Twenty Years of Gut Transplantation for Chronic Intestinal Pseudo-obstruction: Technical Innovation, Long-term Outcome, Quality of Life, and Disease Recurrence.

OBJECTIVE: To define long-term outcome, predictors of survival, and risk of disease recurrence after gut transplantation (GT) in patients with chronic intestinal pseudo-obstruction (CIPO). BACKGROUND: GT has been increasingly used to rescue patients with CIPO with end-stage disease and home parenteral nutrition (HPN)-associated complications. However, long-term outcome including quality of life and risk of disease recurrence has yet to be fully defined. METHODS: Fifty-five patients with CIPO, 23 (42%) children and 32 (58%) adults, underwent GT and were prospectively studied. All patients suffered gut failure, received HPN, and experienced life-threatening complications. The 55 patients received 62 allografts; 43 (67%) liver-free and 19 (33%) liver-contained with 7 (13%) retransplants. Hindgut reconstruction was adopted in 1993 and preservation of native spleen was introduced in 1999. Immunosuppression was tacrolimus-based with antilymphocyte recipient pretreatment in 41 (75%). RESULTS: Patient survival was 89% at 1 year and 69% at 5 years with respective graft survival of 87% and 56%. Retransplantation was successful in 86%. Adults experienced better patient (P = 0.23) and graft (P = 0.08) survival with lower incidence of post-transplant lymphoproliferative disorder (P = 0.09) and graft versus host disease (P = 0.002). Antilymphocyte pretreatment improved overall patient (P = 0.005) and graft (P = 0.069) survival. The initially restored nutritional autonomy was sustainable in 23 (70%) of 33 long-term survivors with improved quality of life. The remaining 10 recipients required reinstitution of HPN due to allograft enterectomy (n = 3) or gut dysfunction (n = 7). Disease recurrence was highly suspected in 4 (7%) recipients. CONCLUSIONS: GT is life-saving for patients with end-stage CIPO and HPN-associated complications. Long-term survival is achievable with better quality of life and low risk of disease recurrence.

The natural history of infantile mitochondrial DNA depletion syndrome due to RRM2B deficiency.

PURPOSE: Mitochondrial DNA (mtDNA) depletion syndrome (MDDS) encompasses a group of genetic disorders of mtDNA maintenance. Mutation of RRM2B is an uncommon cause of infantile-onset encephalomyopathic MDDS. Here we describe the natural history of this disease. METHODS: Multinational series of new genetically confirmed cases from six pediatric centers. RESULTS: Nine new cases of infantile-onset RRM2B deficiency, and 22 previously published cases comprised a total cohort of 31 patients. Infants presented at a mean of 1.95 months with truncal hypotonia, generalized weakness, and faltering growth. Seizures evolved in 39% at a mean of 3.1 months. Non-neurological manifestations included respiratory distress/failure (58%), renal tubulopathy (55%), sensorineural hearing loss (36%), gastrointestinal disturbance (32%), eye abnormalities (13%), and anemia (13%). Laboratory features included elevated lactate (blood, cerebrospinal fluid (CSF), urine, magnetic resonance (MR), spectroscopy), ragged-red and cytochrome c oxidase-deficient fibers, lipid myopathy, and multiple oxidative phosphorylation enzyme deficiencies in skeletal muscle. Eight new RRM2B variants were identified. Patients with biallelic truncating variants had the worst survival. Overall survival was 29% at 6 months and 16% at 1 year. CONCLUSIONS: Infantile-onset MDDS due to RRM2B deficiency is a severe disorder with characteristic clinical features and extremely poor prognosis. Presently management is supportive as there is no effective treatment. Novel treatments are urgently needed.

Pediatric Intestinal Pseudo-obstruction: Impact of Neonatal and Later Onset on Clinical and Nutritional Outcomes.

