A hypotensive 37-year-old man developed the problems of impotence, lack of sweating, orthostatic hypotension, and convulsive syncopal attack. His blood pressure fell to 53 mmHgsystolic following bedtilting from 30 to 60°, but his heart rate remained constant which indicated a diagnosis of acute autonomic neuropathy. With the tilting test, a decrease in serum cathecholamine levels and an increase in bradykinin levels were observed. Four months after admission, anti-nuclear antibody, anti-DNAantibody, and the LE test became positive. The acute autonomic neuropathy appeared to be associated with SLE, and the hyperbradykinism, consequent on orthostatic hypotension.
A 59-year-old woman with nephrotic syndrome was diagnosed as having primary amyloidosis based on the detection of amyloid deposition (AL-protein) in the esophagus and kidneys. Bone marrow aspirate showed plasmocytic proliferation, leading to a diagnosis of multiple myeloma (IgG i,-type). In addition, a very rare translocation t(1; 20) (q21; qil) was seen by chromosomal analysis of both the bone marrow and peripheral blood, multiple myeloma ; amyloidosis ; chromosomal abnormality Then, an azoospermic man with t(1; 20)(q21 ; q13) was reported by Antonelli et al. in 1983. Madan andKleinhout (1987) subsequently showed that the women of a large family that suffered from first trimester abortions had t(1; 20)(p36 ; p11). Most recently, Weh et al. (1990) have reported two patients with multiple myeloma and t(1; 20)(q12.3 ; p13). We here report a woman with multiple myeloma, the nephrotic syndrome and systemic amyloidosis, who also had the chromosomal abnormalities t(1; 20) (q21; q11).
CASE REPORTA 59-year-old house-wife was admitted for treatment of the nephrotic syndrome on 26 May, 1989. The hemogram was normal and the Westergren sedimentation rate was increased. Liver and kidney function was normal. Urinary protein excretion ranged between 10 and 15 g/day, with Bence-Jones protein being detected by immunoelectrophoresis. Her serum total protein level was low (4.9 g/100 ml), but there was an increase of y-globulin (16.9%). This M-protein was shown to be an IgG A-type paraprotein by immuno-electrophoresis. The serum IgG level was elevated (1,473 mg/100 ml), while both the IgA (47 mg/100 ml) and IgM (61 mg/100 ml) levels were low. Bone marrow aspiration microscopically showed the proliferation of plasmocytis (18.0%) with scattered plasmocytic packets, leading to a diagnosis of multiple myeloma. Needle biopsy of the kidney indicated amyloidosis ; with Congo Red staining AL-type amyloid deposition along the capillary walls thickened the basement membrane, resulting in the nephrotic syndrome. Chromosomal analysis by G-banding was performed after the culture of the bone marrow
A 25-year-old man was admitted for examination to determine the cause of hypertension. High levels of noradrenaline in plasma and urine were seen, suggesting that the patient had an adrenal or extra-adrenal pheochromocytoma. However, whole-body scintigraphy using the isotope of [1311] metaiodo-benzylguanidine (1311-MIBG) failed to find the presence of a cathecolamine-secreting tumor. The highest level of noradrenaline was detected in plasma obtained from the left jugular vein after selective venous collections through femoral catheterization. Both the computed tomography and cerebral angiography showed a hen-egg-sized tumor located in the left pterygopalatine. After surgical removal of the tumor, high blood pressure and the levels of noradrenaline in plasma and urine were significantly decreased. Histopathological diagnosis was paraganglioma (cathecolamine-secreting paraganglioma). The patient with cervical cathecolamine-secreting paraganglioma is the first case where the tumor was isolatively located in the pterygopalatine fossa.pheochromocytoma ; paraganglioma ; perygopalatine fossa 1968;Schwartz et al. 1975;Manger and Gifford 1977).
A 49-year-old man with hyperthyroidism caused by the syndrome of inappropriate secretion of TSH (SITSH) is reported. A diagnosis of infertility and hypospermatogenesis due to primary hypergonadotropic hypogonadism had been made 10 years ago. Serum TSH levels were inappropriately elevated with thyrotoxic high levels of thyroid hormone in serum, but no tumorous mass was demonstrated in the pituitary gland. The non-tumorous pituitary resistance of TSH to thyroid hormonal suppression was confirmed by low levels of glycoprotein a-subunit and low a-subunit/TSH ratios in serum before and after the stimulation by TRH. In addition, the patient had elevated levels of LH and FSH in serum, and their exaggerated responses to LH-RH, although serum testosterone concentrations were normal, indicating primary hypogonadism. It is of interest that SITSH appeared in the hypogonadal patient who was preceded by the long standing hypersecretion of LH and FSH.
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