bluuebunny/crossref_metadata_2025_split huggingface dataset:
Potassium Supplementation in Kwashiorkor
Authors: Manary M. J. , Brewster D. R. 
Published: February 2024

Background:Kwashiorkor is an edimatous form of severe malnutrition and is the predominant form of childhood malnutrition in Malawi. Potassium depletion is common and contributes to the high mortality. The aim of this study was to determine if high potassium supplementation improves the outcome of kwashiorkor treatment.Methods:We performed a randomised, double‐blind, placebo‐controlled, clinical trial of high potassium supplementation in 99 children with kwashiorkor. Controls (n = 51) received a standard potassium intake of 4.7 mmol/kg/day. The intervention group (n = 48) received 7.7 mmol/kg/day. All cases (intervention and control groups) were treated in the hospital‐based Nutrition Rehabilitation Center and received a standard treatment regime of mild feeds, mineral and vitamin supplements, and antibiotics.Results:There was no significant difference in length of hospitalization, or time for resolution of oedema between groups. The case‐fatality rate was reduced by 33% in the high potassium intervention group (13/48) compared to controls (21/51). There was a significant reduction in late deaths (13 in controls vs 3 in intervention group; odds ratio 5.3, 95% confidence interval 1.2‐31.0) but no difference in early deaths (0‐5 days). The intervention group also had significantly fewer presumed septic episodes (3 vs 18, odds ratio 8.9, confidence interval 2.2‐50.9), respiratory symptoms, and new skin ulcerations than controls.Conclusions:The high potassium supplementation reduced mortality and significant morbidity in kwashiorkor. This may be due to improved myocardial and immune function from earlier repletion of intracellular potassium. We recommend that the standard potassium supplement for the initial phase of treatment of kwashiorkor be increased from 4 to 8 mmol/kg/day.
Intestinal Permeability in Cystic Fibrosis in Relation to Genotype
Authors: Hallberg K. , Grzegorczyk A. , Larson G. , Strandvik B. 
Published: February 2024

Background:The purpose of this study was to investigate whether the increased intestinal permeability (IP) seen in patients with cystic fibrosis (CF) is correlated with the basic defect, as revealed by the patient's genotype, and/or whether the intestinal disturbance reflects secondary abnormalities such as essential fatty acid deficiency.Methods:Nineteen CF patients were compared with nine age‐ and sex‐ matched healthy controls. IP was evaluated by studying urinary excretion for 5 hours after a test meal containing lactulose, L‐rhamnose and xylose in water. Urine was analyzed for carbohydrates, and blood samples were taken for determination of the fatty acid pattern of serum phospholipids. The CF patients were grouped according to genotype: homozygous for ΔF508, heterozygous for ΔF508, or unidentified.Results:Patients who were homozygous (n = 9) or heterzygous (n = 6) for ΔF508 had significantly higher lactulose/L‐rhamnose excretion ratios (mean(range) values of 0.08(0.05‐0.13) and 0.09(0.03‐0.13), respectively) than patients (n = 4) with unidentified genotypes [0.03(0.02‐0.05); p = 0.005] or healthy controls [0.02(0.003‐0.06); p = 0.002]. CF patients with EFAD (n = 6) did not differ from those with a normal pattern of serum phospholipid fatty acids, the lactulose/L‐rhamnose excretion ratio being 0.08(0.02‐0.13) and 0.07(0.03‐0.12), respectively.Conclusions:These data show that the IP in CF was related to patient genotype; those homozygozous or heterozygous for ΔF508 having a significantly increased IP compared with patients with unidentified genotypes, who had IP values within the normal range.
A High Index of Suspicion: The Key to an Early Diagnosis of Wilson's Disease in Childhood
Authors: Sánchez‐Albisua Icíar , Garde Teresa , Hierro Loreto , Camarena Carmen , Frauca Esteban , de la Vega Angela , Díaz María Carmen , Larrauri Javier , Jara Paloma 
Published: February 2024

