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Jaundice icterus [keywords]
- Coexistence of Congenital Syphilis and Cytomegalovirus Infection: A Case Report. [JOURNAL ARTICLE]
- Acta Dermatovenerol Croat 2014 Sep; 22(3):215-217.
A 22-year-old pregnant woman with an intravenous drug abuse habit delivered a girl in the 26th gestational week with a fetal length and weight of 38 cm/990 g (pc. 75-91). She did not participate in prenatal care that included screening for congenital diseases, syphilis, and human immunodeficiency virus (HIV) infection during the pregnancy. Laboratory examinations revealed positive rapid plasma reagin (RPR) (1:128), Treponema pallidum particle agglutination assay (TPPA) and TpELISA results. Immediately before delivery, labial herpes simplex virus-1 (HSV-1), Streptococcus agalactiae, and genital yeast infections were detected. Hepatitis B surface antigen (HbsAG), HIV, and hepatitis C virus (HCV) serology remained negative. The preterm and immature newborn girl had mild jaundice, minimal edema, and gluteal hematomas with petechiae. The liver and spleen were extremely enlarged (reaching the plevic bones). Increased muscle tone and rigid elbow, knee, and hip joints were found (Figures 1, 2). Additionally, X-ray examination detected multiple jejunal atresia. Brainstem evoked response audiometry ruled out hearing loss. In laboratory investigations anisocytosis, thrombocytopenia, elevated liver enzymes (ASAT: 3850 U/L, ALAT: 558 U/L, GGT: 292 U/L, ALP: 436 U/L), elevated lactate dehydrogenase (LDH) (38180 mmol/L), and creatinine kinase (CK) (7.1 U/L) with elevated bilirubin levels (87.9 μmol/L) were found. In microbiology investigations a high CMV virus number was detected using a quantitative real-time polymerase chain reaction (PCR) method from the urine and blood. Syphilis serology was positive (RPR: 1:16 positive, TPPA, TpELISA, and T. pallidum IgM immunoblot positive). HSV PCR (in the oral mucosa, conjunctiva, and blood) remained negative. Intravenous penicillin-G (100.000 IU/kg/dose for 10 days) therapy was administered. Intravenous ganciclovir was started, but was discontinued after 2 weeks because of progressive thrombocytopenia and elevating liver enzymes. The newborn underwent transfusion due to anemia and extreme thrombocytopenia. Blue light therapy was administered for 3 days because of jaundice. The multiple jejunal atresia was treated by operation (terminoterminal jejuno-jejunostomia and ileal stricturaplastica) in the Semmelweis University Pediatrics Clinic. At the time of writing this report, the girl was 2 months old, growing and developing; her intestinal passage is satisfactory, but the liver enzymes are extremely high due to the CMV infection. Congenital syphilis and congenital CMV are preventable diseases, but they are still the most common causes of perinatal mortality and morbidity worldwide (4,5). Intravenous drug users and mothers of low socioeconomic status belong to the highest risk groups for vertical transmission of infections. Congenital syphilis may induce jaundice, hepatosplenomegaly, wrinkled skin, thrombocytopenia, and anemia, with symptoms that are clinically similar to congenital CMV infection, making the differential diagnosis difficult (1,5,6). Although syphilis screening tests are mandatory in the first trimester of pregnancy in Hungary, at least one congenital syphilis case was observed yearly since the mid-nineties. Therefore, a second syphilis test is strongly recommended after the 28th gestational week or before delivery, particularly in high risk groups (7). The prenatal diagnosis of fetal CMV infection is based on amniocentesis in the 21st gestational week, which is a risky and non-standard method. The widely used ultrasonography examination often yields a uncertain diagnosis (8). Intravenous penicilline-G is effective treatment for congenital syphilis, but there is no gold standard therapy for CMV infection. Treatment with ganciclovir may prevent hearing loss later in life, but it has several severe side effects (neutropenia, anemia, thrombocytopenia, elevated liver enzymes) (9). Furthermore, studies on the effect of prolonged valganciclovir therapy are still ongoing (10). Prevention is the most effective method of reducing the prevalence of congenital CMV: pregnant women should avoid contact with the saliva of young children. In our case, the mother of the newborn belonged to a high risk group and did not participate in the prenatal caring system; mandatory screening tests were not done, so congenital infections were diagnosed only at delivery. The treatment for congenital syphilis was effective, and resulted in decrease of RPR titers. Most of the clinical symptoms did not improve, and the liver enzymes were continuously increased, indicating that CMV infection was a major contributor in clinical manifestation. Further follow up is needed to evaluate the radiological findings of long bones. Our case draws attention to the importance of early and effective prenatal diagnosis, adequate treatment of prenatal infectious diseases, and the necessity of a multidisciplinary approach to congenital infections.
