ABSTRACT
Esophageal stenosis is a relatively uncommon condition in pediatrics and requires an accurate diagnostic approach. Here we report the case of a 9-month old female infant who presented intermittent vomiting, dysphagia and refusal of solid foods starting after weaning. She was treated for gastroesophageal reflux. At first, radiological investigation suggested achalasia, while esophagoscopy revelaed a severe congenital esophageal stenosis at the distal third of the esophagus. She underwent four endoscopic balloon dilatations that then allowed her to swallow solid food with intermittent mild dysphagia. After 17 months of esomeprazole treatment off therapy impedance-pH monitoring was normal. At 29 months of follow-up the child is asymptomatic and eats without problems.Infants with dysphagia and refusal of solid foods may have undiagnosed medical conditions that need treatment. Many disorders can cause esophageal luminal stricture; in the pediatric age the most common are peptic or congenital. Careful assessment with endoscopy is needed to diagnose these conditions early and referral to a pediatric gastroenterologic unit may be necessary.
Subject(s)
Esophageal Stenosis/congenital , Combined Modality Therapy , Deglutition Disorders/diagnosis , Deglutition Disorders/therapy , Diagnosis, Differential , Dilatation/methods , Esomeprazole/therapeutic use , Esophageal Stenosis/diagnosis , Esophageal Stenosis/therapy , Esophagoscopy , Female , Humans , Infant , Proton Pump Inhibitors/therapeutic useABSTRACT
BACKGROUND AND AIMS: Recent epidemiological studies showed an increase in ulcerative colitis among children, especially in its aggressive form, requiring surgical treatment. Although medical therapeutic strategies are standardized, there is still no consensus regarding indications, timing and kind of surgery. This study aimed to define the surgical management of paediatric ulcerative colitis and describe attitudes to it among paediatric surgeons. METHODS: This was a retrospective cohort study. All national gastroenterology units were invited to participate. From January 2009 to December 2013, data on paediatric patients diagnosed with ulcerative colitis that required surgery were collected. RESULTS: Seven units participated in the study. Seventy-one colectomies were performed (77.3% laparoscopically). Main surgical indications were a severe ulcerative colitis attack (33.8%) and no response to medical therapies (56.3%). A three-stage strategy was chosen in 71% of cases. Straight anastomosis was performed in 14% and J-pouch anastomosis in 86% of cases. A reconstructive laparoscopic approach was used in 58% of patients. Ileo-anal anastomosis was performed by the Knight-Griffen technique in 85.4% and by the pull-through technique in 9.1% of patients. Complications after colectomy, after reconstruction and after stoma closure were reported in 12.7, 19.3 and 35% of cases, respectively. CONCLUSIONS: This study shows that there is general consensus regarding indications for surgery. The ideal surgical technique remains under debate. Laparoscopy is a procedure widely adopted for colectomy but its use in reconstructive surgery remains limited. Longer follow-up must be planned to define the quality of life of these patients.
Subject(s)
Attitude of Health Personnel , Colitis, Ulcerative/surgery , Gastroenterology , Proctocolectomy, Restorative/methods , Adolescent , Child , Child, Preschool , Colitis, Ulcerative/drug therapy , Colostomy/adverse effects , Defecation , Drug Resistance , Fecal Incontinence/etiology , Female , Humans , Italy , Male , Pouchitis/etiology , Proctocolectomy, Restorative/adverse effects , Retrospective Studies , Severity of Illness IndexABSTRACT
Children and adolescents with Crohn's disease (CD) present often with a more complicated disease course compared to adult patients. In addition, the potential impact of CD on growth, pubertal and emotional development of patients underlines the need for a specific management strategy of pediatric-onset CD. To develop the first evidenced based and consensus driven guidelines for pediatric-onset CD an expert panel of 33 IBD specialists was formed after an open call within the European Crohn's and Colitis Organisation and the European Society of Pediatric Gastroenterolog, Hepatology and Nutrition. The aim was to base on a thorough review of existing evidence a state of the art guidance on the medical treatment and long term management of children and adolescents with CD, with individualized treatment algorithms based on a benefit-risk analysis according to different clinical scenarios. In children and adolescents who did not have finished their growth, exclusive enteral nutrition (EEN) is the induction therapy of first choice due to its excellent safety profile, preferable over corticosteroids, which are equipotential to induce remission. The majority of patients with pediatric-onset CD require immunomodulator based maintenance therapy. The experts discuss several factors potentially predictive for poor disease outcome (such as severe perianal fistulizing disease, severe stricturing/penetrating disease, severe growth retardation, panenteric disease, persistent severe disease despite adequate induction therapy), which may incite to an anti-TNF-based top down approach. These guidelines are intended to give practical (whenever possible evidence-based) answers to (pediatric) gastroenterologists who take care of children and adolescents with CD; they are not meant to be a rule or legal standard, since many different clinical scenario exist requiring treatment strategies not covered by or different from these guidelines.
