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. Thiopurine metabolites (6-thioguanine and 6-methylmercaptopurine) were measured by high-performance liquid chromatography. Infliximab trough levels and anti-IFX antibody serum levels were measured routinely by ELISA. The risk of loss of response to infliximab therapy was also assessed. RESULTS: A significant association between infliximab serum levels and 6-thioguanine erythrocyte levels was observed when tested as categorical variables (63 patients, 321 observations). To predict infliximab levels > 5 µg/mL, we propose a 6-thioguanine cutoff of 278 pmol/8 × 108 erythrocytes (sensitivity, 0.799; specificity, 0.347). A higher loss-of-response-to-infliximab rate (tested in a subgroup of 51 patients) was observed in patients with undetectable 6-thioguanine levels than in those with detectable levels (p = 0.026). Non-adherence to azathioprine therapy was suspected in 20% of patients. CONCLUSION: Thiopurine metabolite monitoring in pediatric patients with Crohn's disease is useful when optimizing combination therapy. Pediatric patients with undetectable 6-thioguanine levels are more likely to lose response to infliximab therapy. When targeting optimal infliximab levels, the 6-thioguanine cutoff levels in children appear to be higher than in adults.

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. The parameters were examined before treatment (baseline levels) and compared with levels in the follow-up period during maintenance therapy (mean follow-up of 39 months after diagnosis). RESULTS: Patients with CD (n = 30) had higher serum hepcidin levels (expressed as a median and interquartile range) at diagnosis than subjects with UC (n = 13). These levels significantly decreased during the follow-up (from 36.5 (11.5-79.6) to 2.1 (0.9-6.7) ng/mL). In contrast, no significant serum hepcidin level changes were observed in the UC patients (5.4 (3.4-16.6) vs. 4.8 (0.9-8.1) ng/mL). While hepcidin level changes correlated with disease activity and inflammatory parameters (erythrocyte sedimentation rate, C-reactive protein), in CD patients, they correlated only with serum iron levels in patients with UC. Biological therapy was accompanied by a significant decrease in C-reactive protein and interleukin-6 compared to conventional anti-inflammatory therapy in CD patients. CONCLUSIONS: Children with CD had higher serum hepcidin levels on diagnosis compared to subjects with UC. During an anti-inflammatory therapy, serum hepcidin decreased in the CD group but remained consistently low in children with UC.

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. Low AZA dosing was defined as 6-thioguanine levels <125 pmol/8 × 10 erythrocytes and 6-methylmercaptopurine levels <5700 pmol/8 × 10 erythrocytes. Nonadherence was defined as undetectable levels of 6-thioguanine and 6-methylmercaptopurine <240 pmol/8 × 10 erythrocytes. Data were divided into training and testing part. To construct the model predicting low 6-thioguanine levels, nonadherence, and the level of 6-thioguanine, the modification of random forest method with cross-validation and resampling was used. RESULTS: The final models predicting low 6-thioguanine levels and nonadherence had area under the curve, 0.87 and 0.94; sensitivity, 0.81 and 0.82; specificity, 0.80 and 86; and distance, 0.31 and 0.21, respectively, when applied on the testing part of the dataset. When the final model for prediction of 6-thioguanine values was applied on testing dataset, a root-mean-square error of 110 was obtained. CONCLUSIONS: Using easily obtained laboratory parameters, we constructed a model with sufficient accuracy to predict patients with low 6-thioguanine levels and a model for prediction of AZA treatment nonadherence (web applications: https://hradskyo.shinyapps.io/6TG_prediction/ and https://hradskyo.shinyapps.io/Non_adherence/).

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. METHODS: Children with newly diagnosed IBD between January 2012 and September 2016 were enrolled in this comparative cross-sectional study. We analysed levels of serum hepcidin, C-reactive protein, iron, ferritin, soluble transferrin receptors, blood count and faecal calprotectin in all subjects. Serum hepcidin levels were measured by reverse-phase liquid chromatography. The Paediatric Crohn's Disease Activity Index was used to evaluate CD in children, and Paediatric Ulcerative Colitis Activity Index was used for the assessment of UC disease activity. RESULTS: Subjects with CD (n = 53) had significantly higher serum hepcidin levels compared with subjects with UC (n = 23) - 22.6 ng/mL (range 8.5-65.0) versus 6.5 ng/mL (range 2.4-25.8) (P < 0.05). Hepcidin was independently associated with ferritin levels in all IBD patients (P < 0.05). Moreover, there was a significant positive correlation between hepcidin and platelet count (P < 0.05) in children with CD and a negative correlation between hepcidin and faecal calprotectin (P < 0.05) in children with UC. CONCLUSION: Different hepcidin levels between children with newly diagnosed CD and UC suggest the distinct contribution of iron deficiency and/or systemic inflammation to anaemia and may help clinicians choose the best anti-anaemic treatment.

