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Biomedical subjects

Jelka G Orsoni

Publications and source records attributed to Jelka G Orsoni.

7 recordsLinked to original sources

Use of systemic cyclosporin A in a case of severe Toxocara uveitis.

The report describes the case of an adult patient presenting a severe, serologically proven, Toxocara endophthalmitis, unresponsive to the common surgical and medical approach (vitrectomy, anti-parasitic treatment followed by high-dose corticosteroids). The association of oral cyclosporin A to a lower dose of prednisone was successful in achieving a long-term control of the ocular inflammation without systemic side effects.

Adult↗

Cogan syndrome in children: early diagnosis and treatment is critical to prognosis.

PURPOSE: To present two cases of pediatric Cogan Syndrome and to highlight the differences between the adult and pediatric forms of the disease, as well as the importance of early diagnosis and treatment. DESIGN: Interventional case report. METHODS: Institutional setting. RESULTS: Corneal lesions were much more diffuse than those observed in adult Cogan syndrome. Immunosuppressive drug combination therapy successfully resolved systemic and ocular inflammation, but the involvement of the pupillary area caused permanent low vision in one case and amblyopia in the other. CONCLUSION: When chronic ocular inflammation is observed in association with sensory neural hearing loss and any systemic signs of autoimmune inflammation, a diagnosis of Cogan syndrome should be suspected. If immunosuppressive treatment is not initiated as soon as possible, permanent low vision and deafness can result.

Amblyopia↗

Syphilitic interstitial keratitis: treatment with immunosuppressive drug combination therapy.

OBJECTIVE: The following is a case presentation of congenital syphilitic keratitis in a boy 6 years of age who was successfully treated with an immunosuppressive drug combination therapy. METHODS: Congenital syphilitic keratitis was diagnosed by clinical findings and laboratory tests. The child was unresponsive to traditional treatment; thus, systemic immunosuppressive therapy, which consisted of oral cyclosporine 4 mg/kg/d, 6 days per week, and oral low-dose steroids (fluocortolone 0.8 mg/kg a week, given every other day), was initiated. RESULTS: Corneal disease showed great improvement with this therapy, with progressive healing of lesions in the first month of treatment and no signs of toxic renal, hepatic, or growth abnormalities. Recurrences of uveitis have not occurred, and corneal interstitial keratitis episodes have been limited to 3 in an 8-year period. After 6 months with no recurrences, a tapering off of the systemic therapy was initiated, and the child is still asymptomatic and without flare-ups. CONCLUSIONS: Congenital syphilitic keratitis is usually treated with topical steroids and cycloplegic drugs, which not only can be ineffective but can also lead to complications such as cataract and glaucoma. In the present case report, a pediatric patient affected by syphilitic interstitial keratitis was treated successfully with an immunosuppressive drug combination therapy.

Child↗

Effect of a drug combination treatment on ocular perfusion in recurrent idiopathic intermediate uveitis.

PURPOSE: To test the effect of a drug combination therapy on ocular perfusion in human eyes affected by idiopathic intermediate uveitis. METHODS: Seven patients (12 eyes) showing active signs of intermediate uveitis, with at least two more similar episodes reported within the previous 12 months, were enrolled in a prospective case series. Two fellow healthy eyes of two of the enrolled patients were studied as internal controls. Color Doppler imaging of the central retinal artery (CRA), the ophthalmic artery (OA), and the posterior ciliary arteries (PCAs) was performed at the time of enrollment, and at 6 and 12 months after starting treatment with oral fluorocortolone, cyclosporine, and parenteral methotrexate. The best-corrected visual acuity was concurrently measured as a second parameter. RESULTS: In the 12 affected eyes, the mean visual acuity (+/-SD) improved from 0.15(+/-0.12) to 0.04(+/-0.18) LogMAR (paired samples Student's t-test: p = 0.015). The resistivity index (RI +/- SD) of the CRA decreased from 0.81(+/-0.13) to 0.71(+/-0.13)(p = 0.0091). Further, the variation of the RI in the PCAs reached a borderline significance (p = 0.062), decreasing from 0.71(+/-0.12) to 0.61(+/-0.12). No significant changes were observed in the OA. Moreover, eyes showing a visual improvement of > or =0.1 (LogMAR) were more likely to show a > or =10% improvement of the RI for the CRA (Fisher's exact test: p = 0.018; power = 90%; alpha probability = 5%; odds ratio = 2,4). CONCLUSIONS: In eyes affected by idiopathic intermediate uveitis, treated with a systemic drug combination therapy, the improvement of the visual acuity seems to correlate with a proportional improvement of the retrobulbar circulation.