Subject(s)
Adrenal Cortex Hormones/therapeutic use , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Crohn Disease/therapy , Enteral Nutrition , Immunosuppressive Agents/therapeutic use , Maintenance Chemotherapy/methods , Remission Induction/methods , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Adalimumab , Adolescent , Adrenal Cortex Hormones/adverse effects , Algorithms , Aminosalicylic Acids/therapeutic use , Anti-Bacterial Agents/therapeutic use , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Azathioprine/therapeutic use , Child , Humans , Infliximab , Mercaptopurine/therapeutic use , Methotrexate/therapeutic use , Thalidomide/therapeutic useABSTRACT
Therapeutic drug monitoring (TDM) of major metabolites of thiopurine drugs is a widely used tool for assessing treatment efficacy and toxicity in patients with inflammatory bowel disease (IBD). We report the laboratory and clinical validation of a simple and reliable high performance liquid chromatography (HPLC) method for the measurement of 6-thioguanine nucleotides (6-TGN) and 6-methylmercaptopurine (6-MMP) on paediatric patients with IBD. The aim of this paper is to develop and validate a method for the measurement of 6-TGN and 6-MMP applicable to routine practice and to evaluate the usefulness of the TDM of thiopurine drugs in children with IBD attending our Gastroenterology Unit. The HPLC method was validated following international guidelines starting from red blood cells (RBC) and whole blood (WB). A comparison between RBC and WB was assessed. The usefulness of TDM was then evaluated using the new method from WB in 47 paediatric patients with IBD treated with thiopurine drugs. WB and RBC resulted in interchangeable matrices. The majority of patients had the metabolite levels inside the therapeutic ranges. A moderate correlation was found between 6-MMP concentration and the dose of thiopurines. A higher percentage of non responders was found among patients with lower levels of 6-TGN. Toxicity was found in eight patients and was evaluated in respect to the metabolite concentration. The described HPLC method is applicable to routine practice and it is suitable for its use in multicentric studies. Our results of TDM on paediatric IBD patients can contribute to clarify its role in their therapeutic management.
Subject(s)
Anti-Inflammatory Agents/pharmacokinetics , Chromatography, High Pressure Liquid , Drug Monitoring/methods , Gastrointestinal Agents/pharmacokinetics , Inflammatory Bowel Diseases/drug therapy , Mercaptopurine/analogs & derivatives , Thioguanine/pharmacokinetics , Adolescent , Age Factors , Anti-Inflammatory Agents/blood , Anti-Inflammatory Agents/therapeutic use , Biotransformation , Child , Child, Preschool , Erythrocytes/metabolism , Female , Gastrointestinal Agents/blood , Gastrointestinal Agents/therapeutic use , Humans , Infant , Inflammatory Bowel Diseases/blood , Italy , Male , Mercaptopurine/blood , Mercaptopurine/pharmacokinetics , Mercaptopurine/therapeutic use , Reproducibility of Results , Thioguanine/blood , Thioguanine/therapeutic useABSTRACT
Chronic intestinal pseudo-obstruction (CIPO) can occur as a consequence of neuropathies including diffuse Intestinal Neuronal Dysplasia (IND), a relatively rare enteric nervous system (ENS) abnormality. Although various authors reported of diffuse IND associated either with intestinal malrotation or megacystis, the co-existence of these three entities in the same patient has never been described before. The aim of this paper is to report for the first time in literature a series of patient with such association, focusing on one who carries a de novo duplication of chromosome 12, suggesting a new syndromic association (megacolon, megacystis, malrotation).
