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Liver Involvement in Children with Alpha-1 Antitrypsin Deficiency: A Multicenter Study

  • Cakir, Murat;Sag, Elif;Islek, Ali;Baran, Masallah;Tumgor, Gokhan;Aydogdu, Sema
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.23 no.2
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    • pp.146-153
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    • 2020
  • Purpose: Alpha-1 antitrypsin deficiency (A1ATD) in one of the most common genetic causes of liver disease in children. We aimed to analyze the clinical characteristics and outcomes of patients with A1ATD. Methods: This study included patients with A1ATD from five pediatric hepatology units. Demographics, clinical findings, genetics, and outcome of the patients were recorded (n=25). Results: Eight patients (32.0%) had homozygous PiZZ genotype while 17 (68.0%) had heterozygous genotype. Patients with PiZZ genotype had lower alpha-1 antitrypsin levels than patients with PiMZ genotype (37.6±7.7 mg/dL vs. 66.5±22.7 mg/dL, p=0.0001). Patients with PiZZ genotype were diagnosed earlier than patients with PiMZ genotype, but this was not significant (13±6.8 months vs. 23.7±30.1 months, p=0.192). Follow-up revealed the death of one patient (12.5%) with a homozygous mutation, and revealed that one patient had child A cirrhosis, five patients (62.5%) had chronic hepatitis, and one patient (12.5%) was asymptomatic. Nine of the 17 patients with a heterozygous mutation had chronic hepatitis (52.9%), two (11.7%) had child A cirrhosis, and six (35.2%) were asymptomatic. Overall, 18 (72%) of the 25 children had liver pathology in the long-term. Conclusion: Although prevalence is rare, patients with liver disorders should be checked for alpha-1 antitrypsin levels. Moreover, long-term follow-up is essential because most patients have a liver pathology.

Oxytocin Ameliorates Remote Liver Injury Induced by Renal Ischemia-Reperfusion in Rats

  • Hekimoglu, Askin Tas;Toprak, Gulten;Akkoc, Hasan;Evliyaoglu, Osman;Ozekinci, Selver;Kelle, Ilker
    • The Korean Journal of Physiology and Pharmacology
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    • v.17 no.2
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    • pp.169-173
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    • 2013
  • Renal ischemia-reperfusion (IR) causes remote liver damage. Oxytocin has anti-inflammatory and antioxidant effects. The main purpose of this study was to evaluate the protective function of oxytocin (OT) in remote liver damage triggered by renal IR in rats. Twenty four rats were randomly divided into four different groups, each containing 8 rats. The groups were as follows: (1) Sham operated group; (2) Sham operated+OT group (3) Renal IR group; (4) Renal IR+OT group. OT ($500{\mu}g/kg$) was administered subcutaneously 12 and 24 hours before and immediately after ischemia. At the end of experimental procedure, the rats were sacrificed, and liver specimens were taken for histological assessment or determination of malondialdehyde (MDA), total oxidant status (TOS), total antioxidant status (TAS), paraoxonase (PON-1) activity and nitric oxide (NO). The results showed that renal IR injury constituted a notable elevation in MDA, TOS, Oxidative stress index (OSI) and significantly decreased TAS, PON-1 actvity and NO in liver tissue (p<0.05). Additionally renal IR provoked significant augmentation in hepatic microscopic damage scores. However, alterations in these biochemical and histopathological indices due to IR injury were attenuated by OT treatment (p<0.05). These findings show that OT ameliorates remote liver damage triggered by renal ischemia-reperfusion and this preservation involves suppression of inflammation and regulation of oxidant-antioxidant status.

Subtype Distribution of Blastocystis in Communities along the Chao Phraya River, Thailand

  • Palasuwan, Attakorn;Palasuwan, Duangdao;Mahittikorn, Aongart;Chiabchalard, Rachatawan;Combes, Valery;Popruk, Supaluk
    • Parasites, Hosts and Diseases
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    • v.54 no.4
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    • pp.455-460
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    • 2016
  • Blastocystis is a common zoonotic enteric protozoan that has been classified into 17 distinct subtypes (STs). A cross-sectional study was conducted to determine the prevalence and subtype distributions of Blastocystis in villagers living along the Chao Phraya River, Ayutthaya Province, Thailand, and to assess the risk of zoonotic infection. In total, 220 stool samples were collected, and DNA was extracted. PCR and sequencing were performed with primers targeting the small-subunit ribosomal RNA (SSU rRNA) genes. Blastocystis was present in 5.9% (13/220) of samples, and ST3 (5.0%; 11/220) was the predominant subtype, followed by ST2 (0.45%; 1/220) and ST6 (0.45%; 1/220). Phylogenetic trees were constructed with the maximum-likelihood method based on the Hasegawa-Kishino-Yano + G + I model, neighbor-joining, and maximum parsimony methods. The percentage of bootstrapped trees in which the associated taxa clustered together was relatively high. All the sequences of the Blastocystis-positive samples (KU051524-KU051536) were closely related to those from animals (pig, cattle, and chicken), indicating a zoonotic risk. Therefore, the villagers require proper health education, especially regarding the prevention of parasitic infection, to improve their personal hygiene and community health. Further studies are required to investigate the Blastocystis STs in the animals living in these villages.

