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Purpose@#Li-Fraumeni syndrome (LFS) is a hereditary disorder caused by germline mutation in TP53. Owing to the rarity of LFS, data on its clinical features are limited. This study aimed to evaluate the clinical characteristics and prognosis of Korean patients with LFS. @*Materials and Methods@#Patients who underwent genetic counseling and confirmed with germline TP53 mutation in the National Cancer Center in Korea between 2011 and 2022 were retrospectively reviewed. Data on family history with pedigree, types of mutation, clinical features, and prognosis were collected. @*Results@#Fourteen patients with LFS were included in this study. The median age at diagnosis of the first tumor was 32 years. Missense and nonsense mutations were observed in 13 and one patients, respectively. The repeated mutations were p.Arg273His, p.Ala138Val, and pPro190Leu. The sister with breast cancer harbored the same mutation of p.Ala138Val. Seven patients had multiple primary cancers. Breast cancer was most frequently observed, and other types of tumor included sarcoma, thyroid cancer, pancreatic cancer, brain tumor, adrenocortical carcinoma, ovarian cancer, endometrial cancer, colon cancer, vaginal cancer, skin cancer, and leukemia. The median follow-up period was 51.5 months. Two and four patients showed local recurrence and distant metastasis, respectively. Two patients died of leukemia and pancreatic cancer 3 and 23 months after diagnosis, respectively. @*Conclusion@#This study provides information on different characteristics of patients with LFS, including types of mutation, types of cancer, and prognostic outcomes. For more appropriate management of these patients, proper genetic screening and multidisciplinary discussion are required.
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Purpose@#Sentinel lymph node biopsy (SLNB) using both a radioactive isotope (RI) and blue dye is considered highly effective; however, there were limitations with the use of both agents in some hospitals, and blue dye has been shown to have some adverse effects. Additionally, preoperative prediction of sentinel lymph node (SLN) status using the maximum standardized uptake value (SUVmax) on positron emission tomography-computed tomography (PET-CT) can help avoid unnecessary axillary dissection or SLNB. Thus, we evaluated the efficacy and oncologic safety of SLNB using an RI alone in terms of long-term outcomes and determined the association between SLN metastasis and SUVmax of the primary tumor. @*Methods@#This retrospective study was conducted at Konyang University Hospital between March 2011 and May 2018. Overall, 142 patients with breast cancer who underwent SLNB using an RI alone were enrolled. Data on identification and false-negative rates were collected. The SUVmax of primary tumors on PET-CT were analyzed for their association with SLN metastasis. @*Results@#The identification and false-negative rates were 98.6% and 0%, respectively. There was no axillary local recurrence in patients with negative SLN findings. The correlation between the SUVmax of the primary tumor and SLN status was significant (r=0.249, P=0.005); the cutoff value for negative SLN metastasis was <2.15. @*Conclusion@#The single agent method using an RI is not inferior to other methods and serves as a feasible option for SLNB. And the number of excised SLNs could be minimized when the SUVmax of primary tumor is extremely low.
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Purpose@#Sentinel lymph node biopsy (SLNB) using both a radioactive isotope (RI) and blue dye is considered highly effective; however, there were limitations with the use of both agents in some hospitals, and blue dye has been shown to have some adverse effects. Additionally, preoperative prediction of sentinel lymph node (SLN) status using the maximum standardized uptake value (SUVmax) on positron emission tomography-computed tomography (PET-CT) can help avoid unnecessary axillary dissection or SLNB. Thus, we evaluated the efficacy and oncologic safety of SLNB using an RI alone in terms of long-term outcomes and determined the association between SLN metastasis and SUVmax of the primary tumor. @*Methods@#This retrospective study was conducted at Konyang University Hospital between March 2011 and May 2018. Overall, 142 patients with breast cancer who underwent SLNB using an RI alone were enrolled. Data on identification and false-negative rates were collected. The SUVmax of primary tumors on PET-CT were analyzed for their association with SLN metastasis. @*Results@#The identification and false-negative rates were 98.6% and 0%, respectively. There was no axillary local recurrence in patients with negative SLN findings. The correlation between the SUVmax of the primary tumor and SLN status was significant (r=0.249, P=0.005); the cutoff value for negative SLN metastasis was <2.15. @*Conclusion@#The single agent method using an RI is not inferior to other methods and serves as a feasible option for SLNB. And the number of excised SLNs could be minimized when the SUVmax of primary tumor is extremely low.
