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The broad differential diagnosis of neonatal erythroderma often poses a diagnostic challenge. Mortality of neonatal erythroderma is high due to complications of the erythroderma itself and the occasionally severe and life-threatening underlying disease. Early correct recognition of the underlying cause leads to better treatment and prognosis. Currently, neonatal erythroderma is approached on a case-by-case basis. The purpose of this scoping review was to develop a diagnostic approach in neonatal erythroderma. After a systematic literature search in Embase (January 1990 - May 2020, 74 cases of neonatal erythroderma were identified, and 50+ diagnoses could be extracted. Main causes were the ichthyoses (40%) and primary immunodeficiencies (35%). Congenital erythroderma was present in 64% (47/74) of the cases, predominantly with congenital ichthyosis (11/11; 100%), Netherton syndrome (12/14, 86%) and Omenn syndrome (11/23, 48%). Time until diagnosis ranged from 102 days to 116 days for cases of non-congenital erythroderma and congenital erythroderma respectively. Among the 74 identified cases a total of 17 patients (23%) died within a mean of 158 days and were related to Omenn syndrome (35%), graft-versus-host disease (67%) and Netherton syndrome (18%). Disease history and physical examination are summarized in this paper. Age of onset and a collodion membrane can help to narrow the differential diagnoses. Investigations of blood, histology, hair analysis, genetic analysis and clinical imaging are summarized and discussed. A standard blood investigation is proposed, and the need for skin biopsies with lympho-epithelial Kazal-type related Inhibitor staining is highlighted. Overall, this review shows that diagnostic procedures narrow the differential diagnosis in neonatal erythroderma. A 6-step flowchart for the diagnostic approach for neonatal erythroderma during the first month of life is proposed. The approach was made with the support of expert leaders from international multidisciplinary collaborations in the European Reference Network Skin-subthematic group Ichthyosis.

Resume Les principaux agents responsables des reactions anaphylactiques peroperatoires sont bien connus. Cependant lorsque le bilan allergologique initial est negatif, des explorations complementaires doivent etre realisees afin d’identifier un nouvel allergene potentiel comme les antiseptiques. Nous presentons un cas de choc anaphylactique peroperatoire rapporte a la Betadine®.

Resume Une etude en allergologie moleculaire n’est pas necessaire dans la grande majorite des cas pour la prise en charge d’une pollinose lorsque l’interrogatoire et les tests cutanes sont bien correles a l’histoire clinique du patient. Le diagnostic positif ne repose pas sur la biologie. La sensibilisation a des composants allergeniques sans signification clinique augmente au fur et a mesure de l’exposition aux pollens de graminees. Ainsi, les recombinants n’apportent pas d’informations complementaires utiles a la prescription d’une immunotherapie specifique.

Introduction L’ITA peut etre utilisee dans la RAPG selon 2 protocoles : pre-cosaisonnier (PPC) ou perannuel (PPA) meme si le niveau de preuve pour un benefice a long terme est meilleur pour le PPA (Recommandations EAACI 2018). L’objectif de DIAGRAM etait de decrire le choix du medecin entre ces 2 modalites et d’en comprendre le rationnel. Methodes Etude observationnelle, transversale, multicentrique et nationale, realisee par des allergologues, incluant des patients ≥ 5 ans atteints de RAPG et debutant une ITA-SL. Les donnees ont ete collectees avant l’initiation de l’ITA-SL par des questionnaires medecins et patients (pts) d’oct. 2018 a fev. 2020. La frequence des symptomes de la RAPG a ete evaluee par un score retrospectif (SFRS) cote de 0 a 3 pour chaque symptome (min : 0, max : 18). La severite etait classee : legere ou severe. L’impact sur la qualite de vie (QdV) a ete evalue globalement par les medecins et par le mini-RQLQ par les pts. Le test du Chi2 a ete utilise pour comparer les donnees qualitatives et le test de Student les quantitatives. Resultats Au total, 1119 pts ont ete inclus : âge moyen, 27 ans ; anciennete du diagnostic, 2 ans. Le PPC a ete prescrit chez 1010 pts (92 %). Les 3 criteres les plus importants dans le choix du PPC ou du PPA etaient les symptomes de rhinite (respectivement 27 % ; 50 %), l’impact sur la QdV (24 % ; 19 %) et l’observance (16 % ; 14 %). Si 75 % des medecins n’ont prescrit que le PPC et 6 % que le PPA, 19 % ont prescrit les deux. Au total 92 pts (8 %) ont suivi un PPA. Dans ce groupe PPA, le SRFS etait plus eleve (SFRS ≥ 13 : 67 % vs 58 %, p Conclusion Bien que le PAP ait un niveau de preuve plus fort pour un benefice a long terme dans la RAPG, les medecins francais privilegient le PCP et choisissent le PAP pour les cas plus severes.

