Biomedical subjects
C R Fleming
Publications and source records attributed to C R Fleming.
Home parenteral nutrition as primary therapy in patients with extensive Crohn's disease of the small bowel and malnutrition.
UNLABELLED: Seven patients with extensive Crohn's disease have received nightly supplementary home parenteral nutrition (HPN) for a total of 120 patient months. Indications for HPN were short bowel in 5, growth failure in 1, and gastric outlet obstruction in 1. Before HPN, body weight averaged 72% of ideal body weight in 5 patients; 1 adolescent was less than the third percentile in weight. Anthropometry in 6 patients confirmed decrease in fat and lean body mass in all. All patients were anemic and serum albumin was low in 6 of 7 (1.8 to 3.0 g per dl). During HPN average weight gain was 11 kg in the 6 patients treated for 6 months or longer. Hemoglobin increased an average of 1.5 g per dl and serum albumin increased an average of 1.0 g per dl. There were 50% fewer hospitalizations after HPN was begun compared with similar time periods before HPN. HPN did not prevent or reverse complications of Crohn's disease other than malnutrition. Major complications were displacement of the catheter on two occasions in 1 patient and metabolic acidosis in 2 patients. Sepsis did not occur. CONCLUSION: HPN is justified in selected patients with extensive Crohn's disease and malnutrition in that patients gain weight, nutritional parameters improve, and patients' activities increase as number of hospitalizations decrease.
Effect of D-penicillamine on copper retention in patients with primary billiary cirrhosis.
As part of a double blind, randomized trial evaluating D-penicillamine in primary biliary cirrhosis, we monitored urinary copper excretion and hepatic copper concentration during the 1st year of therapy in 46 patients with this disease. The retention of copper in primary biliary cirrhosis was confirmed by finding abnormally high levels of standard copper measurements in almost all patients before treatment. The hepatic copper was elevated in 43 of 45 patients, the urinary copper in 42 of 46, and the ceruloplasmin in 46 of 46. Urinary copper excretion correlated with the hepatic copper concentration (r = 0.68, P less than or equal to 0.001). No significant correlation occurred between hepatic copper and ceruloplasmin. Hepatic copper concentrations greater than 400 microng per g of dry weight were found almost exclusively in patients with advanced histological disease (P less than or equal to 0.01). Therapy with D-penicillamine and a low copper diet sustained increased urinary copper excretion for 1 year in almost all patients (P less than or equal to 0.001). Among patients taking placebo, the median hepatic copper concentration increased 13 microng per g of dry weight after 1 year. In contrast, among the patients taking D-penicillamine, the median hepatic copper concentration decreased 99 microng per g of dry weight (P less than or equal to 0.02). Continued observation of this therapeutic trial may help to clarify the relationship of copper retention and liver injury in primary biliary cirrhosis.
Splenosis: autotransplantation of splenic tissue.
Splenosis is the autotransplantation of splenic tissue that usually follows traumatic rupture of the spleen. Generally, the splenic implants are numerous and are located within the peritoneal cavity; however, extra-abdominal splenosis does occur. The implants are rarely clinically significant and are incidental findings at autopsy or at abdominal operation, unrelated to and distant from the splenic trauma and splenectomy. Splenic implants retain their ability to function, and recurrence of a hematologic disease for which the spleen was previously removed should alter the clinician to the possibility of splenosis. The differential diagnosis includes accessory spleens, endometriosis, hemangiomas and metastatic cancer. We report three cases, two of which represent unusual complications of splenosis: a twisted pedicle of a splenic implant, which apparently caused abdominal pain, and recurrent Felty's syndrome associated with splenosis and an accessory spleen.
A prospective study of serum copper and zinc levels in patients receiving total parenteral nutrition.
Weekly determinations of serum copper (Cu) and zinc (Zn) were made in eight adult patients receiving total parenteral nutrition (TPN) for 3 to 13 weeks. Serum Cu decreased in all eight patients. Five of eight patients had hypocupremia lasting at least 2 consecutive weeks and three of the five had Cu levels of 30 mug/dl or lower. Low levels of serum ceruloplasmin provided supportive evidence of Cu deficiency in the three patients with the lowest Cu levels. Two patients who had Cu less than or equal to 20 mug/dl demonstrated declines in hemoglobin which were probably due to Cu deficiency. The mean rate of decline in serum Cu was 10.8 mug/dl/week. After resumption of oral feedings in five patients, the mean rate of increase in Cu was 14 mug/dl/week. The sharpest rise in Cu was seen during the 2nd week after oral feedings were resumed in four of the five patients. Three of eight patients had serum Zn levels less than 70 mug/dl for at least 2 consecutive weeks. Serum Zn decreased at a mean rate of 6.6 mug/dl/week. There was a further decline in serum Zn in three of five patients in whom measurements were made after resumption of oral intake. Concentrations of Zn in TPN solutions varied between 0.63 and 1.0 mg/liter. Cu was undetectable in TPN solutions.
Essential fatty acid deficiency in adults receiving total parenteral nutrition.
