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1.
Eur J Pediatr ; 183(2): 827-834, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38030929

RESUMO

Retinopathy of prematurity (ROP) is a leading cause of childhood blindness in preterm infants. The incidence of ROP varies widely across countries, with rates as high as 30% in some regions. This study investigated the incidence, risk factors, treatment, and mortality of ROP patients in Germany. Data were extracted from the German Federal Statistical Office (Destatis) diagnosis-related group (DRG) and Institute for the Remuneration System in Hospitals (InEK) databases. Patients with a secondary diagnosis of ROP (ICD-10 code H35.1) in the first 28 days of life were included. Data were extracted for patients admitted between January 1, 2019 and December 31, 2019. The diagnoses and procedures were determined using the German version of the International Classification of Diseases (ICD-10-GM) and the German procedure coding system (OPS). The codes 5-154.xx, 5-155.xx, 8-020.xx, 5-156.9, 6-003.(c&d), 6-007.(2&8) were utilised to denote different ocular treatments. Patient Clinical Complexity Levels were extracted and used to compare ROP with non-ROP patients. A total of 1326 patients with ROP were identified. The incidence of ROP is estimated to be 17.04 per 10,000 live births. The incidence was highest in infants with birth weights less than 500 g and decreased with increasing birth weight. The most common risk factors for ROP were low birth weight, male sex, and prematurity. Of the infants with ROP, 7.2% required ocular treatment. The most common treatment was intraocular injections, followed by photocoagulation. No surgical treatment was required for any of the infants during the study period. The mortality rate for infants with ROP was 60.33 per 10,000. This is higher than the overall neonatal death rate of 24.2 per 10,000. CONCLUSIONS: This study found that the incidence of ROP in Germany is similar to that in other developed countries. The study also found that the mortality rate for infants with ROP is higher than the overall neonatal death rate. These findings highlight the importance of early detection and treatment of ROP in preterm infants. WHAT IS KNOWN: • ROP is a severe eye condition often affecting preterm infants. • Previous data are limited in scope and generalizability. WHAT IS NEW: • Based on a national database, our study found ROP incidence to be 17.04 per 10,000 new births, higher in males (17.71) than in females (16.34). • 7.2% of ROP cases required ocular treatment, inversely correlated with birth weight. • High rates of multimorbidity such as neonatal jaundice (84.69%), respiratory distress syndrome (80.84%), and apnea (78.88%) were observed.


Assuntos
Morte Perinatal , Retinopatia da Prematuridade , Lactente , Feminino , Recém-Nascido , Humanos , Masculino , Recém-Nascido Prematuro , Peso ao Nascer , Estudos de Coortes , Retinopatia da Prematuridade/diagnóstico , Retinopatia da Prematuridade/epidemiologia , Retinopatia da Prematuridade/terapia , Incidência , Idade Gestacional , Fatores de Risco
2.
Graefes Arch Clin Exp Ophthalmol ; 262(8): 2431-2438, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38466396

RESUMO

PURPOSE: This nationwide study aims to delineate the incidence and trends of rhegmatogenous retinal detachment (rRD) in Germany across 17 years (2005-2021). METHODS: We conducted a retrospective cohort study using data from the German Federal Statistics Office and the Institute for the Hospital Remuneration System (InEK). The dataset includes approximately 19 million annual inpatient admissions annually. Retinal detachment was identified through ICD-10 code H33.0. Adjusted incidence rates were estimated after excluding reoperation cases. We used R Statistical Software to calculate estimates to 2021, and Tableau for visualisation. RESULTS: From 2005 to 2021, Germany reported 332,650 rRD cases, with males consistently more affected. Adjusted incidence rose from 15.6 per 100,000 in 2005 to 24.8 in 2021. Variable annual percentage changes in incidence were noted, averaging 4.0% for males and 2.6% for females. The annual mean age of affected individuals ranged from 60.2 to 62 years, with a median age between 62 and 63, suggesting increasing diagnoses at younger ages. Hospital stays declined from 6 to 3.3 days, and higher management rates were observed in Saarland and Rhineland-Palatine. CONCLUSION: The study confirms an increasing incidence of rRD in Germany from 2005 to 2021, particularly among males. These findings call for further research to investigate the underlying causes. Collaboration among healthcare professionals, researchers, and policymakers is essential for effective management and improved visual outcomes.


