Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention.

BACKGROUND: We believe that our experience with patients presenting with Coats disease and macular sparing should be shared with our colleagues. We would like to show the effect of posture and prompt intervention in cases with fovea-threatening and/or fovea-involving peripheral Coats disease (FTPCD). This association has been poorly debated in our specialty and literature. We call the attention for the unexpexted scenario of observing the lost of the fovea during some types of traditional and prompt interventional treatments of these cases with previous 20/20 vision (something that we have been studying and observing for many years). In order to publish our best representative cases, we have chosen 8 Brazilian patients (age range, 7-62 years; 5 male) with FTPCD. All patients underwent multimodal imaging and different treatments (observation, sleep-posture repositioning, laser, intraocular steroids, and/or anti-vascular endothelial growth factor therapy). All patients, initially, informed to adopt a sleeping lateral-down position, favoring exudation shifting to the fovea pre-treatment. Most promptly-treated patients in this way (n = 4), developed subretinal fluid and exudates in the macula and some had irreversible central visual loss (n = 3). Patients with recent fovea-involving exudation who changed postural sleep position (to protect the foveal area) before and during treatment fared better, with some preserved central vision and an intact fovea (n = 5). The fundus status was correlated with the gravitational effects of posture before and after treatment. Despite prepared as an observational/interventional study, with a small number of cases, the most difficult part is documenting the sleep position of these patients and its influence in the outcomes as there is not good way to prove how well or poorly the positioning occurred in our cases. Finally, we also intended to call the attention to the fact that Coats disease must be studied in all its clinical stage variants and not only seen as a potential blinding and incurable ocular disease. CASE PRESENTATION: This study is a retrospective and/or interventional analysis of eight cases with a less severe clinical variant of classic Coats disease that we refer to as fovea-threatening and fovea-involving peripheral Coats disease (FTPCD). All cases were unilateral with no systemic disease or family history of Coats disease. The bilateral anterior segment and intraocular pressure were normal in all patients. The characteristics of all patients are shown in the Table. CONCLUSION: The funduscopic features of FTPCD are fundamental to disease understanding and optimal management. Habitual posturing may affect the fundus morphologic features of retinal exudation as observed in all current patients with exudative peripheral Coats disease. When sleep habitual posture is not observed in patients with FTPCD, the effects of prompt invasive treatments can cause rapid visual loss because of foveal subretinal pooling of exudates post-treatment. Initial vigilant adjusting of the habitual sleep posture for several patients with FTPCD, before the indication of traditional invasive treatments (laser and/or pharmacologic medications) can result in improved vision and fundoscopic morphologic features.

Intermediate-dose cytarabine plus G-CSF as mobilization regimen for newly diagnosed multiple myeloma and heavily pre-treated patients with hematological and non-hematological malignancies.

BACKGROUND: Intermediate-dose cytarabine plus G-CSF has recently emerged as safe and effective mobilization regimen for heavily pre-treated patients with lymphoid malignancies. We prospectively tested this regimen in patients referred to our center in order to collect enough stem cells for hematopoietic rescue in autologous transplantation (auto-HSCT). STUDY DESIGN AND METHODS: cytarabine (1.6 g/m2) plus G-CSF (outpatient administration) was performed in 81 consecutive patients who underwent auto-HSCT. For analyses purposes patients were divided into Group A, consisted of 48 patients with newly diagnosed multiple myeloma (MM) and Group B with 33 heavily pre-treated patients (13 Hodgkin´s lymphoma, 7 non-Hodgkin´s lymphoma, 7 MM, 4 germ cell tumor, 2 non-promyelocytic acute myeloid leukemia). RESULTS: In the Group A, circulating CD34+ cells/µL was significantly higher, 90% started stem cell harvest on day 14, 98% collected ≥5.0 × 106 CD34+cells/kg and a single apheresis was sufficient in 92% of the cases. In the Group B, 85% started leukapheresis on day 14, 88% collected ≥2.0 × 106 CD34+cells/kg which was achieved with a single apheresis in 82% of the cases; a higher proportion of the patients (63.6% versus 33.3%) required platelet transfusions. Both groups exhibited few adverse events and the time to neutrophil and platelet recovery was similar between groups. CONCLUSION: Intermediate-dose cytarabine plus G-CSF mobilization is effective even for heavily pre-treated patients. The outpatient administration of G-CSF, the reliable prediction of the day to begin harvesting, the optimal CD34+ cell yield obtained with a single apheresis and the fewer occurrences of adverse events denoted the benefits of this regimen.

