BACKGROUND AND AIMS:To investigate the incidence and the severity of COVID-19 infection in patients enrolled in the database for home parenteral nutrition (HPN) for chronic intestinal failure (CIF) of the European Society for Clinical Nutrition and Metabolism (ESPEN). METHODS:Period of observation: March 1st, 2020 March 1st, 2021. INCLUSION CRITERIA:patients included in the database since 2015 and still receiving HPN on March 1st, 2020 as well as new patients included in the database during the period of observation. Data related to the previous 12 months and recorded on March 1st 2021: 1) occurrence of COVID-19 infection since the beginning of the pandemic (yes, no, unknown); 2) infection severity (asymptomatic; mild, no-hospitalization; moderate, hospitalization no-ICU; severe, hospitalization in ICU); 3) vaccinated against COVID-19 (yes, no, unknown); 4) patient outcome on March 1st 2021: still on HPN, weaned off HPN, deceased, lost to follow up. RESULTS:Sixty-eight centres from 23 countries included 4680 patients. Data on COVID-19 were available for 55.1% of patients. The cumulative incidence of infection was 9.6% in the total group and ranged from 0% to 21.9% in the cohorts of individual countries. Infection severity was reported as: asymptomatic 26.7%, mild 32.0%, moderate 36.0%, severe 5.3%. Vaccination status was unknown in 62.0% of patients, non-vaccinated 25.2%, vaccinated 12.8%. Patient outcome was reported as: still on HPN 78.6%, weaned off HPN 10.6%, deceased 9.7%, lost to follow up 1.1%. A higher incidence of infection (p = 0.04), greater severity of infection (p < 0.001) and a lower vaccination percentage (p = 0.01) were observed in deceased patients. In COVID-19 infected patients, deaths due to infection accounted for 42.8% of total deaths. CONCLUSIONS:In patients on HPN for CIF, the incidence of COVID-19 infection differed greatly among countries. Although the majority of cases were reported to be asymptomatic or have mild symptoms only, COVID-19 was reported to be fatal in a significant proportion of infected patients. Lack of vaccination was associated with a higher risk of death.

Objectives: The indications, diagnostic yield, complications, and cecal and ileal intubation rates (CIR and IIR) for colonoscopies in children aged <6 years, denoted preschoolers, is unclear since there is limited information for this group. We aimed to describe the above parameters in our cohort of preschoolers undergoing a colonoscopy. Methods: Retrospective review of all colonoscopies in a tertiary pediatric hospital between December 1, 2014 to December 31, 2020 was undertaken. Demographic factors, indication for colonoscopy, extent of colonoscopy, CIR, IIR, and histologic findings were noted. Preschoolers were further subdivided into those aged <2 years, and those aged 2 to <6 years. Results: One thousand six hundred seventy-one total colonoscopies were performed, of which 13% (n = 219) were in preschoolers with median age 3.9 (range 0.3–5.9) years. Most common indications in preschoolers were rectal bleeding 35% (n = 78), inflammatory bowel disease 24% (n = 53), diarrhea 13% (n = 30), iron-deficiency anemia 11% (n = 25), and abdominal pain 7% (n = 16). IIR and CIR were lower in preschoolers compared to older children, 81% vs 92% (P = 0.0001), and 93% vs 96.4% (P = 0.02), respectively, and even lower in those aged <2 years, 48.1% IIR (P = 0.0001) and 85.1% CIR. Juvenile polyps, 31% (n = 27), were the most common positive finding in preschool children. Conclusion: Rectal bleeding was the most common indication and juvenile polyps the most common finding at colonoscopy in preschoolers. A high IIR is achievable in young children but rates are increasingly lower the younger the child.

