Assessing radiation protection knowledge in diagnostic radiography in the Republic of Cyprus. A questionnaire survey.

INTRODUCTION: Radiation protection knowledge is essential for medical personnel in ensuring that the possible risks do not outweigh the benefits of diagnostic imaging. This study aimed to assess the radiation protection knowledge of radiographers in Cyprus. METHODS: A cross-sectional study was carried out among radiographers in Cyprus through the Cyprus Society of Registered Radiologic Technologists & Radiation Therapy Technologists. The study was a quantitative descriptive analysis, using a questionnaire with 22 multiple-choice questions. Analysis of the data was done using the statistical software Stata, by performing mean knowledge score comparisons by different participants' characteristics, using t-test and analysis of variance test. Statistical significance was assumed as a p-value < 0.05. RESULTS: The answers provided for each question indicate that some areas of radiation protection are less known compared to others, as there is quite a wide range of correct-to-incorrect ratios. The analysis based on participant characteristics in relation to overall radiation effects knowledge, identified important determinants, namely the workplace of the participant (p = 0.006), the type of work licence the participant held at the time of the questionnaire (p = 0.024), and the years of clinical experience of the participant (p = 0.021). CONCLUSION: The study showed that the levels of knowledge in radiation protection are of a very good standard. However, work is needed to clarify the specifics of dose limits and the national radiation protection legislation with regards to informing patients about the possible effects of ionising radiation. IMPLICATIONS FOR PRACTICE: The study results indicate the importance of educating radiographers about the requirements of national radiation protection legislation and how this new knowledge can be linked with practise.

Medullary thyroid carcinoma surgical cytoreduction induces an increase in serum calcitonin and carcinoembryonic antigen doubling times.

Serum calcitonin (Ct) and carcinoembryonic antigen (CEA) doubling times (DT) are considered to be strong prognostic markers in patients with medullary thyroid carcinoma (MTC). The Objective of this work is to study the effect of MTC debulking on Ct and CEA DTs. 4 patients with MTC are presented who after an initial neck operation had residual disease were followed-up with serial measurements of serum Ct and CEA for several years before and after a secondary incomplete removal of involved cervical lymph nodes. The patients received no other treatment for MTC. Ct and CEA DTs were determined after fitting the Ct or CEA values to an exponential growth equation. In patient A, Ct DT increased from 1.45 years (1.17-1.89, 95% CI) preoperatively, to 5.72 (3.22-25.77) postoperatively. In patient B, Ct DT was 1.63 years (1.36-2.02) preoperatively, and very long (serum Ct practically ceased increasing) postoperatively. In patient C, Ct DT was 4.03 years (2.22-21.58) before, and very long after the operation. In patient D, Ct DT from 1.16 years (0.82-1.99) before, increased to 4.21 years (3.04-6.86) after the operation. The changes in CEA DTs were similar to those of Ct DTs in 2 patients in whom the tumor was apparently producing the protein. In conclusion, surgical MTC cytoreduction in 4 patients caused an increase in the Ct and CEA DTs, and the patients could be reclassified in new Ct DT-based strata with better prognosis than before the operation. We hypothesize that such tumor burden reduction may slow the growth of any residual MTC and we discuss mechanisms that could be responsible for this phenomenon.

Height, whole Body Surface Area, gender, working outdoors, and sunbathing in previous summer are important determinants of serum 25-hydroxyvitamin D levels.

