[Hirsutism and hypertrichosis].
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Adolescent↗
Biomedical subjects
M Salerno
Publications and source records attributed to M Salerno.
[Adolescents with chronic pathology: body image].
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Acquired Immunodeficiency Syndrome↗
Short and long-term impact of a structured educational program on the patient's knowledge of hypertension.
BACKGROUND: It has been generally accepted that educational programs can be beneficial in the treatment of a number of chronic diseases such as diabetes mellitus as well as of cardiovascular risk factors and hypertension. We organized a structured educational intervention aimed at 1) assessing the patient's baseline knowledge of hypertension, 2) verifying the short and long-term impact of this initiative on patient's education. METHODS: We invited 174 consecutive patients referred to our hypertension outpatient clinic to participate in an educational meeting on hypertension. For organizational reasons, patients were divided into two groups, each attending a single meeting. Each meeting included four sessions: 1) in the first session a multiple choice questionnaire (nine questions, with answers collected by an interactive electronic system) was administered in order to evaluate patients' baseline knowledge of hypertension, 2) a traditional teaching session, 3) an interactive phase aimed at assessing the improvement of knowledge in which the same questions as in the first session were assessed again, 4) a general discussion session. At the end of the meeting a booklet on principal issues related to hypertension was given to each patient. In order to evaluate the long-term impact of this initiative on the patient's knowledge, 6 months later we invited the patients to answer to the same questions in a questionnaire sent to their home address. RESULTS: One hundred thirty-three patients of the 174 invited attended the meeting and 111 (57 males, 54 females, mean age 53 +/- 13 years) completed the questionnaire after 6 months. The answers to the questions in the initial session were correct in a percentage ranging from 60 to 80% (mean 68%) and immediately after the teaching session this rate increased significantly (range 75-98%, mean 90%, p < 0.05 at least, in all questions). A similar good level of knowledge was maintained in the long term (percentage of exact answers ranging from 78 to 97%, mean 88%, p < 0.05 or p < 0.01 compared to baseline). CONCLUSIONS: The findings of the present study show a positive short and long-term impact of a structured educational intervention on the patient's knowledge of issues related to arterial hypertension. The beneficial role on clinical outcomes such as blood pressure control and cardiovascular events will need future controlled trials.
Adult↗
SHC-alpha5beta1 integrin interactions regulate breast cancer cell adhesion and motility.
The oncogenic SHC proteins are signaling substrates for most receptor and cytoplasmic tyrosine kinases (TKs) and have been implicated in cellular growth, transformation, and differentiation. In tumor cells overexpressing TKs, the levels of tyrosine phosphorylated SHC are chronically elevated. The significance of amplified SHC signaling in breast tumorigenesis and metastasis remains unknown. Here we demonstrate that seven- to ninefold overexpression of SHC significantly altered interactions of cells with fibronectin (FN). Specifically, in human breast cancer cells overexpressing SHC (MCF-7/SHC) the association of SHC with alpha5beta1 integrin (FN receptor) was increased, spreading on FN was accelerated, and basal growth on FN was reduced. These effects coincided with an early decline of adhesion-dependent MAP kinase activity. Basal motility of MCF-7/SHC cells on FN was inhibited relative to that in several cell lines with normal SHC levels. However, when EGF or IGF-I was used as the chemoattractant, the locomotion of MCF-7/SHC cells was greatly (approx fivefold) stimulated, while it was only minimally altered in the control cells. These data suggest that SHC is a mediator of the dynamic regulation of cell adhesion and motility on FN in breast cancer cells.
Adaptor Proteins, Signal Transducing↗
Insulin receptor substrate 1 is a target for the pure antiestrogen ICI 182,780 in breast cancer cells.
The pure antiestrogen ICI 182,780 inhibits insulin-like growth factor (IGF)-dependent proliferation in hormone-responsive breast cancer cells. However, the interactions of ICI 182,780 with IGF-I receptor (IGF-IR) intracellular signaling have not been characterized. Here, we studied the effects of ICI 182,780 on IGF-IR signal transduction in MCF-7 breast cancer cells and in MCF-7-derived clones overexpressing either the IGF-IR or its 2 major substrates, insulin receptor substrate 1 (IRS-1) or src/collagen homology proteins (SHC). ICI 182,780 blocked the basal and IGF-I-induced growth in all studied cells in a dose-dependent manner; however, the clones with the greatest IRS-1 overexpression were clearly least sensitive to the drug. Pursuing ICI 182,780 interaction with IRS-1, we found that the antiestrogen reduced IRS-1 expression and tyrosine phosphorylation in several cell lines in the presence or absence of IGF-I. Moreover, in IRS-1-overexpressing cells, ICI 182,780 decreased IRS-1/p85 and IRS-1/GRB2 binding. The effects of ICI 182,780 on IGF-IR protein expression were not significant; however, the drug suppressed IGF-I-induced (but not basal) IGF-IR tyrosine phosphorylation. The expression and tyrosine phosphorylation of SHC as well as SHC/GRB binding were not influenced by ICI 182,780. In summary, downregulation of IRS-1 may represent one of the mechanisms by which ICI 182,780 inhibits the growth of breast cancer cells. Thus, overexpression of IRS-1 in breast tumors could contribute to the development of antiestrogen resistance.
