Pediatric living donor liver transplantation for congenital hepatic fibrosis using a mother's graft with von Meyenburg complex: A case report

This is the first report of living donor liver transplantation(LDLT) for congenital hepatic fibrosis(CHF) using a mother's graft with von Meyenburg complex. A 6-year-old girl with CHF, who suffered from recurrent gastrointestinal bleeding, was referred to our hospital for liver transplantation. Her 38-year-old mother was investigated as a living donor and multiple biliary hamartoma were seen on her computed tomography and magnetic resonance imaging scan. The mother's liver function tests were normal and she did not have any organ abnormality, including polycystic kidney disease. LDLT using the left lateral segment(LLS) graft from the donor was performed. The donor LLS graft weighed 250 g; the graft recipient weight ratio was 1.19%. The operation and post-operative course of the donor were uneventful and she was discharged on post-operative day(POD) 8. The graft liver function was good, and the recipient was discharged on POD 31. LDLT using a graft with von Meyenburg complex is safe and useful. Long-term follow-up is needed with respect to graft liver function and screening malignant tumors.

Inflammatory pseudotumor of the liver: 13 cases of MRI findings

INTRODUCTIONInflammatory pseudotumor of the liver ,a rare benign lesion ,is often confused with the malignant tumors .Until 1998,less than 80 cases had been reported in the world [1,2].The accuracy of the pre-surgical diagnosis was low p[3,4] ,and very few materials on the diagnosis using dynamic MRI were reported [5-7] .We collected thirteen cases of inflammatory pseudotumor of the liver were collected and roved by pathology with MRI studies .Our purpose is to describe and analyze the MRI findings of this lesion and find out the valuable signs suggesting the diagnosis so as to avoid unnecessary operations.

Female gender in the setting of liver transplantation

The evolution of liver diseases to end-stage liver disease or to acute hepatic failure, the evaluation process for liver transplantation, the organ allocation decisionmaking, as well as the post-transplant outcomes are different between female and male genders. Women's access to liver transplantation is hampered by the use of model for end-stage liver disease(MELD) score, in which creatinine values exert a systematic bias against women due to their lower values even in the presence of variable degrees of renal dysfunction. Furthermore, even when correcting MELD score for gender-appropriate creatinine determination, a quantifiable uneven access to transplant prevails, demonstrating that other factors are also involved. While some of the differences can be explained from the epidemiological point of view, hormonal status plays an important role. Moreover, the pre-menopausal and post-menopausal stages imply profound differences in a woman's physiology, including not only the passage from the fertile age to the non-fertile stage, but also the loss of estrogens and their potentially protective role in delaying liver fibrosis progression, amongst others. With menopause, the tendency to gain weight may contribute to the development of or worsening of pre-existing metabolic syndrome. As an increasing number of patients are transplanted for non-alcoholic steatohepatitis, and as the average age at transplant increases, clinicians must be prepared for the management of this particular condition, especially in post-menopausal women, who are at particular risk of developing metabolic complications after menopause.

Combined endovascular-surgical treatment for complex congenital intrahepatic arterioportal fistula: A case report and review of the literature

BACKGROUND Congenital intrahepatic arterioportal fistula(IAPF) is a rare vascular malformation in infants that causes severe portal hypertension(PH) with poor prognosis if untreated. Currently, radiological embolisation is considered the first-line therapy for simple IAPF; however, it might be not resolutive for complex hepatic vascular lesions. When endovascular embolization is not sufficient to completely obliterate the IAPF, surgical intervention is needed, but it has been associated with severe morbidity and mortality in small children.Furthermore, indications are not defined.CASE SUMMARY We present the first case of a 6-month-old girl with trisomy 21 affected by a complex congenital IAFP, which caused severe PH, successfully treated with an endovascular-surgical hybrid procedure. The novel technique comprised a multistep endovascular embolisation, including a superselective transarterial embolisation of the afferent vessels and a direct transhepatic embolisation of the dilated portal vein segment, combined with selective surgical ligation of the arterial branches that supply the fistula, which were too small to be embolised.The complex IAPF was also associated with severe cholestasis and intra/extrahepatic biliary tree dilatation, which was successfully treated by a temporary biliary drainage. At 24-mo follow-up, the hybrid endovascularsurgical procedure achieved complete occlusion of the complex IAPF and resolution of the PH. A comprehensive review of the literature on congenital IAPF management, focussed on alternative treatment strategies, is also reported.CONCLUSION The combined radiological-surgical approach is a safe and effective treatment option for complex IAPF and avoids major invasive surgery.