OBJECTIVE: To investigate if serum 25-hydroxyvitamin D (25(OH)D) is related to the whole Body Surface Area (BSA), and to several other anthropometric and environmental factors. MATERIALS/METHODS: Serum 25(OH)D was determined in 489 apparently healthy subjects (346 women and 143 men, mean age 43.9 years) in April and May. From all subjects the following data were available: height, body weight, waist to hip ratio, BSA, BMI, environment of work (indoors vs. outdoors), habit of regularly sunbathing during previous summer(s), fear of sun, dwelling in city or village, and skin color. RESULTS: Approximately 43% of the participants had serum 25(OH)D levels between 10 and 20 ng/ml, 44% had values between 20.1 and 30 ng/ml, whereas about 5% had values below 10 ng/ml and only 9% had values above 30 ng/ml. There was a significant positive relationship between 25(OH)D, height and BSA, which was more pronounced for BSA in obese subjects even after adjustment for work outdoors and sunbathing during previous summer(s). Outdoor workers and sunbathers had higher 25(OH)D compared to indoor workers and non-sunbathers respectively. Men when compared to women had higher 25(OH)D regardless of BMI and this difference was apparently due to the fact that men were taller, had greater BSA, and worked more often outdoors. CONCLUSIONS: Height, whole BSA, gender, working outdoors and sunbathing in previous summer(s) proved to be significant determinants of serum 25(OH)D. Vitamin D status is higher in taller individuals with greater BSA, and in men when compared to women.

Changes in TNM stage, reoperation and 131-I ablation rate during the use of newer methods for the preoperative diagnosis of differentiated thyroid carcinoma.

OBJECTIVE: To define and discuss the changes of important risk factors and TNM staging over the last 40 years in patients operated on for differentiated thyroid carcinoma (DTC), resulting from the introduction of newer sensitive diagnostic procedures in the preoperative evaluation of thyroid nodules. PATIENTS: We reviewed the medical records of 1251 patients with postoperative diagnosis of DTC who had undergone initial diagnosis, before surgery, at our unit, between 1971 and 2010. According to the period of diagnosis, the patients were divided into four groups (I, II, III, IV) corresponding to the four decades. RESULTS: The mean age at diagnosis was unchanged over time for both papillary (PTC) and follicular thyroid cancer (FTC). A decrease in the proportion of FTC (group I vs group IV P < 0·01) and a concomitant increase in PTC/FTC ratio was observed particularly in group IV. The significant decrease in the proportion of tumour size of PTC (group I vs group IV, P < 0·01), the increase in the proportion of microcarcinomas, from 22·4% in group I to 53·0% in group IV, P < 0·001, and the decrease in the number of cases with features of aggressiveness have changed the TNM stage towards stages I and II. The overall frequency of patients at high risk was significantly decreased, from 8·0% in group I to 1·8% in group IV. The number of patients who underwent reoperation for the completion of tumour resection and/or radioiodine therapy significantly decreased over time. CONCLUSIONS: The evaluation of thyroid nodules using the newer diagnostic methods was useful in identifying DTC early. Consequently, the reoperation and thyroid remnant ablation rates were reduced.

Exercise preconditioning reduces neuronal apoptosis in stroke by up-regulating heat shock protein-70 (heat shock protein-72) and extracellular-signal-regulated-kinase 1/2.

Exercise preconditioning induces neuroprotection after stroke. We investigated the beneficial role of heat shock protein-70 (HSP-70) and phosphorylated extracellular-signal-regulated-kinase 1/2 (pERK 1/2), as they pertain to reducing apoptosis and their influence on Bcl-x(L), Bax, and apoptosis-inducing factor (AIF) in rats subjected to ischemia and reperfusion. Adult male Sprague-Dawley rats were subjected to 30 min of exercise on a treadmill for 1, 2, or 3 weeks. Stroke was induced by a 2-h middle cerebral artery (MCA) occlusion using an intraluminal filament. Protein levels of HSP-70, pERK 1/2, Bcl-x(L), Bax, and AIF were analyzed using Western blot. Neuroprotection was based on levels of apoptosis (TUNEL) and infarct volume (Nissl staining). Immunocytochemistry was used for cellular expression of HSP-70 and pERK 1/2. Significant (P<0.05) up-regulation of HSP-70 and pERK 1/2 after 3 weeks of exercise coincided with significant (P<0.05) reduction in neuronal apoptosis and brain infarct volume. Inhibition of either one of these two factors showed a significant (P<0.05) reversal in the neuroprotection. Bax and AIF were down-regulated, while levels of Bcl-x(L) were up-regulated in response to stroke after exercise. Inhibiting HSP-70 or pERK 1/2 reversed this resultant increase or decrease. Our results indicate that exercise diminishes neuronal injury in stroke by up-regulating HSP-70 and ERK 1/2.