Assuntos
Descolamento Retiniano , Humanos , Alemanha/epidemiologia , Descolamento Retiniano/epidemiologia , Descolamento Retiniano/diagnóstico , Incidência , Estudos Retrospectivos , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Idoso , Distribuição por Idade , Distribuição por Sexo , Adolescente , Adulto Jovem , Criança , Idoso de 80 Anos ou mais , Pré-Escolar
3.
J Neuroinflammation ; 19(1): 260, 2022 Oct 22.
Artigo em Inglês | MEDLINE | ID: mdl-36273134

RESUMO

BACKGROUND: Forkhead-Box-Protein P3 (FoxP3) is a transcription factor and marker of regulatory T cells, converting naive T cells into Tregs that can downregulate the effector function of other T cells. We previously detected the expression of FoxP3 in retinal pigment epithelial (RPE) cells, forming the outer blood-retina barrier of the immune privileged eye. METHODS: We investigated the expression, subcellular localization, and phosphorylation of FoxP3 in RPE cells in vivo and in vitro after treatment with various stressors including age, retinal laser burn, autoimmune inflammation, exposure to cigarette smoke, in addition of IL-1ß and mechanical cell monolayer destruction. Eye tissue from humans, mouse models of retinal degeneration and rats, and ARPE-19, a human RPE cell line for in vitro experiments, underwent immunohistochemical, immunofluorescence staining, and PCR or immunoblot analysis to determine the intracellular localization and phosphorylation of FoxP3. Cytokine expression of stressed cultured RPE cells was investigated by multiplex bead analysis. Depletion of the FoxP3 gene was performed with CRISPR/Cas9 editing. RESULTS: RPE in vivo displayed increased nuclear FoxP3-expression with increases in age and inflammation, long-term exposure of mice to cigarette smoke, or after laser burn injury. The human RPE cell line ARPE-19 constitutively expressed nuclear FoxP3 under non-confluent culture conditions, representing a regulatory phenotype under chronic stress. Confluently grown cells expressed cytosolic FoxP3 that was translocated to the nucleus after treatment with IL-1ß to imitate activated macrophages or after mechanical destruction of the monolayer. Moreover, with depletion of FoxP3, but not of a control gene, by CRISPR/Cas9 gene editing decreased stress resistance of RPE cells. CONCLUSION: Our data suggest that FoxP3 is upregulated by age and under cellular stress and might be important for RPE function.


Assuntos
Degeneração Macular , Epitélio Pigmentado da Retina , Animais , Humanos , Camundongos , Ratos , Células Cultivadas , Células Epiteliais/metabolismo , Células Epiteliais/patologia , Fatores de Transcrição Forkhead/genética , Fatores de Transcrição Forkhead/metabolismo , Inflamação/genética , Inflamação/metabolismo , Degeneração Macular/genética , Degeneração Macular/metabolismo , Degeneração Macular/patologia , Epitélio Pigmentado da Retina/metabolismo , Epitélio Pigmentado da Retina/patologia , Pigmentos da Retina/genética , Pigmentos da Retina/metabolismo , Fatores de Transcrição/genética , Fatores de Transcrição/metabolismo
4.
Graefes Arch Clin Exp Ophthalmol ; 260(5): 1723-1731, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-34605956

RESUMO

PURPOSE: To calculate the overall incidence of uveal melanoma in Germany and to compare incidences in different German states. In addition, we computed the overall and cancer-specific survival rates nationwide. METHODS: Incidence data for the period between 2009 and 2015, covering the entire German population, was collected through the German Center for Cancer Registry. ICD-O-3 topography codes C69.3-C69.4 and histology codes for melanoma subtypes were used to collect the incidence data. Confidence Intervals with a level of 95% (95% CI) were calculated for rates. Survival was calculated using the Kaplan-Meier. The log-rank test was used for survival comparisons. RESULTS: This study comprised 3654 patients with uveal melanomas, including 467 (12.8%) with iridial and ciliary body tumors. The overall age-standardized incidence rate (ASIR) was 6.41 person per million. Generally, the ASIR was higher in males than females (6.67 (95% CI 6.37-6.98) vs. 6.16 (95% CI 5.88-6.45 per million). Higher crude incidence rates were noted in the northeastern states (12.5 per million (95% CI 10.5-14.7) in Mecklenburg-Vorpommern) compared with the southwestern states (2.1 per million (95% CI 1.7-2.6) in Hessen). The 5-year overall survival stood at 47%, while the cancer-specific survival stood at 84%. Multivariate analysis showed that women, younger patients, and patients living in Berlin achieved significantly higher overall survival. CONCLUSION: Overall ASIR of uveal melanoma in Germany indicates that the disease is more common in males and that it follows the same geographical distribution previously noted in central European countries, with the highest incidence in northern parts of Germany.


