Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 23
Filter
1.
RMD Open ; 7(3)2021 09.
Article in English | MEDLINE | ID: mdl-34531304

ABSTRACT

OBJECTIVE: To assess the efficacy and safety of belimumab in paediatric versus adult patients with systemic lupus erythematosus (SLE). METHODS: We performed across-study comparisons of patients with active SLE who received belimumab or placebo, plus standard therapy, in PLUTO (paediatric phase II) and BLISS-52, BLISS-76, BLISS-NEA and EMBRACE (adult phase III). Analysed efficacy data included Week 52 SLE Responder Index (SRI)-4 response rate (EMBRACE: SRI with modified Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) proteinuria scoring (SRI-S2K)); SRI-4 response rate (EMBRACE: SRI-S2K) according to baseline disease activity indicators (Safety of Estrogens in Lupus Erythematosus National Assessment-Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) score; anti-dsDNA/C3/C4 levels); Week 52 SRI-6 response rate; and time to first severe flare (SELENA-SLEDAI Flare Index) over 52 weeks. Safety data were compared for all aforementioned studies along with adult LBSL02 (phase II) and BLISS-SC (phase III). RESULTS: SRI-4 response rates were similar across the paediatric and adult studies; more belimumab-treated patients achieved SRI-4 responses versus placebo (PLUTO: 52.8% vs 43.6%; BLISS-52: 57.6% vs 43.6%; BLISS-76: 43.2% vs 33.8%; BLISS-NEA: 53.8% vs 40.1%; EMBRACE: 48.7% vs 41.6%). Across all studies, SRI-4 response rates were generally greater in patients with baseline SELENA-SLEDAI scores ≥10 than in patients with baseline SELENA-SLEDAI scores ≤9. A similar proportion of belimumab-treated patients achieved SRI-6 across all studies (PLUTO: 41.2%; BLISS-52: 46.2%; BLISS-76: 33.1%; BLISS-NEA: 43.9%; EMBRACE: 37.5%). Belimumab reduced the risk of severe flare versus placebo in all studies. The incidence of adverse events was similar across all studies. CONCLUSIONS: These analyses demonstrate consistent efficacy and safety of belimumab plus standard therapy across paediatric and adult patients with SLE. TRIAL REGISTRATION NUMBERS: PLUTO (NCT01649765); BLISS-52 (NCT00424476); BLISS-76 (NCT00410384); BLISS-NEA (NCT01345253); EMBRACE (NCT01632241); BLISS-SC (NCT01484496); and LBSL02 (NCT00071487).


Subject(s)
Antibodies, Monoclonal, Humanized , Lupus Erythematosus, Systemic , Adult , Animals , Antibodies, Monoclonal, Humanized/therapeutic use , Bees , Child , Humans , Lupus Erythematosus, Systemic/drug therapy , Severity of Illness Index , Treatment Outcome
2.
Retin Cases Brief Rep ; 15(1): 49-51, 2021 Jan 01.
Article in English | MEDLINE | ID: mdl-29578935

ABSTRACT

PURPOSE: To present a case of open globe injury and retained intraocular foreign body secondary to drywall. METHODS: Interventional case report. RESULTS: A 21-year-old man presented with corneal laceration, iris defect, and vitreous hemorrhage after hammering drywall. Computed tomography scan was negative for intraocular foreign body, but a drywall intraretinal foreign body was found on 25-gauge vitrectomy. Intraoperative findings and 6-month follow-up are presented. CONCLUSION: Intraocular foreign body must always be suspected in all cases of penetrating ocular trauma. Although magnetic resonance imaging is ideal in diagnosing nonmetallic foreign bodies, computed tomography scan with Hounsfield units should be used in an emergency setting.


