Deep Jaundice Research Articles

Mifepristone induced liver injury in a patient with Cushing syndrome: a case report and review of the literature

BackgroundMifepristone, also known as RU-486, is an anti-progestational steroid with similar chemical structure to anabolic steroids. Given as a single dose in conjunction with misoprostol, mifepristone is used to induce medical abortion. Mifepristone administered chronically at a higher dose is also approved for the management of hypercortisolism. There have been only 2 reported cases of mifepristone associated liver injury, in both cases, in the setting of Cushing syndrome. We report a third patient with Cushing syndrome with mifepristone induced liver injury with unique histological findings that provide insight to the pathophysiology of liver injury in mifepristone and anabolic steroids.Case presentationPatient is a 63-year-old Caucasian female Cushing disease with no prior history of liver disease. She was started on mifepristone and semaglutide. Ninety days after initiating mifepristone, she developed deep jaundice, severe pruritus, fatigue, and nausea. Liver tests revealed a mixed hepatocellular/cholestatic pattern. Viral and autoimmune serologies were negative and there was no biliary dilatation on imaging. Liver biopsy showed severe cholestasis but no bile duct injury. Focal endothelialitis was present within a central venule. Cholestatic symptoms persisted for one month after presentation before slowly subsiding. Four months after stopping mifepristone, the patient’s symptoms completely resolved, and liver tests became normal. Compilation of Roussell Uclaf Causality Assessment Method score indicated probable causality.ConclusionsMifepristone shares a similar chemical structure as synthetic anabolic/androgenic steroids and there are many similarities in the clinical presentation of liver injury. This case and the 2 other reported cases share similar clinical characteristics. The observation of endothelialitis in our patient may provide a mechanistic link between mifepristone, or anabolic steroids in general, and the development of vascular complications such as peliosis.

S3274 A Rare Case of Benign Recurrent Intrahepatic Cholestasis (BRIC) Presenting as an Unexplained Cholestatic Jaundice

Introduction: Benign Recurrent Intrahepatic Cholestasis (BRIC) is a very rare autosomal recessive disorder characterized by episodes of recurrent cholestatic jaundice followed by complete resolution. BRIC is associated with a mutation in both alleles of ATP8B1 (BRIC 1) or ABCB11 (BRIC2). It causes cholestasis by impairing the function of the bile salt export pump. Recognition of BRIC is important as it can lead to delayed or no diagnosis, also it is underrecognized and challenging diagnosis. Case Description/Methods: A 19-year-old South Asian male presented with nausea, vomiting, deep jaundice and pruritus of 10 days duration. Family history was negative for jaundice. He was not consuming alcohol or any drugs. Past medical records revealed three past episodes of undiagnosed cholestatic jaundice lasting for three to four weeks with complete recovery at the age of 8, 11 and 14 years respectively. Physical examination revealed stable vitals, deep icterus, cutaneous scratch marks and mild tender hepatomegaly. No stigmata of acute or chronic liver disease were identified. PT was prolonged with an INR of 1.8. AST and ALT were initially raised on admission which gradually trended down while serum total bilirubin, direct bilirubin and alkaline phosphatase continued to raise over 8 weeks. GGT remained within normal limits (Table 1). Viral markers for hepatitis A, B, C, E; HIV 1&2; EBV and CMV were negative. Workups for autoimmune hepatitis and Wilson's disease were negative. Ultrasound of the abdomen and MRCP were unremarkable. A liver biopsy was performed which showed intrahepatic and canalicular cholestasis predominantly involving zone 3 suggestive of BRIC (Figure A). He was treated with intravenous fluids, antiemetics, cholestyramine and antihistamines for symptomatic relief. Because of severe pruritus, Rifampicin was added later on for 14 days resulting in significant improvement. Liver function tests returned to baseline after 3 months. The patient was followed up for 6 months and remained asymptomatic. Discussion: Till date very few cases of BRIC have been reported, which may be due to the rarity of the condition compounded by under-recognition by clinicians. Despite being a genetic disease, most cases are sporadic. Diagnosis in our case was based on the past episodes of jaundice, present clinical features, laboratory parameters and liver biopsy findings as proposed by Luketic and Shiffman for the diagnosis of BRIC. This is a self-limiting disease with no residual damage and treatment is symptomatic.Figure 1.: A. Liver Histology (H&E × 400): Intrahepatic cholestasis (black arrow) with preserved liver architecture. Table 1. - Laboratory results T. Bilirubin (mg/dl) D. Bilirubin (mg/dl) ALP (U/L) GGT (U/L) ALT (U/L) AST (U/L) Day – 1 8.2 6.1 388 15.2 415 205 Day – 7 12.4 9.8 421 15.4 354 106 Day – 14 14.6 12.2 510 14.8 156 88 Day – 21 18.0 16.5 615 14.5 80 60 Day – 31 21.5 18.3 830 12 48 32 Day – 45 24.32 22.45 950 11.3 38 22 Day – 60 26.82 24.13 1152 10.1 36 20 Day – 75 11.24 9.12 353 9.2 32 18 Day – 90 1.6 0.8 86 9 28 15

