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The ability of the small intestine to perform various functions, such as digestion/absorption of nutrients, gradually declines with age. However, the mechanism that causes intestinal senescence remains unclear. Therefore, age-related changes in the jejunum and ileum were evaluated using senescence-accelerated mouse (SAM) strains that possess characteristic phenotypes of aging. In particular, to understand how senescence affects the small intestine, we investigated whether age-related changes in the morphology of the intestinal villi and its capability to digest/absorb nutrients are associated with the senescence phenotypes identified in specific SAM strains. Four SAM strains were selected (SAMP1, SAMP6, SAMP10, and SAMR1; of which SAMR1 served as a control of SAMP strain) and age-related changes in the small intestine were evaluated for each strain. A villus morphological analysis, mRNA expression level analysis of the small intestine-specific molecules, and disaccharidase activity measurement were performed. We observed that the mRNA expression levels of the genes involved in the differentiation of intestinal epithelial cells and in the digestion/absorption of nutrients were markedly decreased in all the SAM strains, especially in the SAMP10 strain. Our results revealed that all the SAM strains spontaneously induced senescence of the small intestine, which occurred due to the disorders affecting the differentiation/maturation system of intestinal epithelial cells. In addition, it was evident that senile phenotypes, such as brain dysfunction, enhanced intestinal senescence in the SAMP10 strain. The results of this study suggest that the brain-intestinal nervous system may play role in maintenance of villous morphology and nutrients uptake via the GLP-2 and IGF-2 signaling pathway., (Copyright © 2022 Elsevier Inc. All rights reserved.)

The gyromagnetic factor of the low-lying E=251.96(9)  keV isomeric state of the nucleus ^{99}Zr was measured using the time-dependent perturbed angular distribution technique. This level is assigned a spin and parity of J^{π}=7/2^{+}, with a half-life of T_{1/2}=336(5)  ns. The isomer was produced and spin aligned via the abrasion-fission of a ^{238}U primary beam at RIKEN RIBF. A magnetic moment |μ|=2.31(14)μ_{N} was deduced showing that this isomer is not single particle in nature. A comparison of the experimental values with interacting boson-fermion model IBFM-1 results shows that this state is strongly mixed with a main νd_{5/2} composition. Furthermore, it was found that monopole single-particle evolution changes significantly with the appearance of collective modes, likely due to type-II shell evolution.

In order to verify indications for surgery, 27 patients with refractory epileptic seizures and brain tumor, aged up to 19 years at the time of surgery, were studied between 1996 and 2013 and followed up for at least one year. The mean interval between the onset of seizures and the diagnosis of the tumor was 3.6 years, and from diagnosis to the surgery, 18 months. The location of the tumor was in the temporal lobe in 16, with ganglioglioma and dysembryoplastic neuroepithelial tumors being the most frequent. Among the patients, 92.5% and 90.4% were seizure-free in the first and fifth year after surgery, respectively. Twelve of 16 children were successful in becoming drug-free, with complete withdrawal by 3.2 years. Surgery proved to be potentially curative and safe in these cases, suggesting that the tumor diagnosis and surgery cannot be postponed.

Background: Organic acidurias or organic acidemias are inherited metabolic disorders in which organic acids (carboxylic acids) accumulate in tissues and physiologic fluids of affected individuals. They are considered the most frequent metabolic disorders among severely ill children. Patients frequently present acute symptoms in early life. Metabolic acidosis and neurologic symptoms are the most common signs., Methods: Urine specimens obtained from 1,926 children from January 1994 to July 2001 were used in analyses. Venous blood specimens were also collected from some patients. Samples were initially submitted to screening tests for detection of inborn errors of metabolism. Identification and semi-quantitation of organic acids in urine were performed by gas chromatography or gas chromatography coupled to mass spectrometry using capillary column (DB-5) and flame ionization detection., Results: Ninety three (4.8%) cases of organic acidemias were diagnosed among 1,926 patients investigated from January 1994 to July 2001. Prompt therapy was instituted after diagnosis in a considerable number of patients and resulted in rapid improvement in their symptomatology, distinct from our previous cases diagnosed abroad where patients representing index cases died before any measure could be taken., Conclusions: Results demonstrate the importance of diagnosing organic acidurias in loco in developing countries despite implied extra costs.