Subject(s)
Eye Foreign Bodies/diagnosis , Eye Injuries, Penetrating/diagnosis , Magnetic Resonance Imaging/methods , Ophthalmologic Surgical Procedures/methods , Tomography, X-Ray Computed/methods , Visual Acuity , Eye Foreign Bodies/surgery , Eye Injuries, Penetrating/surgery , Humans , Male , Vitrectomy/methods , Young Adult
3.
Ophthalmic Plast Reconstr Surg ; 34(2): e65-e67, 2018.
Article in English | MEDLINE | ID: mdl-29346169

ABSTRACT

The authors report a case of isolated congenital orbital myofibroma causing sphenoid dysplasia and presenting as global restriction of extraocular motility and ptosis in a neonate. Sphenoid wing dysplasia is most commonly associated neurofibromatosis 1 but this patient had no evidence of neurofibromatosis on clinical examination and genetic testing. Congenital orbital myofibroma can have secondary effects on bone and likely the lesion was present early in development leading to aplasia of the sphenoid bone. To the best of the authors' knowledge, this is the first reported case of sphenoid wing aplasia secondary to congenital orbital myofibroma independent of neurofibromatosis 1.


Subject(s)
Myofibroma/pathology , Orbital Neoplasms/pathology , Sphenoid Bone/abnormalities , Facial Asymmetry/etiology , Humans , Infant, Newborn , Male
4.
Retina ; 38(7): 1432-1435, 2018 07.
Article in English | MEDLINE | ID: mdl-28613214

ABSTRACT

BACKGROUND: The study was inspired after the authors encountered a patient with a penetrating globe injury due to drywall, who had retained intraocular drywall foreign body. Computed tomography (CT) was read as normal in this patient. Open globe injury with drywall has never been reported previously in the literature and there are no previous studies describing its radiographic features. METHODS: The case report is described in detail elsewhere. This was an experimental study. An ex vivo model of 15 porcine eyes with 1 mm to 5 mm fragments of implanted drywall, 2 vitreous only samples with drywall and 3 control eyes were used. Eyes and vitreous samples were CT scanned on Days 0, 1, and 3 postimplantation. Computed ocular images were analyzed by masked observers. Size and radiodensity of intraocular drywall were measured using Hounsfield units (HUs) over time. RESULTS: Intraocular drywall was hyperdense on CT. All sizes studied were detectable on Day 0 of scanning. Mean intraocular drywall foreign body density was 171 ± 52 Hounsfield units (70-237) depending on fragment size. Intraocular drywall foreign body decreased in size whereas Hounsfield unit intensity increased over time. CONCLUSION: Drywall dissolves in the eye and becomes denser over time as air in the drywall is replaced by fluid. This study identified Hounsfield Units specific to intraocular drywall foreign body over time.


Subject(s)
Eye Foreign Bodies/diagnosis , Eye Injuries, Penetrating/diagnosis , Tomography, X-Ray Computed/methods , Vitreous Body/injuries , Animals , Disease Models, Animal , Eye Foreign Bodies/surgery , Eye Injuries, Penetrating/surgery , Swine , Vitrectomy , Vitreous Body/pathology , Vitreous Body/surgery
6.
Mo Med ; 113(2): 131-5, 2016.
Article in English | MEDLINE | ID: mdl-27311224

ABSTRACT

Children often present to health care providers for evaluation of limp. Having the knowledge of the different causes of leg pains both in the acute and chronic settings will help in diagnosis, treatment, and referrals to subspecialists in a timely manner. Taking a detailed history and completing a thorough evaluation will help hone in on the underlying cause. This article will review important causes of limp from the rheumatologist's viewpoint.


Subject(s)
Arthritis, Juvenile/diagnosis , Gait , Rheumatology , Arthritis, Juvenile/complications , Arthritis, Juvenile/therapy , Child , Humans , Joint Instability , Legg-Calve-Perthes Disease/diagnosis , Osteomyelitis/diagnosis , Slipped Capital Femoral Epiphyses/diagnosis , Synovitis/diagnosis
8.
Ophthalmic Genet ; 37(1): 44-52, 2016.
Article in English | MEDLINE | ID: mdl-25007332

