MyMedR

Fulltext Abdominal subcutaneous fat aspiration--an alternative method to diagnose amyloidosis

Ong SG, Rajasingam R

Med J Malaysia, 2007 Mar;62(1):68-9.

A middle aged lady presented with clinical manifestations of primary amyloidosis which included amyloid cardiomyopathy. There was failure to confirm the diagnosis of amyloidosis with biopsies from rectal and tongue tissues. Fat tissue obtained from abdominal subcutaneous fat aspiration eventually demonstrated the presence of amyloid.

Matched MeSH terms: Amyloidosis/diagnosis*

Primary cardiac amyloidosis

Jeyamalar R, Pathmanathan R, Yap SF, Kannan P

Ann Acad Med Singap, 1991 Nov;20(6):795-7.

PMID: 1803972

Cardiac amyloidosis is an uncommon and often unrecognised cause of cardiac failure. It is an infiltrative disease that may mimic either a restrictive or hypertrophic cardiomyopathy, constrictive pericarditis, coronary artery disease or valvular heart disease. The diagnosis should be suspected in a patient with cardiac failure who has low voltage complexes on the electrocardiogram, in association with increased myocardial mass and echogenicity on the echocardiogram. The definitive diagnosis, however, can only be made by endomyocardial biopsy or biopsy of any involved organ in systemic amyloidosis. Prognosis is poor and treatment ineffective.

Matched MeSH terms: Amyloidosis/diagnosis*

Echocardiographic and electrocardiographic presentations of patients with endomyocardial biopsy-proven cardiac amyloidosis

Roslan A, Kamsani SH, Nay TW, Tan KL, Hakim N, Tan AM, et al.

Med J Malaysia, 2018 12;73(6):388-392.

PMID: 30647209

OBJECTIVE: Cardiac amyloidosis is under diagnosed and its prevalence is unknown. This is a retrospective, nonrandomised, single centre study of patients with endomyocardial biopsy-proven cardiac amyloidosis focusing on their echocardiographic and electrocardiogram (ECG) presentations. This is the first case series in Malaysia on this subject.

METHODS: We identified all of our endomyocardial biopsyproven cardiac amyloidosis patients from January 2010 to January 2018 and reviewed their medical records. All patients echocardiographic and ECG findings reviewed and analysed comparing to basic mean population value.

RESULTS: In total there are 13 biopsy-proven cardiac amyloidosis patients. All of the biopsies shows light chain (AL) amyloid. Majority of the patients (8, 61.5%) is male, and most of our patients (8, 61.5%) is Chinese. All seven patients on whom we performed deformation imaging have apical sparing pattern on longitudinal strain echocardiogram. Mean ejection fraction is 49.3%, (SD=7.9). All patients have concentric left ventricular hypertrophy and right ventricular hypertrophy. Diastolic dysfunction was present in all of our patients with nine out of 13 patients (69.2%) having restrictive filling patterns (E/A ≥2.0 E/e' ≥15). On electrocardiogram, 12 (92%) patients have prolonged PR interval (median 200ms, IQR 76.50ms) and 9 (69.2%) patients have pseudoinfarct pattern.

CONCLUSION: Echocardiography plays an important role in diagnosing cardiac amyloidosis. The findings of concentric left ventricular hypertrophy with preserved ejection fraction without increased in loading condition should alert the clinician towards its possibility. This is further supported by right ventricular hypertrophy and particularly longitudinal strain imaging showing apical sparing pattern.

Matched MeSH terms: Amyloidosis/diagnosis

Fulltext Renal-limited AL amyloidosis - a diagnostic and management dilemma

Fuah KW, Lim CTS

BMC Nephrol, 2018 11 06;19(1):307.

PMID: 30400895 DOI: 10.1186/s12882-018-1118-8

BACKGROUND: Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils which may result in a wide spectrum of symptoms depending upon their types, sites and amount of deposition. Amyloidosis can be divided into either systemic or localized disease.

CASE PRESENTATION: We present a case of a middle-aged gentleman who presented with persistent nephrotic syndrome with worsening renal function. Repeated renal biopsies showed the presence of renal-limited AL amyloidosis. Systemic amyloidosis workup was unremarkable apart from a slightly raised band of IgG lambda level with no associated immunoparesis. The nephrotic syndrome and renal histology did not improve over a 3-year period despite being given two courses of chemotherapies.

CONCLUSION: We hope that early recognition of this unusual localised presentation of renal- limited AL Amyloidosis and its poor response to conventional treatment can alert the nephrologist to the potential existence of this rare condition.

Matched MeSH terms: Amyloidosis/diagnosis*

Fulltext Aerodigestive amyloidosis presenting as acute asthma

Sreetharan SS, Prepageran N, Razak A, Valuyeetham KA

Med J Malaysia, 2003 Jun;58(2):290-3.

PMID: 14569753

Aerodigestive amyloidosis is a rare disorder characterized by fibrillar protein deposition in the aerodigestive tree. We present a case of a 19-year-old Chinese gentleman whose diagnosis was initially missed as he presented with features suggestive of severe bronchial asthma and was intubated and ventilated. He subsequently presented 2 years later with severe stridor and required emergency tracheostomy. Current literature is reviewed for the histopathology, common clinical features, radiological findings and treatment options for aerodigestive amyloidosis.

Matched MeSH terms: Amyloidosis/diagnosis*

Fulltext Splenic amyloidosis: a rare cause of spontaneous splenic rupture

Chan RS, Abdul Aziz YF, Chandran P, Ng EK

Singapore Med J, 2011 Nov;52(11):e232-5.

PMID: 22173263

A 62 year-old woman who presented with an atraumatic acute abdomen was discovered to have haemoperitoneum with splenic rupture on urgent computed tomography and was immediately referred for life-saving emergency splenectomy. Histopathological examination revealed secondary splenic amyloidosis. The patient was later found to be suffering from infective endocarditis secondary to her permanent cardiac pacemaker. This report describes a patient who could have suffered from a long-standing infected vegetation on a permanent cardiac pacemaker, which led to splenic amyloidosis and spontaneous splenic rupture.

Matched MeSH terms: Amyloidosis/diagnosis*

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