PubMed Health⌕ Search

Biomedical subjects

E Perrin

Publications and source records attributed to E Perrin.

52 records · Page 3Linked to original sources
PubMed1982

Familial T-cell lymphoblastic lymphoma: association with Von Recklinghausen neurofibromatosis and Gardner syndrome.

A family is described in which three of seven siblings developed a T-cell lymphoblastic lymphoma. Tumor cells formed rosettes with sheep erythrocytes, lacked surface Ig, and expressed human T- but not B-lymphocyte antigens. They lacked the enzyme terminal transferase suggesting a lymph node rather than thymic T-cell origin. Autopsy findings supported this conclusion. All three sibs, like their father, had numerous cafe'-au'lait spots indicative of Von Recklinghausen neurofibromatosis. One child had a subcutaneous fibroma, and another had multiple colonic polyps and exostoses characteristic of Gardner syndrome. Both are autosomal dominant conditions known to predispose to malignancies. The genetic factors responsible for these conditions may also predispose to the development of "post-thymic" T-cell lymphoblastic lymphoma.

Child↗
PubMed1982

A lethal, Larsen-like multiple joint dislocation syndrome.

We describe here two isolated cases of lethal, Larsen-like multiple joint dislocation syndrome. Death occurred shortly after birth with pulmonary insufficiency due to tracheomalacia and/or lung hypoplasia. Both had abnormal dermal collagen bundles. In one case, histochemical studies showed abnormal cartilagenous matrix and striking deficiency in the dense collagen bundles of joint capsules; electronmicroscopic studies of the hyaline cartilage of the trachea documented a relative increase of the small, short fibers with decrease of the matrix collagen fibers. This study confirms that this Larsen-like syndrome is a disorder of connective tissue, possibly involving dysmaturity of the collagen fibers, with a predilection for joint capsules and tracheal cartilage. This lethal condition is characterized by pulmonary insufficiency, tracheomalacia, and collagen fiber dysmaturity.

Collagen Diseases↗
PubMedDate not supplied

[Fluoxetine: an update of its use in major depressive disorder in adults].

The selective serotonin reuptake inhibitors (SSRIs) have emerged as a major therapeutic advance in psychiatry. They have emphasized the pathophysiological role of serotonin (5-HT) in affective disorders. Indeed, SSRIs were developed for inhibition of the neuronal uptake for serotonin (5-HT), a property shared with the TCAs (tricyclic anti-depressants), but without affecting the other various central neuroreceptors (ie, histamine, acetylcholine and adrenergic receptors) that are responsible for many of the safety and tolerability problems with TCAs. In this way, fluoxetine and other SSRIs represent a major advance over tricyclics, because of their lower toxicity. While the position of fluoxetine relative to other selective serotoninergic antidepressants requires further investigation, fluoxetine has a more favorable tolerability profile for a similar efficacy in comparison to tricyclic antidepressants. The pharmacokinetic and pharmacodynamic properties of fluoxetine are well described. After oral administration, fluoxetine is almost completely absorbed. Due to hepatic first-pass metabolism, the oral bioavailability is < 90%. Fluoxetine has a half-life of 2-7 days, whereas the half-life of norfluoxetine ranges between 4 and 15 days. This long half-life of fluoxetine may be advantageous when the patient omits a dose since drug concentrations decrease slightly. On the other hand, in the case of fluoxetine non-response, long washout periods are necessary before switching the patient to a TCA or a MAO inhibitor to avoid drug interactions or the development of a 5-HT syndrome. As a class, SSRIs are considerably more selective in comparison to TCAs in terms of their central nervous system mechanisms, but differ in other clinically relevant aspects. This action affects several specific 5-HT receptors, which, in turn, effects a multitude of neural systems and signalization pathways. However, despite the facilitating serotoninergic neurotransmission, the direct mechanism by which a SSRI exerts its anti-depressant activity remains uncertain. The therapeutic response in major depression for SSRIs (ie 15-20 days) maybe due to a progressive desensitization of somatodendritic 5-HT autoreceptors in the midbrain raphe nucleus. On the other hand, it has also been postulated that 5-HT is a modulator of several neurophysiological pathways, including dopamine, noradrenaline, but also neurotrophic factors, intra-cytoplasmic phosphorylations and nuclear genes expression. Therapeutic activity of SSRIs may finally results in a complex modulation and homeostasis between monoaminergic neurotransmisson and neuronal plasticity. In term of health-care, the introduction of fluoxetine and other SSRIs in the 1980s has radically changed the treatment of depressive disorder worldwide and they have emerged as the first line of treatment for depressive disorders. The efficacy of fluoxetine is now well established in the treatment of major depressive disorder. Indeed, this efficacy has been assessed in numerous clinical controlled trials involving patients with major depressive disorders. Meta-analysis were carried out and confirmed that fluoxetine was as effective as the tricyclic antidepressants, and appeared more effective than placebo in improving the symptoms of depression. However, there is no scientific evidence to suggest that any one SSRI is more effective than another, but not all patients respond to the same agent. Looking to the future, we need further comparative studies of the SSRIs with the next generation of antidepressants such as 5-HT noradrenaline reuptake inhibitors (SNRIs, Venlafaxine). Actually, it is interesting to note that, whereas the emphasis with the SSRIs has been on their selectivity, recent developments have tended to move towards less selective agents, and now to other neurobiological pathways (ie neurotrophic factors). Finally, fluoxetine, in common with other SSRIs, remains today a first-line treatment option for major depressive disorder.

