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PubMed1995

A gene for ulnar-mammary syndrome maps to 12q23-q24.1.

Ulnar-mammary syndrome (UMS) is an autosomal dominant disorder characterized by posterior limb deficiencies or duplications, apocrine/mammary gland hypoplasia and/or dysfunction, abnormal dentition, delayed puberty and genital anomalies. We report the mapping of a gene causing UMS to chromosome 12q23-24.1. Linkage analysis generated a positive lod score of 6.21 at theta = 0.00 with the marker D12S79, and recombinants bracket the UMS gene to a 21 cM region. This region contains a locus for Holt-Oram syndrome (HOS) suggesting that the genes for UMS and HOS may be allelic or closely linked. The identification of the gene causing UMS will be an important step toward understanding the molecular mechanisms that control limb and apocrine gland development.

Abnormalities, Multiple↗
PubMedDate not supplied

Menstrual disorders in adolescence: investigation and management.

Menstrual disorders in adolescence may present diagnostic and management challenges for the gynaecologist. This review will describe the common and uncommon menstrual disorders that may arise in early reproductive life, together with guidance on their investigation and management.

Adolescent↗
PubMed1999

The association between melatonin and sleep stages in normal adults and hypogonadal men.

The role of melatonin in normal sleep-wake regulation has been inferred from the temporal relationships between its cycle and the 24 h cycle in sleep propensity. Pharmacological doses of melatonin were reported to have sleep-inducing effects in insomniacs. The current study investigated the relationship between melatonin and sleep stages in groups of hypogonadal men with abnormal melatonin levels. We were also interested in examining what would happen to these relationships during testosterone replacement therapy. Male patients with hypogonadotropic hypogonadism (IGD, n = 6), constitutional delayed puberty (DP, n = 6), and Klinefelter's syndrome (KS, n = 5) before and during testosterone replacement therapy were studied. Six patients with KS and normal testosterone levels were also studied. Results were compared with those obtained in normal controls (n = 6). Serum samples were obtained at 15 min intervals from 1900-0700h in a controlled light-dark environment with simultaneous polysomnographic sleep recordings. Serum melatonin levels were the highest in IGD and DP and lowest in KS patients. A lower percentage of sleep stage 2 and higher percentage of stage 3/4 were observed in IGD and DP groups while KS patients had higher percentage of stage 2 and lower percentage of stage 3/4 as compared to controls. Slow wave sleep was the highest in IGD and the lowest in KS groups. Serum melatonin levels were lowest in KS groups. Serum melatonin levels were lowest in sleep stage 3/4, higher in stage 2 and highest in REM sleep when all groups were combined and averaged together. However, in the IGD group, melatonin levels were actually lowest in REM sleep. Also in the KS group, melatonin levels were lower in REM than during sleep stage 2. Serum melatonin levels were lowest in sleep stage 3/4 in all groups, higher in stage 2, and highest in REM sleep. During waking periods, melatonin levels were the highest in untreated IGD, DP and KS patients. Testosterone treatment given to these patients, although normalized, their melatonin levels did not statistically significantly change these correlations. These data demonstrate that relative melatonin concentrations are associated with sleep stages in hypogonadal and normal men. The results also indicate that the association between melatonin and the reproductive hormones are independent of the synchronizing effects of melatonin on sleep homeostasis.

Adolescent↗
PubMed2005

Effects of acute and chronic exposure to the aryl hydrocarbon receptor agonist 2,3,7,8-tetrachlorodibenzo-p-dioxin on the transition to reproductive senescence in female Sprague-Dawley rats.

