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J L Schlienger

Publications and source records attributed to J L Schlienger.

At least 55 records · Page 3Linked to original sources
PubMed2000

[Adult celiac disease: importance of delay to diagnosis].

OBJECTIVE: Adult celiac disease is a genetically determined condition resulting from intolerance to gluten. This familial disease is associated with HLA B8 DR3 and is characterized by cytotoxic T-lymphocyte reaction in the digestive wall leading to a maladsorption syndrome. Adult celiac disease would be an expression of a silent disease already present in childhood. PATIENTS AND METHODS: We conducted a retrospective study in 19 patients. Diagnosis was established in a department of internal medicine in adulthood. We analyzed histological findings and the clinical history and course. RESULTS: The age curve at diagnosis peaked at 22 years and 67 years. Female sex predominated. Average delay to diagnosis was 3.1 years. The patients were admitted for reasons other than digestive disorders in 17 out of 19 cases. Careful history taking did however disclose subtle digestive signs. The principal manifestations were hematological disorders such as chronic anemia, abnormal liver function tests, and poor control of associated diseases. Type 1 diabetes mellitus was the main associated disease (4/19). Patients with prolonged nutritional deficiencies experienced serious situations. CONCLUSION: Adult celiac disease is a clinical variant suggesting the presence of other non-digestive disorders. Specific antibodies should be assayed in patients with even minimal signs of deficiency in order to identify associated diseases. The principal cause of mortality is digestive T-cell lymphoma. A gluten-free diet is essential to control the course of this chronic disease.

Adult↗
PubMed2000

[Capillary hyperpermeability syndrome of the face].

BACKGROUND: Capillary leak syndrome is a rare disorder, with various clinical features, characterized by episodes of diffuse edema. CASE REPORT: We report the case of a 83 year-old woman with capillary leak syndrome of the face characterized by recurrent episodes of edamata. We found a follicular non-Hodgkin's lymphoma and high levels of plasma interleukin (IL) 2 and IL 6. Regression of edema and remission of hematology disease have been obtained with corticoids and chloraminophene. DISCUSSION: This case illustrates that capillary leak syndrome may present as an edema of the face and may be in some cases considered as a paraneoplastic syndrome. The role of cytokines seems important. This case suggests that corticoids may be useful in the management of neoplasm induced capillary leak syndrome.

Aged↗
PubMed2000

[Alcoholism].

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Alcoholism↗
PubMed2000

[Anemia caused by vitamin B 12 deficiency in subjects aged over 75 years: new hypotheses. A study of 20 cases].

PURPOSE: New hypotheses have recently been developed on vitamin B12 deficiency and the frequently observed occurrence in the elderly subject of food cobalamin malabsorption, i.e., the non-dissociation of B12 and its carrier protein (ND B12), and the possibility of rectifying this imbalance by oral crystalline B12 supplementation. The aim of this study was therefore to confirm these hypotheses in a series of patients aged over 75 years with anemia due to B12 deficiency. METHODS: A retrospective study was carried out over a 5-year period on patients aged over 75 years presenting with megaloblastic anemia (hemoglobin [Hb] < 12 g/dL) and vitamin B12/cobalamin deficiency (B12 < 160 pg/mL). RESULTS: Twenty cases were analyzed. The average age of the patient population was 82.5 +/- 6 years, and the F/M sex ratio was 1:2. Mean Hb levels were 7.9 +/- 2.4 g/dL, mean serum B12 levels were 83 +/- 24 pg/mL, and mean homocysteinemic levels were 35 +/- 27 mumol/L. The diagnosis was as follows: food cobalamin malabsorption/ND B12 (n = 10), Biermer's disease/pernicious anemia (n = 5), malabsorption due to pancreatic insufficiency (n = 1), and low dietary B12 levels (n = 1). Disorders associated with ND B12 were: atrophic gastritis and Helicobacter pylori infection (n = 6), antacid or biguanide intake (n = 3), alcohol abuse (n = 2), or idiopathic syndrome (n = 2). In the patients who were followed up (n = 10), i.m. (n = 5) or oral (n = 5) administration of crystalline B12 resulted in the correction of hematological abnormalities. CONCLUSION: In the elderly subject, food cobalamin/ND B12 malabsorption appears to be the main cause of B12 deficiency, and is frequently associated with atrophic gastritis. In these cases, administration of oral crystalline B12 may be an efficient means of treating this disorder.

