Biochemical disturbances, nutritional assessment, and interventions in patients with glycogen storage disease in a Beijing-based general hospital / 中华临床营养杂志

Objective To investigate the biochemical disturbances,nutritional assessment and nutritional interventions in hospitalized patients with glycogen storage disease (GSD) in a general hospital in Beijing and provide examples of advanced nutritional management for these patients.Methods We retrospectively reviewed 22 consecutive hospitalized patients with GSD diagnosed from January 1,2013 to January 1,2016 in Peking Union Medical College Hospital and analyzed their medical data,nutritional assessments,and interventions.Results These 22 GSD patients included 2 cases with GSD-Ⅰa,3 cases with GSD-Ⅰb,and 17 cases with GSD-Ⅲ.They were composed of 21 children (including a 6-month old infant and a 7-month old infant) and one 18-yearold man.Biochemical abnormalities:hypoglycemia (5/5),hyperlactacidemia (4/5),hyperlipidemia (4/5),hyperuricemia (4/5) were common in GSD-Ⅰ patients,while hypoglycemia (17/17),hyperlactacidemia (5/17),hyperlipidemia (7/17),and hyperuricemia (3/17) were also present among patients with GSD-Ⅲ.Growth and development assessments:among 5 patients with GSD-Ⅰ,the body weight was below the 10th percentile in of 4 patients and the height was below the 3rd percentile in 5 patients;among 17 patients with GSD-Ⅲ,the body weight was below the 10th percentile in 4 patients and the height was below the 10th percentile in 9 patients.Nutritional management condition:all the patients received nutritional assessment,dietary modulation and nutritional support.Twenty children or adolescents were prescribed with raw corn starch (RCS) every 4-6 hours,with a mean initial dose of (1.58±0.28) g and (1.21±0.33) g for patients with GSD-Ⅰ and GSD-Ⅲ,respectively;the other 2 infant aged 6-month old and 7-month old were regularly administered amino acid-based enteral nutritional agents.In addition,all the patients with GSD-Ⅲ were recommended high protein intake of 3 g/(kg · d) and received dietary guidance and modulation.Follow-up condition:5 cases with GSD-Ⅰ and 2 cases with GSD-Ⅲ stayed in track after discharge from our hospital.The mean follow-up duration was (480.40±246.16) d and (373.00± 108.89) d for patients with GSD-Ⅰ and GSD-Ⅲ,respectively.All the patients reported alleviated symptom of hypoglycemia,and the examinations also indicated improved fasting blood glucose,uric acid,lipid profile,and growth status.Conelusions Hypoglycemia,metabolic disturbances,and growth retardation are prevalent in both GSD-Ⅰ and GSD-Ⅲ patients.Potential biochemical abnormalities should not be ignored in GSD-Ⅲ patients,especially in young patients.Comprehensive nutrition assessment,regular administration of RCS,sufficient protein intake,and continuous patient follow-up and surveillance can help to alleviate symptoms,correct metabolic disturbances,and improve growth status of patients with GSD.

Novo biomaterial composto de EVA (polietileno-co acetato de vinila) para enxerto "onlay" em tecido ósseo / New biomaterial composed of EVA (polyethylene-co-vinyl acetate) for onlay grafting in bone tissue