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Background@#To elucidate the achievement rates of imaging remission and to examine the characteristics associated with imaging remission status among patients with rheumatoid arthritis (RA) who have attained clinical remission. @*Methods@#Ninety-seven patients with RA patients who had attained clinical remission, defined by DAS28-ESR < 2.6 were enrolled. Power Doppler ultrasonography (PDUS) was performed on 16 joints and 2 tendons, including the first to third metacarpophalangeal, second and third proximal interphalangeal, radiocarpal (RC), second and third metatarsophalangeal joints, and extensor carpi ulnaris tendons. They were graded based on a dichotomous assessment. The clinical and laboratory data of patients who had attained imaging remission were compared to those of patients who had attained only clinical remission. @*Results@#The imaging remission rate was 51.5% in patients who had attained clinical remission. Forty-seven patients (48.5%) were PDUS positive. Power Doppler was detected most frequently in the right RC joint (n = 40). PDUS positive patients had higher evaluator global assessment (EGA) scores (P < 0.001) than PDUS negative patients. PDUS positive patients also had higher clinical disease activity index and simplified clinical disease activity index scores than PDUS negative patients. Patients who had attained imaging remission had lower pain scores and used nonsteroidal anti-inflammatory drugs less frequently. Patients who had attained imaging remission had higher rheumatoid factor (RF) and anti-cyclic citrullinated peptide levels. A low EGA score was found to be a predictor of imaging remission achievement among patients who had attained clinical remission. @*Conclusion@#Only 51.5% of the patients with RA who had attained clinical remission were also in imaging remission. Patients who had attained imaging remission had lower EGA scores and higher RF levels than patients who had attained only clinical remission.
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OBJECTIVE@#To compare the efficacy between acupoint selection of meridian diagnosis and regular acupoint selection for chronic atrophic gastritis (CAG).@*METHODS@#A total of 70 cases of CAG were randomly divided into an observation group (35 cases, 5 cases dropped off) and a control group (35 cases, 5 dropped off). In the observation group, according to the hand diagnosis of meridians and the results of 80-channels energy determinator, based on the principle of child-mother relation acupoint combination, the -connecting point and back- points were added for excess syndrome, and the -primary point, front- points and -sea point of foot meridians were added for deficiency syndrome; in addition, the acupoints of the eight extraordinary meridians were added based on the nature of acupoints. In the control group, Zhongwan (CV 12), Neiguan (PC 6), Zusanli (ST 36) and Gongsun (SP 4) were selected as the primary acupoints, and additional acupoints were added according to syndrome differentiation. The two groups were treated twice a week (Tuesday and Thursday, respectively), totally for 6 months. Six months after treatment, the follow-up was conducted. The clinical symptom score, gastroenteropathy patient reported outcomes (PRO) scale score before treatment, after treatment and during follow-up as well as the score of pathological changes of gastric mucosa before and after treatment were compared between the two groups.@*RESULTS@#After treatment and during follow-up, the clinical symptom scores and gastroenteropathy PRO scale scores were decreased in the two groups (<0.01, <0.001); at the follow-up, the gastroenteropathy PRO scale score in the observation group was lower than that in the control group (<0.01). After treatment, the scores of pathological changes of gastric mucosa in the two groups were decreased (<0.01), and the score in the observation group was lower than that in the control group (<0.05).@*CONCLUSION@#The acupoint selection of meridian diagnosis is superior to regular acupoint selection for CAG, which has better efficacy, more significant improvement on gastric mucosa pathology, and more stable long-term effect.
