HRV as a possible marker of chronic distress in children with inflammatory bowel disease (IBD) with HRV frequencies in the healthy controls. Participants were 29 children with IBD (19 Crohn's disease and 10 ulcerative colitis), 25 children were in remission and 4 presented mild disease activity.

Retroperitoneal bronchogenic cyst: prenatal diagnosis of cystoid formation, its progression and surgery.

Aim: Presentation of a rare localization of bronchogenic cyst in retroperitoneum.

Material: A case of a patient with retroperitoneal localization of a bronchogenic cyst with a prenatally diagnosed cystic formation. The surgery was indicated at the age of 6 owing to the progression of lesion.

Pharyngeal perforation after blunt cervical trauma in child.

Pharyngeal perforation caused by non-penetrating cervical trauma is an extremely rare clinical entity both in adults and children. Data concerning management of this type of injury are quite rare in surgical and even scarcer in pediatric literature. Since delay in treatment may be associated with life-threatening complications, prompt diagnosis coupled with appropriate therapy is essential for achieving favorable clinical outcome.

Pediatric acute myeloid leukemia with t(8;16)(p11;p13), a distinct clinical and biological entity: a collaborative study by the International-Berlin-Frankfurt-Munster AML-study group.

In pediatric acute myeloid leukemia (AML), cytogenetic abnormalities are strong indicators of prognosis. Some recurrent cytogenetic abnormalities, such as t(8;16)(p11;p13), are so rare that collaborative studies are required to define their prognostic impact. We collected the clinical characteristics, morphology, and immunophenotypes of 62 pediatric AML patients with t(8;16)(p11;p13) from 18 countries participating in the International Berlin-Frankfurt-Münster (I-BFM) AML study group.