Subject(s)
Abnormalities, Multiple/genetics , Enteric Nervous System/abnormalities , Fetal Diseases/diagnosis , Gastrointestinal Tract/abnormalities , Megacolon/diagnosis , Torsion Abnormality/diagnosis , Child, Preschool , Chromosome Duplication , Chromosomes, Human, Pair 12/genetics , Comparative Genomic Hybridization , Duodenum/abnormalities , Fatal Outcome , Female , Fetal Diseases/genetics , Fetal Diseases/therapy , Gastrointestinal Tract/surgery , Humans , Ileostomy , Megacolon/genetics , Megacolon/surgery , Syndrome , Torsion Abnormality/genetics , Torsion Abnormality/surgery , Urinary Bladder/abnormalitiesABSTRACT
BACKGROUND: The purpose was to assess in Italy the clinical features at diagnosis of inflammatory bowel disease (IBD) in children. METHODS: In 1996 an IBD register of disease onset was established on a national scale. RESULTS: Up to the end of 2003, 1576 cases of pediatric IBD were recorded: 810 (52%) ulcerative colitis (UC), 635 (40%) Crohn's disease (CD), and 131 (8%) indeterminate colitis (IC). In the period 1996-2003 an increase of IBD incidence from 0.89 to 1.39/10(5) inhabitants aged <18 years was observed. IBD was more frequent among children aged between 6 and 12 years (57%) but 20% of patients had onset of the disease under 6 years of age; 28 patients were <1 year of age. Overall, 11% had 1 or more family members with IBD. The mean interval between onset of symptoms and diagnosis was higher in CD (10.1 months) and IC (9 months) versus UC (5.8 months). Extended colitis was the most frequent form in UC and ileocolic involvement the most frequent in CD. Upper intestinal tract involvement was present in 11% of CD patients. IC locations were similar to those of UC. Bloody diarrhea and abdominal pain were the most frequent symptoms in UC and IC, and abdominal pain and diarrhea in CD. Extraintestinal symptoms were more frequent in CD than in UC. CONCLUSIONS: The IBD incidence in children and adolescents in Italy shows an increasing trend for all 3 pathologies. UC diagnoses exceeded CD.
Subject(s)
Colitis, Ulcerative/epidemiology , Crohn Disease/epidemiology , Adolescent , Age of Onset , Child , Female , Humans , Italy/epidemiology , Male , Prognosis , RegistriesABSTRACT
BACKGROUND: Thiopurines are used in the treatment of inflammatory bowel disease. They are metabolised via methylation by thiopurine-S-methyltransferase (TPMT), which displays a genetically determined polymorphic activity. Subjects with reduced TPMT activity have a higher concentration of active thiopurine metabolites and may be at increased risk of bone-marrow suppression. AIMS: To evaluate the relevance of TPMT genotyping in the management of thiopurines therapy in inflammatory bowel disease patients. PATIENTS AND METHODS: Adverse effects and clinical response were determined retrospectively and correlated with TPMT genotype in 70 paediatric inflammatory bowel disease patients. RESULTS: Nineteen patients (27.1%) developed adverse effects; of the 51 who did not, 34 (66.7%) responded to treatment. Five patients (7.1%) were heterozygous for a variant TPMT allele; two of these (40%) were intolerant to thiopurines, compared to 17 of the 65 patients (26.2%) with a wild type gene (O.R. 1.88, 95% CI 0.29-12.2, p=0.61); among the 34 responders, the median dosage of the drug required to obtain remission was lower for mutated than for wild type patients (1.6mgkg(-1)day(-1) versus 2.0mgkg(-1)day(-1), p=0.043). CONCLUSIONS: There was no significant association between adverse effects of thiopurines and TPMT heterozygous genotype, but TPMT genotyping could be useful in establishing the most appropriate dose of thiopurines to start treatment.
Subject(s)
Azathioprine/therapeutic use , Immunosuppressive Agents/therapeutic use , Inflammatory Bowel Diseases/genetics , Mercaptopurine/therapeutic use , Methyltransferases/genetics , Adolescent , Adult , Azathioprine/adverse effects , Bone Marrow Diseases/chemically induced , Chemical and Drug Induced Liver Injury/etiology , Child , Child, Preschool , Dose-Response Relationship, Drug , Female , Genotype , Humans , Immunosuppressive Agents/adverse effects , Infant , Inflammatory Bowel Diseases/drug therapy , Male , Mercaptopurine/adverse effects , Pancreatitis/chemically induced , Polymorphism, GeneticABSTRACT
AIM: To assess the value of long-chain omega-3 fatty acids (FAs) supplementation in addition to amino-salicylic-acid (5-ASA) in pediatric patients with Crohn's disease (CD). METHODS: Thirty-eight patients (20 males and 18 females, mean age 10.13 years, range 5-16 years) with CD in remission were randomized into two groups and treated for 12 mo. Group I (18 patients) received 5-ASA (50 mg/kg/d)+ omega-3 FAs as triglycerides in gastro-resistant capsules, 3 g/d (eicosapentanoic acid, EPA, 400 mg/g, docosahexaenoic acid, DHA, 200 mg/g). Group II (20 patients) received 5-ASA (50 mg/kg/d)+olive oil placebo capsules. Patients were evaluated for fatty acid incorporation in red blood cell membranes by gas chromatography at baseline 6 and 12 mo after the treatment. RESULTS: The number of patients who relapsed at 1 year was significantly lower in group I than in group II (P<0.001). Patients in group I had a significant increase in the incorporation of EPA and DHA (P<0.001) and a decrease in the presence of arachidonic acids. CONCLUSION: Enteric-coated omega-3 FAs in addition to treatment with 5-ASA are effective in maintaining remission of pediatric CD.
