Format
Sort by
Items per page

Send to

Choose Destination

Search results

Items: 1 to 20 of 2875

1.
Zhejiang Da Xue Xue Bao Yi Xue Ban. 2019 Jul 25;48(5):487-492.

[Therapeutic experience of type Ⅲ-b congenital intestinal atresia].

[Article in Chinese]

Author information

1
The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.

Abstract

OBJECTIVE:

To summarize the clinical characteristics and treatment of type Ⅲ-b congenital intestinal atresia (CIA).

METHODS:

The clinical data of 12 type Ⅲ-b CIA treated in the Children's Hospital of Zhejiang University School of Medicine from January 2015 to December 2017 were analyzed retrospectively.

RESULTS:

Of the 12 patients diagnosed as type Ⅲ-b CIA in operation, treatment was refused during operation by their parents in 2 cases. For one child, only the proximal intestine was partly resected in the first operation, dilatation and dysplasia of the duodenum was diagnosed and total duodenum was resected and sutured in the second operation, as the child had postoperative intestinal obstruction. For one child, due to the long distal normal intestine, distal apple-peel like intestine was partly resected without mesenteric reformation. For the rest 8 children total duodenum resection and mesenteric reformation were performed. During the postoperative follow-up, one case was early rejected for further treatment by parents, one case died from complex congenital heart disease, 5 cases had the complication of short bowel syndrome. All 8 survival children received parenteral nutrition support after operation, 5 of whom received parenteral nutrition support for more than 42 days, and they were followed up for 1-3 years after discharge. The short-time efficacy was satisfactory.

CONCLUSIONS:

For children with type Ⅲ-b CIA, the distal apple-peel like intestine should be preserved as much as possible, the mesenteric reformation should be performed and the proximal dilated bowel should be partly resected and sutured. Postoperative nutritional support and early intestinal rehabilitation contribute to the compensation for rest intestines.

PMID:
31901021
[Indexed for MEDLINE]
Icon for Zhejiang University Press
2.
Surg Clin North Am. 2019 Dec;99(6):1209-1221. doi: 10.1016/j.suc.2019.08.010. Epub 2019 Sep 23.

Inflammatory Bowel Disease and Short Bowel Syndrome.

Author information

1
Department of Surgery, Division of General Surgery, University of Nebraska Medical Center, 983280 Nebraska Medical Center, Omaha, NE 68198-3280, USA.
2
Department of Surgery, Division of General Surgery, University of Nebraska Medical Center, 983280 Nebraska Medical Center, Omaha, NE 68198-3280, USA. Electronic address: jthompso@unmc.edu.

Abstract

Short bowel syndrome / intestinal failure (SBS/IF) is a rare and debilitating disease process that mandates a multidisciplinary approach in its management. Inflammatory bowel disease (IBD), in particular Crohn's disease (CD), predisposes patients to development of SBS/IF. This review discusses SBS/IF from the perspective of IBD, with an emphasis on prevention and treatment in the setting of CD. The aims of this review are to emphasize the unique treatment goals of the newly diagnosed SBS/IF patient, and highlight the role of both medical and surgical therapies in the management of IBD-related SBS/IF, including intestinal transplantation.

KEYWORDS:

Crohn’s disease; Inflammatory bowel disease; Intestinal failure; Short bowel syndrome; Ulcerative colitis

PMID:
31676059
DOI:
10.1016/j.suc.2019.08.010
[Indexed for MEDLINE]
Icon for Elsevier Science
3.
Transplant Proc. 2019 Nov;51(9):3178-3180. doi: 10.1016/j.transproceed.2019.07.006. Epub 2019 Oct 13.

Kidney After Intestinal Transplantation Using Two Different Living Donors: A First Case Report.

Author information

1
Department of Surgery, State University of New York, Downstate, Brooklyn, New York.
2
Department of Surgery, State University of New York, Downstate, Brooklyn, New York. Electronic address: Rainer.gruessner@downstate.edu.

Abstract

We describe a unique case of a 53-year-old woman who underwent a nonrelated living donor kidney transplant 9 years after a previous small bowel transplant from her sister. The patient had suffered from short bowel syndrome secondary to volvulus after undergoing bariatric surgery for morbid obesity. Her entire small bowel had to be resected emergently, but she also developed acute kidney failure at the time. This initial kidney injury associated with long-term exposure to calcineurin-inhibitor medication eventually led to end-stage renal disease. A successful kidney transplant from a different, nonrelated adult donor was performed. Of note, the unrelated kidney donor matched exactly the 2 HLA-A and HLA-B antigens that the recipient had not matched with her sister. We discuss the unique HLA configuration between the patient and her 2 living donors, the absence of posttransplant rejection and posttransplant immunosuppressive therapy. To our knowledge this is the first published report of a successful kidney after a previous bowel transplant using (2 different) living donors.

[Indexed for MEDLINE]
Icon for Elsevier Science
4.
Acta Cir Bras. 2019 Sep 16;34(7):e201900705. doi: 10.1590/s0102-865020190070000005.

Botulinum toxin as a treatment for short bowel syndrome in rats.

