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Biomedical subjects

G Lorette

Publications and source records attributed to G Lorette.

At least 235 records · Page 13Linked to original sources
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

[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↗
PubMed1981

[Hypogonadism and alkylating agents (author's transl)].

The authors have studied the toxicity of alkylating agents on the gonads in the light of three cases. The more frequent prescription of these products in chemotherapy when treating cases in Nephrology, Dermatology, Neurology and Ophthalmology as well as general systemic diseases, should make the doctor prescribing them look out for the risks that the reproductive function runs in their use. Although of course there is no valid discussion of the use of these drugs when life is at stake one should all the same, seeing how their efficacy is increasing and survival is becoming more likely, ask oneself about their bad effects on the gonads, and in particular when they are being used for less serious indications. The gonads are often attacked early and insidiously, the the effects on them often starting before the more spectacular secondary effects such as alopecia or neutropenia. A review of the literature brings to light the following : there is a more marked effect on the gonad when it is the testis than when it is the ovary, which seems to be progressively affected; the lesions become worse and increase according to the pubertal state of the male. As far as testicular damage is concerned, it seems debatable whether is is dose-related or related to the length of treatment, and the chances of recovery on stopping chemotherapy with alkylating agents seen to be overall poor. On the practical level the use of sperm banks may resolve some of the worries about later infertility. As far as women are concerned, the greater resistance of the ovary to the effects should make one careful to employ contraceptive measures which are necessary during and after treatment (for fear of teratogenicity).

Adolescent↗
PubMed1980

[Oculo-cutaneous tyrosinosis (Richner-Hanhart syndrome). Histo-pathological study of a case].

The Richner-Hanhart syndrome corresponds to a tyrosine elevation in serum due to deficit in soluble tyrosine aminotransferase in liver cells. This new enzymopathy which is transmitted in an autosomal recessive mode is called oculocutaneous tyrosinosis. It is curable by a poor diet in tyrosine and its precursors. The diagnosis has been invoked in a 18 months old girl, on the association of punctuate palmar and plantar keratosis, dentritic ulcerated keratitis, and mental retardation. The diagnosis is confirmed by elevation of tyrosinemia to 52 mgs/100 mls associated with a high urinary elimination of tyrosine and plenylcetonic acid. Absences of anomaly in the metabolism of methionin and hepatorenal absence of disturbance of hepatorenal system is characteristic. The keratosis accompany orthokeratotic hyperkeratosis. The keratinocytes show 2 types of anomaly ranged in strates 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 aspects have been demonstrated anteriorly in the keratinocytes and the cornea; on the other hand, signs of mitochondrial sulferance 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 the mitochondrial defect occurs outside this mechanism.

Female↗
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↗