Assuntos
Melanoma , Neoplasias Uveais , Feminino , Alemanha/epidemiologia , Humanos , Incidência , Masculino , Melanoma/epidemiologia , Taxa de Sobrevida , Neoplasias Uveais/epidemiologia
5.
Int Ophthalmol ; 42(12): 3857-3867, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35821361

RESUMO

BACKGROUND: Marital status influences the presentation and outcome of various cancers. We explored the relationship between marital status and survival of uveal melanoma (UM) and factors influencing this relationship. METHODS: We conducted a retrospective cohort study on patients diagnosed with UM and registered in the Surveillance Epidemiology and End Results program between 1973 and 2017. Cox regression model was conducted to calculate the hazard ratio of overall and cancer-specific survival rate and delineate the effect of each confounder. RESULTS: The study involved 10,557 patients with a male-to-female ratio of 1:1.1. Most of the diagnosed patients were aged between 40 and 79 years (81%). Married patients (62%) represented the majority, followed by singles (12%), widowed (11%), and then divorced patients (7%). Single patients were the youngest group (mean age of 59.3 years) while widowed patients were the oldest (mean age of 75.8 years). In the Cox regression model for overall survival, married and single patients exhibited the best overall survival (no significant difference in between them), both surpassing divorced and widowed patients. Married patients were at a significantly lower risk to die from UM than divorced patients. Female patients and younger age groups showed the best overall and cancer-specific survival. CONCLUSION: Maintained marriages improved the survival of UM patients. Widowed and divorced patients should be included in specially designed support programs during their cancer management.


Assuntos
Melanoma , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Programa de SEER , Estudos Retrospectivos , Estado Civil , Melanoma/terapia
6.
FASEB J ; 34(3): 4055-4071, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31930599

RESUMO

The BEST1 gene product bestrophin-1, a Ca2+ -dependent anion channel, interacts with CaV 1.3 Ca2+ channels in the retinal pigment epithelium (RPE). BEST1 mutations lead to Best vitelliform macular dystrophy. A common functional defect of these mutations is reduced trafficking of bestrophin-1 into the plasma membrane. We hypothesized that this defect affects the interaction partner CaV 1.3 channel affecting Ca2+ signaling and altered RPE function. Thus, we investigated the protein interaction between CaV 1.3 channels and bestrophin-1 by immunoprecipitation, CaV 1.3 activity in the presence of mutant bestrophin-1 and intracellular trafficking of the interaction partners in confluent RPE monolayers. We selected four BEST1 mutations, each representing one mutational hotspot of the disease: T6P, F80L, R218C, and F305S. Heterologously expressed L-type channels and mutant bestrophin-1 showed reduced interaction, reduced CaV 1.3 channel activity, and changes in surface expression. Transfection of polarized RPE (porcine primary cells, iPSC-RPE) that endogenously express CaV 1.3 and wild-type bestrophin-1, with mutant bestrophin-1 confirmed reduction of CaV 1.3 surface expression. For the four selected BEST1 mutations, presence of mutant bestrophin-1 led to reduced CaV 1.3 activity by modulating pore-function or decreasing surface expression. Reduced CaV 1.3 activity might open new ways to understand symptoms of Best vitelliform macular dystrophy such as reduced electro-oculogram, lipofuscin accumulation, and vision impairment.


Assuntos
Bestrofinas/metabolismo , Canais de Cálcio Tipo L/metabolismo , Epitélio Pigmentado da Retina/citologia , Epitélio Pigmentado da Retina/metabolismo , Animais , Bestrofinas/genética , Western Blotting , Células CHO , Canais de Cálcio Tipo L/genética , Células Cultivadas , Cricetulus , Humanos , Imunoprecipitação , Células-Tronco Pluripotentes Induzidas/metabolismo
7.
Graefes Arch Clin Exp Ophthalmol ; 259(2): 515-526, 2021 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32870371

RESUMO

PURPOSE: The eye and its adnexal structures can give rise to first or consecutive primary malignancies or to encounter metastasis. Our aim was to define the characteristics of the second primary neoplasms affecting the eye and its adnexa and find the risk modifying factors for them after malignancies elsewhere in the body. METHODS: We have queried the Surveillance, Epidemiology and End-Results "SEER"-9 program of the National Cancer Institute for the malignancies of the eye and its adnexa that occurred between 1973 and 2015. The malignancies were ordered chronologically according to their incidence: first or second primary malignancies. The tumors were classified according to ICD-O-3 classification. Standardized incidence ratios (SIR) and survival probabilities were calculated for subgroups. RESULTS: Among 3,578,950 cancer patients, 1203 experienced a second malignancies of the eye and its adnexa. The first malignancy was diagnosed between 50 and 69 years of age in 58.94% of them. The eyelid showed 280 events, while 50 in lacrimal gland, 181 in the orbit, 21 in the overlapping lesions, 15 in optic nerve, 148 in the conjunctiva, 9 in the cornea, 6 in the Retina, 379 in the choroid, and 93 in the ciliary body. The SIR of a second malignancy after a prior non-Hodgkin lymphoma was 2.42, and in case of previous skin carcinomas it was 3.02, melanoma of skin, and 2.13 and 1.58 in oral cavity/pharynx malignancies. The second ocular and adnexal neoplasms increased steadily over the 5-year periods on contrary to first primary neoplasms. The survival of patients affected with first ocular and adnexal neoplasms was significantly higher than those with second ocular and adnexal neoplasms. On the other side, second primary ocular and adnexal tumors showed a better survival than second primary malignancies elsewhere. CONCLUSIONS: The epidemiological differences between first and second ocular and adnexal primaries suggest different underlying mechanisms. Careful ocular examination should be integrated in the long-term follow-up plan of cancer patients. Special attention should be given to patients with non-Hodgkin's lymphoma and melanoma as first primary.


