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1.
BMC Nephrol ; 24(1): 53, 2023 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-36922759

RESUMO

BACKGROUND: Recurrent focal segmental glomerulosclerosis (FSGS) after kidney transplantation (KT) is a serious complication and a significant risk factor for graft failure. However, there is no clear evidence of the effectiveness of pre-transplant treatment using plasmapheresis (PP) or rituximab in preventing post-operative FSGS recurrence after KT. METHODS: This single-center retrospective study included 99 adult patients with biopsy-proven primary FSGS who underwent KT between 2007 and 2018. The patients were divided into the pre-treatment group (N = 53, 53.5%) and no pre-treatment group (N = 46, 46.5%). In the pre-transplant group, prophylactic PP was administered before KT in patients undergoing living donor transplantation and the day after KT in those undergoing deceased donor transplantation. RESULTS: The rate of immediate post-operative recurrence was significantly higher in the no pre-treatment group (16 [34.8%]) than in the pre-treatment group (5 [9.4%]; P = 0.002). There were three cases of graft failure due to recurrent FSGS, all of which were in the no pre-treatment group. After adjusting for possible confounding factors, age (per 10-year increase; OR = 0.61, CI, 0.42-0.90; P = 0.012) and pre-transplant treatment (vs. no pre-transplant treatment; OR = 0.17, CI, 0.05-0.54; P = 0.003) were identified as significant factors associated with FSGS recurrence. The rate of death-censored graft survival was significantly superior in the pretransplant treatment group (P = 0.042). CONCLUSION: Pre-transplant treatment with PP was associated with beneficial effects on preventing FSGS recurrence after KT.


Assuntos
Glomerulosclerose Segmentar e Focal , Transplante de Rim , Adulto , Humanos , Transplante de Rim/efeitos adversos , Glomerulosclerose Segmentar e Focal/cirurgia , Glomerulosclerose Segmentar e Focal/etiologia , Estudos Retrospectivos , Rituximab , Doadores Vivos , Plasmaferese , Recidiva
2.
Ann Transplant ; 29: e942763, 2024 Feb 06.
Artigo em Inglês | MEDLINE | ID: mdl-38319291

RESUMO

BACKGROUND Simultaneous liver-kidney transplantation (SLKT) and kidney transplantation (KT) after liver transplantation (LT) provide potential treatment options for patients with end-stage liver and kidney disease. There is increasing attention being given to liver-kidney transplantation (LTKT), particularly regarding the immune-protective effects of the liver graft. This retrospective, single-center, observational study aimed to evaluate the clinical outcomes of KT in LTKT patients - either SLKT or KT after LT (KALT) - compared to KT alone (KTA). MATERIAL AND METHODS We included patients who underwent KT between January 2005 and December 2020, comprising a total of 4312 patients divided into KTA (n=4268) and LTKT (n=44) groups. The LTKT group included 11 SLKT and 33 KALT patients. To balance the difference in sample sizes between the 2 groups, we performed 3: 1 propensity score matching (PSM). RESULTS There was no significant difference in graft survival between the groups. However, the LTKT group exhibited significantly superior rejection-free survival compared to the KTA group (P.


Assuntos
Transplante de Rim , Humanos , Estudos Retrospectivos , Transplante Homólogo , Fígado , Aloenxertos
3.
Korean J Transplant ; 37(2): 135-140, 2023 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-37435144

RESUMO

Kaposi's sarcoma (KS) is a disease that is not widely known among the general public, but has a high prevalence among organ transplant recipients. Here, we present a rare case of intragraft KS after kidney transplantation. A 53-year-old woman who had been on hemodialysis due to diabetic nephropathy underwent deceased-donor kidney transplantation on December 7, 2021. Approximately 10 weeks after kidney transplantation, her creatinine level increased to 2.99 mg/dL. Upon examination, ureter kinking was confirmed between the ureter orifices and the transplanted kidney. As a result, percutaneous nephrostomy was performed, and a ureteral stent was inserted. During the procedure, bleeding occurred due to a renal artery branch injury, and embolization was performed immediately. Subsequently, kidney necrosis and uncontrolled fever developed, leading to graftectomy. Surgical findings revealed that the kidney parenchyma was necrotic as a whole, and lymphoproliferative lesions had formed diffusely around the iliac artery. These lesions were removed during graftectomy, and a histological examination was performed. The kidney graft and lymphoproliferative lesions were diagnosed as KS based on a histological examination. We report a rare case in which a recipient developed KS in the kidney allograft as well as in adjacent lymph nodes.