- Evaluation of risk factors for development of severe hyperbilirubinemia in term and near term infants in Turkey. [Journal Article]
- Pak J Med Sci 2014 Sep; 30(5):1113-8.
To determine clinical features, etiology and risk factors in term and near term newborns with severe hyperbilirubinemia.During ten years period (2000 - 2009), infants of ≥ 35 gestational weeks who received phototherapy were evaluated retrospectively. The study population was divided into two groups and clinical features, etiology and risk factors were compared. Group 1 defined by those who had bilirubin level ≥25 mg/dl (severe hyperbilirubinemia) and group 2 defined by bilirubin level <25 mg/dl.During the study period 1335 babies were evaluated. Severe hyperbilirubinemia was found in 137 (10.3%) patients. Total serum bilirubin level was 29.7±4.7 mg/dl in group 1 and 18.9±3.5 mg/dl in group 2. Pathological weight loss, vaginal delivery and supplementary feeding were identified as significant risk factors for development of severe hyperbilirubinemia (p <0.001, p <0.001 and p = 0.04, respectively). The time at recognition of jaundice by family and postnatal age at admission were significantly higher in group 1. The ratios of previous sibling received phototherapy and being the second child or after were found higher in group 1.Pathological weight loss, vaginal delivery and supplementary feeding were determined as risk factors for development of severe hyperbilirubinemia. The newborns with severe hyperbilirubinemia had late recognition of jaundice and admission to hospital by their families.
- Postoperative steroids after Kasai portoenterostomy for biliary atresia: A systematic review. [JOURNAL ARTICLE]
- Int J Surg 2014 Sep 12.
Aim: The aim of this systematic review and meta-analysis was to determine if adjunct steroids affect jaundice-free, cholangitis, and survival rates after Kasai portoenterostomy. Methods: The literature was searched using the following terms: biliary atresia, portoenterostomy, steroids, glucocorticoids, dexamethasone, prednisolone, and hydrocortisone. The primary outcome was the jaundice-free rate. Secondary outcomes were cholangitis and survival rates. Results: Ten studies were included in the systematic review and 8 in the meta-analyses. Steroid treatment regimens were inconsistent between studies. The pooled odds ratio (OR) for the jaundice-free rate did not significantly favor steroid over non-steroid treatment (1.95; 95% confidence interval [CI]: 0.91-4.11; P = 0.087), nor did the pooled OR for the cholangitis rate (0.75; 95% CI: 0.48-1.17; P = 0.202). Overall survival ranged from 58 to 95% in the steroid group and from 36 to 96% in the control group. Native liver survival ranged from 30 to 56% in the steroid group and from 31 to 48% in the control group. The survival data were not suitable for meta-analysis. Conclusions: Although these results imply that adjunct steroids after Kasai portoenterostomy for BA may not improve jaundice-free or cholangitis rates, the quality of available evidence is limited and therefore not definitive. Additional high quality studies are needed.
- Clinical features and outcomes of patients with drug-induced autoimmune hepatitis: A retrospective cohort study. [JOURNAL ARTICLE]
- Dig Liver Dis 2014 Sep 12.
Drugs and herbal products can induce autoimmune hepatitis. We assessed frequency and clinical outcomes of patients suffering from drug-induced autoimmune hepatitis.All patients with drug-induced liver injury admitted between 2000 and 2011 were retrospectively studied. Diagnoses of drug-induced autoimmune hepatitis and idiopathic autoimmune hepatitis were made according to simplified criteria. After discharge, all patients had regular follow-up and were contacted to update outcomes.Among 10,270 in-hospital patients, 136 (1.3%) were diagnosed with drug-induced liver injury. Among them, 12 (8.8%) were diagnosed as drug-induced autoimmune hepatitis (41.7% males, age range 17-73); 8 (66.7%) were with jaundice at admission. Liver biopsies showed a pattern compatible with drug-induced autoimmune hepatitis, featured by severe portal inflammation and lymphoplasmacytic infiltrate. Drug-induced autoimmune hepatitis group had a shorter duration of drug intake, and higher values of transaminases and gamma globulins. All patients received immunosuppressive therapy with subsequent clinical remission, and five achieved a steroid-free long-term remission.A diagnosis of drug-induced autoimmune hepatitis was quite rare in our cohort, and clinical pattern was similar to idiopathic autoimmune hepatitis. Severe portal inflammation, prominent portal-plasma cells, rosette formation and severe focal necrosis were significantly more frequent in drug-induced autoimmune hepatitis as compared to drug-induced liver injury.
- [A man in his thirties with icterus and itching]. [English Abstract, Journal Article]
- Tidsskr Nor Laegeforen 2014 Sep; 134(17):1665-8.
