Martha Ford-Adams

Hepatopathy of Mauriac syndrome: a retrospective review from a tertiary liver centre

Background Mauriac syndrome is characterised by growth failure, cushingoid appearance and hepatomegaly which occurs in patients with insulin dependent diabetes and was first described shortly after the introduction of insulin as a treatment for the condition. Objective To describe the clinical features, histological findings and outcome of young people with glycogenic hepatopathy, the hepatic manifestation of Mauriac syndrome (MS). Design Retrospective cohort study. Patients Young people with glycogenic hepatopathy. Setting Tertiary paediatric hepatology unit. Results Thirty-one young people (16 M), median age of 15.1 years (IQR 14–16.2) presented within the study period. Median age of diagnosis of diabetes was 10 years (IQR 8–11). Median insulin requirement was 1.33 units/kg/day; median HbA1c was 96.7 mmol/mol (IQR 84.7–112.0). Growth was impaired: median height z-score was −1.01 (−1.73 to 0.4) and median body mass index (BMI) z-score was 0.28 (−0.12 to 0.67). Hepatomegaly was universal with splenomegaly in 16%. Transaminases were abnormal with a median aspartate aminotransferase (AST) of 76 IU/L and gamma glutamyltransferase of 71 IU/L. Liver biopsy was undertaken in 19 (61%). All showed enlarged hepatocytes with clear cytoplasm with glycogenated nuclei in 17. Steatosis was present in the majority. Inflammation was present in 8 (42%). Fibrosis was seen in 14 (73%) and was generally mild though 2 had bridging fibrosis. Megamitochondria were described in 7. Presence of megamitochondria correlated with AST elevation (p=0.026) and fibrosis on biopsy (p=0.007). At follow-up 17 children had normal or improved transaminases, in 13 there was no change. Transaminases followed the trend of the childs HbA1c. Conclusions Despite modern insulin regimens and monitoring in children with type 1 diabetes, MS still exists. Significant steatosis, inflammation and fibrosis were all seen in liver biopsies.

The impact of the National Patient Safety Agency intravenous fluid alert on iatrogenic hyponatraemia in children.

In March 2007, the National Patient Safety Agency (NPSA) issued an alert regarding intravenous fluid (IVF) prescription to hospitalised infants and children, to be implemented in UK hospitals by September 2007. Previously, the most commonly used IVF (0.18% saline/4% dextrose) has been associated with iatrogenic hyponatraemia, resulting in four deaths and one near miss since 2000. The alert recommended 0.45% (or 0.9%) saline/5% dextrose as maintenance IVF and banned 0.18% saline/4% dextrose. We audited practice and outcome in children receiving maintenance IVF in June 2007 (before guideline implementation) and June 2008 (after guideline implementation). In June 2007, 44 (30%) children were prescribed IVF, six received IVF not recommended by NPSA alert 22 and one became hyponatraemic. In June 2008, 56 (30%) children received IVF; one received IVF not recommended by NPSA alert 22 and became hyponatraemic. The median change in serum sodium levels for all children who received IVF not recommended by NPSA alert 22 [−5 (−15 to 0) mmol/l] was significantly greater than those who received IVF recommended by NPSA alert 22 [0 (−13 to +7) mmol/l, p = 0.002]. In addition, there was a significant (p = 0.04) reduction in the number of children who had electrolytes checked while on IVF after implementation of the guideline. Implementation of a new IVF guideline has been associated with less use of IVF not recommended by NPSA alert 22, resulting in less serum sodium level reduction. The only children who became hyponatraemic received IVF not recommended by NPSA alert 22. Despite the NPSA alert and guideline implementation, less children had electrolyte levels checked while receiving IVF.

