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Biomedical subjects

F Prigent

Publications and source records attributed to F Prigent.

At least 127 records · Page 7Linked to original sources
PubMed1982

[Angiographic study of the ejection fraction by thirds of systole in coronary patients].

The parameters of myocardial function in the initial phase of ventricular ejection are theoretically more sensitive than the indices calculated over the total systolic ejection period. The object of this study was to evaluate whether the calculation of the ejection fraction by thirds of systole, giving a separate assessment of left ventricular performance at the beginning, the middle and end of ejection, could reliably detect minor changes in ventricular function unrecognised by the usual holosystolic indices. Seventy left ventricular angiograms were analysed in 20 normal subjects (Group I) and 50 patients with coronary artery disease whose ventricular function estimated by the usual parameters was either decreased (Group II, 20 patients) or normal (Group III, 30 patients). In Group I, the ejection fraction in the first third of systole (FE1/3) was much higher than the ejection fraction in the second third (FE2/3). On the other hand, in Groups II and III, all patients had a FE1/3 lower than the FE2/3 (specificity: 100 p. 100). In these two groups, the reduction of FE1/3 and the increase of FE2/3 were very significant compared to Groupe I (p less than 0,001). The ejection fraction of the lest third was identical in the 3 groups. This abnormal distribution of ejection was detected in all coronary patients and was the only alteration of ventricular performance in each of the 30 patients in Group III. In this group, this abnormality was detected equally in patients with triple vessel disease (Subgroup III a, 20 patients) and in patients with isolated left anterior descending disease (Subgroup III b, 10 patients) illustrating the high sensitivity of this index for the detection of a minor abnormality of myocardial function.

Adolescent↗
PubMed1982

[Atopy and associated diseases].

In this study of the literature, the authors review the possible association between atopy and other conditions. The mostly reported observations are related to atopic dermatitis, but there is also some real relationship with respiratory allergy: this is true for mucoviscidosis. Some association are significant and interesting from the pathogenetic point of view: they may be classified under three headings (digestive and renal diseases, immunologic disturbances). Therefore, this study refers to intestinal absorption disturbance, gluten sensitive enteropathy, dermatitis herpetiformis, colic diseases, and also to glomerulopathies, immediate and delayed hypersensitivity deficiency and polymorphonuclear functional deficiency. Other associations do exist, but the low number of cases and the absence of a common pathogenesis allow no conclusion to be drawn (ectodermal anidrotic ectodermal dysplasia, Dubowitz syndrome). Some of them are certainly only fortuitous: this should be more definitely established by later studies. Some diseases may be accompanied by an eczematiform dermatitis (phenylketonuria, anomalies of histidin metabolism). Frequency of associations with alopecia areata and dominant ichthyosis vulgaris is also discussed. This review gives the possibility of emphasizing the absence of statistically significant hematopathic evolution of atopic dermatitis and calls attention on the Wiskott-Aldrich-syndrome as a model for the studies on atopy.

Agammaglobulinemia↗
PubMed1982

[MacCune-Albright syndrome. Apropos of a case].

The MacCune-Albright's syndrome as described by these authors in 1936 and 1937 is extremely rare if one considers the complete form consisting in the triad: café-au-lait spots, fibro-osseous dysplasia, endocrine disturbances. We report an additional case in a 5 year old girl with an precocious puberty. Within a 10 year follow up occurred a polyostotic fibrous dysplasia with pathologic bone fractures in cystic areas, and a right exophthalmos induced by a cyst of the large wing of the sphenoid bone. All these lesions led to orthopaedic and neural surgical procedures. The early puberty of central origin (as proved by the LH-RH test) was treated during 7 years by medroxyprogesterone acetate with an excellent result. No peripheral endocrine syndrome was evidenced. This case stresses the multiple problems arising in the disease and the necessity of a long lasting careful follow up.

Child, Preschool↗
PubMed1982

[Prolidase and manganese deficiency. Apropos of a case: diagnosis and treatment].

