Biliary atresia: 20-40-year follow-up with native liver in an Italian centre.

INTRODUCTION: Biliary atresia (BA) represents the leading indication for liver transplantation in childhood. Only few studies reported the outcome of patients who survived more than 20 years on their native liver, and up to date there are no Italian data available. We reported our 40-year single centre experience with long-term follow-up of BA patients. MATERIALS AND METHODS: All consecutive patients who underwent Kasai portoenterostomy (KPE) for BA managed at our Institution between 1975 and 1996 were retrospectively reviewed. Native liver (NLS) and overall survival (OS) were analyzed with Kaplan-Meyer curves and LogRank test. A p value of <.05 was regarded as significant. Quality of life of patients currently surviving with their native liver was assessed through a quality of life questionnaire. RESULTS: During the 22-year period of the study 174 patients underwent surgery (median age 60 days). Clearance of jaundice at 6 months from surgery was achieved in 90 patients (51.7%). NLS was 41% at 5 years, 32% at 10 years, 17.8% at 20 years and 14.9% at 40 years. Cholangitis was recorded in 32%, hepatocellular carcinoma in 0.5%. Twenty-six patients (14.9%) survived with their liver more than 20 years; 84.6% had normal serum bilirubin level and 23% had esophageal varices. Quality of life was comparable with the healthy Italian population in all but one patient. CONCLUSIONS: Our Italian experience confirms KPE represents the cornerstone of treatment for children with BA. Multidisciplinary and meticulous lifelong post-operative follow-up should be guaranteed for these patients because of the possibility of late-onset cholangitis, portal hypertension, hepatic deterioration and liver malignant tumors. TYPE OF THE STUDY: retrospective case series. LEVEL OF EVIDENCE: IV.

Qualitative questionnaire on the psychosocial wellbeing of mothers of children with BEEC.

INTRODUCTION: The bladder exstrophy-epispadias complex (BEEC) represents a spectrum of malformations that affect the anatomical and functional structure of the urogenital system. The parents of patients affected by this condition are subject to particularly stressful situations, such as worrying about their child's health, long hospital stays, concerns about the health and constant need for personal care for their children, that can profoundly compromise the quality of family life. OBJECTIVE: The objective of this explorative qualitative study is to evaluate the social situation and the psychological strategies implemented by the mothers of children between 6 and 10 years of age who are affected by BEEC. STUDY DESIGN: Fourteen mothers of children aged 6-10 years and affected by BEEC (9 boys and 5 girls) were interviewed. Data on the mothers' experiences were collected through semi-structured interviews (Table). RESULTS: The qualitative analysis of the interviews showed that participants described experiences that were characterised by emotions such as fear and anger. Each mother had implemented a different and, sometimes, dysfunctional strategy in order to cope with the complex situation of the son/daughter. The aspects that most clearly emerged from mothers' descriptions were (1) the traumatic situation at the birth of the baby, (2) the sense of embarrassment concerning the pathological condition as the child was growing and the consequent sense of isolation of the mother, and (3) the fluctuation of feelings towards the multidisciplinary staff, which was sometimes seen as an important source of help and some other times as too destabilising and not helpful at all. DISCUSSION: The study provided some insight into the psychological and social conditions experienced by mothers of children with BEEC, which could serve as a basis for developing multidisciplinary teams with greater awareness about families living with this condition and better timing in addressing their needs. CONCLUSIONS: Mothers of children with BEEC show emotional and social difficulties. This is a crucial aspect to consider when planning a multidisciplinary approach to the treatment/therapy, especially considering that children examined in this study are approaching adolescence.

Epithelial Tumors of the Ovary in Children and Teenagers: A Prospective Study from the Italian TREP Project.

STUDY OBJECTIVE: To report the clinical findings and treatment results of a series of patients with epithelial tumors of the ovary, registered and treated prospectively in a multi-institutional Italian network (TREP project) on rare tumors in children and adolescent between 2000 and 2014. DESIGN, SETTING, AND PARTICIPANTS: Data on 16 patients, aged 22 to 206 months, from 7 centers were reviewed. All patients were grouped on the basis of the results of the first surgical approach, according to the Children Oncology Group staging system. RESULTS: The most frequent symptom was abdominal pain; 3 patients were hospitalized for acute pain after ovarian torsion. Initial surgical treatment was complete in 15 of 16, and a biopsy was performed in 1 of 16. The histology examination revealed 8 benign tumors (7 mucinous cystadenomas and 1 serous cystadenoma) and 8 borderline tumors (2 serous and 6 mucinous). Fifteen of 16 patients maintained the complete remission after surgical treatment alone; 1 affected by Proteus syndrome died consequent to the progression of a synchronous Wilms tumor, after a delayed incomplete surgery. CONCLUSION: Our analysis documented the rarity of these tumors in children and adolescents. No malignant histologies were found. Surgery alone was effective to cure the majority of patients. Considering the rarity of malignant entities, ovary-sparing surgery may be planned when an epithelial tumor is suspected on the basis of the preoperative work-up and intraoperative findings.

[Fistulas of the lower urinary tract in children]. / Fistole del basso apparato urinario in età pediatrica.

A lower urinary tract fistula consist in an abnormal connection between bladder, urethra and adjacent abdominal organs or skin. There are several types of urinary fistulas in paediatric age and they may be congenital or acquired. Etiology may be due to embriological defects, infectious processes, malignant tumours, pelvic irradiation as well as complications following surgical procedures, especially postsurgical repair of hypospadia or epispadia. Clinical presentation depends on the type of fistula and diagnosis is based on signs, symptoms and radiological or endoscopic examinations. We performed PubMed research using terms such as lower urinary fistulae, urology and paediatrics and we consulted medical texts. We reviewed selected articles and used the relevant ones to perform our study concentrating on classification, diagnosis and treatment of different types of fistulas. Paediatric lower urinary fistulas are an uncommon pathology, but the knowledge of their etiology and classification is important to recognise them and lead the physician to an appropriate treatment, which is surgical in most cases.

Diagnostic value of serological assays in pediatric inflammatory bowel disorders.

BACKGROUND: Pediatric studies reported that the combined use of the anti-neutrophil cytoplasm autoantibodies (ANCA) and the anti-Saccharomyces cerevisiae mannan antibodies (ASCA) may be a specific useful noninvasive test in the diagnosis of inflammatory bowel diseases (IBD). AIMS: To evaluate the diagnostic accuracy of ANCA and ASCA in children with suspected IBD, and to see whether different commercially available assays (indirect immunofluorescence vs. ELISA) agree well enough in terms of analytical performance. PATIENTS AND METHODS: Sixty-nine children (30 males, 39 females, age range 2-18 years) with suspicion of IBD entered the study. Before colonoscopy, a blood sample was also drawn to assess ASCA and ANCA. RESULTS: A diagnosis of IBD was established in 47 patients; the remainder had infective or other causes of colitis. For ulcerative colitis, the association ASCA-/ANCA+ had 70% sensitivity and 86% specificity, with a positive predictive value of 82%. The association ASCA+/ANCA- had 86% sensitivity and 93% specificity for Crohn's disease, with a positive predictive value of 75%. CONCLUSION: Although more experience is needed to state the diagnostic power of serologic assay, determination of ANCA and ASCA in IBD children may help both in distinguish these conditions from other entities and ulcerative colitis from Crohn's disease, particularly in doubtful cases.