Lactobacilli and acidosis in children with short small bowel

BACKGROUND: In patients with a short small bowel, D-lactic acidemia and D-lactic aciduria are caused by intestinal lactobacilli. The purpose of this study was to obtain a detailed picture of the metabolic acidosis in young children with short small bowel. METHODS: Feces, blood, and urine of children with short small bowel and acidosis were studied microbiologically and/or biochemically. RESULTS: Previous findings were confirmed that more than 60% of the fecal flora of patients with small short bowel, who are not receiving antibiotics, consists of lactic acid-producing lactobacilli. In blood, D-lactic acid was the most prominent metabolite: the highest serum D-lactate (15.5 mmol/l) was observed in a sample taken immediately after the onset of hyperventilation. The highest D-lactate excretion was in urine collected some hours after the onset of hyperventilation, and amounted to 59 mol/mol creatinine. Acidosis in the patients with short small bowel was related to strongly increased serum D-lactate and anion gap and to strongly decreased serum bicarbonate and pH. CONCLUSION: In children with small short bowel and acidosis, the common intestinal flora of mainly lactobacilli abundantly produces D-lactic acid from easily fermentable carbohydrates. Thus, these bacteria directly cause shifts of bicarbonate, pH, and base excess and indirectly cause shifts of the anion gap, as well as hyperventilation. These kinetic parameters are strongly associated.     

Nutritional aspects of the short bowel syndrome

Adequate oral nutrition in patients with short bowel syndrome after operation is limited by the amount of intestine left after resection. The remnant small intestine has an important capacity to compensate for the loss of mucosal tissue and develops intestinal hyperplasia when stimulated by a variety of luminal and extraluminal factors such as nutrients, pancreatic biliary secretions, growth factors and hormones. In order to provide appropriate stimuli for the intestinal adaptation after resection, oral feeding is necessary as early as possible after operation. Among nutrients fat, especially LCT and free fatty acids, are the most stimulating nutrients besides proteins and carbohydrates. They increase pancreatic biliary secretions and the excretion of growth promoting hormones such as enteroglucagon. Because of gastric acid hypersecretion the administration of H2 receptor blocking agents (cimetidine) is recommended in these patients. As oral caloric intake is not sufficient in most patients with short bowel syndrome after operation total parenteral nutrition (TPN) or home-TPN has to be installed for longer periods to supplement the appropriate nutritional needs. Both early oral feeding together with TPN or home-TPN have increased considerably the survival rate of children with short bowel syndrome.     

Liver and intestinal transplantation in a child with cystic fibrosis: a case report

Cystic fibrosis (CF) is an inherited disorder that presents as a multisystem disease with meconium ileus being the presenting symptom in 20% of patients. Approximately half of these patients present with complicated meconium ileus mandating early surgical intervention, potentially resulting in short gut syndrome. Although liver transplantation in children with CF has been described, this is the first report of a combined liver and small bowel transplant in a recipient with CF. A 7-month-old boy with CF presented with short bowel syndrome following extensive small bowel resection for meconium ileus and progressive cholestatic liver failure from intravenous hyperalimentation. He underwent combined liver and small intestinal transplant. He was discharged home three weeks post-transplant on enteral feeds with supplemental intravenous fluid. He has had routine protocol small bowel allograft biopsies with no documented rejection episodes. He has been treated for minor respiratory infections without major sequelae. Improvements in pulmonary therapy have impacted on the survival in the CF population to the point where the need for multiorgan transplantation will be increased in the future. Extrapolating from the excellent experience of liver transplantation in children with CF, early liver and small intestinal multivisceral transplantation, if indicated, can be performed safely in children with CF.     

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.     

Physicochemical characteristics and flora of diarrhoeal and recovery faeces in children with acute gastro-enteritis in Kenya.

Physiochemical characteristics and flora of diarrhoeal and recovery faeces were investigated in 14 Kenyan children with acute gastro-enteritis. Causative micro-organisms were Shigella, Campylobacter, enterotoxigenic Escherichia coli, rotavirus and unknown in 6, 2, 1, 2 and 3 patients, respectively. The mean values of the pH of the diarrhoeal specimens were significantly higher than those of the recovery specimens. Large amounts of acetic acid and many other kinds of fatty acids were detected in the recovery specimens, but small amounts and few kinds of fatty acids were detected in the diarrhoeal specimens. Bacterial counts of anaerobic organisms, such as Bacteroides, Bifidobacterium, Lactobacillus and Eubacterium, were lower in the diarrhoeal specimens than in the recovery ones. The normal anaerobic intestinal flora is remarkably disturbed in patients with acute gastro-enteritis. This may result in changes in fatty acid contents and in the pH of diarrhoeal faeces.     

