Pseudomonas aeruginosa and cystic fibrosis: correlation between exoenzyme production and patient's clinical state.

In this study, we investigated the correlation between the production by Pseudomonas aeruginosa isolates of four exoenzymes (protease, elastase, neuraminidase, and phospholipase C (PLC)) and the clinical state of cystic fibrosis (CF) patients. We studied 212 P. aeruginosa isolates from 22 CF patients chronically infected with this bacterium. Patients were classified into three clinical groups according to a modified Shwachman-Kulczycki-Khaw (SKK) scoring system. The production of enzymes by isolates from patients in the three populations was analyzed and compared using four statistical tests: chi-square, Mann-Whitney U, principal component analysis, and discriminant analysis. Isolates from patients with excellent or good clinical status (group I, SKK score >/=71) had higher elastase and neuraminidase activities than isolates from the other patients. In contrast, PLC activity, a common characteristic of CF isolates, was higher in isolates from patients with poor or weak clinical status (group III, SKK score

Genetic diversity of Pseudomonas aeruginosa strains isolated from patients with cystic fibrosis revealed by restriction fragment length polymorphism of the rRNA gene region.

The restriction fragment length polymorphism patterns of rDNAs from Pseudomonas aeruginosa strains isolated from the respiratory tracts of patients suffering from cystic fibrosis were obtained to evaluate the genetic polymorphism of this population of strains. Eighty-seven P. aeruginosa strains isolated from 87 patients from diverse areas of France and the ATCC 10145 strain were examined. Four restriction enzymes were used: BamHI, ClaI, EcoRI, and PstI. Forty-nine strains (56%) were in the three most frequent ribotypes (ribotypes R1 to R3). In addition, hierarchical clustering analysis of the data showed that 71 of the 88 strains (81%) clustered at a distance of less than one-third of the greatest distance observed in the total population. This indicates that clinical isolates implicated in the pathology of cystic fibrosis present a low degree of heterogeneity of rRNA operons, in contrast to the heterogeneity of strains of P. aeruginosa isolated from patients with various other pathologies. This relative homogeneity of rRNA genes was observed independently of the clinical status of the patient and the colony morphology.

[Insulin resistant diabetes, acanthosis nigricans and Bartter's syndrome, a fortuitous association?]. / Diabète insulinorésistant, acanthosis nigricans et syndrome de Bartter, une association fortuite?

The authors have followed during 10 years a girl with Bartter's syndrome who developed severe insulin resistance with acanthosis nigricans. In this rare association, hypokalemia and renal failure did not appear to be relevant factors triggering the onset of diabetes. The therapeutic difficulties in this case have still not been resolved.

["Saber-cut" scleroderma and Parry-Romberg facial hemiatrophy. Nosologic problems. Neurologic complications]. / Sclérodermie "en coup de sabre" et hémi-atrophie faciale de Parry-Romberg. Problème nosologique. Complication neurologique.

We report two cases of gradual facial hemiatrophy (Parry-Romberg syndrome). The first patient, an adolescent girl under treatment for hypothyroidism, presented with a very severe form combining advanced facial hemiatrophy, epilepsy with hemi-generalized seizures and hemiatrophy of the brain. The second patient was a girl who presented with localized scleroderma resembling a saber injury, homolateral cerebral atrophy and contralateral hemiparesis. Our two cases are evidence in support of a close relationship between saber injury-like scleroderma and the Parry-Romberg syndrome.

[Clinical and etiological aspects of erythema multiforme. A propos of 40 cases]. / Aspects clinique et étiologique de l'érythème polymorphe. A propos de quarante observations.

The authors analyse 40 cases of erythema multiforme (including twenty children under fifteen) seen over a five-year period at the Sick Children's Hospital in Bordeaux, Bullous erythematous target lesions of the skin were associated, in most cases, with pluri-orificial ulcerations on the mucous membranes and, less frequently, with more or less severe systemic or visceral symptoms. Borderline cases were observed, associating features of erythema multiforme simplex and of Lyell disease with variable degrees of dermoepidermal blistering and epidermal necrosis. Infection (32.5% of cases) is a more common etiology in children than in adults; the main pathogens are herpes simplex virus, vaccinia pox virus, and Mycoplasma pneumoniae. Drug-induced forms (37% of cases), which are more often seen in adults than in children, are usually due to sulfonamides or antiinflammatory agents. In 30% of cases, no etiology could be demonstrated. Attention is drawn to the frequency of facial vespertilial erythema, as well as the possible occurrence of severe conjunctival sequellae. The connections between erythema multiforme, fixed drug-induced eruptions, and Lyell disease are discussed: only the last, which implies dermoepidermal cleavage, can be categorized with erythema multiforme. The staphylococcal scalded skin syndrome, in which the epidermolysin of Staphylococcus aureus type II 71 is responsible for a superficial cleavage, proceeds from entirely different mechanisms and should be regarded as totally distinct from erythema multiforme.