ABSTRACT

BACKGROUND: Characterization of retinal degeneration (RD) using high-resolution retinal imaging and exome sequencing may identify phenotypic features that correspond with specific genetic defects. MATERIALS AND METHODS: Six members from a non-consanguineous Indian family (three affected siblings, their asymptomatic parents and an asymptomatic child) were characterized clinically, using visual acuity, perimetry, full-field electroretinography (ERG), optical coherence tomography and cone structure as outcome measures. Cone photoreceptors were imaged in the proband using adaptive optics scanning laser ophthalmoscopy. The exome was captured using Nimblegen SeqCap EZ V3.0 probes and sequenced using lllumina HiSeq. Reads were mapped to reference hg19. Confirmation of variants and segregation analysis was performed using dideoxy sequencing. RESULTS: Analysis of exome variants using exomeSuite identified five homozygous variants in four genes known to be associated with RD. Further analysis revealed a homozygous nonsense mutation, c.1105 C > T, p.Arg335Ter, in the FAM161A gene segregating with RD. Three additional variants were found to occur at high frequency. Affected members showed a range of disease severity beginning at different ages, but all developed severe visual field and outer retinal loss. CONCLUSIONS: Exome analysis revealed a nonsense homozygous mutation in FAM161A segregating with RD with severe vision loss and a range of disease onset and progression. Loss of outer retinal structures demonstrated with high-resolution retinal imaging suggests FAM161A is important for normal photoreceptor structure and survival. Exome sequencing may identify causative genetic variants in autosomal recessive RD families when other genetic test strategies fail to identify a mutation.


Subject(s)
Codon, Nonsense , Eye Proteins/genetics , Retinal Degeneration/genetics , Adult , Aged , Blindness/genetics , DNA Mutational Analysis , Electroretinography , Exome/genetics , Female , Humans , Male , Middle Aged , Pedigree , Phenotype , Retinal Degeneration/diagnosis , Tomography, Optical Coherence , Visual Acuity/physiology , Visual Field Tests , Visual Fields/physiology
9.
Invest Ophthalmol Vis Sci ; 56(10): 6179-89, 2015 Sep 01.
Article in English | MEDLINE | ID: mdl-26416092

ABSTRACT

PURPOSE: To determine short-term variability of adaptive optics scanning laser ophthalmoscopy (AOSLO)-derived cone spacing measures in eyes with inherited retinal degenerations (IRD) and in normal eyes. METHODS: Twenty IRD patients and 10 visually normal subjects underwent AOSLO imaging at two visits separated by no more than 1 month (NCT00254605). Cone spacing was measured in multiple macular regions in each image by three independent graders. Variability of cone spacing measures between visits, between graders, and between eyes was determined and correlated with standard clinical measures. RESULTS: Cone spacing was measured in 2905 regions. Interobserver agreement was high both in normal eyes and eyes with IRD (mean intraclass correlation coefficient [ICC] = 0.838 for normal and 0.892 for eyes with IRD). Cone spacing measures were closely correlated between visits (ICC > 0.869 for both study groups). Mean relative intervisit spacing difference (absolute difference in measures divided by the mean at each region) was 4.0% for normal eyes and 4.9% for eyes with IRD. Cone spacing measures from fellow eyes of the same subject showed strong agreement for all subjects (ICC > 0.85 for both study groups). CONCLUSIONS: Adaptive optics scanning laser ophthalmoscopy-derived macular cone spacing measures were correlated between observers, visits, and fellow eyes of the same subject in normal eyes and in eyes with IRD. This information may help establish the role of cone spacing measures derived from images of the cone mosaic obtained with AOSLO as a sensitive biomarker for longitudinal tracking of photoreceptor loss during disease progression and in response to treatment. (ClinicalTrials.gov number, NCT00254605.).


Subject(s)
Retinal Cone Photoreceptor Cells/pathology , Retinal Degeneration/pathology , Adult , Aged , Case-Control Studies , Female , Humans , Male , Middle Aged , Observer Variation , Ophthalmoscopy/methods , Optics and Photonics/methods , Prospective Studies , Young Adult
10.
Arthritis Care Res (Hoboken) ; 67(4): 529-37, 2015 Apr.
Article in English | MEDLINE | ID: mdl-25331530

