Episodes Of Jaundice Research Articles

An Intriguing Case of Cholestatic Jaundice

Purpose: 18-year-old lady presented with approximately 2 months history of malaise, fatigue, lethargy, right upper quadrant discomfort, jaundice and pruritus. She was not on any medications at home. Past medical history was significant for 1 episode of jaundice at age 15 which lasted several months. Family and social history were non-contributory. Physical examination revealed a young female who was afebrile, hemodynamically stable and visibly jaundiced. On abdominal exam, tenderness was present in the right upper quadrant with no rebound or guarding and normal bowel sounds. No organomegaly was appreciated. Laboratory data revealed conjugated hyperbilirubinemia (total bilirubin 11.7, direct bilirubin 7.6). Her liver enzymes AST (51) and ALP (195) were minimally elevated. Of note, her GGT was normal. Other tests including complete blood count, blood chemistries, prothrombin time were normal. Autoimmune markers, ANCA, LKM antibodies were negative. Ceruloplasmin levels were normal. Ultrasound of the abdomen showed no evidence of ductal dilation. The blood vessels were patent. MRCP revealed normal ducts with no dilation and no masses or hepatic lesions were visualized. Patient subsequently underwent liver biopsy which revealed evidence of intracellular and canalicular cholestasis with minimal fibrosis, no evidence of hepatitis or triaditis. These findings were consistent with metabolic type of intrahepatic cholestasis. The constellation of her clinical presentation, lab and diagnostic workup led to a diagnosis of benign recurrent intrahepatic cholestasis, which is a rare entity. She was treated symptomatically with Ursodeoxycholic acid and cholestyramine. Benign Recurrent Intrahepatic Cholestasis (BRIC) is characterized by intermittent episodes of cholestasis. It resolves spontaneously after periods of weeks to months. It has a recessive inheritance pattern and is caused by mutations affecting different canalicular transporter proteins. There are two types: Type 1involves mutation of FIC protein while Type 2 involves BSEP protein. It is episodic and non progressive. An important diagnostic clue is elevated serum ALP with normal GGT. Treatment is usually symptomatic.

Congenital veno‐venous malformations of the liver: Widely variable clinical presentations

Congenital portosystemic veno-venous malformations are rare abnomalities that often remain undiagnosed. Typically they are classified by their anatomical characteristics according to Morgan (extrahepatic, Abernethy malformations type Ia,b and II) and Park (intrahepatic, types 1-4). However, their clinical presentation is less dependent on the anatomical type. We reviewed the clinical characteristics of six cases drawn from our files (from 1970 to 2006). One patient, a 25-year-old male, had extrahepatic shunting whereby the liver receives only arterial blood because the portal vein (PV) connects with the inferior caval vein (ICV) (Abernethy Ib); he presented with episodes of jaundice and pruritus. Three patients had extrahepatic shunting with patent intrahepatic portal veins, but with shunting of splenomesenterial blood towards the ICV (Abernethy II); these included a 66-year-old male with hepatic encephalopathy, a 17-year-old female with (porto?-)pulmonary hypertension without portal hypertension, and a 33-year-old female with epidsodes of acute pain secondary to spontaneous bleeding within a primary liver tumor. Two patients had intrahepatic shunting; these included an 8-year-old boy who was diagnosed incidentally during work-up for abnormal liver enzymes with a communication between right PV and ICV (Park type 1), and a 59-year-old male with multiple PV-ICV-shunts in several liver segments (Park, type 4) who presented with hepatic encephalopathy. Patients often present with signs of hepatic shunting (encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, and/or hypoglycemia) with relative sparing of the synthetic liver function in the absence of portal hypertension. Some shunts present with space-occupying lesions (focal nodular hyperplasia, hepatocellular carcinoma, nodular regenerative hyperplasia, etc.) or biliary atresia. Finally, some cases are detected incidentally.

