Biomedical subjects
Publications and source records attributed to J J Baron.
In 98 young women with lack of maturity, with menstrual disturbances and with insufficiency of the primary and secondary sexual signs an the basis of clinical and hormonal investigations in 61.2% the primary and in 38.8% the secondary ovarian hypoplasia was established. This allowed in spite of great clinical resemblance of the investigated persons to the proper diagnosis and to the choice of the suitable therapy.
In 32 women with signs of androgenization, menstrual disturbances and distinct elevated values of 17-KS, DHEA, pregnantriole, testosterone and very reduced level of 17-OHCS congenital adrenal hyperplasia was diagnosed which established 12.5% individuals with intersexualism. The method of conservative or/and operative treatment and the prognosis were discussed.
In the last 35 years we noticed 91 cases of Turner's syndrome. To the obligatory symptoms of the syndrome belong primary amenorrhoea, sexual infantilism, elevated values only of FSH and very reduced levels of estrogens, chromosomal aberrations in karyotype, most often 45, X and presence of streak gonads. The facultative symptoms of the syndrome belong short stature (94.5% of patients), presence of congenital defects (short webbed neck in 44.4%) and sterility (98.9%). Therapy above 15 years excepting to cause the menstrual cycles have to development the second sexual symptoms by giving enough large doses of oestrogens and gestagens. The best effects we obtain nearly in 100% in the development of breasts, in slowly growth of sexual hair and in the growth of the stature which does not cross the height of 150 cm and only in six patients attained in average 152.5 cm.
Among 67 women with pure gonadal dysgenesis, karyotype 46XY was found in 46 and karyotype 46XX in 21 (26.3% of all intersexual subjects). Karyotype 46XY was either of pure type or mosaicism 45,X/46,XY (10.9%). Primary amenorrhea, underdevelopment of mammary glands and lack or poor development of pubic hair were the main complaints of the patients. In gonadal dysgenesis 46XY mammary glands were developed in 21.8% and pubic hair in 26% suggesting the presence within the gonads of the hormonally active tumor or the state after hormonal treatment. The patients with gonadal dysgenesis 46XX had lowered levels of estrogens and elevated levels of FSH and LH. Karyotype 46XY was not associated with evident changes in hormonal levels. Estrogens were both low and normal, and FSH was elevated (21.5 + 16.6 ug/ml) or normal (3.2-5.0 ng/ml). Total testosterone values were normal or slightly elevated. Such situation can be explained by the presence in some patients of tumors secreting either estrogens or androgens. Taeniform character of gonads was observed by ultrasonography whenever the presence of gonadal tumor was excluded. Histology of specimens taken from gonads or tumors demonstrated the presence of dysgerminoma or gonadoblastoma type of malignancy in 53.1%, foci or proliferation of the Leydig cells in 31.3% and typical morphology of residual gonads without germinal cells only in 12.5%. The differentiation between pure gonadal dysgenesis 46XX and primary ovarian insufficiency is required whenever no characteristic pattern emerges from clinical, hormonal, cytogenetic or ultrasonographic examination. Diagnosis of pure gonadal dysgenesis 46XX can be finally confirmed by the absence of gonocytes in the residual gonad. Besides of removal of gonads or tumors by surgery, the treatment of patients with 46XY karyotype consists in cyclic administration of estrogens and progestagens restoring menstruation and bringing development of secondary sex attributes.
Inter-sexuality is diagnosed in the age between 8-19 years in 58.5% among all 253 individuals with the signs of hermaphrodite. The diagnosis requires clinical, hormonal, cytogenetic and sometimes histopathological investigations. At the youthful patients with signs of hermaphrodite there are confirmed in 66.3% different kinds of gonadal dysgenesis, in 15.5% androgen insensitivity syndrome, in 12.2% congenital adrenal hyperplasia, in 3.4% transsexualism and in 1.3% adrenal virilizing tumours. To call attention to the specific and different treatment of every kind of hermaphrodite it is necessary a very early proper diagnosis of the disease.
On the basis of clinical and hormonal investigations of 58 girls with hirsutism aged between 15-19 years there were confirmed congenital adrenal hyperplasia in 3.4%, late onset adrenal hyperplasia in 24.1% and polycystic ovary syndrome in 72.4%. The proper diagnosis were established above all on endocrinological investigations especially on determinations of 17- ketosteroids, of DHEA, of 17 OH-corticoids in urine and RIA of total and free testosterone in blood.
The aim to study was the determination the effect of operative treatment of 31 patients with polycystic ovary syndrome. Before the operation primarily the authors confirmed at the patients the presence of menstrual abnormalities in 96.8%, of infertility in 100%, of hirsutism I. in 9.7%, II. in 61.8%, III. in 29% and the average level of 17-ketosteroids 16.0 +/- 6.9, of DHEA 3.7 +/- 1.5 mg and of estrogens 39.0 +/- 15.1 mcg in 24 urine, of total testosterone 0.98 +/- 0.24, of FSH 3.4 +/- 1.5, of LH 44 +/- 2.4, of prolactin 11.4 +/- 5.4 and of estradiol 0.32 +/- 0.35 ng/ml in blood. On the basis of clinical, hormonal or/and ultrasonographic examinations the diagnosis of the polycystic ovarian disease was performed.
