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Biomedical subjects

A Lachaux

Publications and source records attributed to A Lachaux.

At least 55 records · Page 3Linked to original sources
PubMed1996

Idiopathic disseminated bacillus Calmette-Guérin infection: a French national retrospective study.

OBJECTIVE: Disseminated bacillus Calmette-Guérin (BCG) infection after inoculation of live vaccine is considered to result from impaired immunity of the child. However, in half of the cases, regarded as idiopathic, no well-defined immunodeficiency condition can account for the infection. The objective of the present study is to report the prevalence, clinical features, associated infections, and outcomes of children with idiopathic disseminated BCG infection. DESIGN: National retrospective survey during the period from 1974 through 1994 in France. SETTING: All neonatology and pediatrics units in primary care and referral centers throughout France. PATIENTS: Data were collected from 595 (82%) of 721 units, 377 (93%) of 407 centers, and 320 (93%) of 345 cities. Selection criteria included BCG infection, dissemination to at least two areas beyond the inoculation site, and no well-defined immunodeficiency condition. Sixteen children (8 girls and 8 boys), born to families unrelated to each other but often consanguinous (5 of 16) or abroad (5 of 16). RESULTS: The minimal prevalence rate was estimated at 0.59 cases per 1 million vaccinated children born in France. Clinical features included fever and cachexia, disseminated BCG infection to lymph nodes (15 of 16), skin (13 of 16), soft tissues (11 of 16), lungs (11 of 16), spleen (11 of 16), liver (11 of 16), and bones (9 of 16). Eight children had associated or subsequent severe opportunistic infection (50%), with either nontyphi Salmonella enterica serotypes (7 of 16) or Mycobacterium abscessus (1 of 16). The outcome was poor: 8 children (50%) died; the cause of death was BCG infection for most children (7 of 8); 8 survived until the last follow-up (50%). CONCLUSIONS: Idiopathic disseminated BCG infection is a rare but severe complication of BCG vaccination. The infection probably results from an as yet unknown genetically determined immunodeficiency condition that affects the killing of intracellular bacteria such as BCG and Salmonella.

BCG Vaccine↗
PubMed1996

A longitudinal study of a harlequin infant presenting clinically as non-bullous congenital ichthyosiform erythroderma.

Over the past 8 years, we have followed a child born as a harlequin baby, who survived due to treatment with retinoids. His condition evolved clinically towards the erythrodermic form of lamellar ichthyosis (non-bullous congenital ichthyosiform erythroderma, NBCIE). According to ultrastructural and biochemical criteria, our patient originally presented with type II harlequin ichthyosis. Investigations showed an abnormal keratinosome structure and extrusion, a keratin pattern characteristic for epidermal hyperproliferation, and an absence of conversion of profilaggrin to filaggrin. Persisting keratinocyte hyperproliferation, associated with the presence of a dermal infiltrate, is in agreement with the present clinical picture of severe NBCIE. However, abnormal lamellar body production and defective filaggrin processing, which is not one of the diagnostic criteria of NBCIE, persist in the patient's skin. Further studies of the epidermal lipid composition, and of possible mutations of the keratinocyte transglutaminase gene performed on epidermal cell cultures of harlequin ichthyosis, will be necessary before type II harlequin ichthyosis can be accepted as an extremely severe form of NBCIE.

Etretinate↗
PubMed1996

[Waldman's disease. Primary intestinal lymphangiectasis].

INTRODUCTION: Primary intestinal lymphangiectasias are often associated with lymphoedema. OBSERVATION: The diagnosis was performed at 4 months when Maxime presented with lymphoedema, diarrhea, hypoprotidemia and hypolipemia. Duodenum biopsies revealed intestinal lymphangiectasias. An hyperprotidic and low fat diet, medium chain triglyceride-supplemented and an elastic contention allowed a decline of the oedemas. DISCUSSION: We report one case of Waldman's disease. It shows very well the typical circumstances of diagnosis in this disease and the two types of oedema (lymphoedema and hypoprotidic oedema).

Dietary Proteins↗
PubMed1995

Complications of percutaneous liver biopsy in infants and children.

UNLABELLED: In this study, 144 consecutive percutaneous liver biopsies performed with a 1.6 mm Menghini needle, during a 2-year period were reviewed. All the children were aged under 15 years, 57 patients less than 1 year and 87 more than 1 year. All biopsies were adequate and the mean number of portal tracts examined was 17.6 per biopsy (14.3 in patients weighing less than 10 kg and 19.1 in the others). There were no deaths and we observed only bleeding complications. In patients with normal coagulation (128 cases), 1 bleeding requiring transfusion occurred; and in patients with abnormal coagulation (16 cases), we observed 2 bleeding cases requiring transfusion. CONCLUSION: Percutaneous liver biopsy can be performed with 1.6 mm needles in children. For increased safety, ultrasound-guided biopsies are recommended.

