What investigations are appropriate in a teenage girl with chronic renal failure who has primary amenorrhea?
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Adolescent↗
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Results for “Puberty, Delayed”
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Evaluation of growth hormone in thalassaemic boys with failed puberty: spontaneous versus provocative test.
Growth hormone (GH) secretion was determined by evaluating ultradian GH profiles for 12 h and GH responses to insulin stimulated hypoglycaemia (ITT) in 28 stunted boys with beta-thalassaemia major aged 15.2-17.4 years, who presented with pubertal failure (FP). Healthy non thalassaemia prepubertal boys (n = 10) aged 7.5-8.8 years, were studied as controls. All patients had normal responses to ITT with peak GH levels > or = 15 mU/l. Basal GH concentrations (mean +/- sem) (1.65 +/- 0.03 mU/l vs 2.58 +/- 0.27 mU/l; P < 0.05) and the stimulated GH responses (peak GH = 15.4 +/- 0.20 mU/l vs 21.08 +/- 0.78 mU/l; P < 0.001) were significantly lower in the patients with failed puberty than in the controls, indicating that the FP patients had diminished GH reserve and secretory capacity. Moreover, all the GH peak parameters including the maximum spontaneous concentrations (MX-GH) and the area under the GH curve (AUC) were significantly lower in the thalassaemic patients than in the controls (MX-GH = 5.2 +/- 0.21 mU/l vs 20.42 +/- 0.14 mU/l; P < 0.001; AUCb = 421.22 +/- 4.31 mU/l vs 712.20 +/- 3.42 mU/l; P < 0.001). These observations suggest that the thalassaemic patients had endogenous neurosecretory GH deficiency (GHND). Priming with sex steroid did not cause any improvement in the spontaneous or stimulated GH secretory patterns in thalassaemic patients. It was noteworthy that in neither the patients nor the control subjects, was there a significant correlation between the maximum stimulated and the MX-GH concentrations.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
A physiological mode of puberty induction in hypogonadal girls by low dose transdermal 17 beta-oestradiol.
Transdermal 17 beta-oestradiol administration (17 beta-E2), used mainly in menopausal women, allows a continuous 17 beta-E2 delivery through the skin into the systemic circulation, avoiding intestinal and hepatic passage. In order to explore whether transdermal 17 beta-E2 could be used for the induction of puberty, 17 beta-E2 patches with low dose delivery were administered in nine prepubertal girls with Turner syndrome (bone age greater than 10.5 years) for a mean period of 2.2 years. Treatment schedule: 5 micrograms/day for 6-9 months, 10 micrograms/day for 6-9 months, 25 micrograms/day for long-term substitution; addition of cyclic gestagen p.o. after 18-24 months. Breast development started within 3 months of therapy and menstruation occurred after 2 years. Growth rate increased from 3.2 to 5.0 cm/year during the 1st year of therapy, height prediction did not change. Serum oestradiol (E2) and urinary E2 conjugates increased proportionally with 17 beta-E2 doses, serum oestrone (E1) rose much less. The possibility to imitate time course, clinical events and hormonal changes of normal puberty, the absence of adverse drug reactions and the excellent acceptance and easy mode of application suggest that transdermal 17 beta-E2 is optimally suited for hormonal substitution in girls with hypogonadism.
Administration, Cutaneous↗
Long-term outcome in 134 patients with galactosaemia.
