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Biomedical subjects

C Canuel

Publications and source records attributed to C Canuel.

31 records · Page 2Linked to original sources

[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↗

[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↗

[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↗

[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↗

[Diagnostic approach to prolonged fever (apropos of 72 cases)].

A report of 72 case histories of prolonged feven (more than three weeks, diagnosis not obvious on admission). The writers review the frequency of occurrence of the various etiologies. Infections are the most frequent cause (43 cases). A good number of them are easy to diagnose based on clinical exams and routine tests. Others are a great deal more difficult to diagnose, and in particular deep, suppurative foci. Collagen diseases then follow with 10 cases, the diagnosis of which is made by immunology and histology. Cancers are not very numerous (4 cases) and poorly represent the usual breakdown (kidney, lungs, liver, colon, pancreas). There were no cases of involvement of the blood and reticuloendothelial systems. With regard to examination of these patients, the writers discuss the merits of lymphography and laparatomies, and they set limits to therapeutic tests.

Adult↗

[Bony involvement of the calcaneum in sarcoidosis. A case report].

Bone lesions of sarcoidosis occur in 10 to 15% of the cases, most often involving the extremities. These frequently corticosteroid-dependent lesions worsen the prognosis of the disease. We report a case of sarcoidosis of 14 years duration in a 58-year old woman who presented with a bone lesion strictly localized to the calcaneum. To our knowledge, such lesion has not previously been reported.

Bone Diseases↗

[Chemotherapy in cancer of colon. General review].

The results of surgery as sole treatment of colon cancer are summarized before dealing with those of chemotherapy. Curative or palliative chemotherapy remains controverted. The results of single drug treatments and of the conventional protocols with 5-fluorouracil and nitrosoureas have been disappointing. A promising approach is modulation of 5-fluorouracil by folinic acid, with a response rate of up to 45 p 100, and potentiation of 5-fluorouracil by cisplatin. Intra-arterial chemotherapy has been, and still is, an interesting method in liver metastases, but recent studies and the experience acquired with prolonged follow-ups have thrown doubts on some of its results, notably survival. Adjuvant chemotherapy is even more controversial than curative chemotherapy; however, a recent controlled study has yielded favourable results, and the best drug combinations have not yet been tested. It is concluded that cancers of the colon have shown some chemosensitivity, even though it has not reached the same level as that of cancers of other organs.

Antineoplastic Agents↗