Adolescent↗
PubMed1995

[Percutaneous endoscopic gastrostomy in pediatric practice].

Percutaneous endoscopic gastrostomy (PEG) has become a good alternative to nasogastric tube feeding and surgical gastrostomy. The procedure requires two practitioners, one of them being an endoscopist. The technique can be performed either under local or general anesthesia, at bedside or in an operating room or endoscopic room, using a one step button or first setting of a catheter and secondly a button, by pull technique in which the button is pulled through esophagus or push technique were the button is pushed through the parietal wall. Its is used to deliver enteral nutriments in patients who need tube feeding. In paediatrics practice, morbidity rate related to PEG is less than 1% and morbidity less than 10%. PEG appears as a simple, safe, practical, esthetic and cheap procedure.

Child↗
PubMed1995

[Early manifestations of Tangier disease].

BACKGROUND: The diagnosis of Tangier disease in childhood is based on the specific aspect of tonsils or by screening relatives of affected subjects. CASE REPORT: A moderately enlarged liver associated with splenomegaly was found upon routine physical examination of a 3 month-old breast-fed boy, born in Turkey from consanguineous parents. Laboratory studies disclosed moderate increase in serum alanine aminotransferase activity (ALAT 52 UI/l, N < 30). The diagnosis of Tangier disease was confirmed by studies of plasma cholesterol and apolipoprotein A. By 8 months of age, the patient had enlarged orange tonsils. Small cervical, axillary and inguinal lymphadenopathies were present. The tonsilar and adenoidal tissues were removed at 18 months of age because the patient suffered from chronic airway obstruction. Colonoscopic examination revealed tiny flat orange spots, 1 to 2 mm in diameter, scattered throughout the rectosigmoidal and colonic mucosa. Survey of the family led to the discovery of one sister, with asymptomatic apolipoprotein Al deficiency and a normal sister, while the parents were heterozygotes for Tangier disease. CONCLUSION: Enlarged liver associated with a moderate level in serum aminotransferase may be an early manifestation of Tangier disease in infants. Rectosigmoidal and colonic lesions may be convenient for biopsy when tonsillectomy is not indicated.

Hepatomegaly↗
PubMed1994

[Surveillance of children with liver transplantation].

The benefit of liver transplantation in children with end-stage liver disease is now well established. About 80% of the children are alive 5 years after liver transplantation. These good results are obtained not only because of the improvements of the surgical techniques, but also secondary to the reffinements of the follow-up of the patients. In the intensive care unit, clinical, biological and radiological cares must be permanent, allowing graft function assessment, correction of the haemodynamic disturbances, initiation of specific therapies and the research of any complication which must be promptly treated. The main surgical complications are intraperitoneal haemorrhage, biliary and vascular failures. Bacterial and viral infections, rejection represent frequent medical complications. Following the discharge from the intensive care unit, the children are checked daily until patient and graft conditions reach normal status. As time goes, the risk of complications is decreasing, but does not disappear, underlying the need for regular and long follow-up. In almost children, liver transplantation offers normal growth and scooling.

Age Factors↗
PubMed1994

CD23/CD21 interaction is required for presentation of soluble protein antigen by lymphoblastoid B cell lines to specific CD4+ T cell clones.

Previous studies have documented a role for membrane-bound CD23 (the low affinity Fc epsilon RII) in presentation of alloantigens by B cells. The aim of the present study was to examine the involvement of cell surface CD23 in presentation of more conventional soluble protein antigens to T cells. We show that antibodies to CD23 and to its lymphocyte-associated second ligand, CD21, inhibit presentation of the cow's milk allergen casein, by autologous CD23+CD21+ B-EBV cell lines to casein-specific HLA-DP-restricted CD4+ T cell clones obtained from patients with either reaginic or enterophatic forms of cow's milk protein intolerance. Maximal inhibition was achieved when the antibodies were added at the initiation of the culture. The absence of specific inhibition by an anti-DR alpha monoclonal antibody (mAb) argues against a steric hindrance phenomenon impeding access of the T cell receptor to major histocompatibility complex class II molecules. Rather, anti-CD23 and anti-CD21 mAb-induced inhibition of antigen presentation seems to affect at least partly, heterotypic conjugate formation through CD23/CD21 interaction. Double immunofluorescence labeling of the T cell clones and antibody inhibition of T/B conjugate formation shows that functional CD23 and CD21 molecules are induced on T cells following contact with B-EBV cell lines. Taken together, these data indicate that CD23/CD21 interactions between T and B cells are required for presentation of soluble protein antigens by B-EBV cell lines to specific CD4+ T cells. The potential implications of these findings for allergen-specific T cell activation are discussed.

Antigen-Presenting Cells↗