Author information

1
Fellow PhD degree, Postgraduate Program in Pathology, Department of Pathology and Legal Medicine, School of Medicine of Ribeirao Preto, Universidade de São Paulo (USP), Ribeirao Preto-SP, Brazil. Conception, design, and scientific content of the study.
2
Fellow PhD degree, Postgraduate Program in Pathology, Department of Pathology and Legal Medicine, School of Medicine of Ribeirao Preto, USP, Ribeirao Preto-SP, Brazil. Histopathological examinations, statistical analysis.
3
Graduate student, Department of Pathology and Legal Medicine, School of Medicine of Ribeirao Preto, USP, Ribeirao Preto-SP, Brazil. Design and scientific content of the study.
4
Fellow PhD degree, Postgraduate Program in Veterinary Surgery, Faculty of Veterinary Medicine, Universidade Estadual Paulista (UNESP), Jaboticabal-SP, Brazil. Design and scientific content of the study.
5
PhD, Assistant Professor, Faculty of Veterinary Medicine, UNESP, Jaboticabal-SP, Brazil. Conception, design, intellectual and scientific content of the study.
6
PhD, Associated Professor, Department of Surgery and Anatomy, School of Medicine of Ribeirao Preto, USP, Sao Paulo-SP, Brazil. Conception, design, intellectual and scientific content of the study; critical revision.
7
PhD, Full Professor, Department of Pathology and Legal Medicine, School of Medicine of Ribeirao Preto, USP, Sao Paulo-SP, Brazil. Conception, design, intellectual and scientific content of the study; critical revision; final approval.

Abstract

PURPOSE:

The denervation of the intestine with benzalkonium chloride (BAC) reduces mortality and improves weight gain in rats with short bowel syndrome (SBS). Nevertheless, translating these promising findings from bench to bedside is not feasible because BAC promotes peritonitis and irreversible denervation which may be followed by an uncontrolled dilatation of the viscera. The use of botulinum toxin (BT) instead of BAC to achieve the denervation of the remaining small intestine in SBS could be an interesting option because it leads to a mild and transient denervation of the intestine.

METHODS:

Here we evaluated the effects of the ileal denervation with BT in rats with SBS by verifying the body weight variation and intestinal morphological parameters. Four groups with 6 animals each were submitted to enterectomy with an ileal injection of saline (group E) or BT (group EBT). Control groups were submitted to simulated surgery with an ileal injection of BT (group BT) or saline (group C - control).

RESULTS:

We observed that the treatment of the remaining ileum with BT completely reversed the weight loss associated to extensive small bowel resection.

CONCLUSION:

This may provide a new promising approach to the surgical treatment of SBS.

PMID:
31531527
PMCID:
PMC6756011
DOI:
10.1590/s0102-865020190070000005
[Indexed for MEDLINE]
Free PMC Article
Icon for Scientific Electronic Library Online Icon for PubMed Central
5.
J Laparoendosc Adv Surg Tech A. 2019 Oct;29(10):1330-1333. doi: 10.1089/lap.2019.0048. Epub 2019 Aug 22.

Radiologic Image-Guided Tube Stoma Insertion in Neonatal Short Bowel Syndrome: First Case Report in the Literature.

Author information

1
Division of Paediatric and Thoracic Surgery, Sidra Medicine, Doha, Qatar.
2
Division of Paediatric Interventional Radiology, Sidra Medicine, Doha, Qatar.

Abstract

Background: The management of neonatal short bowel syndrome can be challenging, and it is critical that these babies are managed in a multidisciplinary team setting with specialists who are experienced in the complex management of these children. One of the surgical strategies, initially published by the Bianchi team in Manchester, UK, is controlled tissue expansion program (CTE) which is done via the insertion of catheters into the proximal and distal bowel in the form of tube stomas. The clamping of the proximal tube allows for an increase in length and circumferential diameter of the proximal bowel for a period of time, whilst the distal tube stoma allows for easy refeeding of proximal bowel contents into the distal bowel. Method: CTE is associated with the risk of dislodgement and exposing patients to further surgical procedures with the risk of losing more bowel length. This article describes a new method in the management of such a complication through a less invasive approach of an image-guided procedure by interventional radiologists. Conclusion: Radiologically guided tube stoma reinsertion in a child with ultrashort bowel syndrome is a promising technique and should be considered in a CTE program in the management of short bowel syndrome.

KEYWORDS:

bowel expansion; minimal invasive; short bowel; tube stoma

PMID:
31436514
DOI:
10.1089/lap.2019.0048
[Indexed for MEDLINE]
Icon for Atypon
6.
Dtsch Med Wochenschr. 2019 Aug;144(15):1061-1066. doi: 10.1055/a-0651-4481. Epub 2019 Jul 26.

[Intestinal Rehabilitation in Short Bowel Syndrome and Chronic Intestinal Failure].

[Article in German; Abstract available in German from the publisher]

Author information

1
Klinik für Innere Medizin und Gastroenterologie, Asklepios-Klinik St. Georg, Asklepios Medical School, Hamburg.
2
Medizinische Klinik für Hepatologie und Gastroenterologie, Charité Campus Mitte, Charité-Universitätsmedizin Berlin.

Abstract

Patients with severely orally decompensated malsaborption due to surgical rescetion suffer from short bowel syndrome (SBS). The resultant decrease in intestinal surface area after rescetion of parts of the intestines leads to chronic functional intestinal insufficiency or even complete failure (cIF) which requires parenteral fluid and nutrient restoration for establishment of metabolc stability. Since the introduction of glucagon-like peptide (GLP)-2 analogues such as teduglutide functional rehabilitation of the remaining gastrointestinal tract has become a causal treatment option. Teduglutide functionally and effectively enhances intestinal absorption of fluid as well micro- and macronutrients allowing a reduction of parenteral nutrition and/or volume with even complete weaning off in some patients. This results increased metabolic stability, oral autonomy, quality of life and putatively less complications.