Assuntos
Neoplasias Oculares , Aparelho Lacrimal , Linfoma não Hodgkin , Segunda Neoplasia Primária , Túnica Conjuntiva , Neoplasias Oculares/diagnóstico , Neoplasias Oculares/epidemiologia , Humanos , Linfoma não Hodgkin/diagnóstico , Linfoma não Hodgkin/epidemiologia , Segunda Neoplasia Primária/diagnóstico , Segunda Neoplasia Primária/epidemiologia , Órbita
8.
Pediatr Hematol Oncol ; 37(3): 198-210, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31971470

RESUMO

Adrenocortical carcinoma (ACC) is a rare, aggressive endocrine neoplasm. Complete surgical resection is the single most important treatment. Most available information has been learned from experience with its more frequent adult counterpart. In this study, we assessed the features and survival outcome of patients with ACC at Children's Cancer Hospital Egypt (CCHE). Patients diagnosed at CCHE between July 2007 and November 2016 were followed up on until November 2018. Patients with stages I and II were operated upon, while stages III and IV had received combinations of doxorubicin, etoposide, platinol, and mitotane (DEPM) beside the attempt to conduct surgery when feasible. Data belonging to 18 patients (7 men and 11 women) were analyzed; median age at diagnosis was 48.5 months. Sixteen patients had presented with secreting tumors. Six patients were diagnosed with stage I disease; four with stage II; three with stage III; and five with stage IV carcinoma. By the end of this study, 10 patients have survived; five-year overall survival of 66.3%. Surviving patients were all of stage I or II diseases and were all in remission. Seven patients who did not survive died due to tumor progression, while one patient died after chemotherapy. The prognosis of ACC is essentially dependent on a successful complete resection of the tumor and thus on the initial tumor stage. The mitotane and DEP protocols may help control tumor growth in the advanced stages for only short periods. Key pointsInitial stage and resectability are the main indicators of outcomes in adrenocortical carcinoma.Chemotherapeutic agents used in developed countries did not achieve the same outcomes.Further molecular-pharmacology differentiation is needed for various ethnic populations.


Assuntos
Neoplasias do Córtex Suprarrenal , Carcinoma Adenoide Cístico , Neoplasias do Córtex Suprarrenal/mortalidade , Neoplasias do Córtex Suprarrenal/terapia , Carcinoma Adenoide Cístico/mortalidade , Carcinoma Adenoide Cístico/terapia , Criança , Pré-Escolar , Países em Desenvolvimento , Intervalo Livre de Doença , Egito/epidemiologia , Feminino , Humanos , Masculino , Estadiamento de Neoplasias , Taxa de Sobrevida
9.
Cancer ; 125(6): 972-979, 2019 03 15.
Artigo em Inglês | MEDLINE | ID: mdl-30613943

RESUMO

BACKGROUND: The suicide risk after a new cancer diagnosis remains a controversial issue. This study examines the suicide risk within the year after a cancer diagnosis. This is the largest study to assess recent trends in suicide risk after a cancer diagnosis. METHODS: Data were obtained from the Surveillance, Epidemiology, and End Results Program. All patients diagnosed with cancer between 2000 and 2014 were selected. The event was defined as death due to suicide within the first year after a cancer diagnosis, and patients who experienced the event after their diagnosis were observed. The observed/expected (O/E) ratio was assessed as well as the excess risk per 10,000 person-years to determine the suicide risk change after the diagnosis in comparison with the general population. RESULTS: A total of 4,671,989 patients with cancer were included; 1585 committed suicide within 1 year of their diagnosis. The risk of suicide increased significantly with an O/E ratio of 2.52 and with an excess risk of 2.51 per 10,000 person-years. When the risk of suicide was studied according to the cancer site, the highest increases in the O/E ratio came after diagnoses of pancreatic cancer (8.01) and lung cancer (6.05). The risk of suicide also increased significantly after a diagnosis of colorectal cancer with an O/E ratio of 2.08. However, the risk of suicidal death did not increase significantly after breast and prostate cancer diagnoses. CONCLUSIONS: The risk of suicide increases significantly in the first year after a diagnosis of cancer in comparison with the general population, and this increase varies with the type and prognosis of cancer. Close observation and referral to mental health services, when indicated, are important for mitigating such risk.


