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Biomedical subjects

B Husson

Publications and source records attributed to B Husson.

35 records · Page 2Linked to original sources

Subdural sarcoma associated with chronic subdural hematoma. Report of two cases and review of the literature.

The authors report the cases of two children who presented in the first months of life with progressive macrocrania related to chronic pericerebral fluid collection. This condition resolved spontaneously without treatment after a few months in the first case, whereas it required several aspirations of blood-stained fluid via the fontanel in the second case. Both patients developed normally without evidence of disease in the earliest years of life and presented at the ages of 3 1/2 and 4 1/2 years, respectively, with symptoms and signs of rapidly progressing intracranial hypertension. In both cases contrast-enhanced computerized tomography and magnetic resonance imaging revealed masses in the subdural space of the skull base and the cranial vault associated with significant subdural fluid collections. In the first case the lesion was misdiagnosed in the initial phase and treated, by means of multiple craniotomies, as an organized subdural hematoma. After a diagnosis of liposarcoma had been made, the patient was treated with chemotherapy, which resulted in a good resolution of the lesions at 3-month follow-up review. In the second case a biopsy allowed the diagnosis of fibrohistiocytic sarcoma and the patient was treated with chemotherapy. The authors review the literature of the few reported cases and discuss the possible pathophysiological association between pericerebral fluid collection and the subsequent development of a subdural sarcoma.

Biopsy↗

The value of MRI in the early diagnosis of growing skull fracture.

Growing skull fracture (GSF) is a progressive enlargement of a fracture due to an underlying tear of the dura mater. It is a rare complication of severe head injury mainly reported in young children. Classically, the diagnosis is made during follow-up, late after the original injury, when a palpable skull defect or a bulging mass is discovered clinically. Initial skull radiographs show a diastatic fracture developing later into a large bony defect. CT will show the brain damage which is usually present beneath the fracture. We present the MRI findings of GSF in a series of eight children. All patients initially had a large linear fracture and underlying brain damage on CT. In all cases MRI showed a zone of the same intensity as the brain contusion or cerebrospinal fluid advancing through the bone margins of the fracture to the subcutaneous plane. This finding was interpreted as an indirect sign of the dural tear. Seven patients were operated on with surgical confirmation of GSF. MRI can make an early diagnosis of GSF possible so that surgical repair with closure of the dura can be carried out before the dural tear enlarges.

Brain↗

Congenital unilateral perisylvian syndrome: radiological basis and clinical correlations.

DESIGN: Advances in neuroimaging have allowed correlations between radiological patterns and clinical features of brain malformations. This paper reports clinical, prognosis, and electroencephalographic features of six children with a previously unrecognised neuroimaging picture of unilateral widening and verticalisation of the sylvian fossa associated with an abnormal ipsilateral perisylvian cortex. RESULTS: All children had reduced hemisphere size and thalamostriatal hypoplasia ipsilateral to the cleft and hemiplegia. Cognitive development was mostly impaired. Epilepsy occurred in two patients and was mainly characterised by partial seizures. Studies with EEG showed hemispheric slowing of background activity homolateral to the perisylvian dysplasia. Occurrence of the malformation among their siblings was not found. CONCLUSION: Similar brain malformations occasionally reported in older patients confirm the clinical picture, sporadic occurrence, and prognosis found, allowing the validation of a unilateral perisylvian syndrome.

Brain↗

Extensive brain calcifications, leukodystrophy, and formation of parenchymal cysts: a new progressive disorder due to diffuse cerebral microangiopathy.