PMID:
31350749
DOI:
10.1055/a-0651-4481
[Indexed for MEDLINE]
Icon for Georg Thieme Verlag Stuttgart, New York

Conflict of interest statement

U.-F. Pape gibt an, von Shire Pharma und Fresenius Kabi finanzielle Unterstützung bei Forschungsvorhaben erhalten zu haben sowie von Shire Pharma Honorare für Vortragsleistungen und die Teilnahme an Advisory-Boards bezogen zu haben.S. Maasberg gibt an, von Shire Pharma Honorare für die Teilnahme an Advisory-Boards erhalten zu haben.

7.
Nutrients. 2019 Jul 13;11(7). pii: E1587. doi: 10.3390/nu11071587.

Cardiac Changes in Patients on Long-Term Parenteral Nutrition.

Author information

1
Internal Medicine and Clinical Nutrition, Department of Clinical Medicine and Surgery, Federico II University, Via Pansini, 580131 Naples, Italy. lidia.santarpia@unina.it.
2
Cardiovascular Emergencies and Onco-hematologic Complications, Department of Advanced Biomedical Sciences, Federico II University Medical School, 80131 Naples, Italy.
3
Internal Medicine and Clinical Nutrition, Department of Clinical Medicine and Surgery, Federico II University, Via Pansini, 580131 Naples, Italy.

Abstract

Patients with short bowel syndrome (SBS) on long-term home parenteral nutrition (HPN) chronically receive high fluid volumes directly into the right atrium (RA) through the superior vena cava. We retrospectively evaluated cardiac function measured by routine transthoracic echocardiography (TTE) in a population of 26 SBS patients on long-term HPN and compared their data on echocardiograph-derived right heart structure and function, with those of a control group of 26 patients also bearing a central venous catheter (CVC) for other reasons. Results showed that body weight and BMI were significantly higher in the control group. The echocardiographic estimate of RA pressure was higher in HPN patients than in controls (p = 0.01). An increased estimate of RA pressure indicates the need to consider TTE in the follow-up of long-term HPN patients to detect functional impairment early.

KEYWORDS:

cardiac changes; home parenteral nutrition; short bowel syndrome; transthoracic echocardiography

PMID:
31337013
PMCID:
PMC6682873
DOI:
10.3390/nu11071587
[Indexed for MEDLINE]
Free PMC Article
Icon for Multidisciplinary Digital Publishing Institute (MDPI) Icon for PubMed Central
8.
EBioMedicine. 2019 Aug;46:444-451. doi: 10.1016/j.ebiom.2019.07.016. Epub 2019 Jul 17.

Effects of glepaglutide, a novel long-acting glucagon-like peptide-2 analogue, on markers of liver status in patients with short bowel syndrome: findings from a randomised phase 2 trial.

Author information

1
Department of Medical Gastroenterology and Hepatology, Rigshospitalet, Copenhagen, Denmark. Electronic address: rahim.mohammad.naimi@regionh.dk.
2
Department of Medical Gastroenterology and Hepatology, Rigshospitalet, Copenhagen, Denmark.
3
Department of Surgical Gastroenterology, Rigshospitalet, Copenhagen, Denmark.
4
Department of Hepatology & Gastroenterology, Aarhus University Hospital, Denmark.
5
Department of Clinical Biochemistry, Aarhus University Hospital, Denmark.
6
Research & Development, Zealand Pharma, Glostrup, Denmark.

Abstract

BACKGROUND:

With the introduction of glucagon-like peptide-2 (GLP-2) in the treatment of short bowel syndrome (SBS), there is emerging evidence that GLP-2 may play a role in the restoration of the disturbed homeostatic feedback in the gut-liver axis and may ameliorate SBS-associated liver damage. We have previously presented that daily subcutaneous injections with 1 and 10 mg of glepaglutide improved intestinal function in patients with SBS. As exploratory endpoints, we here assessed the effect of glepaglutide on liver function.

METHODS:

Liver tests, transient elastography (TE) with controlled attenuation parameter (CAP), indocyanine green (ICG) kinetics, soluble CD163 (sCD163), soluble mannose receptor (sMR), and lipopolysaccharide binding protein (LBP) were assessed in 18 patients with SBS in a randomised, cross-over, dose-finding phase 2 trial before and after three weeks of treatment with glepaglutide. This trial is completed and registered at ClinicalTrials.gov: NCT02690025.

FINDINGS:

Between Feb 2016 and Jan 2017, 22 patients with SBS were screened. Of these, 18 patients were randomised and treated with glepaglutide; 16 patients completed the trial. Treatment with glepaglutide was associated with increase in TE and ICG-elimination. In the 10 mg dose group, glepaglutide increased sCD163 by 0·44 mg/mL (P = 0·0498), and alkaline phosphatase (ALP) decreased in the 1 mg dose group by 33 U/L (P = 0·032). CAP, sMR, LBP, liver transaminases, and INR were not affected.

INTERPRETATION:

Glepaglutide may improve hepatic excretory function, but at the same time activate resident liver macrophages and increase liver stiffness. The excretory and the stiffness findings may to some extent relate to increased splanchnic blood flow which would not influence the marker of macrophage activation. Thus, glepaglutide exerted diverse effects on liver status that call for attention in future studies.