Subject(s)
Anti-Inflammatory Agents, Non-Steroidal/administration & dosage , Crohn Disease/diet therapy , Crohn Disease/drug therapy , Fatty Acids, Omega-3/administration & dosage , Mesalamine/administration & dosage , Adolescent , Child , Child, Preschool , Crohn Disease/blood , Double-Blind Method , Erythrocyte Membrane/metabolism , Fatty Acids, Omega-3/blood , Female , Humans , MaleABSTRACT
BACKGROUND/PURPOSE: The majority of gastrooesophageal reflux (GER) manifestations in children are supraoesophageal, and "spitting/posseting" is "the tip of the iceberg" because most reflux episodes are not regurgitated. Aim of the present study was to prospectively evaluate the incidence of gastrooesophageal reflux and the incidence of antireflux surgery in patients with difficult-to-treat respiratory symptoms. PATIENTS AND METHODS: Five hundred and ninety-five children with difficult-to-treat respiratory symptoms were prospectively enrolled in a blind study looking for the correlation between clinical presentation (asthma or non-asthma), oesophageal pH monitoring, X-ray barium meal, broncho-alveolar lavage, necessity for surgery, and outcome. RESULTS: pH monitoring was anomalous in 47% of patients with asthma (group A) and in 43% of those who did not have asthma as main symptom (group B). Overall, 48 patients finally underwent anti-reflux surgery (8%) as anti-reflux medical treatment did not ensure stable benefits. No major surgical complications were experienced. Postoperatively, respiratory symptoms improved strongly (Visick 1) in 69% of cases, moderately (Visick 2) in 27%, while clinical worsening (Visick 4) was observed in 4%. CONCLUSIONS: The results of this study stress the importance of symptoms, clinical response to anti-reflux medical treatment and broncho-alveolar lavage compared to classical pH parameters in the decision-making process for patients with difficult-to-treat supraoesophageal symptoms. To date no single tool alone has proved to be diagnostic in these patients. Fundoplication is recommended only when a relationship between supraoesophageal symptoms and gastrooesophageal reflux is strongly suspected.
Subject(s)
Gastroesophageal Reflux/surgery , Asthma/etiology , Case-Control Studies , Child , Child, Preschool , Female , Fundoplication , Gastroesophageal Reflux/complications , Gastroesophageal Reflux/epidemiology , Humans , Hydrogen-Ion Concentration , Incidence , Male , Monitoring, Physiologic , Prospective Studies , Respiratory Tract Diseases/etiologyABSTRACT
BACKGROUND: Infliximab is an effective therapy in adult patients with refractory and fistulizing Crohn's disease. Experience in children is still limited. AIM: : To evaluate the experience in 22 children and adolescents treated with infliximab with refractory and/or fistulizing Crohn's disease, and to compare duration of response in children between early Crohn's disease and late Crohn's disease. METHODS: The experience in 22 children and adolescents treated with a total of 73 infusions was evaluated retrospectively. Treatment indication was refractory Crohn's disease in 9/22 patients, fistulizing Crohn's disease in 7/22 patients and both these conditions in 6/22. All patients with refractory Crohn's disease had late Crohn's disease (> 1 year), whereas 6/13 patients with fistulas had early disease (< 1 year). RESULTS: Mean Paediatric Crohn's Disease Activity Index (PCDAI) decreased from 41.2 to 16.2 at 4 weeks (P < 0.01), and to 15.4 at 18 weeks (P < 0.01). Mean PCDAI at 18 weeks in children with early Crohn's disease and late Crohn's disease was 5.5 and 18.1, respectively (P < 0.05). Complete closure of fistulas was obtained in 5/6 children with early Crohn's disease and in 2/7 children with late Crohn's disease. Immediate adverse reactions were observed in two children. CONCLUSIONS: Infliximab is a highly effective treatment in children and adolescents with both severe refractory or fistulizing Crohn's disease. Children with early Crohn's disease have a higher chance of prolonged response to infliximab than children with late Crohn's disease.
