Abnormal vasculature in intestinal neuronal dysplasia

Intestinal neuronal dysplasia (IND) is an intestinal motility disorder, which clinically resembles Hirschsprung's disease (HD). Adventitial fibromuscular dysplasia (AFMD) consists of proliferation of smooth muscle cells and collagen fibers in the adventitia of blood vessels. The purpose of this study was to investigate vascular abnormalities in large bowel biopsies from patients with isolated HD, IND associated with HD and isolated IND. Large bowel biopsies from patients presenting with isolated HD ( n =23), IND associated with HD ( n =11), isolated IND ( n =16) and normal bowel as controls ( n =6) were investigated using acetylcholinesterase (AChE) histochemistry, van Gieson staining and -smooth muscle actin (-SMA) immunohistochemistry. Increased AChE activity around submucosal vessels was found in 9/16 (56%) cases with isolated IND, 3/11 (27%) cases of IND associated with HD, 5/23 (21%) isolated HD cases and 0/6 controls. AFMD was found in 10/16 (62%) of the isolated IND cases, 4/11 (362) of the cases with IND associated with HD and 4/23 (17%) cases of HD without IND using van Gieson staining. None of the control specimens revealed AFMD. Increased -SMA immunoreactivity filaments were demonstrated in the submucosal vessel wall in 9/16 (56%) of isolated IND and 2/11(18%) of IND associated with HD cases. Normal -SMA immunoreactivity around submucosal vessels was seen in isolated HD and controls. Abnormal submucosal vasculature is a common histological finding in isolated IND and IND associated with HD and may be a useful additional diagnostic feature in these patients.     

Clinical impact of intestinal neuronal malformations: a prospective study in 141 patients

A prospective study of 141 consecutive patients with intestinal neuronal malformations is presented. The single malformation of the autonomic nervous system that always required surgical intervention was aganglionosis. Giant ganglia, reduced parasympathetic tone, immature ganglia, and hypogenetic or heterotopic nerve cells were seen in all forms of malformations. However, the incidence in specific malformations was variable. Multiple giant ganglia were identified in all patients with intestinal neuronal dysplasia (IND) type B, but also in various other malformations. Heterotopic nerve cells in the myenteric plexus were seen in the proximal segment of 15 of 74 patients (20.3%) with aganglionosis and 5 of 9 patients (55.6%) with hypoganglionosis. A significant impact on symptoms was found for IND type B: 34 (45.9%) of 74 children with aganglionosis had associated IND type B, and these children more frequently developed ileus (P < 0.001) and more often needed a second resection (P < 0.05) compared to those with isolated aganglionosis. This indicates an additive effect of both malformations, and therefore, in these patients an extended resection should be carried out.Twelve of 67 patients (17.9%) without aganglionosis needed resection for untreatable constipation. This included 7 of 9 children with hypoganglionosis, both patients with heterotopia of the myenteric plexus, 1 of 20 with isolated IND type B, and 2 of 12 with reduced parasympathetic tone. None of the patients with immaturity, heterotopia of the submucous plexus, or mild dysganglionosis required surgery. Six children (8.9%) without aganglionosis underwent sphincteromyotomy and 2 with IND type B had a temporary colostomy. At follow-up (mean 2.4 ± 1.4 years), the outcome in patients with resected aganglionosis was better than in patients who had resections for other malformations; 49 (69%) of 71 patients with aganglionosis were asymptomatic compared to 4 (33.3%) of 12 with other malformations (P < 0.05). It is concluded that some intestinal malformations have a relevant clinical impact. However, the severity of symptoms in the individual patient may not be explained by specific histochemical findings from a limited number of mucosal biopsies. The pathognomonic histochemical criteria of isolated IND typeB — immaturity, reduced parasympathetic tone, heterotopia of the submucous plexus, and mild dysganglionosis —rarely require surgical therapy and should be treated conservatively.     