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. RESULTS: The S100 family is composed of Ca2+ and Zn2+ binding proteins, which are present only in vertebrates. Some of these proteins can be used as diagnostic markers in cardiology (S100A1, S100A12), oncology (S100A2, S100A5, S100A6, S100A14, S100A16, S100P, S100B), neurology (S100B), rheumatology (S100A8/A9, S100A4, S100A6, and S100A12), nephrology and infections (S100A8, S100A9, S100A8/A9, S100A12). The most useful S100 proteins in pediatrics are S100A8, S100A9, heterodimers S100A8/A9, S100B and S100A12. CONCLUSIONS: The S100 family members are promising biomarkers and provide numerous possibilities for implementation into clinical practice to optimize the differential diagnostic process.

Acute lymphoblastic leukemia in a child with Leri-Weill syndrome and complete SHOX gene deletion: A Case Report.

BACKGROUND: Leri-Weill syndrome (LWS) ranks among conditions with short stature homeobox gene (SHOX) haploinsufficiency. Data on possible association of SHOX aberrations with malignant diseases are scarce. METHODS AND RESULTS: We report a unique case of an 8-year-old girl who was successfully treated for acute lymphoblastic leukemia (pre-B ALL, intermediate risk) and was subsequently diagnosed with LWS due to characteristic clinical appearance (short disproportionate stature, Madelung deformity of the wrist) and molecular genetic examination (complete deletion of SHOX). An identical SHOX deletion was identified also in the patient's mother. Leukemic cells of the patient were retrospectively examined by array comparative genomic hybridization (aCGH), which revealed five regions of deletions at chromosome X, including the SHOX gene locus. CONCLUSION: Growth retardation in children with hemato-oncologic malignancies cannot always be attributed to cytotoxic treatment and should be carefully evaluated, especially with regards to growth hormone therapy.

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. Four items assess the ability of pathway thinking, four items measure agency, and the remaining four items are fillers that are not interpreted. There were 394 adult participants with negative psychiatric history who completed the ADHS and BDI-II. Their mean age was 27.1±11.7 years, most of them were women (n=303; 76.9%). RESULTS: There was no significant relationship between age or sex and hope. Reliability was analyzed by Cronbach alpha (α=0.82) and the split-half method (Spearman-Brown coefficient = 0.81). The factor structure of the scale was approved by the results of exploratory and confirmatory factor analysis, except the ninth item that similarly saturated both subscales. The ADHS moderately negatively correlated with BDI-II. Norms were created for the scores of the entire scale and both subscales. CONCLUSION: The Czech version of the Adult Dispositional Hope Scale shows adequate psychometric properties.

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. A surgeon's or anesthetist's failure to recognize all of these specifics in children may result in substantial morbidity of young patients leading to lawsuits. Fortunately, with an adequate knowledge of surgical anatomy and types of procedures and positions in which the ulnar nerve is particularly vulnerable, and familiarity with measures to minimize the potential for neuropathy, this serious complication can be prevented. The aims of this review are to highlight personal experience and current knowledge of the rare position-related ulnar neuropathy, both from a clinical and anatomical-pathophysiological perspective, and to raise awareness about this rare but serious complication in the pediatric population.

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. METHODS: Sixty-seven TS patients aged between 6.6 and 32.5 years underwent cardiac magnetic resonance imaging (MRI) study. They were divided into four cytogenetic subgroups-45,X karyotype (n=27); 45,X/46,XX mosaicism (n=17); structural abnormalities of the X chromosome (n=10); and 45,X/structural abnormality of the X chromosome mosaicism (n=13). Prevalence of BAV and odds ratio (OR) compared with the general population in the whole study group, and statistical comparison of prevalences of BAV among the individual subgroups were determined. RESULTS: Prevalence of BAV in the whole study group was established as 28.4% [OR 208.3 (95% CI - 103.8-418.0); p-value<0.0001]. Individuals with 45,X karyotype had the highest prevalence of BAV - 40.7%, p-value<0.0001. Presence of any 45,X cell line in karyotype significantly predisposed to BAV (p-value=0.05). CONCLUSIONS: The 45,X karyotype is associated with the highest prevalence of BAV. Also, the presence of the 45,X cell line in any mosaic karyotype increases the probability of BAV.

