Pediatric endoscopy in times of pandemic: A nationwide retrospective analysis.

BACKGROUND: Gastrointestinal endoscopy is a procedure that carries an increased risk of transmission of SARS-CoV-2 infection to medical staff. In patients, COVID-19 is a risk factor for adverse events of medical procedures. This study analyzed the real-life risk of, and factors contributing to, infection transmission to endoscopic personnel, and possible adverse events of the endoscopy procedure and anesthesia in children with COVID-19. METHODS: Nationwide retrospective analysis of medical records of children with confirmed SARS-CoV-2 infection who underwent gastrointestinal endoscopy in Poland between February 2020 and February 2022. RESULTS: Fifty-eight patients were included in the analysis, 35% of whom had COVID-19 symptoms at the time of endoscopy. The dominant indications for endoscopy were foreign body or corrosive substance ingestion and gastrointestinal bleeding. Nine cases of virus transmission were registered among endoscopic personnel. In all of these cases, the endoscopy team was unaware of the patient's infection (p < 0.01), although symptoms were present in 78% of the children. Lack of use of personal protective equipment was the strongest predictor of SARS-CoV-2 transmission (p < 0.01). The risk of infection was not statistically significantly dependent on the method of anesthesia, intubation or the type of endoscopy. No statistically significant correlation was found between symptomatic infection and adverse events of endoscopy or anesthesia occurrence. There was one reported anesthesia-related adverse event involving extubation difficulties due to worsening respiratory infection symptoms. CONCLUSIONS: The risk of transmitting SARS-CoV-2 to endoscopic personnel during procedures in children is low and depends on compliance with infection prevention and control measures. Performing gastrointestinal endoscopy in children with COVID-19 does not appear to be associated with an increased risk of adverse events.

Nutritional status of pediatric patients with inflammatory bowel diseases is related to disease duration and clinical picture at diagnosis.

This cross-sectional study presents the nutritional status of newly diagnosed pediatric patients with Crohn's disease (CD) and ulcerative colitis (UC) and its association with the duration of the disease and selected clinical features. We analyzed the data of 41 pediatric patients with CD and 29 with UC (mean age: 13.1 y, range: 5.2-18.0 y) up to 3 mo. from diagnosis. Anthropometry included body weight, body height, body mass index (BMI), three skinfold thicknesses, mid-upper arm circumference and mid-upper arm muscle circumference adjusted for age and sex using national standards. Anthropometry was linked to the disease duration, location of the disease, symptoms, and blood test results. Both studied groups presented significantly lower BMI compared to the reference population, but only children with CD characterized with significantly worse nutritional status according to arm anthropometry. In CD, better nutritional status was associated mainly with longer disease duration and, to a lesser extent, with extraintestinal manifestations, perianal disease, and small intestinal lesions. In UC, anemia at diagnosis was associated with poor nutritional status. Our finding emphasizes the need for more attentive diagnostic care for pediatric patients who exhibit extraintestinal symptoms or perianal disease with no obvious signs of malnutrition, to avoid diagnostic delays.

Pancreatic Involvement in the Course of Inflammatory Bowel Disease in Children-A Multi-Center Study.

The coexistence of inflammatory bowel disease (IBD) with pancreatic pathology is rare in children. A retrospective analysis of data from 1538 children diagnosed with IBD in 2014-2021 was conducted to determine the frequency and causes of pancreatitis and asymptomatic hyperlipasemia (HL) or hyperamylasemia (HA) in this group of patients. Among the 176 children (11.4%) with pancreatic involvement (PI), acute pancreatitis (AP) was diagnosed in 77 children (43.8%), and HA or HL was observed in 88 children (50.0%). Only a few patients were diagnosed with autoimmune or chronic pancreatitis (6.2%). PI was observed at the time of the IBD diagnosis in 26.1% of the cases. A total of 54.5% of the patients had moderate to severe IBD, and 96% had colonic involvement at the time of diagnosis of PI. Idiopathic PI was the most common (57%), followed by drug-induced PI (37%) and azathioprine (AZA). In patients with AZA-induced AP, the successful introduction of 6-mercaptopurine (6-MP) to therapy was noted in 62.5% of the children. Our results suggest that routine monitoring of pancreatic enzymes in patients with IBD should be performed, especially after the initiation of the AZA treatment. The presence of transient HA/HL in IBD does not necessarily indicate pancreatic pathology.