POS-762 UNUSUAL PRESENTATION OF TACROLIMUS TOXICITY – CHOLESTATIC SYNDROME IN RENAL ALLOGRAFT RECIPIENT

Tacrolimus (TAC) holds a key role to a successful organ transplantation and allograft survival in today’s world, but has a narrow therapeutic window, necessitating therapeutic drug monitoring to maintain efficacy and minimize toxicity. Except transaminitis, hepatotoxicity due to TAC, has rarely been reported in a renal allograft recipient. We report here an unusual presentation of TAC toxicity in in a live donor renal allograft recipient 5 years (yr) post successful engraftment presented as cholestatic hepatitis without transaminitis. 32 year male, sister to brother compatible (O Positive), 3/6 HLA mismatch, crossmatch negative by CDC and flow cytometry, 5 years post renal allograft recipient hospitalized with deep jaundice and unremarkable systemic examination. Pre transplant and recent evaluation was inconclusive for acute/chronic infection/inflammation or acute hematological etiology for cholestasis. Compliance with daily therapeutic dose of maintenance immunosuppression was noted. TAC trough levels was 4.7. In a week long management, treatment included modified immunosuppression - Mycophenolate (MMF) stopped, Intravenous steroids, TAC (3mg/day), antibiotics and ursodeoxycholic acid to relieve cholestasis. Thoroughly evaluated, ultrasound abdomen, graft kidney and doppler of spleno-portal axes was also unremarkable, we planned for MRCP and liver biopsy, but his hyperbilirubinemia improved spontaneously and persistently following decrease in TAC dose (1mg/day) on 4th day of hospitalization and subsequently withdrawal and switching to everolimus. To the best of our knowledge Yadav et al. and Mesar et al. have reported isolated cases of cholestatic syndrome with transaminitis in immediate post-renal transplant period respectively attributed to TAC. Our patient presented with direct hyperbilirubinemia 5yr post-transplant without transaminitis, maintained on therapeutic dose and trough levels of TAC. Evaluated thoroughly for cholestasis, clinical, biochemical improvement occurred immediately following tapering and subsequently stopping TAC. Trend of Bilirubin, Liver enzymes, TAC dose and Creatinine levels Cholestatic syndrome in renal allograft recipients due to TAC has rarely been reported. We thus conclude that cholestatic jaundice in our patient was attributed to therapeutic dose of TAC, having excluded other possible causes. The event can thus occur with therapeutic dose or trough levels of TAC and anytime in the post-transplant course. Thus we propose that transplant Physicians should be vigilant in following up their allograft recipients and further insight on the pharmacokinetic and pharmacodynamic profile of TAC should be thought about.

COVID-19 and Stress: A Young Male Presenting with Central Cyanosis, Dark Urine, Oliguria, Jaundice and Anemia due to Methemoglobinemia as a result of Suicidal Naphthalene Mothball Poisoning Recovered with Exchange Transfusion

A young man presented with abdominal pain and vomiting after eating Naphthalene Mothball. He had dyspnea, central cyanosis (SaO2 on air was 67% on air), marked pallor, deep jaundice and dehydration. His urine color was black; and, his plasma in clotted blood sample was brownish. He was treated as methemoglobinemia due to suicidal Naphthalene Mothball poisoning with fluid and electrolyte replacement, ascorbic acid, N-acetylcystine and exchange transfusion twice with four units of whole blood. Dramatic improvement in central cyanosis immediately following exchange transfusion. Psychiatric consultation and counselling were done; he admitted the main reason for committing suicide was socioeconomic stress due to COVID-19.

