Abdullah Shatnawei

Mortality of Intra-Abdominal Desmoid Tumors in Patients With Familial Adenomatous Polyposis A Single Center Review of 154 Patients

Introduction:Intra-abdominal desmoid tumors are one of the leading causes of death in patients with familial adenomatous polyposis. Their behavior is unpredictable and their biology is poorly understood, accounting for the lack of a standardized medical and surgical approach. The aim of this study was to evaluate the mortality rate of patients with intra-abdominal desmoid tumors and to identify prognostic factors for the evolution of the disease. Materials and Methods:A total of 154 patients with intra-abdominal desmoid tumors were included in the study. Each tumor was staged and each patient was categorized according to the stage of their most advanced tumor. Mortality was analyzed and the univariate risk factors associated with survival were included in a multivariable Cox regression model. A scoring system was derived from the multivariate analysis to refine outcomes within stages. Results:Five-year survival of patients with stage I, II, III, and IV intra-abdominal desmoid tumor were 95%, 100%, 89%, and 76% respectively (P < 0.001). Severe pain/narcotic dependency, tumor size larger than 10 cm, and need for total parenteral nutrition were shown to further define survival within stages. Five-year survival rate of stage IV patient with all of the above-mentioned risk factors was only 53%. Conclusions:Our study confirmed the validity of the staging system to predict mortality in patients with intra-abdominal desmoid tumors and identified additional risk factors able to better define the risk of death within each stage. Risk stratification is crucial in directing patients with advanced disease and poor prognosis to the most appropriate medical and surgical options.

Hypocaloric home parenteral nutrition and nutrition parameters in patients following bariatric surgery.

BACKGROUND Surgical intervention is considered an acceptable treatment for morbid obesity. Complications following bariatric surgery procedures (BSPs) may necessitate home parenteral nutrition (HPN). No studies have been published on patients receiving HPN following BSP complications. The study aim was to determine if hypocaloric HPN has an effect on body mass index (BMI), albumin, and HPN complications. METHODS A historic cohort of patients was identified from a clinical database. Obese patients (BMI ≥35 kg/m(2)) who underwent BSP and received HPN for an anastomotic leak/fistula or bowel obstruction were included. Comparisons for start and end of therapy were made for calorie and protein intake, BMI, white blood cell count, and serum albumin level. Readmissions and metabolic and infectious complications were recorded. Obese patients received hypocaloric feeds to promote weight loss and protein for wound healing. RESULTS Twenty-three patients were included for an average study length of 1.5 months. Patients received an average of 1.2 g of protein and a median of 13.6 kcal per kg actual body weight (ABW) per day. BMI decreased by 7.1% ± 5.2%, from a median of 39.8 to 37.1, and serum albumin increased by 12.5%, from 2.8 ± 0.5 to 3.2 ± 0.6 g/dL. Readmissions occurred in 52.2% of patients with 40.0% of complications related to HPN. CONCLUSIONS Hypocaloric HPN is efficacious in maintaining adequate nutrition while allowing for weight loss in morbidly obese patients following complications of bariatric surgery. Frequency of HPN complications was comparable to those reported in the literature.

Intestinal Failure Management at the Cleveland Clinic

The Cleveland Clinic institutional guidelines for the management of intestinal failure, including long-term or home parenteral nutrition and related complications, intestinal rehabilitation, and small bowel transplantation, were reviewed. PubMed was searched for relevant articles. The search was performed in November 2008; keywords used were home parenteral nutrition, short bowel syndrome, intestinal rehabilitation, and small-bowel transplantation. Randomized, prospective, observational, retrospective reviews and case report articles that contained relevant data for long-term parenteral nutrition, intestinal rehabilitation, and intestinal transplantation were selected. Researchers reviewed 67 selected articles that met our inclusion criteria. Our institution data registries for intestinal rehabilitation and home parenteral nutrition were also reviewed for relevant data. The survival of tens of thousands of children and adults with complicated gastrointestinal problems has been possible because of parenteral nutrition. In selected patients, a program of intestinal rehabilitation may avoid the need for long-term parenteral nutrition.

Gastrointestinal Manifestations, Malnutrition, and Role of Enteral and Parenteral Nutrition in Patients With Scleroderma.

Scleroderma (systemic sclerosis) is an autoimmune disease that can affect multiple organ systems. Gastrointestinal (GI) involvement is the most common organ system involved in scleroderma. Complications of GI involvement including gastroesophageal reflux disease, small intestinal bacterial overgrowth, and chronic intestinal pseudoobstruction secondary to extensive fibrosis may lead to nutritional deficiencies in these patients. Here, we discuss pathophysiology, progression of GI manifestations, and malnutrition secondary to scleroderma, and the use of enteral and parenteral nutrition to reverse severe nutritional deficiencies. Increased mortality in patients with concurrent malnutrition in systemic sclerosis, as well as the refractory nature of this malnutrition to pharmacologic therapies compels clinicians to provide novel and more invasive interventions in reversing these nutritional deficiencies. Enteral and parenteral nutrition have important implications for patients who are severely malnourished or have compromised GI function as they are relatively safe and have substantial retrospective evidence of success. Increased awareness of these therapeutic options is important when treating scleroderma-associated malnutrition.