Introdução: o EVA (polietileno-co acetato de vinila) é um tipo de espuma, com baixo custo e ampla gama de aplicações. Misturas de EVA e amido proporcionam diferentes tipos de estrutura porosa favorecendo seu uso como scaffold para regeneração tecidual óssea. Objetivo: resultados prévios mostraram reação tecidual favorável ao seu uso como biomaterial, sendo assim, os mesmos foram investigados para fins de regeneração óssea. Método: nesse trabalho, 22 ratos linhagem Wistar foram divididos em dois grupos (4 destinados ao experimento piloto e 18 destinados ao projeto). Primeiramente, 4 animais foram submetidos à cirurgia na calota craniana para en xerto onlay em tecido ósseo dos biomateriais: 1) EVA com amido a 15% espumado em alta pressão com ultrassom (EVAMCU), 2) EVA espumado em alta pressão com ultrassom (EVACU), 3) EVA espumado em alta pressão sem ultrassom (EVASU), 4) EVA com amido a 15% espumado em alta pressão sem ultrassom (EVAMSU). Após 30 dias do pós-operatório, os biomateriais EVACU e EVAMCU apresentaram resultados microscópicos com fibrovascularização favorável e bom desempenho. Em sequência, 18 ratos foram submetidos à cirurgia de enxerto e após 7, 14 e 90 dias, 6 animais foram submetidos à eutanásia para coleta dos biomateriais e tecidos adjacentes da calota craniana. Foi realizada análise qualitativa da região de fibrovascularização, bem como do possível potencial osteogênico da região ao redor dos biomateriais ao longo dos períodos. Resultados e Conclusão: os biomateriais testados demonstraram biocompatibilidade e capacidade para regeneração óssea, no entanto, mais estudos precisam ser realizados, como por exemplo, em defeitos ósseos bicorticais.

Introduction: the EVA (polyethylene-co-vinyl acetate) is a kind of foam with low cost and wide range of applications. EVA and starch mixtures provide different types of porous structure favoring its use as scaffold for bone tissue regeneration. Objective: Previous results showed tissue reaction favorable to its use as biomaterial, and thus, they were investigated for purposes of bone regeneration. Method: in this study, 22 male Wistar rats were divided into two groups, 4 of them for the pilot experiment and 18 for the project. First, 4 animals underwent surgery on the skull cap for onlay graft in bone tissue of the biomaterials: 1) EVA with starch to 15% foamed at high pressure with ultrasound, 2) EVA foam at high pressure with ultrasound, 3) EVA foam for high pressure without ultrasound, 4) EVA with 15% starch foamed at high pressure without ultrasound). The results were evaluated microscopically 30 days after surgery and the biomaterials EVACU and EVAMCU presented good performance with favorable fibrovascularization. Eighteen rats were submitted to graft surgery and after 7, 14 and 90 days, 6 animals were submitted to euthanasia for the collection of biomaterials and adjacent tissues of the skullcap. A qualitative analysis of the region of fibro vascularization was performed, as well as the potential osteogenic based on the microscopic findings of the region surrounding the biomaterials throughout the periods. Results and Conclusion: the biomaterials tested demonstrated biocompatibility and capacity for bone regeneration, however, more studies need to be performed, for example, on bicortical bone defects.

Preparation and characterization of sustained release microcapsules of grape polyphenols with porous cornstarch, alginate sodium, and chitosan / 中草药

Objective: In order to improve the stability of grape polyphenols and strengthen slow-release effect, the study on micro- capisulazed grape polyphenols was carried out through the complex coacervation method using porous cornstarch as core material carrier. Methods: With the embedding rate as main index, the effect of all factors on the microencapsulation of grape polyphenols was investigated through the single factor test and orthogonal test, and its preparation technology was also optimized. Results: The best preparation technology was as follows: The experiment materials were 10 mL grape polyphenols solution of 25 mg/mL, 1.5 g porous cornstarch, 30 mL sodium alginate solution of 0.03 g/mL, 50 mL chitosan solution of 0.01 g/mL, and 50 mL calcium chloride solution of 0.05 g/mL, at pH value of 3.5. The microcapsules' appearance was superior with size distribution of the main in 600-850 μm, the embedding rate was 83.2%, and they had very good releasing property in simulated gastric and simulated intestinal environment. Conclusion: The product appearance and embedding rate of grape polyphenols microcapsules which used porous cornstarch as core material carrier and sodium alginate-chitosan as wall materials are better than those only used sodium alginate and chitosan as wall materials. Furthermore, the inclusion complex is proved to be successfully prepared by its structural characterization which is gotten from FTIR and scanning electron microscope (SEM).

Disintegrant properties of banana starch obtained from the unripe fruits of Musa sapientum L.

