ABSTRACT
La ictericia colestásica se debe a la alteración de la secreción de bilirrubina conjugada; es una de las posibles causas la alteración del flujo biliar por obstrucción de la vía biliar extrahepática. El linfoma es la tercera neoplasia más frecuente en pediatría, mientras que los tumores pancreáticos son poco frecuentes y, en su mayoría, lesiones benignas. Las manifestaciones clínicas de los tumores de localización retroperitoneal son poco específicas y suelen ser tardías, por lo que la sospecha clínica debe ser alta. El objetivo del siguiente trabajo es presentar el caso de un niño de 7 años con síndrome colestásico en el que se halló un tumor en la cabeza del páncreas que comprimía la vía biliar extrahepática. El diagnóstico del tumor fue linfoma no Hodgkin (LNH). Se destaca la infrecuencia de este tumor en esta localización en la edad pediátrica
Cholestatic jaundice is due to an alteration in conjugated bilirubin secretion; a possible cause is an altered bile flow resulting from an obstruction of the extrahepatic bile duct. A lymphoma is the third most common neoplasm in pediatrics, while pancreatic tumors are rare and mostly benign. The clinical manifestations of retroperitoneal tumors are not very specific and are usually late, so a high level of clinical suspicion is required. The objective of this study is to describe the case of a 7-year-old boy with cholestatic syndrome with a tumor in the head of the pancreas compressing the extrahepatic bile duct. The tumor diagnosis was non-Hodgkin lymphoma (NHL). It is worth noting that the presence of a tumor in this location in pediatric age is uncommon
Subject(s)
Humans , Male , Child , Lymphoma, Non-Hodgkin/complications , Lymphoma, Non-Hodgkin/diagnosis , Lymphoma, Non-Hodgkin/pathology , Cholestasis/etiology , Jaundice, Obstructive/diagnosis , Jaundice, Obstructive/etiology , Jaundice, Obstructive/pathology , Pancreas , Syndrome , Cholestasis/diagnosisABSTRACT
Cholestatic jaundice is rare in patients with Graves' disease and is generally considered to be a complication of Graves' disease or an adverse reaction to methimazole. We report a case of acute cholestatic jaundice caused by Graves' disease complicated with upper respiratory tract infection in Jinan Central Hospital. After timely treatment with glucocorticoid, the jaundice quickly subsided and the liver function gradually returned to normal. We believe that Graves' disease combined with upper respiratory tract infection can lead to cholestatic jaundice, the pathogenesis of which may be immune dysfunction, and glucocorticoid therapy is beneficial to the regression of jaundice.
ABSTRACT
El neurofibroma de la vía biliar es una enfermedad extremadamente rara y generalmente asintomática, sin embargo, puede ocasionar ictericia obstructiva y simular un tumor de Klatskin conduciendo a un tratamiento quirúrgico radical y mayor morbilidad del paciente. Presentamos el caso de una mujer de 62 años colecistectomizada hace 10 años, con ictericia y dolor en hipocondrio derecho, así como exámenes auxiliares compatibles con colestasis. Se observó dilatación de la vía biliar y presencia de un tumor en el conducto hepático izquierdo mediante colangioresonancia magnética. El diagnóstico clínico preoperatorio fue Colangiocarcinoma hiliar, pero el estudio histopatológico reveló una neoplasia compuesta por células fusocelulares sin atipia ni actividad mitótica, cuya estirpe neurogénica se sustentó por su positividad a proteína S100 en inmunohistoquímica. Reportamos el caso dada su poca frecuencia en la literatura y su relevancia, al ser una entidad benigna, como diagnóstico diferencial de cáncer.