A Review of Ophthalmic Complications in Inflammatory Bowel Diseases.

Inflammatory bowel diseases (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), are chronic immune-mediated conditions caused by various polygenic and environmental factors. Clinical manifestations of IBD primarily occur in the gastrointestinal tract, but many patients are affected by extraintestinal complications, including eye diseases. Ocular disorders are the third most common extraintestinal manifestation (EIM), following musculoskeletal and mucocutaneous involvement. Episcleritis, frequently occurring in IBD patients, may be associated with exacerbation of the intestinal disease. Uveitis does not correlate with IBD activity but may be related to the presence of other EIMs, particularly erythema nodosum and peripheral arthritis. Early detection and specific therapy of ocular manifestations of IBD are fundamental to avoiding sight-threatening complications. Therefore, ophthalmic evaluation should be performed in all IBD patients. Systemic corticosteroids or immunosuppressants may be inevitable in severe cases to control ocular inflammation. Persistent and relapsing conditions usually respond well to TNF-α-inhibitors. Interdisciplinary cooperation between gastroenterologists and ophthalmologists is fundamental in initiating the appropriate treatment for patients.

Etiology of IBD-Is It Still a Mystery?

Inflammatory bowel diseases (IBD), including colitis ulcerosa and Crohn's disease, are chronic diseases of the gastrointestinal tract for which the cause has not been fully understood. However, it is known that the etiology is multifactorial. The multidirectional network of interactions of environmental, microbiological and genetic factors in predisposed persons lead to an excessive and insufficiently inhibited reaction of the immune system, leading to the development of chronic inflammation of the gastrointestinal walls, the consequence of which is the loss of the function that the intestine performs, inter alia, through the process of fibrosis. Detailed knowledge of the pathways leading to chronic inflammation makes it possible to pharmacologically modulate disorders and effectively treatthese diseases. In this review, we described the primary and adaptive immune system response in the gut and the known immune pathogenetic pathways leading to the development of IBD. We also described the process leading to intestinal tissue fibrosis, which is an irreversible consequence of untreated IBD.

Prevalence of haplotype DQ2/DQ8 and celiac disease in children with type 1 diabetes.

Type 1 diabetes (T1D) and celiac disease (CD) coexist very often. Identification of the human leukocyte antigen (HLA) DQ2/DQ8 can confirm the genetic predisposition to CD. Negative result of this test allows to exclude CD with a high probability. It was suggested that in individuals with higher risk of CD, including T1D patients, the implementation of genetic testing should reduce the number of patients requiring systematic immunological screening. The aim of this study was to analyze the prevalence of different haplotypes predisposing to CD in children and adolescents with previously diagnosed T1D. MATERIAL AND METHODS: A retrospective analysis was performed on 166 T1D children (91 girls) in whom HLA DQ2/DQ8 alleles were tested. In 9.6% CD was also diagnosed. RESULTS: In 12.7% both HLA DQ2/DQ8 were negative. In 87.3% patients HLA DQ2 and/or DQ8 was positive, including 27.7% patients with both haplotypes DQ2.5 and DQ8 positive. In all CD patients the disease predisposing alleles were positive, while none of the HLA DQ2/DQ8 negative children were diagnosed with CD. CONCLUSIONS: The prevalence of HLA DQ2.5 and the HLA DQ2.5 / HLA DQ8 configuration is higher in patients with T1D, and CD compared to children with T1D alone. The combination of HLA DQ2 and HLA DQ8 most significantly increases the risk of developing CD. The group of HLA DQ2/DQ8 negative patients with improbable CD diagnosis, is relatively small. Most of T1D patients HLA DQ2/DQ8 positive need further regular antibody assessment. In patients with T1D, who are at high risk of developing CD, genetic testing may be considered to select those who require further systematic serological evaluation. Due to its retrospective nature, the study was not registered in the database of clinical trials and the Clinical trial registration number is not available.

