Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (antiCFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.
Journal of The Korean Society of Clinical Toxicology
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v.18
no.2
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pp.116-122
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2020
Acute respiratory failure is an important risk factor for mortality in patients with acute pesticide poisoning. Therefore, it is necessary to investigate the risk factors to predict respiratory failure in these patients. This study retrospectively investigated the clinical features of respiratory failure among patients with acute pesticide poisoning requiring mechanical ventilation. This study included patients who were admitted with intentional poisoning by pesticide ingestion from January 2017 to December 2019. Paraquat intoxication was excluded. Among 469 patients with acute pesticide poisoning, 398 patients were enrolled in this study. The respiratory failure rate was 30.4%. The rate of respiratory failure according to the type of pesticide was carbamate (75.0%), organophosphate (52.6%), glufosinate (52.1%), glyphosate (23%), pyrethroid (8.9%), and others (17%). The mortality was 25.6% in the respiratory failure group. The risk factors for respiratory failure were old age, low body mass index, and ingestion of more than 300 mL. In conclusion, respiratory failure is a risk factor for mortality in pesticide poisoning. Old age, low body mass index, and ingestion of more than 300 mL are the risk factors for predicting respiratory failure.
Objective: The aim of the study was to investigate pregnancy, obstetric, and neonatal outcomes in women with small (<4 cm) unilateral endometriomas. Methods: This retrospective study included 177 patients: 91 patients with small endometriomas and 86 controls with unexplained or tubal factor infertility who were treated at the Süleymaniye Gynecology and Maternity Training and Research Hospital Infertility Unit between January 2010 and July 2015. The groups were matched with regards to demographic characteristics such as age, body mass index, and infertility duration. All of the women in this study conceived via intracytoplasmic sperm injection. We compared pregnancy, obstetric, and neonatal outcomes between these groups. Results: Women with endometriomas had a higher biochemical pregnancy rate, but lower clinical pregnancy and live birth rates than women with unexplained and tubal factor infertility (p<0.05 for all). However no significant differences were found in terms of obstetric and neonatal complications between the two groups (p>0.05 for all). Conclusion: In this study, we found that women with endometriomas less than 4 cm were more prone to early pregnancy complications. We also showed that this group did not have any increased risks of late pregnancy, obstetric, and neonatal complications.
Purpose: There is inconsistent evidence about the association between fetal nuchal cord and the risk of autism spectrum disorder (ASD). We performed a meta-analysis to investigate whether fetal nuchal cord is associated with an increased risk of ASD in offspring. Methods: Three main English databases were searched until January 2021. The Newcastle-Ottawa Scale was used to assess study quality. Study heterogeneity was determined using the I 2 statistic, while publication bias was assessed using Begg and Egger tests. Results are presented as odds ratios (ORs) and relative ratios with 95% confidence intervals (CI) and were determined by a random-effects model. Results: Five articles (1 cohort, 4 case-control; 3,088 total children) were included in the present meta-analysis. Fetal nuchal cord was not a risk factor for ASD (OR, 1.11; 95% CI, 0.66-1.57). There was homogeneity among studies that reported a risk of ASD (I2=0.0%). Conclusion: Our results showed that fetal nuchal cord is not a risk factor for ASD. Future large cohort studies should confirm this finding.
Background: Oral squamous cell carcinoma (OSCC) remains as one of the most difficult malignancies to control because of its high propensity for local invasion and cervical lymph node dissemination. The aim of present study was to evaluate the efficacy of novel pH and temperature sensitive doxorubicin-methotrexate-loaded nanoparticles (DOX-MTX NP) in terms of their potential to change the VEGF-C expression profile in a rat OSCC model. Materials and Methods: 120 male rats were divided into 8 groups of 15 animals administrated with 4-nitroquinoline-1-oxide to induce OSCCs. Newly formulated doxorubicin-methotrexate-loaded nanoparticles (DOX-MTX NP) and free doxorubicin were IV and orally administered. Results: Results indicated that both oral and IV forms of DOX-MTX-nanoparticle complexes caused significant decrease in the mRNA level of VEGF-C compared to untreated cancerous rats (p<0.05). Surprisingly, the VEGF-C mRNA was not affected by free DOX in both IV and oral modalities (p>0.05). Furthermore, in DOX-MTX NP treated group, less tumors characterized with advanced stage and VEGF-C mRNA level paralleled with improved clinical outcome (p<0.05). In addition, compared to untreated healthy rats, the VEGF-C expression was not affected in healthy groups that were treated with IV and oral dosages of nanodrug (p>0.05). Conclusions: VEGF-C is one of the main prognosticators for lymph node metastasis in OSCC. Down-regulation of this lymph-angiogenesis promoting factor is a new feature acquired in group treated with dual action DOX-MTX-NPs. Beside the synergic apoptotic properties of concomitant use of DOX and MTX on OSCC, DOX-MTX NPs possessed anti-angiogenesis properties which was related to the improved clinical outcome in treated rats. Taking together, we conclude that our multifunctional doxorubicin-methotrexate complex exerts specific potent apoptotic and anti-angiogenesis properties that could ameliorate the clinical outcome presumably via down-regulating dissemination factor-VEGF-C expression in a rat OSCC model.
