Biomedical subjects
Publications and source records attributed to Y Sen.
We describe Adie's pupil and radiologic changes related to Parry-Romberg syndrome in a child who presented with facial hemiatrophy with no neurologic deficit. We suggest that cerebral lesions in Parry-Romberg syndrome without neurologic symptoms must be carefully investigated.
Morgagni hernia (MH) is the least common type of congenital diaphragmatic hernias. Although its course is often asymptomatic, it may be associated with various respiratory and gastrointestinal symptoms. We describe 7 children with MH during a 5-year period in three pediatric centers in Turkey. All children had acute or chronic respiratory symptoms; cough was the most frequent. The diagnosis was made by posterior-anterior (PA) and lateral chest X-rays. The PA chest X-rays showed a homogenous mass in 2 and a gas-filled cystic image in 3 children in the right cardiophrenic angle. A retrocardiac homogeneous density in one child, and bilateral consolidation in lower lung areas in another child were also seen. All lateral chest X-rays showed gas-filled bowel loops above the diaphragm. The diagnosis was confirmed by barium-contrast radiograph. Four patients had five additional anomalies, i.e., ventricular septal defect, right inguinal hernia, congenital hip dislocation, pectus carinatum, and obstruction of the uretero-pelvic junction. All of the hernias were repaired by an abdominal approach. There were no complications or recurrences during follow-up. In conclusion, MH should be considered in the differential diagnosis of cases of long-standing respiratory symptoms and/or when an unexplained radiological image, especially on the right cardiophrenic area, is present.
Polyarteritis nodosa (PAN) is a rare vasculitic syndrome in childhood. There are few reported cases of ischaemic necrosis of the intestine and even fewer survivors in adults. We report the case of a 10-year-old boy with PAN and an acute abdomen that required operative intervention. Evidence was found of mesenteric arteritis with large ischaemic segments resulting in infarction and perforation.
The HPLC system was used to separate and measure 10 kinds of corticoids in adrenal tissues. Calibration curves were drawn as straight lines that ranged from 1.25 to 20ng, or 1.25 to 200ng by peak area calculated with the chromatointegrator. The samples for the assay were extracted from homogenized tissues and treated with methanol to remove non-steroidal contaminants which may interfere with the ultraviolet absorption monitor. The recovery rate during the assay procedure was calculated using testosterone as the internal standard, because testosterone was not detected in any adrenal tissue examined in the present study. Contents of corticoids were measured in normal adrenal glands obtained during radical nephrectomy for renal cancer and in functioning adrenal adenomas. Steroid levels in the adrenal glands and tumors have been measured by radioimmunoassay until now, and the data obtained in the present study were compared with those in previous reports. Main steroids in normal adrenals were cortisol (F) and corticosterone (B), and there were certain amounts of 11-deoxycortisol (S), 11-deoxycorticosterone (DOC) and precursor steroids. 11 beta-hydroxy-androstenedione was the main androgen in the adrenal gland. Mineralocorticoids other than B and DOC were very low in the normal adrenals. There was a certain balance between the production of cortisol and corticosterone in normal adrenals. In functioning adenomas, the levels of F, B and aldosterone, and F to B ratios (F/B) varied according to their biological features. Although with the HPLC system it was possible to obtain the production balance of each steroid clearly in the chromatogram, we could not detect the delta 5-3 hydroxysteroids such as pregnenolone and dehydroepiandrosterone using the ultraviolet absorption monitor.