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OBJECTIVE: Coexisting chronic hepatitis C can be problematic when treating rheumatoid arthritis (RA). This study examined the changes in the transaminase and viral load in hepatitis C virus (HCV)-infected RA patients after initiating biologic agents. METHODS: A multicenter retrospective study was conducted at 12 University Hospitals in Korea between November 2014 and November 2015, and 78 RA patients, who met the 2010 American College of Rheumatology and European League Against Rheumatism classification criteria for RA and were concomitantly infected with HCV, were identified. The baseline and longitudinal clinical data, changes in liver function, and viral RNA titers were evaluated. RESULTS: Seventeen (21.8%) patients were treated with biologic agents, including etanercept (n=8), adalimumab (n=8), infliximab (n=2), tocilizumab (n=2), abatacept (n=1), and golimumab (n=1) (median 1.5 patient-years). Four patients experienced marked increases in transaminase during treatment with adalimumab (n=2) and tocilizumab (n=2). Two patients (one using adalimumab, the other using tocilizumab) were treated with anti-viral agents and showed dramatic improvement in both the viral RNA and transaminase. One patient discontinued adalimumab due to the repeated elevated transaminase levels along with a twofold increase in the viral RNA titer, and the transaminase level subsequently normalized. No case of overt viral reactivation was identified. CONCLUSION: The data support that changes in transaminase and/or viral load associated with biologic agents in HCV-infected RA patients are possible. Therefore, the liver function and viral RNA titer should be followed regularly during biologic therapy.
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Humanos , Abatacepte , Adalimumab , Antirreumáticos , Artrite Reumatoide , Fatores Biológicos , Terapia Biológica , Classificação , Etanercepte , Hepacivirus , Hepatite C , Hepatite C Crônica , Hepatite Crônica , Hospitais Universitários , Infliximab , Coreia (Geográfico) , Fígado , Estudos Retrospectivos , Doenças Reumáticas , Reumatologia , RNA Viral , Carga ViralRESUMO
The longitudinal study sought to examine the dynamic development of cognitive skills for reading among elementary-level students in Mainland China.Two groups of students in first (n=164,mean age=6.65 years at first test) and second grade (n=202,mean age=7.73 years at first test) were followed on orthographic awareness,morphological awareness and rapid automatized naming (RAN) for two years.The children exhibited significant improvement in orthographic awareness,morphological awareness and RAN from grades 1 to 4.More importantly,to the orthographic and morphological awareness,while the children took a leap from grade 1 to 2 and grade 3 to 4,the progress developed at relatively slow rates from grade 2 to 3.In order to assure children's development of orthographic and morphological awareness,evidence-based orthographically and morphologically enhanced instruction is needed for Chinese children in the early elementary years,especially for those at the stage from grade 2 to 3.
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The longitudinal study sought to examine the dynamic development of cognitive skills for reading among elementary-level students in Mainland China.Two groups of students in first (n=164,mean age=6.65 years at first test) and second grade (n=202,mean age=7.73 years at first test) were followed on orthographic awareness,morphological awareness and rapid automatized naming (RAN) for two years.The children exhibited significant improvement in orthographic awareness,morphological awareness and RAN from grades 1 to 4.More importantly,to the orthographic and morphological awareness,while the children took a leap from grade 1 to 2 and grade 3 to 4,the progress developed at relatively slow rates from grade 2 to 3.In order to assure children's development of orthographic and morphological awareness,evidence-based orthographically and morphologically enhanced instruction is needed for Chinese children in the early elementary years,especially for those at the stage from grade 2 to 3.
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PURPOSE: The aim of this study was to synthesize the best available evidence for active warming interventions during cesarean section. METHODS: A database search was done for randomized controlled trials utilizing active warming interventions. Maternal temperature, shivering and neonatal temperature were evaluated as outcome variables. Data were analyzed using Cochrane Review Manager software Version 5.3. RESULTS: Thirteen studies including 1306 patients were reviewed. The degree of lowering of maternal temperature decreased in the warmed fluids (MD 0.51; p=.004) and warming mattress interventions (MD 0.22; p<.001) compared with control groups. Incidence of shivering was also lower in the active warming group (OR 0.55; p=.003). There was no statistically significant difference in maternal temperature with a forced air warming intervention (MD 0.64; p=.15) or in neonatal temperature (MD 0.12; p=.26). CONCLUSION: Findings show that with warmed fluids and warming mattresses applied during cesarean sections maternal temperature decline was reduced and also the incidence of shivering declined, but no significant effect was observed for forced air warming interventions. These findings provide a basis for developing a warming guideline for women having a cesarean section and will help to improve the quality of care for cesarean section patients.