A new cerebral disorder, described in three unrelated children, has recognizable clinical, radiologic, and neuropathologic findings. The onset occurs from early infancy to adolescence with slowing of cognitive performance, rare convulsive seizures, and a mixture of extrapyramidal, cerebellar, and pyramidal signs. CT shows progressive calcifications in the basal and cerebellar gray nuclei and the central white matter. MRI reveals diffuse abnormal signals of the white matter on T2-weighted sequences. A special feature is the development of parenchymal cysts in the cerebellum and the supratentorial compartment, leading to compressive complications and surgical considerations. Neuropathologic examination of surgically removed pericystic samples reveals angiomatous-like rearrangements of the microvessels, together with degenerative secondary changes of other cellular elements. Both the anatomic findings and the course of the disease suggest a constitutional, diffuse cerebral microangiopathy resulting in microcystic, then macrocystic, parenchymal degeneration.

Brain Diseases↗

An improved method for detection of low density lipoprotein receptor defects in human T lymphocytes.

Familial hypercholesterolemia (FH) results from an inherited functional defect of the low density lipoprotein (LDL) receptor and is complicated by premature atherosclerosis. FH diagnosis is obtained by sophisticated techniques or is suggested by clinical criteria. We have developed a technique based on flow cytometry for the measurement of DiI-labeled LDL uptake in human peripheral blood T lymphocytes left for 2 days in a lipoprotein-deficient culture medium. Flow cytometry allowed us to discriminate the uptake of DiI-LDL by T lymphocytes subpopulation from the uptake by the whole mononuclear population using a T cell-specific anti-CD3 antibody. The method appeared to be highly specific for the receptor-mediated pathway of LDL uptake as DiI-LDL uptake was inhibited in the presence of a 10-fold excess of unlabeled LDL and by EDTA. A good relationship was found between the uptake of DiI-LDL and 125I-labeled LDL degradation. The test was applied in three groups of patients: patients with normal cholesterol levels, patients with heterozygous FH, and patients with high cholesterol levels but without clinical criteria of FH. The mean fluorescence intensities were 23.1 +/- 8.9, 6.3 +/- 1.7, and 17.1 +/- 3.5 (mean +/- standard deviation), respectively. The ability to measure the fluorescence in T lymphocytes improved the discrimination between FH and non-FH subjects when compared with values obtained from the whole mononuclear cell population. These results suggest that our method could be useful for LDL receptor defects screening.

Adolescent↗

Clinical evidence for an engraftment syndrome associated with early and steep neutrophil recovery after autologous blood stem cell transplantation.

Seventy autologous peripheral blood stem cell transplants (APBSCT) performed in 61 cancer patients were retrospectively analyzed. Patients were heterogenous with regard to malignancy, conditioning regimens and use of growth factors after transplantation. Six patients developed a non-infectious fever, fluid retention and pulmonary interstitial infiltrates during the early phase of neutrophil recovery. Diarrhea was observed in four of these patients and cutaneous rash in three. The clinical condition improved spontaneously in one patient, and within 48 h after steroid therapy in four. One patient died from multiple organ failure. Age, sex (all patients were female; P = 0.07), and time to platelet recovery did not distinguish the six courses complicated by the hypothetical engraftment syndrome (ES) from the other 64 courses taken as controls. However, neutrophil recovery > 0.5 x 10(9)/l occurred earlier (P = 0.01), and the neutrophil count increment during the early phase of recovery was steeper in ES patients (P = 0.003). ES was also associated with infusion of a high number of CD34+ progenitors (P = 0.03) and conditioning with busulfan (P = 0.03). Although all ES patients received G-CSF after transplantation, an association of ES with G-CSF use could not be demonstrated, possibly because of the small number of courses not supported by G-CSF. However, in one patient, ES did not recur after a second transplant unsupported by growth factors. Our study supports the idea of an engraftment syndrome associated with an early and steep neutrophil recovery after APBSCT.

Adult↗

[Encephalopathy induced by arterial hypertension: clinical, radiological and therapeutical aspects].