Suppressive Effect of Maslinic Acid on PMA-induced Protein Kinase C in Human B-Lymphoblastoid Cells

  • Mooi, Lim Yang;Yew, Wong Teck;Hsum, Yap Wei;Soo, Khoo Kong;Hoon, Lim Saw;Chieng, Yeo Chew
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.4
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    • pp.1177-1182
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    • 2012
  • Protein kinase C (PKC) has been implicated in carcinogenesis and displays variable expression profiles during cancer progression. Studies of dietary phytochemicals on cancer signalling pathway regulation have been conducted to search for potent signalling regulatory agents. The present study was designed to evaluate any suppressive effect of maslinic acid on PKC expression in human B-lymphoblastoid cells (Raji cells), and to identify the PKC isoforms expressed. Effects of maslinic acid on PKC activity were determined using a PepTag$^{(R)}$ assay for non-radioactive detection of PKC. The highest expression in Raji cells was obtained at 20 nM PMA induced for 6 hours. Suppressive effects of maslinic acid were compared with those of four PKC inhibitors (H-7, rottlerin, sphingosine, staurosporine) and two triterpenes (oleanolic acid and ursolic acid). The $IC_{50}$ values achieved for maslinic acid, staurosporine, H-7, sphingosine, rottlerin, ursolic acid and oleanolic acid were 11.52, 0.011, 0.767, 2.45, 5.46, 27.93 and $39.29\;{\mu}M$, respectively. Four PKC isoforms, PKC ${\beta}I$, ${\beta}II$, ${\delta}$, and ${\zeta}$, were identified in Raji cells via western blotting. Maslinic acid suppressed the expression of PKC ${\beta}I$, ${\delta}$, and ${\zeta}$ in a concentration-dependent manner. These preliminary results suggest promising suppressive effects of maslinic acid on PKC activity in Raji cells. Maslinic acid could be a potent cancer chemopreventive agent that may be involved in regulating many downstream signalling pathways that are activated through PKC receptors.

Cholangiocarcinoma Patient Outcome in Northeastern Thailand: Single-Center Prospective Study

  • Luvira, Vor;Nilprapha, Kasama;Bhudhisawasdi, Vajarabhongsa;Pugkhem, Ake;Chamadol, Nittaya;Kamsa-ard, Supot
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.1
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    • pp.401-406
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    • 2016
  • Background: Cholangiocarcinoma is relatively rare worldwide. Most previous reports collected only patients with pathological diagnosis. In fact, however, many patients coming to hospital are diagnosed by clinical suspicion with radiologic imaging and receive treatment without histological confirmation. Real survival data and outcome of each treatment, especially for patients that do not have histologic confirmation, are lacking. In this study, therefore, we aimed to analyze the survival rates of CCA patients and the proportions of patients receiving different treatments. Materials and Methods: A total of 270 patients clinically suspected of CCA and visiting Srinagarind Hospital in May-July 2010, were prospectively followed until December 2014. After checking their clinical records, 163 of 270 patients were finally diagnosed as having CCA, and the data of this group were analyzed for survival rate and received treatments. Results: Of the 163 patients, 96 (58.9%) had intrahepatic, 56 (34.4%) had perihilar and 11 (6.7%) had distal CCA. The majority [107 (65.6%, 95%CI, 57.8-73.0)] received only supportive care. Overall median survival was 4 months (95%CI, 3.3-4.7), and 2-years survival was only 8.1% (95%CI,4.5-12.9). However, the 4 year survival of the R0 resection group was 100%. Conclusions: The present results show that the prognosis of CCA is very poor in North-east Thailand. Most CCA patients receive only treatment to alleviate symptoms due to their advanced stage of disease. Complete surgical resection at the early stage is the only treatment that significantly improves patient survival.