Introduction Si les agents responsables des reactions d’hypersensibilite immediate (HSI) lors d’une anesthesie generale (AG) sont bien connus [1] , il existe d’autres allergenes caches. Methodes Un patient de 62 ans opere d’anevrisme de l’aorte presente 3 episodes d’HSI de grade III/IV, 10 minutes apres induction par propofol, ketamine, sufentanil, atracurium et cefazoline, apres paracetamol, nefopam et apres re-injection d’atracurium. Une nouvelle administration des memes antalgiques est sans complication. Resultats La tryptasemie lors du choc est de 125 μg/L (taux basal 8 μg/L). Les tests cutanes (TC) sont negatifs pour atracurium, sufentanil, ketamine, propofol, cephalosporines, heparine et les produits de contraste iodes. Les IgE specifique (IgEs) ammoniums quaternaires sont positives (FEIA) mais negatives pour atracurium et latex. Le test d’activation des basophiles (TAB) est negatif pour atracurium. Seul le TC Betadine® est positif en prick (solution pure) et en IDR au 1/100. Le TAB a Betadine® et les IgEs (FEIA) Betadine® et povidone sont positifs. Discussion Si l’allergie IgE mediee a la povidone iodee est exceptionnelle, une dizaine de cas sont rapportes, survenant apres contact avec les muqueuses ou lors d’un passage intravasculaire [2] , [3] , [4] . Ici on retient l’implication de la Betadine®, seul allergene identifie (TC, IgEs et TAB positifs) et expliquant l’absence de correlation entre les manifestations d’anaphylaxie et l’administration IV des medicaments. Conclusion Un test de provocation affirmerait le choc anaphylactique a la Betadine® mais refuse par le patient.

Introduction L’allergie alimentaire a la peche est frequente, par reaction croisee pollens-aliments ou comme allergie alimentaire primaire. Au Japon et dans la region de Marseille, la sensibilisation a Pru p 7 a ete proposee recemment comme marqueur de gravite de l’allergie a la peche. Nous avons etudie la distribution et les caracteristiques cliniques de la sensibilisation a Pru p 7 dans la moitie Sud de la France. Methodes Nous avons inclus 316 patients suspects d’allergie a la peche (198 allergies confirmees). Les dosages d’IgE ont ete effectues par methode ImmunoCAP. Les donnees polliniques ont ete obtenues a partir du RNSA. Resultats Parmi les resultats statistiquement significatifs concernant la sensibilisation a Pru p 7 : gradient Sud-Nord, association a 99 % (170/171) avec une sensibilisation au pollen de cypres, frequence plus elevee chez les patients allergiques que chez ceux tolerant la peche (62 vs 41 %), association avec les reactions systemiques (78 %) et correlation inverse avec les reactions de faible gravite, association avec la presence de cofacteurs, monosensibilisation apparente (absence d’IgE detectable vis-a-vis de Pru p 1, Pru p 3, Pru p 4) plus frequente chez les patients allergiques que chez les tolerants (54 % vs 17 %), titres d’IgE Pru p 7 plus eleves chez les patients allergiques que chez les tolerants (mediane 3,4 vs 0,3 kUA/L), correlation entre la gravite des reactions cliniques induites par la peche et la concentration d’IgE Pru p 7. Les tests d’inhibition des IgE entre Pru p 7 et le pollen de cypres suggerent une sensibilisation primaire au pollen de cypres. Conclusion Pru p 7, precedemment ignore en raison de l’absence d’outils biologiques, se revele etre un allergene majeur et severe de la peche. La physiopathologie de la sensibilisation a Pru p 7 en lien avec l’exposition aux pollens de Cupressacees reste a decrire. Compte tenu de la large distribution des pollens de Cupressacees dans le monde entier, Pru p 7 et ses homologues pourraient etre impliques dans des allergies potentiellement severes a de nombreux aliments d’origine vegetale.

