AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Background

Few conditions in pediatric gastroenterology pose as great a challenge as short bowel syndrome (SBS). SBS is the result of the alteration of intestinal digestion and absorption that occurs following extensive bowel resection. It represents a complex disorder that affects normal intestinal physiology with nutritional, metabolic, and infectious consequences.

Pathophysiology

The small intestine of the neonate is approximately 250 cm in length. By adulthood, the small intestine grows to approximately 750 cm. As a consequence, the infant and the young child have a favorable long-term prognosis compared to an adult in regards to potential intestinal growth after intestinal resection. Intestinal adaptation may take weeks to months to be achieved, and, in the interim, children who have had intestinal resection need nutritional support through a variety of therapeutic measures, including parenteral nutrition. The duodenum and jejunum are responsible for the absorption of most dietary constituents except vitamin B-12 and bile acids.

The jejunum is characterized by long villi and a large absorptive area. The tight junctions are relatively large, rendering the epithelium more porous to larger molecules and the free and rapid flux of water and electrolytes. In comparison, the ileum has shorter villi and a less absorptive surface area than the jejunum. Furthermore, the tight junctions are tighter, permitting less flux of water and electrolytes from the vascular space into the intestinal lumen, and, consequently, the ileum is more efficient in the absorption of water. Although nutrients are less well absorbed in the ileum, it has site-specific receptors for the absorption of bile acids and vitamin B12. Moreover, many gastrointestinal hormones that affect intestinal motility, including enteroglucagon and peptide YY, are produced in the ileum. The small intestinal sites of nutrient absorption are as follows:

The small intestinal sites of nutrient absorption are as follows:

• Duodenum - Iron
• Jejunum - Carbohydrates, proteins, fat, vitamins
• Ileum - Bile acids, vitamin B-12

In general, virtually all digestion and absorption is completed within the first 100-150 cm of jejunum in a healthy individual. In the absence of an intact colon, the minimum length of healthy bowel necessary to avoid parenteral nutrition is approximately 100 cm. Patients who have less than 100 cm of jejunum exhibit significant malabsorption. Although the ileum is limited in its capacity to form chylomicrons compared to the jejunum, studies have shown that the ileum has greater adaptive function as far as improving its absorptive function in the presence of SBS. Similarly, studies in animals have shown that intestinal transit time is more likely to improve (ie, increase) in patients with proximal small-bowel resection as opposed to patients with distal small-bowel resection.

The jejunum cannot develop site-specific carriers for the transportation of vitamin B12 and bile salts, and, consequently, these are malabsorbed permanently in patients, following ileal resection. Furthermore, the loss of enteroglucagon and peptide YY cannot be underscored in the regulation of small bowel motility.

Mortality/Morbidity

The leading causes of death in infants with SBS who are being treated with parenteral nutrition are central line sepsis and liver failure with the prolonged use of parenteral nutrition.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

The history of a patient with SBS is typically of a child who was born with a congenital anomaly, such as an omphalocele, gastroschisis, or intestinal atresia, and who required a small-bowel resection. Other patients present with a past medical history of intestinal ischemia from malrotation and volvulus that required intestinal resection.

Children with SBS may present with a variety medical issues depending upon the extent of their bowel resection and the level of medical complexity. The history should consider all the potential clinical ramifications of managing cases of SBS, including the following:

• Parenteral nutrition
• • The degree of home nutrition support necessary in the management of a child on total parenteral nutrition (TPN) is noteworthy.
  • Patients may present with issues entirely separate from the medical problems related to SBS, including problems associated with intravenous access, infection, and signs and clinical symptoms associated with TPN-related liver disease.
• Enteric nutrition
• • Quickly initiate enteric feeding for all children with SBS.
  • Once again, patients may present with a history entirely separate from the medical problems related to SBS, including gastrostomy or nasogastric tube issues. For example, gastrostomy tubes may accidentally fall out. In these patients, the immediate replacement of these tubes is important in maintaining the patency of the tube entry site.
  • Although complications are uncommon, be aware of potential gastric ulceration, gastrostomy tube migrations, and intestinal obstruction, which all may be associated with bilious vomiting and the risk for pancreatitis.
• Nutrition
• • Closely monitor all children on long-term enteral and parenteral nutrition for specific nutritional issues.
  • Patients may present with a variety of symptoms related to specific nutritional deficiency, including specific vitamin (or mineral) deficiencies and the related signs, symptoms, and electrolyte abnormalities and their potential complications.
• Medical and surgical histories: Obtain a detailed account of the patient's past medical and surgical histories.
• • The pathology leading up to the surgical resection
  • The extent and location of bowel resection, the presence or absence of the ileocecal valve
  • Medical complications
  • TPN dependency
  • Enteral nutrition
  • Enteral access
  • Type of nutritional formula used
  • Nutritional supplements
  • Medications
  • Allergies
• History of complications associated with SBS
• • Malabsorptive diarrhea
  • Dehydration
  • Vomiting
  • Bloating
  • Gastroesophageal reflux
  • Failure to thrive
  • Drug toxicities

Physical

On physical examination, pay close attention to these clinical signs.