Length of transplanted small bowel required for adequate weight gain in rats

Progress has been made toward developing a clinically successful small-bowel transplant procedure, but there has been little research concerning the functional aspects of the transplanted small bowel. Using a rat model, our study examined the length of transplanted small bowel required to provide adequate weight gain. The rats were divided into six groups; groups I and 2 were considered controls. Group 1 (n = 6) underwent a gastrostomy. Group 2 (n = 3) underwent a jejunoileectomy followed by re-establishment of intestinal continuity and anastomosis of the native proximal small bowel to an abdominal stoma and the distal portion to the ascending colon. Groups 3 (n = 5), 4 (n = 4), 5 (n = 5), and 6 (n = 4) underwent small-bowel transplantation, receiving 100%, 50%, 25%, and 15% transplants, respectively. The donor small-bowel anastomoses were the same as the native small-bowel anastomoses in group 2. All of the rats began to produce stool within 4 days of becoming dependent upon the transplanted small bowel. By the end of postoperative week 4, there was no significant difference between the percentages of preoperative body weight in groups 1–4 (range 125.7%–130.0%). Although the weight gain in group 5 was significantly less than that in groups 1–4 (P < 0.05), it was adequate (111.8%); group 6 animals lost weight (94.7%). It is concluded that a 50% or more small-bowel transplant with or without an ileocecal valve provides ample weight gain; minimally adequate weight gain is achieved by a 25% transplant without an ileocecal valve; and the graft begins to function soon after transplantation.     

Psychiatric comorbidity in gender dysphoric adolescents

Background: This study examined psychiatric comorbidity in adolescents with a gender identity disorder (GID). We focused on its relation to gender, type of GID diagnosis and eligibility for medical interventions (puberty suppression and cross‐sex hormones). Methods: To ascertain DSM‐IV diagnoses, the Diagnostic Interview Schedule for Children (DISC) was administered to parents of 105 gender dysphoric adolescents. Results: 67.6% had no concurrent psychiatric disorder. Anxiety disorders occurred in 21%, mood disorders in 12.4% and disruptive disorders in 11.4% of the adolescents. Compared with natal females (n = 52), natal males (n = 53) suffered more often from two or more comorbid diagnoses (22.6% vs. 7.7%, p = .03), mood disorders (20.8% vs. 3.8%, p = .008) and social anxiety disorder (15.1% vs. 3.8%, p = .049). Adolescents with GID considered to be ‘delayed eligible’ for medical treatment were older [15.6 years (SD = 1.6) vs. 14.1 years (SD = 2.2), p = .001], their intelligence was lower [91.6 (SD = 12.4) vs. 99.1 (SD = 12.8), p = .011] and a lower percentage was living with both parents (23% vs. 64%, p < .001). Although the two groups did not differ in the prevalence of psychiatric comorbidity, the respective odds ratios (‘delayed eligible’ adolescents vs. ‘immediately eligible’ adolescents) were >1.0 for all psychiatric diagnoses except specific phobia. Conclusions: Despite the suffering resulting from the incongruence between experienced and assigned gender at the start of puberty, the majority of gender dysphoric adolescents do not have co‐occurring psychiatric problems. Delayed eligibility for medical interventions is associated with psychiatric comorbidity although other factors are of importance as well.     