Sodium Butyrate Effectiveness in Children and Adolescents with Newly Diagnosed Inflammatory Bowel Diseases-Randomized Placebo-Controlled Multicenter Trial.

BACKGROUND: Butyric acid's effectiveness has not yet been assessed in the pediatric inflammatory bowel disease (IBD) population. This study aimed to evaluate the effectiveness of oral sodium butyrate as an add-on to standard therapy in children and adolescents with newly diagnosed IBD. METHODS: This was a prospective, randomized, placebo-controlled multicenter study. Patients aged 6-18 years with colonic Crohn's disease or ulcerative colitis, who received standard therapy depending on the disease's severity, were randomized to receive 150 mg sodium butyrate twice a day (group A) or placebo (group B). The primary outcome was the difference in disease activity and fecal calprotectin concentration between the two study groups measured at 12 weeks of the study. RESULTS: In total, 72 patients with initially active disease completed the study, 29 patients in group A and 43 in group B. At week 12 of the study, the majority of patients achieved remission. No difference in remission rate or median disease activity was found between the two groups (p = 0.37 and 0.31, respectively). None of the patients reported adverse events. CONCLUSIONS: A 12-week supplementation with sodium butyrate, as adjunctive therapy, did not show efficacy in newly diagnosed children and adolescents with IBD.

Selective Forms of Therapy in the Treatment of Inflammatory Bowel Diseases.

Selective interference with the functioning of the immune system consisting of the selective blockade of pro-inflammatory factors is a modern, promising, and developing strategy for the treatment of diseases resulting from dysregulation of the immune system, including inflammatory bowel disease. Inhibition of the TNF alpha pathway, group 12/23 cytokines, and lymphocyte migration is used in the treatment of severe or moderate ulcerative colitis and Crohn's disease. Intracellular signal transduction by influencing the phosphorylation of SAT (signal transducer and activator of transcription) proteins remains in clinical trials.

Endoscopic Ultrasonography in Children with Eosinophilic Esophagitis-A Review.

Endoscopic ultrasonography (EUS) is a diagnostic endoscopy of the upper gastrointestinal tract, during which ultrasound of nearby organs is also performed. It is also possible to perform a fine needle aspiration biopsy. Currently, EUS is performed more frequently in adults. Despite some limitations, this diagnostic method is also more and more often performed in pediatric patients. Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus, which also occurs in children, and leads to irreversible fibrosis of the esophagus wall, if left untreated. Traditional methods of diagnosing and monitoring EoE treatment have significant limitations, and the use of EUS and total esophageal wall thickness (TWT) assessment may bring measurable benefits. Several studies have shown an increased thickening of TWT in EoE in children compared to pediatric patients with gastroesophageal reflux disease, and a decrease in TWT in adults who responded to EoE treatment. These results suggest that EUS and TWT measurement may become an important test in diagnostics, monitoring the effectiveness of therapy, assessing disease progression, and in individualizing the method and duration of EoE treatment also in children.

Insolation and Disease Severity in Paediatric Inflammatory Bowel Disease-A Multi-Centre Cross-Sectional Study.

