RESUMO
Multicentric squamous dysplasia of the esophagus is characterized by multiple Lugol-voiding lesions (LVLs) on Lugol chromoendoscopy. Multiple LVLs are associated with a very high risk of multiple cancers arising in the esophagus as well as the head and neck. To gain insight into the pathogenesis of multiple LVLs of the esophageal mucosa, we studied risk factors for the development of such lesions in 76 patients who had a current or previous diagnosis of esophageal squamous cell carcinoma. All patients underwent Lugol chromoendoscopy of the esophageal mucosa. The history of tobacco and alcohol use was documented. Polymorphisms of the aldehyde dehydrogenase type 2 (ALDH2) gene were identified by polymerase chain reaction using sequence-specific primers. Clinical factors related to multiple LVLs were analyzed. All patients with multiple LVLs were drinkers. On univariate analysis, male sex (odds ratio [OR] 15, 95% confidence interval [CI] 1.84-122.45: P = 0.011), presence of the ALDH2-2 allele (OR 4.5, 95% CI 1.55-13.24: P = 0.006), and smoking index ≥1000 (OR 2.6, 95% CI 1.02-6.6: P = 0.045) were associated with multiple LVLs. On multivariate analysis, male sex (OR 10.02, 95% CI 1.13-88.44: P = 0.038) and presence of the ALDH2-2 allele (OR 4.56, 95% CI 1.4-14.82: P = 0.012) were associated with multiple LVLs. Among drinkers, a daily alcohol intake of ≥100 g pure ethanol with the ALDH2-2 allele (OR 17.5, 95% CI 1.97-155.59: P = 0.01) and a daily alcohol intake of <100 g pure ethanol with the ALDH2-2 allele (OR 8.85, 95% CI 1.68-46.69: P = 0.01) more strongly correlated with multiple LVLs than did a daily alcohol intake of <100 g pure ethanol without the ALDH2-2 allele, whereas a daily alcohol intake of ≥100 g pure ethanol without the ALDH2-2 allele (OR 4.0, 95% CI 0.54-29.81: P = 0.18) did not. In conclusion, male sex and the ALDH2-2 allele are associated with an increased risk for multiple LVLs of the esophageal mucosa in patients with esophageal squamous cell carcinoma. Among drinkers with the ALDH2-2 allele, the risk of multiple LVLs increased in parallel to the daily alcohol intake.
Assuntos
Carcinoma de Células Escamosas/patologia , Neoplasias Esofágicas/patologia , Mucosa Respiratória/patologia , Idoso , Consumo de Bebidas Alcoólicas/efeitos adversos , Aldeído Desidrogenase/genética , Aldeído-Desidrogenase Mitocondrial , Alelos , Corantes , Esofagoscopia , Feminino , Humanos , Iodetos , Masculino , Análise Multivariada , Polimorfismo Genético , Estudos Prospectivos , Fatores de Risco , Fatores SexuaisRESUMO
BACKGROUND AND STUDY AIMS: Endoscopic submucosal dissection (ESD) has become a standard treatment. However, the treatment time tends to be relatively long and insufflation and manipulation of the endoscope can increase pain and discomfort. We aimed to find an optimal method for sedation during ESD. PATIENTS AND METHODS: Patients scheduled to undergo ESD for early gastric cancer or adenoma were randomly assigned to sedation with midazolam or propofol, and consciousness level was evaluated by bispectral index (BIS) monitoring. Primary end points of effectiveness (three parameters) and secondary end points of safety during ESD and after return to the ward were compared between the groups. Study registration was in the UMIN Clinical Trial Registry (UMIN 000001497), and the institutional trial number was KDOG 0801. RESULTS: From June 2008 through June 2009, we enrolled 178 patients (90 midazolam, 88 propofol). Regarding safety after ESD, recovery was significantly better in the propofol group immediately after and at 1 hour and 2 hours after return to the ward (P < 0.001). The number of patients who required a continuous supply of oxygen 2 hours after returning to the ward was significantly lower in the propofol group (midazolam 18; propofol 6; P = 0.010). Though propofol seemed to be better for effectiveness and safety, there were no statistically significant differences for all three primary end points and the safety parameters (hypotension, hypoxia, bradycardia). CONCLUSIONS: Propofol with BIS monitoring improved recovery of patients after ESD, though this study was underpowered to prove the effectiveness and safety of propofol.