Faecal short chain fatty acids in breast-fed and formula-fed babies

Edwards CA, Parrett AM, Balmer SE, Wharton BA. Faecal short chain fatty acids in breast-fed and formula-fed babies. Acta Pædiatr 1994;83:459–62. Stockholm. ISSN 0803–5253
The intestinal flora of breast-fed infants differs from that of formula-fed infants. It is thought that this difference in flora may be one important reason why breast-fed babies suffer less from gastrointestinal disease. Differences in intestinal flora are reflected in the profile of faecal short chain fatty acids (SCFA). Very little is known about faecal concentrations of SCFA in babies fed breast milk or infant formula. In this study, faecal SCFA were measured in babies at two and four weeks of age who had been either exclusively breast fed or bottle fed from birth. There was no significant difference in total faecal SCFA concentrations between breast-fed and formula-fed babies when lactate was included. The formula-fed group, however, had less lactic acid and higher concentrations of propionic and n-buytric acids than breast-fed babies. Very few babies had significant levels of n-butyric acid, although this SCFA is believed to be important for the health of the colonic mucosa of adults.     

Surgical options for short bowel syndrome

Most children with short bowel syndrome experience spontaneous small bowel adaptation over time. This allows the majority to be weaned from parenteral nutrition. There are, however, some children who cannot be weaned and are potential candidates for techniques to promote intestinal adaptation and intestinal lengthening. Here, surgical therapeutic options are described, literature reviewed, and reported results evaluated. Surgical procedures for children with short bowel syndrome have high complication and failure rates, but in most cases are a less invasive option than intestinal transplantation.     

MEDIUM CHAIN TRIGLYCERIDES FOR TREATMENT OF SPONTANEOUS, NEONATAL CHYLOTHORAX Lipid Analysis of the Chyle

Abstract Volume and contents of lipid and protein in the pleural fluid from a three weeks old girl with spontaneous chylothorax were studied ( a ) during parenteral, nonfatty nutrition and later ( b ) during administration of a formula (Biosorbin®) containing medium chain triglycerides (MCT). The pleural fluid production could not be correlated to the treatment employed but suddenly ceased after 20 days management. Triglyceride and total esterified fatty acid concentrations in pleural fluid were high on admission when feeded with human milk, and chylomicrons and other lipoproteins were present in the chyle. During parenteral treatment a pronounced decrease in pleural fluid concentrations of triglyceride and total fatty acids occurred concomitant with a disappearance of the chylomicrons. During the following MCT diet a pronounced increase in triglyceride and total fatty acids concentrations appeared and the chylomicrons reappeared in the chyle. The cholesterol and phospholipid concentrations in the pleural fluid showed only small changes during the different treatments. No significant changes in protein and albumin concentrations of chyle were observed. It is concluded, that administration of the Biosorbin® MCT formula, containing 871/2% of the fat as MCT, seems without value in the treatment of spontaneous, neonatal chylothorax.     

Medium chain triglycerides for treatment of spontaneous, neonatal chylothorax. Lipid analysis of the chyle.

Volume and contents of lipid and protein in the pleural fluid from a three weeks old girl with spontaneous chylothorax were studied (a) during parenteral, nonfatty nutrition and later (b) during administration of a formula (Biosorbin) containing medium chain triglycerides (MCT). The pleural fluid production could not be correlated to the treatment employed but suddenly ceased after 20 days management. Triglyceride and total esterified fatty acid concentrations in pleural fluid were high on admission when feeded with human milk, and chylomicrons and other lipoproteins were present in the chyle. During parenteral treatment a pronounced decrease in pleural fluid concentrations of triglyceride and total fatty acids occurred concomitant with a disappearance of the chylomicrons. During the following MCT diet a pronounced increase in triglyceride and total fatty acids concentrations appeared and the chylomicrons reappeared in the chyle. The cholesterol and phospholipid concentrations in the pleural fluid showed only small changes during the different treatments. No significant changes in protein and albumin concentrations of chyle were observed. It is concluded, that administration of the Biosorbin MCT formula, containing 871/2% of the fat as MCT, seems without value ih the treatment of spontaneous, neonatal chylothorax.     