ABSTRACT

OBJECTIVE: Few data are available regarding the rates of serious adverse events (SAEs) and important medical events (IMEs) outside of product-based registries and clinical trials for juvenile idiopathic arthritis (JIA). The Enhanced Drug Safety Surveillance Project (EDSSP) was developed to pilot a novel system to collect SAEs/IMEs in children with JIA. This analysis reports the results from this 4-year (2008-2012) EDSSP. METHODS: Participating physicians were surveyed monthly to ascertain whether their JIA patients experienced an SAE or IME. Sites were surveyed every 6 months to determine the number of unique JIA patients seen at each site during that 6-month period. Reporting rates were calculated per 100 person-years and 95% confidence intervals (95% CIs) were calculated based on a Poisson distribution. RESULTS: Thirty-seven Childhood Arthritis and Rheumatology Research Alliance sites with 115 physicians participated. The mean response rate to the monthly surveys was 65%. There were 147 total SAEs and 145 total IMEs. The largest proportion of SAEs and IMEs occurred in children with polyarticular JIA (39% and 37%, respectively). The majority of SAEs and IMEs were reported for patients receiving therapy with biologic agents (76% and 69%, respectively). The total event rate for SAEs and IMEs combined was 1.07 events per 100 person-years (95% CI 0.95-1.19). The rates for SAEs and IMEs were 0.54 per 100 person-years (95% CI 0.45-0.63) and 0.53 per 100 person-years (95% CI 0.49-0.62), respectively. CONCLUSION: The EDSSP provided a simple tool for SAE/IME reporting within an established research network and resulted in a similar range of reported events as captured by a traditional product-based registry.


Subject(s)
Adverse Drug Reaction Reporting Systems , Antirheumatic Agents/adverse effects , Arthritis, Juvenile/drug therapy , Physicians , Population Surveillance/methods , Rheumatology/methods , Adolescent , Adverse Drug Reaction Reporting Systems/trends , Arthritis, Juvenile/diagnosis , Arthritis, Juvenile/epidemiology , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Longitudinal Studies , Male , Physicians/trends , Research Design/trends , Rheumatology/trends
11.
Invest Ophthalmol Vis Sci ; 56(1): 372-81, 2014 Dec 16.
Article in English | MEDLINE | ID: mdl-25515570

ABSTRACT

PURPOSE: We studied the correlation between outer nuclear layer (ONL) thickness and cone density in normal eyes and eyes with retinitis pigmentosa (RP). METHODS: Spectral-domain optical coherence tomography (SD-OCT) scans were acquired using a displaced pupil entry position of the scanning beam to distinguish Henle's fiber layer from the ONL in 20 normal eyes (10 subjects) and 12 eyes with RP (7 patients). Cone photoreceptors were imaged using adaptive optics scanning laser ophthalmoscopy. The ONL thickness and cone density were measured at 0.5° intervals along the horizontal meridian through the fovea nasally and temporally. The ONL thickness and cone density were correlated using Spearman's rank correlation coefficient r. RESULTS: Cone densities averaged over the central 6° were lower in eyes with RP than normal, but showed high variability in both groups. The ONL thickness and cone density were significantly correlated when all retinal eccentricities were combined (r = 0.74); the correlation for regions within 0.5° to 1.5° eccentricity was stronger (r = 0.67) than between 1.5° and 3.0° eccentricity (r = 0.23). Although cone densities were lower between 0.5° and 1.5° in eyes with RP, ONL thickness measures at identical retinal locations were similar in the two groups (P = 0.31), and interindividual variation was high for ONL and cone density measures. Although ONL thickness and retinal eccentricity were important predictors of cone density, eccentricity was over 3 times more important. CONCLUSIONS: The ONL thickness and cone density were correlated in normal eyes and eyes with RP, but both were strongly correlated with retinal eccentricity, precluding estimation of cone density from ONL thickness. (ClinicalTrials.gov number, NCT00254605.).