4.
Sci Rep ; 13(1): 49, 2023 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-36593273

RESUMO

Simultaneous deceased donor pancreas and living donor kidney transplant (SPLK) has certain advantages over conventional simultaneous pancreas-kidney transplant (SPK) and may be beneficial for overcoming the paucity of organs needed for diabetic patients requiring transplant. We compared the clinical outcomes of patients who underwent either SPK (n = 149) or SPLK (n = 46) in terms of pre- and post-transplantation variables, development of de novo DSA, occurrence of biopsy-proven acute rejection (BPAR), and graft survival rates. There were no significant differences in the baseline characteristics between the SPK and SPLK groups except for the shorter cold ischemic time of kidney grafts, shorter duration of diabetes, older age of pancreas graft-donors, and younger age of kidney graft-donors in the SPLK group. Our results showed that the death-censored pancreas graft survival rate was lower in the SPLK group. In addition, the incidence of BPAR of the pancreas graft was higher in the SPLK group. There was no significant difference in the presence of de novo DSA and the rates of kidney graft failure, kidney BPAR, and mortality. Our results show that SPLK can be considered an alternative option for SPK although higher incidences of BPAR and graft failure of pancreas after SPLK need to be overcome.


Assuntos
Diabetes Mellitus Tipo 1 , Diabetes Mellitus , Transplante de Rim , Transplante de Pâncreas , Humanos , Transplante de Rim/efeitos adversos , Doadores Vivos , Pâncreas/cirurgia , Transplante de Pâncreas/efeitos adversos , Diabetes Mellitus/etiologia , Sobrevivência de Enxerto , Rim , Diabetes Mellitus Tipo 1/etiologia
5.
Korean J Transplant ; 37(3): 210-215, 2023 Sep 30.
Artigo em Inglês | MEDLINE | ID: mdl-37751968

RESUMO

Performing kidney transplantations in patients with morbid obesity presents unique challenges using the conventional retroperitoneal approach. Robot-assisted kidney transplantation (RAKT) offers several advantages, such as better access to hard-to-reach areas. A 56-year-old morbidly obese woman presented with end-stage renal disease due to diabetic nephropathy. The patient had a history of obesity for over 20 years, with a peak body mass index (BMI) of 46.9 kg/m2. Before transplantation, she successfully reduced her BMI to 28.9 kg/m2, but was left with excessive skin folds. The surgery began with the removal of the sac from the incisional hernia and umbilical hernia, which was then used as the site for the GelPOINT port. The da Vinci X robot system was utilized to perform RAKT. After completing RAKT, the plastic surgery team initiated abdominal reconstruction involving panniculectomy, followed by hernial reconstruction and abdominoplasty. The patient's postoperative course was uneventful, and she was discharged on postoperative day 7. Her creatinine level was 0.69 mg/dL, and she did not experience any episodes of rejection during the 16 months following RAKT. This case report describes the successful combination of RAKT with incisional hernia reconstruction and abdominoplasty in a patient with morbid obesity.

6.
Sci Rep ; 13(1): 12560, 2023 08 02.
Artigo em Inglês | MEDLINE | ID: mdl-37532735

RESUMO

Anti-thymocyte globulin (ATG) is currently the most widely prescribed induction regimen for preventing acute rejection after solid organ transplantation. However, the optimal dose of ATG induction regimen in Asian kidney recipients is unclear. Using the Korean Organ Transplantation Registry, we performed a retrospective cohort study of 4579 adult patients who received renal transplantation in South Korea and divided them into three groups according to the induction regimen: basiliximab group (n = 3655), low-dose ATG group (≤ 4.5 mg/kg; n = 467), and high-dose ATG group (> 4.5 mg/kg; n = 457). We applied the Toolkit for Weighting and Analysis of Nonequivalent Groups (TWANG) package to generate high-quality propensity score weights for intergroup comparisons. During four-year follow-ups, the high-dose ATG group had the highest biopsy-proven acute rejection rate (basiliximab 20.8% vs. low-dose ATG 22.4% vs. high-dose ATG 25.6%; P < 0.001). However, the rates of overall graft failure (4.0% vs. 5.0% vs. 2.6%; P < 0.001) and mortality (1.7% vs. 2.8% vs. 1.0%; P < 0.001) were the lowest in the high-dose ATG group. Our results show that high-dose ATG induction (> 4.5 mg/kg) was superior to basiliximab and low-dose ATG induction in terms of graft and patient survival in Asian patients undergoing kidney transplant.