In a retrospective study 134 galactosaemic patients, born between 1955 and 1989 in the Federal Republic of Germany were traced and their long-term outcome evaluated. We investigated 83 galactosaemic patients (78 homozygotes, 5 compound heterozygotes) by clinical, psychometric and laboratory testing; 31 patients were evaluated by medical history, the remaining 20 patients had died due to sequelae of the underlying disease. In 48 out of 78 classical galactosaemia patients galactose-free therapy had been started before the 15th day, in 19 between days 15 and 56 and in 11 patients after the 56th day. Physical findings revealed that puberty was delayed in 1 out of 18 males and 6 out of 11 females. Neurological abnormalities included ataxia (n = 6), intention tremor (n = 11) and microcephaly (n = 10). Speech abnormalities were found in 43 out of 66 patients over 3 years of age and disturbance of visual perception and/or arithmetic deficits in 29. Intelligence declined with age, i.e., a DQ or IQ less than 85 was found in 4 out of 34 patients less than 6 years of age (12%), in 10 out of 18 between 7 and 12 years (56%) and in 20 out of 24 older than 12 years (83%). Metabolite patterns (RBC galactose-1-phosphate and UDP-galactose, plasma and urinary galactitol) did not correlate with DQ or IQ. Dietary compliance was good in almost all patients. Compound heterozygotes (n = 5) had normal mental and growth development and all laboratory parameters were in the normal range.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Pediatric aspects of orthodontics].
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Adolescent↗
Pituitary adenomas in childhood and adolescence. Clinical analysis of 10 cases.
Pituitary adenomas in childhood and adolescence constitute 2-6% of all operated pituitary adenomas. We report the clinical features, treatment and follow-up of 10 pediatric patients affected by pituitary adenomas. All patients underwent clinical evaluation, endocrine tests, magnetic resonance imaging and visual field assessment. Follow-up ranged from 8 to 132 months (median 52.6). All patients were older than 10 years of age; 60% were males. In 50% the initial complaints were headache and/or visual impairment, all except one had clear evidence of endocrine dysfunction. Ninety percent were macroadenomas. According to hormone measurements and immunostaining 50% were prolactinomas, 20% were pure GH-secreting and 30% were non-functioning adenomas. Prolactinomas in two females were successfully treated with cabergoline. The other patients underwent surgery: three prolactinomas are still being treated with dopamine agonists and a GH-secreting adenoma is being treated with octreotide LAR and cabergoline. Two patients were also treated with conventional radiotherapy. Treatments were completely successful in 50% of patients: these have normal hormone secretion, full pubertal development, no significant tumor mass and normal visual field. Hypersecretion of prolactin persists in two cases; partial or complete hypopituitarism is present in four, relevant tumor remnant in another four and impairment of visual field is present in two cases. In conclusion, pediatric adenomas occur mostly in pubertal age, are prevalently macroadenomas and clinically functioning. Medical therapy should be preferred for secreting adenomas, but in some cases, notably prolactinomas in males, surgery and eventual radiotherapy may be needed.
Acromegaly↗
Growth hormone treatment in non-growth hormone-deficient short children.
The unlimited availability of GH obtained by recombinant DNA technology has allowed optimization of treatment in GH-deficient (GHD) children. At the same time it has prompted a number of studies in conditions not characterized by GHD such as Turner syndrome, intrauterine growth retardation, chronic renal failure and other chromosomal and genetic abnormalities associated with short stature. Several controlled and uncontrolled studies have now reported the adult height of patients with short stature and normal GH secretion. Critical reviewing of the data shows that some short non-GHD children may benefit from a prolonged treatment with GH. However, further studies are needed in order to be able to identify the subjects for whom treatment is really beneficial.
Adolescent↗
Partial 17, 20-desmolase and 17 alpha-hydroxylase deficiencies in a 16-year-old boy.
