Pregnancy in a patient with chronic intestinal pseudo-obstruction on long-term parenteral nutrition

Parenteral nutrition support is provided in most instances for short intervals during pregnancy in conditions where oral/enteral intake is severely compromised. Few reports describe the use of parenteral nutrition from conception to delivery. We report the case of a 30-year-old woman suffering from a severe form of chronic intestinal pseudo-obstruction on long-term parenteral nutrition because of malabsorption and malnutrition. Pregnancy and delivery developed uneventfully. The fetus grew normally throughout pregnancy. Our patient needed only slight modifications in her parenteral nutrition regimen during lactation. There were no metabolic complications during pregnancy. We conclude that female patients even with severe forms of gastrointestinal diseases, such as chronic intestinal pseudo-obstruction requiring long-term home parenteral nutrition, can conceive and carry successfully a pregnancy to term.     

Enteral nutrition for severe malnutrition in chronic intestinal pseudo-obstruction

ObjectiveChronic intestinal pseudo-obstruction (CIPO) is a rare intestinal motility disorder. A prolonged avoidance of food due to fear of aggravation of postprandial symptoms leads to severe malnutrition. We report a case of a 21 y old man who was diagnosed as CIPO with a history of recurrent intestinal colic and obstructive symptoms, slow transit type of constipation, bilateral hydronephrosis (non-obstructive), motor dysphagia without any evidence of demonstrable mechanical obstruction. Our aim was to keep his post prandial symptoms to a minimum and nutritionally build him up with enteral nutrition (EN).MethodsHe had life threatening malnutrition (BMI of 11 kg/m²) and significant postprandial distension with an intake more than 100 ml, compromising the quality of life. In view of a normal absorptive function of the gut, TPN was ruled out and the patient was treated with enteral nutrition (oral & tube) only. The EN regimen followed was ad libitum oral intake along with nocturnal NG tube feeding. Initially a full strength semi-elemental formula at 50 ml/hour was given, later shifted to polymeric formula at 100 ml/hour. Serum levels of magnesium, phosphate and potassium were regularly monitored to prevent refeeding syndrome. He ws constantly motivated, counseled and monitored.ResultWith a gradual increase in the intake from 300 Kcal to 1400 Kcal he was discharged. Eight months from discharge he had a weight of 58 kg (BMI = 22.3 kg/m²), with resumption of normal activities and marked improvement in the quality of life.ConclusionCarefully planned EN along with motivation, psychological support and regular monitoring are the keys to nutritional management in CIPO.     

Fluoride exposure and bone status in patients with chronic intestinal failure who are receiving home parenteral nutrition

BACKGROUND AND OBJECTIVE: Metabolic bone disease is frequent in chronic intestinal failure. Because fluoride has a major effect on bones, the status of both fluoride and bone was studied in long-term home parenteral nutrition (HPN) patients. DESIGN: We studied 31 adults aged (x +/- SD) 56.3 +/- 15.1 y, mainly patients with short-bowel syndrome, who had been receiving HPN for >1 y. Bone mineral density (BMD) was measured by absorptiometry, and serum fluoride was measured by using a fluoride-sensitive electrode. All patients ate and drank ad libitum. HPN (3.4 +/- 1.2 times/wk) complemented oral nutrition. Potential explicative factors were estimated by using a linear regression model (mixed-effects model). RESULTS: Of 120 fluoride dosages (2-6/patient), 102 were above the upper normal limit (1.58 micromol/L) at the laboratory. Mean (+/- SD) daily fluoride supply was 8.03 +/- 7.71 mg (US adequate intake: 3.1 mg/d for women and 3.8 for men; tolerable upper normal limit: 10 mg/d); intravenous fluoride varied from 0.06 to 1.45 mg, and oral fluoride varied from 0.09 to 27.8 mg. Serum fluoride concentrations were correlated with creatinine clearance and fluoride supply. BMD was significantly lower in the femoral neck than in the spinal area. After adjustment for sex and the duration of HPN, only the effect of serum fluoride on spinal BMD was significant. Two patients had symptoms of fluorosis, eg, calcaneum fissures, interosseous calcifications, or femoral neck osteoporosis. CONCLUSIONS: In chronic intestinal failure, high intakes of fluoride are frequent because of the beverages ingested to compensate for stool losses. Hyperfluoremia has an effect on bone metabolism and may increase skeletal fragility. The consumption of fluoride-rich beverages for extended periods is therefore not advisable.     

