[Assessment of dermatological emergencies in a French university hospital]. / Evaluation d'une consultation d'urgences en dermatologie.

INTRODUCTION: The aim of our study was to understand the motivations of outpatients who come to dermatological emergencies in a university hospital. PATIENTS AND METHOD: This 6-week prospective study included outpatients who came to the dermatology emergency unit. This consultation is proposed each morning (from 8 to 9), from Mondays to Fridays. A questionnaire was distributed to outpatients. They answered questions on the functioning of this consultation and their own symptoms. The consulting dermatologist answered questions on the referring physician, the really urgent characteristics of the disease and the diagnosis. RESULTS: Patients were satisfied by the functioning of the consultation. Indeed, 59 p. 100 of outpatients thought that the timetable was convenient and 70 p. 100 that the delay before getting a consultation was rapid. 75 p. 100 felt they needed treatment rapidly. Nonetheless, 45 p. 100 did not think they had a serious disease. More than half of the outpatients were referred by their general practitioner; the others came spontaneously, or were referred by other departments or general emergencies. The most frequent diagnoses were cutaneous infections (27.6 p. 100), eczema (21 p. 100), then benign tumors, psoriasis, physical dermatoses, viral eruptions... DISCUSSION: A consultation for dermatological emergencies appears to reply to patients' demands. Nonetheless, most of these outpatients do not present with real dermatological emergencies. Criteria for real emergencies needs to be further defined and understood by citizens.

Left chest cyst in 26-week fetus.

A left chest cyst was found in an 18-week fetus. At autopsy it was found to be a cystic lymphangioma arising from the diaphragm.

Nickel-catalyzed arylation of acrolein diethyl acetal: a substitute to the 1,4-addition of arylhalides to acrolein.

[reaction: see text] In the presence of catalytic amount of NiBr(2) as catalyst precursor, organic halides are reductively coupled at 70 degrees C with acrolein diethyl acetal to give (Z)- and (E)-enolethers by allylic deplacement of an alkoxy group. Subsequent hydrolysis affords beta-arylated aldehydes.

[Bi-symptomatic CINCA syndrome with inaugural urticaria and major articular lesions]. / CINCA syndrome bi-symptomatique avec urticaire inaugurale et lésions articulaires majeures.

BACKGROUND: CINCA syndrome (chronic infantile neurological cutaneous and articular syndrome) observed in young children associates chronic urticaria and rheumatological disorders which may cause deformation of the larger peripheral joints. Neurological signs develop later leading to severe prognosis: chronic meningitis, deafness, mental retardation. We present a bisymptomatic case which was distinctive by the absence of central nervous system involvement after 11 years of course. CASE REPORT: Since the age of six months a female child experienced chronic urticaria with nearly daily episodes. At two years limping was also observed. At nine years, the child had highly deformed knee joints giving a tumoral radiological aspect. The diagnosis of CINCA was made. At the age of 11 years, the child had normal psychomotor development and neurological and biological tests were normal. The knee deformations stablized and the child suffered little from the urticaria. DISCUSSION: This case of infantile chronic urticaria inaugurated a CINCA syndrome, a condition described in France and termed NOMID (neonatal onset multisystem inflammatory disease) in the English literature. Our case was similar to those reported in the literature showing characteristic skin, biological and rheumatological disorders. The absence of neurological involvement is exceptional and has been confirmed by regular surveillance to 11 years of age. Bone pathology showed a pseudotumoral aspect which has not been described previously.

[Universal dyschromatosis: a familial case]. / Dyschromatose universelle: une observation familiale.

INTRODUCTION: Universal dyschromatosis is a generalized leucomelanodermia recognised in Japan in 1933. We report a family with universal dyschromatosis, demonstrating the mode of transmission. The ultrastructural aspects are compatible with a functional melanogenesis anomaly. CASE REPORT: A 9-year-old girl was hospitalized for recently diagnosed insulin-dependent diabetes mellitus. She was born to non-consanguinous parents and her past medical history was uneventful. Her father was of mixed ethnic origin. The physical examination revealed generalized leukomelanoderma identified since the first year of life. Zones of small achromatic maculae alternated with zones of pigmented maculae of variable size and color. Lesions were diffuse but predominated on the trunk and did not involve the face, the hands or the feet. Neither the child nor her father who also has leukomelanoderma were photosensitive. A skin biopsy from the gluteal region revealed alternating zones of hyper- and hypopigmentation. The ultrastructural analysis showed that the number of melanocytes was not significantly different in the different pigmented zones and the pigment transfer to adjacent keratinocytes was intact. There were three other girls in the kinhood and two, as well as a few other individuals in the family, had a localized form of the disease. DISCUSSION: Universal dyschromatosis is a rare genodermatosis. The familial cases reported here illustrate the variable clinical presentations of this pigmentary abnormality. The pedigree in this family demonstrated incomplete penetrance of hereditary leukomelanoderma with autosomal dominant inheritance. The localized forms reported to date under different names would actually appear to correspond to incomplete expression of the dermatosis. The skin manifestations in universal dyschromatosis would appear to be similar to those in a few other skin diseases, mainly xeroderma pigmentosum, especially the localized forms; for generalized forms however, there is little room for confusion as photosensitivity is absent and lesions predominate in unexposed zones. The ultrastructure investigations showed different levels of melanocyte activity without abnormal pigment production or transfer. This abnormality has variable expression, explaining the multitude of clinical presentations.

Rapid routes of synthesis of chemically reactive and highly radioactively labeled alpha- and beta-oligonucleotide derivatives for in vivo studies.

Development of the antisense oligonucleotide strategy for the regulation of gene expression in vivo poses several problems: the stability of oligonucleotides toward intracellular nucleases, labeling of oligonucleotides with high specific radioactivity, improvements of penetration of oligonucleotides into living cells, and enhancement of antisense action by coupling of chemically active groups. In the present paper synthesis of highly radioactively labeled [32P]- and [35S]oligonucleotide derivatives is described starting from both natural (beta) and nuclease-resistant (alpha) anomers of oligonucleotides. Conditions for preparative phosphorylation and thiophosphorylation suitable for oligonucleotides of various lengths, base composition, and anomeric forms were established. The stability of the phosphoramide bond under in vivo experimental conditions was checked. The methods of terminal phosphate chemical activation and terminal thiophosphate alkylation were applied to synthesize oligonucleotides equipped with hydrophobic, intercalating, alkylating, and photoactivatable groups. In the case of porphyrin-oligonucleotide conjugates, a series of new monofunctional porphyrin derivatives bearing a free aliphatic amino group was developed.