Evaluation of the infliximab therapy of severe form of pediatric Crohn's disease in Poland: Retrospective, multicenter studies.

BACKGROUND: Registration of infliximab in Poland has increased chances to induce clinical remission and mucosal healing in the severe form of pediatric Crohn's disease. OBJECTIVES: The aim of this retrospective study was to assess the results and safety of infliximab therapy in the severe form of pediatric Crohn's disease. MATERIAL AND METHODS: The study included 153 children with severe form of non-fistulizing Crohn's disease treated with infliximab. The clinical activity of Crohn's disease was assessed according to PCDAI scale, endoscopic scoring was graded according to SES-CD, body mass was measured with body mass index (BMI). Infliximab was administered at the dose 5 mg/kg body mass in the 0.2 and 6th week, and then, after clinical response, every 8 for the period of 12 months. RESULTS: One hundred thirty-six children (88.89%) achieved clinical response after induction therapy and 75.21% of children after the maintenance therapy. 39.68% of children achieved remission as graded with endoscopic scoring SES-CD. There was a statistically significant increase in body weight following the treatment. Side effects such as anaphylaxis, rash, and the activation of EBV infection appeared in 9 children at the time of infliximab injection. In other children the drug was well tolerated. CONCLUSIONS: Induction and maintenance therapy with infliximab resulted in clinical remission of Crohn's disease in 75.21% of children, and in the intestinal mucosa healing in 39.68% of children.

Induction and maintenance infliximab therapy in children with moderate to severe ulcerative colitis: Retrospective, multicenter study.

BACKGROUND: Pediatric ulcerative colitis (UC) is a severe disease characterised by the presence of extensive inflammatory lesions in the colon. The administration of intravenous corticosteroids is recommended in patients with acute relapse of the disease, whereas early treatment with cyclosporine, tacrolimus or infliximab is recommended if there is no improvement. OBJECTIVES: The aim of this study was to retrospectively evaluate the efficacy and safety of infliximab therapy in the treatment of moderate-to-severe and severe relapse of pediatric UC. MATERIAL AND METHODS: The analysis included 42 children aged 4-18 years (23 girls, 19 boys) treated in 7 pediatric gastroenterology departments in Poland during the past 4 years. The disease duration ranged from 2 to 100 months. The clinical activity of UC ranged from 35 to 85 points according to the PUCAI scale. Twenty-one children were diagnosed with pancolitis, 10 children with extensive UC, and the remaining with the left-sided UC. In the induction therapy infliximab was administered at doses of 5 mg/kg in the 0.2 and 6 weeks, and after the clinical response every 8 weeks to 12 months. Treatment results were assessed in 10 and 54 weeks. RESULTS: After the induction therapy the clinical response was achieved in 14 children (33.33%) and clinical remission in 11 children (26.19%). Two children required surgical treatment, and the remaining 2 suffered from anaphylactic shock. After the maintenance therapy clinical remission was maintained in 12 children (57.14%), whereas 3 children required surgery (colectomy). CONCLUSIONS: Infliximab therapy in children with moderate-to-severe UC induces remission and, in some children, proves to be effective in preventing early colectomy.

Gastrointestinal disorders next to respiratory infections as leading symptoms of X-linked agammaglobulinemia in children - 34-year experience of a single center.

INTRODUCTION: Respiratory tract infections constitute the most frequent manifestation of X-linked agammaglobulinemia (XLA). There are not many papers elucidating gastrointestinal (GI) disorders, including inflammatory bowel disease (IBD), in such patients. The aim of the study was to evaluate the occurrence of gastrointestinal disorders and IBD compared to respiratory tract infections in XLA individuals. MATERIAL AND METHODS: Of 1563 patients with primary immunodeficiencies diagnosed in the Department of Immunology, the Children's Memorial Health Institute (CMHI), 66 boys had a provisional diagnosis of agammaglobulinemia. Forty-four subjects fulfilled definitive ESID (European Society for Immunodeficiencies) diagnostic criteria of XLA. A retrospective analysis of medical history of XLA patients was undertaken. RESULTS: Recurrent respiratory tract infections, particularly bronchitis (73%) and pneumonia (59%), were the most common symptoms of XLA. The GI disorders constituted the next main manifestation (63.6%), followed by upper respiratory tract infections. Twenty-six of 28 XLA patients with GI disorders complained of diarrhea, which was resolved generally after immunoglobulin therapy introduction. Single but prolonged episodes of Campylobacter jejuni diarrhea were reported in two individuals. Inflammatory bowel disease of mild to moderate activity was diagnosed in 1 patient, and local enteritis of mild activity in another one. CONCLUSIONS: Gastrointestinal disorders were one of the main manifestations of XLA, reported almost as often as lower respiratory tract infections. The most common GI symptom was diarrhea, which usually resolved after immunoglobulin therapy was started. Infections caused by Giardia lamblia were reported occasionally. Inflammatory bowel disease was diagnosed quite exceptionally, which presumably may be connected with normal T cell immunity.

