Children with neurological disorders do not always need fundoplication concomitant with percutaneous endoscopic gastrostomy

Whether antireflux surgery should be routinely performed at the time of gastrostomy in children with neurological disorders is debatable because of the risk of gastroesophageal reflux. Some argue that these children should be screened for occult gastroesophageal reflux as this will determine the need for fundoplication. This study retrospectively examines outcome in 29 children with neurological disorders who underwent percutaneous endoscopic gastrostomy (PEG) without concomitant fundoplication. Children were included if they had no clinical evidence of severe gastroesophageal reflux before PEG insertion. The median age of children at PEG insertion was 5.6 years (range 1.1 to 18.0). The children were followed for a median of 2.6 years (range 0.4 to 4.9). Insertion of PEG was technically impossible in two children; and an asymptomatic gastrocolic fistula in another child led to subsequent tube removal. Fourteen of the 26 remaining children developed symptomatic gastroesophageal reflux after PEG; five of these showed no reflux on pH monitoring prePEG. Control of symptoms was achieved by medical intervention in 12, but two required fundoplication. Our findings indicate that in the child with neurological disabilities without symptoms indicating severe gastroesophageal reflux, fundoplication is unlikely to be necessary as a consequence of PEG insertion. We conclude that routine investigation for gastroesophageal reflux in the child without severe vomiting can be avoided and the number of antireflux procedures reduced.     

Hirschsprung's disease: problems with transition-zone pull-through

BACKGROUND/PURPOSE: It is generally accepted that if surgery for Hirschsprung's disease is to be successful, ganglionic bowel must be anastomosed to the lower rectum or anal canal. Above the aganglionic distal bowel lies a transition zone (TZ) where more subtle abnormalities of innervation are apparent. The significance of this transition zone in respect to the functional outcome of surgery has received little attention. The aim of this study was to identify the incidence of transition zone pull-through (TZPT) in a cohort of children who underwent surgery for Hirschsprung's disease, to identify the reasons why TZPTs occurred, and to identify the functional consequences. The authors report the long-term outcome of these children with emphasis on bowel function and the results of subsequent surgery. METHODS: A Retrospective study was conducted of children treated at a single institution from 1979 through 1994. TZPT patients were subject to detailed review of surgical records and histopathologic material. RESULTS: Thirteen children were identified with a TZPT. In 12 cases, histopathologic errors contributed to the TZPT: in 5 cases this was caused by single point biopsies missing an asymmetrical TZ, whereas in 7 cases the histopathologic features of the TZ were not recognized. In 1 case the TZPT was caused by surgical error. As a consequence of the TZPT 7 children underwent repeat pull-through. One child is fully continent, one has daytime fecal continence, and 2 others are incontinent. Two children have permanent stomas. One child is clean with antegrade colonic washouts. Repeat pull-throughs were not attempted in 6 children. Two children have achieved full continence, 2 have permanent stomas, 1 is clean with antegrade colonic washouts, and 1 child receives regular suppositories. CONCLUSIONS: Transition zone pull-throughs occurred because of a combination of surgical and histopathologic errors. The transition zone may follow an asymmetric course around the circumference of the bowel and may be missed if single-point extramucosal biopsy specimens are taken. Recognition of the subtle histologic features of the transition zone requires an experienced pathologist. The functional consequences of a TZPT are severe, with symptoms of constipation, diarrhea, and incontinence. The results of revisional pull-through were disappointing. Serious consideration should be given to alternative procedures such as the antegrade continence enema operation.     

儿科肝移植术前的血管影像学检查

背景/目的:术前通过血管影像学检查对拟行肝移植术的儿童进行评价存在很大的差异。采用二维多普勒超声扫描(US)、磁共振血管造影术(M R A)和常规血管造影术评价血管变化范围。作者通过比较术前的血管成像结果和术中所见,从而确定这些检查的精确度和有效性。方法:将37例接受尸体     

Diagnostic Criteria for a Curable Form of Chronic Rhinosinusitis: The Mucous Recirculation Syndrome

In clinical practice, nonallergic rhinosinusitis (rhinopathy) is a common diagnosis of exclusion. The mucous recirculation syndrome is one incompletely defined condition that masquerades as nonallergic rhinopathy. Mucous recirculation syndrome, a curable condition, should be differentiated from nonallergic rhinopathy. The underdiagnosis of this condition is due in part to a lack of diagnostic criteria. In this article, we review the medical literature to better characterize mucous recirculation syndrome and to establish diagnostic criteria for it.     