Aged↗
PubMed2000

[Do hematopoietic growth factors have a role in the treatment of drug-induced agranulocytosis?].

PURPOSE: Our study was aimed at determining the role of hematopoietic growth factor in drug-induced agranulocytosis. METHODS: Fifty-five cases of drug-induced agranulocytosis were reviewed and subdivided retrospectively into a G-CSF group (n = 15) and an untreated group (n = 40). Mortality and hematological recovery (number of days required for neutrophil counts to exceed 1.5 x 10(9)/L) were studied in the two groups. RESULTS: The mean granulocyte count was 0.09 x 10(9)/L. All patients presented infection. In the G-CSF group, no mortality (0% versus 5%, P = 0.85) and a shorter recovery time (8.1 versus 9.5 days P = 0.39) were observed. No significant difference between the two groups was observed in either the duration of antibiotic therapy (9.3 days in the G-CSF group versus 10.1 days in the untreated group, P = 0.51) or duration of hospitalization (10 days in the treated group versus 11 days in the G-CSF group, P = 0.46). CONCLUSION: Our results as well as a literature review indicate that G-CSF could decrease the time to hematological recovery and perhaps reduce mortality. However, the exact role of hematopoietic growth factors requires further investigations.

Aged↗
PubMed2000

Concordance of eight kits for antithyroid peroxidase autoantibodies determination.

Numerous methods are proposed to quantify antithyroid peroxidase autoantibodies. No standardization exists but most assays use the standard MRC 66/387 with a calibration factor. Costs of the tests vary between the different kits. We evaluated the concordance of eight peroxidase autoantibodies assay kits in two centres, using a panel of sera from 269 subjects: controls (n=100), patients with autoimmune thyroid disease (n=77; Graves' disease, Hashimoto's thyroiditis), patients with non-autoimmune thyroid disease (n=69; nodular goiter, differentiated thyroid carcinoma) and individual sera with thyroglobulin antibodies only (n=23). The concordance between the eight methods was high, ranging from 88.3% to 98.8% with the total panel of sera. The majority of assays demonstrated high diagnostic performance. We encountered some false-positive results at borderline positive levels, and the nonrecognition of some sera by competitive assays.

Autoantibodies↗
PubMed2000

[Unusual cause of chronic iron-deficiency anemia and insulin resistance: cholesterol embolism].

BACKGROUND: Cholesterol embolism disease (CED) is an extremely polymorphous clinical entity. Rare reports mention digestive or endocrine manifestations. CASE REPORT: A 73 year-old female patient developed CED disclosed by iron deficiency anemia with chronic digestive bleeding associated with major insulin resistance. DISCUSSION: Solitary iron deficiency anemia as the inaugural sign of CED is exceptional. Rare reports in the literature suggest it is related to polymorphous nonspecific lesions (ulcers, pseudotumoral lesions...) leading to chronic bleeding. Careful histopathology study of the lesions of fragments of healthy mucosa provides the correct diagnosis showing biconcave and optically empty slits. Insulin resistance has not been reported although prior diabetes is often mentioned as a predisposing factor for atherosclerosis. In our case, the role of CED was not determined, but pancreatic or inflammatory involvement can be envisaged.

Aged↗
PubMed2000

[Hypersensitivity syndrome : a rare but severe adverse effect of antithyroid drugs].

We report the case of a female patient with a severe hypersensitivity syndrome caused by carbimazole. She was treated for Graves' disease for a few weeks when she was referred for skin rash, increased liver enzymes and hypereosinophilia. No immunological abnormalities were detected. Discontinuation of carbimazole resulted in quick recovery. Pathogenesis of the hypersensitivity syndrome is discussed. Antithyroid drugs are rarely involved in the syndrome but cases with vasculitis have been described. In our patient, the role of excessive dose is possible.