Fish oil-based lipid emulsion: current updates on a promising novel therapy for the management of parenteral nutrition-associated liver disease.

Intestinal failure is characterized by loss of enteral function to absorb necessary nutrients and water to sustain life. Parenteral nutrition (PN) is a lifesaving therapeutic modality for patients with intestinal failure. Lifelong PN is also needed for patients who have short bowel syndrome due to extensive resection or a dysmotility disorder with malabsorption. However, prolonged PN is associated with short-term and long-term complications. Parenteral nutrition-associated liver disease (PNALD) is one of the long-term complications associated with the use of an intravenous lipid emulsion to prevent essential fatty acid deficiency in these patients. PNALD affects 30–60% of the adult population on long-term PN. Further, PNALD is one of the indications for isolated liver or combined liver and intestinal transplantation. There is no consensus on how to manage PNALD, but fish oil-based lipid emulsion (FOBLE) has been suggested to play an important role both in its prevention and reversal. There is significant improvement in liver function in those who received FOBLE as lipid supplement compared with those who received soy-based lipid emulsion. Studies have also demonstrated that FOBLE reverses hepatic steatosis and reduces markers of inflammation in patients on long-term PN. Future prospective studies with larger sample sizes are needed to further strengthen the positive role of FOBLE in PNALD.

Prevalence and predictors of vitamin D deficiency and response to oral supplementation in patients receiving long-term home parenteral nutrition.

UNLABELLED Previous studies have suggested a high prevalence of vitamin D deficiency in patients receiving long-term home parenteral nutrition (HPN). The aim of this study was to determine the prevalence and predictors of vitamin D deficiency in long-term HPN patients. METHODS A retrospective, institutional review board-approved study was performed on all adult patients followed by the Cleveland Clinic HPN program receiving HPN therapy >6 months between 1989 and 2013 with a 25-(OH) D3 level reported. Patients were categorized by serum vitamin D status as follows: sufficient, insufficient, and deficient with respective 25-(OH) D3 levels of ≥30 ng/mL, 20-30 ng/mL, and <20 ng/mL. RESULTS Seventy-nine patients were categorized based on serum vitamin D status as follows: 35 (44.3%) deficient, 24 (30.4%) insufficient, and 20 (25.3%) sufficient. The mean age of the cohort at the initiation of HPN was 52.0 ± 12.7 years, and 26 (32.9%) were male. The median HPN duration was 39 months, and the most common indication was inflammatory bowel disease (36.7%). Most (82.3%) patients had at least 1 prescription of oral vitamin D supplement (50,000 International Units) during this time. History of jejunal resection (odds ratio [OR], 5.3; 95% confidence interval [CI], 1.9-15.1; P = .002) and lack of oral vitamin D supplementation (OR, 0.7; 95% CI, 0.52-0.93; P = .038) were the strongest predictors of vitamin D deficiency. CONCLUSION Vitamin D deficiency is common among patients receiving long-term HPN despite oral supplementation.

The Association Between Home Parenteral Nutrition and Patients With FAP-Associated Intra-abdominal Desmoids A Retrospective Study

BACKGROUND Intra-abdominal desmoid tumors (IADTs) are a common complication of familial adenomatous polyposis (FAP). Treatment is not standardized for advanced disease. Medical and surgical treatments may be ineffective in preventing complications, which can cause intestinal failure. Home parenteral nutrition (HPN) can be a life-saving treatment in these patients. The aim of this study was to investigate the association with HPN in FAP-IADTs. METHODS A retrospective review of FAP patients with IADTs at the Cleveland Clinic (CC) between 1980 and 2009 was performed. Patients and tumor characteristics were retrieved from the CC Jagelman Registry for Inherited Neoplasms and CC HPN database. Inclusion criteria were FAP-IADTs and 6-month follow up at CC. Exclusion criteria were <6-month follow-up, lack of 3-dimensional lesion or sheet desmoid, and/or incomplete medical records. Kaplan-Meier curves were analyzed for HPN and non-HPN groups. RESULTS One hundred fifty-four patients were included and divided into 2 groups: HPN (n = 41, 26.6%) and non-HPN (n = 113, 73.4%). The HPN group was more likely to have advanced-stage disease and significantly higher incidence of chronic abdominal pain, narcotic dependency, bowel obstruction, ureteral obstruction, deep vein thrombosis, pulmonary embolism, fistulae, and sepsis (P < .05). The need for HPN represented a strong predictor of mortality (5-year survival HPN = 72% vs non-HPN = 95%), but duration of HPN did not affect mortality. CONCLUSION HPN, although a life-saving treatment, is an independent poor prognostic factor associated with high morbidity and mortality.