Activation of the aryl hydrocarbon receptor (AHR) can occur in polluted environments, either from smoking-related toxicants or from endogenous ligands. We tested whether acute or chronic exposure to the AHR agonist 2,3,7,8-tetrachlorodibenzo-p-dioxin (TCDD) alters the transition to reproductive senescence in female Sprague-Dawley rats. In experiment 1, rats (n = 6 per experimental group) received a single dose of 0 or 10 mug/kg of TCDD orally (p.o.) on Postnatal Day 29. Vaginal cytology was monitored for 1 wk each month until rats were killed at 1 yr of age. The single prepubertal exposure to TCDD hastened the transition to reproductive senescence in female rats and was associated with delayed puberty, abnormal cyclicity, and premature reproductive senescence. In a second experiment, rats were exposed to TCDD chronically through weekly dosing (0, 50, or 200 ng kg(-1) wk(-1) p.o., n = 7 each dose) beginning in utero. Lifelong exposure to these lower doses of TCDD induced a dose- and time-dependent loss of normal cyclicity and significantly hastened the onset of the transition to reproductive senescence (P < 0.05). This premature transition to reproductive senescence was associated with prolonged estrous cycles and, at the highest dose of TCDD, persistent estrus or diestrus. The number and size of ovarian follicles were not altered by TCDD. Diestrous concentrations of LH in rats exposed chronically to TCDD were similar to those in controls, whereas progesterone tended to be elevated at both doses of the dioxin (P < 0.08). Serum FSH was elevated in the group exposed to 50 ng/kg of TCDD (P < 0.02), whereas estradiol was decreased at both doses of dioxin (P < 0.01). Data thus far support endocrine disruption rather than depletion of follicular reserves as a primary mechanism of the premature transition to reproductive senescence following activation of the AHR pathway by TCDD in female rats.

Aging↗
PubMed1983

A new hypoprolactinemic rat strain. Prolactin, luteinizing hormone, follicle-stimulating hormone, testosterone and corticosterone levels in males and effects of two anesthetics.

A new strain of rat, the IPL nude rat, derived by spontaneous mutation from the Sprague-Dawley strain, is characterized by a lack of hair, delayed puberty and a total lack of lactation. To characterize partially the endocrine system in this rat, serum levels of prolactin (Prl), luteinizing hormone (LH), follicle-stimulating hormone (FSH), testosterone and corticosterone were quantified. The content of gonadotropins and Prl in the pituitary gland was also measured. Blood samples were collected following decapitation or by cardiac puncture after ether or ketamine anesthesia. Serum levels of Prl were decreased by approximately 50% in IPL nude compared to normal Sprague-Dawley rats, regardless of the method used for collection of the samples. There were also significantly reduced levels of testosterone, LH and FSH in the serum collected from IPL nude rats. Levels of corticosterone in serum were similar in IPL nude and normal rats. There was the same relative response to anesthesia in the levels of all the hormones measured when the data from IPL nude and normal rats were compared. The quantities of LH, FSH and Prl contained in the pituitary of the IPL nude rats were not significantly different from those observed in normal rats. These data suggest that the serum hormone deficiencies observed in the nude IPL rat may be due to an altered regulation of hormone production by hypothalamic factors.

Anesthetics↗
PubMed1988

Relationship between bioassay and radioimmunoassay measurement of prolactin in the IPL rat, a hypoprolactinemic rat strain.

IPL (Institut Pasteur, Lyon) nude, hypoprolactinemic rats exhibit delayed puberty and a complete lack of lactation. To characterize the secretion of circulating forms of prolactin (PRL) of these rats, PRL concentrations were measured in serum and pituitaries of males and females under various physiological conditions. Two assay methods, a radioimmunoassay (RIA) and a sensitive bioassay (NB2BA) were employed. Normal rats of the Sprague-Dawley strain were tested simultaneously, as controls. The pituitary content of PRL, estimated either by RIA or by NB2BA, in IPL nude males and females was similar to that of normal male and female rats. On the contrary, serum PRL levels of IPL male rats, measured by RIA or NB2BA, were significantly reduced when compared to normal rats. In both groups, there was a close correlation between the results obtained by the two methods, the NB2BA estimates being higher. However, the NB2BA/RIA ratio was significantly decreased in serum from IPL nude rats compared to controls, indicating that the circulating form of PRL was less bioactive in this group. Castrated male rats injected with estradiol showed sharply increased PRL values as estimated by RIA or NB2BA. The increase was greater (35-fold) in IPL nude rats then in normal rats (9-fold), but these increases resulted in serum PRL levels being similar in the two groups. However, the NB2BA/RIA ratio remained significantly reduced in IPL nude rats. In female rats, PRL was measured during different physiological states: estrus, diestrus, proestrus at 1000, 1200, and 1600 h and Days 1 and 21 of gestation and 2 days postpartum.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1990

Alterations in gonadotropin secretion and ovarian function in prepubertal gilts by elevated environmental temperature.