BACKGROUNDWe present a patient from South-East Asia who has been living in Norway for five years. He was referred to our department with jaundice caused by opisthorchiasis/clonorchiasis. This reason for jaundice is highly unusual in Europe. Worldwide, however about 35 000 000 people are infected.CASE PRESENTATION A male in his thirties, originally from South-East Asia, experienced diffuse itching for five weeks and painless jaundice for two weeks. Blood samples showed increasing cholestasis. Abdominal ultrasound, MRCP and computed tomography showed no sign of bile duct obstruction or liver tumour. Serological tests and liver biopsy revealed no infectious or autoimmune liver disease. ERCP showed normal bile ducts, but large quantities of typical hookworms in the duodenum. Stool samples showed at least one egg typical of Opisthorchis/Clonorchis and a large quantity of hookworm eggs. We interpreted these findings as parasite-induced obstruction of some of the small bile ducts. The patient recovered completely after treatment with Praziquantel.INTERPRETATION Jaundice due to bile duct obstruction by opisthorchiasis/clonorchiasis is a well-known problem in South-East Asia. It may become more common in Europe as well as a result of increasing migration. Treatment with Praziquantel is simple and effective.
- Autoantibody status and histological variables influence biochemical response to treatment and long-term outcomes in Japanese patients with primary biliary cirrhosis. [JOURNAL ARTICLE]
- Hepatol Res 2014 Sep 14.
The aim of the present study is to evaluate the factors influencing biochemical response to treatment and the value of biochemical response for predicting long-term outcomes in Japanese patients with PBC.Biochemical response to ursodeoxycholic acid (UDCA) or UDCA plus bezafibrate was defined as good (≤ULN), fair (≤1.5 x ULN), or poor (>1.5 x ULN) at 2 years after initiation of UDCA treatment. Associations between various factors (including age, sex, autoantibody status, and histological variables at baseline), biochemical response to treatment, and long-term outcomes were evaluated in 164 Japanese PBC patients.Anti-gp210 positivity and a higher bile duct loss score were significant risk factors for worse ALP response (OR, 2.78 and 1.85, respectively). Age, anti-gp210 positivity, and anti-centromere positivity were significant risk factors for worse ALT response (OR, 1.05, 4.0, and 2.77, respectively). Anti-gp210 positivity and a higher hepatitis score were significant risk factors for worse IgM response (OR, 2.10 and 2.06, respectively). Worse ALP and IgM response were significant risk factors for progression to late-stage disease without jaundice (OR, 2.27 and 2.32, respectively). Worse ALT response was a significant risk factor for progression to late-stage disease with persistent jaundice (OR, 11.11).Biochemical response to treatment at 2 years, which is influenced by autoantibody status and histological variables at baseline, can predict long-term outcomes in Japanese patients with PBC.
- Endoscopic Retrograde Cholangiopancreatography at Dhulikhel hospital: Outcome Analysis. [Journal Article]
- Kathmandu Univ Med J (KUMJ) 2014 Jan-Mar; 12(45):55-9.
Background Endoscopic Retrograde Cholangiopancreatography (ERCP) is an important but potentially invasive therapeutic procedure in treating various pancreatobiliary conditions. In Nepal, the ERCP services is limited to a few tertiary care centers mostly in the capital, Kathmandu. Dhulikhel hospital has started ERCP since 2011 providing services to patients from all over the country. The study analyzes the outcome from data since August 2011 to 2013 August. Objective To analyze the results of ERCP done in Dhulikhel hospital, Kathmandu University hospital. Method This is a retrospective study. The ERCP records of all the patients done since August 2011 to August 2013 were retrieved. A total of 516 attempts of ERCP were done since August 2011 to August 2013. Out of these, 423 were included for the analysis. The repeat ERCP for the same patient due to failed cannulation or patients undergone ERCP but incomplete documentation were excluded. The demographic data, indications, the findings, the outcome in terms of success or failure to cannulation, success or failure of stone extraction, stenting and complications were all derived and analyzed. SPSS 16 version was employed for data management and analysis. Result The female: male ratio was found to be 1.7 : 1. The mean age of patients were 50.57±17.8 Years. The most common indication for ERCP were: choledocholithiasis 208(49.17%); followed by: obstructive jaundice 69(16.3% ) of undetermined cause, acute biliary pancreatitis 26(6.4%), suspected bile duct injury during cholecystectomy 18(4.2%), periampullary growth 21( 4.96%), Cholangiocarcinoma 12(2.8%), chronic pancreatitis 10( 2.3%), CBD stent exchange 23(5.43%), dilated cbd 21(4.96%) , acute cholangitis 14(3.3%).The most common finding was CBD stone in 308(72.81%), normal in 51(12.1% ), bile duct stricture in 45(10.63%). Bile duct injury during cholecystectomy was found in 13(3.1%), biliary obstruction due to ampullary growth and pancreatic head mass was found in 21(4.96%) and 6(1.41%) respectively. Choledochal cyst was found in 5(1.2%), chronic pancreatitis in 7(1.7%). Biliary fascioliasis was found in one (0.24%); and roundworm in 1(0.24).The most common complication was acute pancreatitis in 17(4%), post-ERCP cholangitis 6(1.4%), bleeding 6(1.4%), duodenal perforation in 1(0.2%), arrhythmia in 1(0.2%) and one death (0.2%). Conclusion ERCP has been a potentially emerging therapeutic tool for various pancreato-biliary disorders in Nepal.
