Long-term nutritional assessment in children with short bowel syndrome

目的 比较不同剩余小肠长度的短肠综合征患儿长期随访营养状况.方法 对我院已脱离肠外营养2年以上的短肠综合征的14例患儿进行随访.随访内容包括人体测量(身高、体重)和血液生化指标测定(血常规;肝功能;血铜、锌、铁、钙、磷、镁;维生素A、维生素E、β-胡萝卜素、维生素B12),并根据剩余小肠长度≤60 cm和＞60 cm分两组进行比较.结果 1例患儿存在轻度营养不良,1例患儿存在重度营养不良,1例患儿超重.≤60 cm和＞60 cm组比较,年龄别身高百分比29.65±19.22、53.31±36.15和Z-评分-0.73±0.90、0.48±2.00、年龄别体重百分比44.39±31.41、47.43±29.42和Z-评分-0.57±1.90、-0.30±0.63以及BMI百分比49.20±39.82、43.71±19.43和Z-评分-0.22±2.11、-0.20±0.57均无统计学意义.剩余小肠≤60 cm组患儿的维生素A、维生素E水平为(46.87±20.07) μg/dl和(4.89±4.57)王g/L均低于剩余小肠＞60 cm组的(152.27±75.98) μg/dl和(11.68±5.97)μg/L,两组比较差异有统计学意义.Hb、MCV、MCH、MCHC、前白蛋白、白蛋白、血铜、锌、铁、钙、磷、镁、β-胡萝卜素、维生素B12水平无差异.结论 短肠综合征患儿脱离肠外营养大于2年的远期营养状况基本正常,剩余小肠长度不同对脱离肠外营养2年后患儿的体格发育情况无明显影响,但是剩余小肠过短脂溶性维生素A、E吸收仍存在问题,需坚持全面随访.     

短肠综合征儿童导管相关性血流感染的单中心回顾性研究

目的评估儿童短肠综合征(SBS)导管相关血流感染(CRBSI)的发生率和危险因素。方法回顾性分析2015年8月至2019年12月收治的19例SBS患儿的临床资料,评估CRBSI的发生率和相关危险因素。结果在19例SBS患儿中,共置管57次,总置管日为1 907天,其中6例患儿共发生11次CRBSI,发生率为5.8次/1000导管日。SBS患儿CRBSI的常见表现为发热(6/11,54.5%)、嗜睡(5/11,45.4%)、体质量不增(7/11,63.6%)和肠造瘘量增加(7/11,63.6%)。与未发生CRBSI组相比,CRBSI组SBS患儿的回盲瓣缺失以及肠外营养持续时间≥120天的比例更高,差异均有统计学意义(P0.05)。SBS患儿发生CRBSI时的血培养共获得11次阳性结果,最常见的病原微生物为肺炎克雷伯杆菌(6/11,54.55%)。结论 CRBSI发生可能与回盲瓣缺失和肠外营养持续时间长有关。     

Endogenous ethanol fermentation in a child with short bowel syndrome

The term "auto-brewery syndrome" has been frequently used to describe patients who show features of alcohol intoxication because of abnormal yeast proliferation after ingesting carbohydrate-rich meals. We present a case of a 3-y-old girl with short bowel syndrome (SBS) who demonstrated signs of alcohol intoxication on repeated occasions. A blood test indicated an ethanol concentration of 15 mmol/l, and cultures from gastric fluid and faeces showed the presence of Candida kefyr. An association was found between the introduction of a carbohydrate-rich fruit drink and the occurrence of symptoms. CONCLUSION: The possibility of endogenous ethanol fermentation should be considered in patients with SBS and the diagnosis of auto-brewery syndrome added to the differential diagnosis list for D-lactic acidosis. Management includes both antifungal treatment and special diet modification.     

Specific IgE antibodies to cow's milk and individual cow's milk proteins in children with suspected allergy to cow's milk

Specific IgE antibodies against whole-milk-antigen mixture and against casein, alpha-lactalbumin and beta-lactoglobulin were determined using the radioallergosorbent test (RAST) in 125 sera of children with an initial diagnosis of suspected cow milk allergy. The results of the RAST were correlated with the clinical diagnosis. No specific IgE antibodies could be demonstrated in 98 children. Of these, 20 (20%) showed clinically intolerance to cow milk presumably on the basis of other immunological mechanisms. Specific IgE antibodies could be demonstrated in the sera of 27 children. Of these, 16 (60%) actually showed allergy to cow milk. A statistically significant association with chi 2 test could be established between positive RAST and clinical diagnosis (p = 0.001).     

