RESUMO
Genetic defects in the development, maturation, and/or function of the immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. The overall risk for cancer in children with IEI ranges from 4 to 25% and the type of malignancy is highly dependent on the specific mutant gene underlying IEI. We investigated 3056 IEI patients registered in the Iranian national registry between the years 1999 and 2020 in this retrospective cohort study. The frequency of malignancy and its association with the type of IEI in these patients were evaluated. A total of 82 IEI patients with malignancy were enrolled in this study. Among them, predominantly lymphoma was the most common type of malignancy (67.1%), followed by leukemia (11%), and cancers of the head and neck (7.3%). Among identified lymphoma cancers, non-Hodgkin's lymphomas were the most frequent type (43.9%) followed by different subtypes of Hodgkin's lymphoma (23.2%). Solid tumors (18.3%) appeared to be very heterogeneous by type and localization. The correlation between the type of malignancy and survival status and the association between the type of malignancy and IEI entities were unremarkable. The awareness of the association between the presence of IEI and cancer highlights the importance of a synergistic effort by oncologists and immunologists in the early diagnosis of malignancy and personalized therapeutic strategies in IEI patients.
RESUMO
PURPOSE: To determine the predictive factors of post-operative stereopsis in patients with strabismus. METHOD: In this retrospective study, records of patients who received surgical treatment for strabismus were reviewed. All types of strabismus were included. Pre- and post-treatment stereoacuity were measured using the Titmus Stereo Fly test, and predictive factors of stereopsis were evaluated. RESULTS: A total of 194 patients (132 females and 62 males) with a mean age of 14.8 ± 8.4 years were included. There was a statistically significant improvement in stereopsis following surgery (p value < 0.001). Patients with a higher amount of deviation at baseline had poorer stereopsis on the final examination (p value < 0.001). Stereopsis improvement was more prominent in the pure horizontal strabismus group, compared to combined horizontal and vertical deviations. Baseline and the final stereopsis were higher in the "exotropia" group as compared to the "esotropia" group (p value = 0.003 and 0.0155, respectively); however, the within group change of stereopsis was not significantly different between these two groups (p value = 0.144). Post surgical residual deviation was associated with a poorer stereopsis (p value = 0.002, r = 0.251). A longer duration of strabismus before surgery was associated with poorer final stereopsis levels (p value = 0.026). The presence of amblyopia before surgery was associated with poorer stereopsis on last examination (p value < 0.001 for both correlations). CONCLUSION: Based on the result of this study, final stereopsis after strabismus surgery could be affected by the type, duration, and the amount of deviation before surgery, amblyopia, and post operative ocular deviation.
RESUMO
PURPOSE: To determine possible complications and efficacy of ptosis surgery in a series of chronic progressive external ophthalmoplegia (CPEO) patients with healthy tear film. METHOD: It is a prospective interventional study on 24 eyes from 12 patients with the diagnosis of CPEO and ptosis. Pre-operatively, tear breakup test (TBUT) and Schirmer test were performed to assess lacrimal function unit. Levator resection was performed for 16 eyes with levator function (LF) more than 4 mm, while eight eyes from four patients with poorer LF underwent frontalis silicone sling surgery. Main outcome measures included change in marginal-to-reflex distance (MRD1) and incidence of post-operative exposure keratopathy. RESULTS: The mean age of the patients was 45 ± 15 years. The mean of TBUT and Schirmer test were 12 ± 1.98 s and 15 ± 2.76 mm, respectively. The mean follow-up period was 34 months. Four eyes encountered mild to moderate exposure keratopathy. All cases were managed medically, with no need for surgical revision. The mean pre-operative MRD1, LF, and chin-up angle were -0.54 ± 1.03 mm, 4.21 ± 1.41 mm, and 21.6 ± 6.01 degrees, respectively. The mean post-operative MRD1, LF, and chin-up angle were 2.42 ± 0.60, 4.46 ± 1.53 mm, and 3.3 ± 1.01 degrees, respectively. At 1-year follow-up visit, improvement in MRD1 and chin-up posture was statistically significant (p-value < 0.05). CONCLUSION: Normal TBUT and Schirmer test results, prophylactic lubricating therapy, and close follow-up can be as important as Bell's phenomenon and palpebral fissure height in predicting post-operative complications.