Comparison of 72-hour fecal fat quantification and the 13C-mixed triglyceride breath test in assessing pancreatic exocrine sufficiency in children with chronic pancreatitis.

INTRODUCTION: Chronic pancreatitis (CP) in children is still a rare, although increasingly recognized entity. Over the duration of the disease several complications can be observed, two of which are major ones: endo- and exocrine insufficiency. In the medical care of children with CP it is crucial to diagnose the decreased endo- and exocrine function of the pancreas, in order to preserve patients from malnutrition and the failure to thrive. The aim of the study was to compare the usefulness of two indirect methods of assessing the pancreas exocrine function in children with CP. MATERIAL AND METHODS: Ninety one patients with CP were enrolled in the study (41 boys, 50 girls, aged 2-17.8 years). Only Patients who had had both the 72-hour fecal fat quantification and the 13C-mixed triglyceride breath test (13C -MTBT) performed were selected. We compared the results of both tests for sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) in detecting exocrine pancreatic insufficiency. RESULTS: Out of 91 patients, 12 were diagnosed with exocrine pancreatic insufficiency (EPI). The sensitivity of the fecal fat quantification was 50%, the specificity for the test was 100%. PPV and NPV were 100% and 93%, respectively. 13C-MTBT had the sensitivity of 42% and the specificity of 99%. PPV and NPV for the breath test were of 83% and 92%, respectively. No statistically significant discrepancy between the values obtained was found. CONCLUSIONS: Although the 72-hour fecal fat quantification remains the gold standard in detecting EPI, both of the methods that had been investigated were shown to be comparable regarding sensitivity, specificity, PPV and NPV in assessing pancreas exocrine sufficiency in children with CP. Due to the easier execution of the breath test, both for the patient and for medical personnel, its importance may increase.

Evaluation of the effectiveness of biofeedback therapy for functional constipation in children.

BACKGROUND/AIMS: Defecation disorders are one of the most common problems in pediatric gastroenterology. Treatment includes changes in the diet, pharmacotherapy, and biofeedback therapy. The aim of the present study was to evaluate the effectiveness of biofeedback therapy as assessed by clinical improvement as well as by changes in manometric parameters in children with constipation and pelvic floor dyssynergia (PFD). MATERIALS AND METHODS: A total of 44 children with constipation and PFD hospitalized between 2000 and 2015 were enrolled in this retrospective study. All patients underwent anorectal manometry, and in case of diagnosed PFD, the patient qualified for biofeedback therapy. Amplitudes between extreme and basic pressures during defecation maneuvers in the first and last sessions as well as the difference between them were compared between groups with and without clinical improvement after the last session. RESULTS: A clinical improvement was achieved in 38 (86%) patients. There were no significant differences found in the amplitudes in the first session (mmHg), 94, 65, 115 vs. 112, 55, 170 (median, first quartile, third quartile, respectively; NS: not significant); last session, 36, 27, 52 vs. 41, -38, 66, respectively; or between them, 71, 11, 124 vs. 81, 17, 109, respectively, in the group with versus the group without clinical improvement, respectively. CONCLUSION: Biofeedback therapy has high clinical efficacy, and despite the lack of manometric improvement, it should be used as a treatment method in children in whom dietary and pharmacological procedures do not work, even if we consider the exercises more as a form of psychological training.

Induction and Maintenance Infliximab Therapy for the Treatment of Crohn's Disease with Perianal Fistulas in Children: Retrospective, Multicenter Study.

