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J Szwarcberg

Publications and source records attributed to J Szwarcberg.

8 recordsLinked to original sources
PubMed2006

[Combined hamartoma of the retina and retinal pigment epithelium. Four case studies].

INTRODUCTION: Combined hamartoma of the retina and retinal pigment epithelium is a rare condition, characterized by a proliferation of the retinal pigmentary epithelium and retinal gliosis leading to a disorganization of the retina and papilla. This study aimed to demonstrate the advantages of early diagnosis and regular monitoring. OBSERVATIONS: We report a series of four children followed between 2001 and 2004 with combined hamartoma of the retina and retinal pigment epithelium, with age of diagnosis ranging from 3 months to 8 years. The main reason for consultation was reduction of vision. The clinical examination objectified the existence of a slightly grayish peripapillary formation with tortuous retinal vessels. This condition was confirmed by angiography with fluorescein in three cases and by optical coherence tomography (OCT) in one case. Progression showed the persistence of low vision in all four cases and the appearance of a neovascular membrane in one case. DISCUSSION: Combined hamartoma of the retina and retinal pigment epithelium is probably a congenital tumor whose pathogenesis has not yet been elucidated. The diagnosis is clinical and the patient can be thoroughly examined by retinal angiography and optical coherence tomography (OCT). In this disorder, it is important to eliminate retinoblastoma and malignant melanoma of the choroid, showing the advantage of radiological exploration. Progression is stationary; nevertheless a reduction in visual acuity can be related to an epiretinal membrane or a neovascular membrane. CONCLUSION: Knowledge of the clinical aspect is essential to differentiate this condition from the malignant retinal processes. This tumor can progress in spite of its benign character. Regular follow-up is essential and can improve the visual prognosis.

Child↗
PubMed2005

[Vogt-Koyanagi-Harada syndrome].

INTRODUCTION: Vogt-Koyanagi-Harada syndrome is a rare, inflammatory disease with manifestations affecting the ocular, central nervous, audito-vestibular, and integumentary systems. Vogt-Koyanagi-Harada syndrome is more frequent in Asia but is also described in Europe. We report three new non asiatic cases of this syndrome. OBSERVATIONS: The three patients had bilateral panuveitis and hypoacusia. Two of them had peripheral facial palsy, two of them had vestibular syndrome and one of them lymphocytic meningitis. In each case we found characteristic HLA II typing and in one case we discovered the simultaneous presence of three auto-antibodies: anti-retina (anti-Arrestin type), anti-choroid and anti-cochlea. These patients were treated by corticosteroids but required an additional treatment by cyclophosphamide (0.8g/m2). The clinical course was favorable with visual sequelae for two and auditory one for one. DISCUSSION: These biological and therapeutic elements and a review of the recent literature are in favor of an autoimmune origin of this syndrome.

Adult↗
PubMed2002

[Ocular toxicity of deferoxamine: description and analysis of three observations].

INTRODUCTION: Deferoxamine is a specific chelating agent of trivalent anions: iron ion and aluminum ion. The main prescriptions for this treatment are primary non-curable by blood letting hemochromatosis, secondary hemosiderosis, and aluminum intoxication associated with chronic kidney failure. Since the early 1980s, ocular toxicity has been documented in several publications. OBSERVATIONS: We recorded three clinical observations of patients presenting symptoms of an ocular toxicity caused by deferoxamine. The prescription of this treatment related to the presence of secondary hemosiderosis (a case of primitive myelofibrosis and a case of chronic myelomonocytic leukemia treated by blood transfusion) and an aluminum intoxication affecting a patient with chronic kidney failure. All three patients presented a gradual loss of visual acuity. The following were predominantly observed at the fundus examination which showed pigmentary anomalies near the macula such as mottling and dispersion affecting the electrophysiological studies. The termination of the treatment did not result in an improvement in the symptomatology. DISCUSSION: Considering the latest literature on the subject, the indications as well as the pharmaceutical properties of deferoxamine, the ophthalmological symptoms of this intoxication, the additional investigations and the anatomicopathological analyses are restated, together with the current pathogenical hypothesis. CONCLUSION: Deferoxamine can cause ocular toxicity resulting in severe and permanent lesions of the retinal pigment epithelium. The occurrence of disorders of the fundus and visual acuity requires, before and during the treatment, regular ophthalmological monitoring combined with electrophysiological explorations. This allows early treatment of the hematological or kidney disorder.

Aged↗
PubMed1999

[Congenital hypertrophy of retinal pigment epithelium: a marker in familial adenomatous polyposis].

PURPOSE: Familial adenomatous polyposis (FAP) is an inherited autosomal dominant disorder with marked propensity for malignant transformation. The potential for congenital hypertrophy of retinal pigment epithelium (CHRPE) as a phenotypic marker for this disease is recognized. MATERIAL AND METHODS: We report our investigations in 11 families with familial adenomatous polyposis. CHRPE characteristics were described and the relations between genotype and phenotype and those between CHRPE and severity of FAP are discussed. DISCUSSION: All members of the family should undergo retinal examination at the earliest age possible. The results give an indication of the severity of the intestinal disease and allow an approximate localization of the mutation in the coding sequence, leading to a more rapid genetic analysis.

Adenomatous Polyposis Coli↗
PubMed1976

Atrial fibrillation developing in the acute phase of myocardial infarction. Prognostic implications.

Atrial fibrillation was observed in 39 (11 percent) of 350 instances of acute myocardial infarction. The mortality among these patients was 41 percent (16/39). Atrial fibrillation was more common in patients with undetermined infarctions and in older people. As opposed to death rates close to 50 percent among patients with anterior, combined, and undetermined infarctions, the presence of atrial fibrillation did not affect the mortality among patients with inferior infarctions (10 percent, 1/10). Ventricular rates higher than 120 beats per minute and duration of the arrhythmia longer than six hours were not associated with increased mortality. Hemodynamic failure was present in almost all of the cases and preceded the arrhythmia in most of them. It is concluded that different mechanisms are responsible for the production of atrial fibrillation in the setting of acute myocardial infarction, and the prognosis of the patient is related to the mechanism of production and not to the arrhythmia itself.

Aged↗