Cognitive Rehabilitation Practice Patterns: A Survey of American Hospital Association

Results of a survey of cognitive rehabilitation (CRT) practice patterns are reported. Of 270 programs contacted, 45% responded. Most patients were under age 65, had a stroke or brain injury, and underwent individual CRT for attention or memory problems while hospitalized. CRT typically lasted 1-6 months and cost $130 per session. Rehearsal and compensation approaches were employed equally, while computer-based procedures were infrequent. Speech and occupational therapists provided services more than neuropsychologists and few programs incorporated neuropsychological testing. Results suggest opportunities for research and expansion of neuropsychological practice and highlight the need for better communication between researchers and clinicians.     

Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis

This report describes a case of chronic neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis. When encountered in children, neutrophilic urticarial dermatosis should raise suspicion of autoimmune or autoinflammatory disease.     

The preterm gut microbiota: changes associated with necrotizing enterocolitis and infection

Aim: To describe gut colonization in preterm infants using standard culture and 16S gene rRNA profiling, exploring differences in healthy infants and those who developed NEC/late onset sepsis (LOS). Methods: Ninety‐nine stools from 38 infants of median 27‐week gestation were cultured; 44 stools from 27 infants had their microbial profiles determined by 16S. Ordination analyses explored effects of patient variables on gut communities. Results: Standard microbiological culture identified a mean of two organisms (range 0–7), DGGE 12 (range 3–18) per patient. Enterococcus faecalis and coagulase negative staphylococci (CONS) were most common by culture (40% and 39% of specimens). Meconium was not sterile. No fungi were cultured. Bacterial community structures in infants with NEC and LOS differed from healthy infants. Infants who developed NEC carried more CONS (45% vs 30%) and less Enterococcus faecalis (31% vs 57%). 16S identified Enterobacter and Staphylococcus presence associated with NEC/LOS, respectively. Conclusions: Important differences were found in the gut microbiota of preterm infants who develop NEC/LOS. The relationship of these changes to current practices in neonatal intensive care requires further exploration.     

Functional abnormalities of heparan sulfate in mucopolysaccharidosis-I are associated with defective biologic activity of FGF-2 on human multipotent progenitor cells

In mucopolysaccharidosis-I (MPS-I), alpha-L-iduronidase deficiency leads to progressive heparan sulfate (HS) and dermatan sulfate (DS) glycosaminoglycan (GAG) accumulation. The functional consequences of these accumulated molecules are unknown. HS critically influences tissue morphogenesis by binding to and modulating the activity of several cytokines (eg, fibroblast growth factors [FGFs]) involved in developmental patterning. We recently isolated a multipotent progenitor cell from postnatal human bone marrow, which differentiates into cells of all 3 embryonic lineages. The availability of multipotent progenitor cells from healthy volunteers and patients with MPS-I (Hurler syndrome) provides a unique opportunity to directly examine the functional effects of abnormal HS on cytokine-mediated stem-cell proliferation and survival. We demonstrate here that abnormally sulfated HS in Hurler multipotent progenitor cells perturb critical FGF-2-FGFR1-HS interactions, resulting in defective FGF-2-induced proliferation and survival of Hurler multipotent progenitor cells. Both the mitogenic and survival-promoting activities of FGF-2 were restored by substitution of Hurler HS by normal HS. This perturbation of critical HS-cytokine receptor interactions may represent a mechanism by which accumulated HS contributes to the developmental pathophysiology of Hurler syndrome. Similar mechanisms may operate in the pathogenesis of other diseases where structurally abnormal GAGs accumulate.