Validation of visual estimation of neonatal jaundice in low-income and middle-income countries: a multicentre observational cohort study

ObjectiveDetermine the sensitivity and specificity of neonatal jaundice visual estimation by primary healthcare workers (PHWs) and physicians as predictors of hyperbilirubinaemia.DesignMulticentre observational cohort study.SettingHospitals in Chandigarh and Delhi, India; Dhaka,...

Efficacy and safety of rifampicin in patients with persistent hepatocellular secretory failure.

Persistent hepatocellular secretory failure (PHSF) is a rare condition of severe cholestasis caused by drugs, toxins, infection, or temporary biliary obstruction. Real-world data on rifampicin in cholestasis, particularly among patients with deep jaundice, are scarce. We aimed to expand the knowledge on the efficacy and safety of rifampicin treatment in PHSF patients. Sixteen patients with PHSF who had received rifampicin treatment (150-300mg/d) at our institution from September 2016 to July 2020 were included. Treatment efficacy was assessed by 20% improvement in serum total bilirubin (TBIL) concentration at 4weeks. Follow-up was continued until the concentration of TBIL returned to normal. Among the 16 enrolled patients, 12 had predisposing factors (drugs, infection, or transient biliary obstruction). ATP8B1 or ABCB11 mutations were detected in the other four patients without trigger events. UGT1A1 mutations were found in 7/10 patients. Before rifampicin treatment, the median TBIL level was 352μmol/L (range 171-591μmol/L). TBIL>20% improvement was observed in 14 patients at 4weeks. TBIL levels of 14 patients eventually returned to normal after 6-12weeks of rifampicin treatment. The remaining two patients who did not respond to rifampicin finally recovered after nasobiliary drainage. Except for one patient with transient drug-induced hepatitis, no other serious adverse events were observed. Rifampicin could be a promising option for most PHSF patients. Most PHSF patients have UGT1A1 deficiency, which may be the target of rifampicin.

A multicenter prospective randomized controlled trial for preoperative biliary drainage with uncovered metal versus plastic stents for resectable periampullary cancer.

Although routine preoperative biliary drainage (PBD) in patients with distal malignant biliary obstruction is generally not recommended, there are still various situations where it may be necessary. The current study aims to compare the uncovered self-expandable metal stent (uSEMS) and plastic stent (PS), where PBD may be necessary. In this multicenter prospective randomized study, patients with resectable periampullary cancer with cholangitis, deep jaundice, or expected long waiting time for surgery were included. PBD was performed endoscopically, but percutaneous drainage was allowed if the initial endoscopic drainage was not feasible. The primary outcome was the reintervention rate; the secondary outcomes were the complication rates, rate of decrease of total bilirubin, waiting time for surgery, and postoperative hospital stay. Of the 60 enrolled patients, 53 were included for analysis (26 PS and 27 uSEMS). Common bile duct cancer was the most common (27, 50.9%), followed by pancreatic head cancer (20, 37.7%). Regarding PBD indication, 36 (67.9%) had cholangitis and 21 (39.6%) had a total bilirubin level of more than 10mg/dL at randomization; 10 (18.9%) were included due to delayed surgery by more than 7days. Fifty (94.3%) patients received pancreaticoduodenectomy, and one (1.9%) patient received palliative hepaticojejunostomy. The median waiting time for surgery was 11.0days. There was no difference in the reintervention rate (3.8% and 3.8% in PS and uSEMS, P>.999), PBD-related complication rate (23.1% and 22.2%, P>.999), PBD- or surgery-related complication rate (57.7% and 48.1%, P=.674), and the rate of decrease of total bilirubin (P=.541). The median hospital stay after surgery was 13.0days without significant difference. For patients who received surgery within the first 2weeks from receiving PBD, there was no superiority of uSEMS to PS. According to the expected waiting time for surgery, selective approach for stent choice should be considered.