HEPATITIS - C

Objectives: 1) To evaluate the overall prevalence rate of viral hepatitiscarrier state among a group of voluntary blood donors. 2) To find the proportion of anti HCV in the study group. 3) Tofind the probable mode of transmission of HCV infection in the study group. Setting: Pathology lab, Jinnah Hospital,Iqra Medical Complex & Hi Tech Lab, Lahore. Period: November 2000 to April 2001. Patients & Methods: A total of890 apparently healthy blood donors (797 males, 93 females) were studied. Clinical details were recorded. The bloodsamples were collected using packed sterile disposable syringes. Sera were analysed by a qualitative enzymeimmunoassy sera diagnostic test using second generation ELISA Kits. Results: Among 890 subjects screened, carrierstate for hepatitis-C was 6.06%. The prevalence of hepatitis-C amongst total cases positive for hepatitis was 33.5%.A higher prevalence rate in males (6~8%) was observed as compared to females (2.22%). The difference wasstatistically significant (p<0.05). The most important mode of transmission was through injections; (males 32.075%,females 100%) followed by blood transfusion. Only 5.88% of the cases had an episode of jaundice in the past.Conclusion: A remarkable positive cases of hepatitis C in this study underline the dire need of comprehensive planfor multi-disciplinary approach for HCV prevention and control.

Recurrent Toxic Hepatitis in a Caucasian Girl Related to the Use of Shou‐Wu‐Pian, a Chinese Herbal Preparation

Recurrent Toxic Hepatitis in a Caucasian Girl Related to the Use of Shou‐Wu‐Pian, a Chinese Herbal Preparation

The Sickly Stuarts—the Medical Downfall of a Dynasty

The Sickly Stuarts—the Medical Downfall of a Dynasty

Mild hemolysis in a girl with G6PD Sumaré (class I variant) associated with G6PD A−

In the present study we describe the clinical and laboratory features of a female child, a compound heterozygote for glucose-6-phosphate dehydrogenase (G6PD) Sumaré (1292T→G) and African variants (202G→A). G6PD Sumaré is a variant causing chronic nonspherocytic hemolytic anemia. The child had neonatal jaundice 2 days after birth and needed phototherapy for 8 days. Since then, she has not had episodes of dark urine or new episodes of jaundice. She has not had hemolytic crises in spite of five respiratory infections and antibiotics administration. Laboratory data showed a reticulocytosis (5.6%) without anemia and serum unconjugated bilirubin at the upper limit of the normalcy. No hemoglobin and hemosiderin in the urine were detected. G6PD activity at 37°C was 1.15 UI/g Hb and G6PD cellulose acetate electrophoresis at pH 9.0 revealed two bands, in equal amounts, with normal and faster migration, respectively. She was homozygous for the normal (TA)6(TA)6 repeat in the UGT1A1 promoter. We conclude that the association of G6PD Sumaré and G6PD A− gave rise to a very mild chronic hemolysis, and the red cell population containing G6PD A− is probably enough to protect against severe chronic hemolysis.

A twenty-year history of alveolar echinococcosis: analysis of a series of 117 patients from eastern France.

Alveolar echinococcosis of the liver is a very rare and severe parasitic disease due to the growth of the larva of Echinococcus multilocularis. The aim of this paper was to describe a 20-year study of the epidemiological, clinical and therapeutic aspects of alveolar echinococcosis in eastern France. One hundred and seventeen consecutive cases, diagnosed and followed in our liver unit, were studied from 1972 to 1993. Data from 85 patients followed since 1983 (period B) were compared to data from a first series of 32 patients (period A) collected from 1972 to 1982; 1983 was chosen as the cut-off year because of the numerous changes that occurred in the diagnosis, follow-up and treatment of the disease at this time, in particular the introduction of parasitostatic benzimidazoles. The results of patient follow-up were evaluated in December 1997. The cumulative prevalence was 2.5 per 100,000 persons in period A whereas it reached 6.6 per 100,000 in period B. The annual incidence in period B was 7.3 on average, compared with 2.7 in period A. Twenty-nine per cent of patients from period B were asymptomatic at the time of diagnosis compared with 10% in period A. This change was correlated with less advanced liver lesions, and was related to the extensive use of abdominal ultrasound, and from 1987, serological screening. Curative resections were performed in 24% of the cases in period B versus only 3% in period A. From 1986, liver transplantations were performed in eight patients from period A and 13 patients from period B. In period B, palliative surgery was frequently replaced by radiological non-operative procedures to treat abscesses and jaundice. From 1982, 73 patients received benzimidazoles for a period of time ranging from 4 to 138 months. Stabilization of the lesions was observed in two-thirds of the patients. Episodes of jaundice or digestive haemorrhage due to portal hypertension were 31.5 and 11 times less frequent respectively in patients from period B compared with period A. Actuarial survival at 5 years improved from 67% in period A to 88% in period B in patients of similar age. Radical changes in the diagnosis and the management of alveolar echinococcosis have occurred during the last decade. Together they have contributed to an improvement in the status of the patients affected by this very severe parasitic disease.