Banana starch obtained from unripe fruits of Musa sapientium L. has been evaluated as disintegrant in comparison with official corn starch. The physicochemical and material properties of the starches were evaluated and the properties of paracetamol tablet prepared by wet granulation using the starches as endo-disintegrants were determined. The results indicated that the physicochemical and material properties of banana starch varied considerably from corn starch. Scanning electron microscopy showed that banana starch granules were oval or ellipsoidal in shape while corn starch granules were angular or polyhedral in shape. The crushing strength (CS) of paracetamol tablets increased with disintegrant concentration while friability decreased. Tablets containing banana starch exhibited higher CS but lower friability than those containing corn starch. Paracetamol tablets containing banana starch had longer disintegration times than those containing corn starch although there were no significant (p >0.05) differences in the disintegration time of tablets. The results showed that banana starch compared well with corn starch as disintegrant.

Agro-industrial residues and starch for growth and co-production of polyhydroxyalkanoate copolymer and -amylase by Bacillus sp. CFR-67

Polyhydroxyalkanoates (PHA) and -amylase (-1,4 glucan-4-glucanohydrolase, E.C. 3.2.1.1) were co-produced by Bacillus sp. CFR-67 using unhydrolysed corn starch as a substrate. Bacterial growth and polymer production were enhanced with the supplementation of hydrolysates of wheat bran (WBH) or rice bran (RBH) individually or in combination (5-20 g L-1, based on weight of soluble substrates-SS). In batch cultivation, a mixture of WBH and RBH (1:1, 10 g L-1 of SS) along with ammonium acetate (1.75 g L-1) and corn starch (30 g L-1) produced maximum quantity of biomass (10 g L-1) and PHA (5.9 g L-1). The polymer thus produced was a copolymer of polyhydroxybutyrate-co-hydroxyvalerate of 95:5 to 90:10 mol%. Presence of WBH and corn starch (10-50 g L-1) in the medium enhanced fermentative yield of -amylase (2-40 U mL-1 min-1). The enzyme was active in a wide range of pH (4-9) and temperature (40-60ºC). This is the first report on simultaneous production of copolymer of bacterial PHA and -amylase from unhydrolysed corn starch and agro-industrial residues as substrates.

Agro-industrial residues and starch for growth and co-production of Polyhydroxyalkanoate copolymer and alfa-amylase by Bacillus. SP CFR67îen

Polyhydroxyalkanoates (PHA) and α-amylase (α-1,4 glucan-4-glucanohydrolase, E.C. 3.2.1.1) were co-produced by Bacillus sp. CFR-67 using unhydrolysed corn starch as a substrate. Bacterial growth and polymer production were enhanced with the supplementation of hydrolysates of wheat bran (WBH) or rice bran (RBH) individually or in combination (5-20 g L-1, based on weight of soluble substrates-SS). In batch cultivation, a mixture of WBH and RBH (1:1, 10 g L-1 of SS) along with ammonium acetate (1.75 g L-1) and corn starch (30 g L-1) produced maximum quantity of biomass (10 g L-1) and PHA (5.9 g L-1). The polymer thus produced was a copolymer of polyhydroxybutyrate-co-hydroxyvalerate of 95:5 to 90:10 mol%. Presence of WBH and corn starch (10-50 g L-1) in the medium enhanced fermentative yield of α-amylase (2-40 U mL-1 min-1). The enzyme was active in a wide range of pH (4-9) and temperature (40-60ºC). This is the first report on simultaneous production of copolymer of bacterial PHA and α-amylase from unhydrolysed corn starch and agro-industrial residues as substrates.