FUNDING:

Zealand Pharma.

KEYWORDS:

Indocyanine green; Short bowel syndrome; Soluble CD163; Soluble mannose receptor; Transient elastography

PMID:
31326433
PMCID:
PMC6710908
DOI:
10.1016/j.ebiom.2019.07.016
[Indexed for MEDLINE]
Free PMC Article
Icon for Elsevier Science Icon for PubMed Central
9.
Expert Rev Gastroenterol Hepatol. 2019 Aug;13(8):785-796. doi: 10.1080/17474124.2019.1640600. Epub 2019 Jul 15.

Restoring gut physiology in short bowel patients: from bench to clinical application of autologous intestinal reconstructive procedures.

Author information

1
a Emergency General Surgery, St. Orsola University Hospital-Alma Mater Studiorum , Bologna , Italy.
2
b Department of Pediatric Surgery, Pediatric Autologous Bowel Reconstruction, Rehabilitation & Regenerative Medicine Unit, Meyer Children's Hospital , Florence , Italy.
3
c Department of NEUROFABRA, University of Florence , Florence , Italy.

Abstract

Introduction: Short bowel syndrome represents the leading etiology that causes intestinal failure both in children and adults. Total parenteral nutrition support has dramatically improved the prognosis for these patients but, if related irreversible complications occur, the alternative is represented by surgery and/or transplantation. Areas covered: Autologous gastrointestinal reconstructive procedures are a feasible, alternative approach with good long-term outcome data inexperienced surgical centers. Expert opinion: Ongoing innovative efforts have driven the surgical options for successful autologous reconstructive surgery: bowel elongation/tapering techniques (LILT, STEP, and the new SILT) together with the 'reversed bowel segment' procedure are now recognized procedures and all must be tailored to the individual patient needs to obtain the optimal result in terms of enteral autonomy. Background laboratory experimentation with new procedures e.g. options for bowel dilation techniques and distraction-induced enterogenesis, may provide additional management and treatment modalities.

KEYWORDS:

Short bowel syndrome; bowel rehabilitation; experimental surgery; parenteral nutrition; surgical rescue

PMID:
31282770
DOI:
10.1080/17474124.2019.1640600
[Indexed for MEDLINE]
Icon for Taylor & Francis
10.
PLoS One. 2019 May 16;14(5):e0215351. doi: 10.1371/journal.pone.0215351. eCollection 2019.

The bacterial communities of the small intestine and stool in children with short bowel syndrome.

Author information

1
Departments of Pediatrics and Microbiology, Immunology, and Cancer Biology, University of Virginia, Charlottesville, Virginia, United States of America.
2
University of Maryland School of Medicine, Institute for Genome Sciences Department of Microbiology & Immunology, Baltimore, Maryland, United States of America.
3
Department of Microbiology, Immunology, and Cancer Biology, George Washington University, District of Columbia, United States of America.
4
Gastroenterology, Hepatology and Nutrition, Children's National Medical Center, District of Columbia, United States of America.

Abstract

Short bowel syndrome (SBS) presents an increasing problem in pediatrics. SBS often results from surgical resection of necrotic bowel following necrotizing enterocolitis or treatment of anatomic gastrointestinal defects. SBS is associated with significant morbidity and mortality, and creates substantial burdens for patients, families, and the health system. Recent reports have demonstrated that the fecal microbiome of children with SBS is significantly different from healthy control and severe intestinal microbial imbalances is associated with poor growth. We hypothesized that children with SBS and adverse clinical features such as PN dependent, shorter bowel length and lack of ileocecal valve would demonstrate more gut dysbiosis compare with the SBS non-PN dependent. An improved understanding of SBS pathogenesis would enhance management and potentially suggest new interventions. We studied microbial communities of SBS and control non-SBS patients from the jejunum, obtained endoscopically or by ostomy aspiration, and stool. We enrolled SBS patients who did and did not require parenteral nutrition (PN), as a surrogate marker for the seriousness of their disease. We studied the microbiota using high-throughput DNA sequencing of 16S rRNA genes and statistical analyses. We found that microbial diversity was significantly greater in jejunal aspirate than in stool samples in SBS patients, unlike non-SBS patients; that SBS patients receiving enteral feeds had greater diversity, and that SBS patients on PN and enteral feeds had lower differences in diversity in jejunal vs. stool samples. We found a trend toward increased diversity in patients with an intact ileocecal valve, and found that certain taxa were more abundant in the certain sample types, and in SBS patients vs. non-SBS patients. SBS patients have lower microbial diversity, especially patients with more severe disease, patients requiring PN, and those lacking an ileocecal valve. SBS patients, particularly those with more complex characteristics, exhibit differences in their intestinal microbiota. Particular individual taxa were over- and under-represented in patients with more unfavorable disease. While diminished diversity and alterations in microbiota composition are likely consequences of SBS, future efforts aimed at increasing microbial diversity and interventions targeting specific microbiota characteristics might constitute a testable approach to ameliorate some clinical SBS clinical consequences.

PMID:
31095575
PMCID:
PMC6521997
DOI:
10.1371/journal.pone.0215351
[Indexed for MEDLINE]
Free PMC Article
Icon for Public Library of Science Icon for PubMed Central
12.
Cir Pediatr. 2019 Apr 22;32(2):74-80.

