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1.
Eur J Pediatr ; 178(12): 1875-1881, 2019 Dec.
Article in English | MEDLINE | ID: mdl-31522315

ABSTRACT

Necrotising enterocolitis (NEC) is often managed with a temporary enterostomy. Neonates with enterostomy are at risk of growth retardation during critical neurodevelopment. We examined their growth using z-score. We identified all patients with enterostomy from NEC in two neonatal surgical units (NSU) during January 2012-December 2016. Weight-for-age z-score was calculated at birth, stoma formation and closure, noting severely underweight as z < - 3. We compared those kept in NSU until stoma closure with those discharged to local units or home (LU/H) with a stoma. A total of 74 patients were included. By stoma closure, 66 (89%) had deteriorated in z-score with 31 (42%) being severely underweight. There was no difference in z-score at stoma closure between NSU and LU/H despite babies sent to LU/H having a more distal stoma, higher birth weight and gestational age. Babies in LU/H spent a much shorter period on parenteral nutrition while living with their stoma for longer, many needing readmission.Conclusion: Growth failure is a common and severe problem in babies living with enterostomy following NEC. z-score allowed growth trajectory to be accounted for in nutrition prescription and timing of stoma closure. Care during this period should be focused on minimising harm.What is Known:• Necrotising enterocolitis (NEC) is a life-threatening condition affecting predominately premature and very low birth weight neonates. Emergency treatment with temporary enterostomy often leads to growth failure.• There is no consensus on the optimal timing for stoma reversal, hence prolonging impact on growth during crucial developmental periods. Both malnutrition and surgical NEC are independently associated with poor neurodevelopment outcome.What is New:• Our study found growth in 89% of babies deteriorated while living with a stoma, with 42% having a weight-for-age z-score < - 3, meeting the WHO criteria of being severely underweight, despite judicial use of parenteral nutrition. Applying z-score to weight measurements will allow growth trajectory to be accounted for in clinical decisions, including nutrition prescription (both enteral and parenteral), and guide timing of stoma closure.• Surgeons who target stoma closure at a certain weight risk waiting for an indefinite period of time, during which babies' growth may falter.


Subject(s)
Brain/growth & development , Child Development , Enterocolitis, Necrotizing/complications , Enterocolitis, Necrotizing/surgery , Enterostomy , Infant, Newborn, Diseases/surgery , Weight Gain , Female , Humans , Infant, Newborn , Male
2.
Dig Liver Dis ; 51(11): 1537-1546, 2019 11.
Article in English | MEDLINE | ID: mdl-31358484

ABSTRACT

BACKGROUND: Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare, severe, sporadically occurring disorder characterized by multiple venous malformations. AIMS: To present and analyze a case series of pediatric patients with BRBNS and to describe diagnostic approaches and management options applied. PATIENTS AND METHODS: Multicenter, retrospective study, evaluating the diagnosis and management of children with BRBNS. RESULTS: Eighteen patients diagnosed with BRBNS were included. Cutaneous venous malformations were observed in 78% and gastrointestinal venous malformations in 89%. Lesions were also found in other organs including muscles, joints, central nervous system, eyes, parotid gland, spine, kidneys and lungs. Gastrointestinal lesions were more common in the small intestine than in stomach or colon. The management varied significantly among centers. Endoscopic therapy and surgical therapy alone failed to prevent recurrence of lesions. In younger children and in patients with musculoskeletal or other organ involvement, sirolimus was used with 100% success rate in our series (5 patients treated) although poor compliance with subtherapeutic sirolimus trough levels led to recurrence in a minority. CONCLUSIONS: Considering the multi-organ involvement in BRBNS, diagnosis and management requires a multidisciplinary approach. The treatment includes conservative, medical, endoscopic and surgical options. Prospective multicenter studies are needed to identify the optimal management of this rare condition.