Background: The European Society for Clinical Nutrition and Metabolism database for chronic intestinal failure (CIF) was analyzed to investigate factors associated with nutritional status and the intravenous supplementation (IVS) dependency in children. Methods: Data collected: demographics, CIF mechanism, home parenteral nutrition program, z-scores of weight-for-age (WFA), length or height-for-age (LFA/HFA), and body mass index-for-age (BMI-FA). IVS dependency was calculated as the ratio of daily total IVS energy over estimated resting energy expenditure (%IVSE/REE). Results: Five hundred and fifty-eight patients were included, 57.2% of whom were male. CIF mechanisms at age 1–4 and 14–18 years, respectively: SBS 63.3%, 37.9%; dysmotility or mucosal disease: 36.7%, 62.1%. One-third had WFA and/or LFA/HFA z-scores < −2. One-third had %IVSE/REE > 125%. Multivariate analysis showed that mechanism of CIF was associated with WFA and/or LFA/HFA z-scores (negatively with mucosal disease) and %IVSE/REE (higher for dysmotility and lower in SBS with colon in continuity), while z-scores were negatively associated with %IVSE/REE. Conclusions: The main mechanism of CIF at young age was short bowel syndrome (SBS), whereas most patients facing adulthood had intestinal dysmotility or mucosal disease. One-third were underweight or stunted and had high IVS dependency. Considering that IVS dependency was associated with both CIF mechanisms and nutritional status, IVS dependency is suggested as a potential marker for CIF severity in children.

Background Hereditary tyrosinemia type 1 is a rare metabolic condition associated with an increased risk of hepatocellular carcinoma. Nitisinone (2-[2-nitro-4-trifluoromethylbenzoyl]-1,3-cyclohexanedione, NTBC) treatment has reduced but not eliminated the risk. The delayed initiation of nitisinone treatment, and persistently abnormal alpha 1-fetoprotein (AFP) levels are recognized to be risk factors for late-onset hepatocellular carcinoma. We report three children diagnosed and treated with nitisinone since infancy who developed hepatocellular carcinoma despite long-term normalization of AFP. Methods A retrospective review of all patients with tyrosinemia on nitisinone managed at our center was undertaken. Patient demographics, age at diagnosis, duration of therapy, timing of AFP normalization, and radiographic imaging findings were noted. Results Three patients at our center with tyrosinemia type 1 developed hepatocellular carcinoma 9-13 years after diagnosis despite long-term nitisinone therapy and normalization of AFP. Two patients developed new nodules on imaging with an elevation of AFP leading to the diagnosis and subsequent liver transplant. The third patient proceeded with liver transplant because of a very nodular liver and increasing splenomegaly despite normal AFP and no change in surveillance gadoxetate magnetic resonance imaging. Early hepatocellular carcinoma was found in her liver explant. All three patients were cirrhotic at diagnosis. Conclusions Patients with hereditary tyrosinemia type 1, especially those already cirrhotic at diagnosis, remain at high risk of developing hepatocellular carcinoma despite long-term nitisinone therapy and AFP normalization, and warrant close monitoring and surveillance.

