Renal amyloidosis in children

Renal amyloidosis is a detrimental disease caused by the deposition of amyloid fibrils. A child with renal amyloidosis may present with proteinuria or nephrotic syndrome. Chronic renal failure may follow. Amyloid fibrils may deposit in other organs as well. The diagnosis is through the typical appearance on histopathology. Although chronic infections and chronic inflammatory diseases used to be the causes of secondary amyloidosis in children, the most frequent cause is now autoinflammatory diseases. Among this group of diseases, the most frequent one throughout the world is familial Mediterranean fever (FMF). FMF is typically characterized by attacks of clinical inflammation in the form of fever and serositis and high acute-phase reactants. Persisting inflammation in inadequately treated disease is associated with the development of secondary amyloidosis. The main treatment is colchicine. A number of other monogenic autoinflammatory diseases have also been identified. Among them cryopyrin-associated periodic syndrome (CAPS) is outstanding with its clinical features and the predilection to develop secondary amyloidosis in untreated cases. The treatment of secondary amyloidosis mainly depends on the treatment of the disease. However, a number of new treatments for amyloid per se are in the pipeline

Adrenal carcinoma causing secondary amyloidosis: report of the first case in the literature

In a 53-year-old male patient with metastatic adrenal carcinoma, treatment with mitotane was instituted but he was lost to follow-up. Two years later, he presented with oedema and nephrotic-range proteinuria. The rectal and renal biopsies revealed an accumulation of secondary amyloid material. The patient died of respiratory failure caused by the progressive pulmonary metastases. This is the first report of a patient with adrenal carcinoma who developed secondary amyloidosis

Chylous ascites and chylothorax due to constrictive pericarditis in a patient undergoing haemodialysis

Chylous ascites and chylothorax are rare clinical entities and usually caused by neoplasms, particularly lymphomas, liver cirrhosis, superior vena cava thrombosis, nephrotic syndrome, and some cardiac events such as dilated cardiomyopathy or right heart failure. Constrictive pericarditis is an extremely rare cause of this clinical state. We report a 41-year-old male patient undergoing haemodialysis who presented with chylous ascites and chylothorax. Echocardiography and heart catheterisation. revealed constrictive pericarditis. He underwent pericardiectomy and after the operation the ascites and pleural effusion resolved rapidly. We suggest that constrictive pericarditis should be considered in the differential diagnosis of chylous ascites and chylothorax

Brucella glomerulonephritis: Review of the literature and report on the first patient with brucellosis and mesangiocapillary glomerulonephritis

Although mild proteinuria is commonly observed during the course of brucellosis, biopsy-proven glomerulonephritis (GN) is quite rare. We present the first case of mesangiocapillary glomerulonephritis (MCGN) associated with brucellosis and summarize all cases of Brucella GN published to date. Our patient, who had a congenital bicuspid aortic valve, also had heart failure, fever, urinary abnormalities and proteinuria. Renal biopsy revealed MCGN. Although the clinical features raised the possibility of GN associated with endocarditis, transesophageal echocardiography did not show any vegetations

Acute renal failure due to carnitine palmitoyltransferase II deficiency

Carnitine palmitoyltransferase II (CPT-II) deficiency is the most common long-chain fatty acid oxidation defect, resulting in rhabdomyolysis and acute renal failure (ARF). There are three forms of CPT-II deficiency: the neonatal, infantile and adult form. We report an adult form of CPT-II deficiency in a patient who presented with attacks of exercise-induced rhabdomyolysis and ARF