Immunosuppression in infants with short bowel syndrome undergoing isolated liver transplantation

BACKGROUND: Little data exist on immunosuppressive drug absorption in children with short bowel syndrome and intestinal failure associated liver disease (SBS-IFALD). AIM: To evaluate the absorption of immunosuppressive medications in children with SBS-IFALD undergoing isolated liver transplantation (iLTx). METHODS: A retrospective review was performed in children with SBS-IFALD undergoing LTx and comparison made with weight, age-matched children undergoing iLTX (extra-hepatic biliary atresia (EHBA) and normal intestinal length and function). RESULTS: Seven children with SBS-IFALD undergoing iLTx (median residual bowel length, 60 cm, range 40-80) were compared with 15 children undergoing LTx for EHBA. SBS-IFALD children had significantly lower trough tacrolimus levels at three months (5.8 vs. 7.9 ng/mL, p<0.05) and six months (5.0 vs. 8.0 ng/mL, p<0.05), but equivalent levels at 12 months after iLTx. The median calculated dose-normalized concentrations indicated that systemic availability of tacrolimus was comparable in two groups at 3, 6, 12 months (33.1 vs. 23.3; 42.4 vs. 36; 51 vs. 52.9) despite the differences in enteral function. The incidence of acute rejection was 1/7 (SBS-IFALD) and 10/15 (EHBA) group (p = 0.06). CONCLUSION: Children with SBS-IFALD demonstrated adequate absorption of oral tacrolimus without significant acute rejection rate after iLTx suggesting that modification of immunosuppression is not necessary.     

Longitudinal study on latex sensitization in children with spina bifida

The course of latex sensitization is rarely documented, and only a few reports about the influence of prophylaxis in the occupational environment have been published concerning health care workers. Therefore we did a follow-up study in the high risk group of patients with spina bifida and evaluated the efficacy of latex prophylaxis. For this purpose we measured IgE antibodies (FEIA) against latex and inhalative allergens in the sera of 67 patients with spina bifida and reevaluated them 0.6 to 4.1 years later, having instructed the patients about prophylactic measures and established a latex-free environment for surgery of all spina bifida patients in our hospital. 37% of the patients did not develop latex antibodies during the follow-up period, 27% showed decreasing levels of antibodies (12% to non-detectable levels), 19% had an increase in latex sensitization (6% newly sensitized), and 9% showed no change in levels of latex antibodies. From our data it may be concluded that surgery without strict latex prophylaxis is the main cause of new sensitization and worsening of preexisting latex antibody levels. Mild sensitization can be reduced by prophylactic measurements to non-detectable antibody levels. With consistent prophylaxis, even symptomatic patients can be operated without risk of allergic complications or increasing antibodies.     

Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: a 25-year experience

OBJECTIVE: To analyze the outcome of children with short bowel syndrome (SBS) who required long-term parenteral nutrition (PN). STUDY DESIGN: Retrospective analysis of children (n=78) with SBS who required PN >3 months from 1975 to 2000. Statistics: univariate analysis, Kaplan-Meier method, and Cox proportional regression model were used. RESULTS: We identified 78 patients. Survival was better with small bowel length (SBL) >38 cm, intact ileocecal valve (ICV), intact colon, takedown surgery after ostomy (all P <.01), and primary anastomosis (P <.001). PN-associated early persistent cholestatic jaundice (P <.001) and SBL of <15 cm (P <.01) were associated with a higher mortality. Intestinal adaptation was less likely if SBL <15 cm (P <.05), ICV was removed, colonic resection was done (both P <.001), >50% of colon was resected (P <.05), and primary anastomosis could not be accomplished (P <.01). Survival was 73% (57), and 77% (44) of survivors had intestinal adaptation. CONCLUSIONS: SBL, intact ICV, intestinal continuity, and preservation of the colon are important factors for survival and adaptation. Adaptation usually occurred within the first 3 years. Need for long-term PN does not preclude achieving productive adulthood. Patients with ICV even with <15 cm of SBL and patients with SBL >15 cm without ICV have a chance of intestinal adaptation.     

Complications of short bowel syndrome

Short bowel syndrome is the commonest reversible cause of intestinal failure. Most of the children are started on parenteral nutrition (PN) after surgery to enable growth and allow time for intestinal adaptation i.e. a process whereby the shorter length of bowel is able to achieve complete function as if the entire length of bowel is present. With advances in management a majority of children with short bowel syndrome are able to discontinue PN and establish on full enteral feeds. This article mainly focuses on the complications of short bowel syndrome that need to be avoided in order for intestinal adaptation to progress and the child to be established on enteral feeds/oral diet.     