Assuntos
Adenoma/cirurgia , Anestésicos Intravenosos/administração & dosagem , Sedação Profunda , Dissecação , Propofol/administração & dosagem , Neoplasias Gástricas/cirurgia , Idoso , Idoso de 80 Anos ou mais , Anestésicos Intravenosos/efeitos adversos , Bradicardia/induzido quimicamente , Distribuição de Qui-Quadrado , Monitores de Consciência , Feminino , Mucosa Gástrica/cirurgia , Gastroscopia , Humanos , Hipotensão/induzido quimicamente , Hipóxia/induzido quimicamente , Masculino , Midazolam/administração & dosagem , Midazolam/efeitos adversos , Pessoa de Meia-Idade , Oxigenoterapia , Propofol/efeitos adversos , Estatísticas não ParamétricasRESUMO
BACKGROUND AND OBJECT: An antiulcer agent, ecabet sodium, is active against Helicobacter pylori. The aim of the present study was to clinically examine whether eradication therapy, which includes ecabet sodium, is effective in eradication of H. pylori after failure of first-line therapy. METHODS: Patients with peptic ulcer who failed with first-line triple eradication therapy containing clarithromycin received quadruple therapy with omeprazole (20 mg, twice daily), amoxicillin (750 mg, twice daily), metronidazole (500 mg, twice daily) and ecabet sodium (1000 mg, twice daily) for 14 days. Eradication of H. pylori was judged by 13C-urea breath test 8 weeks later. RESULTS: Fifty-two patients (36 men and 16 women) were included. Their mean age was 51.4 years (range 28-73). One patient dropped out because of diarrhoea. The eradication rate was 98.0% (50/51) according to the per-protocol analysis and 96.2% (50/52) according to the intention-to-treat analysis. Side effects occurred in seven patients, but none were serious. CONCLUSIONS: Quadruple therapy including ecabet sodium is useful as second-line eradication treatment for H. pylori.
Assuntos
Antibacterianos/uso terapêutico , Antiulcerosos/uso terapêutico , Infecções por Helicobacter/tratamento farmacológico , Helicobacter pylori/efeitos dos fármacos , Abietanos/administração & dosagem , Abietanos/efeitos adversos , Abietanos/uso terapêutico , Adulto , Idoso , Amoxicilina/administração & dosagem , Amoxicilina/efeitos adversos , Amoxicilina/uso terapêutico , Antibacterianos/administração & dosagem , Antibacterianos/efeitos adversos , Antiulcerosos/administração & dosagem , Antiulcerosos/efeitos adversos , Quimioterapia Combinada , Feminino , Infecções por Helicobacter/microbiologia , Helicobacter pylori/isolamento & purificação , Humanos , Masculino , Metronidazol/administração & dosagem , Metronidazol/efeitos adversos , Metronidazol/uso terapêutico , Pessoa de Meia-Idade , Omeprazol/administração & dosagem , Omeprazol/efeitos adversos , Omeprazol/uso terapêutico , Úlcera Péptica/tratamento farmacológico , Úlcera Péptica/microbiologia , Projetos Piloto , Resultado do TratamentoRESUMO
INTRODUCTION: Pediatric hepatocellular carcinoma (HCC) is an uncommon disease with a poor prognosis. There are few reports about liver transplantation for pediatric adult-type HCC. We experienced a case of living donor liver transplantation (LDLT) for a child with recurrent pediatric adult-type HCC. CASE REPORT: A 12-year-old boy was admitted to the Department of Pediatrics in our institution due to HCC in May 2005. He underwent hepatectomy after 3 courses of chemotherapy in July 2005. After the operation, he had 2 more courses of the same chemotherapy. His posttheraputic course was uneventful for 1 year. However, his alpha-fetoprotein level increased and a computed tomography (CT) scan showed recurrent tumor in his remnant liver in October 2006. He underwent another chemotherapy session immediately. However, CT revealed multiple liver tumors after chemotherapy in December 2006. His mother requested to be an LDLT donor, which was performed on January 23, 2007. The donor operation was a right hepatic lobectomy. The postoperative course of the donor was unremarkable and she has now returned to work. The recipient's posttransplantation course was uneventful and he was discharged at postoperative day 53 and is currently doing well. CONCLUSION: Liver transplantation in conjunction with chemotherapy may have an increasing role in the management of pediatric HCC.