OBJECTIVE: The aim of the study was to evaluate long-term nutritional outcomes and clinical characteristics in a cohort of children with pediatric intestinal pseudo-obstruction (PIPO) at neonatal-onset (NO-PIPO) and at later-onset (LO-PIPO). METHODS: All children fulfilling new PIPO criteria over a 30-year period were reviewed. Baseline demographic and clinical features as well as nutritional outcomes were collected. Nutritional outcomes included overall survival, prevalence of enteral autonomy and parenteral nutrition (PN) dependency, rate of major PN complications, and growth course. RESULTS: Forty-four patients were still alive at the end of the follow-up. Twenty-five patients (57%) achieved enteral autonomy, whilst 18 remained on PN. Among the patients requiring PN at the beginning of the study period, we found that 55% (CI 34-70) has the probability of remaining on PN at the latest follow-up. Prevalence of gastrointestinal obstruction symptoms (P < 0.01), urinary involvement (P < 0.05), stoma placements [gastrostomy (P < 0.01), ileostomy P < 0.05)] and complex gastrointestinal surgery (P < 0.05) were significantly higher in NO-PIPO than in LO-PIPO. The number of patients requiring long-term PN (P < 0.001) and the number of PN days (P < 0.05) were significantly higher in NO-PIPO, whilst the number of patients achieving enteral autonomy was significantly higher in LO-PIPO (P < 0.05). CONCLUSIONS: In our study, we have reported the nutritional outcome of a cohort of children with PIPO over a 30-year period showing that about 20% of patients develop irreversible intestinal failure requiring life-long PN. Nutritional and clinical outcomes seem to be influenced by the time of onset of the disease.

Assessment and outcome of pediatric intestinal pseudo-obstruction: A tertiary-care-center experience from Turkey.

BACKGROUND/AIMS: Pediatric intestinal pseudo-obstruction (PIPO) is a severe disorder of gut motility. In this rare and difficult-to-manage disease, complex treatment method, such as intestinal transplantation, is sometimes needed. This study evaluated the management and follow-up results of patients with PIPO who received treatment at our center. MATERIALS AND METHODS: The cases of 13 patients with PIPO were reviewed retrospectively. Demographic data, clinical features, etiologies, pharmacological and surgical treatments, nutritional support, anthropometric findings, small bowel transplantation (SBT), and survival rates were assessed. RESULTS: Two of the patients were diagnosed at 1 and 5 years of age, while other patients were diagnosed during neonatal period. The etiological cause could not be identified for 5 patients. Pharmacological treatment response was observed in 38.4% of patients. Post-pyloric feeding was applied in 4 patients, but no response was observed. Gastrostomy decreased the clinical symptoms in 3 patients during the abdominal distension period. Total oral nutrition was achieved in 38.4% of the total-parenteral-nutrition (TPN)-dependent patients. It was observed that anthropometric findings improved in patients with total oral nutrition. Liver cirrhosis developed in 1 patient. Venous thrombosis developed in 4 patients. The SBT was performed on 3 patients. One of these patients has been followed up for the last 4 years. CONCLUSION: Pediatric intestinal pseudo-obstruction is a rare disease that can present with a wide range of clinical symptoms. While some patients require intestinal transplantation, supportive care may be sufficient in others. For this reason, patients with PIPO should be managed individually.

Causes and prognosis of chronic intestinal pseudo-obstruction in 48 subjects: A 10-year retrospective case series.

The aim of the study was to evaluate the prognosis and survival of pediatric subjects with chronic intestinal pseudo-obstruction (CIPO) and investigate the independent risk factors affecting their prognosis.This was a retrospective case series of all pediatric subjects suffering from CIPO and treated at the Pediatric Surgical ward of Xinhua Hospital between January 2006 and January 2016.The overall mortality was 19/48 (39.6%). Because of delayed CIPO diagnosis, many subjects underwent a variety of surgical procedures. The rate of additional surgical procedures was high (35/48, 72.9%), but the number of surgical procedures, parenteral nutrition, and megacystis did not affect mortality. Mycotic infection was significantly associated with mortality, while onset at <1 year and hypoganglionosis showed a tendency to be associated with mortality.Mycotic infection was associated with mortality of children with CIPO. Despite improving treatment approaches, the overall prognosis of CIPO remains poor. The choice of the surgical intervention could be based on standard criteria.