Use of home parenteral nutrition in patients with intra-abdominal desmoid tumors.

BACKGROUND Fistulae, small bowel obstruction (SBO), and malabsorption are complications of intra-abdominal desmoid (IAD) tumors that require home parenteral nutrition (HPN). HPN outcomes in patients with IAD tumors have not been previously reported. The aim of this study was to compare some of the nutrition parameters and complications of HPN in patients with IAD with a control group of patients on HPN. METHODS This was a case-control study of patients and randomly selected controls who required HPN because of fistulae, SBO, or malabsorption and were managed by the Cleveland Clinic Nutrition Support Team between 1990 and 2008. Variables included demographics, indications, number of episodes, duration of HPN, number of admissions and complications related to HPN, and nutrition parameters. Univariable and multivariable logistic regression analyses were used. RESULTS Eighteen of 1615 HPN patients (1.1%) had IAD. For the study, 58 patients were included: 14 with IAD and 44 controls. Four IAD patients did not have complete medical records. IAD patients had longer duration of HPN (P = .015), were younger (P = .028), and were more likely to receive HPN for malabsorption (P < .001). Body mass index (BMI), serum albumin level, protein intake provided at the beginning of HPN, energy intake provided at the start and end of HPN, mortality, and complications were comparable between groups. At the end of HPN, IAD patients received significantly more protein and had lower serum albumin levels compared with controls. CONCLUSIONS HPN for IAD patients maintained BMI but did not increase serum albumin concentration despite receiving more protein than controls. IAD patients did not have increased HPN-related complications.

Skin cancer in immunosuppressed transplant patients: Vigilance matters

Liver transplantation (LT) is a widely-accepted, definitive therapy of irreversible liver diseases including hepatitis C, alcoholic liver disease and metabolic liver disease. After transplantation, patients generally use a variety of immunosuppressive medications for the rest of their lives to prevent rejection of transplanted liver. Mortality after LT is mainly caused by recurrence of alcoholic hepatitis which is mostly seen in the patients who resume heavy drinking. On the other hand, de-novo malignancies after LT are not seldom. Skin cancers make up 13.5% of the de-novo malignancies seen in these patients. Malignancies tend to affect survival earlier in the course with a 53% risk of death at 5 years after diagnosis. We aimed to report a case who underwent LT secondary to alcoholic liver disease and developed squamous cell carcinoma of the skin eighteen years after transplantation. In summary, transplant recipients are recommended to be educated on self examination for skin cancer; health care providers should be further suspicious during routine dermatological examinations of the transplant patients and biopsies of possible lesions for skin cancer is warranted even many years after transplantation.

New-Onset Gastrointestinal Polyposis

Question: A 60-year-old man presented to the outpatient gastroenterology clinic with a 9-month history of diarrhea. The diarrhea started gradually, intermittent in course; he reported anywhere between 2 to 10 loose bowel movements a day, with a lot of mucous in the stool but no blood. This was associated with upper abdominal discomfort, bloating, early satiety, and nausea. He complained of significant fatigue and lost about 40 lbs during this period of illness. He complained of his fingernails and toenails becoming very brittle and eventually lost all of them, burning of the tongue, decreased taste sensation, hair loss, decreased libido, and development of skin hyperpigmentation over his thighs. The patient’s medical history is remarkable for hypertension, hyperlipidemia, osteoarthritis, and Parkinson’s disease. Family history is negative for inflammatory bowel disease, celiac disease, colon polyps, and colon cancer. Medications included amitriptyline, clonazepam, gabapentin, pramipexole, pravastatin, niacin, meloxicam, esomeprazole, hydrochlorothiazide, and verapamil. His laboratory tests on initial evaluation were significant for a total protein of 5.3 g/dL, albumin of 2.4 g/dL, hemoglobin of 11.2 g/dL, hematocrit of 35.5%, and white blood cells within normal range, at 9.60 cell/mL with 72% neutrophils. Stool studies including culture, ova, and parasites, and Clostridium difficile toxin polymerase chain reaction were all negative. Thyroid-stimulating hormone, total immunoglobulin (Ig)A, and anti-tissue transglutaminase antibodies IgA were normal. The patient underwent a contrast-enhanced computed tomography (CT) of the abdomen (Figure A). He also underwent an upper endoscopy (Figure B, C) and colonoscopy (Figure D, E). Biopsies from the antrum of the stomach, colon, and terminal ileum are highlighted in Figures F, G, and H, respectively. Of note, the patient underwent screening colonoscopy at the age of 50, which was normal. What is the diagnosis? See the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.