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Biomedical subjects

D Lahbil

Publications and source records attributed to D Lahbil.

9 recordsLinked to original sources
PubMed2006

[Fraser syndrome. A case report].

Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptophthalmos, syndactyly of fingers and toes, laryngeal stenosis, and urogenital abnormalities. We report a newborn case at day 1 of life who had multiple abnormalities, born from a consanguineous marriage. Clinically, the newborn had an ankyloblepharon on the left side, a cryptophthalmos on the right side, a syndactyly, anorectal abnormalities with ambiguous genitalia, laryngeal stenosis, and ear malformations. TDM of the cranium and orbits and the transfontanel ultrasound were normal. The abdominal ultrasound showed renal abnormalities. Right eye surgery showed a reduced cornea to an opaque thin plate clinging to the iris without an anterior chamber and a nonindividualized eyeball. The authors discuss the morphological abnormalities, the clinical and paraclinical aspects of this syndrome, its multispecialized clinical management, and the importance of prenatal diagnosis.

Abnormalities, Multiple↗
PubMed2006

[Bilateral optic neuropathy and non-Hodkin's lymphoma].

UNLABELLED: While ocular lesion is commonly known in lymphoma, optic neuropathy is very rare : 1,3% of lymphomas affecting the central nervous systems. OBSERVATION: Authors report the case of a 75 year old patient treated in the haematology department for 8 years, for a large cell B phenotype stage IV lymphoma for which he received 7 chemotherapy courses (CHOP protocol). After a 4 year remission period, he presented a relapse with a rapid progressive bilateral impairment of visual acuity observed for a week before his admission. The ophthalmologic exam revealed no light perception and no afferent reflex on the right eye. There was light perception and weak afferent reflex on the left eye. The anterior segment was normal on both eyes and fundus examination revealed a bilateral stage I papillar oedema. The general exam showed a right facial palsy and an impairment of general condition. The orbital CT scan revealed a significant thickening of both optic nerves caused by lymphomatous infiltration. A chemotherapy with highly dosed IV and intrathecal methotrexate was performed. DISCUSSION: the optic neuropathy is usually associated with a generalized lymphoma with central nervous system involvement, but sometimes can precede the systemic spread of the disease. Apart from infiltration, the optic nerve can be compressed by an intracranial or orbital tumor. The optic neuropathy can also be caused by lymphomatous leptomeningitis.

Adult↗
PubMed2004

[Macular damage in Behçet's disease].

INTRODUCTION: The macular damage in Behçet's disease requires particular attention because of its poor prognosis. PURPOSE: The purpose of our study was to specify the frequency of macular alterations in Behçet's disease and discuss the clinical, physiopathological and therapeutic aspects. MATERIALS AND METHODS: Our report is based on 244 patients with Behçet's disease examined between January 1995 and December 1997. The authors studied macular alterations in detail. RESULTS: Sixty-nine patients had a maculopathy affecting 108 out of 432 eyes (24%). The mean progression of Behçet's disease in these cases was 4 years. Visual acuity was<or=2/10e in 48 eyes (44.4%). Maculopathy was bilateral in 39 cases. Macular edema was the most frequently noted form in 62% of the eyes, followed by cystoid macular edema (17%) and macular hole (3.4%). DISCUSSION: Macular edema is the most frequent sign and complication arising from the macular area during Behçet's disease, which can evolve into cystoid degenerescence and occasionally macular hole. Macular lesions are often irreversible, making prognosis uncertain, despite the various treatments available. CONCLUSION: Macular damage is the major cause of visual morbidity in patients with Behçet's disease and should be systematically sought.

Adult↗
PubMed2004

[A case of a retinitis pigmentosa and asteroid hyalosis].

Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship between retinitis pigmentosa and asteroid hyalosis has been established. We report a case of a 65-year-old male admitted for progressive hemeralopia. Visual acuities were 2/10e in the right eye and 4/10e in the left eye. Ophthalmic examination revealed a large number of asteroid and refringent bodies, a typical retinitis pigmentosa with a cystoid macular oedema confirmed by fluorescein angiography. Electrodiagnostic testing revealed an altered electroretinogram. Visual fields showed a typical generalized constriction. We discuss the clinical aspects and physiopathogenic mechanisms of this rare association through this case and other cases found in the literature.