Assuntos
Neoplasias/diagnóstico , Neoplasias/mortalidade , Suicídio Consumado/tendências , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/diagnóstico , Neoplasias da Mama/mortalidade , Neoplasias Colorretais/diagnóstico , Neoplasias Colorretais/mortalidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias da Próstata/diagnóstico , Neoplasias da Próstata/mortalidade , Medição de Risco , Programa de SEER , Suicídio Consumado/estatística & dados numéricos , Adulto Jovem
10.
Int J Neurosci ; 129(5): 447-454, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30366515

RESUMO

BACKGROUND: Glioblastoma multiforme (GBM) patients with a prior malignancy are usually excluded from clinical trials on GBM based on the assumption that this history will affect their survival outcomes. This practice may affect clinical trial accrual and limit the gathering of knowledge essential to the formulation of therapeutic options for this patient population. However, not much is known about the real impact of these prior malignancies on the survival of patients with subsequent GBM. We aimed to investigate the degree of such an impact. PATIENTS AND METHODS: We used the Surveillance, Epidemiology, and End Results (SEER) Program to analyze data of GBM patients diagnosed between 1973 and 2014. We calculated the overall and GBM-specific survival of these patients using the unadjusted Kaplan-Meier test and the multivariable covariate-adjusted Cox models. RESULTS: Of 51,158 GBM patients, 3,076 had a prior malignancy. The unadjusted Kaplan-Meier test showed worse overall and GBM-specific survivals for patients who had a prior history of cancer. However, after adjusting for age at diagnosis of GBM, sex, race, marital status, and conduction of surgery, multivariable covariate-adjusted Cox models showed that having a prior malignancy did not significantly affect neither overall survival (HR = 1.025, 95%CI = .986 - 1.066, p = .213) nor GBM-specific survival (HR = 1.005, 95%CI = .963 - 1.049, p = .810). CONCLUSIONS: Our findings suggest that the broad practice of excluding patients with a prior history of cancer should be reconsidered as it may adversely affect accrual, trial completion rates, and generalizability of the results.


Assuntos
Neoplasias Encefálicas/mortalidade , Glioblastoma/mortalidade , Segunda Neoplasia Primária/mortalidade , Sistema de Registros , Análise de Sobrevida , Adolescente , Adulto , Fatores Etários , Idoso , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Modelos de Riscos Proporcionais , Adulto Jovem
11.
Int J Clin Oncol ; 23(4): 625-633, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29492793

RESUMO

BACKGROUND: Thyroid carcinoma is a very rare tumor in the pediatric age group, accounting for only 1.5-3% of childhood carcinomas in the United States and Europe. We aimed to identify the risk of a second malignancy among pediatric thyroid cancer survivors. METHODS: The cohort analysis consisted of pediatric cancer patients aged less than 20 years, diagnosed with a primary thyroid cancer, identified by site code ICD-0-3: C739, and reported to the SEER 9 database between 1973 and 2013. They were followed up until December 31, 2013; the end of the study period, or up to death if earlier. RESULTS: Out of 1769 patients diagnosed primarily with thyroid carcinoma, 42 patients had a total of 45 incidences of subsequent malignancies. The mean age of patients at the initial diagnosis of thyroid cancer was 16 years. Females (90.5%) had a significantly higher incidence of second malignancies (SM) than males (9.5%). The overall Standardized Incidence Ratio (SIR) of SM in the study patients was higher than expected (SIR = 1.48). Some specific sites showed significantly higher incidences: the salivary glands (SIR = 33.95), the gum and other parts of the mouth [excluding the lips, tongue, salivary glands and floor of the mouth] (SIR = 24.53)*** and the kidneys (SIR = 5.72). The overall risk of SM in patients who had received radioactive iodine was higher than expected (SIR = 4.41). The cumulative incidence of SM after treatment of thyroid cancer in children increases steadily over 40 years (11.92%). CONCLUSIONS: Race, gender, histological subtypes, and radioactive iodine are potentially significant prognostic factors for the development of SM among pediatric thyroid cancer survivors. Identification of underlying mechanisms that raise the risk of SM is important for both treatment and follow-up strategies.