Comparison of fecal microflora in children with atopic eczema/dermatitis syndrome according to IgE sensitization to food

Atopic eczema/dermatitis syndrome (AEDS) commonly often arises during early infancy. In several intervention studies a beneficial influence on AEDS course of certain intestinal bacteria, administered as 'probiotics', has been described. To evaluate the possible role of the natural intestinal microflora in children with allergic eczema/dermatitis syndrome regarding immediate type hypersensitivity to food allergens, children with food allergy (AAEDS, n = 68) have been compared with children without detectable food allergy (NAEDS, n = 25). All children (n = 93) in preschool age, mean age of 2.6 (+/-1.8) years, diagnosed with AEDS who were treated as inpatients in 2003 in a dermatological hospital were included. The correlation between fecal microflora, parasites and specific immunoglobulin E (IgE) antibodies against common food allergens was analyzed. A similar composition of intestinal microflora in children with AAEDS and NAAEDS was found. The food allergens that were most frequently detected were egg white, cow milk, casein, peanut and hazelnut. Furthermore, a significant association between IgE sensitization against important food allergens and components of the fecal microflora could not be demonstrated. With aging changes occur in the intestinal microbiota [Proteus/Klebsiella and age (rho = -0.607) and Enterococcus and age (rho = -0.428)]. In two subjects of the AAEDS group Blastocystis hominis was found. The composition of natural intestinal microflora in children with AAEDS and NAAEDS was similar. Hence, there is no evidence of a role of the intestinal microflora with regard to the development of infant (food) allergy in children with AEDS. The possible consequences for allergic diseases later in life require further investigation.     

Prediction of the development of tolerance to milk in children with cow's milk hypersensitivity

OBJECTIVES: To investigate whether the development of tolerance to cow's milk (CM) by aged 4 years can be predicted with a skin prick test (SPT) and measurements of total or specific immunoglobulin E (IgE) in the serum, taken at the time of diagnosis of cow's milk hypersensitivity (CMH). STUDY DESIGN: Infants with immediate (n=95) or delayed (n=67) challenge reactions to CM were prospectively followed to aged 4 years. CMH status was assessed annually by CM challenges. RESULTS: By aged 2, 3, and 4 years, children with delayed reactions developed tolerance to CM faster than those with immediate reactions: 64%, 92%, and 96% versus 31%, 53%, and 63%, respectively. A wheal size of <5 mm in SPT correctly identified 83% of 124 infants who developed tolerance to CM by aged 4 years, and a wheal size of >or=5 mm in SPT correctly identified 71% of 39 infants with persistent CMH. Milk-specific IgE <2 kU/L correctly identified 82% of infants who developed tolerance to CM, and milk-specific IgE >or=2 kU/L correctly identified 71% of infants with persistent CMH. CONCLUSION: SPT and milk-specific IgE in the serum are useful prognostic indicators of the development of tolerance to CM in infants with CMH.     

Prevalence of latex sensitization and allergy in Portuguese children

The prevalence of latex allergy has been increasing not only in risk groups but also in the general population, where it is accepted to average 1%. In children, latex sensitization prevalence studies are scarce and involve different population sampling and allergy testing methods, which makes it difficult to compare across studies. Nevertheless, existing studies point towards a low prevalence of latex allergy in children, which still needs to be confirmed in the Portuguese population. Aiming at studying the prevalence of latex sensitization and allergy in a sample of Portuguese children, we studied 182 children from two different hospital outpatient clinics. A standardized questionnaire focusing on atopic background, previous history and allergic signs or symptoms on exposure to latex or fruits was given to all children and parents. Skin prick testing was performed with a battery of common aeroallergens as well as latex. Serum total IgE, Phadiatop, F x 5E and latex-specific IgE were determined in all children. Specific IgE to latex-crossreacting fruits was determined in latex-sensitized children. Based upon the questionnaire, the prevalence of latex allergy would be 0.5%. The prevalence of latex sensitization would be 3.8%, when based solely upon skin prick testing, and 12.1% (>/=0.35 IU/ml) or 6.6% (>/=0.70 IU/ml) when based singly upon determination of latex-specific IgE. When positive results for either test were considered, the prevalence of latex sensitization was 14.3%. All latex-sensitized children were atopic. Sensitivity to latex-crossreacting foodstuffs was demonstrated in 61.5% of latex-sensitized children (16/26). This study shows that the prevalence of latex allergy and sensitization in Portuguese atopic and non-atopic children, as analysed using various diagnostic methods, is similar to that observed in other countries. In addition, the assessment of latex allergy and sensitization should always include skin prick testing and determination of serum IgE.     

