A rare cause of nephrotic syndrome in a 14-year-old boy: Answers

Abstract

Detection of a cystic lesion in the liver suggests simple cysts, multiple cysts arising in the setting of polycystic liver disease (PCLD), parasitic or hydatid (echinococcal) cysts, cystic tumors, and abscesses. These conditions can usually be distinguished on the basis of the patient’s symptoms and the radiographic appearance of the lesion [1]. The presence of constitutional symptoms, such as pallor, anorexia, fever or weight loss, may point toward a malignant lesion or infection, but our patient had no medical history or signs of these upon physical examination. Ultrasound of his abdomen revealed a huge liver cyst of 90 mm occupying almost the whole left lobe with mild internal echoes all suggestive of hydatid cyst. The kidneys, spleen and pancreas appeared normal. Abdominal computed tomography (CT) was used to delineate the cystic lesion within the liver. Indeed, the CT confirmed a huge, thick-walled cyst of 90 mm in diameter, occupying the left lobe of the liver in segment I. Hydatid disease (HD), or echinococcosis, is a zoonotic infection caused by the larval stages of the cestodes belonging to the genus Echinococcus. Cystic echinococcosis (CE) is the most common form of HD, and occurs as the result of infection by the larval stages of E. granulosus, which has a worldwide distribution and continues to be a major health problem in developing countries [2–4]. Oncosphere larvae hatch in the duodenum and are carried to the liver through blood vessels or lymphatics. About 65 % are trapped in the liver (first filter), which is the most commonly involved organ (52–77 % of cases); the remaining 25 % are trapped in the lungs (second filter) and less than 10 % reach various organs through the systemic circulation [5]. The symptoms of liver hydatid cysts differ depending on the size and number of cysts, but also on the mass effect within the liver and upon surrounding structures. Patients are usually asymptomatic, but may present with right/epigastric pain, nausea, and vomiting [1]. Considering that the early stages of infection are usually asymptomatic, the diagnosis of hydatid cyst depends on clinical suspicion. Diagnosis is made by history of exposure, by radiological and ultrasound cyst detection, and by serology [6]. The diagnosis of hydatid disease in our patient was confirmed by a strongly positive serum ELISA and indirect hemagglutination test (IHA) for Echinococcus. Treatment This article refers to the article that can be found at http://dx.doi.org/ 10.1007/s00467-012-2282-x.