Effect of dietary fiber on carbohydrate metabolism in healthy volunteers with 13C breath test / 中华临床营养杂志

Objective To investigate the effect of dietary fiber on carbohydrate metabolism of healthy volunteers after the intake of corn starch meal. Methods Totally 12 healthy volunteers aged (25. 8 ± 5. 3) years were enrolled in this study, and they were equally randomly divided into two groups (group A and group B). This was an open, randomized, cross-over, and two-period study, and each period lasted for one day. In period 1, the subjects in group A received fiber-free corn starch and group B received high-fiber corn starch (containing 16 g dietary fiber). In period 2, the two groups are crossed. There was a one-week wash-out time between the two study days. On the study day, breath samples of fasting and 0. 5, 1.0, 1.5, 2.0, 2.5, 3.0, 3.5, 4.0, 5.0, 6.0,7. 0, 8. 0 hours post meal were collected to measure13 CO2. Results The delta over baseline at 0. 5, 1.5, 2. 0,2. 5, 3. 0, 4. 0 hour after test meal in fiber free group and in high fiber group were 0. 79, 2. 03, 2. 57, 2. 86,3. 02, 3. 18 and 0. 16, 1. 33, 1.77, 2. 10, 2. 34, 2.42, respectively (the P value was 0. 014, 0. 014, 0. 011,0.018, 0. 036, and 0.020, respectively). Peak concentration of delta over baseline of fiber free group and high fiber group was 3.18 and 2. 56 respectively, there was no significant difference between two groups (P > 0. 05) ,peak time of the group at 4. 0 hour and 3. 5 hour respectively, showed significant difference (P = 0. 032). The cumulative percentage dose recovered 0. 5-6. 0 hours after test meal in fiber-free group and in high-fiber group were 0.41, 1.46, 3.15, 5.50, 8.28, 11.30, 14.42, 17. 62, 23. 65, 28. 78 and 0. 09, 0. 55, 1.61, 3.22,5.23,7.53, 10.09, 12.68, 17.60, 22.27 respectively (the P value was 0.014, 0.018, 0.018, 0.014, 0.013,0.014, 0.018, 0.020, 0.025, and 0.044, respectively). However, there was no significant difference 6.0 hours after meal (P > 0. 05 ). Conclusion The dietary fiber used in this study can delay the absorption of carbohydrate 6. 0 hours within intake without influencing its total absorption amount.

The Effect of Silk Fibroin/Nano-hydroxyapatite/Corn Starch Composite Porous Scaffold on Bone Regeneration in the Rabbit Calvarial Defect Model

0.05).CONCLUSION: The rabbit calvarial defect was not successfully repaired by silk fibroin/nano-hydroxyapatite/corn starch composite scaffold and may have been due to an inflammatory reaction caused by silk powder. In the future, the development of composite bone graft material based on various components should be performed with caution.

Studies of conformational changes, crystalline and granular structures, and in vitro digestibility of cross-linked and methylated corn starches / Estudios de los cambios conformacionales, estructura granular y cristalina, y digestibilidad in vitro de almidones de maíz entrecruzados y metilados / Estudos das mudanças conformacionais, estrutura granular e cristalina, e digestibilidade in vitro de amidos de milho entrecruzados e metilados

Starch granular ultrastructure is an important determinant of its functional properties. Its knowledge, control and application should help to produce a wide range of food products. The goal of the study was to measure the modifications produced by cross-linking and methylation on the conformation and the granular and crystalline structures of corn starch, as well as the effects on the in vitro enzymatic digestibility of starch by a-amylase. Cross-linked and methylated starches were produced from commercial corn starch by low degree substitution (DS) methods. Both native and modified starches were analyzed employing NMR, SEM and X-ray diffraction. The morphologic characteristics, crystalline structure and susceptibility to hydrolysis by a-amylase were dramatically affected by the methylation process, while cross-linked corn starch showed non-significant variations that did not change the type A pattern of the native one. However, the 13C CP-MAS NMR spectrum of the cross-linked starch is suggesting a change from a type A to a type B crystalline pattern, while the X-ray diffraction pattern of the methylated starch was completely different from those reported in the literature for any starches. No changes in granular shape were observed (SEM) in the cross-linked starch, while the methylated starch showed larger chunks without granular integrity and with a rough surface due to exo-erosion. It can be presumed that, due to the presence of methyl groups inside the granules, the enzyme-substrate interaction is hindered by steric effects.