A symptomatic cyst of the ligamentum teres of the liver: A case report

Cysts of the liver ligaments are extremely rare and cysts of the ligamentum teres of the liver have been sporadically reported in the literature during the last century. The present report describes a case of a symptomatic patient with a cyst of the ligamentum teres of the liver. The patient presented with right upper quadrant pain and indigestion during the last 2 years. Ultrasound and computed tomography scans revealed a water-density mass attached to the anterior abdominal wall, but definite diagnosis could not be reached. The cyst was completely excised during laparotomy. Cysts of the ligamentum teres of the liver, although infrequent, may produce clinical symptoms and require excision. Ultrasound and computed tomography scan preoperatively cannot rule out malignancy, thus exploratory laparotomy and total resection of these lesions are necessary.

Congenital biliary atresia caused by GPC1 gene mutation in Chinese siblings:A case report

BACKGROUND Congenital biliary atresia(CBA)is a serious hepatobiliary disease in children with unknown etiology.Its outcome is often liver transplantation or death.Clarifying the etiology of CBA is of great significance for prognosis,treatment,and genetic counseling.CASE SUMMARY A male Chinese infant at an age of 6 mo and 24 d was hospitalized because of"yellow skin for more than 6 mo".Soon after birth,the patient developed jaundice,which then progressively intensified.A"laparoscopic exploration"indicated"biliary atresia".After coming to our hospital,genetic testing suggested a GPC1mutation[loss 1(exons 6-7)].The patient recovered and was discharged after living donor liver transplantation.After discharge,the patient was followed up.The condition was controlled by oral drugs,and the patient’s condition was stable.CONCLUSION CBA is a complex disease with a complex etiology.Clarifying the etiology is of great clinical importance for treatment and prognosis.This case reports CBA caused by a GPC1 mutation,which enriches the genetic etiology of biliary atresia.However,its specific mechanism needs to be confirmed by further research.

Type Ⅰ congenital extrahepatic portosystemic shunt treated by orthotopic liver transplantation: A case report

BACKGROUND Congenital extrahepatic portosystemic shunt, also known as Abernethy deformation, is a rare malformation caused by dysplasia in the portal vein system. There are few reports of liver transplantation as a treatment for Abernethy deformation, and our report is the first case in China. This is the second reported case with congenital extrahepatic portosystemic shunt combined with focal nodular hyperplasia and hepatopulmonary treated with liver transplantation.CASE SUMMARY The patient was a 14-year-old girl, diagnosed preoperatively as type Ib Abernethy deformation, intrahepatic multiple space-occupying lesion, and hepatopulmonary syndrome. The patient recovered well after undergoing classic orthotopic liver transplantation. Liver function, pulmonary function, and portal vein computed tomography angiography imaging were reexamined 20 mo postoperatively, and no abnormality was observed.CONCLUSION Liver transplantation is an effective treatment for type I Abernethy deformation combined with focal nodular hyperplasia and hepatopulmonary syndrome.

Long-lasting symptoms and diagnostics in a patient with unrecognized right sided heart failure: Why listening to the heart is so important

M Ebstein is usually diagnosed in early childhood or ad-olescence. The young woman in our case complainted of fatigue and shortness of breath, on physical exami-nation she showed signs of heart failure with raised central venous pressure, palpable liver, an a cardiac murmer. Based on these findings she should have been referred to a cardiologist in an early stage after which transthoracic echocardiography resulted in the correct diagnosis. In this case the anomaly was missed for many years by different specialists and the patient was treated for liver disease, while she was suffering from liver congestion due to right-sided heart failure.

Clinical Observation on the Long-Term Therapeutic Effects of Traditional Chinese Medicine for Treatment of Liver Fibrosis

48 patients with liver fibrosis due to hepatitis B were treated for 2 years with the drugs for tonifying the kidney, supplementing qi, cooling and invigorating the blood and detoxification. The symptoms were markedly improved, and serum ALT and bilirubin were recovered and kept normal in most of the cases. The mean levels of serum hyaluronic acid, procollagen peptide III and circulating immune complex were decreased and returned to normal after the treatment. B-ultrasonography showed that the portal vein kept in normal size in 82% of the patients, the enlarged portal vein diminished in diameter, and the enlarged spleen reduced.