Resume Le chien et le cheval font partie de l’environnement familier des atopiques. Les allergenes du chien sont trouves dans presque tous les habitats meme sans chien, et il n’est pas rare d’avoir des contacts indirects avec les allergenes du cheval par l’intermediaire de vetements contamines. Les tests cutanes au chien et au cheval devraient donc faire partie de la batterie standard des pneumallergenes. L’identification precise des allergenes de chaque espece progresse, ainsi que la connaissance des reactivites croisees entre les differentes familles proteiques allergisantes. Cependant, meme si les recombinants actuellement disponibles ameliorent notre diagnostic, ils sont encore en nombre insuffisants pour faire la part, dans de nombreuses situations cliniques, entre multiples sensibilisations ou reactivites croisees aux animaux domestiques.

PURPOSE: Phase 3 icatibant trials showed that most hereditary angioedema (HAE) (C1 inhibitor deficiency) acute attacks were treated successfully with one injection of icatibant, a selective bradykinin B2 receptor antagonist. We conducted a post hoc analysis of icatibant reinjection for HAE type I and II attacks in a real-world setting by using data from the Icatibant Outcome Survey, an ongoing observational study that monitors the safety and effectiveness of icatibant treatment.METHODS: Descriptive retrospective analyses of icatibant reinjection were performed on Icatibant Outcome Survey data (February 2008 to December 2012). New attacks were defined as the onset of new symptoms after full resolution of the previous attack. Potential associations between the patient and attack characteristics and reinjection were explored by using logistic regression analysis.RESULTS: Icatibant was administered for 652 attacks in 170 patients with HAE type I or II. Most attacks (89.1%) were treated with a single icatibant injection. For attacks that required two or three injections, the second injection was given a median of 11.0 hours after the first injection, with 90.4% of second injections administered ≥6 hours after the first injection. Time to resolution and attack duration were significantly longer for two or three injections versus one icatibant injection (p < 0.0001 and p < 0.05, respectively). Multivariate logistic regression analysis identified sex, attack severity, and laryngeal attacks as significantly correlated with reinjection (all p ≤ 0.05). These factors did not remain predictors for reinjection when two outlier patients with distinct patterns of icatibant use were excluded.CONCLUSIONS: In this real-world setting, most HAE attacks resolved with one icatibant injection. There was no distinct profile for patients or attacks that required reinjection when outliers with substantially different patterns of use were excluded. Because new attacks were not distinguished from the recurrence of symptoms, reinjection rates may be slightly higher than shown here. Clinical trial identifier: NCT01034969.

Bone mass is primarily genetically determined, but exogenous factors also play a major role. The prevention of osteoporosis can start from childhood, and optimal achievement of peak bone mass during childhood and adolescence is important in order to minimise future fracture risks. Chronic inflammatory diseases can have a detrimental effect on bone mass, by means of several mechanisms. Different diagnostic methods for detection and monitoring of osteoporosis are in use or under investigation. The role of calcium and vitamin D supplementation for the prevention and treatment of osteoporosis associated with paediatric rheumatic diseases remains to be established. New treatments such as bisphosphonates and calcitonin are now available, although their use in the paediatric age has been limited.

Resume Introduction. – Le syndrome de Sjogren est la plus frequente des maladies auto-immunes. Les manifestations neurologiques sont polymorphes et parfois revelatrices. L’epilepsie et l’atteinte du systeme nerveux autonome sont moins connues et probablement sous estimees. Exegese. – Nous rapportons le cas d’une patiente qui a presente un syndrome de Sjogren avec atteinte neurologique diffuse comportant des troubles moteurs, une dysautonomie et une epilepsie. Conclusion. – Le syndrome de Sjogren doit etre systematiquement recherche devant tout tableau neurologique complexe et notamment en presence d’une dysautonomie.

