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S Lamberts

Publications and source records attributed to S Lamberts.

4 recordsLinked to original sources

Consensus statement: medical management of acromegaly.

In November 2003, the Pituitary Society and the European Neuroendocrine Association sponsored a consensus workshop in Seville to address challenging issues in the medical management of acromegaly. Participants comprised 70 endocrinologists and neurosurgeons with international expertise in managing patients with acromegaly. All participants participated in the workshop proceedings, and the final document written by the scientific committee reflects the consensus opinion of the interactive deliberations. The meeting was supported by an unrestricted educational grant from Ipsen. No pharmaceutical representatives participated in the program planning or in the scientific deliberations.

Acromegaly↗

In vitro and in vivo detection of somatostatin receptors in pheochromocytomas and paragangliomas.

Fifty-one adrenal pheochromocytomas and 14 paragangliomas were evaluated for somatostatin (SRIH) receptor content with in vitro autoradiography on tissue sections from surgically removed tumors, using iodinated 125I[Tyr]3 octreotide as radioligand. Thirty-seven of 51 pheochromocytomas were SRIH receptor positive (73%), as well as 13 of 14 paragangliomas (93%). These SRIH receptors were of high affinity, pharmacologically specific for SRIH and localized on the tumor tissue. Using in vivo imaging techniques with radiolabeled SRIH analogs, paragangliomas could be visualized in five patients, as well as pheochromocytomas in two of three patients. All tumors tested subsequently in vitro (n = 7) were shown to contain SRIH receptors. A majority of pheochromocytomas were also shown to have a high tumoral SRIH content as measured by immunohistochemical techniques. Detection of SRIH messenger RNA in pheochromocytomas by in situ hybridization indicated that the SRIH was produced in the tumors. A weak inverse correlation was observed between SRIH receptor status and tumoral SRIH content, suggesting that SRIH receptors may be down-regulated by high levels of endogenous SRIH in some tumors. There was no correlation between the SRIH receptor status and sex, age, tumor size, benign vs. malignant tumor, or urinary metanephrine excretion. These tumors were also analyzed for allelic losses on various chromosomes and showed significant loss of heterozygosity (LOH) on chromosomes 1p, 3p, 17p, and 22q. All eight tumors with LOH on chromosome 1p were SRIH receptor positive (100%), whereas only 6 of 11 tumors without LOH on 1p (55%) were SRIH receptor positive, suggesting a correlation between LOH on 1p and SRIH receptor positive status. SRIH receptors thus represent a consistent pathobiochemical marker for most of these adrenal and extra-adrenal tumors. In addition, these receptors may be of potential interest for the in vivo localization of these tumors.

Adrenal Gland Neoplasms↗

Patients with congenital dopamine beta-hydroxylase deficiency. A lesson in catecholamine physiology.

We recently described a case of congenital dopamine beta-hydroxylase (DBH) deficiency. The syndrome is characterized by noradrenergic denervation, adrenomedullary failure, but intact baroreflex afferents, cholinergic innervation, and adrenocortical function. Norepinephrine, epinephrine, and their degradation products were undetectable in plasma, urine, and cerebrospinal fluid, whereas dopamine and its degradation products were elevated. Plasma DBH was not detectable. Studies in this novel syndrome showed evidence for the peripheral production of dopamine from sympathetic nerve terminals noradrenergic in nature. Tyrosine hydroxylase is probably induced in this syndrome, since plasma levels of L-DOPA were also elevated. Absence of hemodynamic effects of sympathicolytic agents in the face of an increase in blood pressure after dopamine antagonists suggest that intrasynaptic concentrations of dopamine are in the range of its plasma concentrations. Hypoprolactinemia, reduced REM sleep, increased slow wave sleep and sodium loss, despite low blood pressure, are further evidence for the biological role of dopamine in man.

Adult↗