Combined branch retinal vein and artery occlusion in toxoplasmosis.

A 22-year-old man complained of low visual acuity and pain in his left eye for five days. His ophthalmological examination revealed 2+ anterior chamber reaction and a white, poorly defined retinal lesion at the proximal portion of the inferotemporal vascular arcade. There were retinal hemorrhages in the inferotemporal region extending to the retinal periphery. In addition, venous dilation, increased tortuosity, and ischemic retinal whitening along the inferotemporal vascular arcade were also observed. A proper systemic work-up was performed, and the patient was diagnosed with ocular toxoplasmosis. He was treated with an anti-toxoplasma medication, and his condition slowly improved. Inferior macular inner and middle retinal atrophy could be observed on optical coherence tomography as a sequela of ischemic injury. To our knowledge, this is the first report of combined retinal branch vein and artery occlusion in toxoplasmosis resulting in a striking and unusual macular appearance.

Combined branch retinal vein and artery occlusion in toxoplasmosis / Oclusão combinada de ramo arterial e venoso retinianos em toxoplasmose

ABSTRACT A 22-year-old man complained of low visual acuity and pain in his left eye for five days. His ophthalmological examination revealed 2+ anterior chamber reaction and a white, poorly defined retinal lesion at the proximal portion of the inferotemporal vascular arcade. There were retinal hemorrhages in the inferotemporal region extending to the retinal periphery. In addition, venous dilation, increased tortuosity, and ischemic retinal whitening along the inferotemporal vascular arcade were also observed. A proper systemic work-up was performed, and the patient was diagnosed with ocular toxoplasmosis. He was treated with an anti-toxoplasma medication, and his condition slowly improved. Inferior macular inner and middle retinal atrophy could be observed on optical coherence tomography as a sequela of ischemic injury. To our knowledge, this is the first report of combined retinal branch vein and artery occlusion in toxoplasmosis resulting in a striking and unusual macular appearance.

RESUMO Um paciente do sexo masculino, com 22 anos de idade, queixou-se de redução da acuidade visual no olho esquerdo por 5 dias. O exame oftalmológico mostrou reação de câmara anterior 2+ e uma lesão retiniana esbranquiçada, pouco definida, na porção proximal da arcada vascular temporal inferior. Foram observadas hemorragias retinianas na região temporal inferior estendendo-se à periferia, assim como ingurgitamento venoso, aumento da tortuosidade e palidez isquêmica da retina no mesmo quadrante. Exames laboratoriais corroboraram o diagnóstico de toxoplasmose ocular. O paciente melhorou lentamente após tratamento apropriado. Foi evidenciada atrofia da retina macular inferior interna e média à tomografia de coerência óptica, como sequela da isquemia retiniana. Para nosso conhecimento, este é o primeiro relato de oclusão retiniana combinada de ramo arterial e venoso em toxoplasmose ocular, levando a um aspecto fundoscópico atípico e peculiar.

Trends in mortality of adult patients diagnosed with myeloid leukemia from 1994 to 2011 in southeastern Brazil.

