Food Protein-Induced Enterocolitis Syndrome.

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-, cell-mediated food allergy of unknown prevalence and pathophysiology. Onset is typically during the first year of life; seafood-induced FPIES may start in adulthood. Acute FPIES manifests within 1-4 hours after ingestion with repetitive emesis, pallor, and lethargy progressing to dehydration and hypovolemic shock in 15% of cases. Chronic FPIES manifests with intermittent emesis, watery diarrhea, and poor growth progressing to dehydration and hypovolemic shock over a period of days to weeks. Chronic FPIES has been only reported in infants aged less than 3 months fed with cow milk (CM) or soy formula. The most common triggers are CM, soy, rice, and oat. Diagnosis of FPIES relies on recognition of a pattern of clinical symptoms and may be missed owing to the absence of typical allergic symptoms (eg, urticaria, wheezing) and delayed onset in relation to food ingestion. Physician-supervised food challenge is recommended if diagnosis or the trigger food is not clear and to evaluate for resolution. Testing for food-specific IgE is usually negative, although a subset of patients, usually with CM-induced FPIES may develop sensitization to foods. Such atypical FPIES tends to have a more prolonged course. Despite the potential severity of the reactions, no fatalities have been reported, and FPIES has a favorable prognosis. In most cases, FPIES resolves by age 3-5 years, although persistence of CM-induced FPIES and soy FPIES into adulthood has been reported. The first international consensus guidelines on diagnosis and management of FPIES were published in 2017. Given that the pathophysiology of FPIES is poorly understood, there are no diagnostic biomarkers and no therapies to accelerate resolution. These unmet needs warrant future investigations to improve the care of patients with FPIES.

Food protein-induced enterocolitis syndrome

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE-, cell-mediated food allergy of unknown prevalence and pathophysiology. Onset is typically during the first year of life; seafood-induced FPIES may start in adulthood. Acute FPIES manifests within 1-4 hours after ingestion with repetitive emesis, pallor, and lethargy progressing to dehydration and hypovolemic shock in 15% of cases. Chronic FPIES manifests with intermittent emesis, watery diarrhea, and poor growth progressing to dehydration and hypovolemic shock over a period of days to weeks. Chronic FPIES has been only reported in infants aged less than 3 months fed with cow milk (CM) or soy formula. The most common triggers are CM, soy, rice, and oat. Diagnosis of FPIES relies on recognition of a pattern of clinical symptoms and may be missed owing to the absence of typical allergic symptoms (eg, urticaria, wheezing) and delayed onset in relation to food ingestion. Physician-supervised food challenge is recommended if diagnosis or the trigger food is not clear and to evaluate for resolution. Testing for food-specific IgE is usually negative, although a subset of patients, usually with CM-induced FPIES may develop sensitization to foods. Such atypical FPIES tends to have a more prolonged course. Despite the potential severity of the reactions, no fatalities have been reported, and FPIES has a favorable prognosis. In most cases, FPIES resolves by age 3-5 years, although persistence of CM-induced FPIES and soy FPIES into adulthood has been reported. The first international consensus guidelines on diagnosis and management of FPIES were published in 2017. Given that the pathophysiology of FPIES is poorly understood, there are no diagnostic biomarkers and no therapies to accelerate resolution. These unmet needs warrant future investigations to improve the care of patients with FPIES (AU)

SEIOA es una alergia alimentaria con patofisiología y prevalencia desconocidas, que típicamente comienza en el primer año de vida, mientras que la producida por pescado suelo tener su comienzo en adultos. La forma aguda se manifiesta entre la hora y 4 horas tras la ingesta del alimento con emesis, palidez, letargia progresiva por deshidratación y shock hipovolémico en el 15% de los casos. La forma crónica se manifiesta con emesis intermitente, diarreas y crecimiento pobre con progresión hacia la deshidratación y shock hipovolémico en un periodo de días o semanas. La forma crónica se ha podido observar únicamente en niños menores de 3 años alimentados con leche de vaca o fórmula de soja. Los desencadenantes más frecuentes son la leche de vaca, la soja, el arroz y avena. El diagnóstico es clínico y puede ser difícil debido a la ausencia de síntomas alérgicos típicos (urticaria, asma…) y a la relación retardada con la ingesta. Es recomendable la provocación controlada si el diagnóstico clínico o el alimento implicado no son claros y también para evaluar la evolución. La IgE específica suele ser negativa, aunque una parte de los pacientes puede desarrollar alergia mediada por IgE. Estos pacientes manifiestan un curso más prolongado. A pesar de la potencial severidad de las reacciones, no se han reportado casos fatales y tiene un pronóstico favorable. La mayoría de los niños consiguen la resolución entre los 3 y 5 años aunque existen casos de persistencia en adultos. Las guías del primer consenso internacional sobre el diagnóstico y tratamiento de esta enfermedad se publicarán en el 2017. Debido a la poco conocida patofisiología, no existen biomarcadores ni terapia que acelere su resolución. Son necesarios estudios que permitan investigar y mejorar la clínica de estos pacientes (AU)

Eosinophilic esophagitis in children: frequency, clinical manifestations, endoscopic findings, and seasonal distribution.

