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Biomedical subjects

A Tenore

Publications and source records attributed to A Tenore.

At least 19 recordsLinked to original sources

Prognostic factors in the intellectual development at 7 years of age in children with congenital hypothyroidism.

The influence of the initial L-T4 dose and some other factors on the intellectual development was evaluated at 7 years of age in 47 congenitally hypothyroid children detected by the regional screening program. All patients were initially treated with 25 micrograms LT4/kg/day that represented a mean replacement dose of 6.8 +/- 1.3 micrograms/kg. Despite the "low" initial dose mean IQ at 7 years resulted within normal range (96 +/- 9). Twenty-eight patients initially treated with 6.0 +/- 0.6 micrograms L-T4/kg/day had a mean IQ (96 +/- 9) which was not different with respect to 19 patients (IQ 94 +/- 7) treated with a significantly higher L-T4 dose (8.1 +/- 0.9 micrograms/kg/day; p < 0.0001). The initial L-T4, dose did not correlate with IQ at 7 years whereas a significant correlation was found between IQ and serum T4 concentration at diagnosis (r = 0.35; p < 0.01) regardless of the fact that serum T4 concentration normalized after the first 2 months of therapy in both groups. Twenty-three patients whose serum T4 at diagnosis was < 2 micrograms/dl (1.0 +/- 0.5) had a mean IQ at 7 years (92 +/- 9) which was significantly lower than the 24 patients (IQ 98 +/- 7; p < 0.02) whose serum T4 was > 2 micrograms/dl (5.7 +/- 2.4; p < 0.001). The present findings suggest that the severity of neonatal hypothyroidism is an important factor in determining subsequent intellectual development of congenitally hypothyroid children.

Child

Periodontal status and selected cultivable anaerobic microflora of insulin-dependent juvenile diabetics.

The periodontal status and subgingival microflora of insulin-dependent juvenile diabetic (JD) patients (n = 16, mean age = 11.3) were compared with that of their non-diabetic cohabiting healthy siblings (HS, n = 16, mean age = 13.2). JD patients were monitored every 3 months for levels of glycosylated hemoglobin (HbA1c) and clinical and microbial parameters were measured 6 weeks before drawing blood for levels of HbA1c (M% = 8.76). Clinical indices, measured for the entire permanent dentition, included: probing depth (PD), attachment level (AL), sulcus bleeding index (SBI), and plaque index (PI). Subgingival plaque samples were obtained at 2 sites from each subject; whenever possible, the site with the deepest probing depth and the mesial aspect of the maxillary right first molar were used. Microbial analyses were determined by cultural characteristics and biochemical tests. No significant differences were detected in any of the clinical indices for the entire dentition. The mean AL for JD sites was 2.32 +/- 0.83 mm and for HS sites was 2.2 +/- 0.85 mm. Mean percentage of total cultivable anaerobic microflora included Capnocytophaga spp. (JD, 13.21%; HS, 11%) and Porphyromonas gingivalis (JD, 5.1%; HS, 7.9%). Differences between the two groups were not statistically significant. When cluster analysis was performed on sampled sites, one cluster group in JD patients showed significantly elevated P. gingivalis and lower Capnocytophaga spp. levels as compared to the overall mean. The clinical parameters of this cluster were characterized by statistically significant greater loss of attachment and probing depth. These data would suggest few differences between JD patients and their HS in this population.

Adolescent

Impairment of T-cell growth-promoting lymphokines in human insulin-dependent diabetes mellitus.

T-cell growth factor (TCGF) activity was studied in phytohemagglutinin (PHA)-stimulated peripheral blood mononuclear cells (PBMC) from 10 type-1 diabetic patients who had been diagnosed within the previous 12 months (group A), from 9 diabetic patients in whom the duration of disease was more than 1 year (group B) and from 12 healthy controls (group C). The effects of indomethacin on PHA-induced TCGF activity and the effects of adherent cells (macrophages) from group A and group C on TCGF production of normal group-matched non-adherent cells (lymphocytes) were also studied. TCGF activity was assayed on TCGF-dependent blast cells and calculated as a stimulation index (SI). TCGF activity in group A (SI 0.86 +/- 0.8) was significantly different from that in group B (SI 1.75 +/- 1.02; P = 0.037) and in group C (SI 1.91 +/- 1.29; P = 0.023). Following the addition of indomethacin, TCGF SI was 1.35 +/- 0.74 in group A, 1.85 +/- 0.73 in group B and 2.06 +/- 1.19 in group C. The responses to indomethacin were found to correlate with the basal TCGF activity in all subjects (r = -0.48; P = 0.006) independently of the disease process studied or its duration. No correlation was found between TCGF activity and parameters of metabolic control (HBA1c and fructosamine). Interestingly, a significant inverse correlation was found between TCGF activity and the required dose of insulin only in group A (r = -0.66; P < 0.05). Adherent cells from diabetic patients were found not to inhibit TCGF production.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Differential expression of surface membrane growth hormone receptor on human peripheral blood lymphocytes detected by dual fluorochrome flow cytometry.