Subject(s)
Antibodies, Monoclonal/administration & dosage , Crohn Disease/drug therapy , Gastrointestinal Agents/administration & dosage , Intestinal Fistula/complications , Adolescent , Antibodies, Monoclonal/adverse effects , Child , Child, Preschool , Crohn Disease/complications , Female , Follow-Up Studies , Gastrointestinal Agents/adverse effects , Humans , Infliximab , Infusions, Intravenous , Intestinal Fistula/drug therapy , Male , Retrospective Studies , Treatment OutcomeABSTRACT
BACKGROUND: Esophageal achalasia is not a frequent disorder in children and different treatments have been proposed during past decades. This study reviews the results of the laparoscopic Heller-Dor procedure performed in pediatric patients in two different surgical units. METHODS: We included the patients aged <14 years with a minimum follow-up of 6 months operated on in the period 1994-2001. A single longitudinal anterior esophageal myotomy (Heller) and a 180 degrees anterior gastropexy (Dor) were laparoscopically performed. The patients were checked to detect intra- or postoperative complications and recurrence. RESULTS: Twenty children were operated on. Mean follow-up was 45 months (range 6-102). Postoperative clinical score was Visick 1 in 15 cases and Visick 2 in five. CONCLUSIONS: As complication and recurrence rates are very low we consider modified Heller myotomy and Dor gastropexy through a laparoscopic approach our first choice to treat esophageal achalasia in the pediatric population.
Subject(s)
Esophageal Achalasia/surgery , Laparoscopy/methods , Adolescent , Child , Child, Preschool , Esophageal Achalasia/diagnosis , Esophageal Perforation/etiology , Female , Humans , Laparoscopy/adverse effects , Male , Treatment OutcomeABSTRACT
AIM: To asses the efficacy and safety of ciclosporin in a paediatric population with inflammatory bowel disease. PATIENTS AND METHODS: Twenty-three Italian children treated with ciclosporin were studied retrospectively. The indications for treatment were severe unresponsive colitis, chronic active colitis or severe fistulizing Crohn's disease. The treatment duration, follow-up and causes of drug discontinuation were assessed. RESULTS: Sixteen patients were treated intravenously for a mean time of 10 +/- 7 days (1-24 days) and 19 orally for a mean time of 133 days (17-660 days). The mean follow-up of all patients was 13.2 months. Ciclosporin was totally ineffective, being discontinued for surgery, in nine of 23 patients (39%); it was discontinued for partial response in three patients (13%). During treatment, clinical remission was achieved in eight children (35%) and maintained after drug withdrawal in four (17%). In severe unresponsive colitis, urgent colectomy was avoided in 12 (85%) of 14 patients who tolerated the drug. Side-effects appeared in six of 23 patients (26%), and three (13%) required ciclosporin to be discontinued due to neurotoxicity. CONCLUSIONS: Ciclosporin shows disappointing long-term results in the treatment of refractory inflammatory bowel disease, but can play an important role in preventing urgent surgery in unresponsive severe colitis. Severe side-effects can occur.
Subject(s)
Cyclosporine/therapeutic use , Immunosuppressive Agents/therapeutic use , Inflammatory Bowel Diseases/drug therapy , Adolescent , Child , Colectomy , Cyclosporine/adverse effects , Elective Surgical Procedures , Female , Follow-Up Studies , Humans , Immunosuppressive Agents/adverse effects , Inflammatory Bowel Diseases/surgery , Male , Retrospective Studies , Treatment Failure , Treatment OutcomeABSTRACT
BACKGROUND: The laparoscopic approach has become increasingly popular for fundoplication over the last few years; however many surgeons are skeptical about its real advantages. METHODS: We conducted a prospective comparative study of children operated on for gastroesophageal reflux (GER). Exclusion criteria included age <1 YEAR AND >14 years, previous surgery on the esophagus or stomach, and neurologic impairment. We compared two groups of patients who met the same inclusion/exclusion criteria. One group was treated via a laparotomic approach between January 1993 and December 1997; the other was treated via a laparoscopic approach between September 1998 and December 2000. A 360 degrees wrap was performed in each group. RESULTS: Group 1 (laparotomic approach) included 17 patients; mean operative time was 100 min and postoperative time was 7 days. Group 2 comprised 49 children operated on via a laparoscopic approach; mean operative time was 78 min and postoperative time was 48 hours. No major complications were encountered in either group. In postoperative period, two patients in group 1 had complications. One had a prolonged bout of gastroplegia, which required nasogastric drainage, and then recovered spontaneously after 20 days; the other had stenosis of the wrap, which required dilation. No relapses occurred during a follow-up of 6 months. Long-term follow-up data are not presented. Comparative analysis of the short-term functional results indicated that there were no differences between the two groups. CONCLUSION: This study confirms that the minimally invasive approach is safe and effective for the treatment of primary gastroesophageal reflux disease in children.