The neurofibroma of the bile duct is an extremely rare and generally asymptomatic disease, however, it maybe cause obstructive jaundice and mimic a Klatskin tumor, leading to radical surgical treatment and increased patient morbidity. We present the case of a 62-year-old woman who underwent cholecystectomy 10 years ago, with jaundice and pain in the right upper quadrant, as well as auxiliary tests compatible with cholestasis. Dilation of the bile duct and the presence of a tumor in the left hepatic duct were observed by magnetic cholangioresonance. The preoperative clinical diagnosis was hilar cholangiocarcinoma, but the histopathological study revealed a neoplasm composed of spindle cells without atypia or mitotic activity, whose neurogenic lineage supported by its positivity to protein S100 in immunohistochemistry. We report the case given its infrequency in the literature and its relevance, as it is a benign entity, as a differential diagnosis of cancer.
ABSTRACT
Symptomatic acute Epstein-Barr virus (EBV) hepatitis, without associated infectious mononucleosis syndrome, is exceptionally rare. A 30-year-old female presented to hospital with jaundice, fevers, and right upper quadrant abdominal pain. Her blood tests demonstrated marked hyperbilirubinemia and mild global liver function test abnormalities consistent with obstructive jaundice. Preliminary imaging with ultrasound showed gallbladder wall thickening and cholelithiasis, suggestive of potential cholecystitis. Authors were concerned for potential ascending cholangitis in the setting of her hyperbilirubinemia. The diagnosis was refuted after magnetic resonance cholangiopancreatography demonstrated no choledocholithiasis. A hepatic panel was performed which revealed positive EBV IgM serology. This case highlights the importance of considering EBV hepatitis as a potential differential diagnosis in patients with right upper quadrant pain, fevers and jaundice in the absence of an obstructing cause.
ABSTRACT
Benign Recurrent Intrahepatic Cholestasis (BRIC) is a rare autosomal recessive disorder characterized by intermittent episodes of jaundice and pruritus. It is also known as Summerskill-Walshe-Tygstrup syndrome. It is a benign disease with no progression to end stage liver disease.. The first episode of cholestatic jaundice occurs early in life and there are asymptomatic periods between attacks lasting weeks to years. This case report presents a young male who presented with severe pruritus and acute onset jaundice. He had his first episode of jaundice at the age of twelve and had several intermittent episodes since then. Diagnosis was made by the unique clinical presentation with exclusion of other causes of cholestatic jaundice. This case report highlights the importance of detecting such cases of rarity and preventing unnecessary invasive diagnostic procedures on such patients.
ABSTRACT
INTRODUCCIÓN: El quiste de colédoco (QC) es una patología infrecuente, caracterizada por una dilatación de vía biliar intra o extrahepática. Constituye una lesión congénita, representado 1% de las lesiones biliares benignas. Tiene una incidencia de 1 en 100.000 a 150.000 habitantes. Es más frecuente en mujeres, y su etiología es desconocida. En adultos los síntomas son inespecíficos; predominando dolor abdominal e ictericia. PRESENTACIÓN DEL CASO: Mujer de 61 años con cólico abdominal en hipocondrio derecho de tres días, vómitos e ictericia. Al ingreso hospitalario presentaba leucocitosis, hiperbilirrubinemia, aumento de fosfatasa alcalina, transaminasas y amilasa. Se plantearon los diagnósticos de ictericia obstructiva, pancreatitis y quiste hidatídico complicado, por lo que se realiza tomografía computada (TC) de abdomen evidenciando dilatación sacular intra y extrahepática, compatible con QC tipo IV-a. Se realizó colecistectomía y coledocostomía con sonda T de urgencia por evolución a colangitis con resultados favorables. DISCUSIÓN: Los QC son una causa rara de ictericia obstructiva. En Chile existen escasos datos estadísticos al respecto. Se manifiesta con una sintomatología inespecífica, sobretodo en adultos. El diagnóstico se realiza con hallazgos de laboratorio concordantes con ictericia colestásica, donde los estudios imagenológicos como ultrasonido y TC tienen un rol importante, pese a que en algunas ocasiones pueden pasar inadvertido. Es primordial un alto índice de sospecha para el diagnóstico y un tratamiento oportuno debido a su importante riesgo de progresión a colangiocarcinoma