This study was to investigate whether the clinical course of inflammatory bowel disease (IBD) in a Polish paediatric cohort fits a seasonal pattern and depends on insolation. Two hundred and fourteen patients diagnosed with Crohn's disease (CD) and 192 with ulcerative colitis (UC) aged from 3 to 18 years, were recruited in seven centres of similar latitude. The seasons were defined as winter (December-February), spring (March-May), summer (June-August), autumn (September-November). The year was also divided depending on insolation threshold (3.0 kWh/m2/day). Patients diagnosed with IBD when the isolation was >3 kWh/m2/day had poorer nutritional status than those diagnosed while insolation was below threshold (lower standardised BMI at diagnosis (-0.81 ([-1.34]-[-0.03]) vs. -0.52 ([-1.15]-0.15); p = 0.0320) and worst flare (-0.93 ([-1.37]-[-0.05]) vs. -0.66 ([-1.23]-0.17); p = 0.0344), with the need for more frequent biological treatment (45.5% vs. 32.7%, p = 0.0100). Patients diagnosed in winter were significantly younger at diagnosis (11.4 vs. 13.0; padj = 0.0180) and first immunosuppressive treatment (11.3 vs. 13.3; padj = 0.0109) than those diagnosed in other seasons. CD patients diagnosed in months with higher insolation spent more days in hospital than those diagnosed in months with lower insolation [4.6 (1.8-11.8) vs. 2.9 (1.3-6.2); p = 0.0482]. CD patients diagnosed in summer had significantly more concomitant diseases. In patients with CD, the occurrence of the worst flare was more frequent in autumn. Furthermore, the season of birth was associated with Pediatric Crohn's Disease Activity Index at worst flare and earlier surgery. In conclusion, several clinical parameters are associated with insolation, the season of diagnosis and season of birth in the clinical course of Crohn's disease.

Laugier-Hunziker syndrome: a case report of the pediatric patient and review of the literature.

Laugier-Hunziker syndrome (LHS) is a rare, idiopathic pigmentary disorder especially affecting the lips and oral mucosa. At present, no more than 200 cases of patients diagnosed with LHS syndrome have been described worldwide. To date, three patients under the age of 20 have been described, including the youngest patient who is a 12-year-old child. The exact etiology of LHS still remains uncertain, as there is no evidence of systemic symptoms or increased cancer risk. The final diagnosis of LHS is possible after the exclusion of other, more serious diseases involving skin-mucosal hyperpigmentation, mainly Peutz-Jeghers syndrome (PJS) and Addison's disease (AD). Herein, we present a 16-year-old patient who has been diagnosed with oral hyperpigmentation since the age of 13. We reviewed the clinical and histological findings. In addition, we discussed the differential diagnosis of mucocutaneous hyperpigmentation.

Correlation between biomarkers (calprotectin, seromucoid, metalloproteinase-3 and CRP) and clinical and endoscopic activity of ulcerative colitis in children.

PURPOSE: The aim of the study was to compare the clinical activity and inflammatory markers with the endoscopic activity of ulcerative colitis (UC) and mucosal healing. PATIENTS AND METHODS: The study included 50 children aged 2-18 years (27 girls, 23 boys) diagnosed with UC at various stages of the disease; 8 children were assessed twice. In 20 children, colonoscopy revealed pancolitis, in 24 - left-sided colitis, and in 6 - ulcerative proctitis. The clinical activity of UC was assessed according to the Pediatric Ulcerative Colitis Activity Index (PUCAI). Endoscopic index of the colon inflammation was assessed according to the Rachmilewitz scoring. We assessed the clinical activity of UC, the concentration of fecal calprotectin (FC), seromucoid, metalloproteinase-3 (MMP-3) and C-reactive protein (CRP). RESULTS: The study demonstrated significant decrease in the clinical activity, FC, seromucoid and MMP-3 in endoscopic remission. We found a strong positive correlation between PUCAI, FC, serum seromucoid and serum MMP-3 with the endoscopic activity. However, we found no relationship between the concentration of CRP and the endoscopic activity of the disease. Among the studied markers, seromucoid exhibited the best performance in distinguishing between patients with endoscopic remission and endoscopically active disease. CONCLUSIONS: The examined inflammatory markers such as FC, as well as serum seromucoid and MMP-3 levels may be helpful in the assessment of large intestine mucosal healing.

New colon anatomy-related ratios used to predict the course of colonoscopy in children.