原发性慢性假性肠梗阻

原发性慢性假性肠梗阻是一种临床罕见的肠蠕动功能障碍性疾病,以病人有明确的肠梗阻症状和体征,但无机械性肠梗阻存在为主要特点。其病因不明,可通过营养支持、药物和手术等治疗,但预后不良。现就近年来该病的临床诊治理念作一综述。     

The role of home parenteral nutrition in the management of intestinal failure - report of 400 cases

Between 1977 and January 1991, 400 patients were entered on the United Kingdom Home Parenteral Nutrition (HPN) Register. Almost half suffered from Crohn's disease. Most commenced HPN while between 20-50 years of age. In terms of life quality while on HPN, most (63%) were able to work or look after their families unaided. A further 89 (22%) were able to cope with HPN independently. 221 patients ceased HPN during the study period, mostly (41%) due to intestinal adaptation. Of 69 patients who died, over half (57%) did so due to disease progression. Complications of HPN resulted in death in 22% of this subgroup. In terms of specific disease groups, those with Crohn's disease had the lowest mortality rate (8%).     

Ambulatory home total parenteral nutrition

Ambulatory total parenteral nutrition (TPN) at home was used in 85 patients within a 6-yr period. Indications include severe malabsorption, fistulas, anorexia nervosa, and malignancies. The median duration of home TPN (HPN) was 67 days (range: 30-4,155 days). HPN duration for patients with benign diseases was longer [357.12 days (range: 30-4,155 days)] than for cancer patients [93.54 days (range: 30-421 days)]. Under HPN, patients gained a good nutritional status with an increase of total protein (p less than 0.001) and serum albumin levels (p less than 0.001). Weight gain was also significant (p less than 0.001). The rehospitalization rate was low (7.8%), but it was higher when HPN lasted for more than 3 months (10.87% +/- 1.58%) compared with short-term HPN (5.69% +/- 1.25%). Metabolic complications were unusual, and rehospitalization was related to the oncological treatment and/or infectious complications. Therefore, ambulatory HPN is a nutritional support that can significantly improve the life of patients with alimentary failure. Moreover, HPN allows significant cost savings compared to the alternative of prolonged hospitalization.     

Home parenteral nutrition with full-time home care nurses

A group of patients exists who cannot manage home parenteral nutrition (HPN) due to debilitating conditions and/or lack of family resources. They are limited to either nursing home placement or extended hospitalization unless home nursing care is provided. A 58-year-old single female with malabsorption secondary to scleroderma was sent home on cyclic HPN under the supervision of full-time home care nurses. A comprehensive patient-centered nurse training program was designed to teach nursing personnel from a private nursing service the theory and practice of HPN. Each nurse must achieve at least 80% in the theory posttest and demonstrate the competent performance of central venous catheter care, mixing of parenteral nutrition solutions, use of infusion pump, and application of the heparin lock. The results of the pre- and posttests have shown that the home care nurses have been able to learn the theory necessary for implementing HPN. The mean pretest value was 46% while the mean posttest value was 90% (n = 12, p = 0.01). The patient has been doing very well at home for 5 months in this psychologically, medically, and financially effective program covered by third party payers. To the best of our knowledge, this program has never before been accomplished.     

Patient compliance with a home parenteral nutrition program

A systematic study of patient compliance to home parenteral nutrition (HPN) was done by an independent nurse who visited 10 patients and families at varying periods after HPN had been started. A compliance scale was based on assessment of aseptic technique, sequence of steps followed, safety precautions, proper use of equipment, and knowledge of complications. Compliance percentiles ranged from 73 to 97, with a median of 95. Seven of the 10 patients scored 90% or more in overall compliance. The only category that clearly showed a deficiency was patient knowledge of potential complications of HPN. Factors that correlated positively with a high compliance score were male sex, absence of children in the home, and wives who were enthusiastic about HPN and who offered the most moral support. This study shows that patients with chronic disabling diseases of the small bowel and severe malnutrition can be managed safely at home with HPN if they are properly trained and supervised.     

