Glycogen storage disease IXa in a 9-year-old Filipino boy with short stature: A case report

@#Glycogen storage disease (GSD) type IXa, due to a deficiency of hepatic phosphorylase b kinase, results in liver enlargement, growth retardation and fasting ketosis. Many are asymptomatic and do not require treatment. This is the first documented GSD IXa in a Filipino boy evaluated for short stature.

Películas comestibles a base de almidón nanoestructurado como material de barrera a la humedad / Edible films based on nanostructured starch as barrier material moisture

RESUMEN Los materiales de empaque proporcionan protección física y crean las condiciones fisicoquímicas apropiadas para proporcionar una vida útil adecuada. Recientemente, la industria alimentaria ha propuesto incorporar nanocompuestos a películas comestibles que se degraden en un periodo corto, sin causar problemas medioambientales. El objetivo de esta investigación fue desarrollar una película comestible resistente a la humedad, utilizando almidón nanoestructurado, que pueda servir de empaque para aumentar la vida útil de los alimentos, sin afectar el medio ambiente. Los efectos del almidón nanoestructurado sobre las propiedades físicas y estructurales de una película comestible fueron estudiados en términos de espesor, solubilidad en agua, difusión, permeabilidad al vapor de agua (PVA), velocidad de transmisión de vapor de agua (VTVA) y comparados a las películas de almidón nativo. Los resultados mostraron que las películas comestibles formuladas con almidón nanoestructurado presentaron menor espesor, comparadas con las elaboradas con almidón nativo, además, los valores de la solubilidad en agua, el coeficiente de difusión, PVA y VTVA fueron menores para las películas nanoestructuradas, con respecto a las de almidón nativo. La nanoestructuración del almidón de maíz permitió obtener películas comestibles con excelentes propiedades de barrera a la humedad, sin modificar las propiedades estructurales de la matriz del polímero, lo que podría constituir una alternativa para el empaque de alimentos.

ABSTRACT The packaging materials provide physical protection and create the appropriate physicochemical conditions to give an adequate shelf life. Recently, the food industry has proposed to incorporate nanocomposites into edible films that degrade in a short period of time without causing environmental problems. The objective of this research was to develop an edible film using nanostructured starch, which can serve as a packaging, resistant to moisture, stable that can extend the shelf life of food and additionally environmental benefits. The effects of nanostructured starch on the physical and structural properties of an edible film were studied in terms of thickness, water solubility, diffusion, water vapor permeability (WVP) and water vapor transmission rate (WVTR). The results showed that the edible films formulated with nanostructured starch had the lowest thickness. Furthermore, the solubility in water, the diffusion coefficient, WVP and WVTR were lower for these films. The nanostructuring of corn starch made it possible to obtain edible films with excellent water barrier properties without modifying the structural properties of the polymer matrix, which could constitute an alternative for food packaging.

New perspectives of glycogen storage disease / 国际儿科学杂志

Glycogen storage diseases (GSD) are a group of inherited disorders characterized by enzyme defects that affect the glycogen metabolism. Hypoglycemia, hepatomegaly, splenomegaly and growth retardation are the main clinical manifestations. The enzymes affecting glycogen synthesis and degradation are varied.Consequently, the clinical manifestations are different because of different enzyme defection. Most patients treated regularly can obtain the normal growth and grow to adults, but still can be accompanied long-term complications by adulthood. Now some scientists find new modified cornstarch is superior to standard therapy in maintaining blood glucose levels. Gene therapy is still in the experimental animals.