Conventional ventilation in neonates : Experience from Saudi Arabia

Objective. To study the clinical profile and immediate outcome of inborn neonates receiving intermittent positive pressure ventilation (IPPV) at the neonatal intensive care unit of Civil Hospital, Khamis Mushayt, Saudi Arabia, a level II nursery.Methods : 78 liveborn neonates who had received IPPV over a 20 months period from January 1999 to August 2000 were reviewed from their charts and nursery registers. The indications for IPPV and the immediate outcome including complications were studied with respect to various weight groups (1 kg or less, >1–1.25 kg, >1.25–1.5, >1.5–2 kg and >2 kg) and gestation groups (28 weeks or less, 29–32 weeks, 33–36 weeks and full term).Result : Hyaline Membrane disease (n=31, 39.7%) and perinatal asphyxia (n=29, 37.2%) were the major indications for IPPV. 67.9% (53 of the 78) ventilated neonates survived. The chances for survival showed a statistically significant increase with increasing birthweight (P = 0.0006) and with increasing gestational age (P = 0.002). (80%) (44 of 55) of neonates weighing more than 1.25 kg survivedvs 39.1% (9 of 23) of those 1.25 kg or less, P=0.0011. Similarly, 79.3% (46 of 58) of neonates of 29 or more weeks of gestation survivedvs 35% (7 of 20) of those 28 weeks or less, P = 0.0007. The complications seen in the study group included blood culture positive sepsis (n=7), pulmonary hemorrhage (n=6), air leak syndromes (n=4), endotracheal tube related problems (n=5), chronic lung disease (n=3) and retinopathy of prematurity (n=2).Conclusion : Gestational age of less than 28 weeks and birth weight less than 1.25 kg can be recommended as the cut off weight and gestation criteria forin utero transfer in this centre and upgradation of existing facilities are urgently called for to improve the survival rates further.     

Thyroglossal duct cyst’s inflammation. When do we operate?

Thyroglossal duct cyst (TGDC) disease, one of the most common developmental neck lesion in the pediatric population, often presents as infected neck mass. The authors reviewed their experience in the management of inflamed TGDC cases, in order to suggest the most efficient approach of these patients regarding the ideal type and time of surgical intervention. The medical records of all the patients with the diagnosis of TGDC treated at our department from 1988 to 2003 were reviewed. Data collected included age, gender, preoperative inflammation, treatment and time of definitive surgery. The outcome of the operation was graded as successful, recurrence, or postoperative infection. Eighty-nine (89) patients with histologically confirmed TGDC were treated at our department. Mean age at operation was 6.0 years (range, 9 months–14 years). Male to female ratio was 1.2:1. All patients underwent Sistrunk operation and the mean follow-up was 3 years. Fifty-four (54) patients (60.6%) presented with an inflamed TGDC. Among them, 24 patients were operated immediately after diagnosis at the phase of acute inflammation, and 30 patients after antibiotic administration and resolution of inflammation. The overall recurrence incidence was 6.7%. Recurrence was noted in 6 of 24 (25%) patients operated during the acute phase of inflammation and none in patients operated after resolution of inflammation (P=0.0052) or with no preoperative inflammation (P=0.0002). Postoperative wound infection was noted in seven cases but none of them developed recurrence of the disease. The presence of inflammation at the time of surgery is an important risk factor for relapse. We suggest that in the inflamed cases of TGDC disease, the initial treatment should be antibiotic administration and after resolution of the inflammation, surgical management should follow.     

Exhaled nitric oxide in asthmatic and non-asthmatic children: Influence of type of allergen sensitization and exposure to tobacco smoke