Subarachnoid hemorrhage is the most common cause of cerebral salt wasting syndrome. There are few reports of this condition in infectious meningitis. We describe a patient with hyponatremia and bacterial meningitis. Hyponatremia rapidly improved after administration of sodium chloride. The purpose of this report is to alert clinicians to the fact that hyponatremic patients with central nervous system disease do not necessarily have a syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but may have cerebral salt wasting syndrome. By contrast with SIADH, the treatment requires saline administration.

INTRODUCTION Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional. EXEGESIS We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted. CONCLUSION The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.

Resume Afin d'etudier la relation entre d'une part la presence de manifestations cliniques evocatrices d'allergie ou les resultats de tests cutanes vis-a-vis des pneumallergenes et, d'autre part, le niveau d'immunodeficience clinique et biologique au cours de l'infection par le VIH, une etude transversale a ete mise en place. Pour chaque patient, un entretien standardise sur les manifestations cliniques evocatrices d'allergie (MCEA) recentes, des tests cutanes a six pneumallergenes communs (pricks) et un dosage des IgE totales etaient realises. Parmi les 115 patients inclus, la moyenne des lymphocytes CD4+ (CD4) etait de 214,7/mm3 (etendue: 0-1 328/mm3). Des MCEA etaient retrouvees chez 8,8 % des sujets ayant un nombre de CD4 inferieur ou egal a 50, chez 30 % des sujets ayant des CD4 entre 51 et 200, chez 36 % des sujets ayant des CD4 entre 201 et 350 et chez 11,5 % des sujets ayant plus de 350 CD4 (p=0,03). Pour les patients ayant entre 51 et 350 CD4, le risque de presenter des MCEA etait 4,8 fois plus eleve que pour les autres patients (intervalle de confiance a 95 %=1,7–13,5) (p=0,003). Il n'y avait pas de difference de prevalence des tests cutanes positifs selon le niveau de CD4. Les MCEA sont plus frequentes quand le taux de CD4 est compris entre 51 et 350, evoquant un terrain allergique acquis au cours de l'infection par le VIH. Le mecanisme physiopathologique expliquant la moindre frequence des MCEA quand l'immunodepression est profonde (CD4 inferieurs ou egaux a 50) reste a preciser.

Resume Nous rapportons 2 cas d'atteinte nasosinusienne a Scedosporium apiospermum, l'une de la fosse nasale, l'autre du sinus maxillaire. L'evolution s'avere variable selon le terrain immunitaire. Elle est simple chez le sujet sain (cas 1), avec guerison totale par traitement chirurgical. Elle est compliquee d'une extension locale avec symptomatologie pseudotumorale et lyse de la cloison intersinusonasale chez le sujet immunodeprime (cas 2), le traitement chirurgical precoce associe a un antifongique specifique in situ assurant la guerison locale apparente, le patient decedant quelques mois plus tard. La revue de la litterature confirme le potentiel invasif de ce champignon qui sur un terrain immunodeprime (lie a un traitement immunosupresseur ou a une affection debilitante) devient un opportuniste redoutable. La culture du prelevement sur milieu de Sabouraud permet d'une part le diagnostic mycologique precis de l'affection, d'autre part la realisation d'un antifongigramme, le miconazole (Daktarin) etant le plus regulierement efficace. Alors que, chez un sujet sain, le traitement de l'atteinte nasale ou sinusienne est seulement chirurgical, celui-ci doit etre associe, sur tout terrain immunodeprime, a un traitement medical antifongique complementaire.

Subarachnoid hemorrhage is the most common cause of cerebral salt wasting syndrome. There are few reports of this condition in infectious meningitis. We describe a patient with hyponatremia and bacterial meningitis. Hyponatremia rapidly improved after administration of sodium chloride. The purpose of this report is to alert clinicians to the fact that hyponatremic patients with central nervous system disease do not necessarily have a syndrome of inappropriate secretion of antidiuretic hormone (SIADH), but may have cerebral salt wasting syndrome. By contrast with SIADH, the treatment requires saline administration.

