[Benign tracheal stenosis treated by T-tube in a patient with autism; report of a case].

A female with autism, aged over 40 years, who had been hospitalized in a nursing home, developed descending necrotizing mediastinitis requiring tracheostomy. Subsequently, tracheal stenosis was observed. She was referred to our hospital. T-tube therapy was selected, and there has been no recurrence during the 3-year follow-up. We report a patient in whom a T-tube was useful for treating benign tracheal stenosis in the presence of autism.

[Nephrotic syndrome after extended thymectomy for thymoma with myasthenia gravis; report of a case].

A 35-year-old man visited our hospital complaining of blepharoptosis and fatigability of the left arm. Under a diagnosis of myasthenia gravis (Osserman Ila), ambenonium was administered and improvements were noted. At 40-year-old, the patient underwent extended thymectomy due to the development of thymoma, which invaded the lung and pericardium (Masaoka stage Ill). Then ADOC therapy (doxorubicin hydrochloride+cisplatin+vincristine sulfate+cyclophosphamide) was initiated. At 47-year-old, we found recurrence of disseminated thymoma in thoracic cavity. Because of multiple metastatic lesions, radiotherapy in combination with chemotherapy was chosen and these lesions were reduced in size. Facial and foot edema developed at 50-year-old. Chest X-ray revealed bilateral pleural effusion. He was diagnosed as minimal change nephrotic syndrome and steroid therapy was started. The changes in cellular immunity due to thymoma is considered to be causative in the development of nephrotic syndrome. We report the clinical course of our case and discuss with reference to the literature.

[Liposarcoma originating in the neck and the mediastinum after removal of mediastinal lipoma].

A 47-year-old man, who had undergone removal of an upper mediastinal lipoma 9 years previously, was referred to our hospital for swelling of the right neck and wheezing. The chest X-ray showed enlargement of the upper mediastinum. Contrast-enhanced chest computed tomography and magnetic resonance imaging demonstrated a tumor with fatty density extended from the upper mediastinim to the right side of the neck. Two-stage tumor resection was carried out. On gross pathological examination the tumor was soft and fatty. Microscopic examination revealed well differentiated liposarcoma. Postoperative radiation therapy (50 Gy) was carried out. The patient remains free of disease 3 years after radiation therapy.

[The frequency and mechanisms of urolithiasis in acromegaly].

It is generally accepted that acromegaly is often associated with hypercalciuria, but there are few reports on the frequency and the mechanisms of urolithiasis. Recently we consecutively experienced 2 cases of acromegaly with urolithiasis, and these experiences made us investigate the association between urolithiasis and acromegaly. Among 18 acromegalies from 1977 to March 1990 (10 males, 8 females, 24-64 years old), 13 cases (72%) fulfilled the criteria of hypercalciuria (urinary calcium (u-Ca) greater than or equal to 200 mg/day or u-Ca/urinary creatinine (u-Ca/u-Cr) greater than or equal to 0.15), and 7 cases (39%) suffered from urolithiasis that was diagnosed by KUB (4 cases) or X-ray computed tomography (CT) (3 cases). Especially in the last 2 years, 5 out of 7 cases (71%) were complicated with urolithiasis and all 7 cases were associated with hypercalciuria. These results suggest that hypercalciuria and urolithiasis are both much more frequent than previously reported. In 6 cases who were treated by pituitary adenomectomy from 1988-1989 (4 males, 2 females, 24-59 years old), we examined Ca metabolism before and after operation. Before operation, the levels of serum growth hormone (GH), u-Ca (mg/day), u-Ca/u-Cr (in all cases) and plasma somatomedin-C (Sm-C) (in 4 cases) were increased above the normal range. To determine the etiology of hypercalciuria, we performed the oral Ca load test under restriction of Ca (400 mg/day) and P (650 mg/day) intake. The results suggested that the hypercalciuria might be mainly due to the increased absorption of Ca from the intestine (so-called "Absorptive hypercalciuria"). However, the levels of serum vitamin D (Vit. D) metabolites were all within the normal range before operation. After operation, GH and u-Ca/u-Cr (in 5 cases) and u-Ca (mg/day) (in all cases) decreased significantly compared with before operation, and the levels of Sm-C (in all cases), serum 25-(OH)D3, 1 alpha, 25-(OH)2D3 (in 4 cases) and 24,25-(OH)2D3 (in 3 cases) were also reduced after operation. Surprisingly, u-Ca and u-Ca/u-Cr normalized only in 4 cases who showed a reduction in 1 alpha, 25-(OH)2D3 levels after operation, although there were no correlations between u-Ca (mg/day) or u-Ca/u-Cr and 1 alpha, 25-(OH)2D3. Significant correlations were found between u-Ca (mg/day) or u-Ca/u-Cr and Sm-C. The parathyroid function evaluated by the rapid Ca infusion test or nephrogenous cyclic adenosine monophosphate (NcAMP) was normal before and after operation.(ABSTRACT TRUNCATED AT 400 WORDS)

Cyclosporine A (CyA)-induced decrease of serum gonadotropin levels in a case of Klinefelter's syndrome.

We report a case of Klinefelter's syndrome who developed a decrease of serum gonadotropin levels, particularly LH, after CyA treatment for complicated focal glomerulosclerosis (FGS). A 38-year-old man suffering from general malaise and pretibial edema was diagnosed FGS by renal biopsy in October 1988, and was referred to our hospital for further evaluation and treatment for FGS in December 1988. He was not married, and closer anamnesis revealed that he had had impaired seminal ejaculation from the age of 30. The physical examination showed 37% obesity, scanty body hair, pretibial edema and small bilateral testes (3.0 x 1.5cm). Laboratory findings included marked proteinuria (5.3g/day) and mild renal dysfunction (serum creatinine 1.3mg/dl, glomerular filtration rate 57.2ml/min). Endocrinologically, high basal levels of LH and FSH (133.6mIU/ml and 93.7mIU/ml, respectively) and the hyperresponses of LH and FSH to LH-RH stimulation were found, but the other pituitary hormone levels, thyroid and adrenal status, were in the normal range. In testicular biopsy, nodularly proliferated Leydig cells and no seminal tubules could be seen. The chromosome analysis showed 47,XXY karyotype, which confirmed the diagnosis of Klinefelter's syndrome in this patient. From 9 January 1989, CyA (6mg/Kg.day) was orally administered for 4 weeks in order to treat for FGS. After CyA administration, basal levels of LH and FSH remarkably decreased, particularly LH, and their decrease lasted for at least 6 weeks after cessation of CyA (final levels; LH 28.2mIU/ml, FSH 69.8mIU/ml). On the other hand, serum testosterone level was low normal or slightly under normal, and no apparent changes could be seen during CyA treatment.(ABSTRACT TRUNCATED AT 250 WORDS)