Asthmatic bronchial inflammation is associated with increased nitric oxide concentrations in exhaled air (eNO). Recent data suggest that this effect arises from atopy. Our aim in this study was to find out whether atopy and sensitization to particular allergens influences eNO levels. A total of 213 subjects (41 asthmatics and 172 controls) (96 boys and 117 girls, 7.3–14 years of age) were studied. Parents completed a questionnaire that sought information on their children's respiratory symptoms and exposure to tobacco smoke. Subjects underwent skin‐prick tests for the following common allergens: Dermatophagoides pteronyssinus (Dpt), cat fur, Aspergillus fumigatus, Alternaria tenuis, mixed grass, mixed tree pollen, Parietaria officinalis, egg, and cow's milk. eNO was collected in 1‐l mylar bags (exhaled pressure 10 cmH₂O, flow 58 ml/s) and analyzed by using chemiluminescence. Atopic and non‐atopic children without a history of chronic respiratory symptoms had a similar geometric mean eNO (atopics, n = 28, 11.2 p.p.b.; non‐atopics, n = 96, 10.0 p.p.b.; mean ratio 1.1, 95% confidence interval [CI]: 0.7–1.6). Conversely, atopic asthmatic subjects had significantly higher eNO values than non‐atopic asthmatic subjects (atopics, n = 25, 24.8 p.p.b.; non‐atopics, n = 16, 11.4 p.p.b.; mean ratio 2.2, 95% CI: 1.2–3.9, p= 0.000). In children with rhinitis alone (n = 15) and those with lower respiratory symptoms other than asthma (n = 33), eNO increased slightly, but not significantly, with atopy. eNO levels correlated significantly with Dpt wheal size (r = 0.51) as well with the wheal size for cat, mixed grass, and Parietaria officinalis (r = 0.30–0.29), and with the sum of all wheals (r = 0.47) (p= 0.000). Subjects sensitized only for Dpt (but not those subjects sensitized only for grass pollen or other allergens) showed significantly higher eNO levels than non‐atopic subjects (16.4 p.p.b. vs. 10.2 p.p.b., mean ratio 1.6, 95% CI: 1.1–2.3, p= 0.002). In asthmatic subjects, Dpt sensitization markedly increased eNO levels (Dpt‐sensitized subjects: 28.0 p.p.b.; Dpt‐unsensitized subjects: 12.2 p.p.b.; mean ratio 2.3, 95% CI: 1.5–3.5, p= 0.000). Non‐asthmatic Dpt‐sensitized subjects also had significantly higher eNO values than non‐asthmatic, non‐Dpt‐sensitized subjects (14.2 p.p.b. vs. 10.1 p.p.b.; mean ratio 1.4, 95% CI: 1.1–1.9, p= 0.008). No difference was found between eNO levels in asthmatic subjects and control subjects exposed or unexposed to tobacco smoke. In conclusion, eNO concentrations are high in atopic asthmatic children and particularly high in atopic asthmatics who are sensitized to house‐dust mite allergen.     

High molecular weight microtubule-associated protein 2 over-expression in neuronal migration disorders caused by N-methyl-D-aspartate receptor activation in hamsters

To evaluate the neuronal cytoarchitectural changes in neuronal migration disorders, the immunohistochemical expression of microtubule-associated proteins (MAPs) was analyzed using the experimental model induced by ibotenate in newborn hamsters. The cortical lesions observed after intracerebral ibotenate injections strongly resembled the following neuronal migration disorders: (1) microgyria; (2) focal subcortical heterotopia; (3) focal subependymal heterotopia; and (4) leptomeningeal glioneuronal heterotopia. Microgyria and leptomeningeal glioneuronal heterotopia had MAP2 (HM-2: high and low-molecular-weight forms of MAP2) immunoreactive dendritic processes or neuronal elements. The high molecular weight isoform of MAP2 (AP-20), which is more characteristic of mature neurons, showed enhanced expression in neurons of focal subcortical or subependymal heterotopia, although MAP1B (AA6: early form of MAP) immunoreactive elements were not detected in these heterotopic areas. We conclude that high molecular weight isoform of MAP2 is closely associated with cytoarchitectural repair and remodeling of neuronal processes, resulting in neuronal heterotopia after NMDA receptor activation.     

Short duration of skin-to-skin contact: effects on growth and breastfeeding

AIM: To compare weight gain and head growth in very-low-birthweight (VLBW, <1501 g) infants with or without exposure to short duration of skin-to-skin contact (STSC) during their stay in a neonatal intensive care unit. METHODS: Stable VLBW infants were randomised into either STSC or control group. Parents of the STSC group were encouraged to provide STSC for at least 1 h daily. RESULTS: One hundred and forty-six infants were randomised, but only 126 were enrolled (STSC group: n = 64; Controls: n = 62). Infants in the STSC group had better mean weekly increase in head circumference (1.0 cm (SD = 0.3) vs. 0.7 cm (SD = 0.3); P < 0.0001) and higher breastfeeding rate at discharge (29.7% vs. 14.5%; P = 0.04). Although the mean duration of maternal education was longer in STSC (13.0 vs. 12.1 years; P = 0.04) than in controls, linear regression analysis showed that the significant predictors associated with weekly head growth were exposure to STSC (unstandardised coefficient: 0.2; 95% confidence intervals (CI): 0.1, 0.3; P < 0.0001) and head circumference of infants at the time of enrollment (unstandardised coefficient: -0.05; 95% CI: -08, -0.03; P < 0.0001); the number of years of maternal education was not a significant predictor. Logistic regression analysis showed that the only significant predictors of successful breastfeeding at discharge were receiving expressed breast milk at enrollment (adjusted OR: 4.1; 95% CI: 1.4, 11.7; P = 0.009) and receiving expressed breast milk during intervention period (adjusted OR: 8.3; 95% CI: 2.8, 24.4; P < 0.0001); exposure to STSC and maternal education were not significant predictors. CONCLUSION: Exposure to short duration of STSC may promote head growth in VLBW infants.     