Resume Introduction Le cadmium est une substance cancerogene connue impliquee dans des neoplasies bronchiques et prostatiques. C'est aussi un produit nephrotoxique et sa responsabilite dans la survenue de cancers de rein a ete suspectee mais tres peu de cas etayant cette hypothese ont ete publies dans la litterature. Exegese Nous rapportons l'observation d'une femme de 59 ans soumise pendant 30 ans a une exposition professionnelle au cadmium. Elle a presente un carcinome epidermoide du rein et les dosages plasmatiques du cadmium effectues alors ont confirme l'existence d'une contamination chronique puis, 3 ans plus tard, un cancer bronchique. Conclusion La notion d'une exposition prolongee au cadmium, sa nephrotoxicite, la rarete des cancers epidermoides du rein et la survenue d'un autre cancer induit nous ont fait retenir la responsabilite de cette substance dans la neoplasie renale de notre malade. Cette hypothese a ete admise par la Commission de reconnaissance des maladies professionnelles.

Familial occurrence of progressive systemic sclerosis is unusual. The occurrence of conjugal scleroderma is exceptional.We report here a case of systemic sclerosis in a wife and husband who both developed the onset of illness within a 10-year period. Solvent exposure was noted.The etiology of systemic sclerosis remains unknown. Environmental factors may play role in its pathogenesis.

Bone mass is primarily genetically determined, but exogenous factors also play a major role. The prevention of osteoporosis can start from childhood, and optimal achievement of peak bone mass during childhood and adolescence is important in order to minimise future fracture risks. Chronic inflammatory diseases can have a detrimental effect on bone mass, by means of several mechanisms. Different diagnostic methods for detection and monitoring of osteoporosis are in use or under investigation. The role of calcium and vitamin D supplementation for the prevention and treatment of osteoporosis associated with paediatric rheumatic diseases remains to be established. New treatments such as bisphosphonates and calcitonin are now available, although their use in the paediatric age has been limited.

Primary Sjogren syndrome is considered as the most frequent connective tissue disease. Neurological complications may affect the peripheral nervous system and to lesser extent the central nervous system. Autonomic system nervous dysfunction and epilepsy have been rarely reported.We present on case of Sjogren's syndrome with epilepsy and autonomic nervous system dysfunction. The epilepsia respond to valproate.Autoimmune investigations for Sjogren's syndrome should be initiated in any patient presenting with unexplained neurologic manifestations.

Hereditary hemochromatosis is inherited as an autosomal recessive trait. It is characterized by increased absorption of dietary iron. The association between pernicious anaemia and hereditary hemochromatosis has never been described.We report a case of paradoxical association of hereditary hemochromatosis and pernicious anaemia.It seems that pernicious anaemia may prevent manifestations of hemochromatosis. We suppose that this protective role is due to atrophic body gastritis with iron malabsorption.

Mycobacterium marinum has been recognized for some forty years. It generally occurs after trauma in a patient who manipulates tropical fish living in an aquarium.We report the case of an exotic fish seller who developed M. marinum nodular lymphangitis after being bitten by one of the fish.Nodular lesions occurring after trauma in subjects living in France who are in contact with exotic fish suggest the diagnosis of M. marinum. Nodular lymphangitis is seen in about 50% of the cases. Prolonged antibiotic therapy is required. Several antibiotics are effective including minocycline used in our case for 2 months after complete healing of the wound. Surgery is exceptionally required.

Background: The safety and efficacy of sublingual-swallow immunotherapy (SLIT) in rhinitis caused by house-dust mite were evaluated in a double-blind, placebo-controlled study including 75 patients for 24 months. Methods: Patients received either placebo or SLIT with a standardized Dermatophagoides pteronyssinus (D.pt.) –D. farinae (D.f.) 50/50 extract. The mean cumulative dose was 90 000 IR, equivalent to 2.2 mg of Der p 1 and 1.7 mg of Der f I. Symptom and medication scores were assessed throughout the study. Exposure to house-dust mite, skin sensitivity, and serum specific IgE and IgG4 were assessed before starting treatment and after 12 and 24 months. Results: Seventy-two patients (36 active–36 placebo) were eligible for intent-to-treat analysis. Thirty-six patients dropped out of the study. The number of patients who dropped out due to lack of efficacy was eight out of 37 (21.6%) in the active treatment group compared to 15 out of 38 (39.5%) in the placebo group (chi-square=2.81, P=0.09). Total symptom and medication scores decreased significantly after 12 and 24 months (P

