Background Gastrointestinal (GI) complications such as constipation, diarrhea and gastroparesis are common in hereditary transthyretin (ATTR) amyloidosis. The mechanisms behind these disturbances have not been fully elucidated and the patients’ small bowel function remains largely unexplored. The aim of the present study was to compare the small bowel motility in patients with hereditary ATTR a...

Introduction AA amyloidosis may develop as a consequence of chronic inflammatory conditions including inherited periodic fever syndromes. Mevalonate-kinase (MVK) deficiency (MKD) appears to be the least frequent underlying condition after FMF, TRAPS and CAPS. Moreover, amyloidosis rarely manifests during childhood. We report a case of a small child in whom renal biopsy performed because of the ...

The larynx is a rare site of involvement of amyloidosis. We report two cases of laryngeal amyloidosis. Both patients were middle aged females with history of persistent hoarseness. Fibreoptic laryngoscopy used for diagnosis in both and debulking of the tumour was performed. Histopathological examination confirmed the diagnosis of amyloidosis. Appropriate follow up is an important part of the lo...

Here, we report a case of insulin-derived amyloidosis in the lower abdomen. The mass continued to develop even after the patient ceased injecting insulin into the mass. Histological examination led to a diagnosis of insulin-derived amyloidosis. Excision is preferable in cases of insulin-derived amyloidosis if patient's condition permits.

Amyloidosis is characterized by the extracellular deposition of highly-organized fibrillar aggregates showing a cross-beta super-secondary structure (1). Several proteins are amyloidogenic in humans, resulting in different clinical presentations, either systemic or localized. Transthyretin-related hereditary amyloidosis (ATTR) is a late-onset, dominantly inherited systemic amyloidosis. Heterozy...

The types of disease associated with secondary amyloidosis have undergone considerable change in recent years. Largely because of modern antibiotic treatment, amyloidosis associated with tuberculosis, osteomyelitis, chronic lung disease, and syphilis is now rarely seen. For this reason rheumatoid arthritis is now one of the commonest causes of secondary amyloidosis. Although amyloidosis may be ...

A review of routine histopathological samples and autopsies examined at the Department of Pathology, University of Malaya revealed 15 cases of amyloidosis of the lung. Two were localized depositions limited to the lung while in the remainder, lung involvement was part of the picture of systemic amyloidosis. Both cases of localized amyloidosis presented with symptomatic lung/bronchial masses and...

BACKGROUND Immunoglobulin amyloid light-chain (AL)-related cardiac amyloidosis (CA) has a worse prognosis than either wild-type (ATTRwt) or mutant (ATTRm) transthyretin (TTR) CA. Detailed echocardiographic studies have been performed in AL amyloidosis but not in TTR amyloidosis and might give insight into this difference. We assessed cardiac structure and function and outcome in a large populat...

Amyloidosis is a protein conformational disease caused by the deposition of extracellular insoluble fibrils which derived from misfolded precursor proteins. The kidney organ most commonly involved in amyloidosis, common types renal amyloidosis include immunoglobulin light chain (AL), amyloid A (AA) and leukocyte chemotactic factor 2 (ALECT2). Previous studies revealed that deposits are often ac...

Background: A rippled type of pigmentation is observed on the arms, forearms, and bony prominences, more commonly in women with a history of chronic rubbing. The terminology (commonly referred to as frictional melanosis) and its relation with cutaneous amyloidosis has been debated. Materials and Methods: Twenty one patients with pigmented skin lesions with manifestations suggestive of frictiona...