Current status of small bowel transplantation in children

Small bowel transplantation is gradually changing from an experimental procedure to a very desirable and viable treatment option in children with irreversible intestinal failure due to either short bowel syndrome or functional impairment. Long term total parenteral nutrition and home parenteral nutrition would be necessary to manage these children in the absence of a small bowel transplant programme. Parenteral nutrition is also associated with complications which can result in chronic liver disease. In India, there is no infrastructure for this treatment option and even if it was there the cost of this method of treatment is likely to be more than the cost of post-operative immunosuppression. Small bowel can be transplanted as an isolated graft, in combination with the liver or as part of a multiviscera! transplant. The operative techniques have been standardised. Major post-operative complications result from sepsis and lymphoproliferative diseases. The best results have been obtained with a combined liver and small bowel transplant.     

Is small bowel bacterial overgrowth of pathogenic significance in persistent diarrhea?

Bacterial overgrowth in the upper small intestine is postulated to cause persistent diarrhea. We compared the fecal and upper intestinal flora in 82 patients with persistent diarrhea aged 36 months and weight-for-length 90% of National Center for Health Statistics standards (NCHS) and 39 non-diarrheal children with age 36 months, nutritionally matched with the patients and residing in the same environment. In the age groups 12 months and Ygt;12 months the duodenal fluid bacterial counts 10⁵/ml were found with similar frequency in patients and controls for aerobic ( p =0.33; p =0.1) and anaerobic ( p =1.0; p =1.0) bacteria. However, the duodenal isolation rates of any aerobic bacteria ( p <0.05) without regard to counts and Enterobacteriaceae ( p = 0.06) were higher in patients than in controls. Colonization with Enterobacteriaceae was directly correlated with increased stool weight over a 7-day observation period ( p <0.05; p <0.01). One or more pathogens were isolated in the feces of 58% of the patients and 43.8% of the controls. Enteroadherent E. coli of the localized [EAEC-L] ( p <0.01) and aggregative [EAggEC] ( p =0.22) phenotypes were isolated more commonly from the feces of patients. The duodenal fluid detection rates for Klebsiella were significantly greater ( p <0.01) in patients, while Giardia lamblia was detected more commonly in the duodenal fluid of controls ( p <0.01). The presence of specific pathogens in the feces did not seem to be related to the extent of small bowel colonization. Thus small bowel overgrowth with aerobes or anaerobes is common in malnourished children with or without diarrhea. However, specific pathogens such as EAEC-L, EAggEC and Klebsiella may be associated with persistent diarrhea.     

Does ileal reverse segment in rats with short bowel syndrome change intestinal morphology?

BACKGROUND: The primary goal of surgical therapy for short bowel syndrome is to increase intestinal absorptive capacity. Many surgical procedures have been described for this purpose. One of these is ileal reverse-segment procedure. This procedure after massive small-bowel resection is an alternative way to treat short bowel syndrome, but how it affects intestinal morphology in short bowel syndrome has not been investigated. The aim of this study is to investigate macroscopic and microscopic effects of reverse-segment procedure on the short bowel. METHODS: Twenty rats underwent resection of 80% of the small bowel. The rats were separated into two groups (n = 10). In the first group (reverse group), a reverse segment was formed by twisting a 2-cm ileal segment 180 degrees, without damaging its vascularity. In the second group (control group), a 2-cm ileal segment was resected, preserving its mesentery, and end-to-end anastomosis was performed to maintain the intestinal passage. The segment was not twisted 180 degrees. The 2-cm proximal (jejunal) and distal (ileal) segments of the resected bowel were reserved for histologic investigation. Two months later, the rats were killed and the jejunal and ileal segments were evaluated morphologically. RESULTS: In the reverse group, body weight and total intestinal length significantly increased (14% more than in the control group). The diameter of both proximal (jejunal) and distal (ileal) segments in the reverse group also increased 53.8% and 22.8%, respectively ( P < 0.05). Histologically, crypt depth and villus height of the ileal segment in the reverse group increased 15.2% and 18.2% more than in the control group ( P < 0.05). No histologic change was observed at the jejunal level except for intestinal muscle thickness. CONCLUSIONS: Ileal reverse-segment procedure in rats with short bowel syndrome 1) does not cause intestinal obstruction, 2) increases total bowel length and body weight, 3) increases the diameter of both jejunal and ileal segments, and 4) increases villus height and crypt depth only at the ileal level. For this reason, reverse-segment procedure positively affects intestinal adaptation.     