A 30-year-old woman developed progressive left sided hemiparesis with intracranial hypertension signs. CT scan and MRI showed a large temporo parietal cystic mass with marked surrounding edema. Surgical excision was performed, and histological analysis revealed an inflammatory granuloma. No disease elsewhere was found and all classical causes of granulomas such as tuberculosis, toxoplasma, fungus infections, inflammatory diseases, lymphomas and cancers were excluded. No treatment was administered and she remained neurologically stable for two years. Afterwards, she developed chronic meningo-encephalitis, hypothalamic-pituitary dysfunction and hydrocephalus requiring decompression. Sarcoidosis was suspected, a steroid therapy was initiated, she gradually improved and a ventricular biopsy confirmed this diagnosis. Nervous system lesions complicate the course of sarcoidosis in 5 to 15% of patients and most commonly involve the cranial and peripheral nerves. CNS involvement is typically meningeal with a predilection for the hypothalamic region. Intracranial mass lesions are rare and their occurrence in the absence of disease elsewhere is still more unusual. Three presentations have been described: an isolated intra parenchymatous mass, multiples nodules, and subdural plaques, that can be mistaken for meningiomas, gliomas or metastases. When systemic manifestations of sarcoidosis are absent, the diagnosis is difficult, and Gd-enhanced MRI is now considered the diagnostic method of choice. However brain biopsy is sometimes necessary. Corticosteroids are the mainstay of therapy. Immunosuppressive agents are also used and brain irradiation has been tried in some refractory cases. Surgical approach may be indicated to establish tissue diagnosis, to perform decompression and to remove brain tumors.

The hyper-IgD syndrome (HIDS) is an autosomal recessively inherited auto-inflammatory syndrome, caused by deficient enzyme activity of mevalonate kinase (MVK), an enzyme in the isoprenoid pathway. Patients present with a long history of recurrent fever attacks, accompanied by abdominal pain, skin lesions, lymphadenopathy and arthralgia. The attacks last about 3–7 days followed by a spontaneous remission, only to recur after a symptom-free period of 4–6 weeks. Factors known to precipitate an attack are infection, trauma, vaccination, and psychological stress. Laboratory examination at the time of attacks reveals a vigorous acute phase response with leucocytosis, raised sedimentation rate, high serum concentrations of C-reactive protein and increased mevalonic acid urinary levels. Almost all patients have an elevated polyclonal immunoglobulin D. Until now, treatment of HIDS patients is largely supportive and very difficult. Various anti-inflammatory drugs have failed to suppress the fever attacks [1].

Cadmium is a carcinogenic substance and bronchial and prostatic cancer may be readily identified as professional diseases. It is also a nephrotoxic substance but renal carcinoma are rarely imputed to this kind of intoxication.We report the observation of a 59-year-old woman with a long unprotected professional exposure to cadmium. She developed an epidermoid carcinoma of the kidney and later of the lung. Levels of cadmium in blood samples were abnormally high, confirming the existence of a chronic impregnation.We have judged cadmium to be responsible for her renal disease: the fact of a long unprotected professional exposure, the scarcity of renal epidermoid carcinoma, the well established nephrotoxicity of this substance and the association with other cancers, appear to us as convincing evidence, and the kidney cancer of our patient has been considered a professional disease.

A cross sectional survey was set up to study the relation between the prevalence of allergic-type reactions during HIV infection course. For each patient, a standardized interview about recent allergic-type manifestations (RATM), skin prick-tests to six common airborne allergens, IgE serum level were done. Among the 115 included patients, the mean CD4 lymphocyte count (CD4) was 214.7/mm3 (range: 0-1328/mm3). RATM were found in 8.8% of patients with CD450, in 30% of patients with CD4 between 51 and 200, in 36% of patients with CD4 between 201 and 350 and in 11.5% of patients with CD4350 (p = 0.03). The risk of presenting RATM was 4.8 times (95% confidence interval = 1.7-13.5) higher in patients with CD4 between 51 and 350 than in other patients (p = 0.003). The proportion of positive prick-tests did not significantly vary according to the level of CD4. The increased frequency of RATM in patients with CD4 between 51 and 350/mm3 could be due to an allergic predisposition acquired during the course of HIV infection. The mechanisms explaining the reduced frequency of allergic manifestations when immunodeficiency is profound (CD450/mm3) remain to be explained.

