[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.

[Treatment of familial erythermalgia with the association of lidocaine and mexiletine]. / Traitement de l'érythermalgie familiale par l'association lidocaïne-mexilétine.

INTRODUCTION: Erythermalgia is a rare acrosyndrome characterized by reddening of the skin, local increase heat and pain. The disease is frequently resistant to treatment. Recently, Kuhnert et al. presented very favorable results using a combination of lidocaine and mexiletine. We used this treatment in 4 patients suffering from familial erythermalgia. OBSERVATIONS: In a family exhibiting severe familial erythermalgia involving 5 members over 3 generations, we treated 4 patients aged 41, 39, 19 and 15 years. In these patients, the erythermalgia known since early childhood, progressed in the form of multiple flares (6 to 7/day) during the day and at night, lasting several hours and often accompanied by headaches. The impact of the disease on their quality of life was major. Only cold-water baths provided temporary relief, obliging them to live with their "feet in cold water". After they had been informed of the modalities of treatment and in the absence of any contraindication, notably cardiologic, 200 mg (100 mg in the youngest patient) of lidocaine were infused in 4 hours in a single intravenous injection on the first day. Mixelitine was introduced on the second day at the dose of 600 mg in 3 oral intakes (200 mg in the youngest patient). The painful paroxistic symptomatology rapidly improved and the flares had disappeared on the 3dr day, thus permitting the progressive reduction in analgesics and major improvement in quality of life. This beneficial effect persisted with oral mexiletine alone, 2 years after the infusion of lidocaine in the first patient treated (and one year after in the other patients). COMMENTS: Primary familial erythermalgia is highly resistant to treatment. The combined action of lidocain and mexiletine, usually well tolerated (class IB antiarrythmic), blocks the sodium channels. The mechanism of action of their analgesic effect is peripheral or central or even mixed. This benefit warrants confirmation in other forms of erythermalgia.

Inverse agonist activity of selected ligands of platelet-activating factor receptor.

The receptor for platelet-activating factor (PAFR) is a member of the G protein-coupled receptor (GPCR) family. According to the allosteric ternary complex model, GPCRs exist in an equilibrium between different conformations. Agonist binding promotes and stabilizes the receptor in an active conformation. On the other hand, ligands that stabilize the inactive conformation are known as inverse agonists. Due to the association of platelet-activating factor (PAF) with diverse physiological and pathological processes, considerable efforts have been invested in the development of antagonists to PAFR. A large number of these molecules has been shown to specifically interact with PAFR but, surprisingly, little is known about their impact on the conformation of the receptor and its activity. By using a constitutively active mutant (L231R) of the human PAFR and by transiently coexpressing the wild-type (WT) receptor with the G(alpha)q subunit of the trimeric G protein, we were able to address this issue with ligands of diverse structures such as phospholipids, benzodiazepines, furans, and others. We demonstrated that some of these molecules are potent inverse agonists. For example, when cells (WT PAFR + G(alpha)q) were exposed to WEB2086, SM10661, or alprazolam, the basal inositol phosphate production was reduced by 53 +/- 6, 44 +/- 3, and 54 +/- 4%, respectively. The decrease in basal inositol phosphate production by WEB2086 was significantly inhibited by a more neutral antagonist BN52021, confirming the specificity of the reaction. We demonstrate here that WEB2086 and other known ligands previously considered as antagonists can act as inverse agonists on the human PAF receptor.

Mental health problems and service use among female juvenile offenders: their relationship to criminal history.

OBJECTIVE: To describe (1) the level of mental health problems and lifetime use of specialty mental health services and special education programs among incarcerated female juvenile offenders and (2) how these indices relate to their criminal history. METHOD: Between 1997 and 1998, fifty-four female youths incarcerated in California were interviewed on-site using standardized self-report measures of depression and anxiety symptoms and substance use problems. RESULTS: Eighty percent of the youths had symptoms of an emotional disorder or substance use problem, and almost two thirds (63%) had a history of recidivism. Of those with emotional symptoms or a substance use problem, 51% had used specialty mental health services and 58% had been in a special education program during their lifetime. In addition, among recidivistic youths, 82% had a history of a substance use problem and 47% had used specialty mental health services during their lifetime. CONCLUSIONS: A substantial proportion of female juvenile offenders merit a mental health evaluation. Interventions for these high-risk youths should include an assessment for substance use disorders because of the association of recidivism and substance use problems in this population.

Conformational studies of sphingolipids by NMR spectroscopy. I. Dihydrosphingomyelin.