Extended transduodenal sphincteroplasty for bile duct stones associated with a periampullary diverticulum.

Extended transduodenal sphincteroplasty has been suggested as an alternative to choledochoduodenostomy for the surgical management of bile duct stones associated with a periampullary diverticulum but its value has not previously been investigated. Over a 3 year period, nine patients underwent extended transduodenal sphincteroplasty for common bile duct calculi associated with a periampullary diverticulum with no operative or post-operative mortality and minimal morbidity. Follow-up ranging from 20 to 60 months has shown remission of pain in all but one patient, who has had a normal endoscopic cholangiogram and no further episodes of jaundice or cholangitis. Extended transduodenal sphincteroplasty is a safe and effective alternative to choledochoduodenostomy for the surgical management of choledocholithiasis associated with a periampullary diverticulum.

Type II Essential Mixed Cryoglobulinaemia: Presentation, Treatment and Outcome in 13 Patients

The long-term clinical course of patients with primary Type II essential mixed cryoglobulinaemia is unclear as many reports fail to separate this group from patients with Type III disease. We have reviewed 13 patients with Type II essential mixed cryoglobulinaemia who presented to the Hammersmith Hospital between 1976 and 1990. All patients had a cryoglobulin level greater than 0.1 mg/ml (range 0.27-6.50 mg/ml), and characterization of the cryoglobulin in all cases revealed the presence of a monoclonal IgM kappa component with rheumatoid factor activity together with polyclonal IgG. All patients had evidence of activation of the classical pathway of complement with greatly reduced levels of C4, while C3 levels were moderately reduced in three patients. All patients had skin disease and joint symptoms were reported by nine patients, with erosive arthritis in one. Eight patients had peripheral sensorimotor neuropathy. Renal disease was observed in 10 patients, manifesting as raised creatinine level, proteinuria or haematuria. Renal tissue was examined in eight patients: in six the appearances were those of a mesangiocapillary glomerulonephritis Type I while in the other two patients there was a mesangioproliferative glomerulonephritis, in one diffuse and in the other focal and segmental. Glomerular capillary 'hyaline thrombi' were found in six biopsies, extracapillary proliferation was found in three and evidence of vasculitis was found in all eight. Liver biopsy showed macronodular cirrhosis in one patient, while a second with recurrent episodes of jaundice showed only chronic inflammatory changes. No patient was positive for hepatitis B surface antigen; however one patient had low titre anti-hepatitis B surface antibody. Normochromic normocytic anaemia was present in nine patients. Bone marrow examination was carried out in 13 patients at presentation to our unit: 10 showed no evidence of a lymphoproliferative disorder, while three suggested the presence of a non-Hodgkin's lymphoma (some years after original presentation in all three). Unusual clinical features included one patient with retinal vasculitis and one patient with severe pulmonary haemorrhage.

Liver Injury due to Verapamil

A 56-year-old female patient on verapamil for hypertension experienced two episodes of jaundice, pruritus and upper abdominal pain with transaminase elevated up to six-fold and alkaline phosphatase up to four-fold when inadvertently re-challenged with the drug. Liver biopsy showed marked cholestasis. Verapamil can occasionally cause mixed cytotoxic-cholestatic liver injury.