In seven adult patients receiving fat-free total parenteral nutrition (TPN) for 4 to 8 weeks, weekly determinations of plasma fatty acids and total plasma tocopherols were made. Four patients were deficient in essential fatty acids, as defined by triene: tetraene ratio greater than 0.4, at the end of the second week of TPN. Six patients were deficient by the end of the third week and all seven were deficient by the end of the fifth week of TPN treatment. One patient who was deficient in both essential fatty acids and zinc developed a scaling, eczemoid dermatitis that disappeared within 3 weeks after cessation of TPN and resumption of oral feedings containing both fat and zinc. After resumption of oral feedings by three patients, the triene: tetraene ratio returned to normal within 2 weeks. The mean of total plasma tocopherols fell over a period of 7 weeks and in three individuals, reached levels generally associated with deficiency. There were not any obvious clinical manifestations of vitamin E deficiency.
Total parenteral nutrition.
Total parenteral nutrition (TPN) is a relatively new innovation in patient care which allows us to replace and maintain essential nutrients in patients in whom oral or tube feedings are contraindicated or inadequate. Insertion of a catheter into a large central vein permits one to concentrate hypertonic dextrose calories in normal daily fluid requirements. In addition, TPN solutions contain synthetic amino acids or protein hydrolysates, macroelements, electrolytes, and vitamins. Indications for TPN include intestinal fistulas, severe short bowel syndrome, unresolving pancreatitis, advanced inflammatory bowel disease, delayed postoperative gastrointestinal function, developmental anomalies of the intestinal tract, protracted diarrhea of infancy, and hypermetabolic states. Complications encountered in patients receiving TPN are catheter-related mechanical problems, infections, and metabolic abnormalities. In select patients, who otherwise would require repeated hospitalizations for malnutrition, encouraging results have been achieved by the use of TPN in the home.
Essential fatty acid, copper, zinc and tocopherol deficiencies in total parenteral nutrition.
Explore the source record for details and available documents.
Pigmented corneal rings in a patient with primary biliary cirrhosis.
A patient with primary biliary cirrhosis who had high hepatic, serum, and urine copper levels was found by slit-lamp biomicroscopy to have pigmented corneal rings similar to Kayser-Fleischer rings. Wilson's disease was excluded on the basis of the elevated serum ceruloplasmin and copper levels, the lack of neurological disease despite advanced liver disease, a negative family history, and the typical clinical features of primary biliary cirrhosis. Although the exact chemical nature of the corneal rings is unknown, the cornea may be another tissue associated with elevated tissue copper levels in primary biliary cirrhosis.
Intraluminal duodenal diverticulum. Report of two cases and review of the literature.
Diverticula rarely occur within the lumen of the duodenum. They arise near the papilla of Vater and extend distally. The diverticulum is lined on both sides with duodenal mucosa, and its eccentric opening is usually proximal in the sac. The diverticulum results from incomplete recanalization of the intestinal lumen after the proliferative epithelial stage in the 7-week embryo and represents either a remnant of one of two channels formed during recanalization or a distal ballooning of a congenital duodenal diaphragm. Although the patient with a diverticulum may be asymptomatic, most patients present with abdominal pain and obstructive symptoms; pancreatitis and gastrointestinal bleeding may be associated with the diverticulum. Barium study shows a characteristic radiolucent halo that represents the wall of the diverticulum. Surgery should be approached with caution because injury to the papilla is a hazard, and in children, coexisting congenital anomalies may be present.
Copper and primary biliary cirrhosis.
Explore the source record for details and available documents.
Home parenteral nutrition in chronic intestinal diseases: its effect on growth and development.
Nine children and adolescents with chronic intestinal diseases and growth failure were treated with home parenteral nutrition (HPN) for 7-63 months. All patients improved their growth velocities on HPN, with an average height gain of 9 cm. The mean growth velocity in six patients with Crohn's disease increased more than fivefold (from 0.67 +/- 0.4 cm/year to 3.8 +/- 1.09 cm/year), and they gained a mean of 11.4 +/- 3.4 kg/year. The three patients without Crohn's disease [intestinal pseudo-obstruction (two), severe short bowel (one)] increased growth velocity from an average of 2.9 +/- 1.1 cm to 6.1 +/- 1.34 cm/year and gained a mean of 10 +/- 2.84 kg/year. Patients without Crohn's disease grew faster on HPN than age comparable patients with Crohn's disease. Also, the two patients with Crohn's disease who were younger and had their disease for a shorter period (less than 3 years) grew better than older patients with Crohn's disease who had their disease for a mean of 8 +/- 1.3 years. All patients improved clinically, and three with Crohn's disease achieved complete remission. Five of the nine patients had psychosocial adjustment problems while on HPN. In conclusion, HPN is a relatively safe and effective means to stimulate growth in children and adolescents who cannot meet nutritional needs by the enteral route.
Hepatobiliary complications in adults receiving nutrition support.
