Clostridium butyricum MIYAIRI is effective for both the treatment and the prophylaxis of AAD in children, as it normalizes the intestinal flora disturbed by antibiotics.
Background. A 12‐year‐old girl with intractable retroperitoneal paraganglioma experienced increased appetite, acne, obesity, “moon face,” and enlargement of the clitoris during the course of the tumor. Plasma cortisol, serum testosterone, and dehydroepiandrosterone sulfate (DHEA‐S) levels were increased to 34.1 μg/dl, 2.0 ng/ml, and 6.628 ng/ml, respectively. Adrenocorticotrophic hormone (ACTH) levels were not increased, and results of dexamethasone suppression tests were negative. Her condition was diagnosed as Cushing syndrome with virilism. Plasma cortisol levels were increased to a level of 107.1 μg/dl before death.
Methods. Tumor samples were obtained at the time of autopsy. The concentrations of cortisol, androgens, ACTH, and catecholamines were assayed in the tumor extracts. The indirect immunoperoxidase procedure was performed on fixed tissues for cortisol, DHEA‐S, testosterone, and ACTH.
Results. Extracts of the tumor masses contained steroid hormones: the amount of immunoreactive cortisol was 1.64 μg/g wet weight; the amount of immunoreactive testosterone was 25.60 ng/g wet weight; immunoreactive DHEA‐S, 579.00 ng/g wet weight: and immunoreactive ACTH, 891.00 pg/g wet weight in the metastatic mass of the lung. Immunohistochemically, immunoreactive cortisol, testosterone, and DHEA‐S were detectable in the tumor cells. The adrenal gland was atrophic.
Conclusions. The patient is the first reported with malignant paraganglioma with the capacity to produce cortisol, androgens, and ACTH.
A 10-year-old boy who presented with hypopituitarism was diagnosed with supraseller germinoma based on the findings of brain magnetic resonance imaging (MRI). Dissemination of the tumor to the spinal cord was also evident. The patient was first treated with irradiation to both the brain and spinal cord, followed by chemotherapy with cisplatin (CDDP) and etoposide . Peripheral blood stem cell transplantation (PBSCT) with extensive chemotherapy was performed when he was 14 years old. During and after the treatment for the brain tumor, he was placed on hormone replacement with corticosteroid hormone, l-thyroxine, and 1-deamino-8-D-arginine-vasopressin (DDAVP). Growth hormone was also replaced after 2 years from PBSCT. The patient was noted to have gait disturbance, osteoporosis and spondylolisthesis of the lumbar vertebrae (L) at 18 years old. His mean bone mineral density (BMD) of the 2nd to 4th lumbar vertebrae was 0.59 g/m 2 . There was no evidence of recurrence of the tumor. It is possible that a high dose of local irradiation, long-term corticosteroid hormone replacement, and a hypogonadal state are related to the onset and progression of the skeletal abnormalities.
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