Intestinal adaptation in short-bowel syndrome

After massive resection of the small intestine the remannt mucosa has an important capacity to enlarge the absorptive surface for the digestion, hydrolysis and absorption of nutrients. This intestinal adaptation is achieved by the interaction of various factors. Oral nutrients together with pancreatic biliary secretions stimulate the mucosa to become hyperplastic. Secondary to these luminal factors hormones play an important role in the adaptive process. Among the hormones, enteroglucagon is the most important growth promoting agent together with other growth factors such as epidermal growth factor, prostaglandin E₂ and human growth hormone analogues, e.g. plerocercoid growth factor from the plerocercoid larvae of the tapeworm Spirometra mansonoides. The intestinal enterocyte is the target of these factors and within the cell the synthesis of polyamines, which are responsible for rapid growth, is the most essential step for the development of hyperplasia after resection. The rate limiting enzyme for polyamine synthesis ornithine decarboxylase (ODC) reacts to trophic stimuli with an increased activity. Thereafter rapid accumulation of tissue polyamines occurs. Blockade of ODC by specific inhibitors is accompanied by absence of intestinal hyperplasia after resection. Therefore it is concluded that ODC plays a key role in the intestinal adaptation of the remnant small bowel. To start and enhance intestinal hyperplasia after resection in patients with short bowel syndrome introduction of oral nutrition as soon as possible after operation is very important. On account of gastric acid hypersecretion the use of H₂ receptor blocking agents is recommended. A decreased intestinal transit time is treated with loperamide. Adequate nutritional support by enteral and (home) parenteral feeding is the prerequisite for the initiation and development of intestinal adaption in short bowel syndrome.Abbreviations CCK cholecystokinin - CCPR crypt cell production rate - DFMO alpha difluoromethyl-ornithine - EGF epidermal growth factor - FFA free fatty acids - hGH human growth hormone - LCT long chain triglycerides - MCT medium chain triglycerides - ODC ornithine decarboxylase - PBS pancreatic biliary secretion - PGE prostaglandin E₂ - PGF pleroceroid growth factor     

Effect of a valine-rich diet on a rat model of short bowel syndrome

It has been recently reported that valine, which was one of the branched chain amino acids, enhanced liver regeneration after a hepatectomy in rats. The aim of this study is to investigate the effect of enteral valine supplementation on the intestinal adaptation of short bowel syndrome using a rat model. Seven-week-old male Lewis rats underwent a 90% small bowel resection. The rats were randomly divided into two groups; Group V (valine-rich diet which contains valine, five times as the normal amount of valine as that found in standard rat chow) and Group S (standard rat chow), according to the diet each group received. The rats were killed and evaluated at the operative day, and postoperative days (POD) 7, 14, 30, and 60, respectively. The parameters of estimation were body weight (BW), a blood amino acids analysis, a urine organic acids analysis and a morphological examination of the residual small intestines. The BW and the intestinal wet weight, jejunal crypt depth and proliferating cell nuclear antigen positive cells in Group V at POD 7 were significantly higher than in Group S, while those in the Group V at POD 30 and 60 were smaller than in Group S. The urine methylmalonic acid (MMA) level in Group V at POD 30 and 60 was much higher than in Group S. The valine-rich diet was thus found to enhance intestinal regeneration after a small bowel resection in the acute phase. However, the long-term valine-rich diet supplementation was found to disturb the intestinal adaptation, which might be caused by the high production of MMA due to the valine-rich diet. This is the first report in which valine was used as a promoter of intestinal adaptation.     

Pharmacotherapy and growth factors in the treatment of short bowel syndrome

A review of the pharmacologic substances and growth factors that have been studied experimentally and administered clinically for the management of short bowel syndrome is presented. The medical management of short bowel syndrome is multifaceted. In the acute phase, efforts focus on fluid and electrolyte management and the reduction of gastric acid output. As enteral feeding is initiated, antimotility and antisecretory agents may be effective in reducing gastrointestinal losses. Additional modalities of management, including nutrients and growth factors, may be directed at maximizing absorptive function beyond that which occurs with intestinal adaptation. Continued research aimed at further elucidating the process of intestinal adaptation may allow us to use the various peptides and hormones that act as growth factors for the bowel mucosa. Knowledge gained from these studies combined with gene therapy techniques will result in the permanent enhancement of intestinal function beyond the normal adaptation process, eliminate the dependence on total parenteral nutrition, and avoid the need for intestine transplantation.     

Congenital short-bowel; a case study and review of the literature.

A congenital short bowel (CSB) is a rare entity in pediatric surgery. We present the case of a newborn boy with a total small intestinal length of 47 cm, malrotation and gastroesophageal reflux, who is 19 months old at the time of this report. Main treatment steps were Ladd's procedure, a fundoplication and long-term parenteral nutrition. We suggest that missing physiological herniation of the gut into the coelomic cavity may impair normal intestinal growth and rotation and lead to congenital short bowel. Review of all cases reported in the literature shows a considerable mortality of 88%. The limiting factor seems to be reduced motility of the short small bowel causing functional obstruction and liver failure.     