• Vitals
• State of hydration
• State of nutrition, as measured by a patient's weight for height and anthropometric measurements
• Signs of sepsis
• Form of nutritional therapy used in the patient, eg, central line access or enteral access
• Specific clinical signs of nutritional deficiency
• Signs of liver disease

Causes

• Necrotizing enterocolitis and midgut volvulus are the most common causes of SBS in the neonatal period.
• Intussusception with ischemic small-intestinal injury is a common cause of SBS in older infants and children.
• Through innovations in the surgical management of patients with chronic inflammatory bowel disease, Crohn disease is a less frequently associated cause of SBS.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

Fungal infections

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Perform standard hematologic and biochemical studies in all children with SBS. Each institution follows its own specific guideline. The following list is not intended to represent an exhaustive list of laboratory evaluations.
• • Electrolytes, BUN, creatinine, calcium, magnesium, phosphorous - Biweekly in both the initial phase and the late period or at the time of presentation for instability
  • Comprehensive panel CBC, triglycerides, cholesterol - Weekly in both the initial phase and the late period or at the time of presentation for instability
  • Folate, vitamin B-12, vitamin E, copper, zinc, selenium - Monthly in both the initial phase and the late period or at the time of presentation for instability
  • Carnitine
• Microbiology
• • Any suspicion of sepsis necessitates blood cultures. Children with SBS are susceptible to intestinal bacterial translocation, with the central line as the most likely source of seeding. They are also susceptible to translocation of skin flora such as Staphylococcus species.
  • Obtain blood cultures from both the central and peripheral sites.
  • Consider opportunistic infections, including fungal infections, in the differential diagnosis of a child presenting with sepsis. Obtain a urinalysis and blood culture in these children specifically to search for fungal infection.

Imaging Studies

• Imaging studies are needed to assess for potential complications, including the following:
• • Infection
    • Abdominal ultrasonography to search for fungal balls in the kidney
    • CT scanning of the abdomen to identify sepsis
    • Ultrasonography of Broviac tip
  • Bowel obstruction
    • Plain radiography of the abdomen
    • Barium imaging of the bowel
  • Liver disease
    • Abdominal ultrasonography to study the liver, biliary tract, and spleen, as well as assess for the presence of ascites
    • Abdominal ultrasonography with Doppler to assess portal flow
  • Potential liver or bowel transplant
    • Volumetric CT scanning of the liver
    • Possible angiography

Procedures

• Children with SBS may require laboratory procedures, including the following:
• • Upper endoscopy to assess for peptic ulcer disease and possible signs of liver disease (eg, esophageal varices, hypertensive gastropathy)
  • Liver biopsy to evaluate the patient for signs of TPN-related liver disease

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical Care

The management of SBS requires an aggressive multidisciplinary approach that is most often tailored to the individual needs of the patient. Nutrition plays an important role in the management of SBS. The institution of early and aggressive enteral therapy is the most important stimulus for intestinal adaptation and the eventual discontinuation of parenteral therapy.

Parenteral nutrition

The length and function of the remaining intestine, as well as the presence of normal physiologic mechanisms that regulate intestinal transit time, including the ileocecal valve and colon, determine whether the patient requires a limited course of specialized enteral therapy or prolonged TPN.

In 1991, Goulet and coworkers studied the relative importance of several clinical factors in predicting the long-term needs for parenteral nutrition. In 54 neonates who underwent extensive small-bowel resection, the presence of less than 40 cm of small intestine in children with either colonic resection or an absent ileocecal valve had a strong association with a prolonged need for parenteral nutrition (>48 mo).