CASE REPORT A 6-year-old girl with Noonan Syndrome (confirmed SOS1 mutation), underwent elective upper gastrointestinal endoscopy for symptoms of gastro-oesophageal reflux disease. She had a normal aorta and no known bleeding diathesis. Endoscopy was undertaken with a 8.9 mm outer diameter pediatric gastroscope. Direct visualization was unremarkable, and 2 biopsies each were obtained from the proximal and distal esophagus and 3 from the second part of the duodenum (D2) with standard biopsy forceps (2.2 mm). The procedure was uneventful, and the patient discharged home that same morning. She presented to the Emergency Department 48 hours later with abdominal pain, vomiting, fever, and tachycardia. Abdominal examination revealed generalized tenderness without peritonism. Hemoglobin dropped from 116 g/L to 97 g/L, with a normal platelet count (216 × 109/L) and normal coagulation profile (INR 1.2, prothrombin time 13 seconds, APTT 28 seconds, fibrinogen 2.7 g/L). D-dimer was elevated at 1.06. Electrolyte profile, liver biochemistry, and serum lipase were unremarkable, while lactate on venous blood gas was 0.5. Abdominal ultrasound revealed a heterogenous mass conforming to the region of D2 tapering to the duodenojejunal flexure, where normal, collapsed jejunum was demonstrated. The mass measured approximately 9 × 5 × 5 cm and appeared avascular centrally, consistent with duodenal hematoma. Furthermore, there was a moderate volume of free-fluid noted within the pelvis and iliac fossae. A computed tomography (CT) was then undertaken, which confirmed the presence of a hematoma, involving D2-D4 segments, with a maximum dimension of 100 × 40 × 45 mm. There was active contrast extravasation into D2/D3 segment during the portal venous phase of the study, and pooling during the delayed phase, consistent with acute bleeding (Figs. 1 and 2).FIGURE 1.: Axial CT image of abdomen demonstrates a large intramural hematoma in second and third part of duodenum with focal contrast extravasation (arrow) consistent with active bleeding.FIGURE 2.: Coronal CT image of abdomen demonstrates a large intramural hematoma in second and third part of duodenum with focal contrast extravasation (arrow) consistent with active bleeding.Given evidence of active bleeding on CT scan as illustrated by contrast extravasation and intraperitoneal free fluid, in the context of a drop in the patient’s hemoglobin, endovascular embolization of the bleeding vessel was agreed upon, following consultation with the interventional radiology and surgical services. This was performed within 24 hours of the patient representing to hospital. The superior pancreaticoduodenal artery was selectively catheterized with a microcatheter via the celiac trunk. Selective angiography revealed vasospasm in a small muscular branch within the wall of the duodenum running toward a bulge, which denoted a large intramural hematoma, seen in D2 and D3, and supplied by small duodenal branches of the pancreaticoduodenal arcade (Fig. 3). Angiography revealed no evidence of active bleeding. The pancreaticoduodenal arcade supplying D2/3 was embolized with three 2 × 3 mm tornado embolization coils (COOK Medical), involving occlusion of the superior (front door) and inferior (back door) pancreaticoduodenal branches (Fig 4).FIGURE 3.: Selective digital subtraction angiogram from coeliac artery with microcatheter in superior pancreaticoduodenal artery outline pancreaticoduodenal arcade. No active bleeding was seen, note the large extrinsic bulge (arrow head) on duodenum representing intramural hematoma with small duodenal branches (**) from arcade running toward hematoma.FIGURE 4.: Final selective digital subtraction angiogram demonstrates coils in superior pancreaticoduodenal (arrow) and inferior pancreaticoduodenal (arrow head) branches with the cessation of flow in the duodenal branches, which were seen running toward intramural hematoma on initial angiogram.Postembolization, the patient was managed on enteral rest, total parenteral nutrition, and proton-pump inhibition. Repeat CT performed 5 days postendoscopy showed interval reduction of the hematoma. A barium swallow 4 weeks postendoscopy showed normal passage of contrast through the duodenum. Over the course of 4 weeks, the patient was transitioned onto full oral feeds before discharge home. Further investigation, performed following consultation with the hematology service and after recovery, showed normal levels of factor VIII, IX, XI, and XII, normal platelet aggregation and function, and normal von-Willebrand profile. DISCUSSION Duodenal hematoma is an uncommon complication of upper gastrointestinal endoscopy occurring at a rate of 0.05–0.06% (1,2), as compared to the overall risk of bleeding, which is 0.3% (3). This procedural