Increased prevalence of constipation in pre-school children is attributable to under-consumption of plant foods: A community-based study

Aim: To evaluate consumption of foods rich in dietary fibre and its relation to the prevalence of constipation in pre‐school children. Methods: In total, 368 children aged 3–5 years were randomly selected from kindergartens in Hong Kong. Constipation was confirmed by Rome‐criteria. Children with normal bowel habits served as non‐constipated controls. Consumption of vegetables, fruits, whole‐grain cereals and fluid were determined using a 3‐day food record. Results: A total of 28.8% children were reported to have constipation. Median dietary fibre intake of constipated children was significantly lower than non‐constipated children (3.4 g/d (inter‐quartile range (IQR): 2.3–4.6 g/d) vs. 3.8 g/d (IQR: 2.7–4.9 g/d); P = 0.044) corresponding to 40% reference dietary fibre intake. Constipated children also had significantly lower intakes of vitamin C (P = 0.041), folate (P = 0.043) and magnesium (P = 0.002). Fruit intake and total plant foods intake were significantly lower in the constipated than non‐constipated children: (61 g/d (IQR: 23.8–115 g/d) vs. 78 g/d (IQR: 41.7–144.6 g/d); P = 0.047) and (142.5 g/d (IQR: 73.7–214.7 g/d) vs. 161.1 g/d (IQR: 98.3–233.3 g/d); P = 0.034), respectively. Total fluid intake did not differ between groups but milk intake among the constipated children was marginally higher than the non‐constipated children (P = 0.055) Conclusion: Insufficient dietary fibre intake is common in Hong Kong pre‐school children. Constipated children had significantly lower intakes of dietary fibre and micronutrients including vitamin C, folate and magnesium than non‐constipated counterparts which was attributable to under‐consumption of plant foods. However, milk intake was marginally higher in the constipated children. More public education is necessary for parents to help develop healthy dietary habit and bowel habit in early life in order to prevent childhood constipation.     

Ineffectiveness of Lactobacillus GG as an adjunct to lactulose for the treatment of constipation in children: a double-blind, placebo-controlled randomized trial

OBJECTIVE: To determine if Lactobacillus GG (LGG) is an effective adjunct to lactulose for treating constipation in children. STUDY DESIGN: Eighty-four children (2-16 years of age) with constipation (<3 spontaneous bowel movements [BMs] per week for at least 12 weeks) were enrolled in a double-blind, randomized placebo-controlled trial in which they received 1 mL/kg/day of 70% lactulose plus 10 9 colony-forming units (CFU) of LGG (experimental group, n = 43) or a placebo (control group, n = 41) orally twice daily for 12 weeks. The primary outcome measure was treatment success, and analyses were performed on an intention-to-treat basis. RESULTS: Treatment success, defined as >or=3 spontaneous BMs per week with no fecal soiling, was similar in the control and experimental groups at 12 weeks (28/41 [68%] vs 31/43 [72%], respectively; P = .7) and at 24 weeks (27/41 [65%] vs 27/42 [64%], respectively; P = 1.0]. Groups also did not differ in their mean number of spontaneous BMs per week or episodes of fecal soiling per week at 4, 8, and 12 weeks. Adverse events and overall tolerance did not differ between groups. CONCLUSION: LGG, as dosed in this study, was not an effective adjunct to lactulose in treating constipation in children.     

