CAROLI DISEASE: CLINICAL CASE PECULIARITY
Journal Title: World Journal of Pharmaceutical and life sciences - Year 2017, Vol 3, Issue 3
Abstract
Objective: Caroli disease is a rare disorder characterized by the saccular segmental dilatations of the intrahepatic bile ducts communicating with the principal biliary tract, without concomitant histological anomalies in the liver parenchyma. The disease has few symptoms and sometimes, it can remain silent. The clinical expression occurs with the development of the complications related to the bile stasis. The sonography examinations consist of the dilatation of the bile ducts and of the characteristic protrusion, which join the duct walls to the intraluminal portal branches. If a diagnostic doubt lasts after the diagnostic sonography, the use of the hepatobiliary nuclear stratigraphy or of the X-ray computed tomography or of the nuclear magnetic resonance can give important discriminatory information. The last diagnosis can be confirmed by the intraoperative cholangiogram procedure. Methods: A woman was admitted to our Hospital because of left flank pain. She reported widespread abdominal pain in the left flank associated with nausea and vomiting in the ER. The diagnostic sonography showed that the volume of the liver was higher than normal with the obvious presence of multiple cystic formations of several dimension (3.5 cm max). The principal biliary duct, detected in its proximal portion, showed an increased caliber (1.4 cm). The intrahepatic biliary ducts were dilated. Both kidneys showed several cystic formations, which altered the dimensions and the ecostructure of both kidneys in toto in a polycystic kidney disease. There is an additional biliary lithiasis of about 6 mm of the left ureter's intramural tract. Therefore, the patient was hospitalized with diagnosis of left-sided colitis on a polycystic kidney with a renal failure and dilatation of the bile ducts TBD and with abnormal blood texts. The woman was submitted to the MRI of the abdominal cholangiography. Results: During the observation we have witnessed the gradual improvement of the symptomatology, so the patient was discharged with a diagnosis of renal colic, lithiasis of the urinary tract in a polycystic kidney, chronic renal failure and multicystic dilatation of the intrahepatic bile ducts (Caroli disease) with medical therapy. Conclusions: Caroli disease, that is part of this group of disorders, has the reputation of being a rare illness, above all in the pure form. This is a real rarity, but it is overestimated by the benign clinical course of the first 5-20 years of life, sometimes longer and, even if in few cases, asymptomatic, insomuch as it is often fortuitously suspected or diagnosed after a liver biopsy or after an ultrasonography survey or a tomography investigation due to another cause. The diagnostic approach of the Caroli disease is based on various anamnestic, clinical, biological and radiological data, which together allow making the correct diagnosis. Therapeutic behaviour recommends in the first instance some cycles of antibiotic therapy to hinder the rare cholangitis episodes and, if it is possible, extracorporeal lithotripsy, and then to pass to the more radical one of the hepatic section when the symptomatology becomes ingravescent and only one lobe is interested
Authors and Affiliations
Dr. Andrea Piccioni
Keywords
Related Articles
- EP ID EP648979
- DOI -
- Views 160
- Downloads 0
A CLINICAL REVIEW ON: HYPOKALEMIC PERIODIC PARALYSIS-DIAGNOSIS AND TREATMENT
Hypokalemic periodic paralysis is a rare, autosomal dominant channelopathy characterized by muscle weakness or paralysis with a matching fall in potassium levels in the blood (primarily due to defect in a voltage-gated c...
CONCEPT OF PATHOGENESIS OF DHATVAGNIVIKARA W.S.R. TO HYPOTHYROIDISM AND THERAPEUTIC EVALUATION OF TRIKATU CHURNA AND KANCHANARA KWATHA
Hypothyroidism refers to the under activity of thyroid gland resulting into deficiency of thyroid hormone which may be considered as malfunctioning of agni (pitta). In india 42 million people are suffering from thyroid d...
EFFECT OF SEED AGE, VARIETIES AND INTRA ROW SPACING ON YIELD OF SOYBEAN (GLYCINE MAX (L.) MERRILL) IN DANGUR DISTRICT, METEKLE ZONE, WEST SOUTHERN ETHIOPIA
The importance of seed age, varieties and spacing on soybean seed yield and quality has not been established in Ethiopia. This study was conducted in Dangur District at Pawe Agricultural Research Site with the objective...
A REVIEW ON STEP-BY-STEP ANALYTICAL METHOD VALIDATION
When analytical method is utilized to generate results about the characteristics of drug related samples it is essential that the results are trustworthy. They may be utilized as the basis for decisions relating to admin...
IMPORTANCE OF SESAME OIL IN HERBAL OIL FORMULATION: A REVIEW ARTICLE
Sesame plant holds its importance from the ancient culture. Sesamum indicum commonly called as sesame, Family: Pedaliaceae mainly cultivated for its seeds. Sesamum indicum Linn. (Pedaliaceae) is an annualshrub with white...