Background: The liver is one of the most common metastatic sites of breast cancer, hepatic metastases developing in 6%-25% of patients with breast cancer and being associated with a poor prognosis. The aim of this study was to analyze the survival and clinical characteristics of patients with hepatic metastases from breast cancer of different molecular subtypes and to investigate the prognostic and predictive factors that effect clinical outcome. Methods: We retrospectively studied the charts of 104 patients with breast cancer hepatic metastases diagnosed at Sun Yat-sen University Cancer Center from December 1990 to June 2009. Subtypes were defined as luminal A, luminal B, human epidermal growth factor receptor 2 (HER2) enriched, triple-negative (TN). Prognostic factor correlations with clinical features and treatment approaches were assessed at the diagnosis of hepatic metastases. Results: The median survival time was 16.0 months, and the one-, two- three-, four-, five-year survival rates were 63.5%, 31.7%, 15.6%, 10.8%, and 5.4%, respectively. Median survival periods after hepatic metastases were 19.3 months (luminal A), 13.3 months (luminal B), 18.9 months (HER2-enriched), and 16.1 months (TN, P=0.11). In multivariate analysis, a 2 year-interval from initial diagnosis to hepatic metastasis, treatment with endocrine therapy, and surgery were independent prognostic factors. Endocrine therapy could improve the survival of luminal subtypes (P=0.004) and was a favorable prognostic factor (median survival 23.4 months vs. 13.8 months, respectively, P=0.011). Luminal A group of patients treated with endocrine therapy did significantly better than the Luminal A group of patients treated without endocrine therapy (median survival of 48.9 vs. 13.8 months, P=0.003). Conclusions: Breast cancer subtypes were not associated with survival after hepatic metastases. Endocrine therapy was a significantly favorable treatment for patients with luminal subtype.
Kim, Hee-Joung;Kim, Wan-Seop;Shin, Kyeong-Cheol;Lee, Gwan-Ho;Kim, Mi-Jin;Lee, Jeong-Eun;Song, Kyu-Sang;Kim, Sun-Young;Lee, Kye-Young
Tuberculosis and Respiratory Diseases
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v.70
no.1
/
pp.21-27
/
2011
Background: Although the gold standard method for research trials on epidermal growth factor receptor (EGFR) mutations has been direct sequencing, this approach has the limitations of low sensitivity and of being time-consuming. Peptide nucleic acid (PNA)-mediated polymerase chain reaction (PCR) clamping is known to be a more sensitive detection tool. The aim of this study was to compare the detection rate of $EGFR$ mutation and EGFR-tyrosine kinase inhibitor (TKI) responsiveness according to $EGFR$ mutation status using both methodologies. Methods: Clinical specimens from 112 NSCLC patients were analyzed for $EGFR$ mutations in exons 18, 19, 20, and 21. All clinical data and tumor specimens were obtained from 3 university hospitals in Korea. After genomic DNA was extracted from paraffin-embedded tissue specimens, both PNA-mediated PCR clamping and direct-sequencing were performed. The results and clinical response to $EGFR$-TKIs were compared. Results: Sequencing revealed a total of 35 (22.9%) mutations: 8 missense mutations in exon 21 and 26 deletion mutations in exon 19. PNA-mediated PCR clamping showed the presence of genomic alterations in 45 (28.3%) samples, including the 32 identified by sequencing plus 13 additional samples (6 in exon 19 and 7 in exon 21). Conclusion: PNA-mediated PCR clamping is simple and rapid, as well as a more sensitive method for screening of genomic alterations in $EGFR$ gene compared to direct sequencing. This data suggests that PNA-mediated PCR clamping should be implemented as a useful screening tool for detection of $EGFR$ mutations in clinical setting.