In addition, the health of the remaining bowel, its absorptive capacity, and its ability to adapt are important. For example, the motility of the existing small bowel may have been affected by the patient's primary illness leading to SBS. Indeed, patients who experience severe intestinal ischemia may be left with small-bowel dysmotility, rendering patients susceptible to bacterial overgrowth. Another example is in patients with Crohn disease who have undergone repeated small-bowel resections for fibrostenotic disease. Postsurgery, these patients may have repeated exacerbations of their disease that may injure the existing small bowel, thereby affecting its absorptive capacity.

Excessive fluid losses

Massive fluid and electrolyte losses are usually observed during the first week after excessive intestinal resection. Patients with SBS most often require aggressive resuscitation with fluids or parenteral nutrition, or both. Instituting enteral therapy as soon as possible is very important in order to facilitate the adaptive intestinal response.

In the early postoperative period, monitor serum electrolytes and a comprehensive biochemical pattern daily. When these values have stabilized, monitor them on a biweekly or triweekly basis. The hypersecretion noted within the first 12 months postresection is usually treated with histamine 2 (H2)–receptor antagonists or proton pump inhibitors.

The provision of adequate parenteral fluid replacement needs may be ongoing depending on the amount of stool or ostomy output. Indeed, enteral nutrition can cause significant osmotic diarrhea.

Malabsorption

Extensive jejunal resection leads to carbohydrate malabsorption. The undigested foods produce an osmotic diarrhea typical of most patients with SBS. The proximal small bowel also is important in the absorption of proteins, fat, and certain micronutrients, including copper.

Extensive resection of the ileum may lead to severe malabsorption of bile salt and vitamin B-12. Bile salt malabsorption produces a choleretic diarrhea. Furthermore, bile salt depletion affects fat absorption, thereby worsening steatorrhea and fat-soluble vitamin malabsorption. Ileal resection leads to the malabsorption of bile salts and an abnormal bile acid pool that leads to the formation of a lithogenic bile and cholelithiasis.

The ileocecal valve is important in preventing bacterial overgrowth. Problems associated with proximal small-bowel overgrowth include deconjugation of bile salts and depletion of bile salt stores. Bacteria often compete for vitamin B-12, which may facilitate a pernicious anemia. Bacteria overgrowth also leads to carbohydrate malabsorption, worsening of osmotic diarrhea, and the risk of metabolic acidosis and dehydration. Treatment is generally aimed at lessening the degree of bacterial overgrowth with antibiotic therapy, including administration of metronidazole alternating with either kanamycin or oral gentamicin.

Motility disturbances

Patients with SBS have a decrease in intestinal transit time. Patients with extensive proximal small-bowel resection have increased gastric emptying, thereby further decreasing intestinal transit time.

The absence of normal physiologic mechanisms that increase intestinal transit, including the ileocecal valve and colon, also shortens intestinal transit time. However, if the existing small bowel or colon shows signs of dysmotility due to fibrosis or surgical narrowing, stagnant bowel contents may aggravate an existing bacterial overgrowth, thereby worsening malabsorption and diarrhea.

Small-bowel overgrowth also leads to d-lactic acidosis and may be associated with CNS disturbances.

SBS associated colitis is not an infrequent complication. Patients often present with hematochezia and have histologic signs of colitis on intestinal biopsies.

Gastric acid hypersecretion

Gastric acid hypersecretion is common in patients with SBS. The degree of hypersecretion is proportional to the degree of bowel resected. Hypersecretion may contribute to malabsorption by inactivating pancreatic enzymes and, thus, interfering with fat absorption. The usual treatment is with either H2 blockers or proton pump inhibitors.

Medical therapies

Codeine and loperamide can be used in pediatric patients to slow intestinal transit time; however, results have been mixed because of concerns for worsening of bacterial overgrowth. Octreotide is rarely used to limit the amount of intestinal losses after bowel resection. Its use in pediatric patients is controversial because of concerns of the effect on growth and worsening cholestatic liver disease. Cholestyramine has been used as a means of binding bile salts in patients with choleretic diarrhea. Antibiotics are used sparingly to prevent small-bowel overgrowth.

Surgical Care

• Surgical care is related to venous access (ie, central line placement to provide TPN). The loss of intravenous access through repeated episodes of sepsis and thrombosis can lead to the early need for intestinal transplantation despite good hepatic function.
• Surgery may be required for gastrostomy tube placement to provide for enteral access.
• Intestinal lengthening procedures and transplantation are always avoided if at all possible. Several attempts at increasing bowel length through surgical means have been made over the last decade. The bowel is transected longitudinally to preserve the blood supply. The largest experience comes from the University of Nebraska. In this experience, surgical intervention improved intestinal adaptability and weaning from parenteral nutrition in a dozen patients. However, with repeated surgical interventions, the risk of intestinal stricture formation increases, as well as the risk of small-bowel obstruction secondary to adhesion formation.
• Small-bowel transplantation has shown mixed success. The problems associated with transplantation, including the need for immunosuppression and the risk for intestinal rejection and lymphoproliferative disease, has limited this treatment option for most patients with SBS unless absolutely necessary in patients with associated severe advanced liver disease and those with major vascular access problems.
• Isolated orthotopic liver transplantation without small-bowel transplantation has been demonstrated to be effective.