BACKGROUND: Infliximab is a biological drug used for the treatment of Crohn's disease in children. OBJECTIVES: The aim of this retrospective study was the estimation of effectiveness and safety of infliximab in the treatment of Crohn's disease with perianal fistulas in children. MATERIAL AND METHODS: Analysis comprised 50 children with Crohn's disease with perianal fistulas aged 9 to 18 years (16 girls and 34 boys) who failed to respond to conventional therapy. The children were divided into two groups: the first group contained 23 children with simple fistulas and the second - 27 children with complex fistulas. All children were treated with infliximab, administered in the dose of 5 mg per kilogram of the body mass. In the induction phase infliximab was administered at weeks 0, 2 and 6 and after clinical response in maintenance phase the drug was administered every 8 weeks; together for 12 months. RESULTS: In 76% of children after induction therapy with infliximab and in 71.87% after maintenance therapy the complete closure of fistula occurred. During the first year after the treatment a recurrence of a fistula was observed in 30.43% of the children. In two children anaphylactic shock was observed during injection of infliximab. The remaining children tolerated the drug well. CONCLUSIONS: The treatment with infliximab was effective in the majority of fistulazing Crohn's disease and caused the closure of perianal fistula which improved quality of life.

The clinical course of hereditary pancreatitis in children - A comprehensive analysis of 41 cases.

BACKGROUND: Available data from adult patients do not reflect natural course of hereditary pancreatitis (HP) in children. To date, no study has assessed the clinical course of HP in children. OBJECTIVE: To investigate the clinical course of HP in children and compare it to non-HP group with chronic pancreatitis (CP). METHODS: A group of 265 children with CP, hospitalized from 1988 to 2014, were enrolled in the study. Medical records of those patients were reviewed for data on presentation, diagnostic findings and treatment. All children were screened for mutations in major pancreatitis-associated genes, i.e. PRSS1, SPINK1, and CFTR. RESULTS: HP was diagnosed in 41 children (15.5%). Family history was positive in 88% of children with HP. Mutations of PRSS1 gene were found in 80% (33/41) of HP patients. We detected p.R122H, p.R122C, p.N29I, and p.E79K mutation in 34% (14/41), 27% (11/41), 12% (5/41), and 7% (3/41) of HP patients, respectively. Patients with paternal inheritance had first symptoms earlier than those with maternal inheritance (5.9 vs. 9.1 years; P < 0.05). Children with HP showed more severe changes in ERCP then those from non-HP group (2.05 Cambridge grade, vs. 1.6°; P < 0.05). ESWL was performed more frequently in HP group (12.2% vs. 3.1%; P < 0.05). There was no difference in age of disease onset (7.98 vs. 8.9 years; NS), pancreatic duct stenting (46.3% vs. 33%; NS), or number of surgical interventions (12.2% vs. 14.3%; NS) between both groups. CONCLUSIONS: Children with HP reveal significantly more severe clinical presentation of the disease than non-HP patients, despite the same age of onset.

MRCP Versus ERCP in the Evaluation of Chronic Pancreatitis in Children: Which Is the Better Choice?

OBJECTIVES: To evaluate the diagnostic accuracy of magnetic resonance cholangiopancreatography (MRCP) in the detection of chronic pancreatitis (CP)-specific changes in the pediatric population. METHODS: The study included 48 children with pancreatic disorders subjected to both endoscopic retrograde cholangiopancreatography (ERCP) and MRCP within a 1- to 4-month interval. The sensitivity, specificity, positive predictive value, and negative predictive value of MRCP in the detection of CP-specific changes were determined using ERCP as a diagnostic standard. RESULTS: Diagnostic ERCP pancreatograms were obtained in 41 (85.4%) of 48 patients and diagnostic MRCP images in all 48 children. The sensitivity and positive predictive value of MRCP were 77.1% and 90%, respectively, and its specificity and negative predictive value amounted to 50% and 27.3%, respectively. The patients with consistent results of MRCP and ERCP (ie, true-positive and true-negative cases) and individuals with incompatible results of the tests (ie, false-positive and false-negative cases) differed in terms of their median age at MRCP (14.17 vs 10.33 years) and median CP stage according to the Cambridge Scale (4 vs 2). CONCLUSIONS: Magnetic resonance cholangiopancreatography provides diagnostic information equivalent to ERCP in a large percentage of pediatric patients with CP and should be used as the imaging method of choice, especially if the likelihood of therapeutic intervention is low.