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). The patients underwent standard endocrine tests - TSH, fT4, IGF-1, PRL, morning cortisol, FSH, LH, and testosterone in boys and estradiol in girls - in the early post-traumatic period (2 to 14 days; T0) and at 3, 6, and 12 months after the injury (T3, T6, and T12). Dynamic tests were carried out in patients with abnormalities in their clinical examination and/or laboratory results. An MRI was performed on all patients at T12. RESULTS: The median age at the time of injury was 11.3 (0.5 to 18.7) years. Of the 58 patients, 23 had GCS < 8, corresponding to severe brain injury. At T0, diabetes insipidus (DI) was diagnosed in 12 patients, and the syndrome of inappropriate antidiuretic hormone secretion (SIADH) was found in 4 patients. Frequent hormonal changes simulated central hypothyroidism (in 45% of patients) and hypogonadotropic hypogonadism (in 25% of adolescents who were already pubertal at the time of injury > Tanner II). Examination at T3 (n = 58) confirmed a combined pituitary hormone deficiency in two boys and DI in another one. At T6 (n = 49), hormonal dysfunctions were diagnosed in two boys (precocious puberty and growth hormone deficiency). At T12 (n = 39), a new endocrine dysfunction was diagnosed in five patients (growth hormone deficiency in two, hypogonadotropic hypogonadism in two, and in one patient, already diagnosed with a growth hormone deficiency, central hypothyroidism, as well). Brain MRI revealed an empty sella in two patients with growth hormone deficiency. Patients with GCS < 8 had more symptoms of SIADH or DI in the early post-traumatic period 11/23 vs. patients with GCS of 8 to 13 (4/35), and more frequent hormonal disorder (6/23) than individuals with moderate trauma (3/35), P = 0.0135. The incidence of endocrine dysfunction at T0 significantly correlated with the severity of injury (P = 0.05), but it was not an indicator for the development of a late hormonal disorder. CONCLUSION: Within a year after injury, a hormonal disorder was found in 17.6% of the patients. Neuroendocrine dysfunction as a late consequence of craniocerebral trauma in children and adolescents was less frequent than in adults. Risk factors for its development are the gravity of the injury, brain scan pathology, and possibly the development of DI, SIADH, or CSWS in the acute post-traumatic phase.

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 (95.3%) patients and chromosomal changes were detected in 78 of the 82 (95.1%) patients: in 69/73 patients with B-cell precursor (BCP)-ALL and in 9/9 patients with T-lineage ALL (T-ALL). The most frequent chromosomal changes in subgroups divided according to WHO classification independent of treatment protocol and leukemia subtype were hyperdiploidy in 36 patients (with ≥50 chromosomes in 23 patients, with 47-49 chromosomes 13 patients) followed by translocation t(12;21) with ETV6/RUNX1 fusion detected by FISH in 18 (22%) patients. Additional changes were detected in 16/18 (88.8%) ETV6/RUNX1-positive ALL patients with predominant deletion or rearrangement of untranslocated ETV6 allele. Unique aberrations were detected in 4 patients and dicentric chromosomes in 8 patients, one with T-ALL. These results demonstrate that cytogenetics and FISH successfully provided important prognostic information and revealed not only recurrent but also new and rare rearrangements requiring further investigation in terms of prognostic significance.

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). The parents completed the BAI (Beck Anxiety Inventory), BDI-II (Beck Depression Inventory, second version), and PedsQL (Pediatrics Quality of Life) Family Impact Module. RESULTS: The parental styles of the parents of the IBD adolescents and controls were without significant differences. The only exception was that fathers' positive parental style was significantly higher in the fathers of the controls. There were no statistically significant differences between the IBD children and controls in the QoL assessed using KidScreen-10. However, the QoL of the parents of the ill children was significantly lower than that of the parents of the controls (PedsQL total scores in mothers 66.84±14.78 vs 76.17±14.65 and in fathers 68.86±16.35 vs 81.74±12.89, respectively). The mothers of the IBD adolescents were significantly more anxious (BAI scores 9.50±10.38 vs 5.26±4.75) and the fathers more depressed (BDI-II scores 7.23±6.50 vs 3.64±3.51) than the parents of the controls, but there was no difference in the levels of anxiety or depression between the IBD adolescents and the controls. The positive parental style of both the parents of the children suffering from IBD positively correlated with the QoL of the adolescents evaluated by KidScreen-10. The positive parental style of the fathers negatively correlated with the children's state and trait anxiety and negatively correlated with the severity of childhood depression. CONCLUSION: The fathers of the IBD adolescents may exhibit low levels of positive parenting style and be mildly depressed, and the mothers tend to exhibit higher levels of anxiety.