Breast Neoplasms↗
Allocation of inner cell mass and trophectoderm cells to the preimplantation blastocyst of the domestic ferret, Mustela putorius furo.
The growth of ferret preimplantation blastocysts in vivo, collected between 156 and 240 hr post coitum, was investigated. A technique, combining immunosurgery and differential fluorochrome staining, was used to discriminate between inner cell mass (ICM) and trophectoderm (TE) cells. Using the stains propidium iodide and bisbenzimide (Hoechst 33342), the ICM was stained blue and the TE was stained pink. The ICM and TE counts for 90 blastocysts, respectively, averaged 25 and 63 at 156 hr and increased exponentially to 2077 and 4137 at 240 hr. The Box-Cox procedure was used for choosing a transformation that minimized the error sum of squares for a linear regression of Y (cell count) on X (time in hr). Logarithmic transformations of the ICM, TE and total cell count gave a good fit, but the following equations obtained by the Box-Cox procedure provided the best fit, where Y is cell count and X is time in hours. For inner cell mass: Y = [(176.06 + 2.45X)/-899.44 + 1]-3.33; trophectoderm: Y = [(301.38 + 14.48X)/-6863.42 + 1]-10; and total: Y = [(2266.97 + 17.0X)/-7837.21 + 1]-5. The R2 values were 0.73, 0.84, and 0.84, respectively. The exponential growth of the ferret embryo during the time interval that measurements were made fits the general pattern described for other mammalian embryos. This report is the first to characterize the pattern of cell allocation and growth in preimplantation blastocysts of the ferret, and the first such report for a carnivore. The pattern of in vivo development provides a standard for judging the quality of in vitro produced and matured ferret embryos and, concomitantly, a means to evaluate culture systems.
Animals↗
Liver abnormalities in Turner syndrome.
UNLABELLED: We evaluated whether hepatic abnormalities represent a specific feature in girls with Turner syndrome (TS) or whether they are related to an increased susceptibility to hormonal therapies and/or other factors. Alanine aminotransferase, aspartate aminotransferase and gamma-glutamyl transferase were monitored in 70 patients with TS for a mean period of 7.6+/-4.2 years. An increase in serum liver enzymes was observed in 14 out of 70 girls (20%) at a mean age of 12.7 years; it was present at entry before hormonal therapy in 3 girls and developed thereafter during the follow up in the other 11. The increase in serum liver enzymes was never observed before the age of 7 years. In the majority of cases (10/14) it was drug related: in 50% the liver abnormalities were transient and self-limiting, in the remaining cases they required interruption of hormonal therapy. Hepatotoxicity was more frequently observed in girls treated with oestrogens or oxandrolone than in those treated with growth hormone. In a small number of cases, liver disease was either auto-immunity-related (2/14), or cryptogenic (1/14) with a benign and self-limiting course. Obesity was a frequent finding, but it played a likely pivotal role only in one patient. CONCLUSION: Hepatic abnormalities are relatively frequent in Turner syndrome and surveillance of liver function should be included in the management of these patients independent of initiation of hormonal treatment.
Alanine Transaminase↗
Long-term zinc and iron supplementation in children of short stature: effect of growth and on trace element content in tissues.
