Edward V. O’Loughlin

Gastrointestinal and nutritional problems in severe developmental disability

The aim of this study was to describe the experience of 452 children and adults with a severe developmental disability who presented to a multidisciplinary clinic with swallowing, nutritional, and gastrointestinal problems. Data were obtained by chart review. Two hundred and ninety‐four children (age range 7mo–19y, 173 males, 121 females) and 158 adults (age range 18–53y; 90 males, 68 females) were assessed over 5 years. One hundred and eighty‐two children and 86 adults had cerebral palsy. Approximately 90% were wheelchair dependent and totally dependent on caregivers for feeding; 60% had epilepsy. Pulmonary aspiration was identified by oesophageal videofluoroscopy in 41% of 174 children and 47% of 34 adults. Chronic oesophagitis and Helicobacter pylori were found in 57% of 182 children and 76% of 66 adults undergoing endoscopy. Chronic suppurative lung disease was identified by computerized axial tomography in 94% of 62 children and all six adults studied. Most patients improved with simple interventions. However, gastrostomy was recommended in 140 children and performed in 91, and in 10 adults but performed in seven, whereas fundoplication was recommended in 111 children and performed in 74, and in six adults but performed in two. In conclusion, chronic oesophagitis, pulmonary aspiration, and chronic lung disease were identified in many patients with a severe developmental disability.

Detection of Helicobacteraceae in intestinal biopsies of children with Crohn's disease.

Background:  Given that members of Helicobacteraceae family colonize the intestinal mucus layer, it has been hypothesized that they may play a role in Crohn’s disease. This study investigated the presence of Helicobacteraceae DNA in biopsies collected from children with Crohn’s disease and controls.

Nutritional rehabilitation increases the resting energy expenditure of malnourished children with severe cerebral palsy

Aim  The aim of this study was to measure resting energy expenditure (REE) and energy intake in children with quadriplegic cerebral palsy (CP), to relate these to anthropometric measurements, and to determine the influence of nutritional rehabilitation on REE.

Isolated liver transplantation in children with cystic fibrosis - An Australian experience

Nightingale S, O’Loughlin EV, Dorney SFA, Shun A, Verran DJ, Strasser SI, McCaughan GW, Jermyn V, Van Asperen P, Gaskin KJ, Stormon MO. Isolated liver transplantation in children with cystic fibrosis – An Australian experience.
Pediatr Transplantation 2010: 14:779–785.

Liver transplantation in children with hyper-reduced grafts - a single-center experience.

Thomas N, Thomas G, Verran D, Stormon M, O’Loughlin E, Shun A. Liver transplantation in children with hyper‐reduced grafts – A single‐center experience. Pediatr Transplantation 2010: 14:426–430.

Biliary strictures and hepatic artery flow abnormalities in split liver transplants.

O’Loughlin EV, Stormon MO, Shun A, Verran D, Jermyn V, Wong C, Lord D. Biliary strictures and hepatic artery flow abnormalities in split liver transplants.
Pediatr Transplantation 2010: 14: 121–125.

Prospective peer-review audit of paediatric upper gastrointestinal endoscopy.

Aim:  To describe the findings of paediatric upper gastrointestinal endoscopy (UGE) and to reduce the rate of normal findings in children undergoing diagnostic UGE.

Immunogenicity and persistence of immunity of a quadrivalent Human Papillomavirus (HPV) vaccine in immunocompromised children.

AIM The aim of this study was to determine the immunogenicity and reactogenicity of HPV vaccine in immunocompromised children. METHODS A multi-centre clinical trial was conducted in three paediatric hospitals in Australia. Unvaccinated children 5-18years of age attending one of three paediatric hospitals with a range of specified conditions associated with immunosuppression were included. Quadrivalent HPV vaccine (Gardasil) was given to the participants and serum anti-HPV antibody levels were measured at baseline (before first dose), 7 and 24months after the first dose of vaccine. RESULTS Fifty-nine participants were enrolled across the three paediatric hospitals and among those one was seropositive to types 6, 11 and 16 at baseline. Seven months after the first dose, seroconversion rates were 93.3%, 100%, 100% and 88.9% for type 6, 11, 16 and 18 respectively. The corresponding rates at 24month follow up were 82.2%, 91.1%, 91.1% and 68.9%. The greatest increase in geometric mean titre (GMT) was for type 16, followed by type 11. GMTs declined over the following months, but remained more than fourfold higher for all serotypes compared to baseline titres at 24months post vaccination. Injection site erythema, pain and swelling were commonly reported local adverse events and were less common after each dose. Few participants reported systemic adverse events, and minor disease flare occurred in two participants. One child developed a squamous cell oral carcinoma during follow up, but tissue was unable to be tested for HPV. CONCLUSION Immunosuppressed children had an adequate immunogenic response to Quadrivalent HPV vaccine regardless of age and the cause of immunosuppression. HPV related cancers occur at higher frequency and earlier in immunosuppressed patients, so early vaccination and optimal scheduling should be further studied in such children. CLINICAL TRIAL REGISTRATION NCT02263703 (ClinicalTrials.gov).

Family Impact and Infant Emotional Outcomes Following Diagnosis of Serious Liver Disease or Transplantation in Infancy.

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.

Melena and Hyperammonemic Seizures in a Turner Syndrome Patient

Gastroenterology 2015;148:302–303 Question: An8-year-old girl with Turner syndrome (45 XO) and a history of repaired coarctation of the aorta as an infant presented with profusemelena and subsequent status epilepticus. There was no previous or family history of gastrointestinal (GI)bleedingorseizures, peptic ulcer, or nonsteroidal anti-inflammatory drug ingestion. Physical examination revealed typical dysmorphism of Turner syndrome and no signs of chronic liver disease, portal hypertension, or external hemangiomata. Laboratory findings revealed an anemia (hemoglobin, 84 g/L; normal range, 115-140) and hyperammonemia (211mmol/ L; normal range, 10-50). Her liver function test, coagulation studies, and urine metabolic screen (including urinary orotic acid) were normal. Abdominal ultrasonography and CT of the abdomen were performed demonstrating abnormal vascular connections (Figure A, B). Upper endoscopy was unremarkable, but colonoscopy to the transverse colon revealed altered blood with no focal lesions (abandoned due to blood obscuration of the lumen). A laparotomy with enteroscopy was subsequently performed (Figure C, serosal surface; D, luminal view). What is the diagnosis? Look on page 303 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.