Kim, Do-Hoon;Park, So-Jeong;Lee, Seung-Yeon;Yoon, Hyun-Seo;Park, Chung Mu
Korean Journal of Clinical Laboratory Science
/
v.50
no.3
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pp.337-344
/
2018
Hepatocellular carcinoma (HCC), a major type of hepatoma, is associated with high recurrence and mortality because of its uncontrolled metastatic feature. Silymarin is a polyphenolic flavonoid from Silybum marianun (milk thistle) and exhibits anti-carcinogenic activity through modulation of the epithelial-mesenchymal transition (EMT) in several cancer cells. In this study, the inhibitory mechanism of silymarin against migration and invasion was investigated in the Huh7 HCC cell line. Wound healing and in vitro invasion assays were conducted to examine the effects of silymarin on migration and invasion. Western blot analysis was also applied to evaluate the inhibitory effects of silymarin on the EMT-related genes and their upstream signaling molecules. Silymarin inhibited the migratory and invasive activities of Huh7 cells. In addition, silymarin attenuated the protein expression levels of vimentin and matrix metalloproteinase (MMP)-9 as well as their transcription factors, Snail, and nuclear factor $(NF)-{\kappa}B$, while the expression of E-cadherin was increased by the silymarin treatment. Among the upstream signaling molecules, the phosphorylation of Akt was inhibited by the silymarin treatment, which was confirmed by the selective inhibitor, LY294002. Consequently, silymarin inhibited the invasive and migratory activities in Huh7 cells through the modulation of EMT-related gene expression by the PI3K/Akt signaling pathway, which may have potential as a chemopreventive agent against HCC metastasis.
Park, Hong-Jun;Gwak, Ho-Shin;Hwang, Sung-Kyun;Kim, Jeong Eun;Lee, Sang Hyung;Jung, Hee-Won;Kim, Dong Gyu;Cho, Byung-Kyu
Journal of Korean Neurosurgical Society
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v.30
no.9
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pp.1103-1107
/
2001
Objective : Atypical and malignant meningiomas(AM, MM) are known to be rare and show aggressive behavior. Limited data are available concerning the clinical features, effectiveness of surgical removal and role of radiation therapy with AM, MM. The authors report our experience of AM, MM, with respect to clinical features. Methods : Twenty-four cases of AM and 28 cases of MM, who were operated between 1988 and 1999 were retro-spectively studied review of medical records and radiological findings. These were compared with control group of 24 cases of menigiomas manifestating usual clinical course, which are selected arbitrarily among operative cases between Apr. 1999 and Dec. 1999. Mean follow-up periods were 26(1-91) months for AM and 23(1-62) for MM. Authors analyzed the prognostic factors for survival, and statistical analysis were accomplished by Kaplan-Meier method and log-rank test. Results : Differences of clinical features between control groups and these atypical and malignant meningioma group were not significant. However, the location of MM was frequent in non-basal area(p<0.01). In AM, there were 4 patients of recurrence, and 3 patients of mortality. Among mortality cases, only one patient died of tumor progression, the other patients died of other causes. The survival at 2 year and 5 year in this group were 88% and 74% respectively, and in MM, 11 patients died due to tumor progression and 2 had spinal metastasis. The survival at 2 year and 5 year were 72% and 20%, respectively. For extent of resection, total removal(Simpson grade 1 or 2) was less often achieved in MM compared with AM(50% vs. 83%). Extent of resection of tumor and postoperative radiation therapy did not affect survival in both AM, MM. Conclusions : Clinical behavior of AM showed more benign than that of MM. Prognostic factor for survival is not related extent of resection of tumor and postoperative radiation therapy. However, further investigation with long-term follow-up and additional cases is mandatory.
Purpose: To identify baseline prognostic factors for survival in patients with disease progression, during or after chemotherapy for the treatment of advanced gastric or gastroesophageal junction (GEJ) cancer. Materials and Methods: We pooled data from patients randomized between 2009 and 2012 in 2 phase III, global double-blind studies of ramucirumab for the treatment of advanced gastric or GEJ adenocarcinoma following disease progression on first-line platinum- and/or fluoropyrimidine-containing therapy (REGARD and RAINBOW). Forty-one key baseline clinical and laboratory factors common in both studies were examined. Model building started with covariate screening using univariate Cox models (significance level=0.05). A stepwise multivariable Cox model identified the final prognostic factors (entry+exit significance level=0.01). Cox models were stratified by treatment and geographic region. The process was repeated to identify baseline prognostic quality of life (QoL) parameters. Results: Of 1,020 randomized patients, 953 (93%) patients without any missing covariates were included in the analysis. We identified 12 independent prognostic factors of poor survival: 1) peritoneal metastases; 2) Eastern Cooperative Oncology Group (ECOG) performance score 1; 3) the presence of a primary tumor; 4) time to progression since prior therapy <6 months; 5) poor/unknown tumor differentiation; abnormally low blood levels of 6) albumin, 7) sodium, and/or 8) lymphocytes; and abnormally high blood levels of 9) neutrophils, 10) aspartate aminotransferase (AST), 11) alkaline phosphatase (ALP), and/or 12) lactate dehydrogenase (LDH). Factors were used to devise a 4-tier prognostic index (median overall survival [OS] by risk [months]: high=3.4, moderate=6.4, medium=9.9, and low=14.5; Harrell's C-index=0.66; 95% confidence interval [CI], 0.64-0.68). Addition of QoL to the model identified patient-reported appetite loss as an independent prognostic factor. Conclusions: The identified prognostic factors and the reported prognostic index may help clinical decision-making, patient stratification, and planning of future clinical studies.
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