Predictors of Gastrointestinal Involvement in Children with IgA Vasculitis: Results from a Single-Center Cohort Observational Study.

Background and objective: IgA vasculitis (IgAV), a predominantly pediatric leukocytoclastic disease, has an unpredictable, though largely benign, evolution. The aim of this study was to retrospectively investigate any potential clinical or laboratory predictors of gastrointestinal involvement in a single-center cohort of children with IgAV. Patients and methods: A total of 195 children with a history of IgAV, regularly followed-up for an average period of 1 ± 2.6 years via outpatients clinics of the pediatric rheumatology unit in our University, were assessed, analyzing their clinical and laboratory variables in relationship with their disease evolution and outcome. Results: Univariate analysis showed that a higher neutrophil granulocyte count and lower lymphocyte count (expressed as a percentage of the total white blood cells) were significantly associated with the presence of gastrointestinal involvement at the first examination (65.2 ± 13% versus 58.8 ± 12%, p = 0.02, and 26.4 ± 11% versus 32.1 ± 11%, p = 0.02, respectively). A positive pharyngeal swab for Streptococcus pyogenes, a deficiency of 25-hydroxyvitamin D, a persistence of purpuric rash for more than 1 month, and purpuric lesions in the genital area were also associated with gastrointestinal involvement (p = 0.0001, p = 0.0001, p = 0.007 and p = 0.001, respectively). However, multiple logistic regressions with correction for the patients' sex and age showed that lower 25-hydroxyvitamin D levels, persistent rash, and genital lesions were independently and significantly associated with signs of gastrointestinal involvement. We then performed a secondary analysis (both univariate and multivariate) to investigate whether vitamin D deficiency was associated with other IgAV manifestations: we found that only 25-hydroxyvitamin D deficiency remained significantly associated with gastrointestinal involvement in IgAV. Conclusions: Patients with IgAV and vitamin D deficiency might be more prone to developing gastrointestinal manifestations of variable severity.

Anorectal Malformations: The Pivotal Role of the Good Clinical Practice.

Anorectal malformations (ARM) without a fistula are a rare congenital condition. Although may seem more simple to repair compared with ARM with fistulas, surgery has proved to be challenging. We report the case of a newborn who presented a well-formed anus and normal genitalia; a blind-ending anal canal was detected after the insertion of a rectal probe, thus allowing the diagnosis of ARM. Anal probing straight after birth avoids the possible complications related to intestinal obstruction due to a missed diagnosis of ARM. Examination of the perineal region is an important step in the evaluation of the newborn and represents the tool for a prompt identification of ARM. Adding anal probing to accurate inspection perineum is a good clinical practice and should always be performed even in presence of a normal-looking perineum.

Persistent Diarrhoea after Percutaneous Endoscopic Gastrostomy (PEG) in Paediatric Patient: Lessons from a Complication.

Percutaneous endoscopic gastrostomy (PEG) is increasingly used in paediatric population. We report a case of a 4-year-old boy who, two weeks after PEG placement, presented persistent diarrhoea interpreted as intolerance to enteral feeding. His CT scan confirmed the correct placement of gastrostomy, but during gastroscopy, gastrostomy could not be found in the stomach, and the following colonoscopy revealed migration of gastrostomy to the transverse colon. The patient required removal of the misplaced PEG and conservative management of the fistula with surgical replacement of gastrostomy. We faced an unusual presentation of PEG placement complication due to colon interposition during blind gastric puncture. In children with anatomical deformities, previous surgery, or low weight or malnutrition (<10 kg), we suggest laparoscopic-assisted gastrostomy to avoid the risk of a major complication.

Prevalence of bladder cancer in Costello syndrome: New insights to drive clinical decision-making.

Costello syndrome (CS) is a rare disorder affecting development and growth characterized by cancer predisposition and caused by mutations in HRAS proto-oncogene. Somatic HRAS mutations drive bladder carcinogenesis. The aim of this study was to analyze prevalence and histological characterization of bladder cancer (BC) in a cohort of patients with CS to help clinicians plan effective management strategies. This study included 13 patients above 10 years of age with molecular diagnosis of CS. Screening cystoscopies (31 total procedures) were performed to exclude BC. Any lesion was analyzed through cold-cup biopsy or trans-urethral resection of the bladder. According to histology, patients were followed-up with urinalysis and abdominal ultrasound yearly, and cystoscopies every 12-24 months. During study enrollment, bladder lesions (often multifocal) were detected in 11/13 patients. Histological analysis documented premalignant lesions in 90% of cystoscopies performed, epithelial dysplasia in 71%, and papillary urothelial neoplasm of low-malignant potential in 19%. BC G1/low grade (Ta) were removed in 10%. Overall, 76% of patients showed a bladder lesion at first cystoscopy. The present findings document that individuals with CS aged 10 years and older have high prevalence of bladder lesions (premalignant/malignant), highlighting the importance of personalized screening protocols.

Intraluminal Duplication of the Terminal Ileum with Ectopic Gastric Mucosa in an Infant: A Rare Cause of Intussusception.

Enteric duplication cysts are rare malformations mostly diagnosed before the age of two, with varied clinical presentations. Ectopic gastrointestinal epithelium can be present, and management involves surgical resection. A three-month-old girl presented with rectal bleeding due to an ileocolic intussusception. Abdominal ultrasound revealed a target sign in the right upper part of the abdomen. At hydrostatic contrast enema, an incomplete reduction of the intussusception was obtained: only a trickle of contrast material entered the terminal ileum. An exploratory laparotomy ensued with manual reduction of the intussusception. At the end of the maneuver, a soft intraluminal mass was palpated within the ileocecal valve. Thus, an ileocecal resection was performed. At histology, an intraluminal enteric duplication cyst was documented, containing ectopic gastric mucosa. Secondary intussusception should be suspected even in infants in case of abnormal findings at hydrostatic contrast enema. Intraluminal enteric duplication cysts may be a rare cause of intussusception.

Laparoscopic Splenectomy in Children: A Modified Technique with Joint Sealing of Segmental Splenic Arteries and Veins Favorably Compared with the Classic Technique.

Introduction Splenectomy in children is nowadays widely performed by laparoscopy, the major risk being operative hemorrhage. We hereby compare the results of two different techniques of vascular control. Materials and Methods We retrospectively reviewed all patients undergoing a laparoscopic splenectomy (LS) for hematologic disease at a single institution from 2004 to 2013. LS was performed by a lateral approach. The 10-mm optic port was placed in the umbilicus and two 5-mm working ports were placed in the left lower quadrant and in the right upper quadrant near the midline.An additional port was added in the epigastrium if needed. The classic technique (CT) consisted in the separate sealing and section of the splenic artery and vein above the pancreas tail. The modified technique (MT) consisted in the dissection of each segmental splenic vessel, at the lower, middle, and upper parts of the spleen. They were then divided without individualizing the artery from the vein. Results Total 69 patients were operated on at a mean age of 7.8 ± 0.6 years, 30 by CT and 39 by MT. In 20 patients (6 CT, 14 MT) a cholecystectomy was associated. The operative time, length of hospitalization, and number of postoperative complications for CT/MT were respectively 139/117 mn (p = 0.04), 3.8/3.0 days (p = 0.01), 12/3 (p = 0.002). An additional port was less needed with MT (p = 0.03). Conclusions The joint sealing of segmental splenic artery and vein appeared safe, efficient and quicker for LS, with no increased risk of operative bleeding and postoperative complications.