[Efficacy of the intestinal rehabilitation program in patients with short bowel syndrome].

[Article in Spanish; Abstract available in Spanish from the publisher]

Author information

1
Servicio de Cirugía Pediátrica. Hospital Universitari i Politècnic la Fe. Valencia.

Abstract

INTRODUCTION:

In recent decades, intestinal rehabilitation programs, advances in parenteral nutrition (PN) and intestinal lengthening techniques have improved the results of patients with short bowel syndrome (SBS).

OBJECTIVE:

To evaluate the growth, the independence of PN and the survival of patients with SBS diagnosed in the last 12 years.

MATERIAL AND METHOD:

Retrospective review between the years 2007-2016. Defining SBS as the inability of the intestine to provide complete absorption via the enteral route being necessary PN. A multivariate analysis was performed to assess the prognostic factors regarding the autonomy of the NE controlled by Cox regression: ileocecal valve presence (qualitative variable: yes/no), small intestine length and gestational age (both quantitative variables).

RESULTS:

18 patients were evaluated. The most frequent causes of SBS: necrotizing enterocolitis (6, 33.3%), jejunal atresia and Hirschsprung (4 cases each pathology, 22.2%) and others (4). The average intestinal length was 51.17 cm at diagnosis, 72.2% lacked an ileocecal valve. The mean PN at the start was 115.8 hours / week, currently: 56.9. The 22.2% achieved complete enteral nutrition (CEN) after an average time of 4.62 years. Serial transverse enteroplasty was performed in 3 patients.The presence of an ileocecal valve was a protective factor to achieve CEN (p<0.018). In contrast, intestinal length and gestational age were not significant. After a follow-up of 5.38 years (3 exitus, 9.6 months on average), no patient was a candidate for intestinal transplantation.

CONCLUSIONS:

The survival of patients with SBS has improved in recent decades due to intestinal rehabilitation programs, advances in PN and intestinal lengthening techniques. It is possible to achieve NEC and avoiding intestinal transplantation.

KEYWORDS:

Intestinal rehabilitation; Intestinal transplant; Parenteral nutrition; Short bowel syndrome

PMID:
31056867
[Indexed for MEDLINE]
13.
Mo Med. 2019 Mar-Apr;116(2):129-133.

Pediatric Intestinal Failure: A Review of the Scope of Disease and a Regional Model of a Multidisciplinary Care Team.

Author information

1
Nisha Mangalat, MD, is the Medical Director, Glennon Intestinal Rehabilitation and Feeding Program, Department of Pediatrics, Saint Louis University, St. Louis, Missouri.

Abstract

The term "intestinal failure" signifies the inability of the body to meet the digestive, absorptive and nutritive needs of the body. In children, intestinal failure is most often due to short bowel syndrome, often a result of necrotizing enterocolitis, a severe GI ischemic pathology that is generally associated with prematurity. With advances in neonatal care, more preterm infants are surviving, and subsequently we care for more children with SBS than ever before. These children require parenteral nutrition (PN) for survival. Neurodevelopmental outcomes are tied to nutrition in early years; thus these children are the most vulnerable to the sequelae of intestinal failure. As such, the development of multi-disciplinary intestinal rehabilitation programs have emerged as the state of the art in the care of children with intestinal failure.

PMID:
31040499
PMCID:
PMC6461325
[Indexed for MEDLINE]
Free PMC Article
Icon for PubMed Central
14.
Pediatr Surg Int. 2019 Jun;35(6):657-663. doi: 10.1007/s00383-019-04475-4. Epub 2019 Apr 1.

Plasma citrulline is not a biomarker for intestinal adaptation in short bowel syndrome, studied in piglets: a model for human neonates.

Author information

1
Department of Pediatrics, Edmonton Health Academy, University of Alberta, 11405-87 Ave, Edmonton, AB, T6G 1C9, Canada.
2
Department of Pediatrics, Edmonton Health Academy, University of Alberta, 11405-87 Ave, Edmonton, AB, T6G 1C9, Canada. Justine.turner@albertahealthservices.ca.
3
Faculty of Medical Sciences, University of Toronto, Toronto, ON, Canada.
4
Department of Agricultural, Life and Environmental Sciences, University of Alberta, Edmonton, AB, Canada.
5
Department of Surgery, University of Alberta, Edmonton, AB, Canada.
6
Department of Pediatric Surgery, Kagoshima University, Sakuragaoka, Kagoshima, Japan.
7
Laboratory Medicine and Pathology, University of Alberta, Edmonton, AB, Canada.
8
Departments of Pediatrics and Nutritional Sciences, University of Toronto, Toronto, ON, Canada.
9
Division of General and Thoracic Surgery, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Abstract

BACKGROUND:

There are no in vivo methods to measure adaptation in neonatal short bowel syndrome (SBS). We evaluated citrulline (Cit) levels in neonatal piglet surgical models of SBS.

METHODS:

Piglets underwent 75% mid-intestinal resection with jejunoileal anastomosis (JI), 75% distal resection of ileum with jejunocolic anastomosis (JC) or sham surgery. Jugular and gastric catheters were inserted for parenteral and enteral nutrition. On D7, small intestine length and weight were measured, jejunum collected for histopathology and Cit level determined.