Autoimmune Thrombotic Thrombocytopenic Purpura, Disseminated Intravascular Coagulation, Haemophagocytic Lympho Histiocytosis—(Following Corona Virus Infection), Secondary to Fragmented Germ Cells {Liberal Global Abortions, Contraception—(Red Riverways, Blood Moon, Weakened Earth’s Magnet)} and Absence of Abstinence—Retrospective Analysis: Proof of Concept Study

Background: Autoimmunity has increased significantly, alarmingly secondary to global contraception, abortion, fragmenting germ cells; herewith three types of life threatening manifestations, are analysed. Case-1: 28-year-old afebrile lady, vegetarian, presented to emergency with impaired responsiveness, deep jaundice, menorrhagia of one day duration and she had been taking native treatment with leaves for primary infertility for the past five years; she had a few ecchymotic patches, investigations showed severe anaemia, thrombocytopenia, haemolytic jaundice, peripheral smear confirmed thrombotic thrombocytopenic purpura; she developed profound hematuria; she received treatment with intravenous immunoglobulin, fresh frozen plasma, platelet transfusions, packed red blood cells transfusions, plasmapheresis was planned; she improved gradually. In her sincere effort to conceive a baby, for the past five years, due to cultural ignorance which teaches only three days abstinence, instead of seven days abstinence after last menstrual periods, exposure of germ cells to raw endometrial surface, from 4th to 7th days of menstrual cycle, resulted in the cumulative autoimmunity-thrombotic thrombocytopenic purpura status. Case-2: 24-year-old lady who had undergone puerperal sterilisation, on the fourth day after last menstrual periods, presented, with unresponsiveness, to emergency, her investigations revealed prolonged prothrombin time, prolonged activated partial thromboplastin time, more than a minute, thrombocytopenia, reduced fibrinogen levels suggesting disseminated intravascular coagulation; her ultra sonogram abdomen showed, absence of gestational sac in the uterus; she received fresh frozen plasma transfusions, but succumbed within few hours. With puerperal sterilisation, [non evidence based medical practice], 20 million germ cells are fragmented to chromatid breaks, a-centric fragments, ring chromosomes, which get exposed to partly regenerated endometrial surface, on the fourth day after last menstrual periods, due to lack of awareness of abstinence upto seven days, initiating severe cumulative autoimmunity, resulting in fatal disseminated intravascular coagulation. Case-3: Fatal haemo-phagocytic lympho-histiocytosis-soaring autoimmunity, detected in the victim host of corona virus infection, correlated directly with extensive tissue damage produced in the victim host, by prior surgical abortions. Conclusion: Cases one and two followed absence of abstinence, with exposure of germ cells, to partly healed endometrial surface in the former culminating in thrombotic thrombocytopenic purpura; in the latter by exposure of fragmented 20 million germ cells [by contraception] to partly healed endometrial surface, culminating in fatal disseminated intravascular coagulation. In case three fatal autoimmune hemophagocytic lymphohistiocytosis followed corona virus infection, secondary to extensive tissue damage, of liberal global abortions. Red Russian riverways, is another evidence for global warming due to global aborted blood pollution, melting Perma-Frost crashing the Diesel tanks, with oil spill reflecting on aborted blood polluted river, turns bright red, similar to blood moon by abortion polluted air. Innumerable human lives with their iron of blood flowing within blood vessels, repelling magnet of earth, is mandatory to strengthen earth’s magnet. Arctic ozone hole closed this May, environmental pollution cleared, by absence of global abortions, contraception, and their ozone depleting blood pollution, due to lock down of corona virus pandemic.