Assuntos
Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/cirurgia , Doadores Vivos , Adulto , Criança , Feminino , Hepatectomia , Humanos , Masculino , Recidiva Local de Neoplasia , Resultado do Tratamento , alfa-Fetoproteínas/metabolismoRESUMO
BACKGROUND: Living donor liver transplantation (LDLT) is a definitive procedure for splenomegaly caused by liver cirrhosis and portal hypertension, but splenomegaly persists in some patients. The aim of this study was to clarify the long-term changes in the spleen volume after LDLT. METHODS: The 13 pediatric patients who survived for >8 years after LDLT were retrospectively analyzed. We calculated the spleen volume/standard spleen volume (SV/SSV) ratio by automated computed tomography (CT) volumetry. We assessed the spleen volumes before LDLT, at roughly postoperative week (POW) 4, at postoperative year (POY) 1, at POY 5, and at POY 10. RESULTS: With regard to SV as evaluated by CT volumetry, there were no consistent trends, with median values as follows: before LDLT, 282.5 (71-641) cm3; POW 4, 252 (109-798) cm3; POY 1, 222.5 (97-948) cm3; POY 5, 263.5 (123-564) cm3; and POY 10, 377 (201-1080) cm3. In contrast, the SV/SSV ratio decreased chronologically as follows: before LDLT, 5.0 (0.7-6.0); POW 4, 3.7 (2.3-4.3); POY 1, 2.2 (1.7-6.3); POY 5, 1.7 (1.1-5.4); and POY 10, 1.4 (1.1-6.9). In the remote phase after LDLT, many cases showed a trend toward an improved SV/SSV ratio, but splenomegaly was prolonged without improvement in 3 cases (23.1%) with portal vein complications and advanced fibrosis. Furthermore, all 3 cases showed a decreased platelet count due to hypersplenism. CONCLUSION: Splenomegaly requires a long time to demonstrate an improvement. In cases without an improvement of splenomegaly, we should suspect abnormalities in the graft liver and portal hemodynamics.
Assuntos
Transplante de Fígado/efeitos adversos , Esplenomegalia/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Doadores Vivos , Masculino , Estudos Retrospectivos , Esplenomegalia/epidemiologiaRESUMO
BACKGROUND: The relationship between smoking cessation and weight gain is well recognized. Examining the link between smoking cessation and weight gain in donor candidates for living donor liver transplantation (LDLT) is an important topic because of the influence of weight gain on the liver. This study assessed body weight (BW) changes after smoking cessation in donor candidates for LDLT. METHODS: The 27 donor candidates were retrospectively analyzed. The smoking status was determined based on questionnaires administered at the initial presentation, and the candidates were divided into 2 groups: recent quitters and nonsmokers. The changes in BW were compared between the groups. RESULTS: The recent quitters group included 10 (37.0%) candidates, and the nonsmokers group included 17 (63.0%). In the nonsmokers group, 1 candidate had gained weight since the initial presentation. In contrast, in the recent quitters group, 70.0% of candidates had gained weight since the initial presentation (P < .01). The change in BW from the initial presentation was greater in recent quitters than in nonsmokers (+1.6 kg [+2.4%] vs -0.5 kg [-0.9%]; P < .01). Two candidates in the recent quitters group gained ≥ 5 kg [8%] of weight. One of these 2 candidates was judged to be in a donor-inadequate status because of the appearance of fatty liver. CONCLUSIONS: Weight gain due to smoking cessation was observed in donor candidates for LDLT. The amount of weight gain after smoking cessation is highly individualized, so everyone concerned with LDLT must be alert to its potential development.
