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Biomedical subjects

C J Partsch

Publications and source records attributed to C J Partsch.

At least 73 records · Page 4Linked to original sources
PubMed1990

Reference ranges of lutropin and follitropin in the luliberin test in prepubertal and pubertal children using a monoclonal immunoradiometric assay.

Reference ranges of lutropin and follitropin in the luliberin test (60 micrograms/m2 body surface) were established for the various pubertal stages in a large sample of endocrinologically healthy children using a monoclonal immunoradiometric assay (LH and FSH MAIA Clone, Serono Diagnostika, Freiburg, FRG). Qualitatively, the results for lutropin were similar to those already reported from studies using polyclonal RIA's. Lutropin increased gradually throughout pubertal development. Quantitatively, however, the immunoradiometric assay system yielded considerably lower results than polyclonal RIA's. In contrast, follitropin showed a very good linear regression between monoclonal and polyclonal assays. Therefore the former reference ranges for follitropin are still applicable.

Adolescent↗
PubMed1989

Hypothalamic hypogonadism in congenital adrenal hypoplasia.

Congenital adrenal hypoplasia (CAHP) in its X-linked form is associated with hypogonadotropic hypogonadism (HH). A 23 year old man with this disorder received substitution therapy with gluco- and mineralocorticoids starting one week after birth and, recently, pulsatile subcutaneous GnRH treatment via a miniature infusion pump with stepwise increasing doses from 50 to 200 ng/kg body weight/2 hours for a total of 394 days. Testosterone levels increased from prepubertal levels to 409 ng/dl after 2 weeks and to 626 ng/dl after 3 months of treatment. The results of pulsatile GnRH therapy in our patient prove the hypogonadotropic hypogonadism to be of hypothalamic origin. Pulsatile GnRH substitution is a successful therapeutic regimen in patients with CAHP leading to pituitary and gonadal maturation.

Adrenal Glands↗
PubMed1989

Growth, bone maturation and pubertal development in children with the EMG-syndrome.

In 7 patients (5 girls, 2 boys) with the EMG or Wiedemann-Beckwith syndrome, statural growth, bone age (BA), weight and pubertal development were studied longitudinally. Height was above the 90th percentile (%) for chronological age (CA) after age 2 years, reaching an average of 2.5 SD above the mean at or after puberty. Adult or attained height also exceeded significantly (P less than 0.015) parental (genetic) target height by 13.2 cm on the average. In one girl, adult height prognosis (190 cm) could be reduced to an adult height of 183 cm by high-dose estrogen treatment. In most children, growth velocity remained above the 90th % up to 4-6 years of age and normalized thereafter. In all patients studied, bone age was markedly advanced and particularly so during the first 4 years after birth. Weight was above the 90th-97th % during infancy and early childhood and remained there, appropriate or slightly subnormal for height, until adulthood, except for 3 girls who reached and maintained the 50th % during or after puberty. Spontaneous pubertal development occurred within normal limits for CA and around the 50th % for BA. Except for the marked bone age acceleration, the reason for the increased statural growth and adult height in patients with the EMG syndrome is still unknown.

Adolescent↗
PubMed1989

Predictive criteria for successful growth promotion in growth hormone therapy of short stature: a comparison between common endocrine parameters and knemometry.

Due to increased availability of growth hormone (GH) for the treatment of short stature, its use has been proposed for a number of conditions besides classic GH deficiency. We have studied growth response during a one year treatment period with 14 IU/m2/week of GH in a heterogenous group of 24 short children with various conditions associated with short stature (SDS for body height ranging between -2.2 and -4.4). Thirteen children could be classified as "responders" with growth rate increments of 2 cm/yr or more above pretreatment growth rates, and 11 children as "non-responders". The children were measured regularly both by stadiometry and knemometry at weekly intervals. GH stimulation by insulin, arginine and spontaneous overnight secretion of GH, and SM-C generation were evaluated in the children and found to be of no predictive value for the individual responsiveness to GH administration except in one boy with classic GH deficiency. However, serial measurements of the lower leg length provided useful information for individual predictions in 21 out of the 24 children as early as 10 weeks after the start of the GH treatment.