Subject(s)
Gastroesophageal Reflux/surgery , Laparoscopy/methods , Child , Child, Preschool , Fundoplication/methods , Humans , Prospective StudiesABSTRACT
AIM: To assess the efficacy and safety of azathioprine in a paediatric population with inflammatory bowel disease. PATIENTS AND METHODS: One hundred and twenty-three Italian children treated with azathioprine were studied retrospectively. The treatment duration and causes of its discontinuation, side-effects and variation in corticosteroid dose were assessed. RESULTS: The mean age at inflammatory bowel disease diagnosis was 9.8 +/- 3.6 years, and at the start of azathioprine therapy 11.8 +/- 4.3 years. The mean duration of treatment was 19 +/- 16 months. Fifty patients (41%) stopped treatment due to surgery (12%), prolonged remission (11%), non-response (7%), severe side-effects (7%) and poor compliance (3%). Of the 73 patients (59%) remaining on azathioprine, 11 had never been treated with corticosteroids, 27 were able to stop them and 35 were still on a very low daily dose (91% < 0.3 mg/kg). The difference in the daily corticosteroid dose between the beginning of azathioprine treatment (1 +/- 0.6 mg/kg) and the conclusion of the study (0.18 +/- 0.16 mg/kg) was statistically significant. Side-effects were recorded in 48 of the 123 patients (39%), but only eight required discontinuation of azathioprine. CONCLUSIONS: Azathioprine was efficacious in 70% of patients, but ineffective in 20% and induced severe toxicity in 7%. Corticosteroids were stopped or markedly reduced in 62% of patients, but they were never given in 9%.
Subject(s)
Azathioprine/pharmacology , Immunosuppressive Agents/pharmacology , Inflammatory Bowel Diseases/drug therapy , Adolescent , Adrenal Cortex Hormones/administration & dosage , Azathioprine/administration & dosage , Azathioprine/adverse effects , Child , Child, Preschool , Female , Health Surveys , Humans , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/adverse effects , Infant , Inflammatory Bowel Diseases/pathology , Italy , Male , Patient Compliance , Retrospective Studies , Severity of Illness Index , Treatment OutcomeABSTRACT
AIM: To obtain clinical data concerning severe attacks of ulcerative colitis in children. PATIENTS AND METHODS: A retrospective chart review of 37 children with ulcerative colitis was carried out in order to assess the prevalence, risk factors, timing of presentation, and outcome of severe attacks of ulcerative colitis. RESULTS: A total of 20 severe attacks occurred in 15 out of the 37 patients. No difference in the occurrence of severe attacks was detected in relation to age or disease extent at diagnosis. The mean interval between disease diagnosis and a severe attack was 9.1 months (range 0-30). Of the 20 severe attacks, 11 were resolved with medical treatment in a mean time of 1 1 +/- 4.6 days while 9 out of 20 needed urgent surgery in a mean time of 7.4 +/- 4.8 days. Of 10 out of the 15 patients who recovered from the first attack 4 required colectomy after a mean time of 6.7 months, another 4 are still in remission at a mean period of 40.7 months, one needed elective surgery after 25 months and one was lost to follow-up. CONCLUSIONS: Severe attacks of ulcerative colitis had a high prevalence rate [40%); age and disease extent at presentation were not predictors of their occurrence. Approximately half the attacks resolved with medical treatment alone, while the other half required emergency surgery. After successful medical treatment of the first attack, 40% of children maintained long-term remission, while 40% required early colectomy
Subject(s)
Colitis, Ulcerative/therapy , Adolescent , Child , Child, Preschool , Colitis, Ulcerative/classification , Colitis, Ulcerative/epidemiology , Female , Humans , Male , Outcome Assessment, Health Care , Prevalence , Retrospective Studies , Severity of Illness Index , Treatment OutcomeABSTRACT