Autoimmune markers in children with chronic pancreatitis.

INTRODUCTION: In the last decade we can observe a gradual increase in the incidence of autoimmune diseases. The aetiology of chronic pancreatitis (CP) in children is varied and includes gene mutations, anatomic anomalies and others. The reported paediatric experience with chronic CP is scarce and little is known about the role of autoimmune pancreatitis (AIP). AIM: To assess the frequency of autoimmune markers in children with CP. MATERIAL AND METHODS: One hundred and twenty-nine children hospitalised between 2005 and 2012 at the Department of Gastroenterology, The Children's Memorial Health Institute, were examined for the presence of AIP; the level of IgG4 was determined, and tests for anti-tissue antibodies (ANA, ASMA, AMA, ANCA, AHA) were conducted. Clinical data were recorded and analysed. RESULTS: Anti-tissue antibodies were detected in 75/129 children (58%), and 24/68 patients (35.3%) showed an increased IgG4 level. Based on the International Association of Pancreatology criteria, a suspicion of AIP was raised in 6 patients (4.6%). We found gene mutations predisposing to CP in 32/75 (42.6%) patients with autoimmune markers. In 16/75 children (21.3%), anatomic anomalies were found. There was no difference in the severity of the disease and clinical course between children with evidence of autoimmune process and patients without autoimmune markers (p = NS). CONCLUSIONS: In children with CP, similarly to adults, there is a high frequency of biochemical markers of autoimmunity. It is worth remembering that AIP can occur in children.

[Biological treatment of inflammatory bowel diseases in children in the years 2004-2013 in Poland]. / Leczenie biologiczne nieswoistych zapalen jelit u dzieci w latach 2004-2013 w Polsce.

UNLABELLED: In the last years an increase in Crohn's disease morbidity in children is observed together with constant morbidity of ulcerative colitis. The course of these diseases is severe, younger children are affected and the diseases are resistant to conventional treatment. Biological drugs are a chance for a longer remission and healing of the intestinal mucosa. OBJECTIVE OF THE WORK: Assessment of the use of biological drugs in treatment of inflammatory bowel disease in Poland was the objective of the work. MATERIAL AND METHODS: Gastroenterological centers treating inflammatory bowel disease during the years 2004-2013 were invited to a questionnaire retrospective study. RESULTS: The questionnaires of biological treatment of Crohn's disease and ulcerative colitis in children were received from 12 centers. In the years 2004-2013 the number of children aged 4 months to 18 years with Crohn's disease treated with biological drugs was 424. In the years 2004-2008--69 children were treated with infliximab and in the years 2009-2013--299 children, which was a four-fold increase. 56 children were treated with adalimumab in the years 2008-2013. In the years 2005-2013--72 children with ulcerative colitis were treated with infliximab and 11 with adalimumab. The age of the children ranged from 2 years to 18 years. The higher number of children treated was in the years 2009-2013: 59 with infliximab and 10 with adalimumab. CONCLUSIONS: In the last decade a significant increase on the number of children with Crohn's disease and ulcerative colitis treated with biological drugs was observed. It is connected not only to greater morbidity but above all to the introduction of a treatment program by the National Health Insurance Fund for children with Crohn's disease. There is an expectation that the introduction of biological treatment in inflammatory bowel disease will prolong clinical and endoscopic remission and diminish the number of surgeries.

Efficiency of pancreatic duct stenting therapy in children with chronic pancreatitis.

