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Biomedical subjects

J Vendrell

Publications and source records attributed to J Vendrell.

At least 91 records · Page 5Linked to original sources

[Evaluation of the treatment of Cushing's syndrome using ketoconazole].

The effectiveness of ketoconazole for the therapy of Cushing's syndrome, both of adrenal and pituitary origin, was retrospectively evaluated. To this end, the clinical and hormonal results in 5 patients were assessed. There were 4 females and one male, with ages ranging between 17 and 64 years. Three of them, who had Cushing's disease, received sustained treatment with ketoconazole when other therapeutic measures had failed. The two remaining patients (one with Cushing's disease and the other with adrenal adenoma) were treated with this drug in the preoperative period to alleviate the deterioration in the clinical condition caused by hypercortisolism. The clinical evolution of the patients is described, as well as the plasma adrenocorticotropin (ACTH) and the free cortisol in 24 hour urine in the subsequent controls. In view of the study results, we feel that ketoconazole is an effective treatment for the long term control of Cushing's syndrome. In addition, it may be also useful as preoperative therapy in those patients severely deteriorated by the sustained hypercortisolism.

Adolescent↗

The separation of pancreatic procarboxypeptidases by high-performance liquid chromatography and chromatofocusing.

Different experimental conditions and chromatographic supports have been selected for the most efficient and rapid purification of procarboxypeptidases from porcine and human pancreas by different high-performance liquid chromatography (HPLC) variants (anion exchange, reversed phase and gel filtration). Anion-exchange chromatography was found to be the most capable and permitted the isolation, in a single step, of three different porcine procarboxypeptidases (2A + 1B forms) and five different human procarboxypeptidases (2B + 3A forms) in a native and pure state from whole pancreas extracts. Other pancreatic proproteases are also cleanly isolated in the same step. Reversed-phase chromatography under mild conditions separated porcine or human procarboxypeptidases A from other pancreatic proteins in a very short time but was unable further to subfractionate the same proteins. The sequential use of gel filtration (or anion-exchange) and reversed-phase HPLC chromatography permitted, in a simple way, the isolation and dissociation of the strongly bound components of the binary complexes between procarboxypeptidases A and proproteinase E in either porcine or human pancreas extracts. Chromatofocusing on a fast protein liquid chromatographic support was also found to be a very efficient technique, showing a slightly lower capability to separate procarboxypeptidases than anion-exchange HPLC though in a much shorter time and in larger quantities.

Animals↗

[Cushing's disease in a patient with primary empty sella turcica].

Primary empty sella is a neuroanatomical condition which is more common in middle aged obese multiparous females with long-standing hypertension. Usually there are no symptoms, but occasionally nonspecific headache may be present. Hormone studies are commonly normal in these patients, although several functional hypothalamic and pituitary abnormalities have been reported. We report a 64-year-old female with primary empty sella in whom Cushing's disease was diagnosed. The association of both disorders is exceptional.

Cushing Syndrome↗

Late-onset hypocalcemia appearing years after thyroid surgery.

We revised four cases of hypocalcemia diagnosed yr after thyroid surgery, including 1 man and 3 women, operated 5-23 yr before the onset of symptoms, which ranged from mild paresthesia to convulsive seizures. Total serum calcium levels ranged from 1.1 to 2.05 mmol/l, and PTH levels were low in the 3 cases in which they were measured. Hypoparathyroidism appearing as late-onset hypocalcemia after extense thyroid surgery is a poorly understood condition, perhaps not as unfrequent as it is considered.

Adult↗

Procarboxypeptidase A activation segment compared to structures of other proteins.

The 94-residue activation segment of procarboxypeptidase A was compared with segments of other proteins. No significant homologies were observed towards other activation segments, but an inter-domain segment preceding the serine-protease part of complement factor B showed some structural relationships with the N-terminal region of the procarboxypeptidase A activation segment. This may reflect common functional and organizational patterns. In contrast, the present comparisons do not give functional or further sequence support to previously proposed structural homologies with helix-loop-helix (EF-hand) calcium-binding proteins.

Amino Acid Sequence↗

Analysis of the conformation and ligand-binding properties of the activation segment of pig procarboxypeptidase A.

The isolated activation segment of pig procarboxypeptidase A binds two Tb3+ ions in a strong and specific way. In contrast, the binding of Ca2+, Cd2+ and Mg2+ is weak. The binding of Tb3+ increases the resistance of the isolated activation segment against proteolysis and competes for the binding of the carbocyanine dye Stains-All. This dye forms complexes with the activation segment showing spectral properties similar to those observed with EF-hand structures. The presented results support a previous hypothesis on the existence of two regions in the activation segment of pancreatic procarboxypeptidases structurally related to Ca2+-binding domains of the EF-hand protein family.

Animals↗

A case with persistent nephrogenic diabetes insipidus following parathyroidectomy for hyperparathyroidism.

A 25-year-old woman with persistent nephrogenic diabetes insipidus (NDI) following parathyroidectomy for primary hyperparathyroidism is described. NDI is a well recognized complication of primary hyperparathyroidism, generally imputed to hypercalcemia, and promptly reversible after correcting it. In our case, the impaired concentrating ability of the renal tubule was irreversible after the removal of the parathyroid adenoma and the correction of the hypercalcemia, presumptively due to the morphological irreversible changes in the kidney. In addition, hypercalciuria persisted and was the cause of a compensatory hyperparathyroidism. Treatment with thiazide diuretic was effective to decrease relative hypercalciuria, thus reversing the compensatory hyperparathyroidism.

Adenoma↗