[AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE: HOW AND WHY SHOULD WE IDENTIFY THE PATIENTS "RAPIDLY PROGRESSING" TO END-STAGE RENAL DISEASE?]. / LA POLYKYSTOSE RÉNALE AUTOSOMIQUE DOMINANTE: comment et pourquoi identifier les patients "rapidement progresseurs" vers l'insuffisance rénale terminale?

Autosomal dominant polycystic kidney disease (ADPKD) is a common inherited disease characterised by the progressive development of multiple and bilateral cysts in kidneys and other organs. Most patients with ADPKD will develop, sooner or later, end-stage renal disease (ESRD). The morbidity and mortality associated with ESRD prompt physicians to identify early ADPKD patients considered as "rapid progressors", who have the greatest risk to rapidly develop ESRD. The rate of progression can be assessed by clinical--especially with the "predicting renal outcome in polycystic kidney disease score" (PROPKD-Score)-, biological (a decline of the glomerular filtration rate (GFR) of 4.4-5.9 ml/min/year and/or the doubling of serum creatinine within a 36-month period), or radiological criteria (total kidney volume (TKV) adjusted for the size > 600 cc/m and/or TKV annual growth rate > 5 %). Nowadays, there is no curative treatment for ADPKD. However, vasopressin-2 receptor antagonists, such as tolvaptan, appear to slow down the growth of renal cysts and the slope of GFR decline. The current management of ADPKD patients is mostly based on correcting the risk factors for progression, i.e. encouraging (over)-hydration, normalizing blood pressure, stimulating smoking cessation.

Bacterial rapid identification with matrix assisted laser desorption/ionization time-of-flight mass spectrometry: development of an 'in-house method' and comparison with Bruker Sepsityper(®) kit.

OBJECTIVE: The objective of this study was to compare an in-house matrix-assisted laser desorption ionization with time of flight (MALDI-TOF) method and a commercial MALDI-TOF kit (Sepsityper(®) kit) for direct bacterial identification in positive blood cultures. We also evaluated the time saved and the cost associated with the rapid identification techniques. METHODS: We used the BACTEC(®) automated system for detecting positive blood cultures. Direct identification using Sepsityper kit and the in-house method were compared with conventional identification by MALDI-TOF using pure bacterial culture on the solid phase. We also evaluated different cut-off scores for rapid bacterial identification. RESULTS: In total, 127 positive blood vials were selected. The rate of rapid identification with the MALDI Sepsityper kit was 25.2% with the standard cut-off and 33.9% with the enlarged cut-off, while the results for the in-house method were 44.1 and 61.4%, respectively. Error rates with the enlarged cut-off were 6.98 (n = 3) and 2.56% (n = 2) for Sepsityper and the in-house method, respectively. Identification rates were higher for gram-negative bacteria. DISCUSSION: Direct bacterial identification succeeded in supplying rapid identification of the causative organism in cases of sepsis. The time taken to obtain a result was nearly 24  hours shorter for the direct bacterial identification methods than for conventional MALDI-TOF on solid phase culture. Compared with the Sepsityper kit, the in-house method offered better results and fewer errors, was more cost-effective and easier to use.

Use of a temperature gradient measuring device in monitoring of diabetic and critically ill patients.

A device realizing the simultaneous measure of the central body temperature (Tc), the superficial one (Ts) and their difference (Dt), was proposed for permanent energetic balance evaluation in humans. A program was elaborated to command the intravenous delivery of insulin by a pump depending on the value and trends of Dt. The use of this device for monitoring of decompensated diabetic patients allowed their easier stabilization. In critically ill patients (post transplantation, myocardium infarction) it had a diagnostic and prognostic value, and was helpful for optimization of conventional and insulin therapy.

[Social aspects of diabetes mellitus]. / Aspects sociaux du diabète sucré.

A diabetic patient may suffer from social discriminations. We will briefly describe the problems related to licence driving, employment and insurances. Diabetic associations work to attenuate such discriminations and try to provide a practical help to diabetic patients confronted to such difficulties.

Vertebral artery dissection. Case report.