Severe vitamin D deficiency in the institutionalized elderly.

Severe vitamin D deficiency has been found to be prevalent in institutionalized elderly persons in several countries. The aim of the present work was to assess the vitamin D status of institutionalized elderly and compare it to that of community-living independent elderly in southern Greece during summer. Serum 25-hydroxyvitamin D [25(OH)D] and plasma PTH were measured in 58 (aged 68-103 yr, median 83.5) elderly inmates of a nursing home (IE) in the town of Kalamata (latitude N 37 degrees ) and in 48 (aged 60-89 yr, median 72) community-dwelling elderly (CDE) in Athens (latitude N 38 degrees ). The CDE had mean serum 25(OH)D 67.6 nmol/l [95% confidence interval (CI) 57.4 to 79.5], not far from the value of 80 nmol/l which is generally considered to be the lower limit of vitamin D sufficiency. The IE had significantly lower mean 25(OH)D 19.0 nmol/l (17.1 to 21.1); values of 25(OH)D below 20 nmol/l characterize severe vitamin D deficiency and may cause osteomalacia. The group of CDE had significantly lower mean plasma PTH 1.5 pmol/l (1.0 to 1.8) compared to 4.5 (3.9 to 5.3) of IE. Ninety percent of CDE had normal plasma PTH whereas 60% of IE had secondary hyperparathyroidism (PTH values >4.0 pmol/l). In conclusion, the majority of institutionalized elderly in southern Greece had severe vitamin D deficiency and secondary hyperparathyroidism in contrast to the fairly good vitamin D status and lack of hyperparathyroidism in the community-living elderly during summer. These findings indicate the need for vitamin D and calcium supplementation of the institutionalized elderly throughout the year.

Heterophilic antibodies causing falsely high serum calcitonin values.

Heterophilic antibodies (HA) may interfere in some immunoassays, causing falsely high hormone values, of which practitioners should be aware when measuring calcitonin (CT) used as tumor marker for medullary thyroid carcinoma (MTC). We studied four patients with thyroid nodules, three of whom underwent surgical neck exploration, after an erroneous diagnosis of MTC because of falsely high serum CT eventually proved to be due to HA. One patient had a lingual thyroid, two autoimmune thyroiditis and the fourth a colloid goiter. The minimal incremental CT response to calcium infusion raised our suspicion of possible false high CT values due to HA. There was no linearity of the CT values obtained by testing serial dilutions of the sera in the CT assay, which employs two monoclonal mouse anti-CT antibodies. Addition of normal mouse gamma globulin eliminated the interference by HA in the sera of two patients. Serum assayed in a polyclonal radioimmunoassay using goat anti-CT antibodies gave normal CT values. Finally, incubation of the sera in Heterophilic Blocking Tubes (HBT) eliminated the false CT immunoreactivity. A spontaneous change of the CT serum concentrations was noticed in three patients over several months, apparently due to changing titles of HA. We suggest that, in patients a) whose CT response to calcium or pentagastrin infusion is minimal despite high basal CT values, b) with autoimmune thyroiditis and c) in whom an unexpected change in serum CT concentrations occurs, the possibility of spuriously high CT values because of circulating HA should be considered.

Urgent thyroidectomy for acute airway obstruction caused by a goiter in a euthyroid pregnant woman.