Assuntos
Adenocarcinoma Folicular/radioterapia , Adenocarcinoma Papilar/radioterapia , Carcinoma Medular/radioterapia , Segunda Neoplasia Primária/epidemiologia , Radioterapia , Sobreviventes/estatística & dados numéricos , Neoplasias da Glândula Tireoide/radioterapia , Adenocarcinoma Folicular/patologia , Adenocarcinoma Papilar/patologia , Adolescente , Adulto , Carcinoma Medular/patologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Incidência , Lactente , Masculino , Prognóstico , Fatores de Risco , Programa de SEER , Taxa de Sobrevida , Neoplasias da Glândula Tireoide/patologia , Estados Unidos/epidemiologia , Adulto Jovem
12.
Childs Nerv Syst ; 34(3): 431-439, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29299687

RESUMO

PURPOSE: Incidence of BS primitive neuroectodermal tumors (BS-PNET) in children is not reported to date. Our main objectives were to estimate the incidence and report the outcome of BS-PNET in children. METHODS: Data were collected using the Surveillance Epidemiology and End Results cancer registry. RESULTS: From 1973 to 2013, we identified 83 pediatric patients (aged 0-21 years). Patients were divided into two age groups (0-3 years and 4-21 years). Median overall survival was 53 months. Patients in the older age group had a significant survival advantage (P < 0.001), as did those who received three modalities of therapy (surgery, chemotherapy, and radiation therapy) (P < 0.001) and patients with gross or subtotal tumor resection (P < 0.001). CONCLUSIONS: This study presents the first estimate of incidence and the largest cohort of pediatric BS-PNETs to date. A high index of suspicion of BS-PNET in similar cases is crucial for diagnosis, treatment, and outcome.


Assuntos
Neoplasias do Tronco Encefálico/diagnóstico por imagem , Neoplasias do Tronco Encefálico/epidemiologia , Análise de Dados , Tumores Neuroectodérmicos Primitivos/diagnóstico por imagem , Tumores Neuroectodérmicos Primitivos/epidemiologia , Programa de SEER/tendências , Adolescente , Neoplasias do Tronco Encefálico/terapia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Tumores Neuroectodérmicos Primitivos/terapia , Adulto Jovem
13.
J Pediatr Hematol Oncol ; 39(2): 150-152, 2017 03.
Artigo em Inglês | MEDLINE | ID: mdl-28060131

RESUMO

Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin. Mitotane was added when a pulmonary metastasis was discovered and then stopped after the patient suffered from an arachnoid cyst and speech difficulties. The ALL protocol was resumed from week 34 of the continuation phase. Progression of pulmonary nodules was noted after week 45. A pulmonary metastectomy was performed. The ALL protocol was resumed up to week 51 with a good response as proven by assessment of minimal residual disease. A further recurrence was diagnosed at the abdominal tumor bed with a paravertebral mass and a pulmonary nodule. The patient was assigned to palliative treatment and died after a 32-month survival. Such rare associations need more extensive discussions of the best possible management in scientific literature.


Assuntos
Neoplasias do Córtex Suprarrenal/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma/diagnóstico , Neoplasias Primárias Múltiplas/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras B/diagnóstico , Neoplasias do Córtex Suprarrenal/diagnóstico por imagem , Neoplasias do Córtex Suprarrenal/tratamento farmacológico , Neoplasias do Córtex Suprarrenal/cirurgia , Adrenalectomia , Carcinoma/diagnóstico por imagem , Carcinoma/secundário , Carcinoma/terapia , Cisplatino/administração & dosagem , Terapia Combinada , Doxorrubicina/administração & dosagem , Etoposídeo/administração & dosagem , Evolução Fatal , Humanos , Transtornos do Desenvolvimento da Linguagem/induzido quimicamente , Neoplasias Pulmonares/secundário , Neoplasias Pulmonares/cirurgia , Mercaptopurina/administração & dosagem , Metotrexato/administração & dosagem , Mitoxantrona/administração & dosagem , Mitoxantrona/efeitos adversos , Recidiva Local de Neoplasia/cirurgia , Neoplasias Primárias Múltiplas/tratamento farmacológico , Neoplasias Primárias Múltiplas/cirurgia , Nefrectomia , Pneumonectomia/métodos , Leucemia-Linfoma Linfoblástico de Células Precursoras B/tratamento farmacológico
14.
J Cancer Educ ; 32(4): 728-733, 2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26847465

RESUMO

Clinical research is an integrated part of medical education. There is a noticeable decrease in the number of physician-scientists in developing countries, which is reflected by a decrease in research output and publications from these countries. We conducted a survey aiming to identify the gaps in clinical research training from the perspective of medical students. The results can be used to customize future clinical research trainings. The survey tool was divided into six modules which represent the cornerstones of clinical research based on similar surveys done for the same purpose. For each module, questions covered the perceived knowledge of its aspects and how much relevant the responder thought it was to clinical research. Five hundred one candidates have filled the survey. Evidence-based medicine (EBM) had the highest knowledge score of 2.20/4, while "clinical trials execution" knowledge got the lowest score of 1.64/4. Responders perceived EBM as the most relevant aspect of clinical research (3.39/4), while research ethics received the lowest score 3.18/4. "Clinical trials execution" had the largest gap of a difference calculated as 1.60, while EBM had the lowest gap of 1.20. More attention must be paid to clinical research training for medical students in developing countries. These trainings have to be customized to focus on clinical trial execution, research methodology, and biostatistics. In parallel, awareness campaigns targeted toward the medical community emphasizing the importance of the ethics as an aspect of clinical research should be established.