PURPOSE: Eosinophilic esophagitis (EoE) is a chronic disease characterized by eosinophilic infiltration of the esophageal mucosa, which is associated with clinical and endoscopic manifestations. The objective of our study was to determine the frequency of EoE and to outline the clinical manifestations of EoE in Polish children. MATERIAL/METHODS: Ten large regional pediatric gastroenterology centers participated in the study. A database of endoscopy reports from January 2004 till December 2009 was reviewed. A total of 35,631 esophagogastroduodenal endoscopy studies in children, aged from 4 months to 18 years, were performed. Data pertaining to the children's age, gender, indications for endoscopy, clinical findings and histopathology diagnosis were made. RESULTS: In 84 children (20 girls and 64 boys), aged between 4 months and 18 years, EoE was diagnosed. This constituted one case per 424 endoscopic studies. In children with changes in the esophageal mucosa the frequency of EoE was higher and reached one case per 73 children. The most frequent symptoms of EoE differed between the younger (1-6 years old) and older children (aged 13-18 years old). Feeding aversion, vomiting and/or regurgitation were most frequently observed in the younger children, while in older children: abdominal pain, dysphagia and chest pain. Granular mucosa, longitudinal furrows, and mucosal rings belong to the findings most often observed in endoscopic studies. EoE was more frequently diagnosed in the spring (45.2%) and summer (28.5%). CONCLUSIONS: EoE was diagnosed in every age, with frequency of 1/424 gastrointestinal endoscopies, more frequently in boys than in girls.

The ghrelin-positive cells number is increased in duodenum in children with celiac disease.

Ghrelin is predominately produced in the stomach, but new findings indicate that the intestinal wall is an important source of the hormone. In patients with shortbowel syndrome, reduction in the intestinal tissue resulted in a decrease in the circulating ghrelin levels. Since in celiac disease (CD) intestinal mucosa atrophy is the main finding, alterations in duodenal ghrelin-positive cell population can be expected. The aim of the study was to evaluate the density of ghrelin-positive cells in the duodenum of CD children and its relationship with body mass index (BMI) and clinical presentation. The study included 31 consecutive patients with newly diagnosed CD [BMI SD scores (BMISDS) -0.926+/-1.496]. The control group consisted of 21 children (BMISDS -0.517+/-1.186], diagnosed with growth retardation, anemia or abdominal pain. All the patients underwent endoscopy with biopsy samples taken from distal duodenum. Immunohistochemistry was performed using rabbit anti- ghrelin (human) antiserum. The number of ghrelin-positive cells in the duodenum was significantly higher in children with CD than in controls (14.82+/-11.12 vs 5.69+/-5.02, p<0.0013). The density of ghrelin-positive cells in the duodenum did not correlate with age, pubertal status, BMISDS or clinical presentation. In the duodenum of CD children, the number of ghrelin-positive cells is increased compared with the control patients. The population of ghrelin-positive cells in the duodenum does not simply reflect an altered mucosal morphology or failure to thrive but is under the influence of other conditions.

Narcolepsy, metabolic syndrome and obstructive sleep apnea syndrome as the causes of hypersomnia in children. Report of three cases.

Hypersomnia is a significant problem in about 5% of the general population. We discussed clinical aspects in 3 patients with hypersomnia diagnosed in our sleep laboratory. All of the patients, both obese and non-obese, presented abnormal oral glucose tolerance test (OGTT) and plasma insulin level. (1) A 17-year-old girl (BMI = 20.3) with a two-year history of daytime sleep attacks (e.g. on the bus, in a classroom, while reading or eating), followed by refreshed feeling. The first symptoms appeared 2 years after spine injury (L2-L3). Total sleep time was > 98 perc. The diagnosis of narcolepsy was confirmed by sleep-onset REM periods in 3 of 4 daytime naps (positive Multiple Sleep Latency Tests) and HLA-DQB1 (alleles *0201, *0602). (2) A 16-year-old girl (BMI = 32.4) with a history of increased sleepiness (Epworth Sleepiness Scale score = 13), not refreshing naps, along with BMI increase, since the age of 13. The metabolic syndrome was diagnosed based on the presence of obesity, hypercholesterolemia (CH = 240 mg/dl, HDL-CH = 49 mg/dl) and insulin resistance (HOMA index = 6.75, hyperinsulinemia--367 microU/mL at 30' after OGTT). (3) A 6-year-old boy (BMI = 16.0) with a 10-month history of daytime sleep attacks and postprandial sleepiness; nocturnal enuresis, high simple carbohydrate diet, low plasma insulin level after OGTT. Diagnosis of food-related hypersomnia and obstructive sleep apnea was confirmed when the boy recovered after his nutrition habits had been changed, which resulted in decreased respiratory disturbance index (RDI) from 17.7/h in October 2005 to 2.9/h in October 2006. Within that time his parents did not observe any episodes of daytime sleepiness, irritability or nocturnal enuresis.