Pregnancy constitutes a significant factor for thyroid enlargement. However, acute respiratory failure as a result of airway obstruction from an enlarged thyroid gland is an unusual incident. The case presented here concerns a 27-year-old black woman in her 20th gestational week who underwent an urgent operation for removal of a nontoxic, multinodular, mildly substernal goiter that was causing severe upper airway obstruction leading to acute life-threatening respiratory failure. Diagnosis of an extrathoracic tracheal stenosis was based on spirometry with analysis of the flow volume curve and was confirmed by magnetic resonance imaging of the neck. Despite operational risks to the mother as well as the fetus during gestation, an urgent thyroidectomy was carried out successfully. The postoperative period progressed normally and the patient completed her pregnancy with no further respiratory symptoms.

Successful pregnancy in a woman with rare compound heterozygoticity for congenital adrenal hyperplasia; case report.

Pregnancy rates in women with congenital adrenal hyperplasia--even when adequately treated--are reported to be low. We hereby describe our recent experience with the pregnancy of a patient who had congenital adrenal hyperplasia due to a rare combination of P30L/I172N mutations in the CYP21 (21-hydroxylase) gene.

Impaired hypothalamic endocrine function in neuroacanthocytosis.

Two sisters aged 28 and 26, suffering from neuroacanthocytosis (NA), had hypothyroidism and showed a flat growth hormone (GH) response to the insulin tolerance test. GH-releasing hormone (GHRH) administration resulted in a normal GH response in the older sister and a partial GH response in the younger sister. A pathological process--attributable to NA--can be suggested, leading to impaired hypothalamic function, but sparing the pituitary.

Successful treatment with carbimazole of a hyperthyroid pregnancy with hepatic impairment after propylthiouracil administration: a case report.

We report the case of a 27-year-old woman with hyperthyroidism during pregnancy. Antithyroid treatment with propylthiouracil (PTU) resulted in elevated hepatic enzymes and after the 12th week of pregnancy treatment was changed to carbimazole (CBZ). The remaining pregnancy, delivery and follow-up period were uneventful for the mother and her offspring. Antithyroid treatment during pregnancy should allow the use not only of PTU but also of CBZ and methimazole.

Ras oncogenes and p53 tumor suppressor gene analysis in cardiac myxomas.

Although ras oncogenes and p53 tumor suppressor gene mutations are implicated in the development of several human tumors, little is known about their role in the pathogenesis of primary cardiac tumors. Paraffin-embedded tissue from 19 cardiac myxomas were investigated for the presence of ras oncogenes and p53 tumor suppressor gene abnormalities. Immunohistochemical analysis was used to identify the accumulation of p21-ras and p53 proteins. A polymerase chain reaction was used to amplify exons 1 and 2 of the ras genes and exons 5 to 8 of the p53 gene. The PCR products were analyzed by single strand conformation polymorphism analysis and by direct DNA sequencing. Three of 19 myxomas showed strong positive staining for the ras p21 protein. In contrast, nuclear p53 was not detectable in any of the myxomas. Among the ras p21 immunopositive myxomas, 2 were heterozygous for a missense point mutation of the K-ras, Gly 12Asp. Further screening of the remaining myxomas showed no mutation or even silent polymorphism in any exon of the ras and p53. The results suggest that although genetic alterations of ras oncogenes and p53 are uncommon events in cardiac myxomas, ras mutations may be involved in the pathogenesis of a subgroup of this type of tumor.

Hormonal reproductive status of women at menopausal transition compared to that observed in a group of midreproductive-aged women.

OBJECTIVE: To compare the serum levels of follicle stimulating hormone (FSH), luteinizing hormone (LH), prolactin (PRL), estradiol (E2), testosterone (T) and dehydroepiandrosterone sulfate (DHEA-S) in perimenopausal women to the levels of the same reproductive hormones in younger women. METHODS: This was a case control study which compared the hormonal status (circulating levels of FSH, LH, PRL, T, E2, DHEA-S) between women at menopausal transition and younger mid-reproductive-aged controls. The t-test for independent samples was used. RESULTS: FSH, LH and E2 were higher, and T and DHEA-S were lower in perimenopausal women. CONCLUSION: The reproductive hormonal patterns in perimenopausal women favor a relatively hypergonadotropic hyper-estrogenic milieu.

