The pathogenic mechanism of diabetic nephropathy has been extensively investigated, and the significance of alteration in glomerular hemodynamics or mesangial cell metabolism has been recently clarified. It is expected t...The pathogenic mechanism of diabetic nephropathy has been extensively investigated, and the significance of alteration in glomerular hemodynamics or mesangial cell metabolism has been recently clarified. It is expected that in the near future these pathologically important alterations can be corrected, and the development diabetic nephropathy can be halted.
We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialograp...We report a rare case of dry eyes and dry mouth caused by primary amyloidosis. A 66-year-old woman with keratoconjunctivitis sicca and xerostomia died of acute respiratory failure. Shirmer's test, gum test, and sialography indicated Sjögren's syndrome. Lip biopsy revealed amyloid deposition around the salivary ducts. Bence-Jones protein was noted in the urine. At autopsy, amyloid deposition was identified histochemically in many organs, mainly on the vessel walls. Primary amyloidosis should be considered as a differential diagnosis of Sjögren's syndrome.
We report a case of IgM-associated mesangial proliferative glomerulonephritis with microscopic hematuria and renal tubular dysfunction. On electron microscopy, a giant spherical deposit was observed in the glomerular par...We report a case of IgM-associated mesangial proliferative glomerulonephritis with microscopic hematuria and renal tubular dysfunction. On electron microscopy, a giant spherical deposit was observed in the glomerular paramesangial area. Deposition of C3 in the glomerular mesangial areas was not observed by immunofluorescence. IgM-associated mesangial proliferative glomerulonephritis is clinically characterized by nephrotic syndrome, mild proteinuria and/or hematuria, tubular dysfunction or renal failure, suggesting that it is considered to be a heterogeneous disorder.
We report a case of HAM/TSP presenting with short stature, mental retardation, skin eruptions, uterine and ovarian hypogenesis and nephropathy. Skin erythema was noted since from the age of three years old and spasticity...We report a case of HAM/TSP presenting with short stature, mental retardation, skin eruptions, uterine and ovarian hypogenesis and nephropathy. Skin erythema was noted since from the age of three years old and spasticity of lower extremities from elementary school age. Serum calcium level showed 4.1 mEq/l. Recombinant human PTH infusion resulted in no response of phosphate excretion. The persistent proteinuria prompted renal needle biopsy, which revealed IgA and C1q deposits in glomerular mesangium. A diagnosis of pseudohypoparathyroidism and IgA nephropathy was entertained. This patient with pseudohypoparathyroidism who has a deficient immune system was seized with the early onset of HAM/TSP and IgA nephropathy.
We report a case of myasthenia gravis (MG) which became worse shortly after the administration of methimazole (MMI) for hyperthyroidism. The activation of immune responses was found during the worsening of MG. The findin...We report a case of myasthenia gravis (MG) which became worse shortly after the administration of methimazole (MMI) for hyperthyroidism. The activation of immune responses was found during the worsening of MG. The findings suggests the possibility that the worsening of MG might be induced by MMI, presumably by its immunomodulatory property.
A 59-year-old male with acute myocarditis presenting symptoms resembling acute myocardial infarction underwent urgent coronary angiography due to difficulties in determining a diagnosis. Coronary artery spasm was demonst...A 59-year-old male with acute myocarditis presenting symptoms resembling acute myocardial infarction underwent urgent coronary angiography due to difficulties in determining a diagnosis. Coronary artery spasm was demonstrated in the proximal right and left anterior descending coronary arteries. The spasm varied from time to time and lesion to lesion. But no fixed occluded lesion was observed. Predischarge coronary angiography showed no abnormal findings. Histological findings of the biopsy specimen from the left ventricle were consistent with myocarditis. The findings of this case indicate that a coronary artery spasm may aggravate the myocardial damage occurring in acute myocarditis. Urgent coronary angiography and endomyocardial biopsy are important to make the diagnosis of myocarditis in patients presenting symptoms resembling acute myocardial infarction.
Mixed connective tissue disease (MCTD) is characterized as a benign rheumatic disease with a favorable response to therapy. When pulmonary hypertension is a complication, however, it is often reported to be fatal. A 32-y...Mixed connective tissue disease (MCTD) is characterized as a benign rheumatic disease with a favorable response to therapy. When pulmonary hypertension is a complication, however, it is often reported to be fatal. A 32-year-old female patient with MCTD who had developed rapidly progressive pulmonary hypertension and disseminated intravascular coagulopathy was admitted to our hospital and was successfully treated with corticosteroids and anticoagulants. The failure of microcirculation due to coagulopathy is considered to be one of the possible entities of pulmonary hypertension.
A 54-year-old man complained of burning pain, warm skin and erythema in his extremities. A diagnosis of primary erythromelalgia was made. Microneurography was used to clarify the role of skin sympathetic nerve activity i...A 54-year-old man complained of burning pain, warm skin and erythema in his extremities. A diagnosis of primary erythromelalgia was made. Microneurography was used to clarify the role of skin sympathetic nerve activity in the pathophysiology of primary erythromelalgia. The patient showed normal skin sympathetic nerve activity but no vasoconstriction response. Aspirin activated the skin sympathetic nerve activity and improved vasoconstriction producing symptomatic relief. These results suggest that the lack of vasoconstriction following vasoconstrictor activity of the skin sympathetic nerves results in increased skin blood flow and burning pain.
We report a 29-year-old male with pelvic lipomatosis that presented with edema of the lower extremities. The patient visited our department because of the marked edema of the lower extremities with body weight gain. The...We report a 29-year-old male with pelvic lipomatosis that presented with edema of the lower extremities. The patient visited our department because of the marked edema of the lower extremities with body weight gain. The pelvic region was generally radiolucent on plain abdominal radiogram. Excretory urogram and pelvic computed tomography scan revealed compression of the bladder by an adipose tissue mass. On lymphograms, lymph vessels were compressed from the inguinal area in the common iliac region. The edema of the lower extremities was thought to be caused by lymph vessel obstruction.
Co-trimoxazole (trimethoprim-sulfamethoxazole) and pyrimethamine-sulfadoxine have been recommended for treatment and prophylaxis, respectively, of Pneumocystis carinii pneumonia (PCP). However, toxic epidermal necrolysis...Co-trimoxazole (trimethoprim-sulfamethoxazole) and pyrimethamine-sulfadoxine have been recommended for treatment and prophylaxis, respectively, of Pneumocystis carinii pneumonia (PCP). However, toxic epidermal necrolysis (TEN) occurred in three of four patients with acquired immunodeficiency syndrome (AIDS) during the course of treatment or prophylaxis of PCP with these agents. On the other hand, 14 patients with PCP treated with pentamidine never developed TEN. Because the incidence of adverse skin reactions is higher among patients with AIDS than those without AIDS, and TEN is a severe, potentially fatal skin reaction, sulfonamide-containing drugs should be given cautiously to patients with AIDS.