Aged↗
PubMed2000

[Guillain-Barré syndrome and Mycoplasma pneumoniae infection].

Guillain-Barré syndrome subsequent to Mycoplasma pneumoniae is uncommon and difficult to confirm. We report one case. Clinical presentation and therapy were unremarkable. The interest of antibiotics (preventive or curative) is unresolved. The pathophysiology remains hypothetical but Mycoplasma pneumoniae and some surface gangliosides of peripheral nerves share homologous epitopes leading to the production of antibodies. Induced antibodies to ganglioside may cross-react with neural tissues.

Adolescent↗
PubMed2000

[Failure of bone marrow autograft in metastatic malignant adrenalcortical carcinoma].

BACKGROUND: Despite different therapeutic modalities (surgery, hormones therapy, chemotherapy), 5-year survival in patients with malignant adrenocortical carcinoma remains only 10% to 20%. Bone marrow grafts may be proposed to intensify treatment. CASE REPORT: In a 29-year-old patient with adrenocortical carcinoma with metastases to the lung and bone, an adjuvant bone marrow graft using peripheral stem cells was unsuccessful. Recurrence was observed at 3 months and overall survival was 2 years. DISCUSSION: Our case illustrates the feasibility of this approach but also emphasizes the relatively poor results obtains with only 2 years survival (1 year after the bone marrow graft), comparable with other classical modalities reported in the literature.

Adrenal Cortex Neoplasms↗
PubMed2000

[Hypothalamo-pituitary-adrenocortical function during and after steroid therapy: recent data and critical review].

Prolonged corticosteroid treatment is considered to be the main cause of adrenal insufficiency (AI). AI is due to adrenal atrophy caused by the inhibition of ACTH secretion. Risk factors for AI after corticosteroid treatment remain unknown. Various functional tests, particularly the "standard" Synacthene 250 microg, demonstrate frequent abnormal responses in these patients. However, these abnormalities are not predictive of the risk of AI in particular in case of stress. More recent tests (CRH, "low dose" Synacthene) need to be further evaluated in this situation. Several recent studies in animals and in humans have demonstrated that the need for glucocorticoid coverage during a surgical stress has been greatly overestimated and that maintenance of the usual daily dose seems to be sufficient and safe in most patients with minor surgical stress. Revision of guidelines for more important surgical stress or for other medical conditions in patient treated with glucocorticoids is necessary.

Adrenal Cortex Hormones↗
PubMed1999

[Sepsis syndrome and bone marrow granulomatosis after intravesical instillation of BCG].

BACKGROUND: BCG therapy is an effective treatment for superficial bladder carcinoma. Exceptionally, systemic disorders including hypersensitivity reactions or infections may occur. CASE REPORT: A 70-year-old man developed septicemia with bone marrow granulomatosis with a favorable course after intravesicle administration of bacillus Calmette-Guérin for bladder carcinoma. DISCUSSION: High grade fever, septicemia, hepatitis or pulmonary granulomatosis as well as bone marrow involvement are reported in less than 1% of all cases of BCG therapy. The pathogenic mechanisms are complex. Anti-tuberculous drugs are generally given.

Administration, Intravesical↗
PubMed1999

Hepatic and pulmonary metastases from a meningeal hemangiopericytoma and severe hypoglycemia due to abnormal secretion of insulin-like growth factor: a case report.

A 42-year-old woman was hospitalized for severe hypoglycemic coma. She had a voluminous hepatic metastasis and multiple small lung metastases from a meningeal hemangiopericytoma initially operated on 11 years earlier. High blood levels of an abnormal form of insulin-like growth factor type 2 (IGF II) associated with low blood levels of insulin, growth hormone, IGF I, and IGF BP3 were observed. After surgical resection of the liver and pulmonary metastases, serum glucose levels and hormonal abnormalities returned to normal.

Adult↗