Upholding Ethical Decision Making in Children With Life Limiting Illnesses.

Emeritus Professor Edward Alan Glasper from the University of Southampton discusses the complexities of care delivery to children in hospital who have life limiting medical conditions.

[Conservative and surgical ileus treatment]. / Konservative und operative Therapie des Ileus.

The diagnosis ileus is one of the most common indications for an emergency laparotomy. In 70% of the cases, the small intestine is affected, and in 30% it is the colorectum. While stuck hernias are a major cause in developing countries, the most common causes in western countries are postoperative adhesions that lead to an acute bowl obstruction. The timeframe for treatment of a complete mechanical obstruction is short as acute ischemia can lead to necrosis with bowl perforation within 6 h. The perioperative lethality for an emergency laparotomy due to an ileus ranges from 5-15%. In addition to the mechanical ileus, primary and secondary paralytic ileus is important in the differential diagnosis. As the genesis of postoperative ileus is multifactorial, a multimodal concept is required for successful treatment.

Gastrointestinal system involvement in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multisystem disorder which can affect the gastrointestinal (GI) system. Although GI symptoms can manifest in 50% of patients with SLE, these have barely been reviewed due to difficulty in identifying different causes. This study aims to clarify clinical characteristics, diagnosis and treatment of the four major SLE-related GI system complications: protein-losing enteropathy (PLE), intestinal pseudo-obstruction (IPO), hepatic involvement and pancreatitis. It is a systematic review using MEDLINE and EMBASE databases and the major search terms were SLE, PLE, IPO, hepatitis and pancreatitis. A total of 125 articles were chosen for our study. SLE-related PLE was characterized by edema and hypoalbuminemia, with Technetium 99m labeled human albumin scintigraphy (99mTc HAS) and alpha-1-antitrypsin fecal clearance test commonly used as diagnostic test. The most common site of protein leakage was the small intestine and the least common site was the stomach. More than half of SLE-related IPO patients had ureterohydronephrosis, and sometimes they manifested as interstitial cystitis and hepatobiliary dilatation. Lupus hepatitis and SLE accompanied by autoimmune hepatitis (SLE-AIH overlap) shared similar clinical manifestations but had different autoantibodies and histopathological features, and positive anti-ribosome P antibody highly indicated the diagnosis of lupus hepatitis. Lupus pancreatitis was usually accompanied by high SLE activity with a relatively high mortality rate. Early diagnosis and timely intervention were crucial, and administration of corticosteroids and immunosuppressants was effective for most of the patients.

Megacystis microcolon intestinal hypoperistalsis syndrome: Case series and updated review of the literature with an emphasis on urologic management.

INTRODUCTION: Megacystis microcolon intestinal hypoperistalsis (MMIHS) is a rare disorder characterized by distended nonobstructed bladder, microcolon, and decreased intestinal peristalsis. MMIHS has a particularly poor prognosis; however, when appropriately managed, survival can be prolonged. STUDY DESIGN: A systematic review (1996-2016) was performed with the key words "megacystis microcolon intestinal hypoperistalsis syndrome." In addition, a case series of four patients is presented as well as algorithms for the diagnosis and treatment of MMIHS. RESULTS: 135 patients with MMIHS were identified in the literature. 73% (88/121) of the patients were female, 65% underwent diagnostic biopsy (64/99), and 63% (66/106) were identified with prenatal imaging. The majority of patients were treated with TPN as well as gastrostomy or ileostomy and CIC, however 15% (18/116) received multivisceral or intestinal transplant, and 30% (22/73) had a vesicostomy. The survival rate was 57% (68/121). CONCLUSION: Appropriate management of MMIHS patients is crucial. An enlarged, acontractile bladder in a child with bowel motility problems should be considered diagnostic. Bladder distension can be managed with CIC or vesicostomy in addition to prophylactic antibiotics if frequent urinary tract infections are present. These patients often require gastrostomy or ileostomy as well as total parenteral nutrition. This management has led to significant improvement in survival rates.