La ultraestructura granular del almidón es un determinante importante de sus propiedades funcionales. Su conocimiento, control y aplicación ayudaría en la producción de una amplia gama de productos alimenticios. El objetivo de este estudio fue medir las modificaciones producidas por el entrecruzamiento y la metilación en la conformación y en las estructuras granular y cristalina del almidón de maíz, así como sus efectos en la digestibilidad enzimática in vitro por la a-amilasa. Almidones entrecruzados y metilados fueron producidos a partir de almidón de maíz comercial por métodos de bajo grado de sustitución. Tanto el almidón nativo como los modificados fueron analizados empleando RMN, MEB y difracción de rayos X. Las características morfológicas, la estructura cristalina y la susceptibilidad a hidrólisis fueron dramáticamente afectadas por el proceso de metilación, mientras que el almidón entrecruzado mostró cambios no significativos que no modificaron el patrón tipo A del almidón nativo. No obstante, el espectro de 13C CP-MAS RMN del almidón entrecruzado sugiere un cambio en su patrón cristalino del tipo A al B, mientras que el patrón de difracción de rayos X del almidón metilado fue completamente diferente de aquellos reportados para almidones en la literatura. No se apreciaron cambios (MEB) en la forma de los gránulos del almidón entrecruzado, mientras que el metilado mostró gránulos más grandes sin integridad y con superficie rugosa debida a exo-erosión. Es posible asumir que, dada la presencia de grupos metilo dentro de los gránulos, la interacción sustrato-enzima es obstaculizada por efectos estéricos.

A ultra-estrutura granular do amido é uma determinante importante de suas propriedades funcionais. Seu conhecimento, controle e aplicação ajudariam na produção de uma ampla gama de produtos alimentícios. O objetivo deste estudo foi medir as modificações produzidas pelo entrecruzamento e a metilação na conformação e nas estruturas, granular e cristalina, do amido de milho, assim como seus efeitos na digestibilidade enzimática in vitro pela a-amilasa. Amidos entrecruzados e metilados foram produzidos a partir de amido de milho comercial por métodos de baixo grau de substituição. Tanto o amido nativo como os modificados foram analisados empregando RMN, MEB e difração de raios X. As características morfológicas, a estrutura cristalina e a susceptibilidade a hidrólise foram dramaticamente afetadas pelo processo de metilação, enquanto que o amido entrecruzado mostrou mudanças não significativas que não modificaram o padrão tipo A do amido nativo. No entanto, o espectro de 13C CP-MAS RMN do amido entrecruzado sugerindo uma mudança de seu padrão cristalino do tipo A ao B, enquanto que o padrão de difração de raios X do amido metilado foi completamente diferente daqueles relatados para amidos na literatura. Não se apreciaram mudanças (MEB) na forma dos grânulos do amido entrecruzado, enquanto que o metilado mostrou grânulos maiores sem integridade e com superfície rugosa devido à exo-erosão. É possível assumir que, devido à presença de grupos metilo dentro dos grânulos, a interação substrato-enzima é obstaculizada por efeitos estéricos.

Production and characterization of glucoamylase from fungus Aspergillus awamori expressed in yeast Saccharomyces cerevisiae using different carbon sources

Glucoamylase is widely used in the food industry to produce high glucose syrup, and also in fermentation processes for production beer and ethanol. In this work the productivity of the glucoamylase of Aspergillus awamori expressed by the yeast Saccharomyces cerevisiae, produced in submerged fermentation using different starches, was evaluated and characterized physico-chemically. The enzyme presented high specific activity, 13.8 U/mgprotein or 2.9 U/mgbiomass, after 48 h of fermentation using soluble starch as substrate. Glucoamylase presented optimum activity at temperature of 55ºC, and, in the substratum absence, the thermostability was for 1h at 50ºC. The optimum pH of activity was pH 3.5 - 4.0 and the pH stability between 5.0 and 7.0. The half life at 65ºC was at 30.2 min, and the thermal energy of denaturation was 234.3 KJ mol-1. The hydrolysis of different substrate showed the enzyme's preference for the substrate with a larger polymerization degree. The gelatinized corn starch was the substratum most susceptible to the enzymatic action.