The effect of chronic exposure to elevated environmental temperature on gonadotropin secretion and ovarian function was studied in prepubertal gilts. Gilts were maintained under control (15.6 degrees C) or elevated temperature (33.3 degrees C) conditions from 150 to 180 days of age. Endocrine and ovarian responses to bilateral (BLO), unilateral (ULO), and sham ovariectomy were evaluated between 175 and 180 days of age. During the 96-h sampling period after BLO, plasma concentrations of follicle-stimulating hormone (FSH) and luteinizing hormone (LH) were suppressed in heat-stressed females. Similarly, elevated temperatures abolished the transient rise in FSH and subsequent follicular growth normally associated with ULO. In contrast, environmental treatment had no effect on the secretion of FSH and LH after sham ovariectomy, yet the number of small follicles was lower in gilts exposed to elevated temperatures than in females maintained under control conditions. These results indicate that a chronic exposure to elevated environmental temperature during pubertal development diminished the ability of the hypothalamo-hypophyseal axis to secrete FSH and LH, which had physiological consequences on follicular growth. When provided an appropriate stimulus (ULO), an acute period of FSH secretion and subsequent development of follicles failed to occur in females exposed to elevated temperatures. Consequently, we propose that delayed puberty in gilts during periods of elevated environmental temperatures is due, in part, to a diminished capacity for gonadotropin secretion.

Animals↗
PubMed1994

Adrenal axis and hypogonadotropism in the growth-restricted female lamb.

Growth retardation induced by dietary restriction in the lamb results in a decrease in LH pulse frequency and therefore in delayed puberty. Increased circulating cortisol levels have been associated with nutritional restriction in a variety of species. The current study tested the hypothesis that hyperactivity of the adrenal axis sustains hypogonadotropism in the growth-restricted lamb. Our approach was to compare the patterns and levels of circulating cortisol and LH in ovariectomized, growth-restricted (n = 8) and ad libitum-fed (n = 6) lambs. At 37 wk of age, after the growth-restricted lambs had been on the reduced diet for 31 wk, basal cortisol levels were determined hourly for 31 h. In addition, during this period, pulsatile LH and cortisol release was determined during a 4-h period (samples every 12 min). Finally, the cortisol response to a physiologic ACTH stimulus and an audiovisual stimulus (barking dog for 2 min) was determined in frequent samples collected during the last 5 h of the 31-h period. As expected, growth-restricted lambs exhibited a low LH pulse frequency (0.25 +/- 0.10 pulses/h) compared with ad libitum-fed lambs (1.37 +/- 0.07 pulses/h). No diurnal cortisol rhythm was observed in either group, and a similar cortisol pulse frequency occurred in the two groups (1.00 +/- 0.07 pulses/h in growth-restricted lambs and 1.05 +/- 0.10 pulses/h in ad libitum-fed lambs). There was no significant difference between the groups in cortisol pulse amplitude. ACTH administration (i.v.) induced a similar cortisol pulse in 4 of 8 growth-restricted lambs and in 5 of 6 ad libitum-fed lambs.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenal Glands↗
PubMedDate not supplied

Hypogonadism and Leydig cell hypoplasia unresponsive to human luteinizing hormone (hLH).