BACKGROUND: Gallbladder stones are a well-known and widely studied problem in children. Hematological disorders are the most common diseases that can cause cholelithiasis. However, in the last few years, the proportion of children with idiopathic cholelithiasis has increased 50%. Herein, we present a prospective study on laparoscopic cholecystectomy in a selected group of patients aged < 10 years. METHODS: Fifty-eight patients aged < 10 years underwent laparoscopic cholecystectomy for stones in the period 1992-99. The female/male ratio was 1.5, the mean age was 8 years (range, 2-10), and the mean weight was 30 kg. In all patients, parenteral nutrition, fasting state, and prolonged use of antibiotics had been suspended for > or = 6 months, and conservative treatment had been tried for > or = 12 months in the absence of symptoms of stone migration. All the patients were followed up after surgery: clinically at 1,6,12, and 36 months and by ultrasound at 1, 12, and 36 months. Liver function and hematological tests were performed in case of symptoms or if hemolytic disorders were the cause of stones. RESULTS: The mean operative time was 63 min (range, 30-120) in children undergoing cholecystectomy alone and 150 min in children undergoing associated splenectomy. There were no major complications or reoperations. Minor complications included bleeding from accessory cystic artery (n = 3) and insufflation of the omentum (n = 2). One case was converted to an open procedure due to technical problems. All the children were followed up and no complications were observed. CONCLUSION: We consider the laparoscopic approach the gold standard for cholecystectomy in children. This procedure does not have a complication rate any higher than open cholecystectomy, and patient follow-up is as good as that of open surgery. Previous abdominal surgery is not a contraindication to laparoscopy.
Subject(s)
Cholecystectomy, Laparoscopic/methods , Child , Child, Preschool , Cholecystectomy, Laparoscopic/adverse effects , Female , Humans , Male , Prospective Studies , Splenectomy/adverse effects , Splenectomy/methodsABSTRACT
OBJECTIVE: This study was aimed at evaluating intestinal permeability (IP) in patients with oligoarticular juvenile idiopathic arthritis (o-JIA), spondyloarthropathy (SpA) associated with inflammatory bowel disease (IBD) and other forms of juvenile-onset chronic arthritiis (OIA) using the lactulose/mannitol (L/M) test in comparison with other non-invasive parameters of gut involvement. METHODS: A series of 26 children affected with o-JIA and 14 with either SpA/IBD or OIA were assessed for IP. The urinary L/M ratio was measured by gas chromatography. The erythrocyte sedimentation rate (ESR), C reactive protein (CRP), and faecal alpha 1 antitrypsin concentrations were also evaluated. Ten o-JIA patients displayed active arthritis while in 16 the disease was under control. Among the OIA patients, 11 were affected with psoriatic arthritis and the remaining 3 with chronic reactive arthritis. 14 patients with SpA-IBD had active synovitis or spine inflammation. 14 eo-pJCA and 22 OIA and SpA-IBD patients, respectively, were receiving NSAID therapy. RESULTS: The mean L/M ratios for the Spa-IBD (0.07 +/- 0.02, mean +/- SD), OIA (0.05 +/- 0.02) and o-JIA (0.04 +/- 0.02) patients were significantly higher (p < 0.001, p = 0.022 and p = 0.01, respectively) than those found in controls (0.02 +/- 0.01). Logistic regression analysis disclosed a positive correlation between the L/M ratio and the presence of gastrointestinal manifestations (p = 0.011). The type of disease (p = 0.28), the disease activity in the JCA patient group (p = 0.24) and NSAID administration (p = 0.210) did not seem to significantly influence the L/M ratio. CONCLUSIONS: All of the subtypes of juvenile chronic arthritides that we studied displayed an increased IP. Hence, gut wall inflammation (albeit asymptomatic) may also be present in o-JIA patients. The SpA-IBD patients with gastrointestinal symptoms displayed the highest mean L/M ratio values. The L/M test seemed to correlate with histopathological features of the gut mucosa. The L/M ratio was shown to be a highly sensitive but poorly specific test for predicting gut inflammatory disease compared to other non-invasive screening tests.