Effect of Dietary Intake of Ultra-fine or Nano-Scale Pulverized Cornstarch on the Growing Performance and Gut Function in Rats

The objectives of this study was to determine whether a new physically modified cornstarch by ultra-fine- or nanoscale pulverizer to reduce particle size offers better bioactive function than native cornstarch in weanling Sprague-Dawley rats. Male weaning Sprague-Dawley rats were fed diets containing native cornstarch (NAC), ultra fine pulverized cornstarch (UFC) or nano-scale pulverized cornstarch (NSC) for 4 weeks. In vitro rate of starch hydrolysis, growth performance, organ weight, intestine length intestinal proliferation and the fermentation by Bifidobacterium of rat cecum were evaluated. The diet with reduced particle size (UFC or NSC) significantly increased body weight gain and organ weight. Feed efficiency was increased in NSC fed rats and was not affected in UFC fed rats. Intestinal proliferation was decreased in NSC group. Reduction of particle size also increased cecal short chain fatty acid concentration and the growth and acidifying activity of Bifidobacterium. It is concluded that a reduction of particle size of starch granules by physically modification may increase growing performance and gut function.

Peritonitis granulomatosa por el almidón en guantes quirúrgicos / Granulomatous peritonitis due to the starch used in surgical gloves

La peritonitis granulomatosa por almidón de guantes quirúrgicos es una entidad de difícil diagnóstico y escasa incidencia. Los pacientes presentan dolor abdominal, fiebre e íleo funcional prolongado tres a cuatro semanas después de una laparotomía con empleo de guantes lubricados con almidón de maíz. En la exploración quirúrgica se evidencia peritoneo y epiplón con múltiples nódulos pequeños blanquecinos y líquido libre inflamatorio. La anatomía patológica muestra tejido inflamatorio con células gigantes multinucleadas englobando material birrefringente, compatibles con cristales de polvo de guantes. Mediante luz polarizada se evidencia el típico patrón en cruz de malta del almidón de maíz. Presentamos el caso de una mujer de 54 años con dolor abdominal, fiebre e íleo funcional inusualmente prolongado. Se realizó histerectomía convencional tres meses antes. Al ingreso se realizó videolaparoscopía exploradora, en la cual se perforó accidentalmente el yeyuno distal lo cual requirió laparotomía para enterorrafia. Un mes después fue relaparotomizada debido a la persistencia de íleo, evidenciándose peritonitis granulomatosa por almidón que confirmó la anatomía patológica. Recibió hidrocortisona endovenosa durante dos semanas y prednisona vía oral durante 4 semanas con disminución gradual hasta su suspensión. La paciente permanece asintomática a siete meses del postoperatorio. El tratamiento con corticoesteroides es una opción eficaz. La prevención es clave y radica en el uso de guantes quirúrgicos libres de almidón.

Granulomatous peritonitis due to surgical glove starch is an entity of difficult diagnosis and low incidence. Patients present with abdominal pain, fever and prolonged functional ileus three to four weeks postoperatory of a laparotomy where corn starch as glove's lubricant had been used. In surgical re-exploration both the peritoneum and omentum are widely affected with multiple small white nodules together with peritoneal fluid. Histopathology shows inflammatory tissue with giant multinucleated cells containing bi-refringent cornstarch particles inside. Under polarized light the typical maltese cross appearance of cornstarch is revealed. We present a 54 year old woman admitted with abdominal pain, fever and an unusually prolonged ileus in postoperatory period after a laparotomy. Conventional hysterectomy was performed three months before. At admission, exploratory videolaparocopy was done, in which accidentally distal jejunum was perforated which required laparotomy for enterorraphy. One month after she was re-laparotomized due to persistence of ileus. Granulomatous peritonitis was evidenciated and confirmed by histopathology. Endovenous hydrocortisone was given during two weeks and shifted to oral prednisone during four weeks with gradual diminution until suppression. Patient is asymptomatic at seven months of postoperatory. Corticosteroid treatment is an efficient choice. The prevention is crucial and is based on the employment of starch free surgical gloves.