Adult↗
PubMedDate not supplied

Renal abnormalities in paucity of interlobular bile ducts.

Paucity of interlobular bile ducts is a common histologic characteristic in persistent conjugated hyperbilirubinemia of infancy. It occurs in two forms: the syndromatic and the nonsyndromatic types. The syndromatic form is also called arteriohepatic dysplasia. The nonsyndromatic type of paucity of interlobular bile ducts occurs less frequently and is usually associated with more severe disease and a less favorable prognosis. We present two unusual renal anomalies in association with paucity of interlobular bile ducts. In the first case, juvenile nephronophthisis was diagnosed in a 4-week-old infant with arteriohepatic dysplasia; the patient died from severe renal disease by 2 months of age. The second case presented with severe bilateral hydronephrosis and hydroureter secondary to posterior urethral valves associated with the nonsyndromatic form of paucity of interlobular bile ducts. He, however, improved after corrective surgery of the urethral valves. Other renal abnormalities previously reported in the literature in association with chronic liver disease are also reviewed.

Bile Ducts, Intrahepatic↗
PubMedDate not supplied

[Determinants of compliance with antidepressive drugs].

A national survey was conducted in a representative sample of the general French population in order to assess antidepressant non compliance frequency and the factors that influence it. Two types of noncompliance were taken into account: 1) the premature interruption of the treatment; 2) the omission of doses. Data were collected by phone interview. Results show that 36.9% of the 423 included subjects (who represent 4.3% of the sample) were noncompliants (15.4% by treatment interruption and 21.5% by dosage modification). The two types of noncompliance have different risk factors. Treatment interruption was more frequent for patients with high level of schooling (OR = 2.5), in case of multiple take regimens (OR = 3), when the physician did not informed the patient about treatment (OR = 3.3) and when the patients' sources of information were exclusively non medical ones (OR = 3.7). Dosage modification was more frequent in female (OR = 2.1), when the treatment duration exceeded six months (OR = 2.5) and when the prescriber did not communicate with the patient relatives (OR = 1.8). The information delivered by the physician about the duration of the treatment was associated with the two types of compliance but with an opposite effect. This information has a protective effect on treatment interruption (OR = 0.5) but increases the risk of dosage modification (OR = 1.1).

Adult↗