An Unusual Case of Klippel-Trenaunay-Weber Syndrome Presenting with Portosystemic Encephalopathy
スポンサーリンク
概要
- 論文の詳細を見る
We report a unique male patient presenting with portosystemic encephalopathy (PSE) due to intrahepatic portohepatic venous (PHV) shunts. He was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS) based on the findings of a hemitruncal port-wine stain with subcutaneous arteriovenous fistulae and varicose veins in the legs. However, limb-hypertrophy, which is one of the most cardinal manifestations of KTWS, was absent, and in KTWS, PSE is quite a rare clinical manifestation. Hence, the clinical picture of this patient was unusual. Our clinical observation suggests that KTWS can be one of the underlying disorders causing PSE.
- JAPAN SOC INTERNAL MEDICINEの論文
著者
-
IKEDA Shu-ichi
Department of Medicine, Shinshu University School of Medicine
-
Shimojima Yasuhiro
Department of Internal Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
-
Miyazaki Daigo
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan
-
Yazaki Masahide
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan
-
Shimojima Y
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
-
Ikeda SI
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
-
UEDA Kazuhiko
Department of Crown and Bridge, School of Life Dentistry at Niigata, The Nippon Dental University
-
KANEKO Kazuma
Department of Medicine, Azumi General Hospital
-
Kaneko Kazuma
Department Of Medicine Azumi General Hospital
-
Tojo Kana
Department of Medicine (Neurology), Shinshu University School of Medicine
-
Tojo K
Department Of Biochemical Engineering And Science Kyushu Institute Of Technology
-
Ueda Kazuhiko
Department Of Crown And Bridge School Of Life Dentistry At Niigata The Nippon Dental University
-
Yazaki M
Department Of Pediatrics Nagoya City Moriyama Municipal Hospital
-
Kaneko Kazuma
Department Of Medicine Hata General Hospital
-
Ikeda Shu-ichi
Department Of Internal Medicine (neurology And Rheumatology) Shinshu University School Of Medicine
-
Ikeda Shu-ichi
Department Of Neurology Shinshu University School Of Medicine
-
Kaneko K
Department Of Aeronautical Engineering
-
Ueda K
Department Of Applied Biological Sciences College Of Bioresource Sciences Nihon University
-
Shimojima Yoshio
Third Department Of Medicine Shinshu University School Of Medicine
-
Ikeda Shu-ihci
Dep. Of Medicine (neurology And Rheumatology) Shinshu Univ. School Of Medicine
-
Tojo Kana
Third Department Of Medicine (neurology And Rheumatology) Shinshu University School Of Medicine
-
Miyazaki Daigo
Department Of Medicine (neurology And Rheumatology) Shinshu University School Of Medicine
-
Ikeda Shu-ichi
Department of Medicine (Neurology), Shinshu University School of Medicine
-
Miyazaki D
Department of Medicine (Neurology), Shinshu University School of Medicine
-
Miyazaki Daigo
Department of Medicine (Neurology), Shinshu University School of Medicine
-
Ueda Kazuhiko
Department of Radiology, Shinshu University School of Medicine
-
Kaneko Kazuma
Department of Medicine (Neurology), Shinshu University School of Medicine
-
Shimojima Yasuhiro
Department of Medicine (Neurology), Shinshu University School of Medicine
関連論文
- Senile Systemic Amyloidosis in an Aged Savannah Monkey (Cercopithecus aethiops) with Tenosynovial Degeneration
- Biopsy-proven Tuberculous Meningitis Mimicking CNS Sarcoidosis
- Flow Cytometric Analysis of Lymphocyte Subpopulations and Th1/Th2 Balance in Patients with Polymyositis and Dermatomyositis
- Serum Levels of Free Light Chain before and after Chemotherapy in Primary Systemic AL Amyloidosis
- BODY SURFACE QRS POTENTIAL MAPS DURING CORONARY ARTERIOGRAPHY : Mapping : 46th Annual Scientific Meeting, Japanese Circulation Society
- Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met familial amyloid polyneuropathy
- The safety of transcranial magnetic stimulation with deep brain stimulation instruments
- Long-term Follow-up of Plasma Cells in Bone Marrow and Serum Free Light Chains in Primary Systemic AL Amyloidosis
- A Rare Lung Nodule Consisting of Adenocarcinoma and Amyloid Deposition in a Patient with Primary Systemic AL Amyloidosis
- Conventional Diet Therapy for Hyperammonemia is Risky in the Treatment of Hepatic Encephalopathy Associated with Citrin Deficiency
- Metabolic derangements in deficiency of citrin, a liver-type mitochondrial aspartate-glutamate carrier
- Risk of Worsened Encephalopathy after Intravenous Glycerol Therapy in Patients with Adult-onset Type II Citrullinemia (CTLN2)
- Hepatocellular Carcinoma in a Case of Adult-onset Type II Citrullinemia
- Myasthenia Gravis with Anti-MuSK Antibody, Showing Progressive Muscular Atrophy without Blepharoptosis
- Reactive Arthritis Due to Asymptomatic Infection of Chlamydia trachomatis
- Analysis of 55 Transplantations from Unrelated Volunteer Donors Facilitated by Tokai Marrow Donor Bank
- Quality of Life in Adult Patients after Bone Marrow Transplantation
- Twenty Years' Experience in Allogeneic Hematopoietic Stem Cell Transplantation for Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia in the Nagoya Blood and Marrow Transplantation Group
- Ten Years of Experience with Liver Transplantation for Familial Amyloid Polyneuropathy in Japan : Outcomes of Living Donor Liver Transplantations
- Cytomegalovirus-induced Infectious Mononucleosis-like Syndrome in a Rheumatoid Arthritis Patient Treated with Methotrexate and Infliximab
- Successful Treatment with Rituximab in a Patient with Stiff-Person Syndrome Complicated by Dysthyroid Ophthalmopathy
- Takayasu's Arteritis in a Patient with Crohn's Disease: An Unexpected Association during Infliximab Therapy
- Therapeutic outcome of cyclic VAD (vincristine, doxorubicin and dexamethasone) therapy in primary systemic AL amyloidosis patients
- Successful treatment with infliximab and low-dose methotrexate in a Japanese patient with familial Mediterranean fever
- Increase in Incidence of Elderly-onset Patients with Myasthenia Gravis in Nagano Prefecture, Japan
- High-dose Melphalan Followed by Autologous Stem Cell Support in Primary Systemic AL Amyloidosis with Multiple Organ Involvement
- Serum Amyloid A as a Potent Therapeutic Marker in a Refractory Patient with Polymyalgia Rheumatica
- Retrospective Analyses of Clinical Features and Therapeutic Outcomes in Thymectomized Patients with Myasthenia Gravis at Shinshu University
- Intractable Skin Necrosis and Interstitial Pneumonia in Amyopathic Dermatomyositis, Successfully Treated with Cyclosporin A
- Hemolysis, Elevated Liver Enzymes and Low Platelet (HELLP) Syndrome Associated with Systemic Lupus Erythematosus
- Abdominal Fat Aspiration Biopsy and Genotyping of Serum Amyloid A Contribute to Early Diagnosis of Reactive AA Amyloidosis Secondary to Rheumatoid Arthritis
- Long-term Follow-up of Systemic Reactive AA Amyloidosis Secondary to Rheumatoid Arthritis: Successful Treatment with Intermediate-dose Corticosteroid
- Cytoplasmic Body Myopathy with Hypertrophic Cardiomyopathy
- Marked shrinkage of amyloid lymphadenopathy after an intensive chemotherapy in a patient with IgM-associated AL amyloidosis
- Rituximab therapy in nephrotic syndrome due to AH amyloidosis
- Hemophagocytic syndrome associated with rheumatoid arthritis: A case report and review of the literature