A 52 year-old man was hospitalised for acute renal failure with thrombocytopenia and hemolytic anemia without oliguria. A haemolytic-uremic syndrome was diagnosed and prostacyclin infusion was started. Twenty-four hours later, the renal function improved as well as thrombocytopenia and anemia. Recovery occurred after 11 days of treatment. Haemolytic-uremic syndrome treatment is not well codified: plasmaphoresis, fresh frozen plasma, transfusions showed inconstant efficiency and data about prostacycline treatment are rare and often contradictory. Multicentric studies must be started in order to determine the precise benefit of this treatment.

The peritoneal equilibration test (PET) is routinely performed in adults treated with chronic peritoneal dialysis to assess the peritoneal transport rate and to optimize treatment prescription. Only a few and not well-standardized studies on the PET have been reported in children. Twenty-six PET's were performed in 16 children, mean age 10.9 +/- 4.9 years, mean body weight (BW) 26.8 +/- 11.9 kg, treated with nightly intermittent peritoneal dialysis (NIPD). The PET was performed according to Twardowski. Forty mL/kg BW of 2.27% glucose solution were infused at a rate of 10 mL/kg BW every 2 minutes. In the simplified test, dialysate samples were taken at dwell times 0, 2 and 4 hours for glucose and creatinine. A blood sample was taken after a 2-hour dwell time for the same parameters. The standard PET (8 patients) consisted of dialysate samples at 0, 15, 30, 60, 120, and 240 minutes and blood samples at 0 and 240 minutes. Mean D/P ratio for corrected creatinine and D/D0 ratio for glucose at 2 hours were 51.6 +/- 11.6 and 50.4 +/- 9, respectively; at 4 hours 69.6 +/- 12 and 34.4 +/- 9.8, respectively. There was good correlation between D/P creatinine and D/D0 glucose at 4 hours (p0.0001). Patients were classified as high (3 cases), high-average (5), low-average (6), and low (2) transporters. A statistically significant difference was found between the curves obtained by the simplified PET and those of the standard PET in the first hour (r = 0.66; p0.05). In conclusion, the PET, modified for use in children, gave reliable and reproducible results.(ABSTRACT TRUNCATED AT 250 WORDS)

We report two cases of nasosinusal infection caused by Scedosporium apiospermum and involving the nasal fossa in one case and the maxillary sinus in the other. The course of the disease varied according to the patient's immune status. The otherwise healthy patient (case n. 1) was completely cured by surgery, whereas the immunosuppressed patient (case n. 2) had local extension with pseudotumoral symptoms and lysis of the inter-sinusonasal septum; an early surgical treatment combined with local administration of a specific antifungal agent resulted in an apparent cure, but the patient died a few months later. A review of the literature confirms the invasive potential of this fungus which in immunosuppressed patients (by therapeutic immunosuppressants or by a debilitating disease) becomes a dangerous opportunistic organism. Cultures on Sabouraud's medium provide an accurate diagnosis and enable antifungal drugs to be tested, miconazole being the most regularly effective of them. In healthy subjects surgery is the sole treatment of nasal or sinusal lesions, while in all immunocompromised patients it must be combined with an antifungal treatment.

Aspirine-sensitive asthma (ASA) is a disease defined only by clinical criteria. It is an intrinsic asthma related to a hypersensitivity to aspirin. The illness is linked to abnormalities in platelet and macrophage arachidonic acid metabolism. We assessed in vitro the platelet chemoluminescence (CL) and lymphocyte proliferative response of ASA patients. We observed that platelets from patients and control do not generate any CL in the presence of aspirin. Concerning the proliferative response of lymphocytes, the in vitro effect of aspirin depends upon the origin of the lymphocytes tested. Thus aspirin clearly enhances the proliferative response of lymphocytes from normal subjects but diminishes the thymidine uptake by lymphocytes from ASA patients. This discrepancy in the in vitro response of lymphocytes from normal subjects and patients might be useful for in vitro diagnosis of ASA.