We reported the clinical results of radical surgeries for 44 patients with prostate cancer performed between November 1984 and December 1992. The patients were aged from 57 to 79-year-old (mean 67.2) and classified as clinical stage A2 (6 cases), B1 (7), B2 (12), C (16) and D1 (3) respectively. Radical prostatectomy was performed in 42 cases and radical cystoprostatectomy with urinary diversion in 2 patients, and thirty-nine of 44 cases underwent endocrine or chemoendocrine therapies prior to the surgeries. In all patients with stage A2-B1, the operations were curative, on the other hand, more than 80% of clinical stage B2 patients had pT3 tumors and 33.3% of them had lymph node involvements. With regard to stage C patients, the incidence of lymph node metastasis and positive margin was more frequent. Postoperative adjuvant therapies were added to the patients with pT3,4 tumors and nodal involvements. Patients without residual tumors (n = 20) remained disease-free for 8-89 months (mean 32.8). Of 24 patients who had incomplete resection of tumors, 2 died of other diseases, other 2 were alive recurrent and 20 were alive free from disease for 3-99 months (mean 33.8). The surgical indication for low-stage (A2-B2) prostate cancer has been widely accepted, however that for high-stage cancer (C,D1) has remained controversial. It was our belief that radical surgeries for high-stage cancer could become a potentially curative therapeutic modality in combination with pre- and post-operative adjuvant therapies.
A 64-year-old man with sudden onset of quadriplegia due to marked hypokalemia was referred to our clinic with suppressed plasma renin activity in the presence of a low aldosterone level. Computerized tomography demonstrated a left adrenal adenoma, shown on adrenal scintigraphy to be functioning. The elevated basal level of plasma corticosterone and its increased response to 1-24 adrenocorticotropic hormone suggested the tumor produced corticosterone. The surgical specimen was a benign adrenocortical adenoma with excess content of corticosterone and 18-hydroxycorticosterone.
The effects of alpha 1-adrenergic blockade with prazosin HCl were examined in 28 patients with benign prostatic hypertrophy and bladder neck contracture. The patients received 2-3 mg of prazosin HCl daily administered orally. Improvement in subjective symptoms was observed in 75% of the patients. We evaluated clinical effects as the minimum time of micturition (calculated as the ratio of voided volume/maximum flow rate) for voided volumes less than 200 ml and the maximum flow rates and average flow rates for voided volumes over 200 ml. Evaluation of the findings on a flow rate nomogram revealed improvement in maximum flow rates and average flow rates in 41% of the patients. Side effects such as dizziness developed in only 1 patient.
We have measured androgen receptor in whole cell, cytosol and nuclear fractions of cultured skin fibroblasts originated from the genital area. Samples were obtained from the following patients: 10 boys with phimosis, 5 boys with cryptorchism, 4 boys and a man with hypospadia, 2 infertile men and 3 normal men. Androgen receptor was measured using methyltrienolone (R1881) as the ligand according to the assay technique of Eil et al. using dispersed cell method. The results were as follows: 1. There were no significant statistical differences in the dissociation constant among the diseases. 2. Hypospadia cases showed lower maximum binding capacities (Bmax) of whole cell than phimotic boys but the difference was not statistically significant. 3. The Bmax of normal men were statistically higher than those of phimotic boys and about 80% of these receptors were seen in the nuclear fraction. We also experienced low Bmax levels in an adult hypospadia case and two infertile men. These results suggest that this method is a simple and rapid method to study both nuclear and cytosol androgen receptor at the same time. It is also suggested that there are some cases with reduced androgen receptor levels in hypospadia and infertile cases.
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Infection-associated hemophagocytic syndrome (IAHS) is a form of the reactive hemophagocytic syndrome. IAHS is associated with viral, bacterial, fungal, mycobacterial, rickettsial and protozoal infections and with various malignant neoplasms. A more accurate designation for this acquired form of the syndrome is reactive hemophagocytic syndrome (HS). Reactive HS is characterized by malaise, fever, hepatosplenomegaly, lymphadenopathy, cytopenia, hypertriglyceridemia, hypofibrinogenemia and hemophagocytosis. Cyclosporin A, VP-16, high-dose steroids, and intravenous immunoglobulin (IVIG) have been used in the treatment of IAHS. Here, a 10-year-old girl with reactive HS due to possible viral infection was treated successfully with cyclosporin A and IVIG. Fever disappeared on the third day, complete blood count reached normal levels on the sixth day and hepatosplenomegaly disappeared on the ninth day after treatment. We believe cyclosporin A and IVIG may be used in the treatment of reactive HS, at least in selected patients. Further studies are required to confirm its role as first-line therapy for children with IAHS.