Assuntos
Protocolos Clínicos , Medicina Baseada em Evidências/educação , Estudantes de Medicina/psicologia , Países em Desenvolvimento , Educação de Graduação em Medicina/métodos , Egito , Feminino , Humanos , Masculino , Inquéritos e Questionários
15.
Int Ophthalmol ; 37(1): 111-118, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27113056

RESUMO

Research on childhood diseases represents a great global challenge. This challenge is maximized in both childhood cancer disciplines and developing world. In this paper, we aim at describing our institution experience in starting a structured childhood cancer research program in one of the developing countries in a short time based on philanthropic efforts. We used retinoblastoma as an example for what was conducted in this program. Starting in 2008, this program included improving clinical practice and its related supporting services besides developing new research services that both complement the clinical activities and pave the way towards creating a research foundation in the country. Results included developing hospital standard treatment protocols, developing national clinical trials, joining international consortia for childhood cancers clinical trials, developing data collection tools and real-time analytics, establishing a biobanking facility, and developing highly qualified team for conducting clinical, epidemiologic, and translational research studies. Moreover, this effort resulted in improving both clinical practice and patients' awareness nationally. This model can be used for other startup facilities that aim at finding answers for their national health problems in low-resource setting.


Assuntos
Pesquisa Biomédica/organização & administração , Retinoblastoma/terapia , Bancos de Espécimes Biológicos , Criança , Serviços de Saúde da Criança/organização & administração , Prestação Integrada de Cuidados de Saúde/organização & administração , Países em Desenvolvimento , Egito , Humanos , Oncologia/organização & administração , Pesquisa Translacional Biomédica/organização & administração
16.
J Pediatr Hematol Oncol ; 37(6): 433-7, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25929613

RESUMO

In the span of the last 48 years, only 33 cases of children with orbital Ewing sarcoma have been reported. This study is to present 3 cases that were admitted to Children's Cancer Hospital Egypt 57357, during the period from 2009 to 2013. We have 2 cases treated using the hospital standard Ewing sarcoma treatment protocol, to completion, whereas the third discontinued treatment. All tumors have confirmed CD99 positivity, although translocation (11;22) was positive in 1 patient and negative in the third. With earlier diagnosis and adequate surgical resection and integration of chemotherapy and radiotherapy 1 patient survived for about 4 years, whereas the other 2 cases died due to disease progression or recurrence.


Assuntos
Neoplasias Renais/complicações , Neoplasias Orbitárias/etiologia , Neoplasias Orbitárias/terapia , Sarcoma de Ewing/complicações , Criança , Pré-Escolar , Terapia Combinada , Humanos , Lactente , Neoplasias Renais/patologia , Masculino , Estadiamento de Neoplasias , Neoplasias Orbitárias/secundário , Prognóstico , Sarcoma de Ewing/patologia
17.
Fetal Pediatr Pathol ; 34(3): 176-84, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25839785

RESUMO

PURPOSE: Phthisis bulbi represents an ocular end-stage disease characterized by shrinkage and disorganization of the eye. We aim at identifying the pathologic changes of phthisis bulbi associated with retinoblastoma. DESIGN: Retrospective observational case series study. METHODS: 16 enucleated eyes were enrolled retrospectively between 2007 and 2012. Pathologic gross and microscopic findings were assessed. RESULTS: Cases showed grossly shrunken eyes with a mean volume of 4.3 cc. Sclera was markedly thickened in the majority of cases with mean of 2272.8 µ. Choroid showed an average thickness of 1029 µ. Necrosis, dystrophic calcification, ossification, gliosis, residual viable tumor was identified in many cases. Pathologic high risk factors were seen in three specimens. CONCLUSIONS: We conclude that retinoblastoma must be considered in the differential diagnosis of phthisis bulbi in the pediatric patients and active tumor was present in half of the patients.