Successful high-dose calcium treatment of aluminum-induced metabolic bone disease in long-term home parenteral nutrition

A patient who developed severe metabolic bone disease is presented. He had received long-term home parenteral nutrition (HPN) following extensive small bowel resection after mesenteric vein thrombosis. Bone disease caused by aluminum intoxication had components of osteomalacia and low-turnover osteoporosis. Aluminum was detected at the surface of mineralized bone and was elevated in the serum, resulting in a positive deferoxamine infusion test. One year of treatment with high doses of calcium (up to 24 mEq per day) significantly diminished the patient's bone pain, increased the serum levels of calcium, abolished aluminum deposits in the mineralized trabecula, improved bone formation, and increased trabecular bone volume as assessed by repeated histomorphometric analysis.     

Stability issues in home parenteral nutrition

Home parenteral nutrition (HPN) is a method of providing nutrients, fluid and electrolytes intravenously to patients with a malfunctioning gastrointestinal tract, at home. Parenteral nutrition (PN) formulations are checked carefully for stability by the hospital pharmacist before the bags are made. However, there are many differences between supplying PN regimens to in-patients on the ward and to patients at home. Transportation between the hospital and home may be prolonged if the patient lives a long way from the hospital and may not be straight-forward. A number of factors can adversely affect the stability of the PN, which in turn can cause complications such as line blockage; or even life-threatening events, such as pulmonary embolism. The hospital team caring for HPN patients needs to be aware of these stability issues if complications resulting from incompatibilities of the solutions in the bags or the infusion line are to be avoided.     

Preparing patients for home parenteral nutrition

A comprehensive, interdisciplinary program for patients requiring home parenteral nutrition (HPN) is described. This program begins with an initial patient assessment, which includes medical prerequisites and evaluation of patient motivation and learning abilities, family supports, emotional status, and individual life-styles. The teaching phase involves instructing the patient in the tasks necessary to self-administer parenteral nutrition at home. Problems that can arise are presented, and patient self-monitoring for adverse reactions and problem solving is emphasized. Outpatient follow-up is provided by the interdisciplinary Nutrition Support Service in a clinic setting. Specific components of these visits in relation to physical and emotional adjustment to HPN are discussed. The Nutrition Support Service recognizes a long-term commitment to patients receiving HPN, and members of the team are available to assist patients when problems occur.     

Catheter-related sepsis during home parenteral nutrition

Catheter-related sepsis (CRS) in patients receiving home parenteral nutrition (HPN) is the most frequent complication, with an annual incidence rate of 0.30-0.50 in investigative/approved centres. CRS is responsible for patient deaths in 2.5% of cases which represent 50% of HPN-related deaths. A detailed and strictly applied protocol is the basis of prevention; therefore a low rate of CRS can be used as a marker of quality of care. Skin and hub cultures are used to rule out CRS since their predictive negative value assessed against (semi) quantitative cultures of cannulae were reported to be almost 100%. In the absence of catheter removal, clinical assessment plus quantitative blood cultures with a threshold ratio of 4:1 in central and peripheral blood is a reasonable basis of established CRS. Removal of the infected external cannula is necessary and immediate in cases of tunnel infection, virulent bacteria (e.g. Candida, Staph aureus or pseudomonas species) and for all occurrences of CRS on implantable chambers since conventional treatment fails to sterilise such devices. Thus standard antibiotic treatment may be effective in the absence of tunnel sepsis in the majority of CRS cases due to non-virulent bacteria (mainly Staph coagulase negative species). In these cases of CRS, our experience suggests that the antibiotic-lock technique offers definite advantages over conventional systemic antibiotic treatment, since external catheters were sterilised without recurrent sepsis in more than 85% of cases.