Bleeding Barrett's ulcer as a complication of GERD [corrected] in physically and intellectually disabled children--report of two cases.

Gastroesophageal reflux disease (GERD) is a problem frequently occurring among physically and intellectually disabled individuals. In this group of patients GERD is often overlooked, since the symptoms are usually non-specific. We present two cases of disabled children, who developed complications of GERD in the form of Barrett's esophagus, Barrett's ulceration and bleeding, the life-threatening events which were not preceded by typical GERD complaints.

Dietary lipid composition modifies intestinal morphology and nutrient transport in young rats.

BACKGROUND: Varying lipid content of the diet of pregnant and nursing dams results in alterations in sugar and lipid uptake into the intestine of their suckling offspring. In this study, we wished to determine whether the same alterations in dietary lipid result in adaptation of intestinal transport in postweaning rats. METHODS: During nursing, the dams were fed the same diet that their offspring were fed for 3 more weeks after weaning. These semipurified diets contained: 1) 15.8% of total fatty acids (w/w) as 18:2n-6 and an n6/n3 ratio of 7.3:1; 2) a diet with 17.6% of total fatty acids as 18:2n-6 and an n6/n3 ratio of 4:1; 3) a diet with 16.2% of total fatty acids as 18:2n-6 and 1.2% arachidonic acid (AA); 4) a diet with 16.8% of total fatty acids at 18:2n-6, 1.2% AA and 0.7% docosahexaenoic acid (DHA); and 5) a diet with 16.0% of total fatty acids as 18:2n-6 and 0.7% as DHA. The in vitro uptake of D-glucose, D-fructose, medium- or long-chain fatty acids and cholesterol was assessed in 6-week-old rats. RESULTS: Feeding AA increased the Vmax for jejunal and ileal uptake of glucose, compared with the high n6/n3 diet. This effect was prevented by adding DHA to the AA diet. The low n6/n3 fatty acid ratio diet decreased uptake of fructose as compared with the high n6/n3 diet, and the increased uptake of fructose with DHA was prevented by adding AA. The incremental change in free energy associated with uptake of medium chain-length fatty acids was lower in the jejunum of animals fed AA plus DHA as compared with the other diet groups. Jejunal uptake of 18:0 was lower for animals fed DHA or AA plus DHA, as compared with AA alone; ileal rate of uptake of long-chain fatty acids was unaffected by diet. CONCLUSIONS: The intestine of young rats modifies its intestinal morphology and adapts its nutrient transport in response to variations in dietary lipids. In postweaning rats, the potentially undesirable effect of one fatty acid on nutrient uptake may be countered by adding a select second fatty acid to the diet.

Early dietary experience influences ontogeny of intestine in response to dietary lipid changes in later life.

This study was undertaken to test the hypothesis that a change in the mother's diet at the time of birth and continued during suckling modifies the intestinal transport of nutrients in the suckling offspring. Pregnant rat dams were fed one of four semisynthetic diets during pregnancy [high or low n-6/n-3 diet or a diet enriched with arachidonic acid (AA) or docosahexaenoic acid (DHA)] and were fed the same diet at the time of birth or switched to another diet. The greatest body weight gain was in the suckling rats (15-16 days of age) fed a low n-6/n-3 diet. Switching from this diet caused weight loss, and the observed weight gain with the low n-6/n-3 diet was prevented by previous exposure of the mother to the high n-6/n-3 diet or the AA- or DHA-containing diet. Although continuous feeding of a high n-6/n-3 diet to the mother during pregnancy and lactation was associated with the lowest in vitro rates of fructose uptake, switching the mother to another diet during lactation did not necessarily correct the low absorption. In contrast, continuous feeding of a high n-6/n-3 diet to the mother during pregnancy and lactation is associated with the highest maximal transport rate of glucose uptake into the jejunum and ileum. Jejunal uptake of fatty acids 12:0, 18:0, 18:3(n-3), and cholesterol was less with the low n-6/n-3 diet compared with the high n-6/n-3 diet, whereas the ileal uptake of 18:0 and 18:3(n-3) was higher with the low n-6/n-3 diet. Thus the ontogeny of the intestine is critically influenced by the mother's diet during gestation as well as during the nursing period. Some of the diet-associated changes in nutrient uptake resulting from the mother's diet during pregnancy could be corrected by dietary interventions introduced after birth.

