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Biomedical subjects

G Cornu

Publications and source records attributed to G Cornu.

At least 91 records · Page 5Linked to original sources

Bone marrow transplantation in five children with sickle cell anaemia.

Five children with severe sickle cell anaemia underwent an HLA compatible allogeneic bone marrow transplantation. In four children the engraftment was rapid and sustained. The fifth child rejected the bone marrow graft and required a second bone marrow transplantation 62 days after the first one. The outcome was then uneventful. In all cases there was complete cessation of vaso-occlusive episodes and haemolysis. The haemoglobin electrophoretic pattern became similar to that of the donor (AA or AS) and cytogenetic studies in three patients confirmed the donor origin of bone marrow cells.

Anemia, Sickle Cell↗

Disseminated BCG in HIV infection.

A boy, born to a mother with AIDS related complex, was immunised with BCG on the 10th day of life. At the age of 4 months he presented with a local enlarged lymph node, fever, hypotonia, and diarrhoea. Mycobacterium bovis, BCG strain, was grown from the lymph node and cerebrospinal fluid; this proved dissemination.

Acquired Immunodeficiency Syndrome↗

[Value of high-dose cytosine-arabinoside in the treatment of resistant acute leukemia].

Sixty-one patients with refractory or relapsed or secondary acute leukemia were treated with high-dose cytosine arabinoside (2-3 g/sq m in intravenous infusion every 12 hr to a 12-36 g/sq m total dose). m-Amsa or another antileukemic drug was given with cytosine arabinoside to 20 patients. Complete remission was achieved in 12 of 27 patients with acute myeloid leukemia, 5 of 8 patients with chemotherapy-induced leukemia, 3 of 7 patients with hematologic disorders in blastic crisis and 5 of 17 acute lymphoblastic leukemia patients. A similar response rate (6/16) was obtained when m-Amsa was given with cytosine-arabinoside. The median duration of remission was short (4 months in acute myeloid leukemia). Bone marrow transplantation was performed in 10 patients during the remission time. This regimen has acceptable toxicity; severe neurologic or hepatic disorders occurred in 18% of patients. These data suggest that high-dose cytosine arabinoside is an effective alternative in the treatment of resistant acute leukemia.

Acute Disease↗

Acute epidermal necrolysis or Lyell syndrome.

Acute epidermal necrolysis may be caused by staphylococcal toxins (staphylococcal scalded skin syndrome and toxic shock syndrome) or may be related to usually drug-induced hyper-sensitivity mechanisms (toxic epidermal necrolysis). Diagnostic certainty can only be obtained by histologic localisation of a cleavage plane which is intraepidermal in staphylococcal scalded skin syndrome but situated at the dermo-epidermal junction in toxic epidermal necrolysis. Steroid therapy is indicated in toxic epidermal syndrome. This report of two cases of acute epidermal necrolysis emphasizes the importance of an early skin biopsy for accurate diagnosis and appropriate treatment.

Child↗

AIDS in two African infants born in Belgium.

Two African infants born in Belgium, dying from the acquired immune deficiency syndrome are reported. The first patient was a premature baby girl born to healthy parents. However, her asymptomatic mother was found to have polyclonal hypergammaglobulinaemia, a reversed T-helper/T-suppressor ratio and a decreased lymphocyte response to mitogens. The second patient's mother was treated for pneumocystis carinii pneumonia during the fourth month of gestation and was subsequently diagnosed with AIDS. The rapid clinical evolution of the acquired immunodeficiency syndrome in infancy suggests that the incubation period is shorter than in adults.

Acquired Immunodeficiency Syndrome↗

Pregnancy-specific beta 1 glycoprotein (SP1) in the cerebrospinal fluid.

Pregnancy-specific beta 1 glycoprotein (SP1) was assayed by Particle Counting Assay in the cerebrospinal fluid (CSF) from 26 non-neurological patients, from 190 patients with various neurological disorders and from 84 patients with malignant hemopathies. With a sensitivity limit of 0.5 microgram/l, SP1 was undetectable in normal CSF. High levels were observed in CSF from one pregnant woman with herpetic encephalitis and from another woman with post-puerperal thrombophlebitis as a result of high serum concentrations and leakage of the blood-brain barrier. SP1 was detected at low levels in the CSF from 1 patient out of 5 with Creutzfeldt-Jakob disease and from a patient with Behçet's disease. Seven patients out of 84 with malignant hemopathies presented cerebral involvement; 3 of them had detectable SP1. However, SP1 was also detected in the CSF of 2 patients in apparently complete remission. The determination of SP1 in CSF appears to be of limited value in the diagnosis of neurological disorders and in the early detection of a cerebral localization of malignant hemopathies.

Burkitt Lymphoma↗

Bone marrow transplantation from an unrelated donor for Fanconi's anaemia: two unusual complications.

An eight-year-old boy with Fanconi's anaemia received a bone marrow transplantation from an unrelated donor. Sustained engraftment was achieved, but he developed major complications including acute grade III graft-versus-host disease, autoimmune haemolytic anaemia with positive Coombs' test and free antibodies against Rhesus group, and a virus-associated haemophagocytic syndrome; this latter complication was successfully treated with acyclovir. Sixteen months after transplantation he has full haematopoietic reconstitution, but with persisting mild autoimmune haemolysis.

