while most cases of intestinal malabsorption syndrome are caused by massive intestine resection due to congenital and acquired diseases , a small portion of patients with structurally intact intestines also display intestinal malabsorption syndrome . autoimmune enteropathy ( aie ) , characterized by protracted diarrhea and weight loss , often occurs in infants and young children and can occasionally be observed in adults.1 fewer than 100 adult aie patients have been reported worldwide . mutations in foxp3 , a transcription factor that controls regulatory t - cell development and function , play an important role in its pathogenesis.2 induction of clinical remission has usually required immunosuppressive drugs such as steroids , azothioprine , cyclosporine , and tacrolimus.3,4 some aie patients who do not respond to immunosuppressive therapy can be treated by infliximab.5 interestingly , though malnutrition is common in aie patients , nutrition support has rarely been reported in these special patients . only one paper described total parenteral nutrition ( pn ) in 10 pediatric aie patients . the results showed five of them were weaned off pn , three died of sepsis after a mean period of 18 months ; one underwent total colectomy and one patient is still dependent on tpn for 24 months.6 our paper describes our experience with glutamine - supplemented pn in managing adult patients with aie and its related malnutrition . from april 2006 to january 2012 , four adult patients were identified from ren ji hospital , shanghai jiao tong university school of medicine . all the patients met the criteria for the diagnosis of adult aie : 1 ) adult - onset chronic diarrhea ( > 6 weeks ' duration ) ; 2 ) malabsorption ; 3 ) specific small bowel histology : partial / complete villous blunting , deep crypt lymphocytosis , increased crypt apoptotic bodies , and minimal intraepithelial lymphocytosis ; 4 ) exclusion of other causes of villous atrophy including crohn 's disease , refractory sprue , and intestinal lymphoma ; 5 ) ae and/or ag antibodies . presence of ae and/or ag antibodies is an important diagnostic support , but their absence does not exclude the diagnosis of aie.7 written informed consent was obtained , and the study was approved by the ethics committee of the same hospital . clinical data are obtained at the first admission to our hospital ( table 1 ) . case 2 had undergone enucleation of an ovarian cyst 3 years before the symptoms appeared . the stool routine test was negative without red blood cells , white blood cells , fat drops , and eggs . all immune tests were negative : indirect immunofluorescence antinuclear antibody ( ifana ) , convection immune assay for detection of ena antibody ( cle ) , immunoblot assay for detection of ena polypeptide antibody spectrum ( ibt ) , detection of anticardiolipin antibodies ( acl ) , and double - stranded dna antibodies ( anti - dsdna ) . flow cytometric detection showed that the percentage of cd and cd increased while the percentage of cd and natural killer cells decreased according to the normal range . malnutrition is classified according to bmi and anemia is defined as serum hemoglobin.8,9 severe malnutrition was found in all of the cases . hypocalcemia , hypomagnesemia , and low phosphorus were common in all aie patients ( table 3 ) . a low - calorie ( 15 to 20 kcal / kg / day ) pn was initiated and total calorie increased slowly . it usually took 1 to 2 weeks to achieve the standard goal . a standard pn solution contained a balanced mixture of amino acids , lipids , and glucose in addition to electrolytes , trace elements , and vitamins . the average caloric intake was 20 to 25 kcal / kg / day and the protein amount was 1.2 to 1.5 g / kg / day for the pn prescription . glutamine ( dipeptiven ; sino - swed pharmaceutical ltd . , wuxi , china ) was given to aie patients ( 100 ml / day , every 100 ml containing 20 g ) for 4 weeks followed by a 2-week break , a cycle which was repeated when pn lasted for more than 6 weeks ( fig . 1 ) . then if the frequency of defecation decreased ( to 3 times per day ) and quality improved ( soft and formed stools were achieved ) , when en increased , pn decreased accordingly . if the oral diet met 60% of the daily calorie goal , pn was stopped . each patient was supplemented orally with 250-ml ensure ( 250 kcal ; abbot laboratories ltd . , abbot park , il , usa ) and two probiotics capsules ( a mixture of viable , lyophilized bifidobacterium , lactobacillus acidophilus , and enterococcus faecalis , each capsule containing 0.510 colonies ) three times a day when en started . nutritional indexes did improve significantly in three cases , but not in case 2 ( table 3 ) . pn - associated liver disease ( pnald ) was found in case 2 when she had received pn for 7 weeks.10,11 the fat emulsion was stopped and drugs ( polyene phosphatidylcholine injection and glutathione injection ) were administered to protect liver function . immune medications were applied in two aie patients who did n't response to glutamine - supplemented pn . immune medications were applied in two aie patients who did n't response to glutamine - supplemented pn . clinical data are obtained at the first admission to our hospital ( table 1 ) . case 2 had undergone enucleation of an ovarian cyst 3 years before the symptoms appeared . the stool routine test was negative without red blood cells , white blood cells , fat drops , and eggs . all immune tests were negative : indirect immunofluorescence antinuclear antibody ( ifana ) , convection immune assay for detection of ena antibody ( cle ) , immunoblot assay for detection of ena polypeptide antibody spectrum ( ibt ) , detection of anticardiolipin antibodies ( acl ) , and double - stranded dna antibodies ( anti - dsdna ) . flow cytometric detection showed that the percentage of cd and cd increased while the percentage of cd and natural killer cells decreased according to the normal range . malnutrition is classified according to bmi and anemia is defined as serum hemoglobin.8,9 severe malnutrition was found in all of the cases . hypocalcemia , hypomagnesemia , and low phosphorus were common in all aie patients ( table 3 ) . a low - calorie ( 15 to 20 kcal / kg / day ) pn was initiated and total calorie increased slowly . a standard pn solution contained a balanced mixture of amino acids , lipids , and glucose in addition to electrolytes , trace elements , and vitamins . the average caloric intake was 20 to 25 kcal / kg / day and the protein amount was 1.2 to 1.5 g / kg / day for the pn prescription . wuxi , china ) was given to aie patients ( 100 ml / day , every 100 ml containing 20 g ) for 4 weeks followed by a 2-week break , a cycle which was repeated when pn lasted for more than 6 weeks ( fig . 1 ) . then if the frequency of defecation decreased ( to 3 times per day ) and quality improved ( soft and formed stools were achieved ) , if the oral diet met 60% of the daily calorie goal , pn was stopped . each patient was supplemented orally with 250-ml ensure ( 250 kcal ; abbot laboratories ltd . , abbot park , il , usa ) and two probiotics capsules ( a mixture of viable , lyophilized bifidobacterium , lactobacillus acidophilus , and enterococcus faecalis , each capsule containing 0.510 colonies ) three times a day when en started . nutritional indexes did improve significantly in three cases , but not in case 2 ( table 3 ) . pn - associated liver disease ( pnald ) was found in case 2 when she had received pn for 7 weeks.10,11 the fat emulsion was stopped and drugs ( polyene phosphatidylcholine injection and glutathione injection ) were administered to protect liver function . immune medications were applied in two aie patients who did n't response to glutamine - supplemented pn . immune medications were applied in two aie patients who did n't response to glutamine - supplemented pn . not surprisingly , severe malnutrition , anemia and deficiency of calcium , phosphate and magnesium were found in all aie patients because of intestinal malabsorption . to resolve the nutritional problem , pn is very important for supplying life - essential micronutrients , vitamins and minerals before intestinal function recovers . if the diagnosis of aie is confirmed , pn should begin immediately to avoid deterioration of nutritional status . secondly , the duration of pn in patients with aie ( in our study , at least 15 days ) was longer than that in patients with other conditions . the prevalence of which is 20% to 75% in patients who require long - term pn.12 one measure to prevent pnald is to start pn in a small amount ( 15 to 20 kcal / kg / day ) and to increase the supply slowly to the goal . a second measure is to modify the pn regimen by reduction of calories and the fat emulsion dose.13 another measure is to initiate en.14 the earlier en began , the less likely was pnald to occur . in aie patients , en need not require fasting because the diarrhea does not respond to complete bowel rest.7 the recovery of intestinal function is the key to treating aie . glutamine , fuel for rapid proliferation of intestinal mucosa cells , is reported to be helpful in promoting intestinal rehabilitation . guo et al.15 reported seven pediatric patients with short bowel syndrome ( sbs ) were treated with growth hormone and supplemental glutamine plus en for 3 weeks . wu et al.16 reported that a 3-week treatment including growth hormone plus glutamine resulted in weight gain and a dramatic decrease in stool output in eight adult patients with sbs . our study showed that glutamine - supplemented pn induced clinical remission without steroids in two patients . steroid and azothioprine were required in patients who did n't response to glutamine - supplemented pn.4,5,7 the limitations of our study were the small number of patients and the lack of a control group . another limitation was that probiotics , which are also considered effective in promoting intestinal rehabilitation , were used in the study ; however , their possibly confounding effects were not evaluated.17,18
to evaluate the effects of glutamine - supplemented parenteral nutrition ( pn ) and probiotics in adult autoimmune enteropathy ( aie ) patients . 
 four adult aie patients were identified from april 2006 to january 2012 . 
 clinical and nutritional data were obtained from the patients ' medical records . 
 glutamine - supplemented pn started immediately when the aie diagnosis was confirmed . 
 the total pn duration was 351 days . according to the pn prescription , 
 the average caloric intake ranged from 20 to 25 kcal / kg / day , and the protein intake ranged from 1.2 to 1.5 g / kg / day . 
 alanyl - glutamine ( 20 g / day ) was administered to aie patients for 4 weeks followed by a 2-week break , and this treatment schedule was repeated when pn lasted for more than 6 weeks . 
 body weight gain and an increased serum albumin level were achieved after pn , and defecation frequency and quality also improved . 
 each patient received oral supplements , 250 ml of ensure and two probiotics capsules ( each capsule containing 0.5108 colonies ) three times a day when enteral nutrition started . 
 three aie patients were successfully weaned off pn , and one patient died of pneumonia . 
 glutamine - supplemented pn and probiotics show promise in managing patients with aie and related malnutrition .