The conformational features of dihydrosphingomyelin (DHSM), the major phospholipid of human lens membranes, were investigated by 1H and 31P nuclear magnetic resonance spectroscopy. Several postulates emerge from the observed trends: (a) in partially hydrated samples of DHSM in CDCl3 above 13 mM, at which lipid-lipid interactions prevail, the amide proton is mostly involved in intermolecular H-bonds that link neighboring phospholipids through bridging water molecules. In the absence of water, the NH group is involved in an intramolecular H-bond that restricts the mobility of the phosphate group. (b) In the monomeric form of the lipid molecule, the amide proton of the major conformer is bound intramolecularly with one of the anionic and/or ester oxygens of the phosphate group. A minor conformer may also be present in which the NH proton participates in an intramolecular H-bond linking to the OH group of the sphingoid base. (c) Complete hydration leads to an extension of the head group as water molecules bind to the phosphate and NH groups via H-bonds, thus disrupting the intramolecular H-bonds prevalent at low concentrations.

Conformational studies of sphingolipids by NMR spectroscopy. II. Sphingomyelin.

Sphingomyelin (SM) is the most prevalent sphingolipid in the majority of mammalian membranes. Proton and 31P nuclear magnetic resonance spectral data were acquired to establish the nature of intra- and intermolecular H-bonds in the monomeric and aggregated forms of SM and to assess possible differences between this lipid and dihydrosphingomyelin (DHSM), which lacks the double bond between carbons 4 and 5 of the sphingoid base. The spectral trends suggest the formation of an intramolecular H-bond between the OH group of the sphingosine moiety and the phosphate ester oxygen of the head group. The narrower linewidth and the downfield shift of the resonance corresponding to OH proton in SM suggest that this H-bond is stronger in SM than in DHSM. The NH group appears to be involved predominantly in intramolecular H-bonding in the monomer. As the concentration of SM increases and the molecules come in closer proximity, these intramolecular bonds are partially disrupted and the NH group becomes involved in lipid-water interactions. The difference between the SM and DHSM appears to be not in the nature of these interactions but rather in the degree to which these intermolecular interactions prevail. As SM molecules cannot come as close together as DHSM molecules can, both the NH and OH moieties remain, on average, more intramolecularly bonded as compared to DHSM.

[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.

[Neonatal toxic erythema: 3 atypical cases]. / Erythème toxique néonatal: trois observations atypiques.

UNLABELLED: Diagnosis of pustular dermatosis occurring during the first days of life is based on clinical findings. Erythema toxicum neonatorum (ETN) is the more frequent benign self limiting eruption in the newborn. CASE REPORTS: Three cases of ETN with localized pustules to the genitals and perineal area are described. COMMENT: When encountering a newborn with a localized pustulosis rash, it is important to separate benign condition as ETN from those that require prompt diagnosis and therapy. Atypical ETN and pustular dermatosis due to bacterial or viral infections or inflammatory diseases (e.g., eosinophilic pustulosis) can be differentiated by cytological and bacterial samples.

Numerous and large Bier's spot associated with pregnancy.

We report a patient with anemic macules over cyanotic skin which started in the 8th month of a normal pregnancy. We thought that these lesions were close to Bier's spot description although it has never been described during pregnancy. The relationship with pregnancy is likely because skin lesions regress in post-partum. It probably represents an exaggerated physiological response of small vessels to venous hypertension observed during pregnancy in predisposed women.

[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.

Shaving: a simple and effective treatment for moderate congenital hypertrichosis.

Congenital hypertrichosis is a rare disorder. It is sometimes associated with facial dysmorphism, and dental and gingival abnormalities. This condition constitutes an esthetic problem and regular shaving with a simple razor can be a simple method of treatment.

New ideas for affordable space missions

In September 1995, NASA-Goddard held a workshop on low-cost access to space for science missions. The workshop provided briefings on balloons, sounding rockets, Shuttle payloads, and low-cost free-flyer concepts, to provide options of getting experiments into space. This report is the result of a panel session organized with the aim of generating new ideas beyond those presented in the workshop. In addition to the authors, Orlando Figueroa and Paul Ondrus of NASA-Goddard and Richard Zwirnbaum of Computer Sciences Corp. participated in the discussions. The ideas presented do not necessarily reflect the current thinking of NASA managers. Although the panel discussion was focused on the kinds of science missions usually funded by NASA, most of the ideas that were generated are relevant to military and commercial missions as well.