Characteristics and outcome of primary Budd-Chiari syndrome due to Behçet's syndrome

Behçet's syndrome (BS) is a known cause of Budd-Chiari syndrome (BCS). We aimed at identifying the prevalence of BS in patients with BCS, analyzing different clinical presentations, treatment modalities and outcome of these patients. We conducted a retrospective cohort study, in which all medical records of patients who were presented to Tropical Medicine Department, Ain Shams University with a confirmed diagnosis of primary BCS from May 2005 to December 2016 were collected and analyzed. In total, 271 patients had a confirmed diagnosis of primary BCS, included Group I: 232 (85.6%) patients with BCS without BS and Group II: 39 patients (14.4%) with BCS due to BS. Male gender (P=0.000), oral ulcers, genital ulcers, Prominent abdominal veins, lower limb swellings, lower extremity deep venous thrombosis (P=0.000) and jaundice (P=0.003) were more frequent in group II patients. The presence of intrahepatic collaterals (P=0.004) and IVC thrombosis (P=0.000) was significant in group II. Medical treatment alone in the form of immunosuppressive drugs and anticoagulation (66.7% vs. 24.1%)±IVC stenting (23% vs. 1.3%) (P=0.000) were the main treatment modalities for BCS related to BS. The frequency of HCC in BS was significantly higher (10.26% vs. 2.59%) (P=0.013). The prevalence of BS in Egyptian patients with BCS is considerably high. The clinical presentation of these patients was different from those without BS. Besides, the incidence of HCC was higher in patients with BS, whereas the mortality did not differ between the two groups.

Deep jaundice in a child with sickle cell disease.

Journal of Paediatrics and Child HealthVolume 53, Issue 6 p. 609-610 Images of the Month Deep jaundice in a child with sickle cell disease First published: 02 June 2017 https://doi.org/10.1111/jpc.2_13367Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume53, Issue6June 2017Pages 609-610 RelatedInformation

Hemophagocytic Lymphohistiocytosis Masquerading as Acute Liver Failure: A Single Center Experience

Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening disorder of extreme inflammation and unregulated immune response which require prompt recognition and early introduction of definitive therapy. HLH can present with wide range of hepatic dysfunction ranging from mild elevation of transaminases to liver failure. This study is carried out to describe the clinical and laboratory presentation of HLH. Patients who were diagnosed with HLH between January 2013 and December 2015 were retrospectively included in this study. Six patients were diagnosed as secondary HLH with median age of 28.5 years at diagnosis. All patients were presented with history of deep jaundice and high grade fever with pancytopenia and splenomegaly. Underlying diagnosis was viral infections in 4 and probable viral infection in remaining two. Bone marrow hemophagocytosis was present in 3 cases. Three patients were treated with corticosteroids only and one each with corticosteroids with cyclosporine or intravenous immunoglobulin (IVIG) and HLH treatment protocol. One patient died due to acute respiratory distress syndrome (ARDS); another patient died in follow-up due to respiratory failure due to pneumonia. HLH is rare and potentially life-threatening cause of prolonged fever, jaundice and pancytopenia. Early diagnosis and initiation of specific therapy can improve clinical outcome.

Conjugated hyperbilirubinemia presenting in first fourteen days in term neonates.

AIMTo describe the etiology and characteristics of early-onset conjugated hyperbilirubinemia (ECHB) presenting within 14 d of life in term neonates.METHODSRetrospective review was performed of term infants up to 28-d-old who presented with conjugated hyperbilirubinemia (CHB) at a tertiary center over a 5-year period from January 2010 to December 2014. CHB is defined as conjugated bilirubin (CB) fraction greater than 15% of total bilirubin and CB greater or equal to 25 μmol/L. ECHB is defined as CHB detected within 14 d of life. “Late-onset” CHB (LCHB) is detected at 15-28 d of life and served as the comparison group.RESULTSTotal of 117 patients were recruited: 65 had ECHB, 52 had LCHB. Neonates with ECHB were more likely to be clinically unwell (80.0% vs 42.3%, P < 0.001) and associated with non-hepatic causes (73.8% vs 44.2%, P = 0.001) compared to LCHB. Multifactorial liver injury (75.0%) and sepsis (17.3%) were the most common causes of ECHB in clinically unwell infants, majority (87.5%) had resolution of CHB with no progression to chronic liver disease. Inborn errors of metabolism were rare (5.8%) but associated with high mortality (100%) in our series. In the subgroup of clinically well infants (n = 13) with ECHB, biliary atresia (BA) was the most common diagnosis (61.5%), all presented initially with normal stools and decline in total bilirubin but with persistent CHB.CONCLUSIONSecondary hepatic injury is the most common reason for ECHB. BA presents with ECHB in well infants without classical symptoms of pale stools and deep jaundice.