Subject(s)
Retinal Cone Photoreceptor Cells/cytology , Retinal Photoreceptor Cell Outer Segment/pathology , Retinitis Pigmentosa/pathology , Visual Acuity , Adult , Cell Count , Electroretinography , Female , Follow-Up Studies , Healthy Volunteers , Humans , Male , Middle Aged , Ophthalmoscopy , Prospective Studies , Retinitis Pigmentosa/physiopathology , Tomography, Optical Coherence , Young Adult
12.
Br J Ophthalmol ; 98(3): 298-301, 2014 Mar.
Article in English | MEDLINE | ID: mdl-24344233

ABSTRACT

AIM: To evaluate if broadening the criteria for retinopathy of prematurity (ROP) screening to include babies with gestational age ≥32 weeks and/or birth weight ≥1500 g, would have an impact on the number of babies diagnosed as having ROP. METHODS: A prospective cohort study was carried out at the Aga Khan University Hospital, Karachi, Pakistan. Infants with gestational age ≤35 weeks or birth weight ≤2000 g born in this hospital from May 2010 to December 2012 were screened for the presence of ROP 4-6 weeks after birth. Subsequent examinations were performed at intervals based on the findings of initial eye examinations. Infants diagnosed as having ROP were treated with argon laser therapy. Neonatal risk factors were also assessed. Cumulative incidence of ROP was calculated for babies falling within and outside current screening criteria. Multivariate logistic regression analysis was performed to examine the predictors of ROP. RESULTS: A total of 301 infants were screened: 27 (9%) babies developed ROP, of which 19 had stage 3 ROP or worse. None of the babies falling outside the current screening criteria developed ROP. The incidence of ROP in the infants meeting the current screening criteria was 11.5%. Multivariate logistic regression analysis showed that only gestational age (adjusted relative risk 0.774, 95% CI 0.603 to 0.994) was independently associated with the development of ROP. CONCLUSIONS: In our population ROP was not seen to occur in infants older than 32 weeks gestational age and/or weighing more than 1500 g.


Subject(s)
Neonatal Screening/standards , Retinopathy of Prematurity/diagnosis , Retinopathy of Prematurity/epidemiology , Vision Screening/standards , Birth Weight , Cohort Studies , Female , Gestational Age , Humans , Incidence , Infant, Low Birth Weight , Infant, Newborn , Infant, Premature , Laser Coagulation , Male , Pakistan/epidemiology , Prospective Studies , Retinopathy of Prematurity/surgery , Risk Factors
13.
Invest Ophthalmol Vis Sci ; 54(12): 7498-509, 2013 Nov 15.
Article in English | MEDLINE | ID: mdl-24135755

ABSTRACT

PURPOSE: To evaluate cone spacing using adaptive optics scanning laser ophthalmoscopy (AOSLO) in eyes with nonneovascular AMD, and to correlate progression of AOSLO-derived cone measures with standard measures of macular structure. METHODS: Adaptive optics scanning laser ophthalmoscopy images were obtained over 12 to 21 months from seven patients with AMD including four eyes with geographic atrophy (GA) and four eyes with drusen. Adaptive optics scanning laser ophthalmoscopy images were overlaid with color, infrared, and autofluorescence fundus photographs and spectral domain optical coherence tomography (SD-OCT) images to allow direct correlation of cone parameters with macular structure. Cone spacing was measured for each visit in selected regions including areas over drusen (n = 29), at GA margins (n = 14), and regions without drusen or GA (n = 13) and compared with normal, age-similar values. RESULTS: Adaptive optics scanning laser ophthalmoscopy imaging revealed continuous cone mosaics up to the GA edge and overlying drusen, although reduced cone reflectivity often resulted in hyporeflective AOSLO signals at these locations. Baseline cone spacing measures were normal in 13/13 unaffected regions, 26/28 drusen regions, and 12/14 GA margin regions. Although standard clinical measures showed progression of GA in all study eyes, cone spacing remained within normal ranges in most drusen regions and all GA margin regions. CONCLUSIONS: Adaptive optics scanning laser ophthalmoscopy provides adequate resolution for quantitative measurement of cone spacing at the margin of GA and over drusen in eyes with AMD. Although cone spacing was often normal at baseline and remained normal over time, these regions showed focal areas of decreased cone reflectivity. These findings may provide insight into the pathophysiology of AMD progression. (ClinicalTrials.gov number, NCT00254605).