Intestinal obstruction due to giant liver cyst:A case report

BACKGROUND Congenital hepatic cysts are relatively rare but are now diagnosed earlier and more frequently with a routine prenatal ultrasound.Solitary liver cysts are divided into simple and solitary intrahepatic biliary cysts,depending on the biliary connection.While some solitary liver cysts are symptomatic in childhood,even in newborns,they are often found incidentally in adults.CASE SUMMARY A 3-mo-old female infant was admitted to Mogadishu Somali Training and Research Hospital with recurrent vomiting,respiratory problems,and abdominal bloating complaints.On examination,the abdomen was greatly distended and extremely tight.She had repeated vomiting for 3 d,no stool output,and decreased urine.The abdominal ultrasonography detected a solitary cystic lesion measuring 10 cm×10 cm×14 cm,extending from the liver or right kidney to the pelvis.In the magnetic resonance imaging examination of the patient,a solitary cystic structure of 10 cm×10 cm×14 cm in the right abdomen was observed,extending to the pelvis and possibly originating from the liver.The patient was operated via fenestration after her fluid and electrolytes improved.Oral nutrition was initiated on the 2nd postoperative day,and the drain was removed on the 5th postoperative day.The patient visited the outpatient clinic control 1 mo later with no clinical complaints.CONCLUSION Congenital liver cysts are usually followed without complications.They rarely reach gigantic dimensions and may cause respiratory distress,intestinal obstruction and recurrent vomiting.Surgery can provide quite successful outcomes in the treatment of giant sized simple liver cysts.

Protective effect of exogenous adenosine triphosphate on hypothermically preserved rat liver

AIM:To clarify the protective effect of exogenous adenosine triphosphate(ATP)on hypothermically preserved rat livers. METHODS:Establishment of continuous hypothermic machine perfusion model,detection of nucleotides in hepatocytes with HPLC,measurement of activities of LDH and AST in the perfusate,observation of histopathological changes in different experiment groups,and autoradiography were carried out to reveal the underlying mechanism of the protective effect of ATP. RESULTS:The intracellular levels of ATP and EC decreased rapidly after hypothermic preservation in control group,while a higher ATP and EC level,and a slower decreasing rate were observed when ATP-MgCl_2 was added to the perfusate (P<0.01).As compared with the control group,the activities of LDH and AST in the ATP-MgCl_2 group were lower(P<0.05). Furthermore,more severe hepatocyte damage and neutrophil infiltration were observed in the control group.Radioactive [α-^(32)P]ATP entered the hypothermically preserved rat hepatocytes. CONCLUSION:Exogenous ATP has a protective effect on rat livers during hypothermical preservation.However,Mg^(2+) is indispensable,addition of ATP alone produces no protective effect.The underlying mechanism may be that exogenous ATP enters the hypothermically preserved rat liver cells.

The Combined Use of Tonification and Purgation in Treatment of Fatty Liver

Fatty liver is a disease caused by excessive accumulation of fat in the liver due to long-term ort repeated affection of various causative factors'When the pathogenic factor prevails,the qi is sure to be deficient',Therefore,deficiency of the vital-qicontributes greatly to the occurrence of fatty liver.For many years,the author has concentrated ont the treatment of fatty liver with the tonificatioin method,or tonification followed by purgation,or the combination of tonificaytioin with purgation,so as to effectively prevent the liver from being injured by harmful substances ,and to benefit the recovery of fatty liver.The following is a report of his experience in treatment of fatty liver with the combined use of tonification and purgation.