Thirteen plasma steroids as well as ACTH, LH and FSH were measured by specific RIAs under basal and dynamic conditions in a 16-year-old boy (normal external genitalia, 46, XY karyotype) who presented slowness and unachievement of pubertal development. On the delta 4-pathway: basal levels of testosterone and dihydrotestosterone were low- with a normal ratio-, delta 4-androstenedione and 11 beta-hydroxyandrostenedione were in the low normal range. Meanwhile, 17 alpha-hydroxyprogesterone and progesterone levels were markedly elevated. On the delta 5-pathway: dehydroepiandrosterone was extremely low while 17 alpha-hydroxypregnenolone and pregnenolone were almost normal; dehydroepiandrosterone sulfate was subnormal while pregnenolone sulfate was normal. Cortisol, aldosterone were normal while ACTH was moderately increased. Basal and responsive levels of LH and FSH were markedly increased. ACTH stimulation induced a subnormal rise of cortisol and 11 beta-hydroxyandrostenedione, a low or absent rise of dehydroepiandrosterone, 17 alpha-hydroxypregnenolone, androstenedione and 17 alpha-hydroxyprogesterone contrasting with a marked rise of pregnenolone and progesterone. After hCG stimulation, responses were low for testosterone, extremely high for 17 alpha-hydroxyprogesterone with a normalisation of the 17 alpha-hydroxyprogesterone/progesterone ratio. Fluoxymesterone dramatically reduced the pathologically high basal levels of progesterone and 17 alpha hydroxyprogesterone. Dexamethasone induced only a minute decrease in the delta 4-progestagens, a marked decrease in pregnenolone, with a more than 80% reduction of 17 alpha- hydroxypregnenolone, dehydroepiandrosterone, dehydroepiandrosterone sulfate and androstenedione. These data suggest a defect involving the cytochrome P450 common to both 17 alpha-hydroxylase and 17, 20-desmolase activities.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Iatrogenic Cushing syndrome and mutatio tarda caused by dexamethasone containing nose drops].
Nasal steroid drops can produce iatrogenic Cushing's syndrome if the recommended dose is exceeded. We now report a 19-year-old male patient for whom dexamethasone nasal drops were prescribed because of nasal obstruction. He had been using these drops for more than 5 years, with a daily dexamethasone dose of 0.7-1.0 mg. It is likely that significant amounts of dexamethasone were swallowed and absorbed by the patient's gastrointestinal tract. As a consequence, the patient developed Cushing's syndrome with panhypopituitarism, growth retardation, osteoporosis and hypertension. A boyish size of the larynx resulted in a fundamental speaking frequency of a child. After discontinuing the intranasal use of the of dexamethasone and adjusting doses of hydrocortisone over 3.5 months a normalization of endocrine function occurred with the beginning of puberty and normal sexual changes. These findings show that the use of nasal steroid drops particularly in children should be limited to a short period and drugs with a low absorption dose employed.
Administration, Intranasal↗
[Global decompensated heart failure in a 21-year-old type 1 diabetic patient with marked diabetic sequelae and retarded growth and sexual development].
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Adult↗
[Do endurance sports lead to osteoporosis in women?].
Numerous studies show that (high) performance sport may reduce the ovarian function by disturbing the distribution of the hormones at the level of the hypothalamic-pituitary axis. Before puberty a delay in sexual maturity (in particular delayed menarche) and after menarche oligomenorrhoea or secondary amenorrhoea may result, most frequently in aerobic-type activities associated with lower body weight such as running, aerobics and gymnastics. Body composition and specificity of sport, intensity of training, previous menstrual history and eating disorders are all important factors. After stopping with performance sport, the regular menstrual cycle will return, but the chronic lack of oestrogen may reduce bone density and increase the prevalence of stress fractures. In conclusion, female performance athletes require medical facts about training and nutrition and individual information and guiding if a long-term hormone substitution is needed.
Adult↗
The role of Doppler evaluation of the uterine artery in girls around puberty.
BACKGROUND: Presently, the only sonographic parameters used to follow puberty in girls are size and morphology of the uterus and ovaries. Doppler of the uterine artery appears a useful complementary parameter to follow puberty. OBJECTIVE: To determine the potential contribution of Doppler evaluation of the uterine artery in girls around puberty. MATERIALS AND METHODS: We investigated 61 healthy female volunteers aged 2-15 years (mean 10.3 years). In each girl we performed a standard pelvic transabdominal US examination, including measurement of the uterus and ovaries. Uterine arteries were visualized by colour Doppler and a pulsed signal was obtained in each patient. The blood flow velocity waveform was analysed and the pulsatility index (PI) was calculated. Growth of the uterus and ovaries was plotted against age, and the PI was compared to each of the other studied variables (age, size of uterus, volume of ovaries). RESULTS: We observed a strong negative correlation between the PI of the uterine artery and the usually studied variables. We also observed a progressive modification of the Doppler signal pattern of the uterine artery during the establishment of puberty: the narrow systolic flow waves found in prepubertal girls were progressively replaced by a systolic-diastolic flow wave. CONCLUSIONS: The demonstration of diastolic flow can confirm the onset of puberty.