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. Despite regular follow-up by a dermatologist and plastic surgeon and repeatedly negative histology of previous partial excisions, diffuse bone marrow infiltration with malignant melanoma was diagnosed. The primary site was identified in the post-excision area. The disease progressed rapidly on ipilimumab immunotherapy and led to death at four months from the diagnosis. CONCLUSION: Surveillance is indispensable in children with a predisposition to melanoma and nonspecific symptoms such as bone pain, gait impairment or cytopenia, should always be taken into account.

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. METHODS: Selected markers of erythropoietic activity (erythropoietin, soluble transferrin receptor - sTfR and growth differentiation factor 15) and iron status parameters (serum iron, ferritin and hepcidin) were evaluated in pediatric patients with erythrocyte membrane defects and thalassemia traits. RESULTS: The patients with erythrocyte membrane defects and thalassemia traits had altered iron homeostasis due to disturbed erythropoiesis. In comparison with healthy controls, they had a normal to low hepcidin/ferritin ratio and concomitantly elevated sTfR. CONCLUSION: The findings suggest that pediatric patients with erythrocyte membrane defects and thalassemia traits are more susceptible to iron overload than the general population and that the (hepcidin/ferritin)/sTfR ratio can be used to monitor any worsening of the disease.

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. The aim of this article is to provide a comprehensive, up-to-date literature review of the unmet needs of patients with IBD and their caregivers. METHOD: A computerized search of MEDLINE publications from 1990 to 2016 using the keywords "inflammatory bowel disease", "Crohn disease", "ulcerative colitis" and "unmet needs". In the period 1990-2016, the MEDLINE searches identified 54 publications. Articles cited in the papers from these searches were also used. The total number of 132 particular articles were collected, sorted by their relevance and key articles (n=72) listed in reference lists were searched. RESULTS: Patients' needs differ at various stages of the illness and may have different origins and goals. Thus, we divided the needs into five groups according to their nature; i.e. needs to be connected with symptoms, treatment, quality of life, family and age-related challenges. We provide implications of the patients' needs for pharmacotherapy and psychotherapy. CONCLUSION: Following the needs of patients with IBD may be a crucial part of the therapeutic process. Due to the better understanding and cooperation, the impact of disease could be reduced, and the physical and mental condition of the patient could be improved. However, many needs remain unmet due to both medical and social factors.

[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]. / Sance na vylécení detí s akutní lymfoblastickou leukémií stoupla v Ceské republicev 21. století na 90 % - výsledky studie ALL IC-BFM 2002.

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. 291 patients aged 1-18 years were enrolled; infants below 1 year entered a separate trial. METHODS AND RESULTS: Patients were stratified into three risk groups according to their age, initial leukocyte count, prednisone response, presence of fusion genes BCR/ABL or MLL/AF4, bone marrow D+15 and remission status D+33. The whole therapy took 24 months. Randomized late intensification compared standard BFM therapy with extended, usually more intensive experimental treatment. The median follow-up was 8.7 years. Complete remission was achieved in 97.9% patients, 1% died in remission. 11% of children relapsed, 1.7% with CNS involvement. Six children (2.1%) developed secondary malignancy. Event free survival (EFS) 8 years from diagnosis was 83.5%, overall survival (OS) 91.4%. EFS and OS of the risk groups were: standard risk: 89.4%; 98.1%; intermediate risk: 82.6%; 89.6%; high risk: 68.8%; 78.1%. Male sex and age above 10 years were adverse prognostic factors. CONCLUSIONS: In comparison with the previous trial ALL-BFM 95, significant improvement was achieved.

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. They were compared with the control group of 35 healthy children of the same age (13-16 years-old). RESULTS: In HRV assessment, adolescents with IBD had significantly lower levels of the spectral activity in an LF band in all three positions; lower levels of VLF in both supine positions; and the ratio of the spectral activity at LF/HF was significantly lower in the second post (standing). CONCLUSION: These results indicate children with IBD have less adaptability to stress.

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). The probands and their parents completed questionnaires measuring the quality of life (KidScreen-10, PedsQL), depression (CDI, BDI-II), and anxiety (SAD, BAI). RESULTS: The QoL measured by questionnaires did not differ between the adolescent participants, but it was significantly lower in the parents of the children with IBD than in the parents of the healthy controls. The parents of the IBD children scored lower in the Family Impact Module Total Scale Score and the parental Health-Related QoL Summary Score. The fathers of the IBD children also had a lower level of the Family Functioning Summary Score. There wasn't any difference in the levels of anxiety and depressive symptoms among the IBD adolescents and the controls. CONCLUSIONS: The parents of the children with IBD experience lower QoL than the parents with the healthy children. The children with IBD show similar symptoms of depression, anxiety, and QoL as the healthy controls.

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. To the best of authors' knowledge, the present study illustrates for the first time the experience with successful treatment of pharyngeal perforation caused by a blunt cervical trauma in a child.