ABSTRACTBackground:To study the clinical features of Wilson's disease in childhood.Methods:Retrospective review of the clinical, laboratory, and histologic features and prognosis of Wilson's disease in 26 Spanish children.Results:The first medical visit, at age 9.8 ± 3.4 years (range, 4‐16 years), was prompted by liver dysfunction detected accidentally (61%), symptoms of liver disease (27%), family screening (8%), and extrapyramidal symptoms and personality changes (4%). There were laboratory data of hepatic failure in 27%. All copper metabolism test results (total serum copper, 24‐hour urine excretion, serum ceruloplasmin) were abnormal in 62%, two in 27%, and one in 11%. All patients in whom extrahepatic involvement was found at diagnosis had severe liver disease. Histologic findings were portal fibrosis with steatosis (29%), cirrhosis (21%), portal fibrosis (17%), chronic active hepatitis (17%), and minimal changes or normality (17%). Penicillamine was administered to all but one patient. Four children underwent liver transplantation, three of them having received penicillamine for 12, 45, and 70 days. Three other patients recovered from liver failure after 1 year of treatment with penicillamine. After a follow‐up of 4.5 ± 3.3 years, all the children survived. Penicillamine caused severe toxicity in one patient.Conclusions:Wilson's disease in childhood is generally detected by maintaining a high suspicion of liver disease in patients who have no or nonspecific hepatic symptoms. Kayser‐Fleischer ring is rare in childhood. Drug therapy is effective and well tolerated even in some cases of hepatic insufficiency.
Cisapride in Management of Chronic Pediatric Constipation
Authors: Halabi Issam M. 
Published: February 2024

ABSTRACTBackground:This study was conducted to explore the use of cisapride in the treatment of chronic idiopathic constipation in children.Methods:Seventy‐nine children were screened. Seventy‐three of them met the selection criteria that included clinical, laboratory, radiologic, and histopathologic investigations. These patients entered a week‐long phase I of disimpaction using lactulose. Four of them were noncompliant and thus were excluded from the next phase. In phase II sixty‐nine patients were assigned to two treatment groups: 0.3 mg/kg cisapride four times a day versus matching placebo for 8 weeks in a double‐blind study. The two groups that completed phase II were similar in age and duration of symptoms, confirmed by statistical analysis. Stool frequency was assessed weekly, beginning at the end of the disimpaction phase and continuing for 9 weeks. Total gastrointestinal transit time was measured twice, on completion of phase I and 9 weeks later. Transit time is the time required for a carmine marker taken orally after overnight fast to appear in the stool.Results:There was a significant difference between stool frequency per week before and after cisapride treatment, stool frequency per week at the end of phase II with cisapride versus placebo, and total gastrointestinal transit time before and after treatment with cisapride (p < 0.05 for all values). No such difference was demonstrated when comparing stool frequency per week or total gastrointestinal transit time before and after placebo (p > 0.05 for both).Conclusions:Cisapride may have a role in the management of chronic idiopathic constipation in children.
Autoimmune Enteropathy With Distinct Mucosal Features in T‐Cell Activation Deficiency: The Contribution of T Cells to the Mucosal Lesion
Authors: Murch Simon H. , Fertleman Caroline R. , Rodrigues Carlos , Morgan Gareth , Klein Nigel J. , Meadows Nigel , Savidge Tor C. , Phillips Alan D. , Walker‐Smith John A. 
Published: February 2024

ABSTRACTBackground:Autoimmune enteropathy is normally characterised by crypt hyperplastic villous atrophy with enterocyte autoantibodies, activation of mucosal lymphocytes and increased epithelial HLA‐DR. This case involved a severely affected Portuguese infant who was found to have lymphocyte activation deficiency and demonstrated correspondingly distinct mucosal features.Methods:A female infant of nonconsanguineous parents was treated for vomiting and diarrhoea, first with milk exclusion and then with parenteral nutrition. Lymphocyte subsets and immunoglobulin concentrations were normal, but in vitro testing showed no activation in response to phytohaemagglutinin, Candida, or purified protein derivative, although the response to interleukin (IL)‐2 was intact. Interleukin‐2 deficiency was excluded. Analysis of jejunal biopsy specimens revealed only mild villous blunting with absent goblet cells, normal epithelial proliferation, and no crypt hyperplasia. The dense infiltrate of CD8+ and CD4+ T lymphocytes showed normal CD2 and CD3 expression but no activation or proliferation markers. HLA‐DR was not increased on epithelium or lymphocytes. Thus, in addition to in vitro evidence for lymphocyte activation deficiency, the mucosal specimens showed no evidence of in situ T‐cell activation.Results:After development of overwhelming septicaemia, the patient died at 18 months, just before a planned bone marrow transplant.Conclusions:These findings confirm significant heterogeneity within autoimmune enteropathy. Formal immune function testing should be performed in all affected infants to identify T‐cell activation deficiencies. The distinct mucosal findings suggest that activated T cells usually induce the crypt hyperplastic villous atrophy characteristic of classic autoimmune enteropathy.
Inflammatory Bowel Disease in Children and Adolescents: Mental Health and Family Functioning
Authors: Engstrom Ingemar 
Published: February 2024