Surgical treatment of the short bowel syndrome

Long-term survival after massive intestinal resection is now possible with parenteral nutritional support. The expense, morbidity, and inconvenience of this therapy, however, has led to continued interest in alternatives for the treatment of the short bowel syndrome. The goals of surgical therapy in the short bowel syndrome are to increase the area of absorption, slow intestinal transit, and reduce gastric hyperacidity. Selected patients with dilated bowel segments benefit from intestinal tapering or lengthening. Growing neomucosa to increase surface area is not yet clinically efficacious. The results of transplantation remain unsatisfactory despite recent advances in immunosuppression. Antiperistaltic segments, colon interposition, and intestinal valves may benefit patients with sufficient absorptive area but rapid intestinal transit. Recirculating loops are associated with prohibitive morbidity and mortality. Intestinal pacing is currently being investigated. Surgical treatment of the short bowel syndrome is not sufficiently safe and effective to recommend its routine use. Operations should be performed only on selected patients to achieve specific goals. Adjunct procedures should not be carried out at the initial intestinal resection. Most important is continued emphasis on the prevention of intestinal resection and conservation of the intestine when massive resection is necessary.     

Management of short bowel syndrome in infancy

Short bowel syndrome (SBS) is a reduction in functioning bowel length which is most often a result of surgical resection. Risk factors in the neonatal period include necrotising enterocolitis, small bowel atresia and gastroschisis. With increasing survival of preterm infants there is an increase in incidence. Management is dependent on the use of parenteral nutrition to maintain fluid and electrolyte homeostasis and promote growth and development with the longer term aim being to promote intestinal adaptation to achieve partial or complete enteral autonomy. In this review we discuss the incidence, aetiology, pathophysiology, medical and surgical treatments and outcome.     

新生儿暂时性短肠综合征治疗体会

目的 探索肠外营养(parenteral nutrition,PN)对新生儿暂时性短肠综合征(temporary short bowel syndrome,TSBS)患儿造瘘期间的治疗作用,并对防治并发症进行探索.方法 回顾性分析2001年1月至2015年12月在我院PN支持治疗的21例新生儿TSBS患儿的诊疗经过,并对其进行随访.结果 符合入组标准的患儿共21例,所有患儿均在新生儿期因各种原因行高位小肠造瘘术,术后需长期依赖PN.其中男12例,女9例.入院年龄1d至8个月(中位年龄2个月),随访3～140个月,中位随访时间21个月.随访至2015年12月时,存活15例,病死6例;存活组中13例关瘘,2例未关瘘;病死组中2例关瘘,4例未关瘘.经Fisher's Exact检验,病死组患儿均发生并发症,存活组仅5例发生并发症(P=0.012);病死组中3例保留回盲部,存活组中9例保留回盲部,两组间差异无统计学意义.经Studentt检验,病死组存留小肠长度(57.5±14.1)cm,显著短于存活组(115.9±46.7)cm(P=0.008).两组患儿的出生体重及入院时年龄别体重z值无统计学意义.结论 新生儿TSBS患儿存留小肠越短,并发症发生率越高,死亡风险越大.合理营养支持对该类患儿后续治疗意义重大,积极随访对预防、早期发现和治疗相关并发症作用明显.     

Blood flow in experimental short bowel syndrome

Mechanisms of intestinal adaptation after major loss of small bowel have been studied extensively but little attention has been paid to related changes in blood flow. In 1972, Touloukian and Spencer were the first to study intestinal blood flow after 50% midintestinal resection in rats [16]. In the present study an 80% midintestinal resection was performed in rats (n = 54). Blood flows to the jejunal and ileal remnants, the anastomosis, and the cecum and colon were measured with the radioactive microsphere method on days 1, 2, 3, 5, and 28 after surgery. Results were compared with blood flow estimates after jejunal or ileal tranection (n = 48) and with normal controls (n = 9). The results showed that the operative stress of intestinal transection is followed by a brief increase in intestinal blood flow. After 80% small-bowel resection, intestinal blood flow remains more significantly elevated for at least 3 days. Blood flow to the ileal remnant remains elevated for at least 4 weeks after surgery. The mechanisms responsible for the increase in blood flow remain to be defined. Offprint requests to: M. E. Höllwarth     

Immunonutritional effects during synbiotics therapy in pediatric patients with short bowel syndrome