Consultations

• If concern for sepsis exists with no identifiable source, the aid of an infectious disease specialist may be requested.
• In the setting of fungemia, consulting an infectious disease specialist to guide antifungal therapy is best.
• In the setting of liver or intestinal failure, consult a transplant surgeon.
• Consult a gastroenterologist.

Diet

Enteral therapy

In infants with massive small-bowel resection, enteral nutrition is initiated very quickly by using elemental formulas. The mixture of monosaccharides and polysaccharides is preferred to disaccharides in order to limit osmotic load, in combination with long-chain triglycerides (LCT) and medium-chain triglycerides (MCT). The authors favor starting with formulas that are either one-fourth or one-half strength, depending upon the patient's tolerability, and increasing in volume before increasing energy density. Oligopeptide formulas are better absorbed than elemental amino acid formulas because di-tripeptide absorption exceeds that of amino acids.

MCTs are important in the dietary management of patients with SBS because they are readily absorbed in the stomach and proximal small bowel, thereby improving fat and total energy absorption. Current recommendations are to use MCTs as the main source of fat and energy needs. Long-chain fatty acids are required to prevent essential fatty acid deficiency and should make up approximately 10% of the patient's energy needs. Long-chain fatty acids may have a trophic effect on the intestinal mucosa.

The question of fat intolerance has always been a point of contention; however, the use of long-chain fats, which have increased energy density, is usually better tolerated than use of carbohydrates. Testing the tolerability of either fats or carbohydrates and adjusting the modular formula accordingly is advisable. In some cases, providing carbohydrates parenterally and providing fats enterally is preferred in order to improve a patient's tolerance. The use of continuous enteral feeds is better tolerated than bolus feeds in patients with small-bowel resection. An increase in stool output with the appearance of fecal-reducing substances is an indication that the patient may have reached the tolerance limit.

Enteral nutrition remains the lone medical therapy that can facilitate intestinal adaptation. The residual bowel must be constantly exposed to nutrients in order to allow the bowel to adapt. Hence, the physician must be able to allow for substantial stool volume and frequency, as long as it does not compromise the child's hydration, acid base balance, and serum electrolyte levels. A common mistake is the tendency to either stop enteral feeds or substantially lower the volume and frequency of feeds in response to changes in stool volume. Most fluid and electrolyte perturbations that result from short bowel syndrome, or in response to modifications in enteral nutritional therapy, can be easily compensated through an adjustment in the parenteral formula. If possible, the physician should avoid altering the rate or the concentration of the enteral formula too aggressively, in order to allow the adaptive process to proceed.

Many animal models have shown that the bowel is very sensitive to starvation. In the absence of enteral nutrition, the crypt cell population decreases and epithelial cell cycle increases, thereby decreasing the proliferation of the intestinal epithelium. In contrast, in response to a continual and large supply of enteral nutrients, crypt cells proliferate, leading to an increase in crypt depth and lengthening of the intestinal villi. An increase in the absorptive area does not always coincide with functional adaptation.

The production of digestive enzymes and nutrient receptors is in direct response to the quality and quantity of intestinal nutrients. The physician must ensure a constant provision of macronutrients, in order to facilitate this adaptive process. The adaptive process may, in large part, also depend on the production of trophic intestinal hormone and secretions that are produced in response to nutritional therapy.

In a healthy individual, other than fluid and calcium absorption, the colon has a limited absorptive capacity; however, in patients with SBS, the colon may assume an increased nutritional role. The colonic flora is capable of metabolizing nonabsorbed starch and fiber into the production of short-chain fatty acids. These short-chain fatty acids are regarded as the preferred fuel for the colon and may actually stimulate water absorption. Therefore, the residual colon may provide an opportunity to improve water absorption in patients with SBS.