OBJECTIVE: To evaluate trends in mortality among adults with myeloid leukemia in the Vale do Paraíba, State of São Paulo. METHODS: Data from the Brazilian National Health Service database DATASUS provided the number of deaths caused by myeloid leukemia and the number of inhabitants per year in the Regional Health Division XVII from 1994 to 2011. Registries were categorized according to gender into four age ranges (over 20 years, 20-49, 50-69 and over 70 years) for an estimation of the annual percent change for age-adjusted mortality rates. The percent changes were calculated using the Joinpoint regression analysis model. RESULTS: Overall, a significant decline per year was demonstrated for the entire sample (over 20 years) across the 18-year period studied (annual percent change: -5.59%; 95% CI: -8.5 to -2.5% for males; p-value<0.05 and -7.02%; 95% CI -11.2 to -2.8% for females; p-value<0.05) with no significant difference between genders. In an analysis using two Joinpoints, significant drops were observed from 1994 to 2001 (annual percent change: -21.22%; 95% confidence interval: -27.9 to -13.9%; p-value<0.05) and from 1994 to 2003 (annual percent change: -12.86%; 95% confidence interval -22.2 to -2.5%; p-value<0.05) for men and women, respectively. The declining trends were greatest for patients aged over 70 years with the age-adjusted mortality rates in younger groups declining non-significantly except for males aged 50-69 years old. CONCLUSION: Our data suggest a significant decline per year in age-adjusted mortality rates of adult patients diagnosed with myeloid leukemia from 1994 to 2011 in the Vale do Paraíba, State of São Paulo.

Prevalence of anemia in a sample of elderly southeastern Brazilians.

OBJECTIVE: To demonstrate the proportion of anemia and its association with demographic and clinical characteristics in a representative sample of elderly people from São José dos Campos, São Paulo. METHODS: Demographic data and blood samples were collected from 398 over 65-year-old male and female individuals. Anemia was defined as hemoglobin concentration <12g/dL in women and <13g/dL in men. Anemic and non-anemic groups were compared using the chi-squared test and a multiple logistic regression model. RESULTS: The prevalence of anemia was 18.6% (20.8% in men and 17.6% in women). The percentages of anemia rose significantly across the age groups >75-80, >85-90 and >90-95 years (p-value=0.0251). There were no significant differences in gender, ethnic background, place of residence, years of schooling, income, comorbidities and use of medications. According to gender, the mean hemoglobin concentration and mean corpuscular volume were 11.5g/dL (range: 8.4-11.9g/dL) and 90.7fL (range: 63.0-111.7fL) for women and 11.9g/dL (range: 8.6-12.8g/dL) and 92.1fL (range: 59.8-100.1fL) for men. The great majority of anemia cases were mild with less than 6% having hemoglobin concentrations below 10.9g/dL. Mean corpuscular volume was lower than 80fL in six cases (8%), between 80 and 100fL in 65 cases (88%) and higher than 100fL in three cases (4%). CONCLUSION: A total of 18.6% of elderly people from São José dos Campos had mild anemia with the majority being normocytic. The percentages of anemia rose as the age increased demonstrating an association between age and anemia.

Trends in mortality of adult patients diagnosed with myeloid leukemia from 1994 to 2011 in southeastern Brazil

Objective: To evaluate trends in mortality among adults with myeloid leukemia in the Vale do Paraíba, State of São Paulo. Methods: Data from the Brazilian National Health Service database DATASUS provided the number of deaths caused by myeloid leukemia and the number of inhabitants per year in the Regional Health Division XVII from 1994 to 2011. Registries were categorized according to gender into four age ranges (over 20 years, 20-49, 50-69 and over 70 years) for an estimation of the annual percent change for age-adjusted mortality rates. The percent changes were calculated using the Joinpoint regression analysis model. Results: Overall, a significant decline per year was demonstrated for the entire sample (over 20 years) across the 18-year period studied (annual percent change: −5.59%; 95% CI: −8.5 to −2.5% for males; p-value < 0.05 and −7.02%; 95% CI −11.2 to −2.8% for females; p-value < 0.05) with no significant difference between genders. In an analysis using two Joinpoints, significant drops were observed from 1994 to 2001 (annual percent change: −21.22%; 95% confidence interval: −27.9 to −13.9%; p-value < 0.05) and from 1994 to 2003 (annual percent change: −12.86%; 95% confidence interval −22.2 to −2.5%; p-value < 0.05) for men and women, respectively. The declining trends were greatest for patients aged over 70 years with the age-adjusted mortality rates in younger groups declining non-significantly except for males aged 50-69 years old. Conclusion: Our data suggest a significant decline per year in age-adjusted mortality rates of adult patients diagnosed with myeloid leukemia from 1994 to 2011 in the Vale do Paraíba, State of São Paulo. .