RESULTS:

JI (n = 5) compared to JC (n = 5) had increased small intestinal length (JC - 17.5 cm; JI +22.0 cm; p = 0.02) and mass (JC 43.1 mg/cm/kg; JI 51.3 mg/cm/kg; p = 0.02), while Cit did not differ (JI 801.0 µM; JC 677.7 µM; p = 0.90). Including non-resected shams (n = 4), Cit correlated with length (R2 = 0.48; p = 0.006), but not for SBS alone (R2 = 0.11; p = 0.4), mass (R2 = 0.05; p = 0.5). A second experiment compared change in Cit levels from baseline to D7. Levels declined in sham (n = 8) and JC (n = 10) (sham - 110.1 µM; JC - 56.6 µM; p = 0.17), regardless of intestinal lengthening (sham 29.9 cm; JC - 10.4 cm; p = 0.002).

CONCLUSION:

Citrulline levels predict large differences in intestinal length and 'identify' SBS. However, citrulline cannot discriminate between adaptation in JI and JC, nor predict intestinal lengthening.

KEYWORDS:

Citrulline; Intestinal adaptation; Neonates; Short bowel syndrome

PMID:
30937511
DOI:
10.1007/s00383-019-04475-4
[Indexed for MEDLINE]
Icon for Springer
15.
Nat Commun. 2019 Apr 1;10(1):1477. doi: 10.1038/s41467-019-09458-6.

Identification of human D lactate dehydrogenase deficiency.

Author information

1
Department of Genetics, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands.
2
Center for Molecular Medicine, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands.
3
Hubrecht Institute-KNAW and University Medical Center Utrecht, Utrecht, 3584, CT, The Netherlands.
4
Bartiméus, Institute for the Visually Impaired, Doorn, 3940, AB, The Netherlands.
5
Department of Metabolic Diseases, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, 3584, EA, The Netherlands.
6
Department of Pediatric Gastroenterology and Nutrition, Academic Medical Center, Amsterdam, 1105, AZ, The Netherlands.
7
Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands.
8
Laboratory of Clinical Chemistry, Deventer Hospital, Deventer, 7416, SE, The Netherlands.
9
Laboratory Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, 1105, AZ, The Netherlands.
10
Section of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, Groningen, 9713, GZ, The Netherlands.
11
Oasi Research Institute-IRCCS, Troina, 94018, Italy.
12
Department of Child Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands.
13
Department of Medical Physiology, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands.
14
Department of Genetics, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands. G.vanHaaften@umcutrecht.nl.
15
Center for Molecular Medicine, University Medical Center Utrecht, Utrecht, 3584, CX, The Netherlands. G.vanHaaften@umcutrecht.nl.

Abstract

Phenotypic and biochemical categorization of humans with detrimental variants can provide valuable information on gene function. We illustrate this with the identification of two different homozygous variants resulting in enzymatic loss-of-function in LDHD, encoding lactate dehydrogenase D, in two unrelated patients with elevated D-lactate urinary excretion and plasma concentrations. We establish the role of LDHD by demonstrating that LDHD loss-of-function in zebrafish results in increased concentrations of D-lactate. D-lactate levels are rescued by wildtype LDHD but not by patients' variant LDHD, confirming these variants' loss-of-function effect. This work provides the first in vivo evidence that LDHD is responsible for human D-lactate metabolism. This broadens the differential diagnosis of D-lactic acidosis, an increasingly recognized complication of short bowel syndrome with unpredictable onset and severity. With the expanding incidence of intestinal resection for disease or obesity, the elucidation of this metabolic pathway may have relevance for those patients with D-lactic acidosis.

PMID:
30931947
PMCID:
PMC6443703
DOI:
10.1038/s41467-019-09458-6
[Indexed for MEDLINE]
Free PMC Article
Icon for Nature Publishing Group Icon for PubMed Central
16.
J Pediatr Surg. 2019 Jun;54(6):1179-1183. doi: 10.1016/j.jpedsurg.2019.02.039. Epub 2019 Mar 1.

Ultrasound Elastography as a Non-Invasive Method to Monitor Liver Disease in Children with Short Bowel Syndrome: Updated Results.

Author information

1
Department of Pediatric Surgery and the Research Institute, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH.
2
Division of Gastroenterology, Hepatology, and Nutrition, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH.
3
Department of Surgery, The Ohio State University College of Medicine, Columbus, OH.
4
Department of Surgery, New York Medical College, Valhalla, NY.
5
Department of Pathology, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH.
6
Department of Radiology, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH.
7
Department of Pediatric Surgery and the Research Institute, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, OH. Electronic address: Peter.Minneci@nationwidechildrens.org.

Abstract

PURPOSE:

The purpose of the study was to determine the accuracy of acoustic radiation force impulse (ARFI) ultrasound elastography in assessing the degree of liver disease in children with short bowel syndrome (SBS).

METHODS:

A prospective observational cohort study of patients with SBS who underwent a liver biopsy and ARFI elastography was performed. Mean shear wave speed (SWS) and stage of fibrosis was evaluated using t-tests. Receiver operating characteristic curves (ROC) were generated and the area under the curves (AUC) estimated in order to assess the accuracy of SWS measurements to discriminate between stages of fibrosis.