Decreasing the amount of carbohydrates within the enteral feeds and decreasing the volume and concentration of feeds help manage the problems with excessive stool volume and abdominal distension in the setting of significant malabsorption. The addition of fiber can also increase stool frequency. Decreasing either the volume or rate of feeds may treat patients with gastroesophageal reflux and vomiting. The advancement of enteral feeds is based on the patient's tolerance.

Parenteral therapy

Provide parenteral nutrition to patients with massive intestinal resections as soon as possible. Increase parenteral nutrition accordingly, based on the patient's tolerance level. Before the availability of parenteral nutrition, most patients with SBS died. The dramatic improvement in patient survival primarily is because of advances in parenteral nutrition. Today, survival has been shown in patients with as little as 11 cm of proximal small bowel and an ileocecal valve to as little as 25 cm of small bowel without an ileocecal valve. Anecdotal reports of children surviving with as little as 12 cm of bowel without an ileocecal valve also exist. The clinical factors that are associated with prolonged (>2 y) parenteral nutritional requirements include the following:

• Residual bowel
• Limited absorptive function
• Bowel adaptation (ileum has greater adaptability than jejunum)
• Dysmotility
• Bowel length (<40 cm)
• Absent ileocecal valve
• Colon resection
• Bacterial overgrowth

Several strategies have been proven to improve a patient's tolerance of parenteral nutrition in the setting of SBS, including limiting the amount of toxic amino acids administered parenterally and providing protein requirements enterally with specialized infant amino acid formulas. Similarly, because enteral feeds are known to facilitate bile flow, the initiation and progression of enteral feeds may actually prevent cholestasis. Choleretic agents, such as phenobarbital and ursodeoxycholic acid, have also been shown to help treat cholestatic liver disease. Patients on long-term parenteral nutrition are at risk for central intravenous catheter infection and sepsis. Patients require aggressive home care nursing and the outpatient execution of investigations, including hematologic, biochemical, and microbiologic testing.

Moreover, these patients are at risk for intestinal bacterial translocation. Approximately 20% of all central venous catheters are removed secondary to recurrent infection. In the author's experience at the Johns Hopkins Hospital, approximately 90% of central venous catheter infections can be cleared with antibiotics alone. The role of prophylactic antibiotic therapy is controversial.

Specific nutrient requirements

Multivitamins and minerals are preferentially administered parenterally in patients with extensive small-bowel resections. In the presence of significant steatorrhea, water-soluble forms of vitamin A, vitamin E, and vitamin D are available commercially. Because calcium supplementation is important in the setting of vitamin D deficiency and malabsorption, provide supplementation enterally in order to allow for bone mineralization and growth. Regularly monitor serum calcium. Dual energy x-ray absorptiometry (DEXA) scanning may be used to monitor bone density. Because enteric bacteria synthesize vitamin K, supplementation is not necessary but can be monitored with the measurement of prothrombin time. The deficiency of water-soluble vitamins is rare.

Vitamin B-12 can be administered parenterally on a monthly basis as needed in patients with extensive ileal resections. It is also available as a nasal gel. Patients with iron deficiency secondary to either bacterial overgrowth or malabsorption should be monitored carefully and can be supplemented with IV iron infusions. Zinc supplements are often needed secondary to increased fecal losses. In individuals who are not on parenteral nutrition, zinc supplements can be provided in tablet form. Other micronutrients, including manganese and selenium, can be provided in pharmacologic doses as required. Although copper deficiency is rare, deficiency has been associated with anemia and cardiomyopathy. Periodic measurements of copper and selenium are merited for individuals on long-term parenteral nutrition.

Outcome

The successful nutritional management of patients with SBS has increased long-term survival rates. The complex pathophysiology of SBS often requires a multidisciplinary approach to patient management. Additional experience with adjunct medical and surgical therapies will potentially expand existing treatment options, thereby improving patient survival and precluding potential complications associated with long-term parenteral nutrition support.

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Drug Category: Antibiotics

These agents are used sparingly to prevent small-bowel bacterial overgrowth. They are used on a biweekly basis to prevent bacterial resistance.