Prevalence of anemia in a sample of elderly southeastern Brazilians

Objective: To demonstrate the proportion of anemia and its association with demographic and clinical characteristics in a representative sample of elderly people from São José dos Campos, São Paulo. Methods: Demographic data and blood samples were collected from 398 over 65-year-old male and female individuals. Anemia was defined as hemoglobin concentration <12 g/dL in women and <13 g/dL in men. Anemic and non-anemic groups were compared using the chi-squared test and a multiple logistic regression model. Results: The prevalence of anemia was 18.6% (20.8% in men and 17.6% in women). The per- centages of anemia rose significantly across the age groups >75-80, >85-90 and >90-95 years (p-value = 0.0251). There were no significant differences in gender, ethnic background, place of residence, years of schooling, income, comorbidities and use of medications. Accord- ing to gender, the mean hemoglobin concentration and mean corpuscular volume were 11.5 g/dL (range: 8.4-11.9 g/dL) and 90.7 fL (range: 63.0-111.7 fL) for women and 11.9 g/dL (range: 8.6-12.8 g/dL) and 92.1 fL (range: 59.8-100.1 fL) for men. The great majority of ane- mia cases were mild with less than 6% having hemoglobin concentrations below 10.9 g/dL. Mean corpuscular volume was lower than 80 fL in six cases (8%), between 80 and 100 fL in 65 cases (88%) and higher than 100 fL in three cases (4%). Conclusion: A total of 18.6% of elderly people from São José dos Campos had mild anemia with the majority being normocytic. The percentages of anemia rose as the age increased demonstrating an association between age and anemia. .

Ophthalmologic findings in hepatitis C patients treated with pegylated interferon α-2b and ribavirin.

PURPOSE: To identify the effect of pegylated interferon ƒ¿-2b and ribavirin treatment in the ocular fundus examination, visual acuity, and visual field. METHODS: Prospective observational study was performed at the Hepatology Clinic of Sao Jose Regional Hospital and at the Vitreoretinal Department at the Sadalla Amin Ghanem Eye Hospital in patients with chronic hepatitis C before and during treatment with pegylated interferon ƒ¿-2b together with ribavirin. RESULTS: Six (37.5%) of 16 patients developed retinopathy during the treatment, two of which (12.5%) presented retinal hemorrhage, and four patients (6 eyes) presented cotton-wool spots (25%) that regressed during the treatment. One patient (6.25%) presented transient decrease in visual acuity during the treatment and recovered spontaneously without specific therapy. CONCLUSION: Recommended treatment methods for hepatitis C may cause transient retinopathy, commonly without any damage to visual function in most patients. Although ocular involvement is rare, follow-up with an ophthalmologist is recommended during the course of the hepatitis C medication.

Ophthalmologic findings in hepatitis C patients treated with pegylated interferon α-2b and ribavirin / Achados oftalmológicos nos pacientes portadores de hepatite C em tratamento com interferon peguilado α-2b e ribavirina

Purpose: To identify the effect of pegylated interferon α-2b and ribavirin treatment in the ocular fundus examination, visual acuity, and visual field. Methods: Prospective observational study was performed at the Hepatology Clinic of São José Regional Hospital and at the Vitreoretinal Department at the Sadalla Amin Ghanem Eye Hospital in patients with chronic hepatitis C before and during treatment with pegylated interferon α-2b together with ribavirin. Results: Six (37.5%) of 16 patients developed retinopathy during the treatment, two of which (12.5%) presented retinal hemorrhage, and four patients (6 eyes) presented cotton-wool spots (25%) that regressed during the treatment. One patient (6.25%) presented transient decrease in visual acuity during the treatment and recovered spontaneously without specific therapy. Conclusion: Recommended treatment methods for hepatitis C may cause transient retinopathy, commonly without any damage to visual function in most patients. Although ocular involvement is rare, follow-up with an ophthalmologist is recommended during the course of the hepatitis C medication. .