BACKGROUND: Chronic pancreatitis (CP) is a rare disease in childhood. Although ERCP is commonly performed in children, the effect of pancreatic duct stenting therapy in children with CP is unknown. OBJECTIVE: To investigate the efficacy of pancreatic duct stenting in children with CP. DESIGN: Retrospective analysis. SETTING: National referral center. PATIENTS: A total of 208 children with CP hospitalized between 1988 and 2012. INTERVENTIONS: ERCP with pancreatic duct stenting. MAIN OUTCOME MEASUREMENTS: Results of endoscopic therapy and number of pancreatitis episodes per year before and after treatment. RESULTS: A total of 223 pancreatic duct stenting procedures were performed in 72 children. The median number of stent replacements was 3 (range 1-21). A statistically significant decrease in the number of pancreatitis episodes per year was observed: from 1.75 to 0.23 after endoscopic treatment (P < .05). Pancreatic duct stenting was performed more frequently in patients with hereditary pancreatitis (61.5%) and in children with CP and anatomic anomalies of the pancreatic duct (65%; P < .05). LIMITATIONS: Retrospective analysis with the assessment of adverse events based on medical history. CONCLUSION: Pancreatic duct stenting therapy is a safe and effective procedure in children with CP. This therapy should be recommended especially for children with hereditary pancreatitis and patients with anatomic anomalies of the pancreatic duct.

Ileal exclusion in children with progressive familial intrahepatic cholestasis.

OBJECTIVES: Children with progressive familial intrahepatic cholestasis (PFIC) rarely benefit from medical treatment and most patients require surgical intervention. Partial external biliary diversion (PEBD) is presently the treatment of choice but for those who cannot benefit from PEBD, an alternative surgical procedure--ileal exclusion (IE)--was introduced. The aim of this study was to analyze our experience with IE in children with PFIC. METHODS: This procedure was performed in 9 patients (6 girls, 3 boys) at the median age of 11 years (range 8-21). In 4 children, it was the primary operation (group 1), and in 5, IE was performed after PEBD (group 2). All of the patients were screened for ABCB11 and ATP8B1 mutations, and in 3 cases, PFIC type 2 was confirmed. RESULTS: Median follow-up after IE surgery was 8.5 years (range 3-14). In group 1, 1 patient had to be converted to PEBD and the remaining 3 children experienced alleviation in pruritus and decrease in bilirubin and bile acids concentrations 2 and 5 years after IE. After 10 years, only 2 children were still accessible for follow-up. In both, pruritus varied and elevated serum bile acids were observed. Of the 5 patients who underwent IE after PEBD, 1 eventually required liver transplantation, 1 developed varying degree of pruritus, and 3 female patients, operated on because of aesthetic reasons, had excellent outcomes. CONCLUSIONS: IE is an alternative rescue option to PEBD and should be offered cautiously, only to patients who cannot benefit from PEBD.

Candidaemia in a Polish tertiary paediatric hospital, 2000 to 2010.

Data on the epidemiology of invasive Candida infections in paediatric patients in Europe are still limited. The aim of this retrospective study was to analyse the epidemiology of candidaemia in a tertiary paediatric hospital in Poland from 2000 to 2010. Using microbiological records, a total of 118 episodes of candidaemia were identified in 114 children, with an annual incidence of 0.35 episodes/1000 discharges. The highest incidences were found in the medical intensive care unit (5.28), and in neonatal intensive care (1.47). The mortality rate was 8.5%. Candida albicans and C. parapsilosis were the most prevalent species (39.8% and 35.6% respectively). The prevalence of non-albicans species increased from 12.5% in 2000 to 70% in 2010. No differences were found between C. albicans and C. non-albicans episodes in terms of demographics, risk factors or mortality. The highest resistance rates (overall 7.6%) were observed for fluconazole (4.3% in C. albicans, 7.1% in C. parapsilosis and 13.8% in other Candida species). Resistance to amphotericin B (2.5%) was limited to non-albicans isolates. The dynamic changes in species distribution and increasing resistance of fungal pathogens confirm the importance of epidemiological surveillance.

The opinions of Polish nurses and patients on nursing protests.

The aim of this study is to explore nurses' and patients' opinions about nurses in Poland going on strike. The study was carried out in Poland between January and June 2009, using 150 nurses and 150 hospitalized patients. The study was conducted using two questionnaire surveys. The main reasons why nursing strikes are organized, in the opinions of nurses, are: higher wages, the improvement of working conditions and the improvement of the image of the nursing profession. The main reasons why nursing strikes are organized, in the opinions of patients, are: higher wages, not abiding standards of employment by government and the improvement of the image of the nursing profession. The main reasons for a lack of active participation in strikes are holidays and occupational and economic matters. Patients and nurses support nursing strikes. Both nurses (53.3%) and patients (42%) said that organizing nursing strikes is right and might improve the occupational situation of nurses.