We report an infant with intermittent urinary excretion of D-2-hydroxyglutaric (D-2-OHG) acid who died at the age of 10 months from cardiogenic shock due to cardiomyopathy. High urinary concentrations of D-2-OHG and succinic acid, as well as increased levels of lactic acid were detected on three different occasions, whereas a normal urinary profile of organic acids was found on one occasion. The clinical findings of our patient consisted of generalized hypotonia, irritability, developmental delay, generalized tonic seizures, lethargy, cardiomyopathy, and respiratory distress. Cerebral MRI revealed bilateral lesions in the substantia nigra, the periaqueductal area, the medial part of the thalamus, the hypothalamus, the caudate nucleus, putamen and globus pallidus. This pattern is suggestive of a mitochondriopathy. However, respiratory chain enzyme activities were normal in fibroblasts. Exogenous supplementation of D-2-OHG acid strongly inhibited cytochrome-c oxidase activity in fibroblasts from the patient and from normal controls in vitro. The results suggest that our patient has an unusual form of D-2-hydroxyglutaric aciduria (D-2-OHGA), different from the patients published so far, and that the increase of lactic acid and some citric acid cycle intermediates encountered in some patients with D-2-OHGA may be due to a functional defect of the respiratory chain caused by D-2-OHG acid.

OBJECTIVE: To determine the prevalence of organic acidurias in high-risk Brazilian patients. METHODS: Laboratory techniques for the detection and quantification of organic acids by gas chromatography/mass spectrometry were implemented in Porto Alegre, Brazil. We investigated 1,480 patients suspected of organic aciduria between January 1994 and June 2000. RESULTS: Seventy three (4.9%) cases of organic acidemias (acidurias) were diagnosed among the tested individuals. In most of these patients, prompt therapy resulted in rapid symptom improvement; these results are completely different from our previous cases diagnosed in other laboratories in Europe and the United States, where several patients died before any measures could be taken. CONCLUSIONS: These results demonstrate the importance of diagnosing organic acidurias in loco even in developing countries, in spite of the extra costs involved.

A personal series (in 94% of the cases) of 102 children who underwent 170 procedures (1.66 procedures/patient) for hydrocephalus has been followed for 5 1/2 years (Jan-83 to June-88). Most of the children were under two years of age (80%) and in these cases brain sonography was the examination of choice for both diagnosis and follow-up (307 examinations, 4.4 per patient). Only occasionally was computed tomography necessary for better study in these cases. Our results suggest that there was no significant difference between our cases and those published in the literature concerning the number or procedures/patient (1.66)., infection rate (5.2%), mortality rate (6.8%) and intellectual performance. We recommend the use of brain sonography both in diagnosis and follow-up studies for hydrocephalic children since this examination is innocuous, inexpensive and easy to perform by neurosurgeons. Also it provides a good interaction between the examiner, the child and the parents, which is of utmost importance for the comprehension of the disease by the parents and early diagnosis of complications by the neurosurgeon.

Antibodies to a trinucleotide, ApApA, were prepared by injecting bovine serum albumin conjugated with ApApA into rabbits. The specificities of the antibodies were determined by estimating the inhibiton of the binding of [14C]ApApA to the antibodies by various nonradioactive mono-, oligo-, and polynucleotides, using the ammonium sulfate precipitation method. The antibodies were found to react with ApApN sequences and oligoadenylic acids, but also reacted slightly with polyadenylic acid, RNA and DNA. Significant crossreactions were observed with other oligonucleotides containing adenosine.