Evaluation of Oxygen Saturation By Pulse Oximetry on Newborn Infant Using Skin Protective Covering

Introduction: Pulse oximetry is a convenient method of monitoring oxygen saturation (SpO2). Pulse oximeter probes have been reported to be associated with injuries in neonates. Objectives: This study was conducted with the objective of examining whether application of a protective covering influences values of SpO2. Materials and Methods: A cross-sectional study was carried out in the Department of Neonatology, BSMMU, Dhaka over a period of one year. Hospitalized 140 neonates within 28 postnatal days were included in the study. Neonates with major congenital malformation, heart disease, severe anemia, shock and deep jaundice were excluded. Readings of SpO2 were taken on right foot and right hand directly and through micropore by researcher himself when the saturation display was steadied. Results: The mean readings of SpO2 on foot directly and through micropore were 94.5±3.4% and 94.2±3.4% respectively. On right hand, the values were 94.3±3.3% and 94.5±3.3% respectively. For each of the 13 subgroups formed on the basis of gestational age, weight and postnatal age of the newborns, oximetry readings of two sites i.e. hand and foot were compared using t test and 95% confidence interval. Thus there were in all 26 statistical comparisons. Of these, in 22 comparisons the differences in the reading with micropore and without micropore were found statistically not significant. In the remaining 4, differences in 2 cases were significant at 0.04 and 0.05 level. The paired mean differences between readings of SpO2 by pulse oximeter without micropore and with micropore on foot (0.25±1.11) and hand (0.22±1.12) were very similar to paired mean difference of SpO2 readings by direct method (0.15±1.22) on hand and foot and were within this limit. Conclusions: The work concluded that the micropore protective covering did not influence oxygen saturation readings, suggesting that a covering can be satisfactorily used for protection of neonates from probe related injuries.

Accumulative Copper Poisoning in Sheep in Northeastern Brazil

Background: Despite the proven copper deficiency in several parts of Brazil, reports of acute and accumulative copper poisoning are increasing in ruminants. Sheep are known to be far more sensible to copper toxicity than cattle, but the sensibility of goats is not quite known. The present paper aimed to describe the epidemiological, clinical, laboratorial and pathological findings of two outbreaks of accumulative copper poisoning in sheep fed poultry litter in Ceará and Rio Grande do Norte, Northeastern Brazil, and to compare these findings with national outbreaks.Cases: Two outbreaks of copper poisoning occurred in the municipalities of Mossoró, Rio Grande do Norte [outbreak 1], and Icapuí, Ceará [outbreak 2], Brazil. A total of 35 sheep [outbreak 1] and 190 small ruminants (175 sheep and 15 goats) [outbreak 2] were fed poultry litter. First clinical signs of poisoning started after two months of litter consumption. Main clinical signs were apathy, jaundice, hepatomegaly, abdominal pain, diarrhea, and dark urine. Clinical evolution varied between one and eight days. Laboratorial findings in ill animals consisted of anemia, highly increased serum AST and GGT activities and urea and creatinine levels; whilst in the non symptomatic sheep and one goat revealed high and light GGT increase, respectively. Poultry litter analysis revealed levels of 449, 2610, 414 and 333 mg/kg of copper, iron, manganese and zinc, respectively. Analysis of copper levels by atomic absorption spectrometry in two sheep showed 730.1 and 1526.5 mg/kg in liver and 485.1 and 519.4 mg/kg in the kidneys. Treatment consisted of oral administration of 100 mg ammonium molibdate and 1 g sodium sulphate for five days. A total of 32 sheep died and necropsy of five animals showed deep jaundice, livers presenting a yellow to orange coloration, splenomegaly, hepatomegaly, blackish kidneys and dark urine. The microscopic examination of liver and kidneys showed degenerative and necrotic lesions.Discussion: This is the first report of copper poisoning in sheep from Rio Grande do Norte and Ceará, Northeastern Brazil. Even though it is not allowed in Brazil, the use of poultry litter for animal feeding still occurs and represents a great risk for copper poisoning in ruminants, especially sheep. The use of poultry litter should be discouraged to producers for feeding ruminants and requires more awareness of them that the use of this product is prohibited for this purpose. Clinical signs were typical of accumulative copper poisoning and the most evident changes in the serum biochemistry analyses were anemia and increased serum AST and GGT activities and urea and creatinine levels. Serum activity of GGT in a non symptomatic goat was slight increased, which is suggestive of an initial liver damage promoted by copper accumulation. Main pathological changes are associated to liver and kidney damage. Measurement of copper levels in liver and kidney fragments by atomic absorption spectrometry confirmed the diagnosis of poisoning by this element. Oral administration of ammonium molibdate associated to sodium sulphate was efficient for the treatment of poisoned sheep because both compounds reduce digestive absorption and increase excretion of copper. The absence of poisoning in goats fed the same poultry litter confirms that this species is more resistant than sheep to copper toxicity. Nevertheless further research must be carried out to test this sensibility. The use of poultry litter should be discouraged to producers for feeding ruminants and requires more awareness of them that the use of this product is prohibited for this purpose.Keywords: jaundice, hemoglobinuric nephrosis, poultry litter, small ruminants.