Objetivo: Identificar possíveis mudanças no exame de fundo de olho após o início do tratamento, bem como alterações na acuidade visual e campo visual. Métodos: Estudo observacional prospectivo realizado na Clínica de Hepatologia do Hospital Regional de São José e no Departamento de Vítreo e Retina do Hospital de Olhos Sadalla Amin Ghanem, em pacientes com hepatite C crônica antes e durante o tratamento com interferon peguilado α-2b associado à ribavirina. Resultados: Six (37,5%) dos 16 participantes desenvolveram retinopatia durante o tratamento, dois dos quais (12,5%) apresentaram hemorragia retiniana unilateral, e quatro pacientes com exsudatos algodonosos (25%), seis olhos, que regrediu durante o tratamento. Um participante (6,25%) apresentou diminuição transitória da acuidade visual durante o tratamento com recuperação espontaneamente sem tratamento específico. Conclusão: O tratamento recomendado para a hepatite C pode estar associado com o desenvolvimento de retinopatia transitória, geralmente sem dano à função visual na maioria dos pacientes. Embora o envolvimento ocular seja raro, o acompanhamento com o médico oftalmologista é recomendado durante todo o uso da medicação. .

Associação de microburaco macular e fosseta de papila / Association of macular microhole and optic disc pit

A fosseta de papila do nervo óptico e o microburaco macular são duas patologias raras, cuja probabilidade de coexistência se torna extremamente baixa, embora não haja relação fisiopatológica entre ambas, descreveremos um caso de associação das mesmas, acometendo comumente um olho, a fim de analisar as manifestações clínicas, os exames de OCT, angiografia, retinografia, biomocroscopia, o tratamento e a correlação entre ambas patologias.

Optic disc pit and macular microhole are two rare pathologies with an extremely low likelihood of coexistence, this paper will report an association of both pathologies in the same eye with the purpose of analyzing clinical manifestations, tests, angiography, OCT, retinography, biomocroscopy, treatment outcome and the connection between the optic disc pit and macular microhole.

Oncohematological diseases in the Vale do Paraíba, State of São Paulo: demographic aspects, prevalences and incidences.

BACKGROUND: Based on the necessity of detailed information that supports effective strategies to improve cancer outcomes in the different regions of Brazil, the aims of this study were to report demographic aspects and to calculate the prevalence and incidence rates of oncohematological diseases in the region of Vale do Paraíba. METHODS: This is a multicentric prospective study carried out from October 2009 to March 2010. A total of 500 over 19-year-old patients were enrolled. Data such as type of healthcare insurance, gender, age, ethnic classification, place of residence, schooling, income, body mass index, new cases and the period between the first symptoms and a definite diagnosis were collected. The prevalence and incidence rates were calculated according to an estimated number of 1,319,800 inhabitants. RESULTS: The prevalence and incidence rates per 100,000 inhabitants in the period of six months were, respectively: acute myeloid leukemia 1.5 and 0.7; acute lymphoblastic leukemia 0.5 and 0.1; chronic lymphocytic leukemia 2.4 and 0.4; chronic myeloid leukemia 6.2 and 0.8; Hodgkin's lymphoma 2.9 and 0.9; non-Hodgkin lymphoma 9.8 and 4.3; multiple myeloma 5.7 and 0.7; myelodysplastic syndromes 2.1 and 0.2 and myeloproliferative syndromes 5.1 and 0.3. CONCLUSION: Giving the paucity of data in this field of investigation, our data may be useful for comparisons with those of other regions of Brazil and will assist in the implementation of treatment programs of oncohematological diseases in this region.