Assuntos
Transplante de Fígado/métodos , Doadores Vivos , Abandono do Hábito de Fumar , Aumento de Peso , Adulto , Peso Corporal , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Inquéritos e QuestionáriosRESUMO
When there is an anatomic anomaly in the biliary tract of the donor for living-donor liver transplantation, the risk of postoperative biliary tract complications increases in both the donor and the recipient. We studied a case of living-donor liver transplantation with a left hepatic lobe graft that had anatomic anomalies, in which the medial segmental branch (B4) joined the anterior segmental branch and the posterior segmental branch formed a common trunk with the lateral segmental branch. A 40-year-old man visited our institution as a candidate organ donor for his mother, who had end-stage liver failure. An anomaly of B4 connecting the anterior segmental branch was suspected on magnetic resonance cholangiopancreatography. On intraoperative cholangiography, confluence of B4 with the anterior segmental branch and connection of the posterior and lateral segmental branches forming a common trunk were confirmed. Accordingly, individual anastomoses of the lateral segmental branch and B4 with the recipient jejunum were planned, and a left-lobe graft was excised. The postoperative recovery was smooth, and the donor was discharged with no complications. Even when an anatomic anomaly is present in the donor bile duct, in urgent cases, accurate evaluation through the use of various modalities may enable living-donor liver transplantation with the use of a graft with an anatomic anomaly.
Assuntos
Sistema Biliar/anormalidades , Transplante de Fígado/métodos , Fígado/anormalidades , Doadores Vivos , Transplantes/anormalidades , Adulto , Ductos Biliares/anormalidades , Ductos Biliares/transplante , Colangiografia , Doença Hepática Terminal/cirurgia , Humanos , Transplante de Fígado/efeitos adversos , Masculino , Complicações Pós-Operatórias/etiologia , Transplantes/transplanteRESUMO
OBJECTIVE: The duration of illness is quite long in some anorexia nervosa (AN) patients. In the present study, we investigated the psychopathological features of patients with prolonged AN as assessed by the Minnesota Multiphasic Personality Inventory-1 (MMPI-1). METHODS: Fifty-five AN patients completed the MMPI-1 on admission to Kyushu University Hospital from 1999 to 2002. The patients were divided into three groups on the basis of their illness duration: a short-term group, less than 5 years of illness duration (n=31); a middle-term group, from 5 to 10 years (n=14); and a prolonged group, 10 years or more (n=10). RESULTS: The prolonged group scored significantly higher on the MPPI-1 scales of hysteria (Hy), low back pain (Lb) and family conflict than the short-term group. DISCUSSION: AN patients whose illness duration was prolonged characteristically had more hysteria with family conflict. This should be considered in their treatment.
Assuntos
Anorexia Nervosa/psicologia , Histeria , Transtornos Mentais/psicologia , Transtornos da Personalidade , Adolescente , Adulto , Criança , Conflito Psicológico , Relações Familiares , Feminino , Humanos , Estudos Longitudinais , Dor Lombar , Inventário de Personalidade , Fatores de TempoRESUMO
Immunochemical studies demonstrated the specific accumulation of subunit c of mitochondrial ATP synthase in the brain homogenates of late infantile and juvenile forms of Batten's disease. It is not stored in the infantile form. Storage of subunit alpha of mitochondrial ATP synthase and cytochrome c oxidase subunit IV, an inner membrane protein of mitochondria was not detected in the brains. There was also no difference in the levels of cathepsin B between the two forms of Batten's disease and controls. In cultured skin fibroblasts subunit c accumulates in the late infantile form, whereas it does not in other lysosomal storage diseases. Crude mitochondrial lysosomal preparations of control fibroblasts were separated into high-density fractions rich in a lysosomal marker and low-density fractions rich in a mitochondrial marker on Percoll density gradients. Subunit c was mostly recovered in low-density mitochondrial fractions, but in cells from the late infantile disease a part of subunit c was recovered in the high-density lysosomal fractions. Immunolocalization studies demonstrated a dot-like staining of storage materials for subunit c in the cells from late infantile patients and the staining pattern of subunit c is similar to that of a lysosomal membrane marker, lgp120. Immunostaining failed to detect subunit c in control cells. These results indicate a specific accumulation of subunit c in lysosomes, and suggest that the two forms of Batten's disease are caused by a specific failure in the degradation of subunit c.