Assuntos
Soro Antilinfocitário , Transplante de Rim , Adulto , Humanos , Basiliximab , Imunossupressores/uso terapêutico , Transplante de Rim/métodos , Anticorpos Monoclonais , Estudos Retrospectivos , Rejeição de Enxerto/prevenção & controle , Sobrevivência de Enxerto , Aloenxertos
7.
Biomed J ; 45(1): 19-26, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34808422

RESUMO

The ketogenic diet (KD) is a high-fat, low-carbohydrate diet, in which fat, instead of glucose, acts as a major energy source through the production of ketone bodies. The KD was formally introduced in 1921 to mimic the biochemical changes associated with fasting and gained recognition as a potent treatment for pediatric epilepsy in the mid-1990s. Recent clinical and scientific knowledge supports the use of the KD in drug-resistant epilepsy patients for its anti-seizure efficacy, safety, and tolerability. The KD is also receiving growing attention as a potential treatment option for other neurological disorders. This article will review on the recent updates on the KD, focusing on its mechanisms of action, its alternatives, expansion on its use in terms of age groups and different regions in the world, and future issues.


Assuntos
Dieta Cetogênica , Epilepsia Resistente a Medicamentos , Epilepsia , Criança , Dieta Hiperlipídica , Humanos , Corpos Cetônicos , Resultado do Tratamento
8.
Biomedicines ; 10(10)2022 Sep 21.
Artigo em Inglês | MEDLINE | ID: mdl-36289608

RESUMO

We aimed to discover and validate urinary exosomal proteins as biomarkers for antibody-mediated rejection (ABMR) after kidney transplantation. Urine and for-cause biopsy samples from kidney transplant recipients were collected and categorized into the discovery cohort (n = 36) and a validation cohort (n = 65). Exosomes were isolated by stepwise ultra-centrifugation for proteomic analysis to discover biomarker candidates for ABMR (n = 12). Of 1820 exosomal proteins in the discovery cohort, four proteins were specifically associated with ABMR: cystatin C (CST3), serum paraoxonase/arylesterase 1, retinol-binding protein 4, and lipopolysaccharide-binding protein (LBP). In the validation cohort, the level of urinary exosomal LBP was significantly higher in the ABMR group (n = 25) compared with the T-cell-mediated rejection (TCMR) group and the no major abnormality (NOMOA) group. Urinary exosomal CST3 level was significantly higher in the ABMR group compared with the control and NOMOA groups. Immunohistochemical staining showed that LBP and CST3 in the glomerulus were more abundant in the ABMR group compared with other groups. The combined prediction probability of urinary exosomal LBP and CST3 was significantly correlated with summed LBP and CST3 intensity scores in the glomerulus and peritubular capillary as well as Banff g + ptc scores. Urinary exosomal CST3 and LBP could be potent biomarkers for ABMR after kidney transplantation.