The role of cytokines and cortisol in the non-thyroidal illness syndrome following acute myocardial infarction.

OBJECTIVE: A number of different hormone changes have been described during the acute myocardial infarction (AMI), including those of the non-thyroidal illness syndrome (NTIS). DESIGN AND METHODS: We assessed the alterations of serum thyroid hormones, cytokines and cortisol levels in 30 patients with a first episode of AMI 4, 24, 48h and 10 days (240h) after the onset of the chest pain and we investigated the possible relationship of these alterations with the severity of AMI. RESULTS: Fifteen patients had left ventricular ejection fraction (LVEF) 50% (group II). A transient decrease of total tri-iodothyronine (T(3)), more prominent in group I (P<0.05, t-test) with a concomitant rise of reverse T(3 )(rT(3)) occurred at 24h. Total thyroxine (T(4)), free T(4) (FT(4)) and free T(4) index did not change significantly, but tended to be higher in group I patients, whereas TSH significantly increased in group II at 48h. Interleukin-6 (IL-6) increased significantly at 24h only in group I and declined thereafter (24 vs 240h, P<0.001) and this temporal change of IL-6 was associated with similar changes of creatine phosphokinase and creatine kinase isoenzyme MB (CK-MB). Tumor necrosis factor-alpha and IL-1beta remained low in both groups. Cortisol was higher at 4h and in 12 patients was above the normal values. Negative correlation was found between LVEF and IL-6 (P<0. 001), whereas T(3), T(4) or cortisol levels were not correlated with the LVEF. CONCLUSIONS: Our data indicate that NTIS, in association with increase of IL-6, occurs in the early post-infarction period. In the NTIS following AMI the high level of IL-6 is the best predictor, among several parameters, of the severity of AMI as assessed by the LVEF and the rise of CK-MB.

Changes in parameters of bone and mineral metabolism during therapy for hyperthyroidism.

Hyperthyroid patients have high bone turnover and negative calcium and phosphorus balance often associated with mild osteopenia. Early during antithyroid treatment bone turnover decreases, the mineral balance is converted to positive, and sometimes hypocalcemia occurs. The aim of this investigation was to study the mechanisms of the changes in some parameters of bone and mineral metabolism after treatment of thyrotoxicosis. Thirteen newly diagnosed patients with Graves' disease (seven postmenopausal women, four premenopausal women, and two men) were studied longitudinally, every 6 weeks, for 1 yr after commencing antithyroid treatment with methimazole. Mean serum calcium and phosphorus were both slightly above the normal mean at week 0 and decreased significantly (by 10% and 24%, respectively) during treatment. Fasting urinary calcium was 236 +/- 4 (mean +/- SEM) mg/g creatinine, and the fractional excretion of Ca was 2.0 +/- 0.33% before treatment; both fell significantly to minimums of 61 +/- 20 mg/g and 0.6 +/- 0.16%, respectively. Urinary phosphorus was 282 +/- 60 mg/g creatinine, and the fractional excretion of phosphorus was 3.3 +/- 0.6% before treatment; both increased significantly to 452 +/- 40 mg/g and 8.4 +/- 1.0%, respectively, during treatment. The z-scores were calculated from the mean and SD ofthe respective control groups. The z-score of urinary N-telopeptides of type I collagen (U.NTx) was 9.3 +/- 1.3 at week 0 and declined exponentially, but failed to normalize after 1 yr of antithyroid treatment. The serum alkaline phosphatase (ALP) z-score was initially 2.2 +/- 0.2, increased to 6.0 +/- 1.0 at week 6, and declined slowly there after to 1.0 +/- 1.1 at week 54. The serum osteocalcin (OC) z-score showed a temporal pattern similar to that of ALP. It was initially 2.2 +/- 0.2, increased to 4.0 +/- 0.6 at week 6, and later declined slowly to 0.7 +/- 0.5 at week 54. The failure of the markers of bone turnover to normalize after 1 yr of therapy indicates an on-going high rate of bone turnover despite the attained euthyroidism. The uncoupling index (UI = z-score of U.NTx minus z-score of OC) was 7.1 +/- 1.2 before treatment, indicating unbalanced bone turnover in favor of bone resorption, and fell close to zero at week 30 of treatment. Pretreatment plasma PTH was suppressed slightly to 2.17 +/- 0.47 pmol/L and rose significantly during treatment, reaching a plateau of 5.27 +/- 0.78 at week 12. In all postmenopausal women PTH increased above the upper limit of normal (6.84 pmol/L). Pretreatment serum 25-hydroxyvitamin D was normal and remained unchanged during treatment, whereas 1,25-dihydroxyvitamin D was initially subnormal and rose to normal level after treatment. There was a significant positive linear correlation between PTH and U.NTx after week 12. PTH was also significantly correlated with ALP, but not with OC. ALP and OC were significantly correlated. A significant positive correlation was found between T3 and U.NTx, and a negative correlation was found between T3 and each of the formation markers (ALP and OC) over the 0- to 12-week interval. The latter correlations and the very high pretreatment UI indicate some inhibitory effect of the high thyroid hormone levels on the osteoblasts. The marked and sustained elevation of PTH, more pronounced in the postmenopausal women, during the first year of treatment of hyperthyroidism seems to play a pivotal role in maintaining a relatively high rate of bone turnover despite euthyroidism, and in the conservation of calcium by reducing renal calcium excretion and increasing calcium absorption (via 1,25-dihydroxyvitamin D). It may also account in part for the additional rise of the bone formation markers by an anabolic effect on the osteoblasts. Endogenous PTH may be important in the restoration of bone mineral density of treated hyperthyroid patients.