Drug Name	Gentamicin (Garamycin, Gentacidin)
Description	Aminoglycoside antibiotic for gram-negative coverage. May be used to prevent bacterial overgrowth in children with SBS. Consider if penicillins or other less toxic drugs are contraindicated. Gentamicin works well when administered PO to prevent intestinal overgrowth. Drug interactions and precautions are likely to be clinically insignificant because PO gentamicin has minimal systemic absorption.
Adult Dose	6-7.5 mg/kg/d PO divided q8h; not to exceed 300 mg/d
Pediatric Dose	2.5 mg/kg/dose PO tid; not to exceed 300 mg/d
Contraindications	Documented hypersensitivity
Interactions	Coadministration with other aminoglycosides, cephalosporins, penicillins, and amphotericin B may increase nephrotoxicity; aminoglycosides enhance effects of neuromuscular blocking agents, thus prolonged respiratory depression may occur; coadministration with loop diuretics may increase auditory toxicity of aminoglycosides; possible irreversible hearing loss of varying degrees may occur
Pregnancy	C - Safety for use during pregnancy has not been established.
Precautions	Narrow therapeutic index (not intended for long-term therapy); caution in renal failure (patient not on dialysis), myasthenia gravis, hypocalcemia, and conditions that depress neuromuscular transmission; adjust dose in renal impairment

Drug Category: H2 blockers

This agent is 1 of 2 treatment modalities used for gastric acid hypersecretion.

Drug Category: Proton pump inhibitors

This agent is 1 of 2 treatment modalities used for gastric acid hypersecretion.

Drug Category: Choleretic agents

These agents improve biliary flow and prevent TPN-induced liver disease.

Drug Name	Phenobarbital (Barbita, Luminal, Solfoton)
Description	Improves bile acid–independent flow.
Adult Dose	90-180 mg/d PO qd or as 2-3 divided doses; not to exceed 400 mg/24h
Pediatric Dose	3-8 mg/kg/d PO divided bid-tid
Contraindications	Documented hypersensitivity; severe respiratory disease; marked impairment of liver function; nephritis
Interactions	May decrease effects of chloramphenicol, digitoxin, corticosteroids, carbamazepine, theophylline, verapamil, metronidazole, and anticoagulants (patients stabilized on anticoagulants may require dosage adjustments if added to or withdrawn from their regimen); coadministration with alcohol may produce additive CNS effects and death; chloramphenicol, valproic acid, and MAOIs may increase phenobarbital toxicity; rifampin may decrease phenobarbital effects; induction of microsomal enzymes may result in decreased effects of PO contraceptives in women (must use additional contraceptive methods to prevent unwanted pregnancy; menstrual irregularities may also occur)
Pregnancy	D - Unsafe in pregnancy
Precautions	In prolonged therapy, evaluate hematopoietic, renal, hepatic, and other organ systems; caution in fever, hyperthyroidism, diabetes mellitus, and severe anemia because adverse reactions can occur; caution in myasthenia gravis and myxedema

Drug Category: Bile salt binders

These agents decrease choleretic diarrhea.

Drug Category: Antisecretin agents

These agents decrease intestinal secretions.

Drug Category: Hypomotility agents

These agents increase intestinal transit time.

Drug Category: Fat-soluble vitamins

These agents supplement fat-soluble vitamins A, D, E, and K.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

• A multidisciplinary team should closely monitor the patient with SBS. The gastroenterologist, nutritionist, and pharmacist who manage the patient's TPN are integral to the success of outpatient management of the patient with SBS. The cooperation between these health care providers and home care nursing services is necessary for the proper surveillance of patients with SBS and the execution of investigative testing and treatments. The frequency of home testing, nurse visitation, outpatient follow-up, and hospitalization often lead to noncompliance, morbidity, and treatment failure.

Complications

• The leading cause of death in infants with SBS who are being treated with parenteral nutrition is central line sepsis and complications of the liver and biliary tract associated with the prolonged use of parenteral nutrition.
• Cholestasis is a frequent complication of patients on long-term parenteral nutrition. To some extent, bacterial overgrowth and specific nutrition deficiency can lead to worsening cholestasis in patients with SBS.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal Pitfalls

• A nutrition support service that is experienced in dealing with patients with SBS should manage these cases because of the complex pathophysiology of SBS. Daily modifications in therapy for these individuals are not unusual.
• The potential risk for complications may have medicolegal implications if the physician or paramedical services do not have the experience to manage cases of SBS.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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• Hofmann AF, Poley JR. Role of bile acid malabsorption in pathogenesis of diarrhea and steatorrhea in patients with ileal resection. I. Response to cholestyramine or replacement of dietary long chain triglyceride by medium chain triglyceride. Gastroenterology. May 1972;62(5):918-34. [Medline].
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• Hylander E, Ladefoged K, Jarnum S. Calcium absorption after intestinal resection. The importance of a preserved colon. Scand J Gastroenterol. Jul 1990;25(7):705-10. [Medline].
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Article Last Updated: Jun 27, 2006