Assuntos
Mitocôndrias/enzimologia , Lipofuscinoses Ceroides Neuronais/enzimologia , ATPases Translocadoras de Prótons/metabolismo , Western Blotting , Encéfalo/enzimologia , Eletroforese em Gel de Poliacrilamida , Fibroblastos/enzimologia , Humanos , Lisossomos/enzimologiaRESUMO
OBJECTIVE: Transcortical approaches to the inferior horn often result in quadrant hemianopsia attributable to the injury to the optic radiation. The inferior temporal sulcus (ITS) has received little attention as an entrance point for the transsulcal approach. We used the method of detecting the ITS with magnetic resonance imaging (MRI) scans and investigated the sulcus pattern of ITS, its incidence rate, and the availability of the ITS to the corticotomy for selective amygdalohippocampectomy. METHODS: The sulcus patterns of the ITS of 100 temporal lobes in 50 healthy individuals were classified according to the number of interruptions by gyral bridges, and the localization of the ITS was characterized in relation to the outer surface by means of the surface anatomy scan of MRI. RESULTS: Most of the ITS was interrupted by one to three gyral bridges (0 bridges, 8%; one bridge, 27%: two bridges, 37%; three bridges, 20%; more than four bridges or no apparent ITS, 8%). When the ITS was present, it was located 15 mm above the orbitotragus line at a point 20 mm anterior to the tragus. The number of gyral bridges was significantly larger in the left temporal lobes than in the right temporal lobes, regardless of the sex of the subject. CONCLUSION: The ITS was clearly identified in 72% of the temporal lobes by the oblique sagittal view of MRI scans; thus, in such cases, the ITS was considered to be a candidate for an entrance point of a small temporal corticotomy. The preoperative observation of the ITS in relation to the orbitotragus line by means of MRI may improve the planning of the transsulcal approaches to deeply seated mesial temporal lesions, such as hippocampal sclerosis.
Assuntos
Craniotomia/métodos , Imageamento por Ressonância Magnética , Lobo Temporal/cirurgia , Tonsila do Cerebelo/patologia , Tonsila do Cerebelo/cirurgia , Mapeamento Encefálico , Hipocampo/patologia , Hipocampo/cirurgia , Humanos , Lobo Temporal/patologiaRESUMO
This 49-year-old man gradually developed a disabling action tremor in the proximal right upper extremity 8 months after suffering a pontine tegmental hemorrhage. The intraoperative microrecording in the nucleus ventralis intermedius (VIM) of the left thalamus revealed tremor-synchronous grouped discharges with a vigorous (2.7 Hz) action tremor predominantly in the shoulder and upper arm. High frequency electrical stimulation in the VIM did not affect the tremor. A posteroventral pallidotomy (PVP) was performed and resulted in the successful alleviation of all tremor activity. Posteroventral pallidotomy is known to alleviate parkinsonian tremors, especially those occurring in the contralateral lower extremity, trunk, and proximal segment of the contralateral upper extremity. The authors consider the pallidoreticular pathway to be an important tremor-mediating pathway for the proximal segment of the upper extremities and believe it can be controlled more effectively by PVP than by VIM thalamotomy, as demonstrated by the PVP-induced resolution of the midbrain tremor observed in this case.
Assuntos
Hemorragia Cerebral/cirurgia , Globo Pálido/cirurgia , Ponte/irrigação sanguínea , Tremor/cirurgia , Hemorragia Cerebral/complicações , Hemorragia Cerebral/diagnóstico , Eletromiografia , Globo Pálido/citologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Vias Neurais , Ponte/citologia , Tremor/etiologiaRESUMO
A 44-year-old man presented to the hospital with multiple intracranial epidermoid cysts. The clinical manifestations of his disease included chronic headaches and one seizurelike episode. Findings determined by magnetic resonance (MR) imaging, surgery, and histological analysis indicated intrathecal and intraventricular seeding of the cysts. Spontaneous (nontraumatic) seeding of multiple daughter cysts from intracranial epidermoid cysts is still very rare and their multiple appearances on MR imaging should be distinguished from the simple scattering of oily contents due to cyst rupture.