We evaluated the effect of one year of supplementation with iron plus zinc (12 mg/day of Fe+++ and 12.5 mg/day of Zn++), zinc alone (12.5 mg/day of Zn++) and placebo on growth and on the iron, zinc, copper and selenium tissue contents in 30 well-selected children of short stature (16 M and 14 F; 4-11 years old). Before and after supplementation, we measured the concentrations of iron, transferrin, ferritin, zinc and copper in serum, of zinc in erythrocytes and leukocytes, and of zinc, copper and selenium in hair, as well as glutathione peroxidase activity in erythrocytes. Before supplementation, ferritin and serum, erythrocyte and hair zinc contents were significantly lower than in age-matched controls, while the other measured indices were in the normal range. Iron plus zinc supplementation caused an improvement in growth rate in all subjects, i.e., the median Z-score increased from -2.22 +/- 0.45 to -0.64 +/- 0.55; (p < 0.01). In the zinc-supplemented group, only the subjects whose ferritin levels were higher than 20 ng/L before supplementation showed a similar improvement of growth rate. Iron plus zinc supplementation could be a reasonable treatment in short, prepubertal children affected by marginal zinc and iron deficiency.
Age Determination by Skeleton↗
Blood pressure control in a hypertension hospital clinic.
OBJECTIVES: First, to evaluate the prevalence of clinic blood pressure (BP) control (BP < or = 140/90 mm Hg) in a representative sample of treated hypertensive patients followed in our hypertension clinic. Second, to assess in a subgroup of these patients: (a) the proportion of BP control with both clinic blood pressure (CBP < or =140/90 mm Hg) and daytime ambulatory blood pressure (ABP) (< or =132/85 mm Hg) criteria, and (b) the prevalence of echocardiographic left ventricular hypertrophy (LVH) (left ventricular mass index, LVMI>125 g/m2 in men and >110 g/m2 in women). DESIGN AND METHODS: Seven hundred consecutive hypertensive patients who attended our hypertension centre clinic during a period of 6 months and who had regularly been followed up by the same medical team were included in the study. BP was taken in the clinic by a doctor using a mercury sphygmomanometer with the participants seated. Seventy-four patients with similar demographic and clinical characteristics to the entire population of participants underwent complete echocardiographic examination and 24 h ABP monitoring. RESULTS: During follow-up, 352 of the treated patients had clinic BP < or =140/90 mm Hg, 198< or =160/95 mm Hg and 150>160/95 mm Hg, indicating that BP control was satisfactory in 50.3%, borderline in 28.3% and unsatisfactory in 21.4% of the cases. In the subgroup of 74 patients, the proportion of individuals with satisfactory clinic BP control (CBP< or =140/90 mm Hg) was higher (50.0 versus 33.6%) than with satisfactory ABP control (daytime ABP values < or =132/85 mm Hg). LVH was found in 21 of the 74 patients (28.3%): 12 of them had unsatisfactory CBP control and 19 had unsatisfactory ABP control. LVMI did not correlate with CBP values but only with ABP values (mean 24 h systolic r = 0.47, diastolic r = 0.40, P<0.001; mean daytime systolic r = 0.45, mean daytime diastolic r = 0.39, P<0.001; mean night-time systolic r = 0.38, mean night-time diastolic r = 0.38, P<0.001). CONCLUSION: This study demonstrates that hypertensive patients managed in a hypertension centre clinic have satisfactory CBP control in 50% of cases, but this rate seems to over-estimate the effective BP control during daily life. A large fraction of patients show persistence of LVH and this evidence of organ damage almost entirely concerns individuals with poor ABP control.
Ambulatory Care Facilities↗
Intellectual outcome at 12 years of age in congenital hypothyroidism.
BACKGROUND: The intellectual outcome in children with congenital hypothyroidism detected by neonatal screening is generally good; however, subtle neurological dysfunctions, subnormal IQ, or both, have been reported. OBJECTIVE: To evaluate the intellectual outcome in 12-year-old patients with congenital hypothyroidism, detected by neonatal screening, in an attempt to identify factors that may affect intellectual development. METHODS: The intelligence quotient (IQ) of 40 children with congenital hypothyroidism was evaluated at 12 years of age, using the Wechsler Intelligence Scale for Children -- Revised, and compared with the IQ of 40 healthy siblings (control group). RESULTS: The mean IQ score (88.4+/-13.1) was not significantly different from that of the control group (93.4+/-10.7). Thirteen patients showed subnormal IQ score (72.4+/-4.9) compared with their siblings (86.7+/-9.6; P<0.0001) and with the other patients (96.1+/-9.6; P<0.0001). The low IQ score was associated with lower serum concentrations of thyroxine at diagnosis, poor treatment compliance during follow-up and lower familial IQ. Interviews with parents of children with congenital hypothyroidism revealed that a refusal to acknowledge the disease was linked to poor attention to the child's emotional life and to poor treatment compliance in some cases (11%). CONCLUSION: Even though the mean IQ score in patients with congenital hypothyroidism falls within normal for the control population, low IQ scores may be present in patients with severe hypothyroidism, inadequate compliance to replacement therapy during follow-up and poor parental pedagogic attitude.