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Feminino , Humanos , Gravidez , Leitos , Cesárea , Hipotermia , Incidência , EstremecimentoRESUMO
Objective To compare the therapeutic efficacy betweenLing Gui Ba Fa(eight magic turtle techniques) acupuncture and ordinary acupuncture in treating irritable bowel syndrome with diarrhea (IBS-D).Method Sixty patients were randomized into a treatment group and a control group, 30 cases in each group. The treatment group was intervened byLing Gui Ba Faacupuncture at Gongsun (SP4) and Neiguan (PC6), and the control group was intervened by ordinary needling Gongsun and Neiguan. For both groups, Tianshu (ST25), Dachangshu (BL25), Shangjuxu (ST37), Fenglong (ST40), and Pishu (BL20) were added. Theclinical efficacies of the two groups were compared by evaluating the irritable bowel syndrome (IBS) clinical symptoms score and IBS Quality of Life (IBS-QOL) questionnaire. The two groups were treated 3 times a week, 10 sessions as a treatment course, andwere evaluated by using the scales before and after the treatment followed by statistical analysis and determination of the therapeutic efficacy.Result The scales were significantly improved in both groups after the treatment (P<0.05), and the improvement in the treatment group was superior to that in the control group (P<0.01); the total effective rate was 90% (27/30) in the treatment group, higher than 80% (24/30) in the control group(P<0.05).Conclusion Ling Gui Ba Faacupuncture can produce a more significant efficacy than ordinary acupuncture in treating IBS-D, and can obviously enhance the quality of life of thepatients, providing an advantageous treatment scheme to clinic.
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Urticarial vasculitis is a rare disorder that principally manifests with recurrent urticarial, sometimes hemorrhagic, skin lesions and/or angioedema. Its clinical presentation is not always limited to cutaneous lesions and it can potentially affect other organs, such as the joints, lungs, kidneys, and eyes. Systemic involvement can either be present at the onset of disease or develop over time. In cases with systemic manifestations, urticarial vasculitis is more likely to be associated with a low complement level. We present the case of a teenage boy with hypocomplementemic urticarial vasculitis syndrome (HUVS) that occurred shortly following swine-origin influenza A virus infection in 2009. Afterwards, HUVS was systemically complicated with myositis and membranous nephropathy that developed several months and about 2 years after its onset, respectively. A combination of glucocorticoid and immunosuppressive agents has been used to effectively control disease activity.
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Humanos , Masculino , Angioedema , Proteínas do Sistema Complemento , Glomerulonefrite Membranosa , Imunossupressores , Vírus da Influenza A , Articulações , Rim , Pulmão , Miosite , Pele , VasculiteRESUMO
OBJECTIVE: The aim of this study was to examine and compare the gastrointestinal (GI) risk factors and treatment patterns of rheumatoid arthritis (RA) and osteoarthritis (OA) patients in Korea. METHODS: This was a cross-sectional, observational study on RA and OA patients taking non-steroidal anti-inflammatory drugs (NSAIDs) for at least 1 month. A total of 1,896 patients (981 RA patients, 915 OA patients) were recruited from 20 university hospitals. Data were collected through medical records and patient surveys. GI risk factors included age, prolonged (over 3 months) or high-dose use of NSAIDs, alcohol drinking, smoking, use of aspirin, anticoagulants or glucocorticoids, comorbidities, and history of Helicobacter pylori infection or other GI complications. Treatment patterns were classified according to groups using, selective cyclooxygenase (COX)-2 inhibitors+/-gastro-protective agents, non-selective COX-2 inhibitors+proton pump inhibitor, or non-selective COX-2 inhibitors+/-other gastro-protective agents. RESULTS: GI risk factors were highly present in both RA and OA patients. The proportion of prolonged use of NSAIDs, smoking, and glucocorticoid use were higher in RA patients (p<0.001). The proportion of comorbidities and use of aspirin were higher in OA patients (p<0.001). The remaining GI risk factors were present in similar proportions in both groups. Use of selective COX-2 inhibitors or gastro-protective agents was higher in RA patients. CONCLUSION: Prolonged use of NSAIDs and concomitant glucocorticoid use were higher in RA patients, while comorbidities and concomitant aspirin use were predominant in OA patients. These results will provide insights for use in development of future guidelines for proper selection of NSAIDs and effective prevention of GI complications in arthritis patients.