Distribution of HCV genotypes in Poland.

UNLABELLED: Available data on prevalence of HCV genotypes in Poland are insufficient. The aim of the study was the analysis of distribution of HCV genotypes in Poland over the period of recent 10 years regarding the age of patients and the regions of the country. MATERIAL AND METHODS: Analysis of HCV genotypes in Poland was carried out between 2003 and 2012, and included 14 651 patients from 22 centers where patients with chronic viral hepatitis C are diagnosed and treated. Genotypes were analyzed in age groups (< 20 years of age, 20-40 years of age, > 40 years of age) as well as in populations of HBV and HIV co-infections. RESULTS: Genotype (G) 1 infection was demonstrated in 79.4%, G2 -0.1%, G3- 13.8%, G4- 4.9%, G6-0.09% and mixed infections in 1.6%. There was no infection with genotype 5. The highest prevalence of G1 was observed in the Lódzkie voivodship (89.2%) and the Slaskie voivodship (86.7%) while the lowest one in the Warminsko-mazurskie (62.0%) and the Podlaskie voivodships (68.2%). Genotype 3 most commonly occurs in the Warminsko-mazurskie (28.1%), and the Podlaskie voivodships (23.0%) and is least common in the Malopolskie (7.9%) and the Lódzkie voivodships (9.0%). Genotype 4 is more common in the Kujawsko-pomorskie (11.7%) and the Podlaskie voivodships (8.6%) and relatively less common in the Lubelskie (1.1%) and the Lódzkie voivodships (1.8%). Prevalence of G1 infection in 2003-2004 was 72% and increased up to 85.6% in 2011-2012, that was accompanied by decrease of G3 prevalence from 17% to 8% in this period. In HBV co-infected (n = 83), G1 infection was demonstrated in 85.5%, G3 - in 7.2%, G4 -4.8%, and mixed genotypes in 6%. Among HIV co-infected (n = 391), a much lower prevalence of G1 (33.0%) and a high of G3 (40.4%) as well as G4 (24.0%) were observed. CONCLUSIONS: There is a geographic variability of HCV genotypes prevalence in Poland. Increase of HCV G1 infections and decrease of G3 and G4 were observed in the last 10 years. Genotypes G3 and G4 occur more often in HCV/HIV co-infected than in HCV mono-infected patients.

[Nutritional guidelines for healthy children aged 1-3 years - Polish Expert Group statement. Part I- energy and nutritional components demand]. / Normy zywienia zdrowych dzieci w 1-3. Rokuzycia . stanowisko polskiej grupy ekspertów.czesc i - zapotrzebowanie na energiei skladniki odzywcze.

Updating of the nutritional guidelines for the Polish population requires updates of the nutritional norms for children. We present the Polish Expert Group statement (2012) on intake of selected nutrients (protein, lipids, carbohydrates, vitamin D and E) essential in nutrition of children aged 1-3 years. For this purpose the Expert Group reviewed available scientific data: the recent guidelines, nutritional norms and recommendations, systematic reviews and expert opinions as well as original publications, in relation to the specific requirements of the Polish population.

The impact of infliximab induction therapy on mucosal healing and clinical remission in Polish pediatric patients with moderate-to-severe Crohn's disease.

OBJECTIVE: To assess the clinical efficacy and the impact of infliximab (IFX) induction therapy on mucosal healing in Polish children with Crohn's disease (CD). METHODS: A total of 66 children (29 boys and 37 girls) aged 14.06 ± 3.59 years with CD diagnosed at the mean age of 8.4 ± 7.3 years were included in the study. Patients received IFX (5 mg/kg) in three repeated infusions at 0, 2, and 6 weeks. The clinical activity of the disease was assessed using the Pediatric Crohn's Disease Activity Index (PCDAI) and the endoscopic activity was scored using the Simple Endoscopic Score for Crohn's disease at baseline and at week 10. RESULTS: Twenty-two (33%) of the studied patients reached clinical remission (PCDAI ≤ 10), 26 (39%) showed a clinical response (PCDAI between 15 and 30), and 18 (28%) did not respond to the therapy. When comparing data at baseline and at week 10, significant decreases were observed in the median PCDAI, C-reactive protein, and platelet count. In addition, a significant increase in BMI was noted. A significant decrease in the Simple Endoscopic Score for CD was observed between the initial and the control colonoscopies. Fifteen out of 66 patients (22.7%) had score 0 in the control endoscopy at week 10. No adverse events leading to therapy termination were observed. CONCLUSION: Biological therapy with IFX enables mucosal healing in pediatric patients with CD. Induction therapy with infliximab was found to be clinically effective in 72% of Polish pediatric patients with CD and induced a remission in 33% of them. Induction therapy with infliximab helps to increase BMI.