BACKGROUND: Neuroradiological features of hypertensive encephalopathy are not yet well-known. PATIENTS AND METHODS: The clinical manifestations, neuroradiological aspects and treatment of four patients suffering from hypertensive encephalopathy were studied; special attention was paid to manifestations presented by one patient which appeared representative of the usual manifestations of hypertensive encephalopathy. RESULTS: The main clinical features were visual disturbances (n = 4); altered consciousness (n = 2) and seizures (n = 3). Visual disturbances resulted from cortical lesions (n = 4) associated with optic nerve damage and retinopathy (n = 2). Cerebral imaging showed low density images (CT scan) and hypersignal (MRI, T2 sequence) located in parieto-occipital cortical ribbon and adjacent white matter from the first 24 hours following the onset of neurologic symptoms. In the studied patient, the abnormal images resolved within 1 month after the onset of the disease. Unilateral infarction of the anterior visual pathway affected two patients in whom: 1) hypertension remaining undetected for a long time was revealed by neurological signs; 2) visual impairment was preceded by an hypotensive episode induced by antihypertensive therapy. CONCLUSIONS: Neuroradiological features of hypertensive encephalopathy are useful in establishing the diagnosis. A gradual reduction of blood pressure could prevent the risk of visual complications.

Adolescent↗

Synthesis of 1,2-diacyl-3-nicotinoyl glycerol derivatives and evaluation of their acute effects on plasma lipids in the rat.

Nicotinic acid (CAS 59-67-6) is the only hypolipidemic agent whose activity has been shown both on atherosclerotic lesions and on long term mortality. Unfortunately, its use is hindered by the frequent occurrence ( > 70%) of adverse reactions (i.e. cutaneous rash, pruritus and, most significantly, flush). New prodrugs of nicotinic acid have been prepared by the use of diacylglycerol esters. In the rat, after acute oral administration of these products, a significant decrease of the free fatty acid plasma levels was obtained without the dramatic increase in nicotinic acid plasma levels observed after the oral administration of an equimolecular dose of nicotinic acid. The most interesting ester, S 16961 ((d,l)-1,2-dipalmitoyl-3-nicotinoyl glycerol, CAS 160555-46-4) is undergoing clinical trials.

Animals↗

Submicroscopic deletions of 17p13.3 in type 1 Lissencephaly.

DNA markers YNZ22.1 and YNH37.3 were studied by Southern blotting in 14 patients with typical (11 cases) and atypical (3 cases) type 1 Lissencephaly, all with normal high resolution karyotype. A submicroscopic deletion was found in 2 typical cases: one with Miller-Dieker Syndrome (MDS), the other with Isolated Lissencephaly Sequence (ILS). These results suggest a genetic continuum between MDS and ILS. The low frequency of such deletions, especially in ILS, will necessitate direct testing of the newly identified LIS 1 gene.

Cerebral Cortex↗

[Imaging of complications of liver transplantation in children].

The authors report their experience of imaging of 286 liver transplantations (LT) in children. Hepatic artery thrombosis is the most serious complication (9%), with a maximum risk during the first two weeks. Its clinical presentation is very variable and its diagnosis is based on Doppler ultrasonography. Emergency surgical disobstruction prevented the development of biliary or ischaemic complications in one half of cases. Portal thrombosis (4.5%) is due, in the majority of cases, to hypoplasia of the recipient's portal vein. Patency of intrahepatic portal branches with inversion of flow can delay the ultrasonographic diagnosis. Biliary complications are frequent (20%) and occur after a very variable interval. Their diagnosis is ultrasonographic, but sometimes delayed compared to the histological signs. Transhepatic cholangiography visualises the lesions and allows external drainage or even percutaneous dilatation. In 1 out of 4 cases, the aetiology was hepatic artery thrombosis. The imaging protocol after LT consists of ultrasonography with daily Doppler (or even twice-daily) in the patient's bed for the first two weeks and then as required. Computed tomography is useful to demonstrate parenchymal ischaemia and intraperitoneal abscess in multi-operated children. Angiography and cholangiography confirm the ultrasonographic signs. Interventional Radiology plays an increasingly important role.

Aortography↗

[Syndrome of delayed maturation of the sucking-swallowing reflex].