Survival after Jaundice: A Prospective Study of 1000 Consecutive Cases

A consecutive series of 1002 jaundiced adult patients covering 23 different causes of jaundice is presented. Patients were followed up for 2 to 7 years. The survival for the 784 patients included during their first episode of jaundice was calculated for each diagnostic category. Examples of decreased survival as compared with the general population were (figures indicate 3 months' and 5 years' survival, respectively): alcoholic cirrhosis 0.81, 0.35; cryptogenic cirrhosis 0.78, 0.32; pancreatic carcinoma 0.54, 0.04; cholangiocarcinoma 0.26, 0.00; and heart failure with liver congestion 0.47, 0.07. Ten of 172 patients with acute viral hepatitis died, 1 of fulminant hepatitis and 9 because of suicide or accidents. Of 105 patients with gallstones 37 died during the study period, but in only 9 of these could death be attributed to the gallstone disease. New diagnostic methods and types of treatment for jaundiced patients have been developed during recent years. To justify fully these diagnostic and therapeutic modalities, knowledge of the prognosis for the various causes of jaundice is essential.

Cholecystohepatic Connections in a Case of Extrahepatic Biliary Atresia

EXTRAHEPATIC biliary atresia is an infrequent entity that presents a variety of clinical features. Although the pathogenesis remains the subject of some debate, it is thought to begin in utero and may continue after birth, resulting in the segmental or total obliteration of the intrahepatic and/ or extrahepatic biliary system.1Clinical experience would suggest that surgical intervention in some manner interrupts this progressive process, particularly in the noncorrectable forms. The patient described herein is illustrative and presents anatomic variations not previously described, to the best of our knowledge. Longterm survival is attributed to cholecystohepatic channels. Report of a Case A 5-year-old girl was seen in 1956 because of slow development and at least three episodes of jaundice, attributed to infectious hepatitis, first described at 3 years of age. At operation in 1956 for a suspected congenital deformity, the gallbladder was noted to be large, thick walled, distended, and moderately

Peritoneoscopy of Peliosis Hepatis

Five cases of peliosis hepatis diagnosed by peritoneoscopy (two confirmed by liver biopsy) are presented. The patients were 4 males and one female, and their ages ranged from 31 to 43 years. Each patient had had an episode of jaundice with fever in the past. At peritoneoscopy, the lesions appeared as reddish-purple spots measuring up to 2.0 mm in diameter. These spots did not show protrusion or retraction, and had no clear relationship with the lobular markings formed by portal triads. In one case, spots were connected to form irregular meshworks in places. In other cases, spots were found or oblong. Histologically, a spot obtained with a punch biopsy forceps had dilated sinusoids filled with red blood cells. Similar dilated sinusoids were observed in deep tissues of the liver obtained with a Vim-Silverman needle. Reconstruction studies of a liver biopsy specimen proved that peliosis lesions were collections of dilated sinusoids. The lesions formed ellipsoids with an irregular margin and were located in the central and intermediate zones of a liver lobule. Other histological features of the liver, mainly observed in the central area were: central fibrosis, fatty metamorphosis, slight liver cell necrosis, and cholestasis. Peliosis hepatis in cases with acute hepatitis and chronic alcoholic liver injury as in the present series is a new finding. The lesion was also seen in a case of primary liver cancer.

Liver disease complicating the management of acute leukemia during remission.

Eight adults who developed persisting liver function abnormalities during remission from acute leukemia (6 acute nonlymphoblastic leukemia and 2 acute lymphoblastic leukemia) are reported. These patients constituted 20% of the adults with acute leukemia attaining remission over the time period studied. Although we were unable to be absolutely certain of the etiology of the liver dysfunction, the following findings characterize the problem: 1) onset following red blood cell and platelet transfusions in all cases; 2) minimal symptoms and signs except for transient episodes of jaundice; 3) fluctuating, but often marked, increases in liver transaminases; 4) no other consistent laboratory abnormalities; 5) liver biopsies showing varying degrees of inflammation; 6) a consistent decrease in transaminase levels following pulse chemotherapy; 7) absence of progression of abnormalities during the observation period. This group of patients did not have a worse prognosis than patients with normal liver function. We feel that it is important to recognize this entity and to not withhold antileukemic therapy.