Caroli Disease: A Case Report Observed at the Cocody Teaching Hospital of Abidjan (Cote D’Ivoire)

Caroli disease is a rare affection. It’s commonly associated to polycystic kidneys. We report a case of a nine-year-old girl. She was hospitalized at the pediatric unit of the Cocody Teaching Hospital for an intermittent fever. During the clinical examination, we found a likely cirrhotic hepatomegaly associated to an edematous-ascitic syndrome. Biological exams showed an infectious syndrome with a hyperleukocytosis at 13.000 per mm3, a hyperneutrophilia at 9.600 per mm3. Serological viral markers of B, C and D hepatitis were negative. We saw an intra-hepatic cystic picture at the liver CT-scan with a vascular lesion at his center called a “dot sign”. The diagnosis of Caroli disease could be evocated.

The deleterious effects and potential therapeutic strategy of fluorene-9-bisphenol on circadian activity and liver diseases in zebrafish and mice

Increasing evidence indicates that disturbance of the clock genes,which leads to systemic endocrine perturbation,plays a crucial role in the pathogenesis of metabolic and liver diseases.Fluorene-9-bisphenol(BHPF)is utilized in the manufacturing of plastic materials but its biological effects on liver homeostasis remain unknown.The impacts and involved mechanisms of BHPF on the liver diseases,metabolism,and circadian clock were comprehensively studied by zebrafish andmouse models.The therapeutic effect of melatonin(MT)was also verified.Zebrafish and mousemodels with either acute exposure(0.5 and 1μmol/L,1-4 days post-fertilization)or chronic oral exposure(0.5 and 50 mg/(kg·2 days),30 days)were established with various BHPF concentrations.Herein,we identified a crucial role for estrogenic regulation in liver development and circadian locomotor rhythms damaged by BHPF in a zebrafish model.BHPF mice showed chaos in circadian activity through the imbalance of circadian clock component Brain and Muscle Aryl hydrocarbon receptor nuclear translocator-like 1 in the liver and brain.The liver sexual dimorphic alteration alongwith reduced growth hormone and estrogens played a critical role in damaged glucose metabolism,hepatic inflammation,and fibrosis induced by BHPF.Besides,sleep improvement by exogenous MT alleviated BHPF-related glucose metabolism and liver injury in mice.We proposed the pathogenesis of metabolic and liver disease resulting from BHPF and promising targeted therapy for livermetabolism disorders associated with endocrine perturbation chemicals.These results might play a warning role in the administration of endocrine-disrupting chemicals in everyday life and various industry applications.

Long-term results of laparoscopic fenestration for patients with congenital liver cysts

BACKGROUND: The feasibility and immediate outcome of laparoscopic fenestration for patients with congenital liver cysts have been generally recognized. The aim of the present study was to assess the long-term results after laparoscopic fenestration. METHODS: A retrospective study was performed on 44 patients with congenital liver cysts who had undergone laparoscopic fenestration between June 1998 and December 2004. Among them, 30 were women and 14 men, aged 57 years on average (range 18-76 years). While 14 patients had solitary cysts, 20 had multiple cysts, and 10 had polycystic liver disease. The results of laparoscopic fenestration, including mortality, morbidity, mean postoperative hospital stay, and recurrences of cysts were evaluated. RESULTS: There was no perioperative death and the morbidity rate was 11% (5/44). All complications were treated conservatively. The mean postoperative hospital stay was 4 days. A mean follow-up of 57 months showed that the rates of cyst recurrence and symptom recurrence were 9% and 4.5%, respectively. CONCLUSION: Laparoscopic fenestration as a feasible and safe treatment procedure for patients with congenital liver cysts can yield very good long-term results.

Epidemiological and histopathological study of relevance of Guizhou Maotai liquor and liver diseases