BACKGROUND: Neonates with neurological diseases often have difficulty in sucking or swallowing. This report describes such difficulties in a group of infants with normal development, suggesting late maturation of sucking and swallowing. POPULATION AND METHODS: Seven infants (four girls, three boys) had suffered from aspiration since their first day of life (four cases) with severe asphyxia (two cases), late repeated episodes of airway obstruction by accumulated secretions (two cases), and weak isolated sucking and swallowing (one case). The lack of sucking and swallowing or difficulty with them were the main manifestations in these newborns: the face lacked expression and the tongue movements were abnormal with, in two cases, hyper-extension of the neck. A second phase during the first months of life was marked by episodes of bradycardia and/or drowsiness. A third phase during the first years of life was characterized by repeated episodes of respiratory infections, recurrent stridor and accumulation of saliva. These manifestations required prolonged hospitalization (mean: 2.5 months), nasogastric feeding, and in two cases, surgery. The investigations during the acute phases included a cineradiographic study of swallowing, laryngoscopy, monitoring of the distal esophagus pH, a barium swallow, polygraphic recording during sleep and MR imaging of the brain stem. The difficulties in sucking or swallowing disappeared before the age of 6 months (two cases), between 6 and 12 months (one case), between 1 and 5 years (four cases) without any sequelae or speech disorders. CONCLUSION: These transient sucking or swallowing difficulties suggest late maturation of praxis. They required specialized prolonged education after careful, essentially clinical, investigation.

Deglutition Disorders↗

[Epidermoid cyst of the 4th ventricle. Apropos of a case in a child and review of the literature].

A 6 year-old child was admitted for a recurrent bacterial meningitis at Proteus Morganii. She was treated with antibiotics. The C.T. Scanner showed a midline low-density lesion, with a mild annular contrast uptake in the posterior fossa. M.R.I. showed the lesion and revealed a skin-bone-dura fistula. The surgical resection was complete. It was an epidermoid tumor. Intracranial epidermoid tumors constitute about 1% of brain tumors and are considered to be congenital. Epidermoid tumors of the 4th ventricle are exceedingly rare in childhood. The authors reviewed the literature and discuss the pathogeny, the radiographic diagnosis and the treatment of these tumors.

Brain Diseases↗

[MRI of the spinal epidural fat in pathology].

The authors report about their experience with the merits of the normal systematization of epidural fat with MRI. On sagittal sections, this fat has a variable appearance, but its location along the spinal canal is constant. On axial sections, its morphology is suggestive of the level of section: cervical, upper or lower thoracic, lumbar. Some changes in this fat are precious data to explain local hypertrophy (scoliosis) or to locate an intra- or extradural process.

Adipose Tissue↗

[Analysis of loco-regional control rate, metastatic remission and survival in 178 breast cancer patients treated by radical modified mastectomy and post-operative irradiation (author's transl)].

178 cases of post-operative irradiation for breast cancer (145 T1, T2, T3 and 33 T4) are analyzed. According to the surgical procedure there are 146 cases of radical modified mastectomy (Patey) and 32 cases of radical mastectomy (Halsted). The axillary status reveals 50% of involved nodes, but radiotherapy has been systematic on chest wall and supra-clavicular, parasternal, axillary lymph nodes areas. Loco-regional failures are only situated on chest wall (3,9%) and in the supraclavicular area (1,7%). The five year actuarial rate of locoregional control is 93% and 98% if we exclude 7 locoregional failures contemporaneous or consecutive to a metastatic evolution. The five year actuarial rate of metastatic remission for patients with negative axillary nodes (N-) is 83% against 55% for N+ (p < 0,001). The five year actuarial survival rate is 92% (N-) and 64% (N+) (p < 0,001). We discuss about indications of selective irradiation of chest wall and parasternal area for patients with negative axillary nodes and also about post-operative immuno-suppression, and chronology of chemotherapy.

Adult↗