Hemophagocytic Lympho Histiocytosis (HLH)

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyper-inflammatory condition caused by a highly stimulated, but ineffective immune response, characterized by a dys-regulated activation and proliferation of macrophages leading to uncontrolled phagocytosis of platelets, erythrocytes, lymphocytes and their hematopoietic precursors throughout the reticulo-endothelial system. HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver, or lymph nodes. HLH also occurs as a complication of rheumatic diseases and of malignancies. Awareness of the clinical symptoms and of diagnostic criteria for HLH (Histiocyte society; HLH study 2004) is crucial to starting life-saving therapy in time. We have reported a case of 7-year-old male child presenting with respiratory distress, distension of abdomen and deep jaundice. This case report may be an eye opener to the pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted in those affected with this disease at the earliest.

Robotic Surgery Twice Performed in the Treatment of Hilar Cholangiocarcinoma With Deep Jaundice

To explore and find a new method to treat hilar cholangiocarcinoma with deep jaundice assisted by Da Vinci robot. A hilar cholangiocarcinoma patient of type Bismuch-Corlette IIIa was found with deep jaundice (total bilirubin: 635 µmol/L). On the first admission, we performed Da Vinci robotic surgery including drainage of left hepatic duct, dissection of right hepatic vessels (right portal vein and right hepatic artery), and placement of right-hepatic vascular control device. Three weeks later on the second admission when the jaundice disappeared we occluded right-hepatic vascular discontinuously for 6 days and then sustained later. On the third admission after 3 weeks of right-hepatic vascular control, the right hemihepatectomy was performed by Da Vinci robot for the second time. The future liver remnant after the right-hepatic vascular control increased from 35% to 47%. The volume of left lobe increased by 368 mL. When the total bilirubin and liver function were all normal, right hemihepatectomy was performed by Da Vinci robot 10 weeks after the first operation. The removal of atrophic right hepatic lobe with tumor in bile duct was found with no pathologic cancer remaining in the margin. The patient was followed up at our outpatient clinic every 3 months and no tumor recurrence occurs by now (1 y). Under the Da Vinci robotic surgical system, a programmed treatment can be achieved: first, the hepatic vessels were controlled gradually together with biliary drainage, which results in liver's partial atrophy and compensatory hypertrophy in the other part. Then a radical hepatectomy could be achieved. Such programmed hepatectomy provides a new treatment for patients of hilar cholangiocarcinoma with deep jaundice who have the possibility of radical heptolobectomy.