Comparison of micro-computerized tomography and cone-beam computerized tomography in the detection of accessory canals in primary molars

  • Acar, Buket;Kamburoglu, Kivanc;Tatar, Ilkan;Arikan, Volkan;Celik, Hakan Hamdi;Yuksel, Selcen;Ozen, Tuncer
    • Imaging Science in Dentistry
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    • v.45 no.4
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    • pp.205-211
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    • 2015
  • Purpose: This study was performed to compare the accuracy of micro-computed tomography (CT) and cone-beam computed tomography (CBCT) in detecting accessory canals in primary molars. Materials and Methods: Forty-one extracted human primary first and second molars were embedded in wax blocks and scanned using micro-CT and CBCT. After the images were taken, the samples were processed using a clearing technique and examined under a stereomicroscope in order to establish the gold standard for this study. The specimens were classified into three groups: maxillary molars, mandibular molars with three canals, and mandibular molars with four canals. Differences between the gold standard and the observations made using the imaging methods were calculated using Spearman's rho correlation coefficient test. Results: The presence of accessory canals in micro-CT images of maxillary and mandibular root canals showed a statistically significant correlation with the stereomicroscopic images used as a gold standard. No statistically significant correlation was found between the CBCT findings and the stereomicroscopic images. Conclusion: Although micro-CT is not suitable for clinical use, it provides more detailed information about minor anatomical structures. However, CBCT is convenient for clinical use but may not be capable of adequately analyzing the internal anatomy of primary teeth.

Comparison of radiographic and computed tomographic acetabular index in small-breed dogs: a preliminary study using Maltese and Shih Tzu

  • Kanthavichit, Kanokporn;Klaengkaew, Auraiwan;Thanaboonnipat, Chutimon;Darawiroj, Damri;Soontornvipart, Kumpanart;Choisunirachon, Nan
    • Journal of Veterinary Science
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    • v.22 no.4
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    • pp.58.1-58.13
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    • 2021
  • Background: The morphometry of the acetabulum is one source of information that assists in the clinical diagnosis of the hip and influences the proper selection of a prosthesis, reducing post-operative complications such as those seen in total hip replacement (THR). However, acetabular parameters in small-breed dogs are rarely reported. Objectives: To investigate acetabular parameters using radiography and computed tomography (CT) in small-breed dogs with Maltese and Shih Tzu dogs used as model breeds. Methods: Standard calibrated, extended hip radiographs and CT images were obtained. Subsequently, acetabular width (AW) in various directions was measured using radiography and CT, whereas acetabular depth (AD) was obtained by CT. Acetabular index (AI) is a ratio calculated from AD and AW. Results: The values of AW and AD were much higher in Shih Tzu than in Maltese dogs. Male Shih Tzus showed higher values of these parameters than females, while sex-based differences in most of the parameters could not be detected in Maltese. Body weight, but not age, influenced AWs and ADs. While AWs and ADs were influenced by several factors, AI was comparable among the assessed factors and between Maltese and Shih Tzu dogs (p = 0.172; 31.42 ± 1.35 and 32.60 ± 1.80, respectively). Also, AI did not vary with breed, sex, or body size. Conclusions: The obtained radiographic and CT acetabular parameters could be useful as guidelines for evaluating the acetabulum of small-breed dogs in clinical practice.

The effect of high fat dietary modification and nutritional status on the outcome of critically ill ventilated children: single-center study

  • El Koofy, Nehal Mohamed;Rady, Hanaa Ibrahim;Abdallah, Shrouk Moataz;Bazaraa, Hafez Mahmoud;Rabie, Walaa Ahmed;El-Ayadi, Ahmed Ali
    • Clinical and Experimental Pediatrics
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    • v.62 no.9
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    • pp.344-352
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    • 2019
  • Background: Ventilator dependency constitutes a major problem in the intensive care setting. Malnutrition is considered a major determinant of extubation failure, however, attention has been attracted to modulating carbon dioxide production through decreasing carbohydrate loading and increasing the percent of fat in enteral feeds. The detected interrelation between substrate oxidation and ventilation outcome became the base of several research to determine the appropriate composition of the nonprotein calories of diet in ventilated patients. Purpose: We aimed to assess the effect of high-fat dietary modification and nutritional status on ventilatory and final outcomes of pediatric intensive care. Methods: Fifty-one ventilated children (1 month to 12 years of age) with pulmonary disease who could be enterally fed, in the Cairo University Pediatric intensive care unit, were divided into 2 groups: group A included 25 patients who received isocaloric high-fat, low-carbohydrate diet; group B included 26 patients who received standard isocaloric diet. Comprehensive nutritional assessment was done for all patients. Results: Group A had a significant reduction in carbon dioxide tension, but no similar reduction in the duration or level of ventilatory support. Assisted minute ventilation was predicted by weight-for-age and caloric intake rather than the type of diet. Poor nutritional status was associated with higher mortality and lower extubation rates. Mild hypertriglyceridemia and some gastrointestinal intolerance were significant in group A, with no impact on the adequacy of energy or protein delivery. Conclusion: The high-fat enteral feeding protocol may contribute to reducing carbon dioxide tension, with mild hypertriglyceridemia and negligible gastrointestinal intolerance as potential adverse effects. Optimization of nutritional status rather than dietary modification may improve ventilatory and survival outcomes in critically ill-ventilated children.