AIM: To explore the relevance of Maotai liquor and liver diseases. METHODS: Epidemiological study was conducted on groups of subjects, each consisting of 3 subjects from the Maotai liquor group consisting of 99 individuals and one from the non-alcoholic control group consisting of 33 individuals. Liver biopsy was performed on 23 volunteers from Guizhou Maotai Distillery who had a constant and long history of drinking Maotai liquor. Experimental histopathological study was conducted as follows: sixty male Wistar rats were divided into 3 groups randomly and fed with Maotai liquor, ordinary white wine, and physiological saline respectively for a period of 8 and 12 weeks. The rats were sacrificed in batches, then serum ALT, AST, TBil, and AKP were measured. Rat livers were harvested to measure the liver indexes, GSH, and MDA. Histopathological examinations were also performed. Another eighty mice were randomly divided into 4 groups and fed with Maotai (at different dosages of 10 ml.kg(-1) and 20 ml.kg(-1)), ethanol, and physiological saline. The animals were sacrificed after 4 weeks and serum ALT was determined. Then the livers were harvested and liver indexes and MDA were measured. RESULTS: The incidence rate of hepatic symptoms, splenomegaly, liver function impairment, reversal of Albumin/Globulin and increased diameter of portal veins in the Maotai liquor group were 1.0% 1/99 , 1.0% 1/99 , 1.0% 1/99 , 1.0% 1/99 , 0 0/99 and 0 0/99 , 0 0/99 ,0 0/99 , 0 0/99 , 0 0/99 , respectively. There was no significant difference between the Maotai group and the non-alcoholic control group P】0.05 . Various degree of fatty infiltration of hepatocytes was found in the 23 volunteers receiving liver biopsy, but there was no obvious hepatic fibrosis or cirrhosis. A comparison was made between the Maotai liquor group and the ordinary white wine group. It was found that hepatic MDA in rats and mice were 0.33+/-0.10 and 0.49+/-0.23 respectively in Maotai group and 0.61+/-0.22 and 0.66+/-0.32 in the ordinary white wine group; MDA had an obvious decrease in the Maotai liquor group (P【0.05); hepatic GSH were 0.12 mg.g(-1)+/-0.06 mg.g(-1) in rats of the Maotai liquor group and (0.08+/-0.02)mg.g(-1) in white wine group, it was obviously increased in the Maotai liquor group (P【0.05). After the 20 rats had been fed with ordinary white wine for 8 weeks consecutively, disarranged hepatocyte cords, fatty infiltration of hepatocytes, and fibrous septa of varying widths due to hepatic connective tissues proliferation were observed; after 12 weeks, the fibrous tissue proliferation continued and early cirrhosis appeared. Compared with the ordinary white wine group, fatty infiltration was observed in the 8-week and 12-week groups, but no necrosis or fibrosis or cirrhosis was found in the Maotai liquor group (P【0.05). CONCLUSION: Maotai liquor may cause fatty liver but not hepatic fibrosis or cirrhosis, and it can strengthen lipid peroxidation in the liver.

Liver cell adenoma with malignant transformation:A case report

A 57-year-old woman was referred to our hospital because of a liver mass detected by computed tomography.She had taken oral contraceptives for only one month at the age of thirty.Physical examination revealed no abnormalities,and laboratory data,including hepatic function tests,were within the normal range,with the exception of elevated levels of those serum proteins induced by the absence of vitamin K or by raised levels of the antagonist (PIVKA)-Ⅱ (3 502 AU/ml). Abdominal ultrasonography revealed a hyperechoic mass measuring 10×10 cm in the left posterior segment of the liver.Because hepatocellular carcinoma could not be completely excluded,this mass was resected.The tumor consisted of sheets of uniform cells with clear cytoplasm, perinuclear eosinophilic granules and round nuclei.These histological findings were consistent with liver cell adenoma. Background hepatic tissue appeared normal.After resection of the tumor,serum PIVKA-Ⅱ fell to within the normal range. An area of hepatocellular carcinoma (HCC) with a mid- trabecular pattern was immunohistochemically found,which was positive for PIVKA-Ⅱ.Sinusoidal endothelial cells were CD34-positive,containing scattered PIVKA-Ⅱ positive cells. This tumor was therefore finally diagnosed as liver cell adenoma with focal malignant transformation to HCC.

Expression of lipopolysaccharide binding protein and its receptor CD14 in experimental alcoholic liver disease