Oncohematological diseases in the Vale do Paraíba, State of São Paulo: demographic aspects, prevalences and incidences

BACKGROUND: Based on the necessity of detailed information that supports effective strategies to improve cancer outcomes in the different regions of Brazil, the aims of this study were to report demographic aspects and to calculate the prevalence and incidence rates of oncohematological diseases in the region of Vale do Paraíba. METHODS: This is a multicentric prospective study carried out from October 2009 to March 2010. A total of 500 over 19-year-old patients were enrolled. Data such as type of healthcare insurance, gender, age, ethnic classification, place of residence, schooling, income, body mass index, new cases and the period between the first symptoms and a definite diagnosis were collected. The prevalence and incidence rates were calculated according to an estimated number of 1,319,800 inhabitants. RESULTS: The prevalence and incidence rates per 100,000 inhabitants in the period of six months were, respectively: acute myeloid leukemia 1.5 and 0.7; acute lymphoblastic leukemia 0.5 and 0.1; chronic lymphocytic leukemia 2.4 and 0.4; chronic myeloid leukemia 6.2 and 0.8; Hodgkin's lymphoma 2.9 and 0.9; non-Hodgkin lymphoma 9.8 and 4.3; multiple myeloma 5.7 and 0.7; myelodysplastic syndromes 2.1 and 0.2 and myeloproliferative syndromes 5.1 and 0.3. CONCLUSION: Giving the paucity of data in this field of investigation, our data may be useful for comparisons with those of other regions of Brazil and will assist in the implementation of treatment programs of oncohematological diseases in this region.

[Posterior segment changes in microphthalmic eyes: case report]. / Alterações do segmento posterior na microftalmia: relato de casos.

Microphthalmos is a developmental ocular disorder defined as a small eyeball. The condition can be associated with abnormalities of anterior and posterior segments. The most common anterior characteristics include corneal opacities, angle-closure and a shallow anterior chamber and cataract. The main findings of posterior segment are uveal effusion, retinal folds, abnormalities of macular capillar vascularization, absence of foveal depression and peripheral retinoschisis. Three patients with microphthalmos were assisted and their OCT features of posterior segment were analyzed. The first case had uveal effusion syndrome, choroidal and retinal detachment treated with parcial sclerectomy at the four quadrants. The other case presented with neurosensory retinal fold at fovea and papillomacular area in both eyes without involvement of retinal pigment epithelium layer and choriocapillaris. The third patient showed absence of foveal depression. The main diferencial diagnosis for this condition is foveal hypoplasia.

Alterações do segmento posterior na microftalmia: relato de casos / Posterior segment changes in microphthalmic eyes: case report

Microftalmia é a alteração de desenvolvimento caracterizada pela redução do tamanho do bulbo ocular. Pode estar associada a manifestações estruturais do segmento anterior e posterior. As alterações mais comuns do segmento anterior são as opacidades corneanas, o estreitamento e fechamento do ângulo da câmara anterior e a catarata, e as mais frequentes no segmento posterior são a efusão uveal, as dobras de retina, a alteração da vascularização capilar macular, a ausência de depressão foveal e a retinosquise periférica. Este estudo descreve o caso de três pacientes com microftalmo e manifestações no segmento posterior documentadas com tomografia de coerência óptica. O primeiro caso apresentou a síndrome de efusão uveal, com descolamento de coróide e de retina, tratada com cirurgia de esclerectomia parcial nos quatro quadrantes. No segundo caso, havia a dobra retiniana neurossensorial na fóvea e feixe papilomacular em ambos os olhos sem o envolvimento de epitélio pigmentado retiniano e coriocapilar. No terceiro paciente, o microftalmo estava acompanhado de ausência da depressão foveal, cujo principal diagnóstico diferencial é a hipoplasia foveal.

Microphthalmos is a developmental ocular disorder defined as a small eyeball. The condition can be associated with abnormalities of anterior and posterior segments. The most common anterior characteristics include corneal opacities, angle-closure and a shallow anterior chamber and cataract. The main findings of posterior segment are uveal effusion, retinal folds, abnormalities of macular capillar vascularization, absence of foveal depression and peripheral retinoschisis. Three patients with microphthalmos were assisted and their OCT features of posterior segment were analyzed. The first case had uveal effusion syndrome, choroidal and retinal detachment treated with parcial sclerectomy at the four quadrants. The other case presented with neurosensory retinal fold at fovea and papillomacular area in both eyes without involvement of retinal pigment epithelium layer and choriocapillaris. The third patient showed absence of foveal depression. The main diferencial diagnosis for this condition is foveal hypoplasia.