STUDY DESIGN: To induce scoliosis in young female Wistar rats using a noninvasive method and to validate this model. PURPOSE: To induce scoliosis in a rat model noninvasively by bracing and to study the corresponding gene-expression profile in the spine and different organs. OVERVIEW OF LITERATURE: Scoliosis involves abnormal lateral curvature of the spine, the causes of which remain unclear. In the literature, it is suggested that scoliosis is genetically heterogeneous, as there are multiple factors involved directly or indirectly in its pathogenesis. Clinical and experimental studies were conducted to understand the etiology of anatomical alterations in the spine and internal organs, as the findings could help clinicians to establish new treatment approaches. METHODS: Twelve female Wistar rats aged 21 days were chosen for this study. Customized braces and real-time polymerase chain reaction (RT-PCR) primers for rats were designed using Primer 3 software. Radiological analysis (X-rays), histopathological studies, SYBR green, and RT-PCR analysis were performed. RESULTS: The spines of six rats were braced in a deformed position, which resulted in a permanent structural deformity as confirmed by X-ray studies. The remaining rats were used as controls. Quantitative studies of the expression of various genes (osteocalcin, pleiotrophins, matrix metalloproteinase-2 [MMP2] and MMP9, TIMP, interleukins 1 and 6, tumor necrosis factor-α) showed their differential expression and significant upregulation (p < 0.05) in different organs of scoliotic rats in comparison to those in control rats. Histopathological findings showed tissue necrosis and fibrosis in the brain, retina, pancreas, kidney, liver, and disc of scoliotic rats. CONCLUSIONS: Bracing is a noninvasive method for inducing scoliosis in an animal model with 100% reliability and with corresponding changes in gene expression. Scoliosis does not just involve a spine deformity, but can be referred to as a systemic disease on the basis of the pathological changes observed in various internal organs.
Animals ; Braces ; Brain ; Congenital Abnormalities ; Female ; Fibrosis ; Gene Expression ; Humans ; Inflammation ; Interleukins ; Kidney ; Liver ; Matrix Metalloproteinase 2 ; Methods ; Models, Animal ; Necrosis ; Pancreas ; Rats ; Rats, Wistar ; Real-Time Polymerase Chain Reaction ; Retina ; Scoliosis ; Spine ; Up-Regulation

BACKGROUND/AIMS: Despite improvements in surgical techniques and postoperative patient care, bile leakage can occur after hepatobiliary surgery and may lead to serious complications. The aim of this retrospective study was to evaluate the efficacy of endoscopic treatment of bile leakage after hepatobiliary surgery. METHODS: The medical records of 20 patients who underwent endoscopic retrograde cholangiopancreatography because of bile leakage after hepatobiliary surgery from August 2009 to September 2014 were reviewed retrospectively. Endoscopic treatment included insertion of an endoscopic retrograde biliary drainage stent after endoscopic sphincterotomy. RESULTS: Most cases of bile leakage presented as percutaneous bile drainage through a Jackson-Pratt bag (75%), followed by abdominal pain (20%). The sites of bile leaks were the cystic duct stump in 10 patients, intrahepatic ducts in five, liver beds in three, common hepatic duct in one, and common bile duct in one. Of the three cases of bile leakage combined with bile duct stricture, one patient had severe bile duct obstruction, and the others had mild strictures. Five cases of bile leakage also exhibited common bile duct stones. Concerning endoscopic modalities, endoscopic therapy for bile leakage was successful in 19 patients (95%). One patient experienced endoscopic failure because of an operation-induced bile duct deformity. One patient developed guidewire-induced microperforation during cannulation, which recovered with conservative treatment. One patient developed recurrent bile leakage, which required additional biliary stenting with sphincterotomy. CONCLUSIONS: The endoscopic approach should be considered a first-line modality for the diagnosis and treatment of bile leakage after hepatobiliary surgery.
Abdominal Pain ; Bile Ducts ; Bile* ; Catheterization ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis ; Common Bile Duct ; Congenital Abnormalities ; Constriction, Pathologic ; Cystic Duct ; Diagnosis ; Drainage ; Hepatic Duct, Common ; Humans ; Liver ; Medical Records ; Patient Care ; Retrospective Studies ; Sphincterotomy, Endoscopic ; Stents