Germ line mutation analysis in families with multiple endocrine neoplasia type 2A or familial medullary thyroid carcinoma.

The RET proto-oncogene has been identified as the multiple endocrine neoplasia type 2 disease gene. An association between specific RET mutation and disease phenotype has been reported. We present the phenotype-genotype of 12 Greek families with multiple endocrine neoplasia type 2A (MEN 2A) or familial medullary thyroid carcinoma (FMTC). Seventy members were studied and DNA analysis for RET mutations was performed in fifty-eight of them. Exons 10, 11, 13, 14 and 16 of the RET proto-oncogene were analyzed by single strand conformation polymorphism analysis, direct DNA sequencing and/or restriction enzyme analysis. No mutations of the RET proto-oncogene were identified in 1 of 9 families with MEN 2A and in the 3 families with FMTC. In 7 MEN 2A families, the mutation was demonstrated in codon 634 and in 1 family it was demonstrated in codon 620. There was a low frequency, about 8%, of hyperparathyroidism associated with MEN 2A. The specific causative mutations for pararthyroid disease were C634R or C634Y. Among the MEN 2A individuals there was one case with de novo C634R mutation and one case, C634Y, with cutaneous lichen amyloidosis which predated by 24 years the diagnosis of MEN 2A. In 2 children who were MEN 2A gene carriers, microscopic medullary thyroid carcinomas were found. These data show a low frequency of hyperparathyroidism in our cases and provide further evidence that individuals with C634R as well as with C634Y mutations of the RET proto-oncogene could be at risk for parathyroid disease. Cutaneous lichen amyloidosis could be an early feature of MEN 2A. Additionally, direct DNA testing provided an opportunity to resect medullary thyroid carcinoma at an early stage.

Calcitonin levels are similar in goitrous euthyroid patients with or without thyroid antibodies, as well as in hypothyroid patients.