complication occurs most commonly in children (4,5). As of 2016, there were 47 published cases of duodenal hematoma, which had occurred in pediatric patients following endoscopic biopsy (6). Apart from the obtainment of mucosal biopsies, therapeutic interventions and coagulation disorders are known risks for the development of hematoma following upper gastrointestinal endoscopy (5). Children with bone marrow transplants and those suspected of having GVHD are additionally recognized as being high risk (5). It has been suggested that aspects of duodenal anatomy, including its relative immobility (due to its retroperitoneal location) and its rich submucosal blood supply may predispose to bleeding when a shearing force, such as biopsy forceps, has been applied (2,6,7). Additionally, it has been suggested that method of sedation and supine positioning may increase the risk of hematoma formation (2). Patients typically present with abdominal pain and vomiting within 72 hours of their endoscopy (2). Proposed management consists of nonoperative care with nutritional support (5). Noonan syndrome is an autosomal dominant, genetic condition, which has a prevalence of 1 in 1000–2500 births (8). It is manifest by a conglomeration of distinctive facial features, developmental delay, short stature, chest deformity, congenital heart disease, renal anomalies, lymphatic malformations, and bleeding difficulties (8,9). There are six reports of patients with Noonan syndrome developing duodenal hematomas. These consist of an adult patient postduodenal biopsy (10), children postduodenal biopsy (6,11–13) as well as a spontaneous occurrence in a child (14). Noonan syndrome has been associated with hemostatic disorders, including factor XI deficiency, thrombocytopenia, and abnormal platelet dysfunction (15). The prevalence of coagulation and platelet disorders is high in Noonan syndrome (87–93%); however, only half present with bleeding symptoms (11). This suggests that coagulation results do not always correlate with a predisposition to easy bleeding (16). In the present case, the patient had no history of easy bleeding nor significant hemorrhage and her extensive hematological screen was essentially normal. A recent publication provides a useful approach to high-risk endoscopy in children (17). The authors highlight patients with leukemia or hematopoietic stem cell transplantation as being at increased risk of bleeding, including for duodenal hematoma. No similar caution is issued with regards to patients with Noonan syndrome. Literature based on anecdotal evidence predominantly report expectant, nonoperative management of duodenal hematomas occurring postbiopsy (5). Surgical management is usually reserved for patients who do not improve with conservative management (4,7). The decision to proceed with angiography in the present case was based on the large size of hematoma and active bleeding seen on CT in the context of a postprocedural complication. Vasospasm is an indirect sign of vascular injury when direct signs of active bleed are not visible on angiography (18)—an invasive procedure with inherent risk. Embolization of mesenteric arterial branches poses risk of bowel ischemia and should be performed carefully to minimize nontarget embolization. Given the rich collateral blood supply to the duodenum, risk of procedural ischemia was judged to be low in the present case. While the patient’s time to recovery and discharge are similar to those previously reported, it is conceivable that her admission would have been longer had the hematoma been bigger—an outcome likely had embolization not occurred. This case provides the first-ever description of radiologically guided embolization of an actively bleeding duodenal hematoma occurring after endoscopy. The increased prevalence of hemostatic abnormalities in children with Noonan syndrome likely warrants extra consideration before and during intestinal endoscopy. Practical changes that could be considered include use of a smaller endoscope (and biopsy forceps) and questioning the necessity for duodenal biopsies at all (6) in children with Noonan syndrome. Intramural duodenal hematoma may be the first presentation of an underlying bleeding disorder, suggesting the need for suspicion in those with Noonan syndrome even without a significant bleeding history (12). Indeed, it has been recommended that patients with Noonan syndrome undergo a hematological review before any invasive procedure (9). While the nonoperative approach to the care of patients who have experienced a duodenal hematoma following endoscopic biopsy has been the primary management approach to-date, an interventional radiological option could be considered when there is concern about ongoing bleeding and or hemodynamic instability.