Prolidase deficiency, transmitted on an autosomic recessive mode upsets skin healing and facilitates the occurrence of chronic cutaneous ulcerations. A 36-year-old woman has been followed since the age of 12 for ulcerations and erythematous erysipelatoid plaques of the lower limbs. Two episodes of agranulocytosis were induced by intake of sulfonamides at the age of 17. The same accident had been observed in her aunt. As the aetiological research of ulcers was negative, a prolidase deficit was suspected. The diagnosis is ascertained by the existence of an immunopeptiduria of 5 mmol/24 hours (normally absent). The predominating dipeptides are glycilproline and phenylalanine proline. R-hydroxyproline dipeptide were present at a lesser degree. Urinary hydrolysis showed elevation of free proline (x 10) and hydroxyproline (x 6). Dosage of erythrocytes prolidase evidenced an activity 2 p. 100 of the normal in one case and 55 p. 100 and 49 p. 100 in the parents. Treatment by cofactors of prolidase (vitamine C and manganese) reduced immunopeptiduria, suppressed inflammatory outbreaks and allowed a transient cicatrisation. This tenth case of prolidase deficiency underlines the character of the disease: recurrent ulcers (7/10), erysipelatoid plaques (3/10), ecchymosis (4/10), telangiectatic scars (7/10), edema (1/10), early canitias (1/10). Partial correction by cofactors evokes a prolidase deficiency by inactivation of the enzymes activating systems.

Adult↗
PubMed1981

[Bullous and hereditary Weary-Kindler's acrokeratotic poikiloderma (author's transl)].

Two observations of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler are studied. They are transmitted in dominance on two generations. The neonatal presentation of the first observation makes us discuss an epidermolysis bullosa; but the spontaneous regression, the appearance of a reticular pigmentation and the analysis of the paternal dyschromia allow the correction of the diagnosis. Forty-one observations are actually known. The blisters are present in 95 p. 100 of the cases, the dyschromia in 90 p. 100 and the keratosis on 60 p. 100. The trisymptomatic and bisymptomatic forms prevail. The mechanism of the disease is unrecognized but the vacuolization of the basal epidermic cells seems to be at the origin of the bullous eruption.

Adult↗
PubMed1981

[Porokeratosis of Mibelli in two monozygotic twins (author's transl)].

Two monozygots twins had porokeratosis Mibelli and a deep mental retardation. The exploration of this nervous components is negative. The porokeratosis in one twin had in several sites the aspect of a skin's horn. The demonstration of the monozygotis was made by the same repartition of 16 characters (HLA, blood groups). The boys had the same aspect and the same skin disease. The twin method is discussed. The existence of the porokeratosis of Mibelli in two monozygotic twins is an argument for the genetic mechanism of the disease. The familial cases of porokeratose Mibelli reported by Bataillard, Civatte, Vigne, Bloom, have consistent features for an dominant autosomic transmission with variations in the penetrance and the expressivity of the skin disease.

Adolescent↗
PubMed1981

[Silvery hair in children: a symptom of leucogranulocytic and melanocytic diseases (author's transl)].

Nine children with silvery hair have been dermatologically studied: eight out nine presented a context of abnormal sensitivity to infections. The study of pigmentation, granulocytes functions and immunity system allows a separation in several types: 1) 4 patients had a characteristic Chediak-Higashi syndrome, this diagnosis being based on pigmentary and immunohematological grounds. 2) Two siblings exhibited functional disturbance of the Chediak-Higashi type, but without its cytological abnormalities. A third patient resembled closely to this picture, but was insufficiently investigated. 3) The eighth patient has been studied by Drs. Griscelli and Pruniéras who had previously described a new disease on immunological, hematological and pigmentary grounds. All these case with defective polymorphonuclear functions have febrile peaks with accelerated aggravation close to Chediak-Higashi syndrome. 4) The ninth patient with silvery hair does not have any immunological abnormalities, nor any accelerated phase at the age of eight but suffers from diffuse epilepsy, cerebellar and chorioretinal atrophy.

Chediak-Higashi Syndrome↗
PubMed1981

[Native favus in Poitou. About 3 cases (author's transl)].