OBJECTIVE: Some authors, after studying retrospectively immunostained sections of thyroid specimens, found C-cell hyperplasia (CCH) unrelated to medullary thyroid carcinoma to be frequent in thyroid glands affected by chronic lymphocytic thyroiditis (CLT); others failed to observe this association. The purpose of the present study was to further investigate prospectively the existence of the proposed relationship between CLT and CCH by measuring circulating calcitonin (CT) levels. DESIGN: Basal serum CT was measured in 174 euthyroid goitrous women with negative thyroid autoantibodies (TA-Neg) and 124 age-matched goitrous euthyroid women with positive antibodies (TA-Pos). A calcium-infusion test was performed in 27 TA-Neg and 31 TA-Pos age-matched euthyroid goitrous women. Basal CT was also determined in 51 hypothyroid women and 40 control normal healthy women. METHODS: Serum CT was measured using a specific and sensitive two-site immunoassay which detects mainly the CT-32 monomer. Thyroid autoantibodies (anti-thyroid peroxidase and anti-thyroglobulin) were measured in serum by quantitative RIAs. RESULTS: Basal serum CT was found to be similar in TA-Neg and TA-Pos euthyroid as well as hypothyroid and normal control groups. A test of independence performed on the frequency distribution of the patients among sequential classes of serum CT showed that basal CT levels are independent of the presence of thyroid autoantibodies or hypothyroidism. Calcium-stimulated CT as well as the increase in CT were similar in TA-Neg and TA-Pos euthyroid goitrous women. There was no correlation between thyrotropin and CT in the hypothyroid group. CONCLUSION: Positive thyroid antibodies in goitrous patients do not predispose to hypercalcitoninemia. Elevated CT found in a patient with goitre and positive thyroid antibodies should not be ascribed to the underlying thyroiditis; this patient should be investigated for some other cause of the high CT.

Circulating levels of immunoreactive parathyroid hormone-related protein and intact parathyroid hormone in human fetuses and newborns.

Undetectable or extremely low levels of circulating immunoreactive parathyroid hormone (PTH) have been reported in human newborns while PTH bioactivity was high. This prompted the hypothesis that the fetal calcemic hormone might be PTH-related protein. The purpose of this study was to measure circulating immunoreactive PTH-related protein in human fetuses and newborns in order to investigate this hypothesis. Parathyroid hormone-related protein (PTHrP(1-86) and intact PTH were measured using two-site immunoradiometric assays in plasma obtained by cordocentesis from 23 fetuses (19-33 weeks of gestation), from 17 newborns at term (38-41 weeks), from their mothers and from 22 normal women of reproductive age. Plasma PTHrP was detectable in all but one of the fetuses and newborns and in all the mothers and the controls. The mean level was similar among fetuses (19-33 weeks) (0.43 +/- 0.18 pmol/l), newborns (0.48 +/- 0.12), mothers (0.48 +/- 0.14) and normal controls (0.46 +/- 0.09). Plasma PTH was found to be significantly higher in fetuses at midgestation (1.0 +/- 0.99 pmol/l) than in the newborns (0.22 +/- 0.21) (p < 0.0025); maternal PTH was significantly higher compared to fetal level at mid-gestation (2.1 +/- 1.0, p < 0.01) as well as at term (2.69 +/- 1.40, p < 0.001). In the control women PTH was 3.07 +/- 1.25 pmol/l. These results showed that plasma amino-terminal PTHrP-(1-86) is detectable during the second half of human fetal life and its level remains unchanged during this period of time, in contrast to changing levels of fetal plasma PTH. The relatively low PTHrP-(1-86) level that we found in the newborns is not responsible for the high PTH-like bioactivity found by some investigators in cord blood at term.

Type II glycogenosis and thyroxine binding globulin deficiency in the same family.

An eight-member family is presented with two female members suffering from the juvenile form of acid maltase deficiency (AMD), the diagnosis confirmed by biochemical study of muscle. Biochemical leucocyte investigation revealed reduced a-glucosidase activity in both patients, a brother and the parents. Endocrinological study of the family disclosed reduced levels of thyroxine binding globulin (TBG) in the father and the three daughters. We consider the co-existence of AMD and TBG deficiency interesting, as thyroxine seems to play a role in the activation of acid maltase.