RESULTS:

Thirty-seven paired elastography and biopsy samples from 31 patients were included. The median age was 0.6 years, and 61% were male. There was a significant positive correlation between stage of fibrosis and mean SWS (β=0.16 m/s increase per stage, p=<0.001). ROC analysis revealed that mean SWS had good accuracy for discriminating between mild liver fibrosis (F0-F1) and moderate to severe fibrosis (F2-F4) (AUC=0.80, 95% CI 0.65-0.95). In addition, ROC analysis demonstrated that mean SWS can also accurately discriminate between mild to moderate fibrosis (F0-F2) and more severe fibrosis (F3-F4) (AUC=0.84, 95% CI 0.71-0.96).

CONCLUSION:

ARFI elastography is an accurate, non-invasive method to monitor liver disease in children with SBS.

TYPE OF STUDY:

Retrospective Cohort Study LEVEL OF EVIDENCE: II.

KEYWORDS:

Elastography; Intestinal failure associated liver disease; Liver disease; Liver fibrosis; Parenteral nutrition; Short bowel syndrome

PMID:
30885560
DOI:
10.1016/j.jpedsurg.2019.02.039
[Indexed for MEDLINE]
Icon for Elsevier Science
17.
J Pediatr Surg. 2019 Jun;54(6):1239-1244. doi: 10.1016/j.jpedsurg.2019.02.026. Epub 2019 Feb 28.

Lymphatic network remodeling after small bowel resection.

Author information

1
Division of Pediatric Surgery, Department of Surgery, St. Louis Children's Hospital, Washington University in St. Louis School of Medicine, St. Louis, MO.
2
Department of Pathology and Immunology, Washington University in St. Louis, MO.
3
Division of Pediatric Surgery, Department of Surgery, St. Louis Children's Hospital, Washington University in St. Louis School of Medicine, St. Louis, MO. Electronic address: brad.warner@wustl.edu.

Abstract

BACKGROUND:

Short gut syndrome (SGS) following massive small bowel resection (SBR) is a major cause of pediatric mortality and morbidity secondary to nutritional deficiencies and the sequelae of chronic total parenteral nutrition use, including liver steatosis. Despite the importance of lymphatic vasculature in fat absorption, lymphatic response after SBR has not been studied. We hypothesize that lymphatic vessel integrity is compromised in SGS, potentially contributing to the development of impaired lipid transport leading to liver steatosis and metabolic disease.

METHODS:

Mice underwent 50% proximal SBR or sham operations. Imaging of lymphatic vasculature in the lamina propria and mesentery was compared between sham and SBR Prox1 ERCre-Rosa26LSLTdTomato mice. mRNA expression levels of lymphangiogenic markers were performed in C57BL/6J mice.

RESULTS:

Lymphatic vasculature was significantly altered after SBR. Mesenteric lymphatic collecting vessels developed new branching structures and lacked normal valves at branch points, while total mucosal lymphatic capillary area in the distal ileum decreased compared to both sham and intraoperative controls. Intestinal Vegfr3 expression also increased significantly in resected mice.

CONCLUSIONS:

Intestinal lymphatics, in both the lamina propria and mesentery, dramatically remodel following SBR. This remodeling may affect lymphatic flow and function, potentially contributing to morbidities and nutritional deficiencies associated with SGS.

KEYWORDS:

Adaptation; Intestinal failure; Lymphatics; Remodeling; Short gut syndrome; Small bowel resection

PMID:
30879758
PMCID:
PMC6545263
[Available on 2020-06-01]
DOI:
10.1016/j.jpedsurg.2019.02.026
[Indexed for MEDLINE]
Icon for Elsevier Science
18.
Pediatr Surg Int. 2019 Jun;35(6):649-655. doi: 10.1007/s00383-019-04468-3. Epub 2019 Mar 13.

Autologous intestinal reconstruction: a single institution study of the serial transverse enteroplasty (STEP) and the longitudinal intestinal lengthening and tailoring (LILT).

Author information

1
Department of General and Thoracic Surgery, Children's National Health System, Washington, DC, USA.
2
Department of Surgery, Howard University Hospital and College of Medicine, Washington, DC, USA.
3
Department of General and Thoracic Surgery, Children's National Health System, Washington, DC, USA. MPetrosy@childrensnational.org.
4
Department of Gastroenterology, Hepatology, and Nutrition, Children's National Health System, Washington, DC, USA.

Abstract

PURPOSE:

To review the effectiveness of the longitudinal intestinal lengthening and tailoring (LILT) and serial transverse enteroplasty (STEP) operations in a cohort of patients with short bowel syndrome (SBS).

METHODS:

We conducted a retrospective analysis of children with SBS treated at our institution from 2004 until 2014. Children aged 0 days to 18 years with SBS who underwent autologous intestinal reconstruction were included in the study.

RESULTS:

Twenty-two SBS patients underwent 31 different lengthening procedures (LP). Seventeen patients underwent their primary lengthening procedures at our institution: 9 (53%) patients underwent a LILT, 7 (41%) underwent a STEP and 1 (6%) had a simultaneous LILT and STEP procedure. 12/22 patients had a second STEP, two had a third STEP and one patient had an intestinal transplantation after the LP. Median intestinal length at the time of surgery was 25 cm (range 12-90 cm). There was no difference in gain of intestinal length after LILT vs. STEP (p = 0.74). Length of stay and initiation of feeds were similar. Serum albumin increased after autologous bowel lengthening (p < 0.001). 50% were weaned off parenteral nutrition (PN) (5/9 of the LILT, 1/7 of the STEP, 1/1 of the combined LILT/STEP). There were no surgical complications or deaths.

CONCLUSION:

In patients with SBS, LILT and STEP procedures are effective for autologous intestinal reconstruction and enable intestinal rehabilitation.