INTRODUCTION: Choledochal cysts (CCs) is a rare disease characterized by dilatation of the intrahepatic or extrahepatic bile duct, which is about 1% of all benign biliary lesions. Its incidence is 1:100,000 to 150,000 habitants. It is more common in females, and its etiology is unknown. In adults the symptoms are nonspecific, predominantly abdominal pain and jaundice. CASE REPORT: 61 year old female patient with three days of severe abdominal colic in the right upper quadrant, whit both vomiting and jaundice. On admission, she presents leukocytosis, hyperbilirubinemia, and increased levels of alkaline phosphatase, transaminases and amylase. Diagnosis of obstructive jaundice, pancreatitis and complicated hydatid cyst arising. The abdominal CT Scan reveals intra and extrahepatic saccular dilatations, compatible with a type IV-a CCs. Both cholecystectomy and T-tube choledochotomy were done by evolution to cholangitis with favorable results and satisfactory postoperative. DISCUSSION: CCs is a rare cause of obstructive jaundice, and in this regard, there are few data described in Chile, Its diagnosis requires a high index of suspicion because of its nonspecific symptoms found mostly in adults. Despite this, the diagnosis is determined with laboratory findings consistent with cholestatic jaundice and support diagnostic imaging such as ultrasound, CT Scan, among others. Although the imaging findings, it may not be detected. A correct diagnosis and appropriate treatment is essential because of its high risk of progression to cholangiocarcinoma. Currently the patient is waiting for resection of extrahepatic bile duct and Roux-en-Y hepatic jejunostomy which is the optimal treatment.
Subject(s)
Humans , Female , Middle Aged , Choledochal Cyst/surgery , Choledochal Cyst/complications , Choledochal Cyst/diagnostic imaging , Cholecystectomy , Tomography, X-Ray Computed , Cholangitis , Choledocholithiasis , Jaundice, Obstructive/etiology , Hyperbilirubinemia/etiologyABSTRACT
Background: The objective was to assess the diagnostic efficacy of hepatobiliary scintigraphy (Tc99 mebrofenin) in case of cholestatic jaundice in pediatric practice. Method: Study conducted on 45 cases from 0-6 yrs. of age. Out of 45 pt 20 pt excluded from study Hepatobiliary Scintigraphy (Tc99m Mebrofinin) was performed in 25 cases. Injection of Tc99m mebrofinin (1-2 mCi) IV was given to each subject. Dynamic hepatic scan was done upto initial 1 hour study and additional delayed images were taken at 4 & 24 hrs only in case of non-visualization of tracer in the intestine after initial 1 hr. study. Results: In this study hepatobiliary scintigraphy was 100% sensitive, 93.73% specific, 80% positive predictive values, 100% negative predictive value, 6.23% false positive result and virtually no false negative result for biliary atresia. Conclusion: It is concluded that Tc99m mebrofenin hepatobiliary scintigraphy has proven to be reliable noninvasive imaging modality in evaluating cholestatic jaundice in pediatric practice as it carries a high sensitive & specific value, good positive & no negative predictive value, few false positive & virtually no false negative results.
ABSTRACT
Justification: Neonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers. Process: Eminent national faculty members were invited to participate in the process of forming a consensus statement. Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. A round table conference was organized; presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft. Objectives: To review available published data on the subject from India and the West, to discuss current diagnostic and management practices in major centers in India, and to identify various problems in effective diagnosis and ways to improve the overall outcome. Current problems faced in different areas were discussed and possible remedial measures were identified. The ultimate aim would be to achieve results comparable to the West. Recommendations: Early recognition, prompt evaluation and algorithm-based management will improve outcome in neonatal cholestasis. Inclusion of stool/urine color charts in well baby cards and sensitizing pediatricians about differentiating conjugated from the more common unconjugated hyperbilirubinemia are possible effective steps. Considering the need for specific expertise and the poor outcome in sub- optimally managed cases, referral to regional centers is warranted.