ABSTRACTBackground:Inflammatory bowel disease in children and adolescents often leads to an extremely complex somatic and psychiatric situation. The psychological effect of inflammatory bowel disease warrants further investigation, especially concerning salutogenetic factors that may lead to good mental health despite bad somatic conditions.Methods:These studies used a multimethod design comprising both semiquantitative measures, such as rating scales and questionnaires, and qualitative in‐depth interviews with both the child and his or her parents. Clinical comparison groups of matched children with diabetes and chronic tension headaches and matched children without chronic physical disease were examined.Results:Inflammatory bowel disease often leads to psychiatric sequelae. Emotional disorders, especially depression and anxiety symptoms, were found to be common. Self‐esteem was lowered. A subgroup of children with good mental health despite bad somatic conditions was found. They exhibited certain characteristics, including good knowledge of the disease, an internal locus of control, a good family climate, and an open social network.Conclusions:This study shows that the well‐being of a chronically ill child depends not only on the course of the physical disease but also on the psychological and social complications that often seem to accompany a disease of this kind. The importance of taking good care of the psychosocial aspects of inflammatory bowel disease within the comprehensive treatment program is discussed.
Variability of Human Milk Neutral Oligosaccharides in a Diverse Population
Authors: Erney Renee M. , Malone William T. , Skelding Mary Beth , Marcon Andrea A. , Kleman–Leyer Karen M. , O'Ryan Miguel L. , Ruiz–Palacios Guillermo , Hilty Milo D. , Pickering Larry K. , Prieto Pedro A. 
Published: February 2024

ABSTRACTBackground:A complex array of free oligosaccharides is a distinctive compositional feature of human milk. Although these oligosaccharides have been studied for several years, their variability and distribution have not been systematically studied, and their nutritional and functional roles have not been elucidated. This report describes a study in which a large number of human milk samples were analyzed for the presence and content of nine neutral oligosaccharides. The resultant data were used to probe for distribution trends by donor groups and stage of lactation.Methods:Milk samples from 435 women residing in 10 countries were analyzed using a simple preparation procedure, gel filtration, and high‐performance anion‐exchange chromatography.Results:All samples contained structures based on lacto‐N‐neotetraose and lacto‐N‐tetraose. This contrasts with the fucosyloligosaccharides tested, none of which was detected in 100% of the samples. Unexpected distribution trends were observed. For example, 100% of the samples from Mexico (n = 156) contained 2´‐fucosyllactose, whereas only 46% of the samples from the Philippines (n = 22) contained this structure. Concentration ranges for the analyzed oligosaccharides revealed quantitative and qualitative distribution trends.Conclusions:The oligosaccharide composition of human milk varied among samples. The geographical origin of the donors was one of the factors that accounted for this variability. This can be explained by genetically determined traits that are not uniformly distributed. Results indicated that further systematic studies are needed to ascertain the effect of other factors, such as lactation stage or diet.
Iron‐Deficiency Anemia Associated With Helicobacter pylori Gastritis
Authors: Konno Mutsuko , Muraoka Shunji , Takahashi Michiko , Imai Toshio 
Published: February 2024