Ontogeny of intestinal adaptation in rats in response to isocaloric changes in dietary lipids.

Alterations in dietary lipids of the nursing mother result in variations in the lipid content of her milk. Maternal rats, weanlings, and 10-wk-old animals were fed chow or a semisynthetic isocaloric diet enriched with either saturated fatty acids (S) or polyunsaturated fatty acids (P). The jejunal and ileal in vitro uptake of varying concentrations (4-64 mM) of D-glucose and D-fructose or single concentrations of medium- and long-chain fatty acids and cholesterol were assessed in 18- to 21-day-old suckling rats, in 5-wk-old weanling animals, and in 12-wk-old young adults. The rate of uptake of D-glucose and D-fructose was unaffected in suckling rats by changing the lipid content of the diet of the nursing dams, whereas sugar uptake was greater in weanlings or adults fed S compared with P. The jejunal uptake of long-chain fatty acids was not influenced in suckling by changing the mother's diet, whereas in weanlings the uptake of 18:0 and 18:3 was higher with feeding S vs. P. In summary, jejunal uptake of cholesterol was greater in sucklings than in weanlings fed S vs. P. In suckling animals there are different adaptive patterns between the jejunum and the ileum and varying patterns of adaptation in response to alterations in the lipids in the diet when comparing suckling vs. weanling rats. These differences in nutrient uptake could not be explained by age- or diet-associated alterations in villus height. It is concluded that the age of the rat influences the intestinal adaptation of nutrient transport, which occurs in response to changes in dietary lipids, and dietary lipids fed to nursing dams and their offspring are important in the development of the intestine.

Small bowel review--Part II.

The small bowel has undergone intense study. Part II of this review of the small bowel summarizes the current knowledge about the permeability of the gastrointestinal epithelium, the brush border membrane, motility, carbohydrates, diabetes, ethanol, diet, and diagnostic procedures.

Inhibition of lipid absorption as an approach to the treatment of obesity.

A reduction in fat intake may be achieved by making educated choices to reduce total calorie intake, to consume a lower quantity of total fats, or to modify the ratio of saturated-to-polyunsaturated lipids. Leptin agonists or NPY or CCK antagonists may prove to be useful to diminish appetite and thereby reduce the total intake of food. But eating has such cultural, social, and hedonistic attributes that such a single-pronged approach is unlikely to be successful. The use of fat substitutes may prove to be popular to provide a wide range of snack food options, but these are likely to be of minimal use in weight reduction programs because of their distribution of additives in only a limited number of foods. The inhibitors of lipid digestion will be modestly successful in the short term; their long-term success will be influenced by gastrointestinal adverse effects and the need to consume fat-soluble vitamin supplements to prevent the development of fat-soluble vitamin deficiencies. The inhibition of lipid absorption is an attractive targeted approach for the treatment of obesity, since this would reduce the uptake of visible as well as invisible fats, which would potentially offer convenient dosing, and could also be a means to inhibit secondarily the uptake of carbohydrate calories.

Small bowel review--Part I.

The small bowel has undergone intense study. Part I of this two-part review of the small bowel focuses on gastrointestinal peptides; intestinal infections and human immunodeficiency virus; drugs; intestinal growth-mucosal proliferation and differentiation; nucleic acids, nucleotides and nucleosides; vitamins and minerals; Whipple's disease; radiation; and early development.

[Molecular collagen content and polymorphism in the breast with benign dysplasia]. / Zawartosc i polimorfizm molekularny kolagenu w sutku objetym procesem lagodnej dysplazji.

A significant increase in collagen content in breast benign dysplasia has been noted in comparison with normal breasts. Type I and type III collagens were isolated from the normal breast. These types constitute 70% and 25% of the total collagen contents in breast, respectively. It was found, that the major part of tumor collagen is present in the form of type I and type III collagens stable complex. This complex does not dissociate under conditions used for collagen isolation. Normal female breast does not contain such a complex.

Collagen of breast with benign dysplasia.

Benign dysplasia is a pathological process in the female breast associated with hypertrophy of interlobular and periductal connective tissue. It leads to disappearance of the lobular structure of this organ. It was found that the mammary tumours contain more collagen in comparison to the normal breast. In the normal breast collagen type I and type III constitute 70% and 25% of the total collagen content, respectively. The investigated tumors contain mainly a complex of collagen type I and type III which cannot be dissociated by the methods used for collagen preparation.