Hepatobiliary dysfunction in patients receiving nutrition support is frequent. Other reasons for elevated enzyme levels including drugs, recent anesthesia and surgery or sepsis often coexist. Liver test abnormalities in adults are usually milder than in children and frequently self-limited and are 10 times more likely to occur with total parenteral nutrition (TPN) than tube enteral nutrition. Patients on short-term TPN usually have mild-to-moderate elevations in transaminase and alkaline phosphatase levels and steatosis or portal triaditis on biopsy. Patients who are infected while on TPN are at greater risk of developing steatosis and intrahepatic cholestasis. Strategies to correct abnormalities include alteration of the caloric mix in the TPN, cyclic infusions, metronidazole, enteral nutrition and inclusion of L-glutamine in the TPN formula. Patients on long-term home parenteral nutrition may develop persistent elevations in liver tests and steatohepatitis. Both acalculus and calculus cholecystitis occur with increased frequency in patients on long-term TPN. Biliary sludge precedes calcium bilirubinate stones: predisclosing factors include nil per os, prior ileal resection and use of narcotics or anticholinergics.
Gastrointestinal motility considerations in patients with short-bowel syndrome.
Short-bowel syndrome results from large resections of the small intestine that result in the malabsorption of nutrients and fluids. Following intestinal resection both morphological and functional adaptations of the residual intestine occur. While we have witnessed progress in the understanding of morphological adaptation, little is known about the effects of gastrointestinal motility in short-bowel syndrome. This article reviews what is currently known about gastrointestinal motility in the context of short-bowel syndrome and the motility considerations that impact on clinical management.
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.
Selenium status in patients receiving home parenteral nutrition.
Selenium (Se) status was evaluated in patients with intestinal failure requiring home parenteral nutrition (HPN). Ninety-two percent of patients (11 of 12) studied just prior to starting HPN had low serum Se values, and the mean value was 42 ng/ml, significantly less than mean values in disease controls with Crohn's disease not on HPN (76 ng/ml) and healthy controls (88 ng/ml). Eighty-five percent of patients (22 of 26) already on HPN for 2 to 109 months when studied had low serum Se levels (mean 38.4 ng/ml). The mean 24-hr urinary Se values were 3.7 micrograms in patients on HPN who did not have Crohn's disease, 10.9 micrograms in HPN patients with Crohn's, and 17.9 micrograms in healthy controls. In patients with Crohn's disease on HPN, a significant direct correlation existed between serum Se and the activity of whole blood glutathione peroxidase, a selenoprotein ; and a significant inverse correlation was found between serum Se and months of HPN. This study confirms that Se deficiency is very common in patients before starting and during HPN. These data and recent reports of cardiomyopathies associated with Se deficiencies in patients on HPN increase the importance of proper Se replacement and maintenance.
Does long-term home parenteral nutrition in adult patients cause chronic liver disease?
Sixty patients with gut failure were treated with home parenteral nutrition for 2000 patient months. Fifty-one of these 60 patients had either no abnormalities or mild and transient elevations of their liver chemistries and did not have liver biopsies. Nine (15%) of 60 patients had abnormalities of liver tests that persisted from 8 to 95 months (median, 18 months) which prompted one or more liver biopsies per patient. Three patients had prolonged jaundice, one died of hepatic encephalopathy, and another with protracted intrahepatic cholestasis died following a biliary tract exploration. A third patient remains ill with signs and symptoms of chronic liver disease. Steatohepatitis was found in eight of the nine patients and was characterized by centrilobular and midzonal microvesicular and macrovesicular fatty changes with fat cysts, focal necrosis, and mixed inflammatory infiltrates. Centrilobular fibrosis was present in three patients and evidence of nodular regeneration in one. In the three patients demonstrating cholestasis, bile pigment was identified both in hepatocytes and canaliculi. Ceroid pigment in Kupffer cells was a consistent finding and much more severe than expected from the mildness of the hepatitis. Persistent abnormalities of liver chemistries in nine patients and progressive liver disease while receiving home parenteral nutrition in three patients are quite worrisome and suggest that home parenteral nutrition-associated steatohepatitis with or without cholestasis may progress to chronic liver disease.
Analytical assessment of Broviac catheter occlusion.
Eight of 92 consecutive silastic central venous catheters used for home parenteral nutrition occluded. Six of the eight had patency restored by the instillation of urokinase or streptokinase into the catheter. The thrombus in one of the two catheters that was not reopened with thrombolytic agents was studied in detail by electron microscopy, x-ray dispersive analysis, solubility in isopropyl alcohol-diethyl ether (1:1, v:v), and thin-layer chromatography of extracted lipids. Electron microscopy found the clot to be an amorphous mass without features to suggest crystalline properties. The x-ray dispersive analysis showed that the only elements which were significantly increased were chloride and silicon and the silicon detected was likely from the underlying catheter. Treatment with isopropyl alcohol-diethyl ether left an insoluble, flaky residue that resembled protein from a thrombus. Thin-layer chromatography detected a lipid profile suggestive of circulating endogenous fat instead of the fat that was infused through the catheter.