Predictors of intestinal pseudo-obstruction in systemic lupus erythematosus complicated by digestive manifestations: data from a Southern China lupus cohort.

OBJECTIVE: To determine factors that may predict intestinal pseudo-obstruction (IpsO) in systemic lupus erythematosus (SLE) patients complicated by digestive manifestations. METHODS: SLE patients with digestive manifestations (n = 135) were followed at Southern Medical University affiliated Nanfang Hospital from 2000 until 2013. Demographic variables, clinical features, and laboratory data were compared between the two groups. Univariate and multivariate logistic regression models were used to establish factors that predispose to IpsO in these patients. RESULTS: At the end of the study period, 32 (23.7%) patients had developed IpsO. Mortality (9 patients) was infrequent and the cause of death was unrelated to IpsO. Independent predictors of IpsO in SLE were ureterectasia, anti-U1 RNP(+), peritonitis, and low C3 levels. CONCLUSIONS: Regular abdominal X-ray examinations are recommended in SLE patients with ureterectasia, anti-U1 RNP(+), peritonitis, or low C3 levels, as early diagnosis and therapy may prevent unnecessary surgical intervention and improve the disease course.

Megacystis microcolon intestinal hypoperistalsis syndrome: A report of a nationwide survey in Japan.

BACKGROUND: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare condition with a poor outcome. A nationwide survey was conducted to identify the clinical features and outcomes of MMIHS in Japan. METHODS: Data were collected via a questionnaire, which was sent to 353 pediatric/pediatric surgical departments in Japan. RESULTS: Of the 28 patients registered as having a certain diagnosis of MMIHS, 19 (male/female, 3/16) patients were analyzed. All of the patients developed functional bowel obstruction in the neonatal period and showed a distended bladder and microcolon in the radiological examination. A histopathology assessment of the full-thickness of intestinal specimens showed no pathological abnormalities in all patients. Although various medications were given, the patients did not show significant improvement. Drainage stomas were created in the jejunum (n=11) and colon (n=5). Sixteen patients were maintained by parenteral nutrition (PN). Nine patients died of sepsis or liver failure. The five- and ten-year survival rates were 63% and 57%, respectively. CONCLUSIONS: MMIHS patients developed severe functional intestinal obstruction in the neonatal period and had no specific therapeutic intervention. The majority of MMIHS patients required long-term PN. Small bowel or multivisceral transplantation may be necessary to improve the outcome of this condition.

[PROGNOSTIC SIGNIFICANCE OF ELEMENTS OF THE MULTIORGAN INSUFFICIENCY SYNDROME AND ESTIMATION OF THE PATIENTS STATE SEVERITY IN ACCORDANCE TO INTEGRAL SCALES IN AN ACUTE NECROTIC PANCREATITIS].

There were examined 113 patients, in whom an acute necrotic pancreatitis was diagnosed. While admittance to hospital a constant organ insufficiency was revealed in 50 (44%), a transient one--in 63 (56%) patients. In total 31 (27.4%) patients died. Respiratory insufficiency have occurred in 67% patients, and almost with similar rate--cardio-vascular (in 59%), renal (in 56%) and enteral (in 54%), dominating in the lethality structure. The BISAP (Bedcide Index for Severity in Acute Pancreatitis) scales applied permit with high probability to prognosticate the organ insufficiency and lethality in patients, suffering an acute necrotic pancreatitis.

Mortality, recurrence, and hospital course of patients with systemic sclerosis-related acute intestinal pseudo-obstruction.