ABSTRACTBackground:Recent studies have suggested an association of Helicobacter pylori and iron‐deficiency anemia (IDA). This is a report of six cases of IDA associated with H. pylori gastritis.Methods:Six patients with IDA were studied (5 boys and 1 girl; mean age 13.6 years; range 13–15 years). Five had a medical history of long‐standing IDA and of oral iron supplementation at outpatient clinics. The anemia recurred after the iron therapy had been discontinued. The sixth patient was admitted to the hospital with severe IDA. An extensive work‐up was ordered that included technetium‐99m (99mTc) scans for Meckel's diverticulum, total colonoscopy, and gastrointestinal endoscopy. After biopsy‐based H. pylori test results were confirmed to be positive, anti‐H. pylori therapy consisting of lansoprazole, clarithromycin, and metronidazole was administered for 2 weeks with no iron supplementation. The hematologic profile and iron status were assessed periodically after the end of the eradication regimen.Results:Upper gastrointestinal endoscopy revealed a marked antral nodularity but no evidence of bleeding lesions in all the patients. Given the histology and the fact that rapid urease test results were positive, chronic active gastritis with H. pylori was diagnosed in all these cases. H. pylori was successfully eradicated in all the patients. There was no evidence of IDA in any of the follow‐up examinations between 27 and 50 months after anti‐H. pylori therapy.Conclusions:H. pylori infection may be involved in cases of IDA of unknown origin, and the eradication of H. pylori can be associated with the resolution of anemia.
Predicting verbal and performance intelligence quotients from multimodal data in individuals with attention deficit/hyperactivity disorder
Authors: He Ningning , Kou Chao 
Published: February 2024

AbstractDespite the importance of understanding how intelligence is ingrained in the function and structure of the brain in some neurological disorders, the alterations of intelligence‐associated neurological factors in atypical neurodevelopmental disorders, such as attention deficit/hyperactivity disorder (ADHD), are limited. Therefore, we aimed to explore the relationship between the brain functional and morphological characteristics and the intellectual performance of 139 patients with ADHD. Resting‐state functional and T1‐weighted structural magnetic resonance imaging (MRI) data and intellectual‐performance data of the patients were collected. The MRI data were preprocessed to extract four indicators characterizing the participants' brain features: fractional amplitude of low‐frequency fluctuation, regional homogeneity, and gray and white matter volumes. Then, we used a two‐layer feature‐selection method with support vector regression models based on three kernel functions to predict the verbal and performance intelligent quotients of the patients, along with ten fold cross‐validation to evaluate the models' predictive performance. All models showed good performance; the correlation coefficients between the predicted and observed values for each predictive phenotypic variable were >0.41, with statistical significance. The brain features that could best predict the intellectual performance of the patients were concentrated in the superior and inferior frontal gyrus of the prefrontal areas, the angular gyrus and precuneus of the parietal lobe, the inferior and middle temporal gyrus of the temporal lobe, and part of the cerebellar regions. Thus, the voxel‐based brain‐feature indicators could adequately predict the intellectual performance of patients with ADHD, providing a foundation for future neuroimaging studies of this disorder.
A proposed modification to the Kellgren and Lawrence classification for knee osteoarthritis using a compartment‐specific approach
Authors: Perico Diego Alarcón , Uribe Abelardo Camacho , Niño Sara Jaimes , Mayorga María Camila Peñaloza , Sundfeld Christian , Lievano Jorge Rojas , Mendoza Cristal Castellanos , Ramirez Rafael Gómez , Rapalino Oscar Rivero , Zayed Gamal , Arango German Carrillo , Mieth Klaus 
Published: February 2024

AbstractPurposeSince Kellgren and Lawrence (KL) originally classified knee osteoarthritis, several authors have reported varying levels of reliability and a lack of uniformity in the use of this classification system. We propose several modifications to the KL classification including the use of a compartment‐specific approach that we hypothesize will lead to a better understanding of knee OA while maintaining an adequate interobserver and intraobserver reliability.MethodsWe propose the addition of the lateral and skyline‐view radiographs to the standard anteroposterior (AP) and lateral projections in the evaluation. Also suggest a more precise definition of the evaluated parameters; the addition of the subchondral cancellous bone as parameter of evaluation; and the assessment of medial tibiofemoral compartment (MTFC), lateral tibiofemoral compartment (LTFC) and patellofemoral compartment (PFC) separately resulting in a compartment‐specific KL staging score rather than a single overall KL score. Six evaluators (two knee surgeons, two radiologists and two knee fellows) used the modified KL classification to classify 230 randomly selected knees on two separate occasions. Reliabilities were assessed by calculating Krippendorff's ⍺ coefficients.ResultsTwo hundred and ten knees were included for final evaluation and analyses (53% left knees; 65% females; mean age 56 years old). Average interobserver reliability was moderate for all compartments (0.51 for the MTFC; 0.51 for the LTFC; and 0.56 for the PFC). Average intraobserver reliability was substantial for all compartments (0.63 for the MTFC; 0.65 for the LTFC; and 0.7 for the PFC). Experienced evaluators showed a higher intraobserver reliability than less‐experienced evaluators.ConclusionsA modified compartment‐specific KL classification enables a practical and detailed description of knee OA involvement and demonstrates acceptable interobserver and intraobserver reliability.Level of Evidence: Level III
Melatonin injection and red light irradiation affect the antioxidant response and cell damage in disk abalone (Haliotis discus hannai) exposed to high water temperatures
Authors: Kim Jin A , Kim Min Ju , Park Young‐Su , Kim Jun‐Hwan , Choi Cheol Young 
Published: February 2024