9.
Am J Physiol Endocrinol Metab ; 300(1): E86-93, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-20959529

RESUMO

Tryptophan 2,3-dioxygenase (TDO) is expressed in endometrium and catabolizes tryptophan, a precursor in the biosynthesis of serotonin. Tryptophan metabolism is an important mechanism for regulation of serotonin levels. Preimplantation mouse embryos are known to express serotonin receptors, specifically the 5-HT1D and 5-HT7 serotonin receptor subtypes. Here we demonstrate that Hoxa10 regulates endometrial TDO expression and improves embryo viability through increased serotonin production. Transfection of pcDNA-Hoxa10 to the murine uterus increased total TDO expression. In vitro, epithelial cell TDO expression was decreased after transfection with Hoxa10. Decreased glandular TDO in response to HOXA10 may augment serotonin production by increasing tryptophan availability. Conversely, stromal TDO expression increased with constitutive Hoxa10 expression. In mice, epithelial serotonin was increased in response to constitutive expression of Hoxa10. Embryo quality was impaired after treatment with Hoxa10 antisense. Blockade of serotonin receptors 1D and 7 also resulted in impaired embryo development, indicating an essential role for Hoxa10 induction of TDO and subsequent serotonin production in embryo development. Transfection of pcDNA-TDO also decreased the number of T cells in the endometrial stroma. We have shown a novel mechanism by which HOXA10 regulates endometrial TDO expression. In the endometrial stroma, HOXA10 increases TDO mRNA, which may increase tryptophan catabolism, allowing for immune tolerance at the time of embryo implantation. In endometrial glands, HOXA10 decreases TDO mRNA leading to increased serotonin that in turn acts to promote normal embryo development.


Assuntos
Embrião de Mamíferos/metabolismo , Proteínas de Homeodomínio/metabolismo , Receptor 5-HT1D de Serotonina/metabolismo , Receptores de Serotonina/metabolismo , Triptofano Oxigenase/metabolismo , Animais , Linhagem Celular , DNA Antissenso/genética , DNA Antissenso/metabolismo , Implantação do Embrião/imunologia , Embrião de Mamíferos/citologia , Embrião de Mamíferos/efeitos dos fármacos , Desenvolvimento Embrionário/efeitos dos fármacos , Endométrio/imunologia , Endométrio/metabolismo , Feminino , Proteínas Homeobox A10 , Proteínas de Homeodomínio/genética , Humanos , Tolerância Imunológica , Camundongos , Especificidade de Órgãos , RNA Mensageiro/metabolismo , Distribuição Aleatória , Antagonistas do Receptor 5-HT1 de Serotonina/farmacologia , Antagonistas da Serotonina/farmacologia , Transdução de Sinais , Linfócitos T/imunologia , Linfócitos T/metabolismo , Triptofano Oxigenase/genética , Útero/citologia , Útero/metabolismo
10.
Korean J Transplant ; 35(4): 253-256, 2021 Dec 31.
Artigo em Inglês | MEDLINE | ID: mdl-35769849

RESUMO

The impact of the coronavirus disease 2019 (COVID-19) vaccination on humoral and cellular immunity in transplant recipients remains unknown. We report the case of a 78-year-old kidney transplant recipient who experienced acute T cell-mediated rejection after receiving the second dose of the BNT162b2 mRNA COVID-19 vaccine (Pfizer-BioNTech). She had no history of acute rejection throughout the 13 years after deceased donor kidney transplantation. Fifteen days after receiving the second dose of the BNT162b2 vaccine, the recipient visited our center with a mild headache and fever. Her serum creatinine level had increased from 0.61 to 4.95 mg/dL. Kidney allograft biopsy indicated acute T cell-mediated rejection (grade IB) with no pathologic evidence of antibody-mediated rejection. Anti-severe acute respiratory syndrome coronavirus 2 spike-immunoglobulin G and -immunoglobulin M measurements were weak positive and negative, respectively. Careful monitoring of kidney allograft function is vital for transplant recipients undergoing COVID-19 vaccination.

11.
Ann Surg Treat Res ; 101(3): 187-196, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34549042

RESUMO

PURPOSE: Dipeptidyl peptidase-4 (DPP-4) inhibitors lower blood glucose levels and enhance the function of pancreatic ß cells. Yet, it is unknown whether posttransplant administration of DPP4 inhibitors is beneficial for pancreas transplant recipients. METHODS: We thus retrospectively analyzed the records of 312 patients who underwent pancreas transplantation between 2000 and 2018 at Asan Medical Center (Seoul, Korea) and compared the metabolic and survival outcomes according to DPP-4 inhibitor treatment. RESULTS: The patients were divided into the no DPP-4 inhibitor group (n = 165; no treatment with DPP-4 inhibitors or treated for <1 month) and the DPP-4 inhibitor group (n = 147; treated with DPP-4 inhibitors for ≥1 month). There were no significant differences in levels of glucose, hemoglobin A1c, and insulin between the 2 groups during 36 months of follow-up. However, the level of C-peptide was significantly higher in the DPP-4 inhibitor group at 1, 6, and 24 months posttransplant (all P < 0.05). Moreover, the DPP-4 inhibitor group had significantly higher rates of overall (log-rank test, P = 0.009) and death-censored (log-rank test, P = 0.036) graft survival during a 15-year follow-up. CONCLUSION: Posttransplant DPP-4 inhibitor administration may help improve the clinical outcomes including ß cell function after pancreas transplantation.

