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Biomedical subjects

F Buzi

Publications and source records attributed to F Buzi.

15 recordsLinked to original sources
PubMed1998

Isolated premature thelarche and thelarche variant: clinical and auxological follow-up of 119 girls.

Age of thelarche presentation, breast evolution, growth and puberty patterns were retrospectively evaluated in 119 girls with premature thelarche (PT). Thelarche spontaneously presented before 2 yr of chronological age in 80% of girls and completely regressed in 60% of them. Breast regression was significantly more frequent in the patients with more precocious thelarche presentation. In 40% of the girls breast size did not significantly change during a follow-up period ranging from 12 to 134 months (40.6 +/- 32.5). Among the 38 girls who were followed up to an age greater than 8 yr, seven (18.4%) developed central precocious puberty (PP). These 7 patients who progressed into true PP were indistinguishable from girls with normal puberty for age of thelarche presentation, breast size, evolution and auxological features. On the contrary, a subgroup of patients (28.5%) with accelerated height velocity and/or bone age at diagnosis of PT showed an auxological pattern different from normally growing girls during a 3-yr follow-up, but they did not seem to have higher risk of progressing into precocious puberty. In conclusion, PT encompasses different clinical, and perhaps hormonal situations, therefore requiring careful clinical follow-up.

Aging↗
PubMed1998

Pelvic ultrasonography in normal girls and in girls with pubertal precocity.

UNLABELLED: This prospective study sought to evaluate the role of pelvic ultrasonography in differentiating between various types of pubertal precocity. A control group of 117 normal girls (aged 1.1-15.6) was studied and compared with 87 girls with premature sexual maturation (aged 1.1-9.2 y). Of these patients 19 had central precocious puberty (CPP), 48 had isolated premature thelarche (IPT) and 20 had premature adrenarche (IPA). Pelvic ultrasound variables evaluated were: (i) uterus: longitudinal diameter (uterine length), cross-sectional area (CSA) and fundo-cervical ratio; and (ii) ovaries: volume and morphology. Ovarian morphology was subdivided in 6 different appearances: solid, microcystic, paucicystic, multicystic, macrocystic, and major isolated cyst. In normal control girls, uterine length and CSA increased with age, although no cut-off values could be defined between different age ranges, and they were correlated with breast stage; fundo-cervical ratio was stable through childhood and increased after age 9. Ovarian volume was significantly greater in pubertal girls with breast stage 2 than in those with only pubic and/or axillary hair. There was a clear predominance of solid ovarian appearances in the age range 2-7, with the multicystic appearance being seen only after age 7, a minority being macrocystic. After age 10 all the different patterns were observed, and after age 13 the frequency of a macrocystic pattern increased. Significantly more mature ovarian appearances were observed in subjects with breast development compared with those without, independently of the presence of pubic hair. Patients with IPT had no significant differences in pelvic ultrasound measurements when compared with age-matched controls. All the different morphological ovarian appearances were observed in IPT, in contrast to age-matched controls, where only the less mature patterns (solid, micro- and paucicystic) were seen. Patients with CPP had significantly more mature patterns of ovarian morphology compared with age-matched controls, but did not differ from pubertal pre-menarcheal controls. Those patients with IPA differed from age-matched controls only in having significantly greater uterine length and CSA. Comparison of the pelvic ultrasound parameters between patient groups (IPT, CPP, IPA) and age-matched controls revealed significantly higher values in CPP for uterine length, uterine CSA and ovarian volume. Ovarian volume was also greater in IPT than in IPA. Ovarian morphology was significantly different in patients (IPT, CPP, IPA) compared with age-matched controls, but none of the ovarian morphological appearances was exclusive to a single condition. IN CONCLUSION: (i) pelvic ultrasound parameters increase progressively from birth to maturity, but no clear cut-off values can be established between age ranges; (ii) pelvic ultrasound variables reach adult values during puberty, with differences in the timing that may reflect geographical variations; (iii) the multicystic ovarian appearance occurs just before the onset of puberty; (iv) pelvic ultrasonography cannot always differentiate clearly between different disturbances of puberty and therefore cannot supersede other observations and investigations in the evaluation of pubertal disorders; and (v) in this study we propose a more detailed pelvic ultrasound terminology that can avoid apparent confusion in defining ovarian ultrasound appearance.

Analysis of Variance↗
PubMed1998

Growth, insulin-like growth factor I (IGF-I), and IGF-binding proteins 1 and 3 in children with severe liver disease before and after liver transplantation: a longitudinal and cross-sectional study.