Adolescent↗
The genital tract in female children with imperforate anus.
A retrospective study was undertaken to determine the type and frequency of genital tract anomalies that occur in female children with imperforate anus. The functional outcome and optimal management of the genital tract anomalies were also studied. Primary vaginal anomalies occurred in 22 of 72 patients assessed, and uterine anomalies occurred in 18 of 51 patients assessed. For 17 patients, who were 13 years of age or older, information was available on genital tract function. Of these 17 patients, five had severe vaginal scarring. A previous vaginoplasty or cloacal abnormality was a predictor of poor vaginal function. It is suggested that vaginoplasty, in cases in which it is possible, be delayed until puberty.
Abnormalities, Multiple↗
Gynecologic and obstetric aspects of Gaucher's disease: a survey of 53 patients.
OBJECTIVE: We report our experience on the gynecologic and obstetric features of 53 female patients with Gaucher's disease. STUDY DESIGN: Each patient was interviewed for a detailed medical history, and all underwent a complete physical examination and laboratory work-up. RESULTS: Delay of puberty onset was encountered in two thirds of the patients without subsequent infertility. Heavy menstrual bleeding was a major problem and was best treated with low-dose oral contraceptives. Of the 102 spontaneous pregnancies' 25 (24.5%) ended in spontaneous first-trimesters abortions; 72 continued beyond the twenty-second week. Nine patients (27.7%) were diagnosed as having Gaucher's disease during their first pregnancies. Aggravation of thrombocytopenia and anemia were prominent features, but antepartum blood transfusion was not required. Early postpartum hemorrhage and fever were increased after both cesarean and vaginal deliveries. Development of bone crisis in seven women during the third trimester and early postpartum periods recurred in subsequent pregnancies. Genotypes had not influenced the gynecologic or obstetric manifestations. CONCLUSIONS: Gynecologic and obstetric complications play a significant role in this patient population, representing an additional burden to female patients with Gaucher's disease.
Abortion, Spontaneous↗
Emergence of the mature myosin phenotype in the rat diaphragm muscle.
Immunohistochemical analysis of myosin heavy chain (MHC) isoform expression in perinatal and adult rat diaphragm muscles was performed with antibodies which permitted the identification of all known MHC isoforms found in typical rat muscles. Isoform switching, leading to the emergence of the adult phenotype, was more complex than had been previously described. As many as four isoforms could be coexpressed in a single myofiber. Elimination of developmental isoforms did not usually result in the myofiber immediately achieving its adult phenotype. Activation of genes for specific adult isoforms might be delayed to puberty. For example, two of the three fast MHCs, MHC2X and MHC2A appeared perinatally, while MHC2B did not appear until 30 days postnatal. By Day 60 this isoform was present in approximately 27% of the myofibers, but in most myofibers expression of this isoform was transient (i.e., at Day greater than or equal to 115, less than 4% of the myofibers expressed MHC2B). Fibers which contained MHC beta/slow during the late fetal and early neonatal period coexpressed MHCemb. A marked increase in the frequency of fibers containing MHC beta/slow occurred between 4 and 21 days postnatal. These slow fibers arose from a population of myofibers which expressed MHCemb and MHCneo during their development, and they accounted for the majority of slow fibers found in the adult diaphragm. The adult myosin phenotype of the diaphragm myofibers (as determined with immunocytochemistry, and 5% SDS-PAGE) was not achieved until the rat was greater than or equal to 115 days old.
Age Factors↗
Behavioral masculinization is independent of genital masculinization in prenatally androgenized female rhesus macaques.