Reply to: doi:10.1007/s00464-013-3395-4: Re: “Laparoscopic bile duct exploration via choledochotomy followed by primary duct closure is feasible and safe…” (2013 (27):4164–4170)

To the editor, We agree that it is best to avoid laparoscopic common bile duct exploration (LCBDE) in patients with small diameter bile ducts (amongst our 120 patients who underwent LCBDE, one had a diameter of 3 mm, 3 of 4 mm and 4 of 5 mm). However, exploration of these is possible with the use of a 3-mm choledochoscope or via a trans-cystic duct approach. Whilst single-stage LCBDE is not the preferable option in high-risk patients, at times it becomes unavoidable when the endoscopic approach to management of ductal stones has failed. In our experience, LCBDE with primary closure can be performed in both elective and emergency cases. We performed emergency LCBDE in ten patients and encountered a postoperative chest infection in one and urinary tract infection in another but no bile leaks or duct strictures. Clearly though, patients with cholangitis, deep jaundice and coagulopathy should be optimised preoperatively and consideration should be given to preoperative percutaneous transhepatic biliary drainage as a temporising measure before embarking on LCBDE. With the use of a transverse choledochotomy and interrupted 4–0 Vicryl suture closure of non-dilated bile ducts (\6 mm diameter) at LCBDE (n = 8), we have encountered no postoperative bile leaks or bile duct strictures. We agree that endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic nasobiliary drainage have their application in the management of bile leak due to retained stones after LCBDE. However, re-laparoscopy has its role in patients with generalised abdominal signs and systemic disturbance as it facilitates a thorough abdominal washout as well as removal of retained duct stones with re-suturing of the bile duct. Nasobiliary drainage is not commonly adopted in the United Kingdom.

Extracorporeal liver support in severe alcoholic hepatitis.

The severity of alcoholic hepatitis (AH) which may coexist with cirrhosis varies greatly, from asymptomatic forms which are detected in alcoholic patients without any sign of liver disease, except laboratory abnormalities, to severe forms characterised by deep jaundice, ascites, hepatic encephalopathy and low prothrombin index. In hospitalized patients the mortality could be as high as 75%. The elevated number of therapeutic proposals reported for more than forty years reveals the lack of efficacy of a particular modality. Even in the most favorable trials, the survival is already very poor and in some cases related to the development of renal failure or hepatorenal syndrome. There are some motivating reports concerning albumin dialysis as a support treatment in patients with severe AH, either alone or in combination with other pharmacological therapies. The favorable effects of albumin dialysis in patients with severe AH suggest that the procedure used alone or in combination with other therapies may have a role in this clinical condition. This will be particularly relevant to offer an alternative therapy in these patients, thus being a potential bridge to recovery or to be listed for liver transplantation.

Is liver biopsy necessary in the management of alcoholic hepatitis?

Acute alcoholic hepatitis (AAH) is characterised by deep jaundice in patients with a history of heavy alcohol use, which can progress to liver failure. A clinical diagnosis of AAH can be challenging to make in patients without a clear alcohol history or in the presence of risk factors for other causes of acute liver failure. Other causes of acute on chronic liver failure such as sepsis or variceal haemorrhage should be considered. Liver biopsy remains the only reliable method to make an accurate diagnosis. However, there is controversy surrounding the use of liver biopsy in patients with AAH because of the risks of performing a percutaneous biopsy and limitations in access to transjugular biopsy. We review the existing literature and find there are few studies directly comparing clinical and histological diagnosis of AAH. In the small number of studies that have been conducted the correlation between a clinical and histological diagnosis of AAH is poor. Due to this lack of agreement together with difficulties in accessing transjugular liver biopsy outside tertiary referral centres and research institutions, we cannot advocate universal biopsy for AAH but there remains a definite role for liver biopsy where there is clinical diagnostic doubt or dual pathology. It also adds value in a clinical trial context to ensure a homogeneous trial population and to further our understanding of the disease pathology. Further prospective studies are required to determine whether non-invasive markers can be used to accurately diagnose AAH.

Extraosseous Bone Tracer Accumulation of the Liver on 99mTc-HDP Bone Scintigraphy in Intermittent Bleeding After Iatrogenic Liver Injury

A 69-year-old man with common bile duct cancer and deep jaundice had complained of abdominal pain after percutaneous transhepatic biliary drainage. The laboratory workup showed reduction of serum hemoglobin level after the procedure. A 99mTc-hydroxy diphosphonate bone scan was performed for further evaluation of common bile duct cancer, and there was no abnormality of the skeletal structures. However, diffuse and hazy increased radiotracer uptake was detected on the liver. Follow-up contrast-enhanced 99mCT revealed hematoma and subcapsular hemorrhage of the liver.