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.
Adult ; Antiphospholipid Syndrome* ; Budd-Chiari Syndrome* ; Congenital Abnormalities ; Edema ; Female ; Hematemesis ; Humans ; Hypogonadism ; Klinefelter Syndrome* ; Liver Cirrhosis ; Lower Extremity ; Lupus Erythematosus, Systemic*

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.
Adult ; Antiphospholipid Syndrome ; Budd-Chiari Syndrome ; Congenital Abnormalities ; Edema ; Female ; Hematemesis ; Humans ; Hypogonadism ; Klinefelter Syndrome ; Liver Cirrhosis ; Lower Extremity ; Lupus Erythematosus, Systemic

BACKGROUND/AIMS: Chronic liver disease leads to liver fibrosis, and although the liver does have a certain regenerative capacity, this disease is associated with dysfunction of the liver vessels. C-reactive protein (CRP) is produced in the liver and circulated from there for metabolism. CRP was recently shown to inhibit angiogenesis by inducing endothelial cell dysfunction. The objective of this study was to determine the effect of CRP levels on angiogenesis in a rat model of liver dysfunction induced by bile duct ligation (BDL). METHODS: The diameter of the hepatic vein was analyzed in rat liver tissues using hematoxylin and eosin (H&E) staining. The expression levels of angiogenic factors, albumin, and CRP were analyzed by real-time PCR and Western blotting. A tube formation assay was performed to confirm the effect of CRP on angiogenesis in human umbilical vein endothelial cells (HUVECs) treated with lithocholic acid (LCA) and siRNA-CRP. RESULTS: The diameter of the hepatic portal vein increased significantly with the progression of cirrhosis. The expression levels of angiogenic factors were increased in the cirrhotic liver. In contrast, the expression levels of albumin and CRP were significantly lower in the liver tissue obtained from the BDL rat model than in the normal liver. The CRP level was correlated with the expression of albumin in hepatocytes treated with LCA and siRNA-CRP. Tube formation was significantly decreased in HUVECs when they were treated with LCA or a combination of LCA and siRNA-CRP. CONCLUSION: CRP seems to be involved in the abnormal formation of vessels in hepatic disease, and so it could be a useful diagnostic marker for hepatic disease.
Angiogenic Proteins/genetics/metabolism ; Animals ; Bile Ducts/surgery ; C-Reactive Protein/*analysis/genetics/metabolism ; Cells, Cultured ; Disease Models, Animal ; Hepatic Veins/abnormalities ; Hepatocytes/cytology/metabolism ; Human Umbilical Vein Endothelial Cells ; Humans ; Lithocholic Acid/pharmacology ; Liver/metabolism/pathology ; Liver Cirrhosis/etiology ; Liver Diseases/metabolism/*pathology ; Male ; Microscopy, Fluorescence ; Mitochondria/drug effects/metabolism ; RNA Interference ; RNA, Small Interfering/metabolism ; Rats ; Rats, Sprague-Dawley ; Real-Time Polymerase Chain Reaction ; Serum Albumin/genetics/metabolism

No abstract available.
Arteriovenous Fistula/complications ; Fatty Liver/complications/radiography/ultrasonography ; Hemangioma/etiology/radiography/*ultrasonography ; Hepatic Artery/abnormalities ; Humans ; Liver Neoplasms/radiography/ultrasonography ; Male ; Middle Aged ; Portal Vein/abnormalities ; Tomography, X-Ray Computed

Hepatopulmonary syndrome (HPS) is a serious complication of end-stage liver disease, which is characterized by hypoxia, intrapulmonary vascular dilatation, and liver cirrhosis. Liver transplantation (LT) is the only curative treatment modality for patients with HPS. However, morbidity and mortality after LT, especially in cases of severe HPS, remain high. This case report describes a patient with typical findings of an extracardiac pulmonary arteriovenous shunt on contrast-enhanced transesophageal echocardiography (TEE), and clubbing fingers, who had complete correction of HPS by deceased donor LT. The patient was a 16-year-old female who was born with biliary atresia and underwent porto-enterostomy on the 55th day after birth. She had been suffered from progressive liver failure with dyspnea, clubbing fingers, and cyanosis. Preoperative arterial blood gas analysis revealed severe hypoxia (arterial O2 tension of 54.5 mmHg and O2 saturation of 84.2%). Contrast-enhanced TEE revealed an extracardiac right-to-left shunt, which suggested an intrapulmonary arteriovenous shunt. The patient recovered successfully after LT, not only with respect to physical parameters but also for pychosocial activity, including school performance, during the 30-month follow-up period.
Adolescent ; Anoxia ; Arteriovenous Fistula/etiology ; Biliary Atresia/*diagnosis/etiology ; Cyanosis/complications ; Dyspnea/complications ; Echocardiography, Transesophageal ; End Stage Liver Disease/complications/*surgery ; Female ; Hepatic Artery/abnormalities ; Hepatopulmonary Syndrome/*diagnosis/ultrasonography ; Humans ; *Liver Transplantation ; Osteoarthropathy, Secondary Hypertrophic/complications