12.
Metab Brain Dis ; 25(2): 185-9, 2010 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-20424904

RESUMO

Mitochondrial disease is an energy metabolic disorder with various organ involvement. Iron is widely known to be one of the most important nutriments required for normal brain development and several essential metabolic functions. We retrospectively studied the laboratory data on iron deficiency (ID) in 69 children with mitochondrial respiratory chain complex (MRC) defects by biochemical enzyme assay using muscle tissue. We analyzed the differences between groups of mitochondrial disease based on the presence of ID. ID has higher prevalence in children with mitochondrial disease than in the normal population. There were 6 (9%) patients with low hemoglobin, 12 (17%) with low serum ferrtin, and 22 (32%) with low transferrin saturation levels among children with MRC defects. In comparisons between the ID and the non-ID group of MRC-defect patients, the frequency of MRC I defect was significantly higher in the ID group while that of MRC IV defect was higher in the non-ID group. Abnormal brain magnetic resonance imaging (MRI) findings were more frequently detected in the ID group. The incidence of failure to thrive and gastrointestinal symptoms were significantly higher in the ID group. Early diagnosis and proper treatment of ID are recommended. Especially in cases with risk factors such as failure to thrive or gastrointestinal manifestation, active evaluation of ID should be encouraged.


Assuntos
Anemia Ferropriva/metabolismo , Encefalopatias Metabólicas/metabolismo , Deficiências de Ferro , Doenças Mitocondriais/metabolismo , Fatores Etários , Anemia Ferropriva/sangue , Anemia Ferropriva/epidemiologia , Encefalopatias Metabólicas/sangue , Encefalopatias Metabólicas/epidemiologia , Criança , Pré-Escolar , Comorbidade , Feminino , Ferritinas/sangue , Ferritinas/deficiência , Hemoglobinas/deficiência , Humanos , Lactente , Ferro/sangue , Masculino , Doenças Mitocondriais/sangue , Doenças Mitocondriais/epidemiologia , Prevalência , Estudos Retrospectivos , Fatores de Risco , Transferrina/metabolismo
13.
Seizure ; 82: 50-55, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-33002834

RESUMO

PURPOSE: The observation of mild malformation of cortical development (mMCD) has yet to have a major clinical impact due to the lack of clinical and research data. We characterized the clinical features, surgical outcomes, and postoperative seizure control patterns in pediatric patients with mMCD. METHODS: We examined 40 patients with isolated mMCD who underwent resective surgery during a 10-year period. RESULTS: The median age at seizure onset was 1.2 years, and the median age at surgery was 7.9 years. Twenty-seven patients (67.5%) presented with childhood-onset epileptic encephalopathy (21 Lennox-Gastaut syndrome, 6 West syndrome), and 13 patients (32.5%) presented with intractable focal epilepsy (10 extratemporal lesions, 3 temporal lesions). Twenty-one patients (52.5%) showed "suspected focal cortical malformation" on MRI, whereas 16 patients (40.0%) and 3 patients (7.5%) showed normal MRI findings or mild brain atrophy, respectively. The most common surgical procedures were two lobar resections (18 patients, 45.0%), followed by unilobar resections (12 patients, 30.0%) and resections exceeding two lobar boundaries (10 patients, 25.0%). As a final surgical outcome, 24 patients (60.0%) were ILAE Class 1-3. Discontinuation of all AEDs was possible for 36.8% of ILAE Class 1 patients. Regarding the seizure control pattern, fluctuating seizure control was observed most frequently (21 patients, 52.5%). CONCLUSION: Our results suggest that mMCD is an important pathological finding in children related to a significant degree of epileptogenicity, and resective surgery can have positive outcomes. However, these patients showed unstable postoperative seizure control patterns with a high rate of late recurrence, suggesting difficulties in the surgical treatment of intractable epilepsy.