Granular cell myoblastoma of the biliary tree

A granular cell myoblastoma of the common bile duct is described which caused episodes of jaundice in a young woman. Histological examination demonstrated a close association of the tumour with nerve fibres, tending to support the theory of origin from Schwann cells. The lesion is benign and after excision carries an excellent prognosis.

Non-B hepatitis in Melbourne: a serological study of hepatitis A virus infection.

A study was performed to establish the value of immune adherence haemagglutination tests for antibody to hepatitis A virus in the diagnosis of non-B hepatitis. Infection with hepatitis A virus was confirmed in 14 out of 16 patients from six families and seven out of nine patients in whom the source of infection was unknown. One additional patient, who had had two episodes of jaundice, was shown to have had an attack of hepatitis A followed by an attack of hepatitis B. In patients with acute hepatitis A antibody detectable by immune adherence haemagglutination becomes detectable three or four weeks after the onset of symptoms and reaches peak levels about five weeks later.

Fusiform dilatation of the common bile duct with pancreatitis

We have seen two girls, with fusiform dilatation of the common bile duct. One child also had saccular dilatation of her intrahepatic bile ducts. Each child had suffered from recurrent bouts of abdominal pain and an episode of jaundice, and had an elevated serum amylase prior to operation. Unexplained pancreatitis in children should lead to investigation of the biliary tract. It may be wise to perform an operative choleangiogram on all patients with choledochal cysts to delineate the intrahepatic bile ducts, which may also be involved with saccular dilatation.

Total haemolytic complement after liver transplantation and in liver disease.

To investigate the relationship between serum complement and the development of jaundice after liver transplantation, serial measurements of total serum haemolytic complement have been made in four patients during the 1st month after operation. In two cases, abnormally low levels were found during episodes of jaundice, shown to be attributable to rejection on histological grounds and on the results of leucocyte migration studies carried out concomitantly. In contrast, high levels of total serum haemolytic complement were found in the other two cases when liver function had deteriorated secondary to cholangitis and extrahepatic obstruction, respectively. The relevance of these findings to the differential diagnosis of jaundice after liver transplantation is discussed and related to serum complement levels in 95 other patients with various acute and chronic liver disorders.

HALOTHANE HEPATITIS???A PREVENTABLE DISEASE?

Eleven patients with acute hepatitis following multiple anaesthetics with halothane have been seen at two hospitals during the last four years. Six had massive hepatic necrosis and died. Unexplained fever occurred in nine of the patients; two of these and one other had had previous episodes of jaundice after halothane anaesthesia. Thus, in ten patients the disease could have been avoided.

IDIOPATHIC, RECURRENT CHOLESTASIS

Idiopathic, recurrent cholestasis is characterized by episodes of obstructive jaundice often preceded by pruritus, steatorrhea, and purpuric rash. Between episodes of jaundice the liver histology is normal. We report a case of idiopathic, recurrent cholestasis occurring in a 14-year-old Caucasian girl who has experienced seven episodes of jaundice since 8 years of age. Serum alkaline phosphatase elevation preceded clinical jaundice. During remission the bromsulphalein excretion was normal. Response to oral steroids occurred when used after 4 weeks of jaundice. Cholestyramine resin and cyproheptadine relieved pruritus and in combination may have prevented progressive jaundice when administered early. Light microscopy during exacerbation showed centrilobular cholestasis with a slight mononuclear infiltration of the portal triad. The electron microscopic examinations demonstrate dilated bile canaliculi with altered microvilli. Serial liver biopsies over 6 years show no fibrosis or cytoplasmic alterations of hepatocytes. Of the 24 reported cases of idiopathic, recurrent cholestasis, two-thirds have been in males and had onset of jaundice under 20 years of age. This younger age of onset merits a greater awareness by the practicing pediatrician. After the diagnosis is established by clinical, biochemical, and morphologic studies, a benign course can be anticipated.