Although several reports indicate proliferative and functional effects of human GH (hGH) on peripheral blood lymphocytes (PBL), no information is available about hGH receptor (GHR) expression in PBL subsets. Here, the surface membrane GHR levels were investigated in different human PBL subpopulations using a fluorescein isothiocyanate (FITC)-conjugated monoclonal antibody specific for the GHR (mAb263) in dual fluorochrome flow cytometric assays. Strong GHR expression was found in B-cells (CD20+), whereas CD2+ lymphocytes, including T-cells as well as natural killer cells, exhibited considerably lower levels of receptor expression. Similarly, using FITC-labeled recombinant hGH, receptor expression on CD20+ cells was significantly higher than that on CD2+ cells. Abundant expression of GHR in B-lymphocytes was confirmed by reverse transcriptase-polymerase chain reaction analysis of GHR messenger ribonucleic acid from isolated B-cells. Accordingly, the B-cell merits greater consideration as a GH target cell. The use of FITC-labeled mAb263 and hGH is of potential use for the study of GHR levels in patients exhibiting different types of growth disorders. Because of its high specificity for GHR, FITC-labeled mAb263 is also of considerable value for specifically demonstrating the presence of GHR, because hGH may interact with and act through PRL receptor, as shown previously in human neutrophils.

Antibodies, Monoclonal

[L-T4 malabsorption determined by intolerance to cow's milk proteins and celiac disease in a patient with congenital hypothyroidism. A clinical case].

An infant with congenital hypothyroidism (CH) is described who in the course of thyroxin replacement therapy developed cow's milk protein intolerance (CMPI) and subsequently coeliac disease (CD). The presence of these two pathologies interfered with the intestinal absorption of L-thyroxin (L-T4) and made the appropriate management of CH difficult. A male patient, formula fed from birth, was screened for CH at 5 days of age. CH was confirmed and L-T4 treatment (6.8 micrograms/kg/day per os) begun at 35 days of age. Seen after 3 weeks because of feeding problems and impaired growth and with a serum T4 concentration of 4.7 micrograms/dl and a TSH of 56 mU/ml, his replacement dose was increased to 12 micrograms/kg/day. One week later, the child was hospitalized because of vomiting, acidosis and shock. CMPI was diagnosed and the child began a diet with a hydrolyzed milk protein food. After 7 weeks the serum T4 improved to 11 micrograms/dl, the TSH decreased to 10 mU/ml, while maintaining the 12 micrograms/kg dose. After 2 months an oral challenge with cow's milk confirmed the diagnosis of CMPI with a concomitant decrease in serum T4 to 6.4 micrograms/dl, while TSH rose to 64 mU/ml. Weight and T4 levels normalised with CMPI diet. At 12 months of age the patient presented anorexia and impaired growth.(ABSTRACT TRUNCATED AT 250 WORDS)

Celiac Disease

Prognostic signs in the evolution of premature thelarche by discriminant analysis.

Since premature thelarche (PT) can be a first sign of precocious puberty (PP), the aim of our study was to identify simple items in the course of the first 6 months of follow-up that could help predict if PT would evolve to PP. Thirty-two girls with PT were studied. First evaluation included bone age (BA), basal estradiol, FSH, LH and prolactin. GnRH was performed in 15 subjects and BA was checked at 6 month intervals in 30. Based on clinical outcome after a mean follow-up of 33.4 +/- 16.5 (SD) months, patients were divided into 2 groups: Group I (G-I) included subjects whose breast development either remained unchanged, increased or regressed; Group II (G-II) included subjects who progressed to PP. The multivariate combination of the items which was able to best discriminate between the two groups was chosen in predicting the evolution of PT. The items considered included four variables available at the time of diagnosis [chronological (CA) at onset less than 3 years, basal FSH, basal LH and BA/CA ratio] and two additional variables after a 6-month follow-up (delta BA/delta CA and growth velocity); 88% of G-I and 14% of G-II had CA less than 3 yr. Basal FSH levels were elevated in both G-I (7.6 +/- 3.0 mIU/ml) and G-II (12.1 +/- 4.1) with respect to controls (2.6 +/- 1.2); however, approximately 20% of G-I had low FSH levels. Basal LH levels were consistently higher in G-II (8.0 +/- 1.3 mIU/ml) than in G-I (2.9 +/- 1.5) or controls (2.8 +/- 1.2). Although initial BA was advanced (greater than 2SD) in 21% of G-I and in all of G-II, an acceleration of BA was seen only in G-II. The mean growth velocity of G-I (44.1 +/- 31.5%) was significantly less than G-II (92 +/- 32%; p less than 0.0025). With the help of the discriminant equations derived from data obtained at diagnosis and during the first 6 months of follow-up, all subjects with isolated premature thelarche could be sharply distinguished from those who subsequently progressed to precocious puberty.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

X-linked ichthyosis, due to steroid sulphatase deficiency, associated with Kallmann syndrome (hypogonadotropic hypogonadism and anosmia): linkage relationships with Xg and cloned DNA sequences from the distal short arm of the X chromosome.