KEYWORDS:

Autologous intestinal reconstruction; Intestinal rehabilitation; Short bowel syndrome

PMID:
30868210
DOI:
10.1007/s00383-019-04468-3
[Indexed for MEDLINE]
Icon for Springer
19.
Pediatr Surg Int. 2019 May;35(5):547-550. doi: 10.1007/s00383-019-04462-9. Epub 2019 Mar 7.

Nationwide survey of outcome in patients with extensive aganglionosis in Japan.

Author information

1
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Fukuoka, 812-8582, Japan.
2
Department of Pediatric Surgery, Field of Developmental Medicine, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima, 890- 8520, Japan.
3
Department of Pediatric Surgery, Hiroshima City Hiroshima Citizen Hospital, 7-33, Moto-machi, Naka-ku, Hiroshima, 730- 8518, Japan.
4
Department of Pediatric Surgery, Shizuoka Children's Hospital, 860, Urushiyama, Aoi-ku, Shizuoka, 420-0953, Japan.
5
Department of Pediatric Surgery, Hamamatsu University Hospital, 1-20-1, Handayama, Higashi-ku, Hamamatsu, Shizuoka, 431-3192, Japan.
6
Department of Pediatric Surgery, Niigata University Graduate School of Medicine and Dental Sciences, 1-757, Asahimachi- Dori, Chuo-ku, Niigata, 951-8510, Japan.
7
Department of Pediatric Surgery, Kanazawa Medical University, 1-1, Daigaku, Uchinada-machi, Kahoku-gun, Ishikawa, 920-0293, Japan.
8
Department of Pediatric Surgery, Kyorin University School of Medicine, 6-20-2, Shinkawa, Mitaka, Tokyo, 181-0004, Japan.
9
Department of Gastroenterological Surgery I, Hokkaido University Graduate School of Medicine, 5, Kita-14jo-nishi, Kita-ku, Sapporo, Hokkaido, 060-8648, Japan.
10
Department of Pediatric Surgery, Tohoku University School of Medicine, 1-1, Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980- 0872, Japan.
11
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Faculty of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, Fukuoka, 812-8582, Japan. taguchi@pedsurg.med.kyushu.

Abstract

PURPOSE:

Hirschsprung's disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz's band. The condition is reported be associated with a high mortality rate of 50-80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan.

METHODS:

Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan.

RESULTS:

A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel's procedure in one). Ziegler's myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes.

CONCLUSION:

HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.

KEYWORDS:

Hirschsprung’s disease; Mortality; Nationwide survey; Short bowel syndrome

PMID:
30847604
DOI:
10.1007/s00383-019-04462-9
[Indexed for MEDLINE]
Icon for Springer
20.
J Pediatr Surg. 2019 May;54(5):964-967. doi: 10.1016/j.jpedsurg.2019.01.025. Epub 2019 Feb 2.

Long-term outcomes of ultrashort bowel syndrome due to malrotation with midgut volvulus managed at an interdisciplinary pediatric intestinal rehabilitation center.

Author information

1
Center for Advanced Intestinal Rehabilitation, Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
2
Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
3
Center for Advanced Intestinal Rehabilitation, Center for Nutrition, Division of Gastroenterology, Hepatology, and Nutrition; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
4
Center for Advanced Intestinal Rehabilitation, Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. Electronic address: tom.jaksic@childrens.harvard.edu.

Abstract

PURPOSE:

The purpose of this study was to describe long-term outcomes of pediatric-onset ultrashort bowel syndrome owing to midgut volvulus managed at an interdisciplinary intestinal rehabilitation center.

METHODS:

Patients with a history of malrotation and pediatric-onset midgut volvulus causing extensive bowel loss (<20% residual small bowel length expected for postconception age) and treated between 2010 and 2017 were reviewed. Data are expressed as median (IQR).

RESULTS:

Twenty-three patients had midgut volvulus at age 1 (0-21) day leading to 9 (8-12) percent predicted residual bowel length. Eight (35%) had gastroschisis. Follow-up was 8.5 (6.6-12.2) years from volvulus. Five (22%) patients underwent intestinal/multivisceral transplantation, and all achieved enteral autonomy. Eighteen (78%) patients remained transplant-free, 7 of whom achieved enteral autonomy after 718 (682-1030) days of parenteral nutrition. Transplant-free enteral autonomy was achieved by 0/6 patients with gastroschisis, compared to 7/12 without gastroschisis (p = 0.04). For the overall group, 18 (78%) patients had small bowel bacterial overgrowth, and 7 manifested symptomatic D-lactic acidosis. We observed 2 mortalities, one awaiting transplant and one 4 years following transplantation.

CONCLUSION:

Midgut volvulus owing to malrotation with extensive bowel loss is associated with favorable long-term survival. Transplant-free enteral autonomy may be feasible, particularly in the absence of gastroschisis.

TYPE OF STUDY:

Prognosis study.

LEVEL OF EVIDENCE:

IIb, retrospective cohort study.

KEYWORDS:

Intestinal failure; Intestinal rehabilitation; Intestinal transplantation; Midgut volvulus; Parenteral nutrition; Ultrashort bowel syndrome

PMID:
30826119
DOI:
10.1016/j.jpedsurg.2019.01.025
[Indexed for MEDLINE]
Icon for Elsevier Science

Supplemental Content

Loading ...
Support Center