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Humanos , Consumo de Bebidas Alcoólicas , Anti-Inflamatórios não Esteroides , Anticoagulantes , Artrite , Artrite Reumatoide , Aspirina , Comorbidade , Inibidores de Ciclo-Oxigenase 2 , Glucocorticoides , Helicobacter pylori , Hospitais Universitários , Coreia (Geográfico) , Prontuários Médicos , Estudo Observacional , Osteoartrite , Prostaglandina-Endoperóxido Sintases , Fatores de Risco , Fumaça , FumarRESUMO
Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by high heritability. Recently, autism, the most profound form of ASD, has been increasingly attributed to synaptic abnormalities. Postsynaptic density 95 (PSD95), encoding PSD protein-95, was found essential for synaptic formation, maturation and plasticity at a PSD of excitatory synapse. It is possibly a crucial candidate gene for the pathogenesis of ASD. To identify the relationship between the rs13331 of PSD95 gene and ASD, we performed a case-control study in 212 patients and 636 controls in a Chinese population by using a polymerase chain reaction-restriction fragment length polymerase (PCR-RFLP) assay. The results showed that in genetic analysis of the heterozygous model, an association between the T allele of the rs13331 and ASD was found in the dominant model (OR=1.709, 95% CI 1.227-2.382, P=0.002) and the additive model (OR=1.409, 95% CI=1.104-1.800, P=0.006). Our data indicate that the genetic mutation C>T at the rs13331 in the PSD95 gene is strikingly associated with an increased risk of ASD.
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Idoso , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtorno do Espectro Autista , Genética , Estudos de Casos e Controles , China , Proteína 4 Homóloga a Disks-Large , Peptídeos e Proteínas de Sinalização Intracelular , Genética , Proteínas de Membrana , Genética , Polimorfismo de Fragmento de Restrição , Polimorfismo de Nucleotídeo ÚnicoRESUMO
Developmental dyslexia in children is one of the neurodevelopmental disorders and is affected by various susceptible genes. In recent years, researchers have found some susceptible genes for dyslexia via chromosome analysis, genome-wide association studies, association analysis, gene function research, neuroimaging, and neurophysiological techniques. This article reviews the research advances in susceptible genes for developmental dyslexia, and with the study on susceptible genes for dyslexia, it lays a foundation for in-depth studies on the "gene-brain-behavior" level and provides scientific clues for exploring etiology and pathogenesis of dyslexia.
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Criança , Humanos , Dislexia , Genética , Fatores de Transcrição Forkhead , Genética , Predisposição Genética para Doença , Proteínas Associadas aos Microtúbulos , Genética , Proteínas do Tecido Nervoso , Genética , Proteínas Nucleares , Genética , Receptores Imunológicos , GenéticaRESUMO
Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by high heritability. Recently, autism, the most profound form of ASD, has been increasingly attributed to synaptic abnormalities. Postsynaptic density 95 (PSD95), encoding PSD protein-95, was found essential for synaptic formation, maturation and plasticity at a PSD of excitatory synapse. It is possibly a crucial candidate gene for the pathogenesis of ASD. To identify the relationship between the rs13331 of PSD95 gene and ASD, we performed a case-control study in 212 patients and 636 controls in a Chinese population by using a polymerase chain reaction-restriction fragment length polymerase (PCR-RFLP) assay. The results showed that in genetic analysis of the heterozygous model, an association between the T allele of the rs13331 and ASD was found in the dominant model (OR=1.709, 95% CI 1.227-2.382, P=0.002) and the additive model (OR=1.409, 95% CI=1.104-1.800, P=0.006). Our data indicate that the genetic mutation C>T at the rs13331 in the PSD95 gene is strikingly associated with an increased risk of ASD.