- Malignant Lymphoma Associated with Rheumatoid Arthritis, Developing Shortly after Initiation of Oral Methotrexate
- Senile Systemic Amyloidosis Starting as Bilateral Carpal and Left Ulnar Tunnel Syndrome
- Expression of Various Glutamate Receptors Including N-Methyl-D-Aspartate Receptor (NMDAR) in an Ovarian Teratoma Removed from a Young Woman with Anti-NMDAR Encephalitis
- Huntington's disease with onset ages greater than 60 years
- Clinical Evaluation of Tooth Reduction Using Piezotome
- Risk Factors for Hospital-acquired Bacteremia
- An Unusual Case of Klippel-Trenaunay-Weber Syndrome Presenting with Portosystemic Encephalopathy
- A Novel RNA Splice Site Mutation in the C1 Inhibitor Gene of a Patient with Type I Hereditary Angioedema
- Unilateral Oculomotor Nerve Palsy Induced by Combination Therapy of Interferon-α2b and Ribavirin
- Diflunisal stabilizes familial amyloid polyneuropathy-associated transthyretin variant tetramers in serum against dissociation required for amyloidogenesis
- Multiple Brain Tumors of Diffuse Large B Cell Lymphoma in a Patient with Waldenstrom's Macroglobulinemia/Lymphoplasmacytic Lymphoma : PCR and DNA Sequence Analysis Show Evidence of Differences in Clonality of the Two B Cell Malignancies
- Establishing the Independent Culture of a Strictly Symbiotic Bacterium Symbiobacterium thermophilum from Its Supporting Bacillus Strain
- Periodic Peritonitis due to Familial Mediterranean Fever in a Patient with Systemic Lupus Erythematosus
- Abscess of the Round Ligament of the Liver Associated with Acute Obstructive Cholangitis and Septic Thrombosis
- Nationwide survey on predictive genetic testing for late-onset, incurable neurological diseases in Japan
- Skin Penetration Enhancement by the Synergistic Effect of Supersaturated Dissolution and Chemical Enhancers
- Effect of Supersaturated Dissolution and Crystallization on In Vitro Skin Penetration of 17-β-Estradiol from Acrylic Pressure Sensitive Adhesives
- Distinctive Multidrug Sensitivity and Outcome of Acute Erythroblastic and Megakaryoblastic Leukemia in Children With Down Syndrome
- Herbimycin A Inhibits Cell Growth at S Phase in the Synchronized Philadelphia Chromosome Positive Cell Line BV173
- Biological characteristics and prognostic value of in vitro three-drug resistance to prednisolone, L-asparaginase, and vincristine in childhood acute lymphoblastic leukemia
- Characteristic Spinal MRI Findings of HIV-associated Myelopathy in an AIDS Patient
- Fasciitis in Mixed Connective Tissue Disease Successfully Treated with High-dose Intravenous Immunoglobulin
- Severe Depression as an Initial Symptom in an Elderly Patient with Acute Disseminated Encephalomyelitis.
- Paraneoplastic Neurologic Syndrome Associated with Small Breast Cancer : Diagnostic Value of FDG-PET for Detection of Underlying Malignancy
- Phenol Congo Red Staining Enhances the Diagnostic Value of Abdominal Fat Aspiration Biopsy in Reactive AA Amyloidosis Secondary to Rheumatoid Arthritis
- Accurate and simple method for quantification of hepatic fat content using magnetic resonance imaging : a prospective study in biopsy-proven nonalcoholic fatty liver disease
- The Characteristics of Food Intake in Patients with Type II Citrullinemia
- Nephrotic Syndrome Due to Primary AL Amyloidsis, Successfully Treated with VAD and Subsequent High-dose Melphalan Followed by Autologous Peripheral Blood Stem Cell Transplantation
- A Japanese case of SCA14 with the Gly128Asp mutation
- Intestinal Angina Due to Atherosclerosis in a 45-year-old Systemic Lupus Erythematosus Patient
- Magnetooptical Kerr Effect of Semiconductor-Based Multilayer Structures Containing a GaAs:MnAs Granular Thin Film