Subject(s)
Geographic Atrophy/diagnosis , Retinal Cone Photoreceptor Cells/pathology , Retinal Drusen/diagnosis , Tomography, Optical Coherence/methods , Aged , Disease Progression , Female , Fluorescein Angiography , Follow-Up Studies , Humans , Male , Middle Aged , Ophthalmoscopy , Visual Acuity
14.
Invest Ophthalmol Vis Sci ; 54(2): 950-61, 2013 Feb 01.
Article in English | MEDLINE | ID: mdl-23299470

ABSTRACT

PURPOSE: To study retinal structure in choroideremia patients and carriers using high-resolution imaging techniques. METHODS: Subjects from four families (six female carriers and five affected males) with choroideremia (CHM) were characterized with best-corrected visual acuity (BCVA), kinetic and static perimetry, full-field electroretinography, and fundus autofluorescence (FAF). High-resolution macular images were obtained with adaptive optics scanning laser ophthalmoscopy (AOSLO) and spectral domain optical coherence tomography (SD-OCT). Coding regions of the CHM gene were sequenced. RESULTS: Molecular analysis of the CHM gene identified a deletion of exons 9 to 15 in family A, a splice site mutation at position 79+1 of exon 1 in family B, deletion of exons 6 to 8 in family C, and a substitution at position 106 causing a premature stop in family D. BCVA ranged from 20/16 to 20/63 in carriers and from 20/25 to 5/63 in affected males. FAF showed abnormalities in all subjects. SD-OCT showed outer retinal layer loss, outer retinal tubulations at the margin of outer retinal loss, and inner retinal microcysts. Patchy cone loss was present in two symptomatic carriers. In two affected males, cone mosaics were disrupted with increased cone spacing near the fovea but more normal cone spacing near the edge of atrophy. CONCLUSIONS: High-resolution retinal images in CHM carriers and affected males demonstrated RPE and photoreceptor cell degeneration. As both RPE and photoreceptor cells were affected, these cell types may degenerate simultaneously in CHM. These findings provide insight into the effect of CHM mutations on macular retinal structure, with implications for the development of treatments for CHM. (ClinicalTrials.gov number, NCT00254605.).


Subject(s)
Choroideremia/pathology , Fluorescein Angiography/methods , Image Processing, Computer-Assisted/methods , Ophthalmoscopy/methods , Tomography, Optical Coherence/methods , Adaptor Proteins, Signal Transducing/genetics , Adaptor Proteins, Signal Transducing/metabolism , Adolescent , Adult , Aged , Choroideremia/genetics , Choroideremia/metabolism , DNA/genetics , Female , Fundus Oculi , Genetic Predisposition to Disease , Humans , Male , Middle Aged , Mutation , Pedigree , Phenotype , Polymerase Chain Reaction , Protein Prenylation , Retinal Cone Photoreceptor Cells/pathology , Young Adult
15.
Arch Ophthalmol ; 130(10): 1301-8, 2012 Oct.
Article in English | MEDLINE | ID: mdl-23044944

ABSTRACT

OBJECTIVES: To describe the clinical phenotype and identify the molecular basis of disease in a consanguineous family of Palestinian origin with autosomal recessive retinal degeneration. METHODS: Eight family members were evaluated with visual acuity and perimetry tests, color fundus photographs, full-field electroretinography, and optical coherence tomography. Cone photoreceptors surrounding the fovea were imaged in 2 members, using adaptive optics scanning laser ophthalmoscopy. Exome was captured using probes and sequenced. Readings were mapped to reference hg19. Variant calls and annotations were performed, using published protocols. Confirmation of variants and segregation analysis was performed using dideoxy sequencing. RESULTS: Analysis detected 24 037 single-nucleotide variants in one affected family member, of which 3622 were rare and potentially damaging to encoded proteins. Further analysis revealed a novel homozygous nonsense change, c.1381 C>T, p.Gln461X in exon 13 of the CDHR1 gene, which segregated with retinal degeneration in this family. Affected members had night blindness beginning during adolescence with progressive visual acuity and field loss and unmeasurable electroretinographic responses, as well as macular outer retinal loss, although residual cones with increased cone spacing were observed in the youngest individual. CONCLUSIONS: Exome analysis revealed a novel CDHR1 nonsense mutation segregating with progressive retinal degeneration causing severe central vision loss by the fourth decade of life. High-resolution retinal imaging revealed outer retinal changes suggesting that CDHR1 is important for normal photoreceptor structure and survival. CLINICAL RELEVANCE: Exome sequencing is a powerful technique that may identify causative genetic variants in families with autosomal recessive retinal degeneration.