Adolescent↗
PubMed1989

[Transitory precocious puberty caused by autonomous ovarian cysts. Clinical, endocrinologic and sonographic course].

Precocious pseudopuberty in girls may be due to autonomous ovarian cysts. The spontaneous course in two girls aged 3.5 and 5.75 years is described. For differential diagnosis of the precocious pubertal development the following parameters were particularly helpful: growth curves, bone age determinations, repeated LHRH tests and serial sonographic examinations of ovaries and uterus. Since spontaneous regression of pubertal signs could be seen after a few weeks with complete resolution of the ovarian cysts and endocrinological parameters returning to normal after a few months, laparotomy and ovariectomy could be avoided in these two patients.

Body Height↗
PubMed1989

[The significance and characteristics of the LHRH test in diagnosing precocious puberty development in girls: the stimulated LH/FSH quotient differentiates between central precocious puberty and premature thelarche].

LHRH tests (100 micrograms i.v.) were performed in 31 girls with central precocious puberty (PP); the girls were participating in an international multicentre trial for the treatment of PP with the LHRH agonist decapeptyl in microspheres, together with 18 girls with premature thelarche (PT). Assignment to these two groups was made after 6 months to 5 years of clinical follow-up. LH and FSH were determined centrally using a polyclonal RIA. Basal LH and FSH levels and stimulated LH levels were significantly higher in PP patients (p less than 0.001), but the stimulated FSH levels were not significantly different between the two groups. In the PP group, all stimulated LH levels were above the prepubertal range, whereas in the PT patients all stimulated LH levels were within the prepubertal reference limits. In PP and PT patients 52% and 56%, respectively, of the stimulated FSH levels were increased above the range for prepubertal girls. In 55% of the PP patients, stimulated LH levels were also above the reference range for the corresponding Tanner breast stage. In contrast, all stimulated LH levels of the PT group were within the reference limits for their breast stage. For FSH, 45% and 56% of the stimulated levels were above the normal ranges for the corresponding breast stages in the PP and PT groups, respectively. The LH-to-FSH ratio after LHRH stimulation was significantly higher in the PP than in the PT group (p less than 0.001). All but one of these ratios were above 1 in the PP patients and all ratios in the PT patients were below 1.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Determination by Skeleton↗
PubMed1987

Short-term pulsatile administration of luteinizing hormone releasing hormone in male adolescents with multiple idiopathic pituitary hormone deficiencies.

In order to define both level and severity of defect in patients with idiopathic multiple pituitary hormone deficiencies (MPHD) and to find out which patient might benefit from pulsatile LHRH substitution therapy, the effect of short-term pulsatile LHRH infusion in 6 affected male adolescents was studied. Controls were 9 boys with constitutional delay of puberty (CD). During a spontaneous nocturnal plasma profile LH and FSH levels were prepubertal with little evidence of pulsatile secretory LH activity in all MPHD patients. During short-term pulsatile LHRH stimulation (36 h), however, all showed a significant rise in mean LH and FSH levels (p less than 0.0001). Linear regression analysis revealed significant continuous increases of FSH (p less than 0.001) in all patients and of LH (p less than 0.01) in all but one patient. These changes were not accompanied by an increase of testosterone, androstenedione and DHAS levels. Since all MPHD patients showed steadily increasing gonadotropin levels if stimulated in a pulsatile manner, we conclude that the defect might only in part be located at the pituitary level. Long-term pulsatile substitution therapy with LHRH is likely to be successful in these patients as has been demonstrated in patients with known hypothalamic defect.

Adolescent↗
PubMed1986

Treatment of Silver-Russell type dwarfism with human growth hormone: effects on serum somatomedin-C levels and on longitudinal growth studied by knemometry.