Anemia, Aplastic↗

Diffuse reflectance technique for infrared analysis of urinary calculi.

We investigated the application of diffuse reflectance infrared Fourier transform spectroscopy to analysis of urinary calculi and compared its operation with that of older sampling techniques (pellet, mull, and attenuated total reflectance). The new method requires shorter sample preparation time and less sample (30 micrograms). Quantitative measurements are easier. Because of the additivity of the Kubelka-Munk functions, a quasi-exhaustive collection of infrared spectra of mixtures of possible components of stones can be compiled, facilitating a computerized intrepretation of infrared spectra of urinary calculi.

Computers↗

Computer-aided infrared analysis of urinary calculi.

We have created a library of 497 digitized infrared spectra of 58 components of urinary calculi and of their usual binary and ternary mixtures. We tested the operation of the "Birsy" search program (Bruker Analytische Messtechnik) with this library for the interpretation of infrared spectra of 50 urinary calculi, selected from both classical and difficult cases. This program correctly identifies the two first components 98% of the time and the third (minor) component 70% of the time. Using this program, those without training or experience in infrared analysis can routinely use the infrared method of analysis of urinary calculi.

Humans↗

Congenital fibrosarcoma. Preoperative chemotherapy and conservative surgery.

Three cases of congenital fibrosarcoma are reported. The use of preoperative chemotherapy, a VAC regimen, allowed conservative surgery in two of them. The three children are well, with no evidence of disease and without sequelae after completion of postoperative chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗

Prevalence and treatment of silent gastro-oesophageal reflux in children with recurrent respiratory disorders.

Thirty-six infants and children presenting with recurrent respiratory disorders (RRD) as the sole clinical symptom including bronchial asthma (6), recurrent obstructive bronchitis with or without wheezing (18), chronic nocturnal cough (3), recurrent episodes of pneumonia (3), recurrent pharyngitis (3) and recurrent laryngitis (3) were investigated for associated gastro-oesophageal reflux (GER) by oesophagram, endoscopy and continuous 24 h pH monitoring of the distal oesophagus. The pH monitoring criteria were selected on the basis of a preliminary study comparing statistically measurements of 32 variables recorded in 15 patients who all had clinical, radiological and endoscopic evidence of GER and in 8 asymptomatic controls. Although patients with symptomatic GER differed significantly from the asymptomatic ones for 27 variables examined, 6 variables emerged as having the highest value for discrimination (overlap score 0-1). Among these, the Euler-Byrne index (number of reflux pH less than 4 + 4 times the number of reflux episodes of more than 5 min), the percentage of total reflux time and the number of reflux episodes 1 h post-cibal scored 0 (no overlap). GER was considered to be present when at least five of these six parameters were abnormal. The overall incidence of GER in children with RRD was 41% (15) when detected by oesophagram and 61% (22) when diagnosed by pH monitoring criteria. In the children with bronchial asthma or with recurrent laryngitis, the percentage of reflux time during sleep was about 40 times higher than in asymptomatic controls and 2 times higher than in those with symptomatic GER.(ABSTRACT TRUNCATED AT 250 WORDS)

Child↗

A variant of the congenital dyserythropoietic anaemia type II with structural abnormalities in the granulocytic series.

Typical features of congenital dyserythropoietic anaemia (CDA) were found in a 13-year-old girl admitted for chronic recurrent multifocal osteomyelitis. The findings on light microscopy were in agreement with those described in CDA type II, whereas on electron microscopy, the ultrastructure findings were compatible with both types I and II. The acidified serum lysis test (Ham test) performed with eight normal sera was negative. The patient's red blood cells showed an increased agglutinability with anti-i antibodies. Morphological changes were also shown in the mature neutrophilic granulocyte suggesting that the primary disorder exists already in the multipotent stem cell.

Adolescent↗

Affinity of corticosteroids for mineralocorticoid and glucocorticoid receptors of the rabbit kidney: effect of steroid substitution.

Corticosteroid derivatives coupled in the C3, C7 or C17 position with a long aliphatic chain were synthesized in order to select a suitable ligand for the preparation of a biospecific affinity adsorbent for mineralocorticoid receptor purification. The affinity of these derivatives for mineralocorticoid receptors (MR) and glucocorticoid receptors (GR) was explored in rabbit kidney cytosol. In this model, aldosterone bound to a single class of receptors with high affinity (Kd 1 nM) and mineralocorticoid specificity. RU26988, a highly specific ligand for GR, did not compete for these sites. The C7 and C17 positions were found to be of crucial importance in the steroid's interaction with the mineralocorticoid receptors, since the linkage of a long side chain in these positions induced complete loss of affinity. Hence, deoxycorticosterone no longer bound to MR after 17 beta substitution with a 9-carbon aliphatic chain. This loss of affinity was not observed for glucocorticoids. The 17 beta nonylamide derivative of dexamethasone still competed for GR. Increasing the length of the C7 side of the spirolactone SC26304 suppressed its affinity for MR. Finally, C3 was an appropriate position for steroid substitution. The 3-nonylamide of carboxymethyloxime deoxycorticosterone bound to MR but not to GR, and therefore constitutes a suitable ligand for the preparation of a mineralocorticoid adsorbent.

Adrenal Cortex Hormones↗