Adaptation, Psychological↗
Treatment of hypopituitarism in infancy. Effect on head circumference growth.
BACKGROUND: Head circumference of children with multiple pituitary-hormone deficiency (MPHD) may be subnormal for age. Moreover it is known that linear growth in infancy is growth-hormone (GH) dependent. Therefore, aim of the study has been to compare head circumference measurements in children with hypopituitarism after L-thyroxine (L-T4) therapy alone, before therapy with GH, and after GH was added to the therapy. METHODS: Five infants (2 girls, 3 boys) with MPHD, diagnosed and treated before the age of 2 years and whose auxological parameters records during L-T4 therapy alone were available, were retrospectively studied. Head circumference and length measurements were expressed as standard deviation score (SDS). Weight measurements were expressed as weight for length ratio percentage. RESULTS: Initially treated with L-T4 alone for a mean period of 4, 5 months, there were neither positive effects on head circumference nor on linear growth. A significant catch-up growth was observed only employing GH therapy in addition to L-T4: mean head circumference SDS and length SDS increased respectively from -2.20 to -0.89 SDS and from -4.16 to -0.87 SDS after a mean period of 18 months of combined GH and L-T4 therapy. CONCLUSIONS: Therefore, head circumference growth, in infancy, is growth hormone dependent as well as linear growth, and during GH treatment, monitoring head circumference growth is important as much as monitoring linear growth.
Adolescent↗
Role of IRS-1 signaling in insulin-induced modulation of estrogen receptors in breast cancer cells.
Cross-talk between steroid hormones and polypeptide growth factors regulates the growth of hormone-responsive breast cancer cells. For example, in the MCF-7 human breast cancer cell line, insulin up-regulates estrogen receptor (ER) content and binding capacity. Since the insulin receptor (IR) substrate 1 (IRS-1) is one of the core signaling elements transmitting mitogenic and metabolic effects of insulin, we investigated whether IRS-1 is also required for the insulin-induced function of the ER. The effects of insulin on the ER were compared in MCF-7 cells and MCF-7-derived cell lines with decreased levels (by approximately 80%) of IRS-1 due to the expression of IRS-1 antisense RNA. The severe IRS-1 deficiency in MCF-7 cells was associated with (1) reduced mitogenic response to 20 ng/ml insulin and 10% calf serum (CS), but not to 1 nM estradiol (E2); (2) loss of insulin-E2 synergism; (3) up-regulation of ER protein expression and binding capacity; and (4) loss of insulin-induced regulation of ER tyrosine phosphorylation. In conclusion, the data confirm the existence of the IR-ER cross-talk and suggest that IRS-1-dependent signaling may contribute to the negative regulation of the ER expression and function in MCF-7 cells.
Breast Neoplasms↗
Interactions with single-stranded and double-stranded DNA-binding factors and alternative promoter conformation upon transcriptional activation of the Htf9-a/RanBP1 and Htf9-c genes.
The murine Htf9-a/RanBP1 and Htf9-c genes are divergently transcribed from a shared TATA-less promoter. Transcription of both genes is initiated on complementary DNA strands and is controlled by cell cycle-dependent mechanisms. The bidirectional promoter harbors a genomic footprint flanking the major transcription start site of both genes. Transient promoter assays showed that the footprinted element is important for transcription of both genes. Protein-binding experiments and antibody assays indicated that members of the retinoid X receptor family interact with the double-stranded site. In addition, distinct factors interact with single DNA strands of the element. Double-stranded binding factors were highly expressed in liver cells, in which neither gene is transcribed, while single-stranded binding proteins were abundant in cycling cells, in which transcription of both genes is efficient. In vivo S1 analysis of the promoter depicted an S1-sensitive organization in cells in which transcription of both genes is active; S1 sensitivity was not detected in conditions of transcriptional repression. Thus, the same element is a target for either retinoid X receptor factors, or for single-stranded binding proteins, and form distinct complexes in different cellular conditions depending on the DNA conformation in the binding site.
3T3 Cells↗
Fetal goiter in a iodine-deficient area.
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Female↗
Central precocious puberty: a single blood sample after gonadotropin-releasing hormone agonist administration in monitoring treatment.