A 22-year-old man with hypogonadism, Leydig cell hypoplasia, high plasma LH, and normal plasma FSH was given human chorionic gonadotropin (hCG) and human luteinizing hormone (hLH) in order to assess testicular testosterone responses to exogenously administered gonadotropins. The maximal testosterone level detected after six doses of hCG, 5000 IU per dose given intramuscularly (IM) was 256 ng/dl. After eight doses of hLH, 550-1100 IU per dose given IM, the plasma testosterone level was unchanged, 100-200 ng/dl. The lack of a response to exogenously administered gonadotropins in our patient suggests that there is a primary testicular lesion causing the hypogonadism. Our patient's own LH is probably biologically active since there was no response to human LH, a pituitary extract with a known biopotency (LER 1549). Our patient's endocrine abnormality is apparently another facet in the spectrum of findings described in patients with Leydig cell hypoplasia or aplasia. Other features of this entity can include female pseudohermaphroditism, ambiguous genitalia, hypogonadism, delayed puberty, and the presence of biologically inactive LH.

Adult↗
PubMed1996

Case report: olfactory function in a fertile eunuch with Kallmann syndrome.

The olfactory and gonadal dysfunction in Kallmann syndrome share a common embryologic pathophysiology. To characterize further the linkage between the hypogonadotropic hypogonadism and anosmia, the authors performed a detailed evaluation of olfactory function in a patient with Kallman Syndrome having the rare variant of partial gonadotropin deficiency (fertile eunuch). The subject was seen initially at age 16 years because of delayed puberty. He received testosterone replacement therapy and subsequently completed pubertal development. As an adult, while untreated, he had subnormal levels of serum testosterone, low gonadotropins, and normal response to luteinizing hormone- releasing hormone. He also had impotence that was reversible with testosterone therapy, and a normal sperm count. Despite the mild degree of hypogonadism, olfactory function was completely absent, and the response to nasal trigeminal stimulants was markedly attenuated. Complete anosmia may therefore be associated with gonadotropin deficiency that is only partial; the presence of anosmia does not predict the need for gonadotropin therapy to attain fertility.

Adolescent↗
PubMed1987

The role of sonography in pediatric gynecology.

Sonography has a major role in the evaluation of children with gynecologic disorders. The size, consistency, and origin of pelvic masses and the status of the internal genitalia can be evaluated. Sonography is useful in evaluation of children with precocious puberty because it can demonstrate adult-size ovaries in true precocious puberty, which distinguishes this from premature thelarche and adrenarche. Specific causes of precocious sexual development such as adrenal and ovarian tumors and cysts also can be diagnosed.

Child↗
PubMed2004

Bone mineral density and bone acquisition in children and young adults with cystic fibrosis: a follow-up study.

OBJECTIVE: To investigate bone mineral density and bone homeostasis in cystic fibrosis (CF) and to assess changes in a 2-year period. METHODS: Thirty-eight patients with clinically stable CF (11 children, 16 adolescents, 11 young adults) were enrolled. No patient was treated with corticosteroids before or during the study. Weight and height Z scores and bone mineral density (BMD) Z-score at the femoral neck and the lumbar spine were recorded at the beginning of the study and after 2 years. Osteocalcin and cross-link excretion, both measurements of bone turnover were also measured. Correlations between BMD, bone turnover parameters, disease severity, pubertal stage, and nutritional state were calculated. The maternal BMD was also determined and related to that of the child. RESULTS: Height and weight Z scores were normal in children and below normal in adolescents. Puberty was delayed in most patients. Bone age was lower than chronological age in adolescents. Lumbar spine and femoral neck BMD Z scores were below normal in each age group. Disease severity determined by Schwachman score correlated with lumbar BMD (r = 0.45, P < 0.02). BMD Z scores did not change during 2 year follow-up. Maternal and patient lumbar and femoral BMD correlated significantly (r = 0.51, P < 0.01, and r = 0.54, P < 0.01, respectively). CONCLUSIONS: Bone deficit is present in patients with CF who have never received steroid treatment. Delay of puberty, chronic inflammation, or genetic susceptibility might be responsible for this phenomenon which was found in patients who had never received steroids and who were in relatively good clinical state.

Absorptiometry, Photon↗
PubMed2005

Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition.