Subject(s)
Cadherins/genetics , Codon, Nonsense , Genes, Recessive/genetics , Nerve Tissue Proteins/genetics , Polymorphism, Single Nucleotide/genetics , Retinal Degeneration/genetics , Adult , Aged , Cadherin Related Proteins , Color Vision/physiology , Consanguinity , DNA Mutational Analysis , Electroretinography , Exome/genetics , Female , Humans , Male , Middle Aged , Ophthalmoscopy , Pedigree , Phenotype , Photoreceptor Cells, Vertebrate/pathology , Polymerase Chain Reaction , Refraction, Ocular/physiology , Retinal Degeneration/physiopathology , Tomography, Optical Coherence , Visual Acuity/physiology , Visual Fields/physiology , Young Adult
16.
Diagn Cytopathol ; 40(1): 7-13, 2012 Jan.
Article in English | MEDLINE | ID: mdl-22180232

ABSTRACT

Although percutaneous biopsies of the spine at thoracolumbar levels have proven value, the benefit in the sacrococcygeal region has not been presented thoroughly in the literature. This region has unique anatomic and oncologic properties. The cytopathologic files at The Johns Hopkins Hospital over a 20-year-period were searched. Imaging studies and medical records were also reviewed. Ninety-one cases of sacral/presacral FNA were identified. There were 46 males and 45 females; 62% had a prior history of malignancy. The most common presentation was lower back pain (44%). Imaging revealed lytic and/or soft tissue lesions masses from 1 to 12 cm (mean = 5.0 cm). Of the 71 (78%) diagnostic cases, 19 (27%) were nonneoplastic, 2 (3%) were suspicious for neoplasm, while 50 (70%) cases were neoplastic. Of the nonneoplastic cases, 10 (53%) showed nonspecific inflammation. Two (4%) of the 50 neoplastic lesions were benign tumors (schwannoma and neurofibroma), and of the malignant cases, 11 (23%) were primary, and 37 (77%) were metastatic/secondary. The most common primary malignant tumor was chordoma (four cases, 36%). Of the 37 secondary tumors, the most common were colorectal carcinoma (8, 22%) and plasmacytic tumors (5/9, 56%). The overall accuracy, sensitivity and specificity of FNA were 96, 95, and 100%, respectively. Sacral and presacral lesions represent rare targets for FNA. Benign tumors are rare (2%). Most cancers are metastatic (41%), with colorectum being the most common primary site (9%). Primary malignancies are uncommon (14%), with chordomas being the most frequent of these entities (4%).


Subject(s)
Inflammation/diagnosis , Sacrococcygeal Region/pathology , Soft Tissue Neoplasms/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Biopsy, Fine-Needle , Child , Child, Preschool , Female , Humans , Infant , Inflammation/surgery , Male , Middle Aged , Retrospective Studies , Soft Tissue Neoplasms/secondary , Soft Tissue Neoplasms/surgery , Young Adult
17.
Arthritis Care Res (Hoboken) ; 64(3): 375-83, 2012 Mar.
Article in English | MEDLINE | ID: mdl-22162255

ABSTRACT

OBJECTIVE: To formulate consensus treatment plans (CTPs) for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (SLE). METHODS: A structured consensus formation process was employed by the members of the Childhood Arthritis and Rheumatology Research Alliance after considering the existing medical evidence and current treatment approaches. RESULTS: After an initial Delphi survey (response rate = 70%), a 2-day consensus conference, and 2 followup Delphi surveys (response rates = 63-79%), consensus was achieved for a limited set of CTPs addressing the induction therapy of proliferative LN. These CTPs were developed for prototypical patients defined by eligibility characteristics, and included immunosuppressive therapy with either mycophenolic acid orally twice per day, or intravenous cyclophosphamide once per month at standardized dosages for 6 months. Additionally, the CTPs describe 3 options for standardized use of glucocorticoids, including a primarily oral, a mixed oral/intravenous, and a primarily intravenous regimen. There was consensus on measures of effectiveness and safety of the CTPs. The CTPs were well accepted by the pediatric rheumatology providers treating children with LN, and up to 300 children per year in North America are expected to be candidates for the treatment with the CTPs. CONCLUSION: CTPs for induction therapy of proliferative LN in juvenile SLE based on the available scientific evidence and pediatric rheumatology group experience have been developed. Consistent use of the CTPs may improve the prognosis of proliferative LN, and support the conduct of comparative effectiveness studies aimed at optimizing therapeutic strategies for proliferative LN in juvenile SLE.