Assuntos
Encefalopatias/diagnóstico , Cisto Epidérmico/diagnóstico , Imageamento por Ressonância Magnética , Adulto , Aracnoidite/patologia , Encefalopatias/patologia , Ventrículos Cerebrais/patologia , Doença Crônica , Dura-Máter/patologia , Cisto Epidérmico/patologia , Epilepsia Generalizada/diagnóstico , Lobo Frontal/patologia , Cefaleia/diagnóstico , Humanos , Masculino , Ruptura EspontâneaRESUMO
We reported a 37-year-old man who presented complex partial status epilepticus as the initial symptom of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS). He showed fluctuating consciousness disturbance, left homonymous hemianopsia, and paroxysmal conjugated eye deviation to the left. The lactic acid level was elevated in blood and CSF, and ragged-red fibers were observed in the biopsied muscle. MRI demonstrated T2-prolonged lesions in the right occipito-parieto-temporal lobes. Since a mutation of mitochondrial DNA (A3243G) was identified, he was diagnosed as having MELAS. On an ictal record, high amplitude, rhythmic sharp waves were observed at right parieto-temporo-occipital region. High amplitude slow waves were also observed on the right hemisphere, especially in the right frontal lobe. These ictal discharges gradually decreased at their amplitude and in frequency, and then ictal EEG turned to the interictal EEG. During an ictal period, conjugated eye deviation to the left side and consciousness loss were observed. These seizures were observed once every several minutes. During the interictal period, sharp waves and sharp-wave complexes were observed frequently at right parietal and posterior temporal lobes. The venous injection of diazepam (10 mg) normalized EEG quickly. When consciousness loss, especially fluctuating, was observed in the patients of MELAS, complex partial status epilepticus should be considered.
Assuntos
Síndrome MELAS/complicações , Estado Epiléptico/etiologia , Adulto , Diagnóstico Diferencial , Eletroencefalografia , Humanos , Síndrome MELAS/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Estado Epiléptico/diagnóstico , Inconsciência/etiologiaRESUMO
Ventrolateral (VL)-thalamotomy and posteroventral pallidotomy were carried out in 37 patients with idiopathic dystonia, including 23 with dystonia musculorum deformans, 12 with Meige syndrome and 2 with focal dystonia. There were 11 patients with the onset age of dystonia below 10 years and 26 with the age above 11 years. Dystonia tended to generalize in the pediatric patients and to localize to the palmo-mental region in the adult patients. VL-thalamotomy in 30 patients improved the upper body dystonia especially in the adult patients, but, often failed to benefit the pediatric patients. Posteroventral pallidotomy in 18 patients dramatically alleviated the generalized dystonia especially in the pediatric patients. In one adult patient, pallidotomy caused hemiparesis due to intracerebral hemorrhage. The internal pallidum projects the efferents to the thalamic motor nuclei and also to the brainstem reticular formation that connects to the spinal motoneurons. The present findings implicate the descending pallido-reticular pathway in the mechanism of dystonia of pediatric patients, and the ascending pallido-thalamic pathway in that of adult patients.
Assuntos
Gânglios da Base/fisiopatologia , Distonia/fisiopatologia , Distonia/cirurgia , Técnicas Estereotáxicas , Adolescente , Adulto , Vias Aferentes/fisiologia , Fatores Etários , Idoso , Criança , Vias Eferentes/fisiologia , Feminino , Globo Pálido/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Tálamo/cirurgiaRESUMO
Adult-onset type II citrullinemia (CTLN2), an autosomal recessive disorder caused by a mutation in the SLC25A13 gene, is characterized by increased serum citrulline and ammonia levels. Patients with CTLN2 also display various neuropsychiatric symptoms. Many individuals with CTLN2 are fond of protein-rich and/or lipid-rich foods with an aversion to carbohydrate-rich foods. We herein report two cases of CTLN2 treated with living donor liver transplantation (LDLT) and provide a review of the pertinent literature. Case 1 was a 43-year-old man admitted to our hospital for repetitive episodes of consciousness disturbance. Case 2 was a 37-year-old man admitted to our hospital because of abnormal behavior associated with hyperammonemia. A definitive diagnosis of CTLN2 was accomplished by DNA analysis in both patients, who successfully underwent LDLT using liver segments from donor siblings with confirmed heterozygous gene expression. Case 2 also underwent conservative therapy with arginine and a high-fat, carbohydrate-restricted diet prior to LDLT. Postoperative recovery was uneventful and food was unrestricted in both patients. We also identified 77 cases of CTLN2 in the literature and reviewed them in terms of outcome of both liver transplantation and conservative therapy. The survival rate in patients treated by liver transplantation was 100%, whereas that in patients treated by conservative treatment showed improvement from 39.5% to 76.5% over the years. Liver transplantation is a practical treatment that fundamentally improves patient quality of life after transplantation. However, recent studies have suggested that arginine and sodium pyruvate administration combined with intensive nutritional support is also an effective therapy for CTLN2. Further development of conservative therapy may provide a safer, more affordable alternative to liver transplantation in the near future.