Comparison of ampicillin plus gentamicin vs. penicillin plus gentamicin in empiric treatment of neonates at risk of early onset sepsis

Aim: We aimed to compare the clinical efficacy of ampicillin (AMP) vs. penicillin (PEN) both combined with gentamicin in the empirical treatment of neonates at risk of early onset neonatal sepsis (EOS). Methods: We performed an open label cluster randomized equivalence study in both Estonian neonatal intensive care units, including neonates with suspected EOS, aged less than 72 h. Primary end‐point was clinical failure rate, expressed by need for change of antibiotic regimen within 72 h and/or 7‐day all cause mortality. Bowel colonization was followed with biweekly perineal swab cultures. Results: Incidence of proven EOS was 4.9%. Among neonates receiving AMP (n = 142) or PEN (n = 141) change of antibiotic regimen within 72 h (10/142 vs. 10/141; OR 1.02; 95% CI 0.40–2.59), 7‐day mortality (11/142 vs. 14/141; OR 0.76; 95% CI 0.33–1.75) and over‐all treatment failure (20/142 vs. 20/141; OR 1.01; 95% CI 0.52–1.97) occurred at similar rates. The only differences in gut colonization were lower number of patients colonised with enterococci, S. aureus and AMP resistant Acinetobacter spp. in AMP and lower number of those with S. haemolyticus and S. hominis in PEN arm. Conclusions: AMP and PEN combined with gentamicin have similar effectiveness in the empiric treatment of suspected neonatal EOS.     

A successful short-bowel syndrome model in neonatal piglets.

BACKGROUND: With the higher survival rate of premature neonates as a result of improved neonatal intensive care, the incidence of necrotizing enterocolitis, and thus the incidence of short-bowel syndrome, is increasing. An appropriate animal model resembling the (premature) neonate with short-bowel syndrome suitable for clinically relevant neonatal bowel adaptation and intervention studies, is not available at present. The purpose of this study was the development of a short-bowel syndrome model that mimics the clinical state of the affected neonatal patient. METHODS: Sixteen 7-day-old piglets received either a small bowel transection (group A) or a 75% resection (group B). The piglets were fed 125 kcal/kg body weight per day, including additional electrolytes. The animals were weighed daily and were killed 28 days after surgery. Bowel samples were obtained at both time points. RESULTS: Mortality rates in groups A and B were 0% and 8%, respectively. Body weight gain was significantly higher in group A than in group B (156% vs. 93%; P = 0.01). Jejunal villus length was higher in group B than in group A (74% vs. -2%; P = 0.006), and crypt depth was higher in group B in both jejunum (201% vs. 67%; P = 0.001) and ileum, (197% vs. 20%; P = 0.001), than in group A. CONCLUSIONS: In 7-day-old piglets 75% small bowel resection leads to a clinical short-bowel syndrome, demonstrated by reduced weight gain and typical changes in bowel adaptation parameters. The excellent survival of the animals provides a possibility for the study of bowel adaptation in a neonatal model as well as in intervention studies.     

Chronic suppurative otitis media in children of Luanda, Angola

Aim: Evaluation of clinical characteristics, bacteriology and hearing in paediatric patients with and without chronic suppurative otitis media (CSOM) in Luanda, Angola. Methods: Interview, clinical examination, ear‐discharge culture, open air pure‐tone audiometry and brainstem auditory‐evoked potentials of 23 outpatients with CSOM and 23 controls in a paediatric hospital. Results: Of the CSOM vs. control children, 35% vs. 26% had running water, 70% vs. 70% electricity, 64% vs. 0% HIV (p < 0.0001) and 36% vs. 0% tuberculosis in history (p = 0.002). Ten (43%) children had bilateral CSOM. The major ear‐discharge pathogens were Proteus spp. (44%) and Pseudomonas (22%). Hearing impairment of >25 dB was present in 52% of CSOM‐affected ears and bilateral hearing loss in 7 (30%) CSOM children vs. zero control child (p = 0.009). Only one hearing‐impaired child’s family had previously detected the handicap. Conclusion: CSOM occurred in children with high co‐morbidity. Persistent otorrhoea was usually caused by Proteus spp. or Pseudomonas, and often suggestive of either HIV or hearing impairment. In the developing countries, prompt diagnosis and treatment of CSOM would enhance the children’s linguistic and academic development.     