Celiac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten in genetically susceptible individuals. It occurs in children and adolescents with gastrointestinal symptoms, dermatitis herpetiformis, dental enamel defects, osteoporosis, short stature, delayed puberty and persistent iron deficiency anemia and in asymptomatic individuals with type 1 diabetes, Down syndrome, Turner syndrome, Williams syndrome, selective immunoglobulin (Ig)A deficiency and first degree relatives of individuals with celiac disease. The Celiac Disease Guideline Committee of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition has formulated a clinical practice guideline for the diagnosis and treatment of pediatric celiac disease based on an integration of a systematic review of the medical literature combined with expert opinion. The Committee examined the indications for testing, the value of serological tests, human leukocyte antigen (HLA) typing and histopathology and the treatment and monitoring of children with celiac disease. It is recommended that children and adolescents with symptoms of celiac disease or an increased risk for celiac disease have a blood test for antibody to tissue transglutaminase (TTG), that those with an elevated TTG be referred to a pediatric gastroenterologist for an intestinal biopsy and that those with the characteristics of celiac disease on intestinal histopathology be treated with a strict gluten-free diet. This document represents the official recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition on the diagnosis and treatment of celiac disease in children and adolescents.

Adolescent↗
PubMed1998

Growth hormone therapy for non-growth hormone-deficient children with short stature.

A summary of the majority of the available uncontrolled studies of 322 children with idiopathic short stature treated with growth hormone showed that the final height attainment over predicted adult height was only +2.85 cm (+0.49 SD score). Furthermore, a summary of seven studies reported that the spontaneous outcome in children with untreated idiopathic short stature was more than +1 SD score in final height compared to height at presentation; patients with delayed puberty spontaneously gained more than +2 SD score as adults. Recent reevaluations have concluded that short stature is not associated with clinically significant psychologic morbidity, and the psychologic outcome in response to growth hormone treatment of the short normal child showed no discernable difference in psychologic benefit, despite a difference in height gained. A recent editorial has strongly advised against the expanded use of growth hormone in the normal short child.

Adult↗
PubMed1998

Two cases of Townes-Brocks syndrome with previously undescribed anomalies.

Townes-Brocks syndrome is characterized by the association of anorectal, radial ray and outer ear malformations and deafness. We describe two patients affected with several typical clinical signs of Townes-Brocks syndrome in addition to growth and puberty delays and vertebral anomalies not previously reported.

Abnormalities, Multiple↗
PubMed2001

Bone mineral content and density in asymptomatic children with coeliac disease on a gluten-free diet.

OBJECTIVES: Osteoporosis is a complication of coeliac disease. A gluten-free diet improves but does not normalize bone mineral density in adult patients. Only limited data are available regarding the influence of the disease and diet on bone mineralization in children. The aim of this study was to evaluate the radial bone mineral content and density in children and adolescents who are asymptomatic on a gluten-free diet. SUBJECTS AND METHODS: The bone mineral content (BMC) and density (BMD) values of the non-dominant radius midshaft in 91 children (53 girls, 38 boys, mean age 11.7 years, mean duration of disease 8.7 years) were determined by single-photon absorptiometry. At the diagnosis and at least three years after commencement of a gluten-free diet, serum calcium, phosphorus, albumin concentrations and alkaline phosphatase activities were measured in all patients, and intact parathormone concentrations in 16 patients. RESULTS: The mean BMC Z-score value in the female adolescent group only was significantly lower than normal (mean Z-score -1.04, P < 0.01). In contrast, the mean BMD Z-score was significantly higher compared to a healthy population both in girls (mean Z-score +1.36, P < 0.001) and in boys (mean Z-score +0.53, P < 0.02), as well as in the total patient group (mean Z-score +1.01, P < 0.001). The radial diameter was significantly smaller than normal in both pre-pubertal and adolescent groups. Serum laboratory parameters of asymptomatic patients were in the normal range. The parathormone mean value was significantly lower after at least three years of gluten-free diet than at diagnosis (mean +/- SD 3.77 +/- 1.07 versus 7.89 +/- 2.54 pmol/l, P < 0.01), but significantly higher compared to controls (2.89 +/- 0.90 pmol/l, P < 0.05). CONCLUSIONS: These data indicate that treated, asymptomatic coeliac children and adolescents have normal or even higher radius mineral density values than controls, but the bone size remains reduced. Although there is no direct evidence of calcium malabsorption in this cohort of coeliac patients, the slightly higher parathormone levels, together with some other factors, particularly delayed puberty, may result in reduced bone size.