We aimed to study the growth and growth factors of children with liver disease before and after liver transplantation (LT). Three observation intervals: 1) before LT (preLT), 2) after LT on daily prednisone treatment (dP), and 3) on alternate day prednisone (adP). A longitudinal study (LS) involved 17 infants (9 male) aged 0.73-2.38 y at LT; mean (+/- SD) height (Ht) SD score (SDS) at LT was -2.02 (+/- 1.25). In a cross-sectional study, there were 123 children (73 male) aged 0.16-14.88 y (mean 3.72 y). IGF-I and IGF binding proteins (BP) 1 and 3 were measured at the same intervals. The results were, for LS, preLT height velocity (HV) SDS (X +/- SD -0.8+/-1.4) lower (p < 0.01) than adP-HV SDS (3.1+/-1.8) but not different from dP-HV SDS (-1.0+/-1.9). For the cross-sectional study, dP-Ht SDS (X+/-SD -1.94+/-1.31) lower (p < 0.001) than preLT-Ht SDS (-1.03+/-1.06) and adP-Ht SDS (-0.98+/-1.20). Parental target SDS was not different from adP-Ht SDS. (Similar observations were made in the LS.) The dP- sitting height (SH) and subischial leg length (SLL) SDS were significantly lower than both preLT- and adP-SH SDS and SLL SDS (p = 0.02 and 0.002, respectively). There was a significant improvement of head circumference SDS and arm circumference SDS from preLT to adP. The dP and adP IGF-I and IGF-BP3 levels were greater than preLT levels (p < 0.001); no differences were found between preLT, dP, and adP IGF-BP1 levels. We conclude that growth in children with liver disease does not improve after LT on dP, but catch-up growth is shown on adP, appearing to depend mainly on the clinical course and corticosteroid regimen. IGF-I and IGF-BP3 increment on dP (and sustained on adP) is possibly due to liver regeneration, in contrast with inhibition of body growth on dP, possibly due to central and peripheral effects of corticosteroid.

Adolescent↗
PubMed1997

Loperamide test: a simple and highly specific screening test for hypercortisolism in children and adolescents.

The overnight dexamethasone (DXM) test can give false-positive results in a few conditions (e.g. stress, strenuous exercise, depression, anorexia, anxiety, anticonvulsive therapy) in diagnosing simple obesity and hypercortisolism (HC). The loperamide (LP; a peripheral opioid agonist) test has proven useful in such conditions in adults. Thirty-one obese subjects (age 10.0-19.7 y) were studied by both overnight DXM test and LP test (8 mg orally, samples for cortisol at 0, 90, 150, 180 and 210 min) on 2 separate days. LP suppressed cortisol (< or = 138 nmol l-1) at a dose of 0.1 mg kg-1 bw (half the minimum recommended dose for the drug's antidiarrhoea effect) in 14 subjects who had normal urinary (< 4970 nmol l-1) and serum (< 552 nmol l-1) cortisol, in the absence of signs and symptoms of HC (group A). The DXM test failed to suppress cortisol in three subjects in group A, two of whom were on anticonvulsive treatment. The LP test suppressed cortisol in all of 13 subjects with elevated urinary and/or serum cortisol and/or with signs or symptoms of HC (but in whom HC was subsequently excluded on clinical grounds) (group B), while the DXM test failed to suppress cortisol in three subjects of this group. One of these was under anticonvulsive treatment and one suffered from anxiety and depression. In four patients with Cushing's syndrome (group C) neither DXM nor LP could suppress cortisol levels. Therefore, the sensitivity was 100% for both DXM and LP, while the specificity was 84% for DXM and 100% for LP. No side-effects were observed with either drug. In conclusion, LP is a useful alternative to DXM in those particular conditions that can affect its specificity in children.

Adolescent↗
PubMed1997

[Graves' disease].

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Antithyroid Agents↗
PubMed1996

[Precocious puberty].

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Antineoplastic Agents, Hormonal↗
PubMed1994

X-linked agammaglobulinemia, growth hormone deficiency and delay of growth and puberty.