Assuntos
Epilepsia Resistente a Medicamentos , Epilepsias Parciais , Epilepsia , Malformações do Desenvolvimento Cortical , Criança , Epilepsia Resistente a Medicamentos/diagnóstico por imagem , Epilepsia Resistente a Medicamentos/cirurgia , Eletroencefalografia , Epilepsia/diagnóstico por imagem , Epilepsia/cirurgia , Humanos , Imageamento por Ressonância Magnética , Malformações do Desenvolvimento Cortical/complicações , Malformações do Desenvolvimento Cortical/diagnóstico por imagem , Malformações do Desenvolvimento Cortical/cirurgia , Estudos Retrospectivos , Convulsões , Resultado do Tratamento
14.
J Epilepsy Res ; 9(2): 87-92, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32509543

RESUMO

Surgery has been and is now a well-established treatment indicated for adults and children with drug-resistant epilepsy (DRE). The surgical landscape for children with DRE appears to be expanding, and surgical cases of pediatric epilepsy have increased significantly in the past decade, contrary to adult epilepsy. Several fundamental changes have led to the widespread surgical treatment for DRE in children, based on a risk-benefit analysis of pediatric epilepsy surgery, and a change in our overall approach to evaluation. There are unique and age-related differences associated with pediatric epilepsy surgery, characterized by different types of etiologies, concerns for developmental progress, and safety issues. Indications for "pediatric epilepsy surgery" have been broadened to include a wide spectrum of etiologies without excluding children with "generalized" seizures, "generalized or multifocal eletroencephlography", or patients with contra-lateral epileptiform activity or magnetic resonance imaging abnormalities. Furthermore, epilepsy surgery is increasingly considered in infancy and early childhood, which has similar surgical outcomes as the case of late childhood, in an effort to improve the eventual development outcome. Seizure freedom, or at least seizure reduction, is an excellent result with resolution of the associated epileptic encephalopathy, normalization of the EEG, and decrease in the total epileptic burden in the pediatric field.

15.
Medicine (Baltimore) ; 98(17): e15293, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31027091

RESUMO

The aim of this study was to evaluate the effectiveness of using Neoveil and TachoSil sponges at the pancreaticojejunostomy anastomosis site in reducing the rate and severity of postoperative pancreatic fistula (POPF).In this study, we retrospectively evaluated data that were prospectively collected on pancreaticoduodenectomy (PD) procedures. Patients were divided into 3 groups: no patch application, Neoveil patch application, and TachoSil patch application. Demographic and surgical data were analyzed.Around 165 patients with PD were enrolled in this study and were divided into 3 groups. In the standard group (n = 43), no patch was applied, while in the Neoveil and TachoSil groups (n = 84 and n = 38, respectively), the pancreaticojejunostomy anastomosis site was covered with Neoveil and TachoSil patches, respectively. POPF grade B or above occurred in 37.2% (16/43), 14.3% (12/84), and 18.8% (6/38) of patients in the standard, Neoveil and TachoSil groups, respectively, with a significant difference between patients with and without patch application (P = .004). On multivariate logistic analysis of predictive factors for POPF, male sex, patch application, and hospital day were found to be the significant independent predictors of POPF grade B or above.Significant independent predictors of POPF were male sex and patch application. This study demonstrated that the use of Neoveil or TachoSil patches may reduce the incidence of POPF after PD.


Assuntos
Fibrinogênio/uso terapêutico , Esponja de Gelatina Absorvível/uso terapêutico , Fístula Pancreática/cirurgia , Pancreaticoduodenectomia/métodos , Trombina/uso terapêutico , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Perda Sanguínea Cirúrgica , Índice de Massa Corporal , Combinação de Medicamentos , Feminino , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Duração da Cirurgia , Ácido Poliglicólico , Estudos Retrospectivos , Fatores Sexuais
16.
Pediatrics ; 142(4)2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30194277