HRQOL is a key outcome following pediatric LT. Parent‐proxy reports may substitute for patients unable to report their own HRQOL. This study compared parent‐proxy and self‐reported HRQOL in children who have undergone LT.

Liver disease in children tends to present either as: (i) an acute hepatitis with or without jaundice; (ii) incidental finding of abnormal liver function tests; or (iii) from a complication of portal hypertension with either haematemesis and/or incidental splenomegaly. Acute hepatitis may result from acute infection, prescribed or other drugs, ischaemia or vascular causes, autoimmune hepatitis, or idiopathic liver failure. Non‐alcoholic fatty liver disease is now the most likely reason for abnormal liver function tests but medications, metabolic disease, cholangiopathy and non‐liver causes should be considered. Autoimmune hepatitis and alpha‐1‐antitrypsin deficiency are the most likely causes of insidious liver disease. An international normalised ratio uncorrected by vitamin K reflects the severity of liver synthetic dysfunction, but not propensity to bleed. Creatine kinase helps to differentiate muscle from liver disease in patients with raised transaminases. Doppler ultrasound of hepatic vasculature is useful when assessing splenomegaly to differentiate extra‐hepatic portal hypertension from inherent liver disease.

Quality indicators for colonoscopy in adults are well established, and mainly target colorectal cancer screening as it is by far the most common indication. In contrast, the most common indication for colonoscopy in children is for diagnosis or review of inflammatory bowel disease (IBD). High rate of ileal intubation has been proposed as a more meaningful quality indicator in pediatric colonoscopy. Whether this also applies to very young children is unclear since there is very limited information on colonoscopy in this age group. IBD is less likely in the very young, and they would potentially, by virtue of their small size, have a greater risk of adverse events if ileal intubation is mandated. Our study aims therefore were to assess the indications for colonoscopy in pre-school children aged <6years, and whether high rates of cecal and ileal intubation are achievable. Retrospective review of all colonoscopy procedures performed in a tertiary pediatric hospital between Dec 1, 2014 until Nov 30, 2018 was undertaken. Only children aged <6 years, designated pre-schoolers, were included in this review. Demographic factors, indication for colonoscopy, extent of colonoscopy, and histologic findings were noted. Cecal and ileal intubation were noted and compared to the whole cohort. Colonoscopy was considered incomplete if caecal intubation was not achieved. 1085 total colonoscopies were performed at our centre during the four-year study period, with median age 12.22 (range 0.33-19.48) years. Of these, 13% (143/1085) were performed in children aged <6years; median age 4.07 (range 0.33-5.95) years, and weight 15.75 (range 6.2-24.4) kg. Most common indications for colonoscopy in pre-school children were rectal bleeding 36% (52/143), potential or follow up IBD 21% (30/143), iron deficiency anemia 13% (19/143), diarrhea 8% (11/143) and pain 7% (10/143). 1 patient had colonoscopy to perform faecal microbial transplant. Incomplete colonoscopy was more likely in pre-schoolers, 8% (12/143) compared to the whole cohort 4% (39/1085, p= 0.013). Ileal intubation rate (IIR) and cecal intubation rate (CIR) were also lower in pre-schoolers compared to whole cohort, 76% vs 88% (p=0.001) and 92% vs 96% (P=0.013) respectively. Positive findings were found in 31% (44/143), of which juvenile polyps 43% (19/44) was most common, followed closely by IBD 41% (18/44). Other findings include perianal disease (n=2), eosinophilia (n=2), lymphocytic colitis (n=2) and 1 with vascular ectasia. Rectal bleeding is the most common indication for colonoscopy in pre-school children with juvenile polyps the most common positive finding. High rates of CIR and IIR are achievable in preschool children, albeit at slightly lower rate than in older children, but may not be as essential since IBD is less common in this age group.

Objective To assess long term graft and patient survival after donor liver retransplantation in children in Australia and New Zealand during 1986-2017; to determine the factors that influence survival. Design Retrospective cohort analysis (registry data). Setting, participants Australia and New Zealand Liver Transplant Registry data for all liver retransplantations in children (under 18 years of age), 1986-2017, in all four paediatric and six adult liver transplantation centres in the two countries. Main outcome measures Graft and patient survival at one, 5, 10 and 15 years. Results 142 liver retransplantations were undertaken in children (59 during 1986-2000, 83 during 2001-2017). Kaplan-Meier survival analysis indicated that survival was significantly greater during 2001-2017 than 1986-2000 (P < 0.001). During 2001-2017, graft survival one year after retransplantation was 84%, at 5 years 75%, at 10 years 70%, and at 15 years 54%; patient survival was 89% at one year, 87% at 5 years, 87% at 10 years, and 71% at 15 years. Median time between transplantations was 0.2 years (IQR, 0.03-1.4 years) during 1986-2000, and 1.8 years (IQR, 0.1-6.8 years) during 2001-2017 (P = 0.002). The proportion of graft failures that involved split grafts was larger during 2001-2017 (35 of 83, 42%) than 1986-2000 (10 of 59, 17%). Graft type, cause of graft failure, and number of transplants did not influence survival following retransplantation. Conclusion Survival for children following retransplantation is excellent. Graft survival is similar for split and whole grafts. Children on the liver waiting list requiring retransplantation should have the same access to donor grafts as children requiring a first transplant.