Two patients, aged 5 and 12 years, with Silver-Russell type dwarfism are presented. As shown by standard tests and examination of the spontaneous nocturnal hGH secretion, there was only mild regulative hGH deficiency. HGH treatment was started with daily subcutaneous injections at age 5.5 years (height 90.3 cm, -6.1 SDS, bone age 2.75 years) and 12.6 years (height 125.7 cm, -3.7 SDS, bone age 8.75 years), respectively. Treatment was monitored by serial somatomedin-C (SM-C) determinations and by knemometry (lower leg measurement). SM-C values increased in both patients by 10.5 and 4.8 fold, respectively, and remained above the prepubertal range (greater than 2.5 U/ml) during the treatment periods of 1.5 years. Pretreatment knemometric growth rate was high (after a somatomedin generation test) in patient 1 (0.7 mm/week) and low in patient 2 (0.31 mm/week). It remained at the same level in patient 1 (0.67 mm/week) and increased markedly in patient 2 (0.46 mm/week). During a treatment interruption, in both patients, knemometric growth rates fell to 0.33 and 0.30 mm/week, respectively. After resumption of treatment, now with biosynthetic hGH, growth rates increased again in patients 1 and 2 to 0.64 and 0.48 mm/week, respectively. This lower leg growth pattern was parallelled by similar changes in total body growth velocity. Even after the relatively short treatment period of 14 to 16 months, a slight net gain in statural height could be observed, as standard deviation scores for bone age increased.

Age Determination by Skeleton↗
PubMed1985

Differentiation of male hypogonadotropic hypogonadism and constitutional delay of puberty by pulsatile administration of gonadotropin-releasing hormone.

It is not possible to differentiate reliably between male idiopathic hypothalamic hypogonadism (HH) and severe constitutional delay of puberty (CD) on the basis of a standard GnRH bolus test (GBT) or other known endocrine or clinical parameters. Therefore, we studied the response of 17 hypogonadal men, 8 with a diagnosis of HH (age, 15.5-41; bone age, 12.5-19 yr; testes, 1-4 ml) and 9 with CD (age, 14.5-20; bone age, 11-15 yr, testes, 2-10 ml) to pulsatile GnRH stimulation. Basal and peak LH and FSH levels after a single dose of GnRH greatly overlapped between the two groups. In each patient, a spontaneous nocturnal plasma profile of LH and FSH, sampled every 20 min, was followed by a pulsatile GnRH stimulation (5 micrograms iv every 90 min) via a portable minipump for 36 h. Before and after this pulsatile GnRH stimulation, a GBT (60 micrograms/m2 iv) was performed and plasma LH, FSH, testosterone, androstenedione, and dehydroepiandrosterone sulfate were measured. Pulse analysis revealed 0-5 spontaneous nocturnal LH peaks in the CD patients but only one in all of the HH patients. During the 36 h of pulsatile GnRH, mean LH and FSH levels were significantly higher (P less than 0.0001) than during the spontaneous nocturnal profile in all patients (except 1 from each group for LH). The GBT after pulsatile stimulation caused significantly higher (P less than 0.001) LH increments in CD than in HH patients, with no overlap between the two groups (range, 4.1-15.6 in CD vs. 0.8-2.4 mIU/ml in HH). Plasma testosterone rose significantly (P less than 0.01) during pulsatile GnRH from 67 to 155 ng/dl (median) in the CD men, but did not change in the HH group (21 to 22.5 ng/dl). Plasma androstenedione and dehydroepiandrosterone sulfate did not rise in either group. We conclude that, in contrast to other parameters investigated so far, the LH increment in the second GBT after 36 h of pulsatile GnRH allows clear-cut differentiation between CD and HH. These results indicate significantly lower pituitary LH reserve in patients with permanent HH after short term priming of the pituitary by pulsatile GnRH administration.

Adult↗
PubMed1977

[Cogans's syndrome in childhood (author's transl)].

A case--report is given of a patient with Cogan's syndrome in childhood. This syndrome consists of non-syphilitic interstitial keratitis and vestibuloauditory symptoms. Visual loss is rare but hearing loss is rapidly progressive. A relation to autoimmune diseases especially to panarteriitis nodosa has been suggested by a number of authors.