OBJECTIVE: Acute intestinal pseudo-obstruction is a rare gastrointestinal manifestation of systemic sclerosis (SSc) with few data existing as to its demographics, clinical course, outcomes, and mortality. METHODS: We undertook a case-control study to describe 64 cases in 37 unique patients, of whom 70% had spontaneous resolution with conservative measures of intravenous hydration and bowel rest, 9% underwent surgical resection, and 25% required prolonged total parenteral nutrition (TPN). RESULTS: Hospital course was for a mean of 12 ± 12.5 days and there was a 16% patient mortality in our population. In a subgroup analysis, patients who had recurrent episodes of pseudo-obstruction were less likely to have esophageal involvement from SSc, and more likely to need prolonged TPN. Mortality tended to be higher in male patients and patients who did not have SSc-related esophageal involvement, and also in patients who had low hemoglobin and serum albumin at presentation. The need for a nasogastric tube for decompression and a surgical intervention correlated with a more prolonged hospital stay. CONCLUSION: To the best of our knowledge, ours is the largest study looking at this rare manifestation of SSc.

Deoxynucleoside stress exacerbates the phenotype of a mouse model of mitochondrial neurogastrointestinal encephalopathy.

Balanced pools of deoxyribonucleoside triphosphate precursors are required for DNA replication, and alterations of this balance are relevant to human mitochondrial diseases including mitochondrial neurogastrointestinal encephalopathy. In this disease, autosomal recessive TYMP mutations cause severe reductions of thymidine phosphorylase activity; marked elevations of the pyrimidine nucleosides thymidine and deoxyuridine in plasma and tissues, and somatic multiple deletions, depletion and site-specific point mutations of mitochondrial DNA. Thymidine phosphorylase and uridine phosphorylase double knockout mice recapitulated several features of these patients including thymidine phosphorylase activity deficiency, elevated thymidine and deoxyuridine in tissues, mitochondrial DNA depletion, respiratory chain defects and white matter changes. However, in contrast to patients with this disease, mutant mice showed mitochondrial alterations only in the brain. To test the hypothesis that elevated levels of nucleotides cause unbalanced deoxyribonucleoside triphosphate pools and, in turn, pathogenic mitochondrial DNA instability, we have stressed double knockout mice with exogenous thymidine and deoxyuridine, and assessed clinical, neuroradiological, histological, molecular, and biochemical consequences. Mutant mice treated with exogenous thymidine and deoxyuridine showed reduced survival, body weight, and muscle strength, relative to untreated animals. Moreover, in treated mutants, leukoencephalopathy, a hallmark of the disease, was enhanced and the small intestine showed a reduction of smooth muscle cells and increased fibrosis. Levels of mitochondrial DNA were depleted not only in the brain but also in the small intestine, and deoxyribonucleoside triphosphate imbalance was observed in the brain. The relative proportion, rather than the absolute amount of deoxyribonucleoside triphosphate, was critical for mitochondrial DNA maintenance. Thus, our results demonstrate that stress of exogenous pyrimidine nucleosides enhances the mitochondrial phenotype of our knockout mice. Our mouse studies provide insights into the pathogenic role of thymidine and deoxyuridine imbalance in mitochondrial neurogastrointestinal encephalopathy and an excellent model to study new therapeutic approaches.

Non-transplantation surgical approach for chronic intestinal pseudo-obstruction: analysis of 63 adult consecutive cases.

BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) is a recurrent disorder caused by intestinal dysmotility. Although, CIPO is not a surgically remediable condition, surgery is a common issue in CIPO patients. The aim of this study was to assess postoperative morbidity and mortality of patients operated on for CIPO and risk of re-operation. METHODS: Adult patients that have undergone surgery for CIPO since 1980 were included in this retrospective study using a prospective database. Postoperative morbidity, according to Clavien-Dindo classification and CIPO-related re-operation rates were evaluated with univariate and multivariate analysis. KEY RESULTS: Sixty-three patients (33 women, median age 37 [range: 15-79] years) were included. Median follow-up was 6 (0.2-28) years. Overall postoperative mortality rate was 7.9%. Overall morbidity rate was 58.2% (Clavien-Dindo ≥3 in 20.7%) leading to re-operation in 17% of cases. In multivariate analysis, major postoperative morbidity (Clavien-Dindo ≥3) was significantly increased when there was an intraoperative bowel injury (HR = 15.7 [2.4-102], P = 0.004), idiopathic CIPO (HR = 4.2 [1.5-12], P = 0.007) and emergency procedure (HR = 3 [1.3-6.8]. After the first surgery, probabilities of CIPO-related re-operation were 44%, 60%, and 66% at 1, 3, and 5 years respectively. In multivariate analysis, CIPO-related reoperation for CIPO was significantly increased when there was a major postoperative morbidity (HR = 2.1 [1.1-4.4], P = 0.040) and intraoperative bowel injury (HR = 33.1 [2-553], P = 0.015) after the first procedure. CONCLUSIONS & INFERENCES: The surgical management of CIPO patients was associated with high postoperative morbidity and mortality rates and frequent re-operation. Attempts should be made to avoid surgery when possible and optimize nutritional status.

Characteristics of intestinal pseudo-obstruction in patients with mitochondrial diseases.

AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and 18 females) were diagnosed with mitochondrial diseases at our hospital. We conducted a retrospective review of the patients' sex, subclass of mitochondrial disease, age at onset of mitochondrial disease, frequency of CIP and the age at its onset, and the duration of survival. The age at onset or at the first diagnosis of the disorder that led to the clinical suspicion of mitochondrial disease was also examined. RESULTS: Twenty patients were sub-classified with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), 8 with chronic progressive external ophthalmoplegia (CPEO), and 3 with myoclonus epilepsy associated with ragged-red fibers (MERRF). Nine patients were diagnosed with CIP, 8 of the 20 (40.0%) patients with MELAS, 0 of the 8 (0.0%) patients with CPEO, and 1 of the 3 (33.3%) patients with MERRF. The median age (range) at the diagnosis and the median age at onset of mitochondrial disease were 40 (17-69) and 25 (12-63) years in patients with CIP, and 49 (17-81) and 40 (11-71) years in patients without CIP. During the survey period, 5 patients (4 patients with MELAS and 1 with CPEO) died. The cause of death was cardiomyopathy in 2 patients with MELAS, cerebral infarction in 1 patient with MELAS, epilepsy and aspiration pneumonia in 1 patient with MELAS, and multiple metastases from gastric cancer and aspiration pneumonia in 1 patient with CPEO. CONCLUSION: Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP.

Prognostic yield of esophageal manometry in chronic intestinal pseudo-obstruction: a retrospective cohort of 116 adult patients.

BACKGROUND: Chronic intestinal pseudo-obstruction (CIPO) refers to a wide and heterogeneous group of neuromuscular disorders, which classically involve the small intestine. However, further investigation is required to determine if motility disturbances involve all parts of the gastrointestinal (GI) tract. METHODS: Medical records and follow-up examinations of 116 adult CIPO patients [70F, median age 28 (0-79) years] were reviewed and performed at our institution since 1980. Manometry (esophageal, small bowel and anorectal) and gastric emptying scintigraphy reports were retrieved and analyzed. Survival, home parenteral nutrition requirement, and the inability to maintain sufficient oral feeding was analyzed using univariate and multivariate analysis. KEY RESULTS: The median follow-up time was 6 (0.1-30) years. In all, 90% of patients who underwent at least one motility test, with the exception of small bowel manometry, exhibited at least one abnormal pattern. Esophageal manometry was abnormal in 73% of the cases, including 51% with severe ineffective esophageal motility. Anorectal manometry was abnormal in 59% of the cases, including only 17% with severe abnormalities. Gastric emptying was abnormal in 61% of the cases. Only esophageal motor disorders had significant predicting values for survival, home parenteral nutrition requirement, and an inability to maintain sufficient oral feeding. CONCLUSIONS & INFERENCES: Our study showed that CIPO was associated with a diffuse involvement of all parts of the GI tract and was not restricted to the small intestine in 90% of the cases studied. Esophageal manometry had a significant prognostic yield and should be systematically performed in CIPO patients.