[Pilomatrixoma (Malherbe's calcifying epithelioma). Apropos of 13 cases observed in 6 years]. / Le pilomatricome (épithélioma calcifié de Malherbe). A propos de treize cas observés en six ans.

Thirteen pilomatrixomas were seen over a six year period. These small benign cutaneous tumors are infrequent though not exceptional and occur mainly in children (mean age six years). The tumor's hard consistency, irregular shape and frequently cephalic location (nearly 50% of cases) are suggestive. Diagnosis is established upon histological examination which demonstrates irregularly shaped clusters of basophilic epithelial cells; these develop into highly suggestive momified areas with shadow cells and secondary calcification. Attention is drawn to the presence of hemorrhagic modifications in recent tumors and telangiectasia. Pilomatrixomas must be considered as suborganoid hamartomas arising from the primary hair-matrix germ cells. The treatment is minimal surgical excision.

[Urticarial rash in cow-milk allergy (author's transl)]. / Manifestations urticariennes chez le nourrisson au cours de l'intolérance aux protéines du lait de vache.

Five recent cases of urticarial rash with cow-milk allergy are reported. They all occur at the time of weaning in children who have been sucking during at least three weeks. In three of those cases, digestive manifestations were also found. The histological pattern of the skin showed a vasculitis with deposits of fibrinogen and complement. The steadily high rate of IgE and positive cow-milk RAST in five cases give arguments for reaginic allergy in extra digestive manifestations of cow-milk proteins allergy. The treatment is the suppression of cow-milk and of any products which might contain milk proteins. Our cases had a favorable evolution.

Dermatomiositis infantil: apreciacion de su evolucion por captacion muscular de difosfonato de metileno. / Child dermatomyositis: estimation of its evolution by means the muscular uptake of methylene diphosphonate

La clinica y las exploraciones para-clinicas clasicas no alcanzan para evaluar el pronostico en un caso de dermatomiositis infantil, ni para guiar eficazmente la corticoterapia. Se realizaron tres centellografias con centellometria durante 14 meses, a fin de lograr un abordamiento metabolico de las lesiones musculares. La evolucion se pudo deducir de estos examenes: la fijacion muscular del tecnecio siguio bastante fielmente la evolucion clinica, atestiguando la lesion celular y la calcinosis. Sugerimos la utilizacion de este metodo desde el comienzo de la enfermedad, como elemento de pronostico para guiar la terapeutica

Dermatomiositis infantil: apreciacion de su evolucion por captacion muscular de difosfonato de metileno. / Child dermatomyositis: estimation of its evolution by means the muscular uptake of methylene diphosphonate

La clinica y las exploraciones para-clinicas clasicas no alcanzan para evaluar el pronostico en un caso de dermatomiositis infantil, ni para guiar eficazmente la corticoterapia. Se realizaron tres centellografias con centellometria durante 14 meses, a fin de lograr un abordamiento metabolico de las lesiones musculares. La evolucion se pudo deducir de estos examenes: la fijacion muscular del tecnecio siguio bastante fielmente la evolucion clinica, atestiguando la lesion celular y la calcinosis. Sugerimos la utilizacion de este metodo desde el comienzo de la enfermedad, como elemento de pronostico para guiar la terapeutica

[Scalded skin syndrome in children associated with S. aureus of non phage group II. Report of a case contracted in West Africa (Togo)]. / Syndrome "des enfants ébouillantés" lié à un Staphylococcus aureus n'appartenant pas au groupe phagique II. A propos d'un cas contracté en Afrique de l'Ouest (Togo).

The authors report the case of a 19 m. old girl developing a Lyell syndrome associated with a S. aureus belonging to phage group I-III. A survey of the literature is made: the toxic exfoliation is not a characteristic of S. aureus, phage group II. It seems that there are two different toxins for which some physicochemical features are well known. An immunologic immune deficiency is frequently observed in adults. The child reported, manifested some signs of depressed cellular immunity.