Coinheritance of X-linked agammaglobulinemia and growth hormone deficiency (XLA/GHD) has been classified as an independent primary immune deficiency. We evaluated the pattern of growth and endocrine function in seven XLA subjects (ages 10.9-20.1 years); four belonged to two different XLA pedigrees and three represented sporadic XLA cases. Three had reached adulthood (final stature 176.0, 173.5 and 165.0 cm, respectively) and their retrospective growth showed delay in growth and puberty during adolescence. In the other four subjects, growth hormone production was measured by growth hormone pharmacological stimulation tests (clonidine, arginine): three of four patients had insufficient growth hormone responses (peak growth hormone < 10 micrograms/l); all three had delayed puberty; their growth hormone responses increased after "priming" with testosterone, reaching values > 10 micrograms/l in two of them and allowing diagnosis of "true" growth hormone deficiency in the third. The fourth was a normally growing subject who showed a normal growth hormone response both before and after testosterone priming. Six out of the seven subjects showed a growth pattern consistent with delay in growth and puberty. Our results suggest that true XLA/GHD is rarer than previously supposed and that subnormal responses to growth hormone stimulation tests may be found without sex steroid priming of the test in adolescence. The most probable growth pattern in XLA appears to be delay in growth and puberty, as has already been described for other chronic diseases.

Adult↗
PubMed1993

Overnight growth hormone secretion in short children: independence of the sleep pattern.

PURPOSE: to analyze the interrelationships between GH secretion and pattern of sleep. PATIENTS: 18 children (10 male, 8 female; mean age 9.1 yr, range 5.1-14.3 yr), with short stature (mean height standard deviation score (SDS) -2.52, range -3.86-(-)1.88; mean height velocity SDS -1.1, range -2.40-(-)0.08), including 9 children with genetic short stature and 9 with idiopathic short stature. METHODS: blood samples were taken every 15 min from 2000 h-0800 h, and GH profiles were analyzed by the PULSAR computerized peak identification algorithm; simultaneous sleep was analyzed by electroencephalogram recording. RESULTS: no significant correlation was noted between GH secretion parameters and any of the electroencephalogram parameters evaluated: stage 1 (S1) percent, stage 2 (S2) percent, slow-wave sleep (SWS) percent, rapid eye movement sleep percent, wakefulness (W) percent, and sleep efficiency (EFF); there was no significant difference in GH secretion between children with EFF less than 76% and those with EFF more than 76% (P > 0.5). Maximal GH peak coincided 9 times (50%) with SWS, 3 times (17%) with S2, 3 times with W, twice (11%) with S1, and once (6%) with rapid eye movement sleep. First GH peak coincided 12 times (67%) with W, 3 times with S2, twice with SWS, and once with S1. There was no significant difference comparing the percentage of sleep stages occurring in the 15 min of maximal GH increment, in the 15 min preceding it, and in those following it; there was no significant difference comparing the percentage of sleep stages occurring in the 15 min preceding the onset of a GH peak and in those following it. CONCLUSIONS: GH secretion in short children seems independent of the sleep stage and efficiency; in children it is possible that GH secretion relates with sleep per se and with neurohormonal changes occurring at nighttime rather than with a specific sleep stage or sleep stage sequence.

Adolescent↗
PubMed1992

Carrier detection in X-linked adrenoleukodystrophy by determination of very long chain fatty acid levels and by linkage analysis.

Diagnosis of X-linked adrenoleukodystrophy is based upon demonstration of high levels of very long chain fatty acids. More recently, in addition to biochemical analysis, closely linked DNA probe St14 has been used for prenatal diagnosis in informative families. Identification of heterozygotes is particularly important, both in order to specifically address only carrier females to prenatal diagnosis, and because appropriate dietary therapy is now available to treat those heterozygotes presenting with neurological symptoms. We report two pedigrees in which carrier detection was performed by a combination of biochemical and molecular genetic analysis. Such approach should allow extremely high accuracy in carrier detection.

Adrenoleukodystrophy↗
PubMed1991

Effects of pyridostigmine on spontaneous and growth hormone-releasing hormone stimulated growth hormone secretion in children on daily glucocorticoid therapy after liver transplantation.