BACKGROUND: In children, colonoscopy is a safe procedure, although it is more difficult to perform in patients whose body mass index (BMI) is under 25. OBJECTIVES: The aim of the study was to establish the relationship between children's age, body mass and height and incomplete colonoscopies due to colon anatomy. MATERIAL AND METHODS: A retrospective evaluation of diagnostic endoscopies in 403 children aged 3-18 years (192 girls and 211 boys) was performed. New ratios were introduced: the incomplete colonoscopy anatomy-related ratio (ICAR) and the modified incomplete colonoscopy anatomy-related ratio (MICAR). RESULTS: The terminal ilium was not reached in 59 children: 27 girls and 32 boys (14.6% of patients). In 13 girls and 18 boys (comprising 7.69% of the study population) no pathological causes were found for the incomplete colonoscopy. There were statistically significant differences concerning colon anatomy-related incomplete colonoscopies in relation to the children's weight. No significance was found in relation to height or age. Incomplete examinations were more frequent in patients weighing less than 30 kg (p = 0.0006), both in boys (p = 0.0090) and girls (p = 0.048). The risk of incomplete colonoscopy (odds ratio - OR) in boys and girls weighing less than 30 kg was 3.995 (95% CI = 1.489-10.720) and 3.373 (95% CI = 1.078-10.560), respectively. For this group of patients, the ICAR ranged between 0.0309 and 0.1889, while the MICAR range was 0.0-0.1889. CONCLUSIONS: Body mass is a statistically significant factor for evaluating the risk of incomplete colonoscopies in children. The lower the ICAR and MICAR values, the lower the risk of non-completion of a colonoscopy due to anatomical (i.e., disease-unrelated) causes.

The large intestine from fetal period to adulthood and its impact on the course of colonoscopy.

The human large intestine in the living adult has a total length of about 1300 mm, ranging from 1100 to 2108 mm. The development of the gut continues after birth, up to the age 4-5. The large intestine ascends at the beginning in the right abdominal quadrant, then it traverses the abdominal cavity, and finally it descends to the anus. The left and right colic flexures are the basic flexions between the transverse, ascending and descending colon, respectively. Additionally, there are secondary bendings between intestinal segments. The angles between the neighbouring parts can vary between examined subjects. Most of the angulations can be found in the transverse (range 2-9) and sigmoid colon (range 1-9), making them the most troublesome parts to pass with a colonoscope. Colonoscopy (usually performed in the left lateral or supine position) is one of the most important examination of the large intestine mucus membrane. During this procedure the endoscope is passed through the colon into the cecum or terminal ilium. The individual anatomical features (tortuosity, supernumerary loops and elongation) may slow down or interfere with the progress of the scope. We summarize current knowledge on the human large intestine from the fetal period to adulthood and carve out some aspects that are currently less known to colonoscopists.

[Ganglioneuroblastoma in a child with chronic abdominal pain - a case report].

Chronic abdominal pain is a very common complaint in the population of children and adolescents. In most cases, the usual cause are functional gastrointestinal disorders. However, in a few percent of children, the reason for persistent chronic stomach pain are organic diseases occurring in the gastrointestinal tract, as well as parenteral diseases, including uro-genital tract abnormalities, inflammation of the lower respiratory tract and cancer processes. Among organic causes, in addition to those commonly encountered, such as: intolerances and food allergies, gastroesophageal reflux disease, chronic gastritis or duodenitis, or urinary tract infections, the diagnosis should also include very rare causes, for example, neoplastic diseases, among them tumors of the abdominal cavity. In the case described in the present article, a 6-year-old girl with chronic abdominal pain, symptoms of gastro-oesophageal reflux and constipation, and previously diagnosed food allergy and lactose intolerance, was referred for widening the diagnosics due to the occurrence of alarm symptoms. The nodule revealed in the chest X-ray, in CT scan, turned out to be a paravertebral tumor with the specific features of neuroblastoma. After a macroscopically complete tumor resection based on the result of histopathological examination, the diagnosis of ganglineuroblastoma was established. The presence of alarm symptoms in anamnesis and physical examination in children with abdominal pain suggests a higher probability of the organic origin of the disease and should always lead to extended diagnostics. Ganglioneuroblastoma is a very rare disease, in most cases is located primarily in the abdominal cavity, and the most common associated symptom is abdominal pain.