BACKGROUND AND OBJECTIVES: Variety of pathological factors including viral hepatitis, alcohol and drug abuse, metabolic diseases, autoimmune diseases and congenital abnormalities can cause hepatic injury. Liver transplantation is the treatment of choice for end-stage liver diseases, however, it faces several difficulties. So the aim of the work is to evaluate the effect of bone marrow derived mesenchymal stem cells (BM-MSCs) on the liver structure in carbon tetra chloride CCL4 induced liver fibrosis in rats. MATERIALS AND RESULTS: BM-MSCs were isolated and characterized from long bones of twenty male albino rats. Sixty female rats were divided into the following two groups: Group I; thirty rats which were the control group. Group II; thirty rats were injected intra-peritoneal (IP) by CCL4 twice weekly for four weeks and was further subdivided into the following three subgroups: Subgroup IIA (CCL4 alone); included ten rats which were sacrificed after this four weeks. Subgroup IIB (CCL4/MSCs); included ten rats which were IP injected by a single dose of BM-MSCs and were sacrificed after four weeks. Subgroup IIC (CCL4/recovery); included ten rats which were left for another four weeks without any intervention. Histological examination of liver specimens showed that CCl4 caused variable pathological changes with elevated liver enzymes. Injection of BM-MSCs revealed an improvement in the histological picture of the liver and its enzymatic profile. On the other hand, most of the pathological lesion were still detected in rats of recovery group. CONCLUSIONS: BM-MSC could restore the liver structure and function in experimental model of liver fibrosis.
Animals ; Autoimmune Diseases ; Bone Marrow* ; Carbon ; Characidae ; Congenital Abnormalities ; Female ; Fibrosis ; Hand ; Hepatitis ; Humans ; Liver ; Liver Cirrhosis* ; Liver Diseases ; Liver Transplantation ; Male ; Mesenchymal Stromal Cells* ; Metabolic Diseases ; Models, Theoretical ; Rats* ; Substance-Related Disorders

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 microg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension.
Echocardiography, Doppler ; Humans ; Hypertension, Pulmonary/*diagnosis/drug therapy ; Iloprost/therapeutic use ; Liver/blood supply/radiography ; Magnetic Resonance Imaging ; Male ; Thoracic Arteries/ultrasonography ; Tomography, X-Ray Computed ; Vasodilator Agents/therapeutic use ; Vena Cava, Inferior/*abnormalities/ultrasonography ; Young Adult

Toxoplasmosis is an important cause of foodborne, inflammatory, as well as congenital abnormalities. There is an urgent need for safe and effective therapies to eliminate or treat this cosmopolitan infectious disease. A medicinal herbal plant, Meliae fructus, has been used to soothe the liver and kills worms in Chinese medicine. In this study, Meliae fructus ethanol extract was examined and screened for its anti-T. gondii activity. For anti-T. gondii activity screening, in vitro study of Meliae fructus extract using tachyzoit of T. gondii RH strain-infected HeLa cells was performed. Further, in vivo anti-T. gondii study using a mouse infection model was conducted. Safety of herbal compounds was evaluated in SD rats by treatment with Meliae fructus extract for 28 days. As a result, selectivity of Meliae fructus ethanol extract was 5.85, which was higher than sulfadiazine selectivity (2.06). We also performed an in vivo study to evaluate the anti-T. gondii activity of Meliae fructus extract in a mouse model. The inhibition rate of Meliae fructus extract was as high as that of sulfadiazine. These results demonstrate that Meliae fructus can successfully cure T. gondii infection and could be a promising native herb treatment for prevention of T. gondii infection.
Animals ; Asian Continental Ancestry Group ; Communicable Diseases ; Congenital Abnormalities ; Ethanol* ; HeLa Cells ; Humans ; Liver ; Mass Screening ; Melia* ; Mice ; Plants ; Plants, Medicinal ; Rats ; Sulfadiazine ; Toxoplasma ; Toxoplasmosis