After the wedge resection of the ovaries the authors obtained the normal menses in 100%, the reduction of total testosterone in blood of 41.8% and of 17-KS in urine of 20%, an increase of fertility of 62.5% and in hirsutism a correction only of 12%, the stopping of 12% and no correction of 76%. The authors conclude that the cause of the healing or of the correction is placed in the resection of 3/4 of the changed ovaries with cysts follicles with distinct thecal layer what is accompanied by the reduction of testosterone on average of 41.8%. On contrary a weak correction in hirsutism has the explanation that the existent hirsutism is supported even by the normal level of testosterone in organism.
One hundred and fourteen nontumorous hirsute women were investigated. The degree of hirsutism, the menstrual abnormalities and the level of 17-ketosteroids, of DHEA, of 17-OH-corticoids in urine and RIA of total and free testosterone and of sex binding globulins (SHBG) in blood were performed. On the basis of endocrinological and ultrasonographic determinations 3 patients were diagnosed as congenital adrenal hyperplasia, 35 patients as late onset adrenal hyperplasia and 57 women as polycystic ovary syndrome (PCO). The maximal mean level (X + SD) of total and free testosterone above 0.5 ng/ml relatively above 5.05 pg/ml were performed in 94.3% relatively 97.1% in late onset adrenal hyperplasia and in 78.9% relatively 87.7% in PCO. The diagnosis of PCO is possible among 75% of all hirsute patients after exclusion the patients with late onset adrenal hyperplasia. The PCO is confirmed by hormonal or/and ultrasonographic examination. The diagnosis of late onset adrenal hyperplasia is allowable only by hormonal investigation.
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37 lumbar discal herniations situated in the intervertebral foramen were operated on, out of a total of 525 operations for lumbar discal herniations during the same period, that is to say 7%. Perhaps the habitual negativity of the contrast neuroradiologic investigations (saccoradiculography, discography, phlebography) explains its relative rareness. In the futur, the scanner, always positif in our cases, will perhaps enable us to appreciate its exact frequency. The radicular pain may be simple in the territory of a root emerging at the upper discal level, which explains the difficulty of diagnosis. It may also interest two roots, including in that case, the root emerging at the discal herniation level. The great frequency of L4-L5 herniations explains the high number of crural pains by compression of the L4 root. Finally, crural pain, in our series of all the lumbar herniations operated on, seems to be linked to a herniation of the upper discs (L3-L4, L2-L3 : 18 cases) as often as to a herniation of the intervertebral foramen of L4-L5 (17 cases).
The authors report 2 cases of primary biliary cirrhosis associated with scleroderma (calcinosis, Raynaud's syndrome, sclerodactyly, telangiectasia) in one case, and associated with pseudopolyarthritis in the other; they recall the conditions under which the rheumatologist may be led to make the diagnosis of this disease. Apart from liver diseases including acute or chronic rheumatic signs, one may observe in primary biliary cirrhosis without symptoms certain other rheumatological syndromes: e.g. scleroderma, Sjögren's syndrome, rheumatoid arthritis, Charcot's joint. The laboratory examinations may give unexpected results: e.g. high levels of IgM, the presence of antinuclear antibodies or may give unexpected results: e.g. high levels of IgM, the presence of antinuclear antibodies or cryoglobulins which sometimes orient wrongly the diagnosis in other directions. One may thus note that a high level of alkaline phosphatase should suggest in any case of inflammatory rheumatism, primary biliary cirrhosis, and attempt to prove this by seeking antimitochondrial antibodies.
Extensive junctional complex formation was observed between cells of an insulinoma with an acinar pattern. Cytolysosomes, multivesicular bodies, and small vesicles were present in the luminal aspect of acinar cells that were shedding cytoplasm. These and other ultrastructural features suggest a recapitulation of intestinal development by this tumor and this is consistent with an endodermal origin of pancreatic beta-cells or their precursors. Psammoma body formation, a rare occurrence in apudomas, was probably due to the accumulation of intraluminal cytoplasmic debris.
A double-blind study was conducted on the effects of nylidrin hydrochloride versus placebo in 60 patients with mild to moderate symptoms of chronic brain syndrome. Their ages ranged from 65 to 99 years. Preliminary results indicated that nylidrin HCl (a vasodilator) is a relatively safe therapeutic agent and more effective than placebo in ameliorating the symptoms of cognitive impairment associated with aging, on both a short term (3-month) and a long-term (9-month) basis. In a dosage of 24 mg daily, it reached its peak effectiveness after 3 months of use.
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In an attempt to find out the becoming of patients with sciatica who have been medically treated, 112 previously hospitalized patients have been questioned and examined by the same physician, two to eight years later. The criteria used were the presence of pain and the level of social and professional activity. The good and excellent results (58%) were slightly better than the poor results and failures (42%). However, painful sequelae were very frequent (83.5%) in the form of lumbago, sciatica or a combination of both, and obliged one subject out of three--manual workers or laborers--to change their occupation. Neither age nor sex, ethnic origin nor occupation, nor the circumstances surrounding the onset (whether or not an accident at work) influenced the course. On the other hand, a long standing history of sciatica decreases the chances of a cure and the older the history, the worse the long-term prognosis. Thus, it seems reasonable not to prolong medical treatment over two months and to advise surgery after a well conducted course of treatment in the recurring cases of sciatica, and in those that do not respond to the usual therapeutic measures. To persist in following a medical treatment threatens to leave definite sequella from prolonged radicular compression.