- Surface Dissolution-Bulk Erosion Model of Drug Release from Biodegradable Polymer Rods
- Fatal Acute Pancreatitis with Cystic Formation in Reactive Systemic AA Amyloidosis Secondary to Rheumatoid Arthritis
- Characteristics of Long Size Thermosyphons Made of Corrugated Tube
- An Arranging Method on L/D of Two-Phase Closed Thermosyphons
- Dynamic Heat Transfer Mechanism of Two-phase Closed Thermosyphon
- 4 Cardiac Amyloidosis : Heterogenous Pathogenic Backgrounds(New Insights of Specific Cardiomyopathies, The 71st Annual Scientific Meeting of the Japanese Circulation Society)
- Leukoencephalopathy Induced by Low-dose Methotrexate in a Patient with Rheumatoid Arthritis
- Pneumatosis cystoides intestinalis in neuropsychiatric systemic lupus erythematosus with diabetes mellitus : case report and literature review
- 69 FUNCTIONAL STUDY OF THE Sl79R PIT1 MUTANT
- 232 TREATMENT OF PROGRESSIVE OSSEOUS HETEROPLASIA WITH ETIDRONATE DISODIUM
- 62 A CASE OF INFANTILE MALIGNANT OSTEOPETROSIS TREATED WITH ALLOGENEIC BONE MARROW TRANSPLANTATION
- Buerger's disease manifesting nodular erythema with livedo reticularis
- Spinal Nerve Root Hypertrophy on MRI: Clinical Significance in the Diagnosis of Chronic Inflammatory Demyelinating Polyradiculoneuropathy
- Spinal Epidural Abscess with Osteomyelitis as a Cause of Bacterial Meningitis
- 209 A Case of 3-Year-Old Girl Diagnosed as Psychosocial Dwarfirsm with Svere Growth Disturbance
- Intravenous Immunoglobulin (IVIg) with Methylprednisolone Pulse Therapy for Motor Impairment of Neuralgic Amyotrophy: Clinical Observations in 10 Cases
- A novel nonsense mutation in a Japanese family with ataxia with oculomotor apraxia type 2 (AOA2)
- Cerebral hemorrhage in Fabry's disease
- Characterization of deletion breakpoints in patients with dystrophinopathy carrying a deletion of exons 45-55 of the Duchenne muscular dystrophy (DMD) gene
- Two Siblings Diagnosed to Have Transthyretin-related Familial Amyloid Cardiomyopathy Around the Same Time at Different Hospitals
- A Case of Mikulicz's Disease Complicated by Malignant Lymphoma: A Postmortem Histopathological Finding
- ANCA-associated Vasculitis with Central Retinal Artery Occlusion Developing during Treatment with Methimazole
- Clinical and Serial MRI Findings of a Sialidosis Type I Patient with a Novel Missense Mutation in the NEU1 Gene
- Liver Dysfunction and Thrombocytopenia Diagnosed as Intravascular Large B-cell Lymphoma Using a Timely and Accurate Transjugular Liver Biopsy
- Adult Onset X-Linked Chronic Granulomatous Disease in a Woman Patient Caused by a de novo Mutation in Paternal-Origin CYBB Gene and Skewed Inactivation of Normal Maternal X Chromosome
- Pure Red Cell Aplasia Developing after Treatment of Pleural Recurrence of Thymoma, Successfully Treated with Cyclosporin A but not with Tacrolimus
- Corpus Callosum Atrophy in Patients with Hereditary Diffuse Leukoencephalopathy with Neuroaxonal Spheroids: An MRI-based Study
- Nephrotic Syndrome Due to Primary Systemic AL Amyloidosis, Successfully Treated with VAD (Vincristine, Doxorubicin and Dexamethasone) Alone
- Systemic AL Amyloidosis Mimicking Rheumatoid Arthritis
- An 85-year-old Case with Hashimoto's Encephalopathy, Showing Spontaneous Complete Remission
- Patient with adult-onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma : Indication for liver transplantation
- Marked and Rapid Regression of Hepatic Amyloid Deposition in a Patient with Systemic Light Chain (AL) Amyloidosis after High-dose Melphalan Therapy with Stem Cell Transplantation
- Spinal Intradural Extramedullary Cavernous Angioma Presenting with Superficial Siderosis and Hydrocephalus: A Case Report and Review of the Literature