AimAerodigestive clinics (ADCs) are multidisciplinary programmes for the care of children with complex congenital or acquired conditions affecting breathing, swallowing and growth. Our objective was to describe the demographic, clinical, etiological and investigational profile of children attending the inaugural ADC at a tertiary paediatric centre in Queensland.MethodsChildren referred to the ADC at Queensland Children's Hospital from August 2018 to December 2019 were included. Data on clinical, growth and lung function parameters, bronchoscopy and upper gastrointestinal endoscopy findings, thoracic imaging and comorbidities were retrospectively analysed.ResultsFifty‐six children (median (range) age 4 years (3 months–15 years); 18 female) attended the ADC during this 17‐month period. Forty‐six (82%) children had previous oesophageal atresia with tracheo‐oesophageal fistula; 43 of these were type C. Previous isolated oesophageal atresia, congenital diaphragmatic hernia and congenital pulmonary malformation were the underlying disorder in three (5%) children each, with one child having a repaired laryngeal cleft. Vertebral Anal Tracheo Esophageal Renal Limb anomalies (VACTERL)/Vertebral Anal Tracheo Esophageal renal anomalies (VATER) association was seen in 21 (38%) children. Growth was adequate (median weight and body mass index z‐score −0.63 and −0.48, respectively). Thirty‐four (61%) children reported ongoing wet cough, with 12 (21%) requiring previous hospital admission for lower respiratory tract infection. Fourteen (25%) had bronchiectasis on computed tomography chest and 33 (59%) had clinical tracheomalacia, apparent on bronchoscopic examination in 21 patients. Dysphagia was reported in 15 (27%) children, 11 (20%) were gastrostomy feed‐dependent and 5 (9%) had biopsy‐proven eosinophilic oesophagitis.ConclusionHigh proportion of children attending the ADC have ongoing respiratory symptoms resulting in chronic pulmonary suppuration and bronchiectasis. Potential benefits of this model of care need to be studied prospectively to better understand the outcomes.

ABSTRACTRecurrent abdominal pain (RAP) in children is common, with most functional in origin. Colonoscopy has sometimes been performed to exclude pathology but its role is unclear. Our aim therefore was to assess the diagnostic yield and role of colonoscopy in these children. Retrospective review of consecutive colonoscopies in a tertiary pediatric hospital between November 2011 and October 2015 was undertaken. Only those with RAP as an indication for procedure were included. Chart review of patients with pain was undertaken to ensure they fulfilled Rome IV criteria. Patient demographics, indication for procedure, and adjunct preprocedure tests were noted. Statistical analyses were performed with SPSS software. A total of 652 colonoscopies were performed, of which 68 (10%) had abdominal pain as one of the indications, and was the sole indication in 15 (2%) patients. All 68 patients had preprocedure serum inflammatory markers measured and 53% (36/68) had stool calprotectin. Positive histology was found in 10% (7/68) including Crohn disease (n = 3), polyps (n = 2), and microscopic colitis (n = 2). The remaining 61 patients had normal colonoscopy and ileocolonic biopsies. Of the 36 patients 5 had raised fecal calprotectin, and all had abnormal histology. Serum inflammatory markers were raised in 4 patients and all also had abnormal calprotectin. No patient with isolated abdominal pain had positive histology. Rectal bleeding was the only associated indication to predict abnormal histology (P = 0.019). Colonoscopy is likely not warranted in children with RAP without bleeding, weight loss, or altered bowel habit. Fecal calprotectin is useful in helping predict positive findings.

