Epidemiological, clinical, and therapeutic characteristics of Behçet's disease: a monocentric study in Tunisia

Introduction: to describe the epidemiological, clinical, therapeutic and evolving characteristics of Behçet´s disease and identify prognostic factors. Methods: we have realized a retrospective, single-center study, conducted over a period of 26 years and including 130 patients presenting Behçet´s disease and hospitalized in an Internal Medicine Department. Results: the mean age of the Behçet´s disease at onset was 30.3 ±8.8 years and that at diagnosis was 34.6 ±9.4 years. The sex ratio (male/female) was 2.5. The mean delay of diagnosis was 53.5 months. Oral aphthosis was constant. The frequency of the manifestations was: genital aphtosis 71.5%, pseudofolliculitis 84.6%, erythema nodosum 11.5%, positive pathergy test 50%, ocular disease 36.9%, venous thrombosis 30%, arterial disease 4.6%, joint damage 30.8%, neurological disease 19.2% and digestive disease 0.8%. The male gender was significantly associated with ocular involvement (p =0.02), venous disease (p =0.01) and occurrence of relapses (p =0.01). The mean follow up was 68.5 ± 77.3 months. The poor survival prognostic factors were male gender, ocular involvement, venous disease, cardiovascular disease, a duration of follow up ≤12 months and a diagnostic delay ≤ 24 months. Conclusion: improving the prognosis of Behçet´s disease requires a shortening of the time to diagnosis, multidisciplinary collaboration, intensive treatment of functional threats, regular monitoring, and patient adherence

Unusual cause of cerebral calcifications in an 8‐year‐old girl

Key Clinical Message Genetic counseling and genetic screening for hyperoxaluria should be recommended for children with urinary lithiasis for early management to avoid progression to oxalosis especially if there is a family history of lithiasis. Primary hyperoxaluria type 1 (PH1) is caused by a deficiency of the liver peroxisomal enzyme alanine–glyoxylate aminotransferase (AGT) resulting in overproduction of calcium oxalates. In its later stage, a systemic deposit of calcium oxalates is observed. We present the case of an 8‐year‐old girl with exceptional neurological involvement secondary to this disease

Surgical management of lifebuoy cataract

Lifebuoy cataract is a rare form of congenital cataract with anatomical characteristics. Herein, we present a case of an otherwise healthy 42-year-old female with a long-standing history of blurred vision. Examination showed the presence of esotropia and bilateral horizontal nystagmus. Visual acuity was limited to light perception in both eyes. Slit-lamp examination showed a calcified lens capsule with the absence of lens material in the right eye and the presence of an annular cataract in the left eye, corresponding to a unilateral lifebuoy cataract. She underwent cataract surgery with intraocular lens implantation. We report the clinical findings, anterior segment optical coherence tomography (AS-OCT), and surgical management tips. We noticed that during surgery, both anterior capsulorhexis and central membrane removal were the most challenging steps due to the absence of central nucleus and the strong adherence of central membrane to the anterior hyaloid