Assuntos
Citrulinemia/terapia , Transplante de Fígado , Adulto , Citrulinemia/cirurgia , HumanosRESUMO
INTRODUCTION: Transplantation in Japan still depends on living donors even after the new revised law. We must pay attention to protect living donors. PATIENTS AND METHODS: Perioperative qualities of life after living donation for liver transplantation were assessed with questionnaires including the Medical Outcomes Study 36-Item Short-Form Health Survey version 2 (SF36-v2). Nonparametric Mann-Whitney tests were used to determine statistical significance. P values<.05 were considered significant. RESULTS: Thirty-one among 33 donors answered questionnaires (93.9%). The 15 men and 16 women of average age of 39.7 years had a median hospital stay of 16 days and median duration after surgery of 78 months. Ten of 33 (35.7%) donors considered themselves to be the only possibility. The decision to a donor was established prior to informed consent in 23 donors (74.1%). Six months were required for them to experience a full recovery after donor surgery. Hamilton depression/anxiety score was significantly increased among donors who considered themselves to be the only possibility or those who had decided prior to informed consent. SF36-v2 revealed a significant decrease in social functioning among donors who did not have sufficient time to decide before surgery. General health was significantly decreased among donors who required more than 6 months for full recovery. Perioperative management of pain influenced general health, physical role, bodily pain, and physical functioning. CONCLUSION: We must pay attention to depression and anxiety among living donors. More care should be focused on pain control and sharing of information of postoperative courses.
Assuntos
Conscientização , Hepatectomia , Transplante de Fígado , Doadores Vivos , Qualidade de Vida , Fatores Socioeconômicos , Adulto , Ansiedade/etiologia , Comportamento de Escolha , Estudos Transversais , Depressão/etiologia , Feminino , Hepatectomia/efeitos adversos , Hepatectomia/psicologia , Humanos , Consentimento Livre e Esclarecido , Japão , Tempo de Internação , Transplante de Fígado/efeitos adversos , Transplante de Fígado/psicologia , Doadores Vivos/psicologia , Masculino , Saúde Mental , Dor Pós-Operatória/etiologia , Dor Pós-Operatória/psicologia , Período Perioperatório , Inquéritos e Questionários , Fatores de TempoRESUMO
AIM: Living donor liver transplantation (LDLT) has been widely accepted because of the severe shortage of hepatic grafts. However, the healthy donor is exposed to risks of morbidity and mortality. In this study, we analyzed medical, functional, and psychological outcomes of donors after hepatectomy for liver donation. PATIENTS AND METHODS: Among 41 donor hepatectomy cases for LDLT performed in our institute from January 1994 to May 2011, we reviewed the medical records (liver function tests, complications, etc) of 27 subjects who donated to recipients older than 12 years. We also performed a questionnaire survey based on the Japanese Short Form-36 version 2 Health Survey scales as a measure of physical and mental health, to which 31 subjects responded. RESULTS: Six of the 27 donors experienced prolonged jaundice. Their ratios of graft volume/standard donor liver volume (GV/SDLV) were higher than those of the 21 donors without prolonged jaundice (60.0% vs 41.5%). According to the questionnaires, social functioning among those having undergone emergency hepatectomy as well as general health perceptions declined in those with postoperative complications. Physical component summary declined among those having undergone emergency hepatectomy and with postoperative complications. CONCLUSION: In liver donation from a living donor, massive hepatectomy should be avoided. A ratio of GV/SDLV around 50% seems reasonable. Donors with emergency transplantations or postoperative complications must be more carefully followed after donor hepatectomy.
