St Bartholomew's Hospital

About: St Bartholomew's Hospital is a healthcare organization based out in London, United Kingdom. It is known for research contribution in the topics: Population & Cancer. The organization has 11054 authors who have published 13229 publications receiving 501102 citations. The organization is also known as: St. Bartholomew's Hospital & The Royal Hospital of St Bartholomew.

Characterization of Classical and Nonclassical Fabry Disease: A Multicenter Study.

Abstract: Fabry disease leads to renal, cardiac, and cerebrovascular manifestations. Phenotypic differences between classically and nonclassically affected patients are evident, but there are few data on the natural course of classical and nonclassical disease in men and women. To describe the natural course of Fabry disease stratified by sex and phenotype, we retrospectively assessed event-free survival from birth to the first clinical visit (before enzyme replacement therapy) in 499 adult patients (mean age 43 years old; 41% men; 57% with the classical phenotype) from three international centers of excellence. We classified patients by phenotype on the basis of characteristic symptoms and enzyme activity. Men and women with classical Fabry disease had higher event rate than did those with nonclassical disease (hazard ratio for men, 5.63, 95% confidence interval, 3.17 to 10.00; P<0.001; hazard ratio for women, 2.88, 95% confidence interval, 1.54 to 5.40; P<0.001). Furthermore, men with classical Fabry disease had lower eGFR, higher left ventricular mass, and higher plasma globotriaosylsphingosine concentrations than men with nonclassical Fabry disease or women with either phenotype (P<0.001). In conclusion, before treatment with enzyme replacement therapy, men with classical Fabry disease had a history of more events than men with nonclassical disease or women with either phenotype; women with classical Fabry disease were more likely to develop complications than women with nonclassical disease. These data may support the development of new guidelines for the monitoring and treatment of Fabry disease and studies on the effects of intervention in subgroups of patients.

Clinical features and endocrine status in patients with growth hormone insensitivity (Laron syndrome).

Abstract: Twenty-seven patients with GH insensitivity were identified from 44 possible cases, using a scoring system based on height standard deviation score (SDS), basal GH, insulin-like growth factor-I (IGF-I), IGF-I response to IGF-I generation test, and GH-binding protein (GH-BP) determinations. The 27 cases were from 8 European countries and Australia. Clinical features were as follows: age 2.8-22.6 yr; 12 male, 15 female, 19 prepubertal. Birth weight was median -0.72 SDS (1.75(-)-3.29) and birth length, median -1.59 SDS (0.63(-)-3.63). Hypoglycemia had been documented in 33% of the cases, and micropenis was present in 58% of the males. At assessment, height was median -6.1 SDS (-3.8(-)-10.2), weight was median -3.2 SDS (-0.1 to -5.2), and percentage weight for height, median 111.3 (72-271). Puberty was absent in 2 boys aged 15 yr and in 3 girls aged 13 yr. Bone age was delayed in 19 of the 27 patients. Endocrine investigations showed basal serum GH median 17 micrograms/L (0.5-79), IGF-I values less than 5th p...

Possible placental origin of ACTH in normal human pregnancy.

Abstract: IT is well known that the free fraction of plasma cortisol is increased in pregnancy1, but it has not been established whether this is a result of maternal pituitary or placental adrenocorticotrophic hormone (ACTH) secretion. Maternal plasma ACTH levels in human pregnancy have been variously reported as elevated2, or depressed3; but there is no information on the relationship between ACTH levels and the stage of gestation. We now report that maternal ACTH levels increase progressively throughout pregnancy, that urinary free cortisol levels are raised and show resistance to suppression by dexamethasone and we present evidence suggesting that ACTH may be produced by the placenta.

The collagen and elastin content of the arterial wall in the dog.

Abstract: A survey has been made in adult dogs of the collagen and elastin content of the walls of arteries varying in size from the thoracic aorta to the saphenous artery. In all arteries, except the smallest, collagen and elastin together formed about 50% of the dry weight. In the smallest the proportion was somewhat higher. On the basis of relative proportions of elastin and collagen the systemic arterial tree was found to be divided rather sharply into two regions: in the intrathoracic aorta there was about twice as much elastin as collagen in the wall; in all other vessels the relation was reversed, there being about twice as much collagen as elastin. The transition between the more elastic intrathoracic aorta and the more collagenous extrathoracic vessels was abrupt and took place over a distance of only 5 cm or so. The pulmonary artery resembled the extrathoracic vessels rather than the thoracic aorta in ratio of elastin to collagen. Newborn and young puppies showed a similar difference to adults between thoracic and abdominal aorta, although we did not investigate the relation between the two vessels in detail.

Sex-Hormone-Binding Globulin is an Oestrogen Amplifier

Abstract: WE suggest a novel biological role for a specific plasma-binding protein in the regulation of oestrogen and androgen activity in man. The biological activity of oestradiol and testosterone, as with other steroids1, is probably exerted by the unbound fraction in plasma; this is only 1–3% of the total concentration. The plasma proteins that bind testosterone and oestradiol include sex-hormone-binding β globulin (SHBG)2, which has high affinity for both steroids; and albumin, which has low affinity but is present in three thousand times the concentration. Further, corticosteroid-binding globulin (CBG) binds testosterone3 though its main affinity is for cortisol and progesterone.