Vertebral artery dissection is an uncommon but important cause of posterior stroke in young and middle aged adults. We report a case with a long term fluctuating ischemic symptoms due to bilateral spontaneous dissection. Etiology and treatment are discussed.

[Lack of benefit from the intermittent administration of insulin in treatment using subcutaneous perfusion pump in type 1 diabetes]. / Absence de bénéfice de l'administration intermittente de l'insuline lors d'un traitement par pompe a perfusion sous-cutanée chez le diabétique de type-1.

Our study is based on two constatations: 1) Hyperinsulinaemia, a possible atherogenic factor, is frequent under continuous subcutaneous insulin infusion. 2) Pulsatile intravenous insulin delivery improve the insulin's hypoglycaemic activity. To test if equivalent metabolic control can be obtained with a reduced intermittent subcutaneous infused insulin dose, we compared nocturnal metabolic control of 8 c-peptide negative type 1 diabetic patients under three experimental conditions: Continuous usual dose test (1.0 +/- 0.1 u/h); Intermittent half dose test (1.0 +/- 0.1 u/h, 30 min/h); Continuous half dose test (0.5 +/- 0.05 u/h) Five parameters were monitored: blood glucose, plasma free insulin and beta-hydroxy-butyrate, free fatty acid and glycerol plasma level. No significant differences were found between intermittent and continuous half-dose tests. We conclude that, in our experimental conditions, intermittent subcutaneous insulin infusion does not reduce the metabolic degradation induced by insulin dose reduction.

[Peroperative localization of insuloma using staged portal vein insulin assay. Apropos of a case]. / Localisation peropératoire de l'insulinome par dosage étagé de l'insuline portale. A propos d'un cas.

The diagnosis of insulinoma is easily done now but, in many cases, it is still difficult to locate it without the use of invasive procedures. With selective angiography and percutaneous transhepatic catheterization of the portal vein however, failure is reduced to about 4%. We report a case of a double localisation of insulinoma, revealed by an intraoperative catheterization of the splenic vein. The advantages of this method are discussed.

[Value of the pentagastrin test in familial hypercalcitoninemia]. / Intérêt du test à la pentagastrine en cas d'hypercalcitoninémie familiale.

This report concerns the history of a portugesian family including one case of MEN II a (female, born 1932, presenting medullary carcinoma of the thyroid--MCT--, hyperparathyroidism and suspected pheochomocytoma), and three others with MCT alone or in association with pheochromocytoma. The diagnosis of MCT has been made possible investigating six members of the kindred by use of IR - calcitonin measurements and pentagastrin injection. This provocative screening test is also of interest to follow operated patients.

[Effects of propranolol and indomethacin upon urinary hydroxyproline in hyperthyroid patients (author's transl)]. / Effets du propranolol et de l'indométacine sur l'hydroxyprolinurie dans l'hyperthyroïdie.

Hydroxyproline excretion was increased by hyperthyroid patients. This increase was related to thyroid hormone levels. Propranolol administration induced a decrease in hydroxyproline excretion and in immunoreactive triiodothyronine. Indometacin administration induced a decrease in urinary excretion of prostaglandins but had no effect on hydroxyproline excretion. These data suggest that the increased bone resorption of hyperthyroidism may involve a catecholamine beta receptor-dependent mechanism and is probably independent of prostaglandins.

[Assessment of biological and hormonal diagnostic criteria in acromegaly (author's transl)]. / Validité des données biologiques, hormonales ou autres, pour le diagnostic de l'acromégalie.

A review of 30 cases of acromegaly has confirmed the limited diagnostic interest of serum phosphorus determination as it was elevated in 40% of cases only; urine hydroxyproline excretion was more useful, since it was high in 80% cases. As for the baseline plasma concentration of somatotropin, it was at or above 10 ng/ml in 80% cases. Among dynamic tests meant to evaluate the responsiveness of the growth hormone-secreting adenoma, inhibition after L-Dopa ingestion and/or stimulation after T.R.H. injection occurred in 2/3 cases. Lack of inhinition after a glucose load was noted in half of the cases only. An abnormal (yet typical for acromegaly) response can be obtained for anyone of these tests, which points at the variable degree of (de) differenciation of the adenoma cells from one case to the other.