Subject(s)
Gastrointestinal Neoplasms/diagnosis , Gastrointestinal Neoplasms/therapy , Nevus, Blue/diagnosis , Nevus, Blue/therapy , Skin Neoplasms/diagnosis , Skin Neoplasms/therapy , Child , Child, Preschool , Diagnosis, Differential , Endoscopy, Digestive System , Female , Humans , Infant , Interdisciplinary Communication , Male , Neoplasm Recurrence, Local , Retrospective Studies , Sclerotherapy , Sirolimus/therapeutic use , Vascular Malformations/diagnosis , Vascular Malformations/therapy
3.
BMJ Paediatr Open ; 1(1): e000075, 2017.
Article in English | MEDLINE | ID: mdl-29637116

ABSTRACT

OBJECTIVE: To determine whether during-exercise rehydration improves swimming performance and whether sports drink or water have differential effects on performance. DESIGN: Randomised controlled multiple crossover trial. SETTING: A UK competitive swimming club. SUBJECTS: 19 club-level competitive swimmers, median age (range) 13 (11-17) years. INTERVENTIONS: Subjects were scheduled to drink ad libitum commercial isotonic sports drink (3.9 g sugars and 0.13 g salt per 100 mL) or water (three sessions each) or no drink (six sessions) in the course of twelve 75 min training sessions, each of which was followed by a 30 min test set of ten 100 m maximum-effort freestyle sprints each starting at 3 min intervals. MAIN OUTCOME MEASURE: Times for the middle 50 m of each sprint measured using electronic timing equipment in a Federation Internationale de Natation (FINA)-compliant six-lane 25 m competition swimming pool. RANDOMISATION: Software-generated individual random session order in sealed envelopes. Analysis subset of eight sessions randomly selected by software after data collection completed. MASKING: Participants blind to drink allocation until session start. RESULTS: In the analysis data set of 1118 swims, there was no significant difference between swim times for drinking and not drinking nor between drinking water or a sports drink. Mean (SEM) 50 m time for no-drink swims was 38.077 (0.128) s and 38.105 (0.131) s for drink swims, p=0.701. Mean 50 m times were 38.031 (0.184) s for drinking sports drink and 38.182 (0.186) s for drinking water, p=0.073. Times after not drinking were 0.027 s faster than after drinking (95% CI 0.186 s faster to 0.113 s slower). Times after drinking sports drink were 0.151 s faster than after water (95% CI 0.309 s faster to 0.002 s slower). Mean (SEM) dehydration from exercise was 0.42 (0.11)%. CONCLUSIONS: Drinking water or sports drink over 105 min of sustained effort swimming training does not improve swimming performance. TRIAL REGISTRATION: ISRCTN: 49860006.

4.
Clin Sci (Lond) ; 129(7): 515-27, 2015 Oct.
Article in English | MEDLINE | ID: mdl-25948052

ABSTRACT

The physical and immunological properties of the human intestinal epithelial barrier in aging are largely unknown. Ileal biopsies from young (7-12 years), adult (20-40 years) and aging (67-77 years) individuals not showing symptoms of gastrointestinal (GI) pathologies were used to assess levels of inflammatory cytokines, barrier integrity and cytokine production in response to microbial challenges. Increased expression of interleukin (IL)-6, but not interferon (IFN)γ, tumour necrosis factor (TNF)-α and IL-1ß was observed during aging; further analysis showed that cluster of differentiation (CD)11c(+) dendritic cells (DCs) are one of the major sources of IL-6 in the aging gut and expressed higher levels of CD40. Up-regulated production of IL-6 was accompanied by increased expression of claudin-2 leading to reduced transepithelial electric resistance (TEER); TEER could be restored in in vitro and ex vivo cultures by neutralizing anti-IL-6 antibody. In contrast, expression of zonula occludens-1 (ZO-1), occludin and junctional-adhesion molecule-A1 did not vary with age and overall permeability to macromolecules was not affected. Finally, cytokine production in response to different microbial stimuli was assessed in a polarized in vitro organ culture (IVOC). IL-8 production in response to flagellin declined progressively with age although the expression and distribution of toll-like receptor (TLR)-5 on intestinal epithelial cells (IECs) remained unchanged. Also, flagellin-induced production of IL-6 was less pronounced in aging individuals. In contrast, TNF-α production in response to probiotics (VSL#3) did not decline with age; however, in our experimental model probiotics did not down-regulate the production of IL-6 and expression of claudin-2. These data suggested that aging affects properties of the intestinal barrier likely to impact on age-associated disturbances, both locally and systemically.


Subject(s)
Age Factors , Gene Expression Regulation , Ileum/metabolism , Immunity, Innate , Adult , Aged , Biopsy , Caco-2 Cells , Child , Culture Media, Conditioned/chemistry , Cytokines/metabolism , Dendritic Cells/metabolism , Electric Impedance , Endoscopy , Epithelium/metabolism , Gene Expression Profiling , Humans , Organ Culture Techniques , Young Adult
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