We report a large Italian pedigree in which five out of six males are affected by a syndrome, following an X-linked inheritance pattern, characterized by ichthyosis, hypogonadotropic hypogonadism, and anosmia. The concurrence of features of X-linked ichthyosis (XLI) with those of Kallmann syndrome, another disease often inherited as an X-linked trait, prompted us to perform biochemical, cytogenetic, and molecular studies in relation to the short arm of the X chromosome (Xp). Steroid sulphatase (STS) activity was found to be completely deficient in all affected members of the family. Prometaphase chromosome analyses of two obligate heterozygous women and one affected male showed normal karyotypes. Xg blood group antigen analysis and molecular studies employing cloned DNA sequences from the distal segment of the Xp (probes RC8, 782, dic56, and M1A), did not provide evidence for deletions or rearrangements of the X chromosome. The linkage analysis showed no crossovers between the disease, Xg, and DXS143, the locus defined by probe dic56, thus suggesting the possibility of a linkage between these two markers of the distal segment of Xp and the X-linked ichthyosis, hypogonadism, and anosmia syndrome.

Adolescent

Congenital giant diverticulum of the posterior urethra in a 4-year-old boy.

A congenital giant diverticulum of the posterior urethra in a 4-year-old boy is reported. The theories on development of congenital urethral diverticula are considered and the importance of the clinical, radiological, endoscopical and histological examination for the differential diagnosis of the acquired diverticula and enlarged utricle is emphasised. In the majority of diverticula transurethral unroofing is most appropriate. The larger and symptomatic diverticula require open excision and eventually urethral reconstruction. Small asymptomatic diverticula may not require any treatment.

Child, Preschool

Vasculitis, pulmonary cavitation, and anemia during antithyroid drug therapy.

Two thyrotoxic girls, aged 12 and 16 years, had a clinical picture consisting of purpuric skin rash, nephritis, pulmonary cavitation, hemoptysis, and profound anemia during antithyroid drug therapy. These major toxic manifestations prompted discontinuation of antithyroid drugs and institution of antibiotic and glucocorticoid therapy before the resolution of the severe illness. The girls eventually required ablative doses of radioactive iodine, and one of them had a thyroidectomy for the treatment of thyrotoxicosis. The skin biopsy specimen in one patient was indicative of mononuclear cell cutaneous vasculitis. After recovery, the patients had no evidence Of persistent anemia, nephritis, or pulmonary parenchymal disease.

Adolescent

Separation and radioimmunoassay of T3 and T4 in human breast milk.

There is little agreement among published reports of the radioimmunoassayable thyroid hormone content of breast milk, likely due to wide variations in methodology applied. In order to achieve a higher degree of specificity in the determination of T3 and T4 concentrations in breast milk, samples were ethanol-extracted and then chromatographed on an LH-20 column. Using this method, all T3 and T4 RIA activity eluted with the void volume. Following pancreatin digestion and subsequent extraction of whole milk samples, void volume T3 RIA activity decreased, and T3 co-eluted primarily with a standard preparation of T3 or 125I-T3, at a concentration of 275 +/- 132 ng/dl (mean +/- SD) (n = 9). In contrast, the elution volume of T4 RIA activity appeared unaffected by pancreatin. These data indicate that immunoreactive T3 and T4 are differentially bound to a thyroid hormone 'binding' substance present in breast milk. They further support the hypothesis that thyroid hormone sufficient to supplement the thyroid economy of the thyroid-deficient suckling infant is present in human breast milk.

Adult

A thyroxine-binding protein in human breast milk similar to serum thyroxine-binding globulin.

A thyroid hormone-binding substance (TBGw) similar to serum T4-binding globulin (TBG) has been identified in the whey fraction of human breast milk. TBGw coeluted with serum TBG, as determined by Bio-Gel P-100 chromatography, and has an isoelectric point of 4.2-4.8, similar to that of serum TBG. The affinity constant of TBGw for T4 was similar to that of serum TBG(Ka, 1.54 +/- 0.38 X 10(9) M-1). Marked inhibition of TBGw binding of [125I]T4 was achieved by the addition of 1.5 X 10(-3) M 8-anilino-1-naphthalene-sulfonic acid. An albumin-like low affinity site (Ka, greater than 10(7) M-1) was also found. RIA of whey concentrates serially diluted in TBG-depleted serum indicated nearly identical binding curves for TBGw and TBG, with slopes of 2.33 (n = 15; r = 0.947) and 2.54 (n = 7; r = 0.996), respectively. Using a specific TBG RIA, a TBGw concentration of 0.29 +/- 0.08 microgram/ml (mean +/- SD) in breast milk (n = 26) was determined. Paired serum and whey specimens from individuals between 6 and 20 weeks of lactation were analyzed by TBG RIA; in these individuals, mean serum TBG concentrations were 25.0 +/- 3.4 micrograms/ml (n = 7); corresponding TBGw levels were 0.26 +/- 0.08 micrograms/ml (n = 7), i.e. approximately 1% of serum levels. Nonetheless, linear regression analysis of the data revealed no significant correlation between serum and whey TBG concentrations in these individuals.

Albumins