Oral cavity health among cystic fibrosis patients: Literature overview.

Cystic fibrosis is a genetic disorder in which the mutation of the Cystis Fibrosis Transmembrane Conductance Regulator (CFTR) gene that codes the protein forming a chloride channel of epithelial cells results in its distorted functioning. The manifestations of the disorder are mainly observed in the respiratory and digestive system. Accumulation of sticky and thick mucus is the dominant clinical symptom; it leads to chronic infections and gradual tissue destruction. Although cystic fibrosis remains incurable, it is currently feasible to extend patients' life expectancy thanks to modern therapy possibilities. As cystic fibrosis is no longer the domain of pediatricians, health care to CF patients needs to be provided by doctors of various specializations. The multidisciplinary team of doctors should include a dentist aware of specific prevention and treatment needs of this group of patients. It results from the fact that in the course of cystic fibrosis it is possible to observe a variety of changes in the oral cavity environment. The study presents dental issues observed in CF patients and reported in literature. Particular attention was paid to dental caries, mineralization disorders of hard dental tissues, gingivitis and the change in the content and properties of saliva; moreover, prevention and treatment options regarding oral cavity health is this group of patients were taken into consideration.

Celiac disease and its impact on the oral health status - review of the literature.

The paper presents the most recent reports on celiac disease, especially its manifestations within the oral cavity. Particular attention should be paid to typical dental enamel defects with a various degree of advancement: discolorations, horizontal groves and pits, and even significant structural destruction causing the change of the dental crown. Symmetric location of defects within all dentition sections, and within the same anatomic groups of teeth (the most frequently: incisors and first permanent molars), is specific for celiac disease. The changes described above may be the only manifestation of celiac disease; therefore, in the case of their occurrence further studies towards gluten intolerance are recommended even when other symptoms are not present. In celiac patients, recurrent aphthae and other disorders of the oral mucosa such as ulceration, erythema, atrophic glossitis, as well as dryness and a burning sensation (particularly of the tongue) may be present, which may be caused by malnutrition. Delayed tooth eruption may also be a consequence of alimentary deficiency in celiac disease.

[The frequency of anemia in children with newly diagnosed Crohn's disease in children]. / Czestosc wystepowania niedokrwistosci u dzieci z nowo rozpoznana choroba Lesniowskiego-Crohna.