Genetic female fetuses were exposed transplacentally to testosterone propionate injected into their mothers either early (Days 40 through 64) or late (Days 115 through 139) in gestation. Early and late androgenized females (EAFs and LAFs, respectively) were raised with normal males and females that served as criteria for evaluating degree of behavioral masculinization induced by the prenatal androgen. EAFs were genitally virilized and LAFs were not. Males and untreated females differed reliably on five behavioral measures: males showed more mother-mounting, more peer-mounting, more rough play with peers, a preference for initiating play with male partners, and less grooming of mothers. Neither type of prenatally androgenized female showed masculinization of all five types of behavior. Compared with females, EAFs showed more mother-mounting, more peer-mounting, less mother-grooming, did not differ from females in rough play, and did not manifest a preference for male partners. LAFs, like females, groomed but did not mount their mothers, and did not show a preference for male partners; but unlike females they showed elevated rough play and mounting with peers. EAFs showed a statistically significant delay in puberty onset (menarche), but LAFs did not. Mothers inspected genitalia of their offspring more often if they were males than if they were females. Mothers of EAFs inspected their offspring's genitalia as often as mothers of males, but mothers of LAFs did not. No aspect of maternal behavior was associated with either the amount or kind of masculine behavior shown toward peers. We interpret the results to mean that genital virilization is independent of, and largely irrelevant to, the expression of those behavioral traits that characterize the juvenile male social role. Moreover, the individual behavior traits that are components of the juvenile male role are independently regulated by the organizing actions of androgen and have separable critical periods. Of the two major traits, mounting peers and rough play with peers, the latter has a greater requirement for androgenic stimulation late in prenatal life.
Aggression↗
Sexual behavior of men with isolated hypogonadotropic hypogonadism or prepubertal anterior panhypopituitarism.
Sexual behavior of men with secondary hypogonadism was studied. Seven of the thirteen subjects presented with hypogonadism secondary to isolated gonadotropin (Gn) deficit, whereas the other six had idiopathic prepubertal anterior panhypopituitarism. Testosterone (T) levels were low and did not differ between the two groups. All subjects were evaluated both during replacement therapy (Gn in the first group; Gn plus cortisone and thyroxine in the second group) and 2 months after withdrawal of Gn therapy. During and after withdrawal of Gn administration, men with isolated deficit of Gn retained sexual activity and nocturnal penile tumescence, although they were partially compromised compared with a control group; on the other hand, panhypopituitarics reported compromised sexual function during Gn treatment and no sexual function when Gn therapy was not given. We conclude that different lesions of the hypothalamus-pituitary axis were accompanied by varying degrees of sexual impairment in the two groups of men presenting both secondary hypogonadism and very low T levels.
Adult↗
beta-Endorphin in genetically hypoprolactinemic rat: IPL nude rat.
Beta-endorphin has been reported to regulate not only stress- and suckling-induced but also basal prolactin secretion. In the aim to better evaluate the endogenous beta-endorphin-prolactin interrelation, we measured beta-endorphin levels in a new rat strain, genetically hypoprolactinemic and characterized by a total lack of lactation: IPL nude rat. Beta-endorphin was measured using a specific anti-h-beta endorphin in plasma and extracts of anterior and neurointermediate lobes of the pituitary, hypothalamus and brain. Pituitary extracts were also chromatographed on Sephadex G50 column. Results obtained showed that in IPL nude females on diestrus and males, the beta-endorphin contents of the neurointermediate lobe was significantly lower than in normal rats, while the values found in the other organs and plasma were similar. However, elution pattern of the anterior pituitary extract from male rats showed greater immunoactivity eluting as I125 h-beta-endorphin than in normal rat; this was not the case for the female rat. These results are consistent with a differential regulation of beta-endorphin levels of anterior and neurointermediate lobe by catecholamines. Moreover they suggest that PRL secretion was more related to neurointermediate beta-endorphin.
Animals↗