Uveal melanoma(UM)is the most common intraocular cancer,with approximately 5.2 individuals per million affected annually in the United States.It represents approximately 3%of the global malignant melanoma cases,accoun...Uveal melanoma(UM)is the most common intraocular cancer,with approximately 5.2 individuals per million affected annually in the United States.It represents approximately 3%of the global malignant melanoma cases,accounting for 80%of the overall noncutaneous melanomas.Clinically,it remains silent in about 30%of the cases;when symptomatic,it generally causes metamorphopsia(painless loss or distortion of vision)and/or photopsia(flashing or flickering of light in the visual field).Discoloration of the iris,astigmatism,glaucoma,and even blindness are other,less common clinical manifestations.Several pathophysiological mechanisms underlie the development of UM.Genetic mutations,involving especially the G protein subunit alpha q(GNAQ),guanine nucleotide-binding protein subunit alpha-11(GNA11),BRCA1 associated deubiquitinase 1(BAP1),splicing factor 3b subunit 1(SF3B1),and eukaryotic translation initiation factor 1A,X-linked(EIF1AX)genes as well as the MAPK/ERK signaling pathway genes,have been largely associated with the development of UM.Chromosomal aberrations,inflammatory and immunological alterations are often concurrent factors for the development and progression of UM.Therapies targeting specific genetic alterations and immunotherapy agents have been recently developed and introduced in clinical practice for the management of advanced-stage UMs.This review aims to present the latest advances in the clinical molecular pathology of UM,along with the resulting targeted,immunological,and other therapies that have been introduced or are currently under investigation.展开更多
Crohn's disease (CD) is a chronic inflammatory disease of the digestive tract with systemic manifestations. Etiology is unknown, even if immunological, genetic and environmental factors are involved. The majority ...Crohn's disease (CD) is a chronic inflammatory disease of the digestive tract with systemic manifestations. Etiology is unknown, even if immunological, genetic and environmental factors are involved. The majority of CD patients require surgery during their lifetime due to progressive bowel damage, but, even when all macroscopic lesions have been removed by surgery, the disease recurs in most cases. Postoperative management represents therefore a crucial mean for preventing recurrence. Several drugs and approaches have been proposed to achieve this aim. Endoscopic inspection of the ileocolic anastomosis within 1 year from surgery is widely encouraged, given that endoscopic recurrence is one of the greatest predictors for clinical recurrence. A strategy should be planned only after stratifying patients according to their individual risk of recurrence, avoiding unnecessary therapies when possible benefits are reduced, and selecting high-risk patients for more aggressive intervention.展开更多
Pediatric glaucoma is a potentially sight-threatening disease and is considered the second leading cause of treatable childhood blindness.Pediatric glaucoma is a clinical entity including a wide range of conditions:pr...Pediatric glaucoma is a potentially sight-threatening disease and is considered the second leading cause of treatable childhood blindness.Pediatric glaucoma is a clinical entity including a wide range of conditions:primary congenital glaucoma,glaucoma secondary to ocular(e.g.,aniridia,Peter’s anomaly),or systemic disease(e.g.,Sturge Weber)and glaucoma secondary to acquired condition(pseudophakic,traumatic,uveitic glaucoma).The treatment algorithm of childhood glaucoma is a step-by-step approach,often starting with surgery,as in primary congenital glaucoma cases.Medical therapy is also crucial in the management of pediatric glaucoma.Here we reported the results of the randomized,controlled,clinical trials carried out in children treated with topical anti-glaucoma drugs.It is worth knowing that prostaglandin analogues showed an excellent systemic safety profile,while serious systemic events have been reported in children taking topical beta-blockers.Angle surgery is the first surgical option in patients diagnosed with primary congenital glaucoma,with ab interno and ab externo approaches showing similar outcomes.Trabeculectomy in children can be troublesome,as mitomycin C(MMC)can lead to bleb complications and a higher endophthalmitis rate than in adults.Glaucoma drainage devices(GDD)are no longer a last resort and can be considered a suitable option for the management of uncontrolled pediatric glaucoma after angle surgery failure.展开更多
文摘Uveal melanoma(UM)is the most common intraocular cancer,with approximately 5.2 individuals per million affected annually in the United States.It represents approximately 3%of the global malignant melanoma cases,accounting for 80%of the overall noncutaneous melanomas.Clinically,it remains silent in about 30%of the cases;when symptomatic,it generally causes metamorphopsia(painless loss or distortion of vision)and/or photopsia(flashing or flickering of light in the visual field).Discoloration of the iris,astigmatism,glaucoma,and even blindness are other,less common clinical manifestations.Several pathophysiological mechanisms underlie the development of UM.Genetic mutations,involving especially the G protein subunit alpha q(GNAQ),guanine nucleotide-binding protein subunit alpha-11(GNA11),BRCA1 associated deubiquitinase 1(BAP1),splicing factor 3b subunit 1(SF3B1),and eukaryotic translation initiation factor 1A,X-linked(EIF1AX)genes as well as the MAPK/ERK signaling pathway genes,have been largely associated with the development of UM.Chromosomal aberrations,inflammatory and immunological alterations are often concurrent factors for the development and progression of UM.Therapies targeting specific genetic alterations and immunotherapy agents have been recently developed and introduced in clinical practice for the management of advanced-stage UMs.This review aims to present the latest advances in the clinical molecular pathology of UM,along with the resulting targeted,immunological,and other therapies that have been introduced or are currently under investigation.
文摘Crohn's disease (CD) is a chronic inflammatory disease of the digestive tract with systemic manifestations. Etiology is unknown, even if immunological, genetic and environmental factors are involved. The majority of CD patients require surgery during their lifetime due to progressive bowel damage, but, even when all macroscopic lesions have been removed by surgery, the disease recurs in most cases. Postoperative management represents therefore a crucial mean for preventing recurrence. Several drugs and approaches have been proposed to achieve this aim. Endoscopic inspection of the ileocolic anastomosis within 1 year from surgery is widely encouraged, given that endoscopic recurrence is one of the greatest predictors for clinical recurrence. A strategy should be planned only after stratifying patients according to their individual risk of recurrence, avoiding unnecessary therapies when possible benefits are reduced, and selecting high-risk patients for more aggressive intervention.
文摘Pediatric glaucoma is a potentially sight-threatening disease and is considered the second leading cause of treatable childhood blindness.Pediatric glaucoma is a clinical entity including a wide range of conditions:primary congenital glaucoma,glaucoma secondary to ocular(e.g.,aniridia,Peter’s anomaly),or systemic disease(e.g.,Sturge Weber)and glaucoma secondary to acquired condition(pseudophakic,traumatic,uveitic glaucoma).The treatment algorithm of childhood glaucoma is a step-by-step approach,often starting with surgery,as in primary congenital glaucoma cases.Medical therapy is also crucial in the management of pediatric glaucoma.Here we reported the results of the randomized,controlled,clinical trials carried out in children treated with topical anti-glaucoma drugs.It is worth knowing that prostaglandin analogues showed an excellent systemic safety profile,while serious systemic events have been reported in children taking topical beta-blockers.Angle surgery is the first surgical option in patients diagnosed with primary congenital glaucoma,with ab interno and ab externo approaches showing similar outcomes.Trabeculectomy in children can be troublesome,as mitomycin C(MMC)can lead to bleb complications and a higher endophthalmitis rate than in adults.Glaucoma drainage devices(GDD)are no longer a last resort and can be considered a suitable option for the management of uncontrolled pediatric glaucoma after angle surgery failure.