Home parenteral nutrition in patients with systemic sclerosis: a retrospective review of 12 cases.

OBJECTIVE: To evaluate the effect of home total parenteral nutrition (HTPN) on the nutritional and functional status of patients with systemic sclerosis (SSc). METHODS: Retrospective review of SSc patients from the HTPN database at the University Health Network, Toronto, Canada, from 1998 to 2010. RESULTS: In total 12 subjects (9 females, 3 males) with SSc were identified. At initiation of HTPN, the age was 48.75 ± 3.94 years and the median body mass index was 16.8 kg/m(2) (range 12.3-21.3). This significantly increased to 18.3 kg/m(2) (17.0-21.7) at 6 months and to 19.7 kg/m(2) (17.0-24.6) at 12 months. The mean Karnofsky score at baseline was 58.00 ± 3.27, and the score at 12 months of 39.00 ± 13.29 did not change significantly. CONCLUSION: HTPN improved the nutritional status of these patients with SSc but it did not improve their functional status.

Nationwide survey on adult type chronic intestinal pseudo-obstruction in surgical institutions in Japan.

BACKGROUND: No appropriate management of chronic intestinal pseudo-obstruction (CIP) has been established. PATIENTS AND METHODS: The clinicopathological parameters of 103 cases collected by a nationwide questionnaire study were reviewed. RESULTS: The CIP cases were primary in 86 (83%) cases and secondary in 15 (15%) cases. The age of onset of the primary type was significantly younger than that of the secondary type (p = 0.011). The diseased segments of the bowel were the large bowel in 60 (58%), the small bowel in 17 (17%), and both in 23 (22%) cases, respectively. Abdominal distension and pain were common symptoms regardless of the types of the diseased bowel; however, constipation was frequently seen in the large bowel type (p = 0.0258). Vomiting and diarrhea were seen with marginally higher frequency in the small bowel type (p = 0.0569, 0.0642). Surgical treatment was most effective in the large bowel type, less effective in the small bowel type, and least effective in the large and small bowel type. The prognosis of the primary CIP was significantly better than that of the secondary CIP (p = 0.033). CONCLUSIONS: The segments of the diseased bowels should be considered in determining the indications for surgical treatments in CIP patients.

Long-term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition.

OBJECTIVES: Chronic intestinal pseudo-obstruction (CIPO) is a rare, disabling disorder responsible for motility-related intestinal failure. Because it induces malnutrition, CIPO is a significant indication for home parenteral nutrition (HPN). The objective of the study was to evaluate long-term outcome of CIPO patients requiring HPN during adulthood. METHODS: In total, 51 adult CIPO patients (18 men/33 women, median age at symptom occurrence 20 (0-74) years, 34/17 primary/secondary CIPO) followed up at our institution for HPN management between 1980 and 2006 were retrospectively studied for survival and HPN dependence rates using univariate and multivariate analysis. RESULTS: Follow-up after diagnosis was 8.3 (0-29) years. Surgery was required in 84% of patients. The number of interventions was 3 +/- 3 per patient (mean +/- s.d.), leading to short bowel syndrome in 19 (37%) patients. Actuarial survival probability was 94, 78, 75, and 68% at 1, 5, 10, and 15 years, respectively. Multivariate analysis showed that lower mortality was associated with the ability to restore oral feeding at baseline (hazard ratio (HR) = 0.2 (0.06-0.65), P = 0.008) and symptom occurrence before the age of 20 years (HR=0.18 (0.04-0.88), P = 0.03). Higher mortality was associated with systemic sclerosis (HR=10.4 (1.6-67.9), P = 0.01). Actuarial HPN dependence was 94, 75, and 72% at 1, 2, and 5 years, respectively. CONCLUSIONS: In this large cohort of CIPO adult patients with severe intestinal failure, i.e., those requiring HPN, we found a higher survival probability than previously reported. These results should be taken into account when considering intestinal transplantation.