RESUMO

OBJECTIVE: We aimed to evaluate the long-term outcome of resective epilepsy surgery in patients with Lennox-Gastaut syndrome (LGS). METHODS: We reviewed the case reports of 90 patients with LGS who had undergone resective surgery between 2003 and 2014 at the Severance Children's Hospital and managed them for a minimum period of 2 years. RESULTS: At the time of surgery, the patients were between 3.0 and 23.5 years old (mean ± SD: 9.3 ± 4.4). The time from seizure onset to surgery ranged from 0.7 to 20.1 years (7.2 ± 4.3). On postoperative follow-up for an average period of 6.1 ± 2.2 years (range: 2.1-11.4 years), 45 patients (50.0%) had no seizures, and 15 (16.7%) reported infrequent seizures. Seizure-free outcomes were achieved in 15 of the 21 (71.4%) hemispherectomies, 23 of the 51 (45.1%) multilobar resections, and 7 of the 18 (38.9%) single lobar resections. On high-resolution MRIs, 20 patients (22.2%) had negative findings, 8 of whom (40.0%) became seizure-free after resective surgery. Malformation of cortical development was the most common pathologic finding and was noted in 57 patients (63.3%). Seizure-free patients achieved better adaptive behavior and social competence than did patients with persistent seizures at the second (2-3 years after surgery) and third (4-6 years after surgery) follow-ups, as indicated by social quotients (P < .05). CONCLUSIONS: Resective surgery is a viable option in some patients to treat seizures that are associated with LGS, with a high probability of seizure control and better adaptive function.


Assuntos
Síndrome de Lennox-Gastaut/diagnóstico por imagem , Síndrome de Lennox-Gastaut/cirurgia , Procedimentos Neurocirúrgicos/tendências , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Eletroencefalografia/tendências , Feminino , Seguimentos , Humanos , Síndrome de Lennox-Gastaut/fisiopatologia , Imageamento por Ressonância Magnética/tendências , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
17.
Neurology ; 87(9): 945-51, 2016 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-27466475

RESUMO

OBJECTIVE: To analyze the clinical presentation and outcomes of surgically treated focal cortical dysplasia (FCD) in children. METHODS: We reviewed 75 cases of confirmed FCD by pathology after resective surgery. We used the pathologic classification proposed by the International League Against Epilepsy and included clinical profile and seizure and neurodevelopmental outcomes in analyses. RESULTS: There were 11 cases of FCD type I, 34 of type IIa, 19 of type IIb, and 11 of type III. Fifty-one cases (68.0%) presented clinically as focal epilepsy (FE) and 24 (32.0%) as epileptic encephalopathy (EE), including 16 with Lennox-Gastaut syndrome and 8 with West syndrome. We observed EE in 7 cases (63.6%) in FCD type I, 14 (41.2%) in type IIa, 2 (10.5%) in type IIb, and 1 (9.1%) in type III. We found the following more frequently in EE: seizure onset occurring at younger than 2 years (EE: 20 [83.3%], FE: 19 [37.3%]; p < 0.001), presence of intellectual disability before surgery (EE: 22 [91.7%], FE: 29 [56.9%]; p = 0.003), and multilobar resections (EE: 19 [79.2%], FE: 15 [29.4%]; p < 0.001). Forty-eight cases (64.0%) were seizure-free; 66.7% (34/51) in FE, 58.3% (14/24) in EE. Neurodevelopmental level showed more improvement (11/48 vs 0/27, p = 0.011) and less deterioration (2/48 vs 9/27, p = 0.001) in the seizure-free group compared to the non-seizure-free group. CONCLUSIONS: FCD can cause FE and EE in pediatric age, and resective surgery should be considered as a treatment option for both types of epilepsy.


Assuntos
Malformações do Desenvolvimento Cortical/cirurgia , Procedimentos Neurocirúrgicos/métodos , Resultado do Tratamento , Adolescente , Anticonvulsivantes/uso terapêutico , Criança , Pré-Escolar , Eletroencefalografia , Feminino , Humanos , Estimativa de Kaplan-Meier , Estudos Longitudinais , Masculino , Malformações do Desenvolvimento Cortical/tratamento farmacológico , Testes Neuropsicológicos , Estudos Retrospectivos
18.
Korean J Pediatr ; 57(5): 232-9, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-25045366