Ewing sarcoma: favourable results with combined modality therapy and conservative use of radiotherapy

BACKGROUND: At the Hospital for Sick Children (HSC), we have treated Ewing sarcoma (ES) with multi-agent chemotherapy, surgery and conservative use of radiotherapy for local control. Our objective was to describe the outcome and prognostic factors associated with this strategy. PROCEDURE: We performed a retrospective chart review of children diagnosed with ES at HSC from Feb 1984 to June 1999. RESULTS: Seventy-two evaluable children were identified. All received chemotherapy. Local control administered was surgery (n = 37), radiation (n = 23), both (n = 10) or neither (n = 2). The 7-year EFS was 66.4%. Recurrence occurred in 23 patients, 7 locally and 16 distantly. Better EFS was associated with male gender (78.5% vs. 52.1%; P = 0.007), localised disease (77.0% vs. 39.4%; P = 0.0004), extremity primary (88.2% vs. 52.8%; P = 0.005) and non-pelvic primary (75.7% vs. 18.2%; P < 0.0001). CONCLUSIONS: Favourable outcomes were seen for patients treated with multi-agent chemotherapy, surgery and conservative use of radiotherapy. Metastatic disease rather than local control was the major cause of failure.     

儿童结肠镜检查前聚乙二醇电解质散联合饮食控制肠道准备质量的影响因素分析北大核心CSCD

目的 探讨儿童结肠镜检查前肠道准备质量的影响因素,以及末次服完泻药至开始结肠镜检查的时间间隔(简称等待时间)与肠道准备质量的关系。方法 回顾性分析2020年1~11月入住南京医科大学附属儿童医院消化科使用聚乙二醇电解质散联合饮食控制进行肠道准备并行结肠镜检查的患儿,按照Boston肠道准备评分分为肠道准备充分组(337例)和肠道准备不充分组(30例)。收集两组患儿的一般资料、肠道准备质量可能的影响因素、肠道准备相关的不良反应、结肠镜检查持续时间、术后诊断等,采用单因素及多因素分析探索肠道准备质量的影响因素。结果 单因素分析显示,年龄、体重、等待时间与肠道准备不充分有关(均P<0.05)。多因素分析显示年龄大(OR=2.155,95%CI:1.087~4.273,P=0.028)及等待时间长(OR=1.559,95%CI:1.191~2.041,P=0.001)是儿童肠道准备不充分的独立危险因素。受试者工作特征曲线分析显示等待时间区分肠道准备充分与否的临界值为5.5 h (灵敏度、特异度分别为90.0%、50.7%;曲线下面积为0.708)。进一步按不同等待时间分组,发现≥5.5 h组肠道准备不充分的发生率明显高于<5.5 h组[(27/193,14.0%) vs(3/174,1.7%),P<0.001]。结论 使用聚乙二醇电解质散联合饮食控制进行肠道准备的患儿中,年龄大是结肠镜检查前肠道准备不充分的独立危险因素,可能与较大儿童存在聚乙二醇剂量不足有关。等待时间长亦是肠道准备不充分的独立危险因素,建议等待时间不要超过5.5 h。     

Lack of an association between E‐selectin gene polymorphisms and risk of Kawasaki disease