BACKGROUND AND AIM:Capsule endoscopy (CE) offers a method of directly visualizing areas of the small bowel not accessible by conventional endoscopy. Some children are unable to swallow the capsule requiring endoscopic placement under general anesthesia. The aim of the present study was to identify any differences between children requiring endoscopic placement and those able to swallow the capsule. METHODS:Retrospective chart review of consecutive CE in a tertiary pediatric center was conducted. Patient demographics, outcomes, and complications between the two groups were noted. Paired t-test comparing continuous variables and Fisher exact test for categorical data were used. RESULTS:A total of 104 CEs were performed in 88 patients, median age 12.8 (range: 1.6-18.5) years. Almost half, 49% (51/104), swallowed the capsule. Children requiring endoscopic placement were significantly younger (9.8 vs 14.2 years; P < 0.001), lighter (34.5 vs 54.9 kg; P < 0.0001), and had longer small intestinal transit time (308 vs 229 min; P < 0.0001). Positive findings were more likely in those who swallowed the capsule (50% vs 30%, P = 0.017), likely a reflection of the indications for procedure. Poor views were found in 30% (16/53) of patients in the endoscopic placement group due to iatrogenic bleeding from biopsies taken from concurrent procedures but did not affect outcome or subsequent patient management. CONCLUSIONS:CE is safe and well tolerated in children. Children requiring endoscopic placement were significantly younger, lighter, had longer small intestine transit time, and less likely to have positive findings. Concurrent biopsies during capsule placement increase the likelihood of inadequate views but did not affect outcome or management.

ABSTRACTObjectives:Liver transplant patients are at risk of osteopenia and fractures but limited information is available in long‐term survivors after childhood transplantation. This study aimed to assess bone mineral density (BMD) of very long‐term, >5 years, survivors after liver transplantation in childhood.Methods:Patients aged <18 years at transplant, having survived >5 years after transplant were potentially eligible but only those with ongoing review in our state were included. Dual‐energy x‐ray absorptiometry (DXA) was used to measure BMD. Patients aged <20 years had lumbar spine (LS) and total body (TB) measurements whereas those aged 20 years or more had LS and femoral neck but not TB. BMD z‐scores for LS and TB, if available, were used in this study. BMD z‐score ⩽−2.0 was considered reduced. Pre‐pubertal children had radiologic bone age assessment.Results:Forty‐two patients, 17 boys, participated of whom 64% had biliary atresia. Median age at transplant was 2.22 (range 0.38–14.25) years; time since transplant 10.10 (5.01–25.98) years; and age at DXA 14.64 (6.59–38.07) years. Mean BMD z‐scores were LS −0.15 ± 1.07, and TB −0.76 ± 1.14, with no sex difference noted. Four (9.5%) patients had reduced LS BMD, and although ongoing steroid use was more frequent in these patients, other comorbidities were likely important. Age at transplant, time since transplant, height, weight, and body mass index at DXA did not predict LS BMD. Pathologic fractures occurred in 2 of 42 (5%) patients; all within 18 months of transplant.Conclusions:Very long‐term survivors after childhood liver transplant have LS BMD within the normal range.

Objectives: Quality indicators for colonoscopy in adults are largely driven by colorectal cancer screening, and include cecal intubation rates, with rates of >90% recommended. In contrast, colorectal cancer is rare in childhood, with paucity of data on relevant quality indicators for pediatric colonoscopy. It is also unclear whether high rates of cecal intubation are achievable in small children. Our aim was to audit all colonoscopies performed in a tertiary pediatric center to examine clinical indications for procedure, completeness of examination with cecal and ileal intubation, significant findings, and complications.Methods: Retrospective review of colonoscopies performed between November 2011 and October 2015 was undertaken.Results: Total colonoscopy was performed in 652 patients, 53% male, with median age 13.0 (range 0.4-18.2) years. The most common indications for colonoscopy were assessment of inflammatory bowel disease (IBD) 57.9% (378/652), rectal bleeding 10% (68/652) and abdominal pain 10% (68/652). Trainees performed 69.8% (452/652) of procedures. Quality of bowel preparation was mentioned in 63% (410/652), of which 22% (90/410) were considered inadequate. Cecal intubation rate was 96.3% (628/652) and ileal intubation rate was 92.4% (603/652). Extent of procedure was confirmed in 99.2% of patients with photographs and/or ileal biopsy. Poor quality of bowel preparation (p=.001) and age <5years (p=.007) were inversely related to successful ileal intubation.Conclusions: High rates of cecal and ileal intubation are achievable in pediatric colonoscopy. Ileal intubation should be considered a quality indicator since the main indicator for pediatric colonoscopy is to investigate IBD.