Bariatric Surgery: A Potential Treatment for Type 2 Diabetes in Youth

Type 2 diabetes, once referred to as “adult-onset” diabetes, has now emerged as a formidable threat to the health of obese adolescents. Although there is growing evidence regarding the epidemiology of type 2 diabetes in youth and its multisystem health consequences, treatment options have lagged and progression of disease occurs even with aggressive medical therapy. Increasing interest in the application of bariatric surgery for adolescents with type 2 diabetes has evolved in part because of the evidence demonstrating improvement or remission in many adults with diabetes after surgery. Here, we review the burden of type 2 diabetes in youth including its associated complications, discuss the outcomes and complications of bariatric surgery in adolescents with diabetes, and conclude with recommendations for future research and options for refinement of the use of bariatric surgery in this patient population.

Assessment of Diet and Physical Activity in Paediatric Non-Alcoholic Fatty Liver Disease Patients: A United Kingdom Case Control Study

Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children, with prevalence rising alongside childhood obesity rates. This study aimed to characterise the habitual diet and activity behaviours of children with NAFLD compared to obese children without liver disease in the United Kingdom (UK). Twenty-four biopsy-proven paediatric NAFLD cases and eight obese controls without biochemical or radiological evidence of NAFLD completed a 24-h dietary recall, a Physical Activity Questionnaire (PAQ), a Dutch Eating Behavior Questionnaire (DEBQ) and a 7-day food and activity diary (FAD), in conjunction with wearing a pedometer. Groups were well matched for age and gender. Obese children had higher BMI z-scores (p = 0.006) and BMI centiles (p = 0.002) than participants with NAFLD. After adjusting for multiple hypotheses testing and controlling for differences in BMI, no differences in macro- or micronutrient intake were observed as assessed using either 24-h recall or 7-day FAD (p > 0.001). Under-reporting was prevalent (NAFLD 75%, Obese Control 87%: p = 0.15). Restrained eating behaviours were significantly higher in the NAFLD group (p = 0.005), who also recorded more steps per day than the obese controls (p = 0.01). In conclusion, this is the first study to assess dietary and activity patterns in a UK paediatric NAFLD population. Only a minority of cases and controls were meeting current dietary and physical activity recommendations. Our findings do not support development of specific dietary/ physical activity guidelines for children with NAFLD; promoting adherence with current general paediatric recommendations for health should remain the focus of clinical management.

Weight loss surgery improves quality of life in pediatric patients with osteogenesis imperfecta

BACKGROUND Osteogenesis imperfecta (OI) is an inherited disorder, which causes brittle bones resulting in recurrent fractures. The associated poor mobility of children with OI increases susceptibility to obesity, and obesity further dramatically limits mobility and increases fracture risk. OBJECTIVES The aim of this report is to describe outcomes of weight loss surgery (WLS) in 2 adolescents with severe obesity and OI. SETTING Two University Hospitals (in the United Kingdom and in the United States). METHODS Two cases of OI treated with WLS were identified. Pre- and postoperative anthropometric and biochemical data and clinical course were reviewed. RESULTS In these 2 cases, preoperative Body Mass Index (BMI) values were 38 and 46 kg/m2. Following laparoscopic sleeve gastrectomy (LSG), BMI decreased by 55% and 60% by 26 and 24 months, respectively. There were no surgical complications, and both patients experienced improvement in their mobility and ability to perform activities of daily living. CONCLUSION WLS effectively treated severe obesity in 2 OI patients and substantially improved mobility and quality of life, theoretically reducing fracture risk.

P1239 : Assessment of diet and physical activity in paediatric non-alcoholic fatty liver disease patients: A UK case control study