Subject(s)
Arthritis, Juvenile/metabolism , Intestinal Mucosa/metabolism , Arthritis, Juvenile/diagnosis , Child , Child, Preschool , Female , Gastrointestinal Diseases/etiology , Humans , Inflammatory Bowel Diseases/complications , Inflammatory Bowel Diseases/diagnosis , Lactulose/urine , Male , Mannitol/urine , Multivariate Analysis , Permeability , Reference Values , Sensitivity and Specificity , Spinal Diseases/complications , Spinal Diseases/diagnosisABSTRACT
Pituitary diseases are often unrecognized in the elderly, although if they can be at the basis of several pathological conditions. We report three clinical cases. Patient n. 1: 87 years old woman, in poor general condition. Thyroid function evaluation showed secondary hypothyroidism. Subsequent pituitary function evaluation demonstrated hypopituitarism with empty sella. The diagnosis was "hypopituitarism with secondary hypothyroidism and adrenocortical insufficiency, in empty sella" starting substitutive treatment with glucocorticoids and L-thyroxine, with improvement in her clinical conditions. Patient n. 2: 74 years old woman, with severe congestive heart failure. Her clinical history revealed hypothyroidism. An endocrine evaluation (in absence of therapy) demonstrated panhypopituitarism with secondary hypothyroidism and adrenocortical insufficiency in presence of empty sella. The patients was started on substitutive treatment and her conditions improved. Patient n. 3: 74 years old man with several atrial fibrillation episodes and hyperthyroidism. Thyroid function evaluation suggested secondary hyperthyroidism confirmed by the presence of a pituitary macroadenoma. The patient underwent surgical adenomectomy by trans-sphenoidal route. The clinical conditions of the patient improved, but a slight secondary hyperthyroidism was still present caused by the persistence of residual pathological tissue in the right cavernous sinus region confirmed by octreoscan suggesting the presence of a lesion endowed with somatostatin receptors. The patient was started on long acting octreotide treatment, which is still in progress. In conclusion, pituitary diseases pass often unrecognized in the elderly. Their prompt recognition and treatment can resolve dangerous situations for the patients.
Subject(s)
Adenoma/diagnosis , Hypothyroidism/diagnosis , Pituitary Neoplasms/diagnosis , Adenoma/complications , Adenoma/metabolism , Adrenocorticotropic Hormone/blood , Aged , Aged, 80 and over , Biomarkers/blood , Female , Growth Hormone/blood , Hormones , Humans , Hypothyroidism/blood , Hypothyroidism/drug therapy , Magnetic Resonance Imaging , Octreotide , Pituitary Neoplasms/complications , Pituitary Neoplasms/metabolism , Thyrotropin/blood , Tomography, X-Ray ComputedABSTRACT
We report the case of a girl with a severe perianal fistulizing Crohn's disease who during intravenous infusion of cyclosporine developed headache, general seizures and cortical blindness. Head magnetic resonance imaging showed spread, cortical-subcortical nonenhancing signal changes. Full neurological recovery was achieved in 24 hours. Cyclosporine was stopped and a single dose of infliximab was infused with rapid improvement of the fistula that at 7 months' follow-up is still closed.
Subject(s)
Brain Diseases/chemically induced , Crohn Disease/diagnosis , Crohn Disease/drug therapy , Cyclosporine/adverse effects , Immunosuppressive Agents/adverse effects , Rectal Fistula/diagnosis , Brain Diseases/diagnosis , Brain Diseases/drug therapy , Child , Crohn Disease/complications , Cyclosporine/administration & dosage , Female , Follow-Up Studies , Humans , Immunosuppressive Agents/administration & dosage , Infusions, Intravenous , Magnetic Resonance Imaging , Rectal Fistula/complications , Severity of Illness Index , Treatment Outcome , Tumor Necrosis Factor-alpha/administration & dosageABSTRACT
The authors present the investigations and surgical treatment of two cases of duodenal cystic duplication. Abdominal pain and gastroesophageal reflux were the most important symptoms and signs associated with an history of recurrent acute pancreatitis. Computed tomography scan, ultrasound examination, and cholangiography confirmed preoperatively the diagnosis, and a transduodenal surgical approach was carried out in both children. A simple marsupialization of the cyst was performed in the former, and a sphincterotomy with papillosphincteroplasty was associated in the latter. The diagnosis was confirmed by microscopy, and both the children are asymptomatic after a 14 and 18 months of follow-up. This report focuses on the importance of the cholangiopancreatography for every child presenting with recurrent, unexplained bouts of acute pancreatitis, and underlines the technical surgical aspects on the basis of the anatomic identification of the malformation.