Assuntos
Neoplasias da Retina/patologia , Retinoblastoma/patologia , Calcinose/patologia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco
18.
Pediatr Blood Cancer ; 61(12): 2185-90, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25175045

RESUMO

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is a rare and aggressive tumor accounting for 5% of pediatric renal tumors with an incidence of 20 patients per year in the USA. It is bone metastasizing with poor prognosis. Our aim was to show characteristics of patients in relation to improved outcome in one of the developing countries. PROCEDURE: We included all patients diagnosed as CCSK in the period between July 2007 and March 2012 at Children's Cancer Hospital, Egypt. Patients' demographics, clinical presentation, pathology, and management were reviewed. Follow up was continued until April 2013. RESULTS: Twenty-five patients were identified in the defined time interval, accounting for 7% all renal tumors diagnosed at the hospital. Mean age was 36 months. Abdominal swelling and hematuria were the most common presentations. Stages I, II, III, IV, and V represented 9 (36%), 3 (12%), 8 (32%), 3 (12%), and 2 (8%), respectively. Twenty-four patients had radical nephrectomy either upfront or after neo-adjuvant chemotherapy. Surgery was followed by adjuvant chemotherapy. Abdominal radiotherapy was given for local stages II and III. Twenty-two patients reached complete remission, while one patient had stationary disease and two patients died due to progression and relapse. Overall survival was 88.5% and event-free survival was 87.8% at 45 months. CONCLUSION: Although previous studies indicate poor prognosis of CCSK, our experience shows that those patients can be treated using extensive chemotherapy combined with proper local control.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/terapia , Neoplasias Renais/terapia , Neoplasias Hepáticas/terapia , Neoplasias Pulmonares/terapia , Sarcoma de Células Claras/terapia , Adolescente , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/secundário , Quimioterapia Adjuvante , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Neoplasias Renais/mortalidade , Neoplasias Renais/patologia , Neoplasias Hepáticas/mortalidade , Neoplasias Hepáticas/secundário , Neoplasias Pulmonares/mortalidade , Neoplasias Pulmonares/secundário , Masculino , Terapia Neoadjuvante , Estadiamento de Neoplasias , Nefrectomia , Prognóstico , Radioterapia Adjuvante , Sarcoma de Células Claras/mortalidade , Sarcoma de Células Claras/secundário , Taxa de Sobrevida
19.
J Cancer Educ ; 29(3): 536-40, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24395632

RESUMO

The use of Web 2.0 tools in education and health care has received heavy attention over the past years. Over two consecutive years, Children's Cancer Hospital - Egypt 57357 (CCHE 57357), in collaboration with Egyptian universities, student bodies, and NGOs, conducted a summer course that supports undergraduate medical students to cross the gap between clinical practice and clinical research. This time, there was a greater emphasis on reaching out to the students using social media and other Web 2.0 tools, which were heavily used in the course, including Google Drive, Facebook, Twitter, YouTube, Mendeley, Google Hangout, Live Streaming, Research Electronic Data Capture (REDCap), and Dropbox. We wanted to investigate the usefulness of integrating Web 2.0 technologies into formal educational courses and modules. The evaluation survey was filled in by 156 respondents, 134 of whom were course candidates (response rate = 94.4 %) and 22 of whom were course coordinators (response rate = 81.5 %). The course participants came from 14 different universities throughout Egypt. Students' feedback was positive and supported the integration of Web 2.0 tools in academic courses and modules. Google Drive, Facebook, and Dropbox were found to be most useful.


Assuntos
Pesquisa Biomédica/educação , Instrução por Computador/métodos , Educação de Graduação em Medicina/métodos , Mídias Sociais , Estudantes de Medicina/estatística & dados numéricos , Centros Médicos Acadêmicos , Países em Desenvolvimento , Egito , Humanos , Indústrias
20.
Ophthalmic Epidemiol ; : 1-10, 2024 Jul 05.
Artigo em Inglês | MEDLINE | ID: mdl-38968083

RESUMO

PURPOSE: This study aims to evaluate the epidemiological patterns, treatment strategies, and survival outcomes of conjunctival malignancies in Germany between 2009 and 2019. METHODS: A total of 1,532 cases were analyzed, with the crude incidence rate calculated. The survival rates were investigated using life tables and Cox regression analysis. RESULTS: The overall incidence rate was 1.7 per million. Incidence rates varied across age groups, peaking in the 75+ age group. Carcinomas (43%), melanomas (30%), and lymphomas (20%), were the most prevalent malignancies. Of the total cases with reported treatment, surgical intervention was undertaken in 64.5% of the patients. The 5-year overall survival rates were 90.4% for lymphomas, 73.8% for melanomas, and 72.9% for carcinomas. Age at diagnosis emerged as a significant prognostic factor in the Cox regression analysis. CONCLUSIONS: This study provides the first population-based incidence data on conjunctival malignancies in Germany, noting a generally low incidence with survival rates comparable to other regions. The findings underscore the importance of consistent reporting and further research into risk factors for a deeper understanding of these malignancies. The study calls for improved reporting systems and further investigations into genetic factors and targeted prevention strategies for high-risk groups.

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