This study was designed to evaluate whether a single blood sample drawn after the home injection of a long-acting gonadotropin-releasing hormone (GnRH) agonist (GnRHa) in patients treated for central precocious puberty (CPP) could be a more simple and inexpensive test with respect to the conventional GnRH stimulating test in assessing adequate suppression of the pituitary-gonadal axis. The response to the first therapeutic injection of the GnRHa triptorelin was studied in 14 newly diagnosed untreated females with CPP. The results were compared with the response that the same patients had to the conventional GnRH stimulation test performed at the time of diagnosis. A significant increase in LH, FSH and E2 levels was observed 12 h after the triptorelin intramuscular injection; serum peak values of LH (70.3 +/- 58.5 IU/l), FSH (44.2 +/- 21.7 IU/l) and E2 (489.7 +/- 263.9 pmol/l) were significantly greater than those obtained with the conventional GnRH test (LH 31.4 +/- 21.7, p = 0.002; FSH 19.8 +/- 10. 7, p = 0.001; E2 83.3 +/- 25, p < 0.001). In particular, the E2 response, 12 h after triptorelin injection, was clearly consistent with gonadal activation compared to the modest E2 increase in response to the GnRH test. Thereafter 22 girls who were already being treated with triptorelin for CPP were evaluated to see whether a single blood sample drawn 12 h after the therapeutic home injection of GnRHa could be informative in assessing adequate suppression of the pituitary-gonadal axis. This response was also compared to the conventional GnRH stimulation test performed 2 days before the therapeutic triptorelin injection. In 7 girls with evidence of pubertal progression, the E2 response following the GnRHa injection (136.3 +/- 44.4 pmol/l) was significantly higher with respect to the response after the GnRH stimulation test (73.0 +/- 0.0; p < 0.02) indicating an inadequate suppression of the pituitary-gonadal axis. The present data suggest that a single blood sample drawn 12 h after the therapeutic home administration of triptorelin provides a simple, comfortable and inexpensive means of monitoring pituitary as well as gonadal function in girls treated for CPP.
Child↗
Expression of growth hormone receptor by peripheral blood lymphocytes in children: evaluation in clinical conditions of impaired growth.
OBJECTIVE: It is widely accepted that the haematopoietic system is a target of growth hormone action and that GH may act as a lymphokine. The expression of GH receptors (GHR) on human peripheral blood lymphocytes (PBL) has been reported previously in adult donors by dual fluorochrome flow cytometry. The aim of this study was to apply the cytofluorimetric method to the analysis of GHR expression on PBL in various human conditions characterized by different patterns of growth due to age or physiopathological conditions. SUBJECTS AND DESIGN: PBL from 38 normal (control) subjects (7 newborns, 18 prepubertal children, 13 adults) were studied in order to provide age-related physiological data. Twenty-two short children (18 with idiopathic short stature, 4 with Ullrich-Turner syndrome) were studied to determine the expression of GHR in conditions of impaired longitudinal growth which may or may not require GH treatment. METHODS: Analysis was performed using a fluorescein isothiocyanate (FITC)-conjugated antibody specific for the GHR (mAb263) and phycoerythrin (PE)-anti CD2 (T and natural killer cells) or PE-anti CD2 (B cells) in dual fluorochrome flow cytometric assays. Results were expressed as mean fluorescent intensity (MFI). RESULTS: Adult CD2+ coils exhibited a significantly higher GHR expression (MFI 347 +/- 40) than that expressed in children and newborns (MFI 285 +/- 36 and 299 +/- 41, respectively, P < 0.001). A significantly increased expression of GHR on CD2+ cells was also found in short children (MFI 330 +/- 42 vs 285 42- 36, respectively; P < 0.002), whereas Ullrich-Turner syndrome patients did not show any difference from their age and gender matched controls (254 +/- 52 and 288 +/- 40, respectively). A negative relationship was found between GHR expression on CD2+ cells and height-SDS (r - 0.54, P < 0.0001) or BMI (r - 0.4, P < 0.015) in controls and short children, independent of their GH secretory status. Expression of GHR and CD20+ cells was higher than that expressed on CD2+ cells in all subjects. No appreciable differences were found in the MFI levels of GHR expression on CD20+ cells either among the different age group controls or between short children or Ullrich-Turner syndrome patients. A significant downregulation of expression was shown in CD20+ (P < 0.008) but not CD2+ cells after 6 months of GH treatment in 6 short children who had a poor response to GH provocative tests. CONCLUSIONS: GH receptor expression on immune cells in non-syndromic short children appears to be inversely related to the linear growth expression and BMI of the subjects, contrary to findings with hepatic derived serum GHBP. This finding may reflect alternate exon usage in lymphoid cells, and indicates that GH has a distinctive role in the immune system.