RESUMO

PURPOSE: Mitochondrial disease (MD) and Duchenne muscular dystrophy (DMD) are often associated with cardiomyopathy, but the myocardial variability has not been isolated to a specific characteristic. We evaluated the left ventricular (LV) mass by echocardiography to identify the general distribution and functional changes of the myocardium in patients with MD or DMD. METHODS: We retrospectively evaluated the echocardiographic data of 90 children with MD and 42 with DMD. Using two-dimensional echocardiography, including time-motion (M) mode and Doppler measurements, we estimated the LV mass, ratio of early to late mitral filling velocities (E/A), ratio of early mitral filling velocity to early diastolic mitral annular velocity (E/Ea), stroke volume, and cardiac output. A "z score" was generated using the lambda-mu-sigma method to standardize the LV mass with respect to body size. RESULTS: The LV mass-for-height z scores were significantly below normal in children with MD (-1.02±1.52, P<0.001) or DMD (-0.82±1.61, P=0.002), as were the LV mass-for-lean body-mass z scores. The body mass index (BMI)-for-age z scores were far below normal and were directly proportional to the LV mass-for-height z scores in both patients with MD (R=0.377, P<0.001) and those with DMD (R=0.330, P=0.033). The LV mass-for-height z score correlated positively with the stroke volume index (R=0.462, P<0.001) and cardiac index (R=0.358, P<0.001). CONCLUSION: LV myocardial atrophy is present in patients with MD and those with DMD and may be closely associated with low BMI. The insufficient LV mass for body size might indicate deterioration of systolic function in these patients.

19.
J Epilepsy Res ; 3(2): 48-53, 2013 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-24649473

RESUMO

BACKGROUND AND PURPOSE: The aim of the current study is to review the clinical features, surgery outcomes and parental satisfaction of children with destructive encephalopathy who underwent epilepsy surgery due to medically intractable seizures. METHODS: 48 patients who underwent epilepsy surgery from October 2003 to August 2011 at Severance Children's Hospital have been reviewed. The survey was conducted for functional outcomes and parental satisfaction at least 1 year after the surgery. RESULTS: Epileptic encephalopathy including Lennox-Gastaut syndrome and infantile spasms was more prevalent than symptomatic focal epilepsy. Hypoxic ischemic injury accounted for most of the underlying etiology of the destructive encephalpathy, followed by central nervous system infection and head trauma. 27 patients (56.3%) underwent resective surgery and 21 patients (43.7%) underwent palliative surgery. 16 patients (33.3%) achieved seizure free and 27 parents (87.5%) reported satisfaction with the outcome of their children's epilepsy surgery. In addition, 14 parents (77.8 %) whose children were not seizure free reported satisfaction with their children's improvement in cognitive and behavior issues. CONCLUSIONS: Epilepsy surgery in destructive encephalopathy was effective for controlling seizures. Parents reported satisfaction not only with the surgical outcomes, but also with improvement of cognitive and behavior issues.

20.
J Assist Reprod Genet ; 19(3): 144-8, 2002 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-12005310

RESUMO

Twin pregnancy consisting of complete hydatidiform mole (H-mole) and a coexisting fetus occurs with an estimated incidence of 1 per 22,000-100,000 pregnancies. The incidence of this unusual twin pregnancy with complete H-mole and a coexisting fetus after in vitro fertilization and embryo transfer (IVF-ET) is not thought to be greater than that of general population. We present an unusual twin pregnancy with complete H-mole and a coexisting fetus that occurred following IVF-ET, which was terminated at 21 weeks of gestation and developed into nonmetastatic gestational trophoblastic tumor.


Assuntos
Transferência Embrionária , Fertilização in vitro , Mola Hidatiforme , Complicações Neoplásicas na Gravidez , Gravidez Múltipla , Neoplasias Uterinas , Aborto Terapêutico , Ameaça de Aborto/etiologia , Adulto , Antimetabólitos Antineoplásicos/efeitos adversos , Antimetabólitos Antineoplásicos/uso terapêutico , Biomarcadores Tumorais/sangue , Gonadotropina Coriônica Humana Subunidade beta/sangue , Terapia Combinada , Feminino , Humanos , Mola Hidatiforme/tratamento farmacológico , Mola Hidatiforme/terapia , Recém-Nascido , Leucovorina/uso terapêutico , Masculino , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Gravidez , Hemorragia Uterina/etiologia , Neoplasias Uterinas/tratamento farmacológico , Neoplasias Uterinas/terapia
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