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<p><b>BACKGROUND</b>Pediatric dental fear, if left unchecked, can persist for a lifetime and adversely impact the physical and psychological health of a patient. In this study, a feasible nonmedical method for relieving pediatric dental fear was investigated.</p><p><b>METHODS</b>A randomized, single-blind, controlled trial model was applied. The juvenile patients experiencing dental fear, whose parents or guardian had signed an informed consent form, were randomly divided into two groups. Group A (n = 50) was the control group, while Group B (n = 50) was the reward group. Participants in Group A accepted routine treatment. Participants in Group B were told that they would obtain a gift as a rewarda for their good behavior if they were compliant during their dental treatments. The Chinese version of the Children's Fear Survey Schedule-Dental Subscale (CFSS-DS) was used to evaluate the level of dental fear of each patient both before and after each treatment. A contrast analysis and a correlation analysis of the results were used to assess the efficacy of the reward mechanism.</p><p><b>RESULTS</b>All participants in Group B, were obedient during the dental treatment, and they also successfully chose the present they wanted at the end of their dental treatment. Children at different ages showed different reward preferences. Significant difference in the fear scores of the participants in Group B before the treatment and after receiving the reward was found (independent samples t-test, t = 14.72, P < 0.001). In Group A, 86% children's fear score did not undergo a noticeable change.</p><p><b>CONCLUSIONS</b>A reward system is proved feasible to relieve pediatric dental fear, and the form of reward should meet the demand of patients.</p>
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Criança , Pré-Escolar , Feminino , Humanos , Masculino , Comportamento Infantil , Comportamento Cooperativo , Ansiedade ao Tratamento Odontológico , Relações Dentista-Paciente , Recompensa , Método Simples-CegoRESUMO
SAPHO syndrome is a rare inflammatory, pseudoinfectious disease. Initially it was an acronym for Syndrome Acne Pustulosis Hyperostosis Osteitis, and the meaning of S was later changed to synovitis. It occurs predominantly in children and adults and is not common over 60 years. The most common clinical presentation is osteoarticular involvement at the anterior chest wall and skin manifestations may be evident, but it could occur years earlier or develop later. We report on two cases of mother and daughter. A 51-year-old female was diagnosed with SAPHO syndrome with costochondritis and palmoplantar pustulosis. Five years later, her 31-year-old daughter presented with similar skin manifestations of the hand and foot.
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Adulto , Criança , Feminino , Humanos , Pessoa de Meia-Idade , Acne Vulgar , Síndrome de Hiperostose Adquirida , Pé , Mãos , Hiperostose , Mães , Núcleo Familiar , Osteíte , Psoríase , Manifestações Cutâneas , Sinovite , Parede TorácicaRESUMO
Type 1 myotonic dystrophy (DM1) is an autosomal-dominant inherited disorder with a multisystem involvement, caused by an abnormal expansion of the CTG sequence of the dystrophic myotonia protein kinase (DMPK) gene. DM1 is a variable multisystem disorder with muscular and nonmuscular abnormalities. Increasingly, endocrine abnormalities, such as gonadal, pancreatic, and adrenal dysfunction are being reported. But, Electrolytes imbalance is a very rare condition in patients with DM1 yet. Herein we present a 42-yr-old Korean male of DM1 with abnormally elevated serum sodium and potassium. The patient had minimum volume of maximally concentrated urine without water loss. It was only cured by normal saline hydration. The cause of hypernatremia was considered by primary hypodipsia. Hyperkalemic conditions such as renal failure, pseudohyperkalemia, cortisol deficiency and hyperkalemic periodic paralysis were excluded. Further endocrine evaluation suggested selective hyperreninemic hypoaldosteronism as a cause of hyperkalemia.
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Adulto , Humanos , Masculino , Hiperpotassemia/complicações , Hipernatremia/complicações , Hipoaldosteronismo/complicações , Capacidade de Concentração Renal , Distrofia Miotônica/complicações , Potássio/sangue , Proteínas Serina-Treonina Quinases/genética , Sódio/sangueRESUMO
An overlap syndrome is a combination of major features of more than one connective tissue diseases which is presented in the same patient. An overlap syndrome of rheumatoid arthritis (RA) and polymyositis (PM) which involved the upper pharyngeal muscle has not been reported in Korea. Herein, we report a rare case of a patient with a long-history RA presenting proximal muscle weakness and swallowing difficulty, who was successfully treated with a high-dose of corticosteroid, azathioprine and tacrolimus.
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Humanos , Artrite Reumatoide , Azatioprina , Doenças do Tecido Conjuntivo , Deglutição , Coreia (Geográfico) , Debilidade Muscular , Músculos Faríngeos , Polimiosite , TacrolimoRESUMO
Behcet's disease (BD) is a systemic vasculitis of unknown etiology that is rarely observed in association with leukemia and other hematologic disorders. We recently encountered a patient who presented with BD associated with myelofibrosis that was not attributable to other underlying causes. The patient was a 49-year-old man with a 3-year history of probable BD diagnosed by clinical findings; he was admitted because of anemia and splenomegaly. Bone marrow examination revealed myelofibrosis. After steroid therapy, the patient's symptoms of BD improved, and his hemoglobin level recovered. Therefore, we believe that the patient had BD with bone marrow involvement.