The aim of this study was to evaluate the effects of synbiotic therapy in patients with short bowel syndrome (SBS). Four pediatric patients with SBS, who were receiving synbiotics therapy including Bifidobacterium breve, Lactobacillus casei and galactooligosaccharides, were enrolled in this study. We evaluated changes in immunonutritional parameters before and after receiving synbiotics therapy. Four normal, healthy, age-matched children were enrolled as controls. Fecal samples from patients and controls were collected and analyzed for fecal bacterial flora and organic acid (OA) contents. Levels of short chain fatty acids (SCFA) such as butyrate, propionate, and acetate increased in one patient, and SCFA/total OA levels increased in three patients. Serum lymphocyte counts and concentrations of pre-albumin increased after beginning synbiotics therapy, reaching a statistically significant level at the ninth month compared to the pre-treatment level. There was an increasing trend in height and weight gain velocity during the study compared with the pre-treatment period. The patients’ fecal bacterial flora improved as a result of synbiotics therapy. Synbiotics therapy may be very effective at improving the intestinal flora and systemic immunonutritional status of patients with SBS.     

Surgical therapy for short bowel syndrome

Patients with short bowel syndrome (SBS) suffer tremendous morbidity secondary to prolonged hospitalization and chronic parenteral nutrition (TPN). Overall, the majority of infants will adapt and ultimately become independent of TPN, but this process may require many months or years. Reasons for continued TPN dependency include bowel dysmotility, bacterial overgrowth, insufficient adaptation, or very short bowel length. It is this subpopulation of patients who may benefit from surgical procedures that optimize intestinal adaptation and increase the mucosal absorptive surface area. The goal of this review article is to summarize the process of intestinal adaptation and then to outline the surgical principles and techniques available to surgeons who treat this complicated group of patients.     

Basophil Activation Test and specific IgE measurements using a panel of recombinant natural rubber latex allergens to determine the latex allergen sensitization profile in children

There are no documented studies that describe natural rubber latex (NRL) sensitization in children with a history of surgical intervention but without any congenital malformation (urogenital anomalies, spina bifida, etc.), although some authors have studied NRL allergy in children without a history of surgical intervention. The aim of this work was to evaluate the sensitization profile to single NRL allergens in children without spina bifida and without repeated surgical interventions, by using different recombinant and natural latex allergens in two analytical techniques: specific serum immunoglobulin E (IgE) quantification and flow cytometry determination of activated basophils expressing CD63, after stimulating cells from patients with NRL allergens. A total of 23 patients and 10 healthy children were selected. Conjunctival and in-use NRL provocation tests were carried out, as well as specific IgE determination in all patients' and controls' sera with the recombinant NRL allergens: rHev b 1, rHev b 2, rHev b 3, rHev b 5, rHev b 6.01, rHev b 6.02, rHev b 8, rHev b 9 and rHev b 11 and with NRL (k82) using appropriate ImmunoCAPs. The Basophil Activation Test (BAT) was performed with whole latex extract and with the recombinant allergens rHev b 5 and rHev b 6.01, as well as with the natural allergen Hev b 6.02. The sensitivity and the specificity of NRL-specific IgE (k82) were 100%. Positive IgE responses to rHev b 5 were found in sera of 10 children, to rHev b 6.01 in 16 and for rHev b 6.02 in 15 children's sera. Specific IgE to rHev b 8 was found in four sera of the children. We only found significant differences in sensitization to rHev b 5 in children with two or more surgical interventions compared with the non-intervened group or those with only one intervention. Specific IgE in sera of children with latex-fruit syndrome recognized rHev b 6.02, but not to rHev b 11. The patients sensitized to Hev b 8, Hev b 9 and/or Hev b 11 were atopic. The four patients presenting a positive response to the NRL profilin Hev b 8 were allergic to pollen. The BAT against whole NRL extract was positive in 22 of 23 children; against rHev b 5 in 14 of the patients studied; against rHev b 6.01 in seven cases and against nHev b 6.02 in 19 children. In all the control subjects, the results using this technique were negative. If combined rHev b 5, rHev b 6.01 and nHev b 6.02 together, BAT could detect 20 of the 23 children with latex allergy. The combined use of ImmunoCAP with all the recombinant NRL allergens and BAT with rHev b 5, rHev b 6.01 and nHev b 6.02, enabled the identification of NRL allergy in 22 of 23 patients. There is a positive and significant correlation between sensitization to Hev b 5 and the number of interventions. BAT and allergen-specific IgE determination could be used as first-line in vitro diagnostic tests in patients with NRL allergy.