ABSTRACT
Carnitine (beta-hydroxy-gamma-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Subject(s)
Humans , Infant, Newborn , Carnitine , Esters , Fatty Acids , Jaundice, Obstructive , Live Birth , Weight GainABSTRACT
Carnitine (beta-hydroxy-gamma-trimethylaminobutyric acid) is involved in the transport of long-chain fatty acids into the mitochondrial matrix and the removal of potentially toxic acylcarnitine esters. Transient carnitine transport defect is a rare condition in newborns reported in 1/90,000 live births. In this paper, we describe a case of transient carnitine transport defect found in a premature baby who had prolonged cholestatic jaundice and poor weight gain, and who responded dramatically to oral carnitine supplementation.
Subject(s)
Humans , Infant, Newborn , Carnitine , Esters , Fatty Acids , Jaundice, Obstructive , Live Birth , Weight GainABSTRACT
The authors report a case of spontaneous perforation of the biliary tract (S.P.B.T) in a three-month-old infant. The diagnosis was suspected before the operation by clinical signs and diagnostic tests. The importance of paracentesis and scintigraphy is stressed. A surgical approach was chosen and drainage procedure of the area around the perforation and a cholecystostomy were done. S.P.B.T. is rare and its etiology is controversial, but cannot be forgotten in association between biliary ascites and cholestatic jaundice during the first months of life.
ABSTRACT
Methimazole is a widely used and generally well-tolerated antithyroid agent. Adverse reactions occur in 1~5% of patients taking methimazole medication, but these are most commonly transient, benign leukopenia and a skin rash. Severe cholestatic jaundice, combined with agranulocytosis, has been known as a rare complication. Herein, a case of methimazole induced cholestatic jaundice, with agranulocytosis, is reported.
Subject(s)
Humans , Agranulocytosis , Exanthema , Jaundice, Obstructive , Leukopenia , MethimazoleABSTRACT
ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized due to pneumonia at four months of age. Corpus callosum hypoplasia and atrial septal defect were detected. The case was treated with a mechanical ventilator because pneumonia was aggravated and respiratory failure occurred. The patient expired at five months of age.
Subject(s)
Humans , Acidosis, Renal Tubular , Arthrogryposis , Contracture , Corpus Callosum , Heart Septal Defects, Atrial , Heart , Jaundice, Obstructive , Joints , Muscular Atrophy , Pneumonia , Respiratory Insufficiency , Ventilators, MechanicalABSTRACT
PURPOSE: Magnetic resonance cholangiopancreatography(MRCP) is a noninvasive method for imaging the pancreaticobiliary tree. The aim of this study was to evalute the usefulness of MRCP for the diagnosis of pancreaticobiliary diseases in children. METHODS: From October 1996 to May 2001, 67 patients with obstructive jaundice and three patients with chronic recurrent pancreatitis were evaluated with abdominal ultrasonography and MRCP. The final diagnosis was based on the operative and pathologic findings with biopsy specimen including clinical and laboratory findings. RESULTS: A total of 70 patients, consisting of 31 males and 39 females, with a mean age of 2.6+/-3.3 years were studied. The final diagnosis was biliary atresia in 25, neonatal cholestasis in 18, choledochal cyst without anomalous pancreatobiliary duct union(APBDU) in nine, choledochal cyst with APBDU in seven, cholestatic hepatitis in five, chronic recurrent pancreatitis in three, sclerosing cholangitis in two, and secondary biliary cirrhosis in one case. The overall diagnostic accuracy of abdominal ultrasonography was 75.7% and that of MRCP was 97.1%. The sensitivity and specificity of MRCP were 100% and 98% for biliary atresia, 87.5% and 100% for choledochal cyst with APBDU, 100% and 100% for choledochal cyst without APBDU, sclerosing cholangitis and chronic recurrent pancreatitis, respectively. CONCLUSION: MRCP is a fast, non-invasive and reliable method for diagnosing pancreaticobiliary diseases in children and will be the standard diagnostic procedure in the future.