Adult↗

Cochlear implantation and Cogan syndrome.

OBJECTIVE: To evaluate outcomes and issues pertaining to cochlear implantation in a group of subjects affected by Cogan syndrome. STUDY DESIGN: Prospective cohort. SETTING: Department of Ophthalmology and Otorhinolaryngology, University of Parma. PATIENTS: Five postlingually deafened adults suffering from a typical form of Cogan syndrome who underwent cochlear implantation. MAIN OUTCOME MEASURES: Benefit from cochlear implantation as measured by word and everyday sentence recognition tests. Surgical issues and postoperative complications were also evaluated. RESULTS: In two cases, intracochlear electrodes were inserted into the scala vestibuli because of the ossification of the scala tympani. Two patients experienced a recurrence of keratitis the day after surgery. To date, with a follow-up of 1 to 4 years, no patient has experienced flap complications or other local or systemic complications. At the 12-month postoperative evaluation, all patients had gained useful open-set speech perception, achieving a mean score of 91% and 95% on word and everyday sentence recognition tests, respectively. CONCLUSIONS: Patients deafened by Cogan syndrome demonstrated high levels of speech understanding after undergoing cochlear implantation. Obliteration of the cochlea may complicate electrode implantation, requiring modifications of the surgical technique. Stress consequent to the surgical procedure may instigate an acute phase of the basic illness.

Adult↗

Autoimmune uveitis in children: clinical correlation between antinuclear antibody positivity and ocular recurrences.

OBJECTIVE: The aim of this study was to identify the correlation between antinuclear antibody (ANA) titre and the onset and clinical course of uveitis in children with juvenile idiopathic arthritis (JIA) or without any other systemic autoimmune disease, i.e., idiopathic uveitis (IU). METHODS: Twenty-two patients affected by uveitis were examined. Ten had JIA-associated uveitis, 12 had IU. Follow-up ranged from 7 to 101 months. The ANA were titrated three times per year and additionally in case of ocular recurrences. All patients were treated with immunosuppressive drug combination therapy (IDCT). RESULTS: JIA-associated uveitis: ocular recurrences were noted in three ANA-positive patients and in one ANA-negative patient. IU uveitis: ocular recurrences were noted in one ANA-positive and in one ANA-negative patient. No significant rise in ANA titre was noted in either group during uveitis recurrence. CONCLUSIONS: (1) ANA had no value in predicting the recurrence of uveitis. (2) IDCT does not influence ANA production.

Age Distribution↗

Cogan syndrome.

PURPOSE: To lead ophthalmologists to consider Cogan syndrome when managing a patient presenting with keratitis or other ocular inflammation accompanied by sensorineural hearing loss. METHODS: Seven patients affected by Cogan syndrome were studied: two males and five females, ranging from 27 to 65 years of age (mean age: 41 years). Subjects were evaluated for a period ranging from 22 to 46 months (mean follow up time: 29.2 months). All patients were treated with immunosuppressive drug combination therapy (IDCT). RESULTS: Three patients were affected by classic Cogan syndrome (i.e., vestibuloauditory symptoms and later sensorineural hearing loss and interstitial keratitis). Four patients presented atypical Cogan syndrome (i.e., sensorineural hearing loss and chronic ocular inflammation such as uveitis, scleritis, conjunctivitis, retinal vasculitis, etc.). Four of these patients had a late diagnosis. Two of them were diagnosed when they already had a cochlear implant, one with bilateral deafness underwent cochlear implantation 1 year after the beginning of IDCT, one had severe bilateral hearing loss that improved during the first year of IDCT, and then rapidly worsened to total deafness in 1 month following an episode of severe systemic hypotension. Three patients who had an early diagnosis of Cogan syndrome had no worsening of vestibuloauditory dysfunction during the follow up period. CONCLUSION: Diagnosis of Cogan syndrome should not be overlooked by ophthalmologists in all patients with recurrent ocular inflammatory disease associated with vestibuloauditory symptoms. Early diagnosis is essential to commence the appropriate immunosuppressive therapy that may prevent permanent hearing loss and ocular dysfunction.

Adult↗