- A Rare Finding During a Common Procedure: Xanthogranulomatous Cholecystitis. [JOURNAL ARTICLE]
- Int Surg 2014 September-October; 99(5):595-599.
Abstract Xanthogranulomatous cholecystitis is a rare variant of chronic cholecystitis characterized by severe proliferative fibrosis and accumulation of lipid-laden macrophages in regions of destructive inflammation. Xanthogranulomatous cholecystitis clinically and radiologically mimics early-stage gallbladder cancer, with wall thickening on computed tomography. The study included 14 xanthogranulomatous cholecystitis patients that were identified following retrospective analysis of the records of 1248 patients that underwent cholecystectomy between 2005 and 2011. Mean age of the 5 male and 9 female patients was 56.7 years. All 14 patients had gallbladder stones; 10 had a history of acute cholecystitis, 1 had cholangitis, and 2 presented with obstructive jaundice. A right-upper quadrant mass was palpable in 2 patients. All patients underwent cholecystectomy. Open surgery was planned and performed in 6 of the 14 patients, and laparoscopic cholecystectomy was planned in 8 patients, but was converted to open surgery in 1 case. In total, 1 patient developed wound infection, 1 patient had postoperative pneumonia, and 1 patient developed intraabdominal hematoma. None of the patients in the series died. Xanthogranulomatous cholecystitis is difficult to diagnose, both preoperatively and intraoperatively, and definitive diagnosis depends exclusively on pathological examination. Xanthogranulomatous cholecystitis should be a consideration in all difficult cholecystectomy cases.
- Single-stage definitive surgical treatment for portal biliopathy. [JOURNAL ARTICLE]
- S Afr J Surg 2014; 52(2):57-60.
The term portal biliopathy (PB) is used to describe the biliary abnormalities associated with portal hypertension. Between 5% and 30% of patients with PB develop biliary obstruction. We report on a patient with extrahepatic biliary obstruction caused by PB that was successfully managed with an intrahepatic segment 3 bypass. The traditional surgical approach for a patient with extrahepatic biliary obstruction caused by PB would be a portosystemic shunt followed by a hepaticojejenostomy if the jaundice persited. An intrahepatic segment 3 bypass provides definitive treatment ensuring biliary decompression and stone removal in a single procedure in appropriately selected patients.
- Quantitative assessment of the multiple processes responsible for bilirubin homeostasis in health and disease. [Journal Article, Review]
- Clin Exp Gastroenterol 2014.:307-28.
Serum bilirubin measurements are commonly obtained for the evaluation of ill patients and to screen for liver disease in routine physical exams. An enormous research effort has identified the multiple mechanisms involved in the production and metabolism of conjugated (CB) and unconjugated bilirubin (UB). While the qualitative effects of these mechanisms are well understood, their expected quantitative influence on serum bilirubin homeostasis has received less attention. In this review, each of the steps involved in bilirubin production, metabolism, hepatic cell uptake, and excretion is quantitatively examined. We then attempt to predict the expected effect of normal and defective function on serum UB and CB levels in health and disease states including hemolysis, extra- and intrahepatic cholestasis, hepatocellular diseases (eg, cirrhosis, hepatitis), and various congenital defects in bilirubin conjugation and secretion (eg, Gilbert's, Dubin-Johnson, Crigler-Najjar, Rotor syndromes). Novel aspects of this review include: 1) quantitative estimates of the free and total UB and CB in the plasma, hepatocyte, and bile; 2) detailed discussion of the important implications of the recently recognized role of the hepatic OATP transporters in the maintenance of CB homeostasis; 3) discussion of the differences between the standard diazo assay versus chromatographic measurement of CB and UB; 4) pharmacokinetic implications of the extremely high-affinity albumin binding of UB; 5) role of the enterohepatic circulation in physiologic jaundice of newborn and fasting hyperbilirubinemia; and 6) insights concerning the clinical interpretation of bilirubin measurements.