Absorptiometry, Photon↗
PubMed2002

Alcohol suppresses insulin-like growth factor-1 gene expression in prepubertal transgenic female mice overexpressing the bovine growth hormone gene.

BACKGROUND: Alcohol (ALC) delays puberty in female rats and alters the development of a normal menstrual pattern in rhesus monkeys. These actions are associated with depressed serum levels of growth hormone (GH), luteinizing hormone and insulin-like growth factor-1 (IGF-1). The mechanism of this ALC-induced depression in IGF-1 is not known, however, could be due to depressed GH and, possibly, to an alteration in the hepatic GH receptor. To assess whether ALC has a direct action at the liver, we used a transgenic mouse model that overexpresses GH, allowing assessment of potential direct actions of ALC on the level of either the GH receptor or the IGF-1-synthesizing machinery within the hepatocyte. METHODS: One group of transgenic mice was fed a liquid diet containing ALC. The second group was pair-fed the companion isocaloric control liquid diet. The third group of transgenic mice was fed Lab Chow and water. The fourth group consisted of normal (nontransgenic) littermates fed Lab Chow and water. Animals received their respective diets for 5 days. Mice were killed during their late juvenile stage of development, and tissues and blood collected and frozen. RESULTS: The ALC-fed transgenic mice showed a decrease (p < 0.01) in hepatic IGF-1a and IGF-1b messenger RNA levels compared with transgenic controls, and this paralleled a decrease (p < 0.01) in serum IGF-1. ALC did not alter the circulating levels of bovine GH held constant by the promotor and did not alter mouse GH receptor protein levels as analyzed by Western blotting. CONCLUSIONS: Using this transgenic animal model that maintains circulating GH in the presence of ALC, we found that the ability of ALC to suppress prepubertal Igf1 gene expression can also occur independently of any alterations in the level of circulating GH. This direct effect on the hepatocyte is a postreceptor event because the GH receptor protein levels were not altered by ALC exposure.

Aging↗
PubMed2004

Treatment of bone disease in cystic fibrosis.

PURPOSE OF REVIEW: As individuals with cystic fibrosis (CF) have experienced marked improvements in longevity over the last three decades, bone disease has emerged as a new problem. Bone disease in CF has not been previously reviewed in this journal. Therefore, this review will give a brief overview of bone disease in CF and then concentrate on treatment options. RECENT FINDINGS: In some series, as many as three fourths of adults with CF have low bone density. Decreased absorption of fat-soluble vitamins due to pancreatic insufficiency, altered sex hormone production, chronic inflammation, physical inactivity, and glucocorticoid treatment are some of the factors that contribute to this problem. Vitamin D depletion most likely contributes to bone disease, but identifying the safest and most efficacious vitamin D supplementation has yet to be resolved. Calcium and vitamin K supplementations are important if the diet contains less than the recommended amounts. Treatment of delayed puberty and adult hypogonadism with hormone replacement is recommended to achieve peak bone mass and maintain bone density. Bisphosphonates, including pamidronate and alendronate, are beneficial in improving bone mineral density before and after transplantation in CF adults. Bisphosphonates have not been studied in CF children. SUMMARY: Although much progress has been made in our understanding of the pathogenesis, natural history, and clinical manifestations of bone disease in CF, treatment options are still evolving. More attention to nutrition, in terms of the maintenance of lean body mass and vitamin D and calcium supplementation, is likely to decrease bone complications. Bisphosphonates can be of value in CF adults with low bone density. Several clinical trials are under way to help optimize the treatment of CF bone disease.

Adult↗