[Show abstract][Hide abstract] ABSTRACT: Abstract We report a Japanese woman with variegate porphyria accompanied by amyloid A (AA) amyloidosis. Arthropathy involving multiple joints occurred at 35 years old and persisted. C-reactive protein was 4.0 mg/dL, but rheumatoid factor was negative. Radiographs did not reveal any loss or narrowing of the joint spaces. Two years later, blister formation after sun exposure and reddish urine were first noted. At the age of 45 years, she developed abdominal pain, nausea, vomiting and seizures. After administration of phenobarbital, reddish urine was noted and muscular weakness progressed to atonic quadraparesis. Porphyria attack was diagnosed from high urinary levels of ∂ aminolevulinic acid and porphobilinogen. At the age of 47 years, hemodialysis was started. At the age of 49 years, progression of her gastrointestinal event resulted in death. Autopsy showed massive deposits of AA amyloidosis in various organs, including the kidneys and digestive tract. Thus, amyloid deposition may have contributed to both end-stage renal failure and her gastrointestinal symptoms. This is the first report about the coexistence of porphyria and AA amyloidosis. Chronic inflammation related to this patient's seronegative arthropathy, although atypical for porphyria, might have contributed to the development of AA amyloidosis.
Amyloid: the international journal of experimental and clinical investigation: the official journal of the International Society of Amyloidosis 10/2013; 20(4). DOI:10.3109/13506129.2013.837390 · 2.01 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Diabetic nephropathy can be regarded mainly as a type of microangiopathy, but is a disease that may also include aspects of macroangiopathy. This is especially true of renal disease in non-insulin dependent diabetes mellitus (NIDDM), which is characterized not only by diabetic glomerulosclerosis, but also by atherosclerosis. We performed morphological studies on the kidney, using computed tomography (CT), focusing on such points as: (1) abdominal aortic calcifications at the level of kidney, (2) calcifications in the renal artery, and (3) wedge-shaped defects on the renal surface. We noted that these findings became more prominent in NIDDM patients during end-stage renal failure than during normal renal function, and were significantly more common in those two NIDDM groups than in age-matched nondiabetic patients without hypertension, hyperlipidemia or gout. NIDDM patients exhibited these features more frequently than IDDM patients.
[Show abstract][Hide abstract] ABSTRACT: Radionuclide ventriculographic and echocardiographic assessments of left ventricular cardiac function were studied in 46 long-term maintenance hemodialysis patients, and comparison of cardiac function pre- and post-parathyroidectomy in 10 hemodialysis patients with secondary hyperparathyroidism was investigated. In long-term hemodialysis patients, impairment of cardiac function was observed in 80.4%. In an overall study of 46 patients, no correlation between intact parathyroid hormone (iPTH) level and left ventricular ejection fraction (LVEF) was observed, but a significant (p < 0.05) negative correlation was observed in patients with an iPTh blood level over 200 pg/ml. A negative correlation between fractional fiber shortening and an iPTh level over 200 pg/ml was observed (p < 0.05). There was neither a correlation between the iPTH level and left ventricular (LV) mass, nor was there a correlation between the iPTH level and wall thickness. After parathyroidectomy, systolic and diastolic blood pressure, cardiothoracic ratio, LVEF, and LV mass decreased significantly (p < 0.05), but no significant difference was detected in cardiac thickness. In summary, the present data suggest that high levels over 200 pg/ml of the iPTH in long-term hemodialysis patients adversely affect the myocardial function, induce cardiac hypertrophy and cause high arterial blood pressure. After parathyroidectomy, the cardiac function improved with a reduction of cardiac mass and an improvement of cardiac contraction.
Mineral and Electrolyte Metabolism 01/1995; 21(1-3):72-6.
[Show abstract][Hide abstract] ABSTRACT: Risk factors for retardation of renal function in 22 patients with non-insulin-dependent diabetes mellitus (NIDDM) were studied and compared with those in 16 patients with insulin-dependent diabetes mellitus (IDDM). The annual decline rate of reciprocal serum creatinine was calculated from the rise of creatinine to the commencement of dialysis. The annual decline rate was compared with levels of blood pressure, fasting blood glucose, HbA1, and lipids, and clinical findings in patients with or without nephrotic syndrome during the same period. There was no significant difference in the rate of decline in levels of fasting blood glucose and HbA1, in IDDM and NIDDM. In NIDDM, the major risk factor is hypertension, as in IDDM. Triglycerides and total cholesterol also play roles in the retardation of renal function. Nephrotic syndrome also influenced the retardation of renal function in both IDDM and NIDDM.
Journal of Diabetic Complications 04/1991; 5(2-3):131-3. DOI:10.1016/0891-6632(91)90044-P