Adult↗
A time course study on the "in vitro" effects of T3 and testosterone on androgen and estrogen receptors in peripuberal primary rat Sertoli cells.
The effect of Tri-iodothyronine (T3) administration leading to the precocius differentiation of Sertoli cell in prepuberal rats has been previously shown. The functional maturation of Sertoli cells is associated with changes in androgen metabolism. We have recently demonstrated that T3 influences androgen metabolism in Sertoli cells by inhibiting aromatase activity and reduces drastically the ER contents in peripubertal hypothyroid rats. To better understand the role of T3 in modulating steroid action on Sertoli cells, we performed a time course study evaluating the in vitro effects of T3 and testosterone (T) on androgen (ARs) and estrogen (ERs) receptor content in Sertoli cells isolated from two weeks old Wistar rats. ARs and ERs basal levels did not change during the time course study indicating that the exposure to culture medium per se did not affect either receptor type. After 24 hrs of incubation with either T3 or T, a decrease of ERs in both nucleus and cytosol was observed. Such a decrease was augmented by the simultaneous administration of both hormones. ARs displayed a different temporal pattern in the two cellular compartments and exhibited an earlier rise in the cytosol induced by either T3 or T. At 36 hrs, ARs were significantly enhanced in both compartments in response to either T or T3 exposure while combined hormonal treatment caused an additive increase compared with the single treatment group. As a consequence of the opposite behaviour pattern displayed by ARs and ERs, the ratio between total ARs and ERs contents was increased after 24 hrs of exposure to hormonal treatment. To evaluate if treatments performed induced a functional maturation of Sertoli cells, transferrin levels in culture medium were measured. The increase of this protein paralleled that of ARs content as well as that of ARs/ERs ratio. This study demonstrates that thyroid hormone induces a progressive increase of (AR)/(ER) ratio in the differentiating Sertoli cells bringing them to a prevalent androgen dependency along their functional maturation.
Animals↗
Growth pattern during the first 36 months of life in congenital adrenal hyperplasia (21-hydroxylase deficiency).
The longitudinal growth pattern during the first 36 months of life was studied in 24 patients (17 females) with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency by analyzing the mean required daily dose of cortisone with respect to steroid suppression, height and weight growth velocities and bone age maturation. All patients were treated with cortisone acetate and 9-fluorohydrocortisone. The standard deviation score for length (SDS-L), the percentage of ideal body weight (% IBW) and biochemical parameters, 17-hydroxy-progesterone (17-OHP) and androstenedione (A) were evaluated every 3 months; bone age (BA) was evaluated annually. At diagnosis, the female population of patients with respect to the males were younger (chronological age (CA): 15 +/- 14 vs. 45 +/- 16 days, p < 0.005) and had a higher % IBW (91.7 +/- 8.0 vs. 76.3 +/- 16.7%, p < 0.05). At 3 months of age (45 days after initiating treatment) the % IBW in males normalized (97 +/- 19%) and was similar to that found in females (101 +/- 12.8%). No differences were noted in SDS-L at the moment of diagnosis (females-1.1 +/- 1.1 vs. males -0.5 +/- 0.7); however, at 3 months of age the SDS-L in females increased (0.41 +/- 0.88, p < 0.005 vs. diagnosis SDS-L) whereas that of males progressively decreased to reach the nadir at 6 months (-1.41 +/- 0.96). No differences between males and females were noted throughout this time with regard to: (a) A or 17-OHP levels (neither of which were suppressed to 'control values'); (b) the dosage of cortisone received (13.5-17.8 mg/m2/day), and (c) change in BA/CA ratio. In all patients the SDS-target height (TH) correlated with the SDS-L at 2 years (r = 0.74, p < 0.0005) and at 3 years (r = 0.60, p < 0.02) of age. In 12 patients who reached 7 years of age the SDS-L correlated with both SDS-predicted adult height (PAH) (r = 0.75, p < 0.001) and SDS-TH (r = 0.80, p < 0.005). Although the commonly accepted definition of "good control' for patients with CAH has generally included, in addition to adequate suppression of hormone markers, normal growth and skeletal maturation, the present data suggest that normal growth and BA maturation are the most useful parameters to follow and not necessarily strive for hormone suppression. Early diagnosis and replacement therapy using cortisone doses less than those currently recommended allow normal growth within the genetic potential at least for the first 7 years of life.
17-alpha-Hydroxyprogesterone↗