Bone metabolism in cholestatic children before and after living-related liver transplantation--a long-term prospective study.

Bone disorders are common in children with end-stage liver diseases, especially those associated with cholestasis. Abnormal hepatocyte function, disordered vitamin D metabolism and calcium-phosphorous homeostasis, malnutrition, and immunosuppressive treatment are potential risk factors of bone tissue pathology before and after transplantation. The aim of the study was to analyze the long-term effect of successful living-related liver transplantation (LRLTx) on skeletal status and bone metabolism in cholestatic children. Eighteen cholestatic children (1.4±0.5yr old; 12 females [F]/6 males [M]) qualified for LRLTx were analyzed; 16 (5F/11M) of them participated in long-term observation (V4). Serum levels of osteocalcin (OC), procollagen type 1 N-terminal propeptide (P1NP), cross-linked telopeptide of type 1 collagen (CTx), insulin-like growth factor I (IGF-I), IGF-I binding protein 3 (IGFBP-3), parathyroid hormone (PTH), 25-hydroxyvitamin D (25(OH)D), and 1,25-dihydroxyvitamin D (1,25(OH)(2)D) were assayed before (V0) and 6mo (V1), 12mo (V2), 18mo (V3), and 4.4yr (V4) after LRLTx. Total body bone mineral content (TBBMC) and total body bone mineral density (TBBMD) were measured by dual-energy X-ray absorptiometry (DXA) at the same pattern. Before LRLTx, the OC, P1NP, CTx, IGF-I, and IGFBP-3 levels as well as TBBMC and TBBMD were decreased compared with age-matched control group. The mean serum levels of 25(OH)D and 1,25(OH)(2)D were within reference ranges from V0 to V4. After LRLTx, the OC, P1NP, CTx, IGF-I, and IGFBP-3 as well as TBBMC and TBBMD reached the age-matched reference values. At V4, the level of P1NP decreased below and the PTH increased above the reference range that coincided with reduced Z-scores of both TBBMC (-1.11±1.24) and TBBMD (-1.00±1.19). P1NP and CTx, both measured at V3, correlated with IGF-I at V2 (R=0.86, p=0.014 and R=0.78, p=0.021, respectively) and PTH at V3 for P1NP and V1 for CTx (R=0.64, p=0.048 and R=0.54, p=0.038, respectively). The TBBMC changes between V0 and V4 correlated with IGF-I (R=0.68, p=0.015) and 1,25(OH)(2)D (R=0.54, p=0.025), both assayed at V1. The change of TBBMC Z-scores between V0 and V4 correlated with P1NP at V1 (R=0.69, p=0.002). The TBBMD changes between V0 and V4 correlated with CTx at V1 (R=0.54, p=0.027) and P1NP change between V0 and V1 (R=0.51, p=0.038). In short-term observation, successful LRLTx led to bone metabolism normalization triggered by probable anabolic action of IGF-I and PTH and manifested by TBBMC and TBBMD increases. In long-term horizon, moderately impaired DXA assessed bone status coincided with disturbances in bone metabolism. Bone metabolism markers, especially P1NP and CTx, appeared to be good predictors of changes in bone status evaluated by DXA.

Successful sirolimus rescue in tacrolimus-induced thrombotic microangiopathy after living-related liver transplantation.

TMA is a rare complication of tacrolimus. Disruption of endothelial cells, platelet aggregation, and intravascular mechanical fragmentation of red cells are core mechanisms of injury; however, exact pathways of toxicity are not clear. The clinical presentation may vary but TMA is a potentially life-threatening condition usually demanding aggressive treatment. We present the case of TMA in a child after living-related liver transplantation (LRLTx) on tacrolimus-based immunosuppressive regiment successfully converted to sirolimus.