AIM: To evaluate the relationship between the expression of lipopolysaccharides (LPS) binding protein (LBP) and CD14 mRNA and the severity of liver injury in alcohol-fed rats. METHODS: Twenty Wistar rats were divided into two groups:ethanol-fed group (group E) and control group (group C). Group E was fed with ethanol(5-12 g x kg(-1) x d(-1)) and group C received dextrose instead of ethanol. Rats of the two groups were sacrificed at 4 weeks and 8 weeks. Levels of endotoxin and alanine transaminase (ALT) in blood were measured, and liver pathology was observed under light and electronic microscopy. Expressions of LBP and CD14 mRNA in liver tissues were determined by RT-PCR analysis. RESULTS: Plasma endotoxin levels were increased more significantly in group E(129+/-21) ng x L(-1) and (187+/-35) ng x L(-1) at 4 and 8 wk than in control rats(48+/-9) ng x L(-1) and (53+/-11) ng x L(-1), respectively (P【0.05). Mean values of plasma ALT levels were (1867+/-250) nkat x L(-1) and (2450+/-367) nkat x L(-1) in Group E. The values were increased more dramatically in ethanol-fed rats than in Group C after 4 and 8 weeks. In liver section from ethanol-fed rats, there were marked pathological changes (steatosis, cell infiltration and necrosis). In ethanol-fed rats, ethanol administration led to a significant increase in LBP and CD14 mRNA levels compared with the control group (P【0.05). CONCLUSION: Ethanol administration led to a significant increase in endotoxin levels in serum and LBP and CD14 mRNA expressions in liver tissues. The increase of LBP and CD14 mRNA expression might wake the liver more sensitive to endotoxin and liver injury.

Plasma erythropoietin levels in anaemic and non-anaemic patients with chronic liver diseases

AIM: To investigate the serum erythropoietin (Epo) levels in patients with chronic liver diseases and to compare to subjects with iron-deficiency anaemia and healthy controls.METHODS: We examined 31 anaemic (ALC) and 22 non-anaemic (NALC) cirrhotic patients, 21 non-anaemic subjects with chronic active hepatitis (CAH), 24 patients with iron-deficiency anaemia (ID) and 15 healthy controls. Circulating Epo levels (ELISA; R&D Systems, Europe Ltd, Abingdon,UK) and haemoglobin (Hb) concentration were determined in all subjects.RESULTS: Mean±SD of Epo values was 26.9±10.8 mU/mL in ALC patients, 12.5±8.0 mU/mL in NALC subjects,11.6±6.3 mU/mL in CAH patients, 56.4±12.7 mU/mL in the cases of ID and 9.3±2.6 mU/mL in controls. No significant difference (P>0.05) was found in Epo levels between controls, CAH and NALC patients. ALC individuals had higher Epo levels (P<0.01) than these groups whereas ID subjects had even higher levels (P<0.001) than patients suffering from ALC.CONCLUSION: Increased Epo values in cirrhotics, are only detectable when haemoglobin was lesser than 12 g/dL.Nevertheless, this rise in value is lower than that observed in anaemic patients with iron-deficiency and appears blunted and inadequate in comparison to the degree of anaemia.

Peripheral blood lymphocytes DNA in patients with chronic liver diseases

BACKGROUND: Viral replication in blood cells with nucleases may lead to the damage of lymphocytes genetic apparatus and the beginning of immunopathological reactions. AIM: Of this investigation is to reveal the damage to peripheral blood lymphocytes (PBL) DNA in the patients with chronic liver diseases. MATERIALS AND METHODS: Sixteen-nine patients with chronic liver diseases (37 patients with chronic viral hepatitis, 2 patients with liver cirrhosis of mixed etiology (alcohol+virus G), 30 women with primary biliary cirrhosis-PBC) were examined. The condition of DNA structure of PBL was measured by the fluorescence analysis of DNA unwinding (FADU) technique with modification. Changes of fluorescence (in %) reflected the DNA distractions degree (the presence of DNA single-stranded breaks and alkalinelabile sights). RESULTS AND CONCLUSION: The quantity of DNA single-stranded breaks and alkalinelabile sights in DNA in all patients with chronic viral hepatitis didn't differ from the control group, excluding the patients with chronic hepatitis (CH) C+G. Patients with HGV and TTV monoinfection had demonstrated the increase of the DNA single-stranded breaks PBL quantity. This fact may be connected with hypothesis about the viruses replication in white blood cells discussed in the literature. Tendency to increase quantity of DNA PBL damages in the patients with primary biliary cirrhosis (PBC) accordingly to the alkaline phosphatase activity increase was revealed. Significant decrease of the DNA single-stranded breaks and alkalinelabile sights in the PBC patients that were treated with prednisone was demonstrated. Probably, the tendency to increase the quantity of DNA single stranded breaks and alkalinelabile sights in lymphocytes of the PBC patients was depended on the surplus of the blood bile acid content.