In vitro hydrogen production by enteric bacteria cultured from children with small bowel bacterial overgrowth.

Lactulose breath hydrogen test and Enterotest string test were carried out simultaneously on 19 children 3-5 years old. Bacteria isolated from the jejunal fluid in upper small intestines of these children were incubated with lactulose at neutral pH. Anaerobes were present in all but one child, and in 15 children they were present in numbers greater than 5 log10 organisms per ml. Most of these bacteria did not produce hydrogen in vitro. Hydrogen production (greater than 100 ppm) was observed with the following bacteria: Bacteroides (5%), clostridia (8%), anaerobic corynebacteria (5%), Escherichia coli (67%), Lactobacillus (8%), Staphylococcus (8%), and Streptococcus (9%). The following bacteria did not produce hydrogen in vitro: Actinobacter, Actinomyces, anaerobic cocci, Bifidobacterium, Fusobacterium, micrococci, Neisseria, Sarcina, and Veillonella. This study suggests that in the diagnosis of small bowel bacterial overgrowth using lactulose breath hydrogen test, it is important to consider that patients with a flat breath hydrogen response to a carbohydrate challenge during the first 60 min may be infected with enteric bacteria which are not capable of producing H2.     

Outcome and long-term growth after extensive small bowel resection in the neonatal period: a survey of 87 children.

This retrospective study aims to analyze the outcome, the prognosis factors and the long-term growth of children after extensive small bowel (SB) resection in the neonatal period. PATIENTS AND METHODS: 87 children, born between 1975 and 1991 who had undergone extensive neonatal small bowel resection, were followed up over a mean period of 15 years. Anatomical data influencing PN dependency and duration were analyzed. Data on height and weight were collected and compared using growth standards. Final heights were studied for patients who achieved their puberty and compared to predicted height based on Tanner's formula. Patients were analyzed according to PN weaning and growth: children still receiving PN (group A), patients weaned from initial PN but requiring PN once again or enteral feeding (group B), and children with permanent intestinal autonomy (group C). RESULTS: The overall survival is 89.7 %, depending on the date of birth. The duration of PN-dependency varies according to the intestinal length and the presence of the ileocaecal valve (ICV). All patients who remain PN dependent had less than 40 cm of small bowel and/or the absence of ICV. Patients in group B had a mean small bowel length of 35 +/- 19 cm, resection of the ICV in 50 % of cases, and a PN duration of 47.4 +/- 23.8 months. There was a significant decrease in height and weight gain within the 4 years after cessation of PN, requiring enteral or parenteral feeding. Patients in group C had a mean small bowel length of 57 +/- 19 cm, presence of ICV in 81 % of cases and a PN duration of 16.1 +/- 11.4 months. After PN weaning, they grow up normally with normal puberty and final height as predicted from genetic target height. CONCLUSION: PN duration is influenced by the length of residual SB and the absence of ICV. With good anatomic prognosis factors and short duration of initial PN, normal long-term growth may be predicted. Conversely, poor anatomical factors and protracted initial PN require careful monitoring of growth and may sometimes require nutritional support to be restarted. The last group, permanently dependent on PN, might be candidates for intestinal transplantation.     

Low-saturated fat, low-cholesterol diet in 3-year-old children: effect on intake and composition of trans fatty acids and other fatty acids in serum phospholipid fraction-The STRIP study. Special Turku coronary Risk factor Intervention Project for children

OBJECTIVE: We evaluated whether replacing a proportion of saturated fat with vegetable oils in the diet of young children increases trans fatty acid intake. STUDY DESIGN: Dietary counseling aimed to reach a dietary fat ratio of unsaturated to saturated fat of 2:1 within a total fat intake of 30% to 35% of energy (E%). Four-day food records of 813 3-year-old children were analyzed, and serum phospholipid fatty acid compositions of 25 randomly selected intervention children and 17 control children were analyzed. RESULTS: trans fatty acid intake of the intervention and control children was small (0.8 E% and 0.6 E%, respectively; P <.001). The relative content of serum phospholipid trans 18:1 was closely similar in intervention and control children (1.0% and 0.9% of all fatty acids, respectively). Trans fatty acid intake and serum trans 18:1 correlated poorly with children's serum cholesterol and HDL cholesterol concentrations and inversely with serum phospholipid arachidonic to linoleic acid ratio (r = -0.373). CONCLUSIONS: Trans fatty acid intake of children in Finland is minimal. Dietary intervention replacing saturated with unsaturated fatty acids is safe because it does not increase trans fatty acid intake or the relative content of trans fatty acids in the serum phospholipid fraction.