OBJECTIVES: We aimed to investigate both nocturnal spontaneous and morning growth hormone (GH)-releasing hormone (GHRH)-induced GH secretion in children on daily glucocorticoid treatment after liver transplantation and to evaluate the effect of pyridostigmine (an acetylcholinesterase inhibitor thought to reduce hypothalamic somatostatin tone) on GH secretion in these patients. DESIGN: We performed a randomized, single-blind, cross-over study. PATIENTS: We studied three male and three female juvenile patients, within a year of orthotopic liver transplantation and under immunosuppressive glucocorticoid therapy (mean dose +/- SEM, 5.92 +/- 0.63 mg/day) and five normal children (four males, one female). MEASUREMENTS: Both nocturnal spontaneous and morning GHRH-induced GH secretion were evaluated after administration of placebo, 1 tablet p.o., or pyridostigmine, 2 mg/kg p.o. RESULTS: Spontaneous GH. Placebo: in liver transplanted children nocturnal GH secretion (mean GH level 10.8 +/- 2.0 mU/l) was not significantly different with respect to normal children (mean GH level 12.8 +/- 1.2 mU/l); pyridostigmine: nocturnal GH secretion was significantly increased as compared to placebo in subjects with liver transplantation but not in normal children. GHRH test. Placebo: liver transplanted patients showed a blunted GH response to GHRH with respect to normal children; pyridostigmine: the GH responses to GHRH (P less than 0.05) increased as compared to placebo and did not differ significantly in the two groups. CONCLUSIONS: Our data suggest a steroid-mediated increase in hypothalamic somatostatin tone in liver transplanted children.

Child↗
PubMed1989

Antigenicity and efficacy of authentic sequence recombinant human growth hormone (somatropin): first-year experience in the United Kingdom.

Twenty-one children were treated for GH deficiency with authentic sequence biosynthetic human GH (somatropin), 60 micrograms/kg body weight, subcutaneously, three times weekly for 1 year or longer. The magnitude of growth response and rise in serum insulin-like growth factor I levels were similar to those expected from experience with pituitary GH and somatrem. Three patients developed serum antibodies to GH with a binding capacity greater than 0.02 mg/l, but in only one patient was the GH binding capacity greater than 1.0 mg/l and he showed no attenuation of growth response. Escherichia coli polypeptide antibodies did not rise significantly and no clinically important side-effects occurred. Somatropin is safe, effective and of low immunogenicity in the treatment of GH deficiency.

Adolescent↗
PubMed1987

The effect of cyproterone acetate on the growth of children with central precocious puberty.

We have examined the growth and skeletal maturation of 19 children (6 male, 13 female) with central precocious puberty. The aetiology in nine patients (5 male, 4 female) was secondary to a hypothalamic hamartoma. Six children (2 male, 4 female) received no treatment whereas 13 children (4 male, 9 female) were treated with cyproterone acetate in a mean dose of 68 mg/m2 per day (range, 34-260) for a mean duration of 4.5 years (range, 0.8-7.9). There was no significant difference between height SDS for bone age at the beginning and end of observation in either treated or untreated groups. No significant relationship between the mean dose of cyproterone acetate used and change in height SDS for bone age could be determined. We conclude that cyproterone acetate has no beneficial effect on the growth prognosis of children with central precocious puberty.

Body Height↗
PubMed1987

Physiological growth hormone secretion during slow-wave sleep in short prepubertal children.

The usefulness of a limited nocturnal GH profile has been evaluated for the assessment of physiological GH secretion. We have analysed the complete overnight GH and sleep profiles of 20 short prepubertal children, sampled at 15-min intervals. The mean age was 9.1 years (range 4.3-11.7 years) and mean height velocity standard deviation score (SDS)-1.0 (range -2.4 to +0.4). The sleep stage in which the maximal GH value was reached varied considerably between the patients. Only 55% achieved the maximal GH value during the first slow-wave sleep period, and 65% in the first 2 h of sleep. The maximal values during the first slow-wave period correlated weakly with the sum of the nocturnal peak values and the total area under the curve of the complete GH profile. None of these parameters correlated with height velocity. We conclude that at present there is no substitute for complete overnight or 24 h GH profiles for the assessment of physiological GH secretion, but the clinical significance of the variations seen remains unclear.

Body Height↗
PubMedDate not supplied

Pseudohypoaldosteronism: report of a case presenting as failure to thrive.

We report a 2 month-old infant referred for failure to thrive. At birth, weight was 3820 g and length 52 cm. After physiologic weight loss, the patient showed no further weight gain for the next two months. On admittance (age 2 mo), weight was 3340 g and length 53 cm; the infant had severe dystrophy, generalized hypotonia and dehydration; blood chemistry showed hyponatremia, hyperkalemia and hypochloremia. A salt losing syndrome of adrenal origin was hypothesized. However, rehydration and hydrocortisone administration failed to correct hyponatremia and hyperkalemia. Endocrine assessment showed high levels of aldosterone and plasma renin activity, suggesting pseudohypoaldosteronism. Oral sodium chloride supplementation normalized electrolyte balance and the patient showed progressive weight gain and catch-up growth, confirming the diagnosis.

Administration, Oral↗