We report the case of a 1-day old, 3.2 kg girl who presented to a regional hospital with massive haematemesis, melaena and haematochezia. She was delivered by spontaneous vaginal delivery at term with no antenatal or perinatal stressors identified, and had been discharged from hospital aged 14 h. On presentation, she was pale, tachycardic, with poor peripheral perfusion. She was resuscitated with crystalloid, fresh frozen plasma, and subsequently packed red cells as her haemoglobin was 82 g/L. Intravenous proton pump inhibitor (PPI) was started and she was retrieved to a tertiary paediatric centre. Emergency endoscopy showed erythematous friable mucosa at the duodenal bulb and duodenal cap ulcers. No haemostatic intervention was required. Biopsies confirmed duodenitis and oesophagitis. She remained stable with no further bleeding and was discharged 48 h after endoscopy on high dose (3 mg/kg) oral PPI, aged 4 days. Massive upper gastrointestinal (GI) bleeding in neonates is rare. Reported causes include gastritis, oesophagitis and ulcers, mainly gastric or oesophageal.1 The incidence of duodenal ulcers (DU) as the cause of bleeding in neonates is reported to be 1.2%.1 Perinatal factors such as preterm birth, sepsis, coagulopathy, metabolic acidosis, hypovolaemia, hypoxaemia, raised intracranial pressure, hypoglycaemia, milk protein intolerance and nasogastric tube trauma have all been reported to increase the risk of bleeding.1 Medications, particularly non-steroidal anti-inflammatory drugs, Helicobacter pylori infection, and Zollinger-Ellison syndrome are also implicated, similar to adults and older children.2 Management of GI bleeding in neonates is anecdotal, and previous reports have mainly used histamine antagonists. Unfortunately, 10% still had ulcers after 1 month of histamine antagonist treatment.1 Other reported therapies in neonates include somatostatin analogues and surgery. Haemostatic treatments, such as sclerotherapy, thermocoagulation and clips have been used in adults and children but are problematic in the neonate due to lack of appropriate equipment and technical expertise. In addition, neonates, due to their size, are considered high risk for complications such as perforation after haemostatic treatment.3 PPIs, however, have been shown to be superior to histamine antagonists in healing bleeding ulcers.4 Our case supports the efficacy of high dose PPI in controlling bleeding ulcers in neonates. It serves to highlight the importance of prompt management of upper GI bleeding and that these acute bleeds are not confined solely to the realm of the sick neonate.

Recurrent abdominal pain in children is common, and may result from a multitude of conditions including functional disorders, celiac disease, constipation and inflammatory bowel disease (IBD). While pain is not considered to be an indication for colonoscopy, many are performed to exclude significant pathology. Several reviews have reported abdominal pain to be the primary indication for colonoscopy in 13-20% of pediatric patients. The utility of colonoscopy in recurrent abdominal pain in children remains unclear. Our aim was to assess the diagnostic yield and role of colonoscopy when assessing children with recurrent abdominal pain.

OBJECTIVES:Research is lacking into the emotional effects on families of serious chronic illness in infants. We examined the effect of the diagnosis of serious liver disease in infants upon parent psychological symptoms and family functioning. We hypothesized that parent psychological symptoms, family functioning, and father engagement will predict infant emotional outcomes.METHODS:Parents of infants recently diagnosed with serious liver disease completed validated questionnaires about parent stress, family function, impact of the illness on the family, and father engagement. The measures were repeated after 1 year, with the addition of the Child Behavior Checklist (CBCL).RESULTS:Parents of 37 infants participated. Parent stress and family functioning scores were not elevated. Parent psychological symptoms, family function, and father engagement did not predict infant outcome. For mothers, infant diagnosis other than biliary atresia, number of outpatient visits, and impact of the illness on the family explained 32% of the variation in CBCL (P = 0.001). For fathers, socioeconomic status, infant diagnosis other than biliary atresia, whether the infant had had a transplant, and impact of the illness on the family explained 44% of the variation in CBCL (P < 0.001).CONCLUSIONS:Parents and families appear to be resilient in coping with serious infant illness. Infant diagnosis other than biliary atresia and parental perceptions of high impact of the illness on the family are indicators of negative emotional outcomes for infants with serious liver disease. Psychosocial interventions for infants with chronic illness should target reducing the impact of illness on the family.