Non-alcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children, with prevalence rising alongside childhood obesity rates. This study aimed to characterise the habitual diet and activity behaviours of children with NAFLD compared to obese children without liver disease in the United Kingdom (UK). Twenty-four biopsy-proven paediatric NAFLD cases and eight obese controls without biochemical or radiological evidence of NAFLD completed a 24-h dietary recall, a Physical Activity Questionnaire (PAQ), a Dutch Eating Behavior Questionnaire (DEBQ) and a 7-day food and activity diary (FAD), in conjunction with wearing a pedometer. Groups were well matched for age and gender. Obese children had higher BMI z-scores (p = 0.006) and BMI centiles (p = 0.002) than participants with NAFLD. After adjusting for multiple hypotheses testing and controlling for differences in BMI, no differences in macro- or micronutrient intake were observed as assessed using either 24-h recall or 7-day FAD (p > 0.001). Under-reporting was prevalent (NAFLD 75%, Obese Control 87%: p = 0.15). Restrained eating behaviours were significantly higher in the NAFLD group (p = 0.005), who also recorded more steps per day than the obese controls (p = 0.01). In conclusion, this is the first study to assess dietary and activity patterns in a UK paediatric NAFLD population. Only a minority of cases and controls were meeting current dietary and physical activity recommendations. Our findings do not support development of specific dietary/ physical activity guidelines for children with NAFLD; promoting adherence with current general paediatric recommendations for health should remain the focus of clinical management.

Reply to correspondence letter ‘don't forget potassium!’ by K. Kaneko

Dear Editor, We thank Dr. Kaneko for his comments. While our audit [2] did not examine potassium levels in children receiving maintenance fluids and looked only at sodium levels, we would agree in general with Dr. Kanekos comments [3]. The addition of potassium to maintenance fluids should certainly be considered in most children to avoid hypokalaemia. As Armon et al. [1] showed in their audit, a quarter of children were hypokalaemic and not receiving supplementary potassium in their maintenance fluid. However, in line with what is stated in NPSA alert 22 [4], we would encourage the checking of serum potassium levels prior to prescribing potassium in maintenance fluid to avoid hyperkalaemia and its consequences. We would also recommend checking serum electrolyte levels (including sodium and potassium) at least daily in children receiving intravenous fluid, again reinforcing NPSA alert 22 [4].

The impact of the National Patient Safety Agency intravenous fluid alert on iatrogenic hyponatraemia in children

Aim To assess the impact of the National Patient Safety Agency (NPSA) alert 22 on prescription of maintenance intravenous fluid (IVF) and development of hyponatraemia in children. Methods In December 2007, in response to NPSA alert 22, a guideline for the prescription of IVF to children was introduced to the hospital paediatric wards. The authors audited practice and outcome in children receiving maintenance IVF in June 2007 (before guideline implementation) and June 2008 (after guideline implementation). The authors used a standard proforma for data collection which included age, weight, diagnosis, comorbidities, medication, details of IVF administered and the results of any serum sodium levels taken during admission. The audit was registered with the hospitals audit department. Results Prior to the implementation of the guideline, 44 (30%) children received maintenance IVF (25 males; median (range) age 6.0 (0.08–15.0) years, weight 18.0 (3.7–70.0) kg). Six children received either 0.18% saline/4% glucose (n=5) or 5% dextrose (n=1) as maintenance IVF (IVF not recommended by NPSA alert 22), and one became hyponatraemic. After the implementation of the guideline, 56 (30%) children received maintenance IVF (34 males; median (range) age 6.9 (0.001–17.1) years, weight 22.2 (3.2–65.0) kg). One child received IVF not recommended by NPSA alert 22 (5% dextrose) and also became hyponatraemic. The median change in serum sodium levels for all children who received IVF not recommended by NPSA alert 22 (−5 (0 to −15) mmol/L) was significantly greater than those children who received IVF recommended by NPSA alert 22 (−0.5 (−13 to +7) mmol/L) p=0.002). In addition, there was a significant (p=0.04) reduction in the number of children who had electrolytes checked while on IVF after implementation of the guideline. Conclusion Implementation of a new IVF guideline has been associated with less use of IVF not recommended by NPSA alert 22, resulting in less serum sodium level reduction. The only children who became hyponatraemic received IVF not recommended by NPSA alert 22. Despite the NPSA alert and guideline implementation, less children had electrolyte levels checked while receiving IVF.