Fecal Calprotectin and Phenotype Severity in Patients with Cystic Fibrosis: A Systematic Review and Meta-Analysis

  • Talebi, Saeedeh;Day, Andrew S.;Rezaiyan, Majid Khadem;Ranjbar, Golnaz;Zarei, Mitra;Safarian, Mahammad;Kianifar, Hamid Reza
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.25 no.1
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    • pp.1-12
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    • 2022
  • Inflammation plays an important role in the outcome of patients with cystic fibrosis (CF). It may develop due to cystic fibrosis transmembrane conductance regulator protein dysfunction, pancreatic insufficiency, or prolonged pulmonary infection. Fecal calprotectin (FC) has been used as a noninvasive method to detect inflammation. Therefore, the aim of the current meta-analysis was to investigate the relationship between FC and phenotype severity in patients with CF. In this study, searches were conducted in PubMed, Science Direct, Scopus, and Embase databases up to August 2021 using terms such as "cystic fibrosis," "intestine," "calprotectin," and "inflammation." Only articles published in English and human studies were selected. The primary outcome was the level of FC in patients with CF. The secondary outcome was the relationship between FC and clinical severity. Statistical analysis was performed using Comprehensive Meta-Analysis software. Of the initial 303 references, only six articles met the inclusion criteria. The mean (95% confidence interval [CI]) level of FC was 256.5 mg/dL (114.1-398.9). FC levels were significantly associated with pancreatic insufficiency (mean, 243.02; 95% CI, 74.3 to 411.6; p=0.005; I2=0), pulmonary function (r=-0.39; 95% CI, -0.58 to -0.15; p=0.002; I2=60%), body mass index (r=-0.514; 95% CI, 0.26 to 0.69; p<0.001; I2=0%), and Pseudomonas colonization (mean, 174.77; 95% CI, 12.5 to 337.02; p=0.035; I2=71%). While FC is a reliable noninvasive marker for detecting gastrointestinal inflammation, it is also correlated with the severity of the disease in patients with CF.

DNA microarray-based characterization and antimicrobial resistance phenotypes of clinical MRSA strains from animal hosts

  • Schmitt, Sarah;Stephan, Roger;Huebschke, Ella;Schaefle, Daniel;Merz, Axel;Johler, Sophia
    • Journal of Veterinary Science
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    • v.21 no.4
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    • pp.54.1-54.11
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    • 2020
  • Background: Methicillin-resistant Staphylococcus aureus (MRSA) is a leading cause of severe infections in humans and animals worldwide. Studies elucidating the population structure, staphylococcal cassette chromosome mec types, resistance phenotypes, and virulence gene profiles of animal-associated MRSA are needed to understand spread and transmission. Objectives: The objective of this study was to determine 1) clonal complexes and spa types, 2) resistance phenotypes, and 3) virulence/resistance gene profiles of MRSA isolated from animals in Switzerland. Methods: We analyzed 31 presumptive MRSA isolates collected from clinical infections in horses, dogs, cattle, sheep, and pigs, which had tested positive in the Staphaurex Latex Agglutination Test. The isolates were characterized by spa typing and DNA microarray profiling. In addition, we performed antimicrobial susceptibility testing using the VITEK 2 Compact system. Results: Characterization of the 31 presumptive MRSA isolates revealed 3 methicillinresistant Staphylococcus pseudintermedius isolates, which were able to grow on MRSA2 Brilliance agar. Of the 28 MRSA isolates, the majority was assigned to CC398 (86%), but CC8 (11%) and CC1 (4%) were also detected. The predominant spa type was t011 (n = 23), followed by t009 (n = 2), t034 (n = 1), t008 (n = 1), and t127 (n = 1). Conclusions: The results of this study extend the current body of knowledge on the population structure, resistance phenotypes, and virulence and resistance gene profiles of MRSA from livestock and companion animals.