A Case of Dumping Syndrome Following Nissen Fundoplication in an Infant / 대한소아소화기영양학회지

The dumping syndrome has been a known complication of gastric surgery in adults, but it is recognized as a very rare disease in the pediatric population, especially in Korea. We report a case of dumping syndrome in a 10-month-old infant, who underwent Nissen fundoplication for the treatment of gastroesophageal reflux(GER). He was admitted because of aspiration pneumonia, and diagnosed as GER by 24-hour ambulatory esophageal pH monitoring test. For the treatment of GER, Nissen fundoplication was performed. After the operation, symptoms occurred within 30 minutes of meals, such as diaphoresis, palpitation, weakness, abdominal fullness, nausea, and diarrhea. The gastric emptying scan showed very rapid gastric emptying. His oral glucose tolerance tests revealed early-onset hyperglycema followed by delayed-onset hypoglycemia, which was the characteristic finding of the dumping syndrome. We introduced uncooked cornstarch to resolve symptoms and maintain the serum glucose level. After the feeding of uncooked cornstarch, his symptoms subsided and normal oral glucose test was restored. After the six months of treatment, his weight and height were increased dramatically from below 3 percentiles up to the normal range. The dumping syndrome should be considered when an infant suffers from the feeding difficulties after the gastric surgery like Nissen fundoplication, and the diet therapy including uncooked cornstarch could be applied as an effective measure.

Uncooked Cornstarch Therapy in Type I Glycogen-Storage Disease (GSD-I)

OBJECTIVE: A few years ago it was shown that uncooked cornstarch feeding(UCS) could correct the biochemical abnormalities resulting from a deficiency of glucose-6-phosphatase in children with type ment. So far, little experience is available with UCS in Korea. We studied to compare the effects on biochemical control and growth of therapy with portacaval shunt and therapy with UCS. METHODS: Enghteen patients with GSD-I, ranging in age from 2 to 17 years, were included in these studies. They were thirteen male and five female children, and were diagnosed as GSD-I in Seoul National University from 1982 to 1994. Six patients (age 6.75+/-3.06, range 4.33~12.75 years) received portacaval shunt after preoperative intravenous hyperalimentation, and fifteen patients(age 7.08+/-4.09, rnage 2.00~17.00years) received UCS(1.75~2g/kg, four times daily). They included three patients who have had hepatic adenoma during follow-up after portacaval shunt. Height standard deviation score(SDS), liver size, blood glucose, serum cholesterol, serum uric acid, and serum transaminase were measured. RESULTS: After UCS, the changes of height SDS, liver size, blood glucose, and serum uric acid were significant but the changes of cholesterol, SGOT, and SGPT were not significant. The SDS of height increased significantly form -2.61+/-1.01 at onset of UCS to -1.93+/-0.86 after UCS. The liver size decreased significantly form 10.1+/-2.7cm at onset of UCS to 7.9+/-3.0cm after UCS. Blood glucose levels increased significantly form 50.6+/-14.3mg/dl at onset of UCS to 90.1+/-17.0mg/dl after UCS. Serum uric acid levels decreased significantly form 8.03+/-2.12mg/dl at onset of UCS to 5.67+/-2.34mg/dl after UCS. Statistically, significant difference were not found in those values after portacaval shunt. CONCLUSIONS: UCS was effective to maintain blood glucose, to minimize biochemical abnormalities and to optimize clinical outcome in patients with GSD. Individuals with GSD-I commonly developed hepatic adenoma and malignant transformation of these ademonas has occurred. So in any case, it is advisable to screen all GSD-I patients periodically by imaging techniques for tumor formation, and once tumors have been detected, to adopt strict dietary measures and observe them closely for evidence of suggestive malignant transformation.

Verificação das condições higiênicas de biscoitos por microscopia / Verification of hyginec conditions of biscuits by microscopy

Foram analisadas pelo método da digestão com pancreatina, 109 amostras de biscoitos adquiridas no comércio da cidade de São Paulo, sendo 67 do tipo "Cream-Cracker" e 52 do tipo "Maizena". Chegou-se à conclusão que 100% dos biscoitos "Cream-Cracker" estavam em condições higiênicas insatisfatórias, contaminados com fragmentos de insetos, sendo 3,5% impróprios para o consumo por conterem pelos de roedores e que 96,15% dos biscoitos "Maizena" estavam em condições higiênicas insatisfatórias, sendo 5,77% impróprios para o consumo pelos mesmos motivos que os do tipo "Cream-Cracker" (AU).