AbstractThe effects of red light‐emitting diode (LED) light irradiation (630 nm, 0.5 W/m2) and melatonin (10−8 and 10−7 M) on oxidative stress and physiological responses in abalones exposed to high temperatures (28°C) were investigated. Changes in messenger RNA (mRNA) expressions of melatonin receptor (MT‐R), heat shock protein 70 (HSP70), and antioxidant enzymes, as well as alterations in H2O2 levels in the hemolymph, were examined. The results revealed that high‐temperature‐stressed abalones treated with melatonin injections or exposed to red LED light showed a significant increase in MT‐R mRNA expression, while HSP70 mRNA expression decreased. Notably, HSP70 mRNA expression levels in the red LED light‐irradiated group were similar to those in the group injected with 10−8 M melatonin after 24 h exposure. Abalones treated with melatonin at 20°C or irradiated with red LED light exhibited decreased H2O2 levels and reduced antioxidant enzyme mRNA expression compared with those of the control group. However, the high‐temperature environment induced oxidative stress in abalones, leading to increased antioxidant enzyme mRNA expression compared with that under 20°C conditions. Moreover, abalones exposed to high‐temperature stress exhibited hepatopancreatic DNA damage, which was attenuated by melatonin treatment or red LED light irradiation. Hence, red LED light reduces oxidative stress, boosts antioxidant enzymes, and alleviates DNA damage in high‐temperature‐stressed abalones, akin to 10−8 M melatonin treatment. Therefore, considering the practical challenges of continuous melatonin administration to abalones, utilizing red LED light emerges as a practical, effective alternative to protect abalones from oxidative stress compared to 10−8 M melatonin treatment.
Physicochemical properties of esterified/crosslinked quinoa starches and their influence on bread quality
Authors: Wang Donghai , Tang Zhiyi , Chen Rui , Zhong Xu , Du Xiaoyao , Li Yi‐Xuan , Zhao Zhengtao 
Published: February 2024

AbstractBACKGROUNDStarch is the main component of quinoa seeds. However, quinoa starch has poor solubility in cold water and poor mechanical resistance and is easily aged, which limit its application. Therefore, modification of its structure to improve its functional properties is necessary.RESULTSThis research used acetic anhydride and sodium trimetaphosphate to modify the structure of starch molecules and investigated their influence on bread quality. The results showed that both esterification and crosslinking prevented the aggregation behavior of starch molecules. Moreover, they both decreased the gelatinization enthalpy change and relative crystallinity of the starch. Compared with native starch, modification significantly decreased the gelatinization temperature from 57.01 to 52.01 °C and the esterified starch exhibited the lowest enthalpy change with a 44.2% decrease. Modified starch increased the specific volume and decreased the hardness and chewiness of bread. Modification did not influence the moisture content in bread but impacted the water retention capacity, depending on the degree of modification. Low and medium degrees of modification improved the water retention capacity during storage. By contrast, a high degree of modification (10 g kg−1 crosslinking agent) decreased the water retention capacity. The dually modified quinoa starch (esterified and crosslinked) showed no influence on the textural properties of bread.CONCLUSIONThis study demonstrated that both esterification and crosslinking significantly improved the functional properties of quinoa starch. Crosslinked or esterified quinoa starches have the potential to improve the textural properties of bakery products. © 2024 Society of Chemical Industry.