UNLABELLED: The aim of the study was to analyze the occurrence of anemia in children with newly diagnosed Crohn's disease and 3 months after the initiation of treatment depending on location and activity of the disease. MATERIALS AND METHODS: 36 children aged from 2 to 18 years with newly diagnosed Crohn's disease treated in the years 2005-2011. According to the classification of Paris children were divided into 2 groups: II - 9 children (0-10 years); II - 27 children (11-17 years). We analyzed RBC, Hb, HCT, MCV, Fe, PCDAI at diagnosis and 3 months after the treatment, depending on the location and phenotype of the disease. Anemia was defined according to WHO criteria. Children were treated in accordance with the standards and activity of the disease. During the observation period supplementation of iron was not used. We analyzed the potential factors that could affect on the incidence of anemia. RESULTS: In the group I mean activity of the disease was lower than in group II in both observed periods. No statistically significant differences in hemoglobin, red blood cell count, hematocrit and volume of erythrocytes at diagnosis and after 3 months were noted. The average iron concentration increased from 6.98 microg/dl to 9.95 microg/dl (p < 0.05) after the treatment. A statistically significant higher incidence of iron deficit in group II in both analyzed periods was observed, respectively, 66.7% vs 92.59% and 55.5% vs 77.77% (p < 0.05). Anemia was more frequent in group II (p > 0.05). After 3 months of treatment the incidence of anemia reduced from 63.88% to 44.4% (p < 0.05) and iron deficit from 83.3% to 72.2% (p > 0.05). Inflammatory changes in upper gastrointestinal tract were found in 61.1% of children with Helicobacter pylori infection in 8.3% of cases. Ileal location was observed in 52.7% of children, more often in group II than I, 62.9% vs 22.22% (p < 0.05). Isolated location of inflammatory lesions in the colon more frequently observed in group I than II, respectively 55.5% vs 29.6% (p < 0.05). Thiopurine were used in 38.8% of children more often in group II than I, respectively 48.1% vs 11.1% (p < 0.05). Corticosteroids were used in 50.0% of children, often in group II than I, respectively 55.5% vs. 33.3% (p > 0.05). CONCLUSIONS: In children with newly diagnosed Crohn's disease anemia was diagnosed in 63.88% of the children and in 47.2% of children after 3 months of treatment (p < 0.05), iron deficiencies, respectively, in 83.3% and 72.2% of children (p > 0.05). 3-month treatment period of Crohn's disease has increased the iron concentration from 6.98 microg/dl to 9.95 mg/dl although the absence of supplementation (p < 0.05). In group II more often than in group I risk factors of anemia like changes in the upper gastrointestinal tract, the location of the ileum, intestinal villous atrophy and the use of immunosuppressive drugs were observed (p < 0.05). Anemia and iron deficiency were more often observed in the ileocolonic location than when the upper gastrointestinal tract was involved (adquately 55.5% vs 30.5%; p < 0.05 and 75.0% vs 41.6%; p < 0.05).

[Blue rubber bleb naevus syndrome--a rare cause of bleeding from the gastrointestinal tract--case report]. / Zespól naczyniaków gumiastych--rzadka przyczyna krwawienia z przewodu pokarmowego--opis przypadku.

Blue rubber bleb nevus syndrome (BRBNS or Bean's syndrome) is a rare disease characterized by the interaction of hemangiomas of rubber-like tenacity in the skin and gastrointestinal tract, rarely in other organs. Cutaneous malformations are usually asymptomatic and not require any treatment. The most common symptoms of gastrointestinal tract are iron deficiency anemia and bleeding. Hemangiomas can occur in any organ and cause a wide spectrum of symptoms. BRBNS can cause massive bleeding and even death. In this article we present a case of Bean's syndrome in a 7,5-year-old girl with bleeding from the lower gastrointestinal tract, which has been caused by hemangiomas located in the skin, large intestine, chest and armpit.

Clostridium difficile infection in newly diagnosed pediatric patients with inflammatory bowel disease: prevalence and risk factors.

BACKGROUND: Epidemiological and microbiological data suggest that Clostridium difficile infection (CDI) plays a substantial role in the clinical initiation of inflammatory bowel disease (IBD). The aim of the present study was to investigate the prevalence and risk factors of CDI in newly diagnosed pediatric patients with IBD. METHODS: The current investigation was a retrospective study. All patients newly diagnosed with IBD in the pediatric gastroenterology clinic in Warsaw between 2007 and 2010 were included in the present study. The patients were diagnosed according to Porto criteria and microbiology evaluation screening tests for CDI were conducted. Risk factors including prior hospitalization, use of antibiotics within 2 months of CDI detection, colonic involvement, and the duration of symptoms were evaluated. CDI diagnosis was based on a positive stool enzyme immunoassay. RESULTS: In the present study, 134 patients were evaluated (54 patients with Crohn's disease, and 80 with ulcerative colitis; 87% of the patients had colonic disease). The average age of the patients was 12.3 years, and the prevalence of CDI was 47% (95% confidence interval [CI], 38%-56%). Significant differences in the prevalence of CDI between patients with Crohn's disease and ulcerative colitis (P = 0.72; odds ratio [OR] = 1.187, 95% CI, 0.56-2.52) were not observed. The risk of CDI was associated with an increase in the age of the patient and the severity of the disease. CONCLUSIONS: The prevalence of CDI in newly diagnosed IBD patients was high and was independent of the type of disease.