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Propionic acidemia
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop
Patients with propionic acidemia usually present first with life-threatening illness very early in life (Figure 2.2). Many patients have died in the course of one of these episodes of illness. Patients with metabolic disease, which presents this way in the neonatal period, may appear to have sepsis, ventricular hemorrhage or some other catastrophic process. It is likely that most patients die undiagnosed. A typical episode is heralded by ketonuria. The initial symptom is often vomiting, and some patients have had such impressive vomiting that they have been operated on with a diagnosis of pyloric stenosis [1, 11, 12]. Massive ketosis leads to acidosis and dehydration. Lethargy is progressive to coma. Unless the patient is treated vigorously with intubation and assisted ventilation, as well as very large quantities of fluid and electrolytes, shock intervenes and the outcome is death [13]. Presentation of a gravely-ill infant can be with hypothermia. In an experience with 30 patients [14], 90 percent presented with severe acidosis.
The Digestive (Gastrointestinal) System and Its Disorders
Published in Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss, Understanding Medical Terms, 2020
Walter F. Stanaszek, Mary J. Stanaszek, Robert J. Holt, Steven Strauss
The stomach (see Figure 6.2) in an adult is usually up to ten inches long and six inches wide with a capacity of approximately 1.5 liters, although this varies greatly. The stomach is a single distinct cavity, but anatomists divide it into four primary areas on the basis of function and physiology. The cardiac region is nearest the esophagus and forms the entry into the stomach; the fundus is the body or large central portion; the antrum, which may be viewed as the upper part of the pylorus, is the portion where narrowing begins; and the pylorus leads out of the stomach.
The gastrointestinal tract
Published in Simon R. Knowles, Laurie Keefer, Antonina A. Mikocka-Walus, Psychogastroenterology for Adults, 2019
Christopher F.D. Li Wai Suen, Peter De Cruz
Sphincters also occur at various points in the GI tract. These circular muscles act as gateways that regulate the passage of food products. By contracting, they stop the flow of content whereas by relaxing, they allow the passage of contents. The pyloric sphincter (or pylorus), for example, is at the exit end of the stomach and regulates passage of food into the duodenum (the first part of the small intestine connected to the stomach).
Comparative study on the gastrointestinal- and immune- regulation functions of Hedysari Radix Paeparata Cum Melle and Astragali Radix Praeparata cum Melle in rats with spleen-qi deficiency, based on fuzzy matter-element analysis
Published in Pharmaceutical Biology, 2022
Yugui Zhang, Jiangtao Niu, Shujuan Zhang, Xinlei Si, Tian-Tian Bian, Hongwei Wu, Donghui Li, Yujing Sun, Jing Jia, Erdan Xin, Xingke Yan, Yuefeng Li
The upper part of the small intestine starts from the pylorus of the stomach, and its lower part is connected with the large intestine via the ileocecal valve, which is divided into duodenum, jejunum and ileum. The HE staining results of duodenum, jejunum and ileum showed obvious injuries in SQD model compared with normal. The main reason may be related to the diarrhoea symptoms of SQD rats. Three parts of the small intestine had more crypt cells, the villi were shortened and indistinct, the villi tips were partially necrotic and detached, the villus epithelial cells were damaged and detached, and edoema was obvious. The intestinal glands were obviously degenerated, and the submucosa was slightly congested and severely oedematous, with a small amount of inflammatory cell infiltration. After treatment, the number, arrangement and morphological structure of glandular cells in each part of the small intestine were significantly improved, the length of villi increased, and edoema was relieved. In particular, HRPCM (18.9 g/kg) and ARPCM (18.9 g/kg) were more significant (Figure 10).
Ascorbic acid-2 glucoside mitigates intestinal damage during pelvic radiotherapy in a rat bladder tumor model
Published in International Journal of Radiation Biology, 2022
Yasutoshi Ito, Tetsuo Yamamoto, Kosuke Miyai, Junya Take, Harry Scherthan, Anna Rommel, Stefan Eder, Konrad Steinestel, Alexis Rump, Matthias Port, Nariyoshi Shinomiya, Manabu Kinoshita
Rats were euthanized with pentobarbital one week after the last X-irradiation fraction, and the bladder and the GI tract were resected. The bladders were inflated with 0.8 mL of 20% formalin and immersed in formalin for two days, followed by paraffin embedding. Slides were prepared from the processed specimens and stained with hematoxylin and eosin (H. E.). The tumor stage was scored according to the TNM classification guidelines (Spiess et al. 2017). The bladder samples were stained with Berlin blue to observe hemosiderin deposits in macrophages. Remained bladder tumors in a rat that died from anesthetic accident during radiotherapy were stained with TUNEL (terminal deoxynucleotidyl transferase-mediated dUTP nick end labeling), using an in situ apoptosis detection kit (MK500, Takara, Tokyo, Japan) as described elsewhere (Ito et al. 2013). Four parts of the small intestine were taken at the 10–15 cm from the pylorus, 25–30 cm, 40–45 cm, and 55–60 cm that was the part at 10–15 cm from the ileocecal valve, and were similarly fixed with formalin, paraffin-embedded, and stained with H. E.
Safety considerations when managing gastro-esophageal reflux disease in infants
Published in Expert Opinion on Drug Safety, 2021
Melina Simon, Elvira Ingrid Levy, Yvan Vandenplas
In a large retrospective cohort study including more than 14,000 infants, an increased risk of developing pyloric stenosis was noticed in infants who received erythromycin before the age of 2 weeks (relative risk = 10.51 95% CI 4.48, 24.66) [161]. Another study comparing the adverse effects of metoclopramide and erythromycin showed that 0.9% (14/1587) of the infants receiving erythromycin developed pyloric stenosis and 0.4% (77/19.200) of these receiving metoclopramide [162]. The incidence of pyloric stenosis in the overall population is 0.2–0.4% [154]. There are also a few case reports of severe arrhythmias associated with the use of erythromycin in neonates but only when administered intravenously [163]. In addition to the concern of antimicrobial resistance, unnecessary use of antibiotics should be avoided because of potential later metabolic effects, thought to be due to perturbation of the host microbiome [164]. Overall, neither low-dose regimes nor prophylactic trials have been shown to be useful [165]. Theoretical risks of prolonged antibiotic use, such as emergence of antibiotic resistance and abnormal intestinal microbiota, have not been fully evaluated [164].