Assuntos
Hepatectomia/psicologia , Transplante de Fígado/psicologia , Doadores Vivos/psicologia , Qualidade de Vida , Adulto , Idoso , Feminino , Hepatectomia/efeitos adversos , Humanos , Japão , Icterícia/etiologia , Icterícia/psicologia , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-Idade , Inquéritos e Questionários , Fatores de Tempo , Resultado do TratamentoRESUMO
Transplantation for Wilson's disease occupies 1/3 of the cases for metabolic diseases in Japan. At the end of 2009, 109 transplantations had been performed including three deceased donor cases in the Japanese registry. We herein discuss problems of transplantation for Wilson's disease as well as its indication, timing, and social care. We retrospectively reviewed four fulminant cases and two chronic cases who underwent living donor liver transplantation. There were two boys and two girls. Four adolescents of average age 11.3 years underwent living donor liver transplantation. Duration from onset to transplantation ranged from 10 to 23 days. Average Model for End-stage Liver Disease (MELD) score was 27.8 (range=24-31). All patients were administrated chelates prior to transplantation. MELD, New Wilson's index, Japanese scoring for liver transplantation, and liver atrophy were useful tools for transplantation decision making; however, none of them was an independent decisive tool. Clinical courses after transplantation were almost uneventful. One girl, however, developed an acute rejection episode due to noncompliance at 3 years after transplantation. All patients currently survive without a graft loss. No disease recurrence had been noted even using living related donors. Two adults evaluated for liver transplantation were listed for deceased donor liver transplantation. Both candidates developed cirrhosis despite long-term medical treatment. There were no appropriate living donors for them. There are many problems in transplantation for Wilson's disease. The indications for liver transplantation should be considered individually using some decision-making tools. The safety of the living donor should be paid the most attention.
Assuntos
Degeneração Hepatolenticular/cirurgia , Cirrose Hepática/cirurgia , Falência Hepática Aguda/cirurgia , Transplante de Fígado , Doadores Vivos , Seleção de Pacientes , Doença Aguda , Adolescente , Quelantes/uso terapêutico , Criança , Técnicas de Apoio para a Decisão , Feminino , Rejeição de Enxerto/etiologia , Hemodiafiltração , Degeneração Hepatolenticular/complicações , Degeneração Hepatolenticular/diagnóstico , Humanos , Japão , Cirrose Hepática/diagnóstico , Cirrose Hepática/etiologia , Falência Hepática Aguda/diagnóstico , Falência Hepática Aguda/etiologia , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-Idade , Plasmaferese , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Listas de Espera , Adulto JovemRESUMO
BACKGROUND: The incidence of hepatic venous outflow obstruction (HVOO) has been reported to be 5%-13% when a partial graft is used for orthotopic liver transplantation (OLT). HVOO leads to graft congestion, portal hypertension, and finally cirrhosis, which jeopardizes both graft and recipient survivals. In this study, we sought to identify perioperative factors influencing HVOO and to investigate conditions that require stent placement. PATIENTS AND METHODS: From February 1994 to December 2010, we performed 40 living donor liver transplantations (LDLT). HVOO occurred in 5 cases (12.5%), all of which were left lobe grafts. Because HVOO was not observed in patients with body weight (BW) <30 kg, we investigated the other 28 cases with BW >30 kg. RESULTS: There was no difference from unaffected subjects except for cold ischemic time (CIT), which was significantly longer: 86.2 ± 10.4 minutes vs 46.0 ± 4.8 minutes (P = .001). Balloon angioplasty, which was selected as the initial treatment for all stricture patients, improved 2 patients after 1 and 5 treatments, respectively, but 3 subjects underwent repeated HVOO, finally being treated with self-expandable metallic stents at 9, 6, and 10 years after LDLT, respectively. All patients finally resolved their strictures. CONCLUSION: HVOO reflects intimal hyperplasia and fibrosis at the anastomotic sites or compression and twisting of the anastomosis caused by graft regeneration. In addition, progression of chronic rejection and fibrosis are possibly responsible for late-onset HVOO. Longer CIT possibly reflects difficulties in the venoplasty before anastomosis. No bleeding or thrombosis complications were observed during dilatation among our cases. The selection of the stent size for each case and careful stent deployment are important to prevent complications. Stent placement should be considered in patients with chronic rejection who are refractory to several balloon angioplasties with early-onset or late-onset HVOO.