Background: Coronary artery lesions (CAL) are a serious complication of Kawasaki disease (KD). The increased serum E‐selectin level during the acute phase of KD and the association of E‐selectin gene (SELE) polymorphisms with the prevalence of coronary artery disease in adults suggest a possible association between SELE polymorphisms and the development of CAL in KD patients. Methods: The subjects consisted of 177 KD patients, including 59 with and 118 without CAL, and 305 healthy controls. Two single nucleotide polymorphisms (SNP) of SELE, 98G>T (rs1805193) and Ser128Arg (rs5361), were genotyped by direct sequencing and the high‐resolution melting curve method, respectively. The allele distributions were assessed using the chi‐squared test. Results: There were no significant differences in the T allele frequency at 98G>T between KD patients and controls (1.4% vs 1.0%, P= 0.55) or between KD patients with and without CAL (1.7% vs 1.3%, P= 0.77). Similarly, there were no differences in the distribution of the C allele (128Arg) at Ser128Arg between KD patients and controls (4.5% vs 3.4%, P= 0.40) or between KD patients with and without CAL (4.2% vs 4.7%, P= 0.86). Conclusion: Although no association was detected between these SELE polymorphisms and the prevalence of KD or the development of CAL, this may have been due to the study limitations, including a low frequency of the minor alleles and a small sample size. A larger‐scale association study is needed in order for a definitive conclusion to be made as to whether these SNP are associated with susceptibility to KD or not.     

To tube or not to tube: do infants and children need post-laparotomy gastric decompression?

The purpose of this study was to evaluate the role of nasogastric (NG) decompression after laparotomy in pediatric surgical practice: 94 children who underwent abdominal surgery by a single surgeon were consecutively prospectively managed without postoperative NG tubes. Patients with either bowel obstruction or intra-abdominal infection were excluded from the study. These children were compared with 94 retrospective, matched controls who were routinely managed with postoperative NG decompression by the same surgeon. Data were analyzed with regard to patient, operative, and outcome variables. There was no difference in gender, age (3.8 ± 0.5 vs 3.5 ± 0.4 years, P > 0.7), or postoperative complications (P > 0.8) between the two groups. However, there was a higher incidence of postoperative vomiting (22% vs 11%, P > 0.05) in the children who did not have postoperative NG decompression. Nevertheless, a significant decrease in time to first feed, first stool, and discharge was noted in the group of patients managed without NG tubes (P < 0.05). NG decompression thus need not be routinely used in the pediatric patient undergoing abdominal surgery, as there is no difference in postoperative complications and the hospital stay is shortened. Accepted: 6 November 1997     

Spectrum of skeletal abnormalities in a complex malformation syndrome with "cutis tricolor" (Ruggieri-Happle syndrome)

Background: The term cutis tricolor describes the combination of congenital hyper‐ and hypopigmented skin lesions in close proximity to each other in a background of normal complexion. This phenomenon has been reported: (i) as a purely cutaneous trait; (ii) as a part of a complex malformation syndrome (Ruggieri‐Happle syndrome ‐ RHS); (iii) as a distinct type [cutis tricolor parvimaculata]; (iv) in association with other (e.g. vascular) skin disturbances. Objectives: To delineate the spectrum of skeletal defects in cutis tricolor. Methods: Retrospective and prospective analysis of skeletal surveys in 14 subjects (eight men; six women; aged 2–28 years) with cutis tricolor [4 purely cutaneous trait; 10 syndromic (RHS)]. Results: Bone abnormalities were recorded in 71.4% (10/14) of patients [100% (10/10) of cases with (other‐than‐skeletal) extra‐cutaneous manifestations vs. null (0/4) in cases with purely cutaneous traits] and included overall small skull (n = 6); prognathism (n = 6); ‘J’‐shaped pituitary fossa (n = 1); absence of atlas posterior arch (n = 3); frontal bossing (n = 6); scoliosis (n = 9) with kyphosis (n = 6) and/or lordosis (n = 6); vertebral (n = 9) and ribs (n = 4) defects. Negative ZFHX1B gene analyses excluded overlaps with Mowat–Wilson syndrome. Conclusions: Cutis tricolor may be a marker of underlying skeletal involvement particularly in subjects with a complex syndromic (RHS) phenotype.