Three cases of tinea favus of pityriasic aspect and without a "scutulum" sign are diagnosed in a small town of "Poitou" region. They correspond to an autochtonal focus. The various enquiries show that favus has become rare in France but the local focus as are still active, since 43 p. 100 of recorded favus are of local origin.

Child↗
PubMed1981

[Dermatological manifestations of synovial cysts of the lower limbs (author's transl)].

Two cases of synovial cysts of the knees are reported. Thier emergeing point located far away from the joint they originate from can make the diagnosis be difficult. Puncture allows to remove synovial fluid and to inject a radio-active iodine compound to realise a cystography. The radiography of the joint demonstrates an already existing joint disease. The atypical aspects of synovial cysts are emphasized on the basis of then two cases such as pseudo-phlebitic, pseudo-arteritic, inflammatory and fistulised. The presentation of synovial cysts of the legs is frequently dermatological. The diagnosis must be suspected in order to avoid a dermatological biopsy.

Adult↗
PubMed1980

[Acute haemorrhagic edema of the skin of the new-born with lethal intestinal complication (author's transl)].

A child, eleven months old, presented with an A. H. O. after an otitis. The dermatologic aspect is typical with oedema and target-like hemorrhagies. The disease evolved in three successive episodes. During the second one two intestinal intussuceptions occurred. It was necessary to proceed to an intestinal resection of twenty centimeters of the small bowel and of the right part of the large bowel. The post-operative period has been complicated by an intravascular coagulation, perforation of small bowel and peritonis due to Proteus bacillus. The child died during these complications. This evolution looks like an anaphylactoid purpura of Schönlein-Henoch. But this does not allow to affirm that these two allergic vasculitis are related to the same etiology. Our own observation suggests the necessity of watching the coagulation rate when an acute haemorrhagic oedema is complicated by bowel's intussusception.

Acute Disease↗
PubMed1980

[Changes in the keratinocytes in oculo-cutaneous tyrosinosis: Richner-Hanhart syndrome (author's transl)].

The Richner-Hanhart syndrome corresponds to a tyrosine elevation in serum due to a defect in soluble tyrosine amino-transferase in liver cells. This new enzymopathy which is transmitted in an autosomal recessive mode is called oculo-cutaneous tyrosinosis. It is curable by a low diet in tyrosine and its precursors. The diagnosis has been clinically suggested in an 18 months old girl, by the association of punctate palmar and plantar keratosis, dendritic ulcerated keratitis, and mental retardation. The diagnosis was established by elevation of tyrosinemia up to 52 mg/100 ml associated with a high urinary elimination of tyrosine and phenylcetonic acid. Absence of anomaly in the metabolism of methionin and hepatorenal lesion is characteristic. The diagnosis was confirmed by the absence of soluble tyrosine aminotransferase in liver cells and by the effectiveness of the diet. The clinical keratosis corresponds histologically to a orthokeratotic hyperkeratosis. The keratinocytes show 2 types of anomalies ranged in the epiderm. Intracytoplasmic vacuoles which include or lead to pseudomyelinic formations extend progressively from the mitochondrial alterations in the epidemial basal layers. Bulky polyhedral electron dense particles are found in the cytoplasm of the superficial keratinocytes. Most of these images have been demonstrates anteriorly in the keratinocytes ant the corned; on the other hand, signs of mitochondrial anomaly had not been observed. The genesis of these cellular alterations based on the liberation of lysosomial enzymes by the action of crystals of tyrosine has been suggested by Goldsmith from experimental facts. However, it seems that the mitochondrial defect occurs outside this mechanism.

Amino Acid Metabolism, Inborn Errors↗
PubMed1979

[Septic arthritis due to a nontoxigenic diphtheria bacillus].

Septic arthritis of the hip in a 2 year old child is described. A nontoxigenic diphtheria bacillus was isolated in large numbers from the articular fluid. The same organism was isolated from excoriated skin lesions of the toes. The bacteriology, epidemiology and pathology of the infection are discussed.

Arthritis, Infectious↗