Aged↗
PubMed2003

[Ocular and systemic complications of homocystinuria: a report of five cases].

Homocystinuria is an autosomal recessive disorder of methionine metabolism due to cystathionine B-synthetase deficiency. It is the second most common inborn error of amino acid metabolism after phenylketonuria. In addition to the eyes, the skeletal, central nervous and vascular systems are usually affected by homocystinuria. We report two family cases of two sisters and two brothers with homocystinuria revealed by lenticular dislocation into the anterior chamber, associated with mental and growth retardation, and an isolated case of homocystinuria revealed by poor vision with Marfan syndrome and mental retardation. They all underwent surgical lensectomy with anterior vitrectomy under general anesthesia with anaesthesic precautions to prevent vascular thrombosis. Ocular complications are common in patients with homocystinuria. Treatment must include dietary changes to reduce the incidence of ectopia lentis and mental retardation. Surgical treatment with modern microsurgical techniques should be considered in advanced ocular manifestations of homocystinuria.

Child↗
PubMed2002

[Ocular impairment during type II membranoproliferative glomerulonephritis].

Membranoproliferative glomerulonephritis type II (MPGN) is characterized by dense deposits within glomerular basal membrane and Bruch's membrane which result in retinal lesions similar to drusens. We observed a 50-year-old patient with chronic renal deficiency who developed central bilateral serous retinopathy with diffuse punctiforme yellow subretinal lesions. Ophthalmoscopic and angiographic aspects led to an MPGN type II diagnosis. Specific posterior segment lesions are described during MPGN type II. Dense deposits concerned both lamina densa of glomerular basal membrane and Bruch's membrane with choriocapillaris. The main ocular complications were central serous chorioretinopathy and choroidal neovascularization. We review the clinical and evolutive aspects of this disease.

Bruch Membrane↗
PubMed2000

[Gyrate atrophy of the choroid and retina: a case report].

BACKGROUND: Gyrate atrophy of the retina and choroid is a rare disease, with recessive autosomal transmission, characterized by progressive chorioretinal atrophy causing blindness. It results from a congenital deficit in aminotransferase ornithine. CASE REPORT: The authors present the case of a young patient aged 15 years old consulting for a progressive fall of visual acuity with hemeralopia. Eye funduscopy showed regions of confluent rounded chorioretinal atrophy. The visual field, the electroretinogram and the retinal angiography were all alterated. Gyrate atrophy of the retina and choroid was evocated. DISCUSSION: It is a systemic and rare metabolic disease where ocular features are dominating. Differencial diagnosis are pigmentary retinopathies. Cataract and/or myopia are often joined to the retinal lesions. General signs could consist in muscular weakness, thin and rare hairs and mental retardation. More than; visual fields, electroretinogram, retinal angiography that are alterated; the plasmatic dosage of the ornithine is often high. The treatment is based on the dietetics with uncertain results. The genic therapy would be the treatment of future.

Adolescent↗
PubMed1998

[Intermediary syndrome in acute malathion poisoning].

BACKGROUND: Acute poisoning by an organophosphorus insecticide is frequently observed in Morocco. We report a case of malathion poisoning complicated by an intermediate syndrome. CASE REPORT: A 42-year-old woman was hospitalized 3 hours after ingestion of 50 g of malathion. Intubation and ventilatory assistance was required due to failing consciousness and bronchial plugging. On day 4, a neurological syndrome suggestive of the intermediary syndrome with diaphragmatic palsy made it necessary to continue ventilatory assistance to day 20. DISCUSSION: The intermediary syndrome occurs 24 to 96 hours after the acute cholinergic phase of organophosphorus poisoning. It consists of an exclusive neuromuscular involvement. The intermediate syndrome is confined to an abnormality of neuromuscular function in specific muscle groups: proximal limb muscles, neck flexors, motor cranial nerves and respiratory muscles, with difficult weaning from respiratory assistance. The intermediary syndrome is quite uncommon. The risk of respiratory failure emphasizes the need for close monitoring in an intensive care setting for at least 96 hours, depending on the intensity of the cholinergic syndrome.

Acute Disease↗