A glucoamilase é amplamente utilizada na indústria de alimentos no processamento do amido para a produção de xarope com alto teor de glicose e também muito empregada nos processos de fermentação para produção de cerveja e etanol. Neste trabalho a glucoamilase de Aspergillus awamori expressa em Saccharomyces cerevisiae produzida sob fermentação líquida foi avaliada quanto à produtividade em diferentes amidos e caracterizada físico-quimicamente. A enzima apresentou alta atividade específica de 13,8 U/mg proteína e de 2,9 U/mg biomassa ao final de 48 h de fermentação em meio contendo amido solúvel. A glucoamilase apresentou temperatura ótima de atividade a 55ºC, e temperatura de desnaturação térmica na ausência de substrato por 1h a 50ºC. O pH ótimo de atividade foi na faixa de 3,5 - 4,0 e a estabilidade ao pH entre os valores 5,0 e 7,0. A meia vida a 65ºC foi 30,2 min., e a energia de desnaturação foi de 234.3 KJ mol-1. A hidrólise em diferentes substratos mostrou a preferência da enzima pelos substratos com maior grau de polimerização, sendo o amido de milho gelatinizado o substrato preferencial à ação enzimática.

Clinical Manifestation and Effect of Corn Starch on Height Growth in Korean Patients with Glycogen Storage Disease Type Ia / 대한소아내분비학회지

PURPOSE: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disease characterized by hepatosplenomegaly, short stature, hypoglycemia, hyperuricemia and lactic academia. It is caused by mutations of glucose-6-phosphatase (G6Pase) gene located on chromosome 17q21. The study were undertaken to investigate clinical manifestations and genotype as well as to evaluate the effects of uncooked corn starch (UCCS) on height growth of pubertal and prepubertal subjects with GSD Ia. METHODS: We analyzed clinical data from 24 GSD Ia patients retrospectively by medical record review. Height standard deviation score (Ht-SDS) was calculated from 13 GSD Ia patients under age 15 treated with UCCS and followed-up over 1 year. DNA isolation, PCR reaction and DNA sequencing analysis were performed in all studied patients. RESULTS: Hypertriglyceridemia (100%), elevated liver enzyme (85%), hyperuricemia (48%), hypercholesterolemia (45%), anemia (45%) were major laboratory findings in studied population. Four different mutations of G6Pase gene in 48 alleles were identified. C.648G>T mutation was the predominant mutation, allele frequency of which was 78.6% (33 alleles). The other mutations were p.Phe51Ser, p.Gly222Arg, p.Gly122Asp. The p.Phe51Ser was a novel mutation. Mean Ht-SDS at diagnosis and two years after UCCS treatment were -2.04+/-1.69 and -0.72+/-1.12 respectively, which were statistically significant (P=0.036). CONCLUSION: The genotype of the G6Pase gene was nearly homogeneous in Korean patients with GSD Ia. Molecular analysis of the G6Pase gene will be the diagnosis of choice since the c.648G>T mutation accounts for 78.6% of mutations in Korean patients with GSD Ia. UCCS treatment has a beneficial effect on height growth of children and adolescents with GSD Ia.

Dumping Syndrome in a Child with Gastrojejunal Tube Feeding / 대한소아소화기영양학회지

Dumping syndrome is a known complication of gastric surgery in adults, but a very rare disease in the pediatric population. We report on a case of dumping syndrome in a 19-month-old child, who underwent gastrojejunal feeding tube insertion for the treatment and prevention of gastroesophageal reflux and frequent aspiration pneumonia. At 17 months of age, 2 months after the beginning of gastrojejunal tube feeding, postprandial diaphoresis, palpitation, lethargy, bloating, and diarrhea occurred, and a single episode of convulsion with hypoglycemia were noted. Early and late dumping syndrome was confirmed by an abnormal oral glucose tolerance test with early onset hyperglycemia followed by delayed onset hypoglycemia. Diet therapy including uncooked corn starch then improved the postprandial diaphoresis, abnormal glucose levels, and her nutritional status. We conclude that dumping syndrome may be considered as a complication of gastrojejunal tube feeding in a child.