Autoimmune Diseases↗
PubMed1977

[Objective determinations of individual sound protectors (author's transl)].

A method for objective measurements of the sound attenuation provided by ear protectors is described, utilizing a head model which contains all normal human sound properties. The difficulties experienced in localizing sound sources when wearing ear protectors is explained by changing the characteristics of the individual frequency bands.

Ear Protective Devices↗
PubMed1976

[Cervical-nystagmus as related to vertebral receptors].

Two patients with absent peripheral labyrinthine function are reported, in whome cervical nystagmus could be demonstrated. Return of labyrinthine function with simultaneous disappearance of the cervical nystagmus in one of the patients demonstrates existence of receptors in the upper three vertebral joints and their potential influence on the vestibular system. Pathogenetic as well as diagnostic functions of the sensory afferent systems of the upper cervical region are discussed.

Adult↗
PubMed1975

[The acute cervical vertigo under otologic and osteopathic view (author's transl)].

The acute cervical vertigo with the single symptome of rotary vertigo is most probably caused by a functional disturbance in the upper third of the cervical spine. Distinct patho-anatomical changes could not be observed. The findings on the cervical spine are based on an osteopathic examination. The therapy of choice is a manipulation. Three cases are reported.

Adult↗
PubMed1975

[Different forms of deafness in Treacher - Collins-Syndrome, shown on a family over three generations (author's transl)].

A Treacher Collins-syndrome has been followed up in a family over 3 generations. We found a change in deafness from grand parents- and parents-generation to the third one. The first two showed an inner ear deafness, the latter the well-known conduction deafness. The phenotype becomes more and more extensive in the following generations. Simultaneous with this development the deafness becomes more typical. In theory the inner ear deafness could either be explained as the final stage of a long standing conductive deafness or it is part of an incomplete developed syndrome.

Abnormalities, Multiple↗
PubMed1975

[The difference between the noise deafness of the pitmans and the typical noise-induced hearing loss of the worker in metals (author's transl)].

Reffering to 92 expert evidences on the noise-deafness the signs are shown, in which the mining noise-deafness differs from the typical noise deafness in the metallurgical industry. In more than 80% of the cases a slope of the curve could be observed in the audiogram beginning at 125 cps. Therefore the hearing loss early impairs the ability to understand conversational speech. Only every second patient shows a positive result at the SISI-test; this is the reason, that the mining noise deafness frequently is misinterpreted. In these cases the cochlear hearing loss will be surely proved by the Langenbeck-audiogram and Békésy-audiogram. A secondary finding was the typical "mining ear-drum". These ear drums are bilaterally whitish, without reflex and thickened; they don't show any air-bone gap in the audiogram. The differences between the mining noise-deafness and the typical noise-induced hearing loss are explained by an additional baro-trauma which occurs, when the pitmans work at a depth of about 2000 feet.

Audiometry↗
PubMed1975

[The Cogan syndrome].

Exactly 30 years ago D. C. Cogan reported 4 cases of "nonsyphilitic interstitial keratitis associated with vestibulo-auditory symptoms". Report of 57 of these cases have come to our attention. They mostly concern relatively young people, although the age range is now 4-1/2 to 63 years. The etiology of Cogan's syndrome is unknown. It very frequently has been associated with some generalized vascular diseases. In our first case, a 13 year old boy, we found the symptoms of a serous meningitis with pathological signs in the EEG. No vascular changes were encountered. During 4 weeks the rapidly progressive hearing loss was accompanied by tinnitus and vertigo and an interstitial keratitis. The recession of vestibular symptoms is followed by the loss of function of the endorgans. The voice seemed high-pitched and monotonous. In our patient the eye condition improved, but the cochleo-vestibular disturbances did not. The second case, a 27 year old woman, could be observed over a period of 19 years. The ocular symptoms disappeared nearly completely after 1 year, but the cochleo-vestibular disturbances remained. The audition shows even after 19 years some fluctuations.

Adolescent↗