Subject(s)
Child , Female , Humans , Male , Biliary Atresia , Biopsy , Cholangitis, Sclerosing , Choledochal Cyst , Cholestasis , Diagnosis , Hepatitis , Jaundice, Obstructive , Liver Cirrhosis, Biliary , Pancreatitis , Sensitivity and Specificity , UltrasonographyABSTRACT
When jaundice persists for more than 14 days postnatally, the early diagnosis of surgical jaundice is important for the prognosis in extrahepatic biliary atresia after draining procedure. The role of diagnostic laparoscopy to differenctiate medical causes of jaundice from biliary atresia is evaluated in this report. Four patients with prolonged jaundice have been included in this study. When the gallbladder was not visualized we proceeded to laparotomy. In patients with enlarged gallbladder visualized at laparoscopy, laparoscopic guided cholangiogram was performed, and laparoscopic liver biopsy was done for those who had a patent biliary tree. Two patients had small atretic gallbladder and underwent a Kasai hepato-portoenterostomy. One patients showed a patent gallbladder and common bile duct with atresia of the common hepatic and intrahepatic ducts, and they underwent a Kasai hepatic-portoenterostomy. One patient showed an enlarged gallbladder and laparoscopic-guided cholangiogram were normal. Laparoscopic liver biopsy was performed. There were no complications. Laparoscopy wth laparoscopic-guided cholangiogram may be a valuable method in accurate and earlier diagnosis in an infant with prolonged jaundice.
Subject(s)
Humans , Infant , Biliary Atresia , Biliary Tract , Biopsy , Common Bile Duct , Diagnosis , Early Diagnosis , Gallbladder , Jaundice , Jaundice, Obstructive , Laparoscopy , Laparotomy , Liver , PrognosisABSTRACT
PURPOSE: To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. METHODS: Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was based on non-visualization of either the common bile duct or common hepatic duct. The final diagnosis of biliary atresia or non-biliary atresia was made with operations or clinical follow-up until jaundice resolved. RESULTS: MRC could clearly visualized the gallbladder, cystic duct, common hepatic duct, common bile duct, both intrahepatic ducts and second order intrahepatic ducts in small neonates and infants. MRC had accuracy of 98%, sensitivity of 100% and specificity of 96% for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. CONCLUSIONS: MRC is a very reliable noninvasive imaging study for diagnosis of biliary atresia in infants with cholestatic jaundice.
Subject(s)
Humans , Infant , Infant, Newborn , Biliary Atresia , Cholangiography , Common Bile Duct , Cystic Duct , Diagnosis , Follow-Up Studies , Gallbladder , Hepatic Duct, Common , Jaundice , Jaundice, Obstructive , Magnetic Resonance Imaging , Sensitivity and SpecificityABSTRACT
Cholestatic jaundice caused by imidazole derivatives is a rare complication of antithyroid drug therapy. We present a case of cholestatic jaundice with systemic hypersensitivity reaction, which developed in a 27-year old male one day after exposure to methimazole. The patient showed clinical improvement and gradual resolution of jaundice after the discontinuation of methimazole and treatment with prednisolone. Histologic findings of liver revealed bile pigment, predominantly in centrilobular area, and infiltration of chronic inflammatory cells in a few portal area without evidence of degeneration or necrosis of hepatocytes. Methimazole could be presumed as etiologic agent from clear chronological relationship and the lack of other causative factors. We report this unusual case with review of literature.
Subject(s)
Adult , Humans , Male , Bile Pigments , Drug Therapy , Hepatocytes , Hypersensitivity , Jaundice , Jaundice, Obstructive , Liver , Methimazole , Necrosis , PrednisoloneABSTRACT
Objective: This article had been ready for creating Matrine capsule by reducing enzyme and receding jaundice of Matrine. Matrine would be not only by intravenously guttae but also by oral use in clinical treatment, so the oral drug could reduce the pain and use more easy for patients. Methods: The experiment used cholestatic jaundice model for ? naphthy isothiocyanate of mice to make high cholerythrin symptom in healthy mice. Results: Pharmacodynamic experiment proved: TBIL and DBIL of high cholerythrin symptom of mice had been reduced, comparing with control groups by Matrine for oral use(75mg/kg and 100mg/kg), and so do SGPT and SGOT. Conclusion: Matrine for oral use had obvious effect on reducing enzyme and receding jaundice.