Subject(s)
Immunosuppressive Agents/therapeutic use , Lupus Erythematosus, Systemic/drug therapy , Lupus Nephritis/drug therapy , Remission Induction/methods , Child , Humans , Lupus Nephritis/diagnosis , Male
18.
BMC Psychiatry ; 8: 56, 2008 Jul 17.
Article in English | MEDLINE | ID: mdl-18637176

ABSTRACT

BACKGROUND: There is a cultural variability around the perception of what causes the syndrome of schizophrenia. Generally patients with schizophrenia are considered dangerous. They are isolated and treatment is delayed. Studies have shown favorable prognosis with good family and social support, early diagnosis and management. Duration of untreated psychosis is a bad prognostic indicator. We aimed to determine the perceptions regarding the etiology of schizophrenia and the subsequent help seeking behavior. METHODS: This cross-sectional study was carried out on a sample of 404 people at the out patient departments of Aga Khan University Hospital Karachi. Data was collected via a self-administered questionnaire. Questions were related to a vignette of a young man displaying schizophrenic behavior. Data was analyzed on SPSS v 14. RESULTS: The mean age of the participants was 31.4 years (range = 18-72) and 77% of them were males. The majorities were graduates (61.9%) and employed (50%). Only 30% of the participants attributed 'mental illness' as the main cause of psychotic symptoms while a large number thought of 'God's will' (32.3%), 'superstitious ideas' (33.1%), 'loneliness' (24.8%) and 'unemployment' (19.3%) as the main cause. Mental illness as the single most important cause was reported by only 22%. As far as management is concerned, only 40% reported psychiatric consultation to be the single most important management step. Other responses included spiritual healing (19.5%) and Sociachanges (10.6) while 14.8% of respondents said that they would do nothing. Gender, age, family system and education level were significantly associated with the beliefs about the cause of schizophrenia (p < 0.05). While these variables plus 'religious inclination' and 'beliefs about cause' were significantly associated with the help seeking behavior of the participants. CONCLUSION: Despite majority of the study population being well educated, only a few recognized schizophrenia as a mental illness and many held superstitious beliefs. A vast majority of Pakistanis have non-biomedical beliefs about the cause of schizophrenia. Their help seeking behavior in this regard is inappropriate and detrimental to the health of schizophrenic patients. Areas for future research have been identified.


Subject(s)
Attitude to Health , Patient Acceptance of Health Care/statistics & numerical data , Schizophrenia/ethnology , Schizophrenia/etiology , Adolescent , Adult , Aged , Cross-Sectional Studies , Culture , Female , Humans , Male , Middle Aged , Pakistan/epidemiology , Population Surveillance/methods , Prognosis , Social Support , Surveys and Questionnaires
19.
J Pak Med Assoc ; 58(4): 186-90, 2008 Apr.
Article in English | MEDLINE | ID: mdl-18655427

ABSTRACT

INTRODUCTION: To determine the frequency of Retinopathy of Prematurity (ROP) in the premature infants born in a tertiary care hospital and to determine the risk factors associated with it. METHODS: This was a retrospective analysis of premature infants with birth weight < or = 1500 grams or gestational ago < or = 32 weeks, admitted to the Neonatal Intensive Care Unit of a tertiary care hospital in a three year period. The infants underwent eye examinations between their 4th and 7th week of life. Univariate and multiple logistic regression analysis were performed to determine the association between risk factors and ROP development. RESULTS: Sixty eight infants were included in the analysis. Out of these, 22 (32.4%) developed ROP (inclusive of all stages) and 14 (20.6%) developed threshold disease. Multiple logistic regression analysis revealed that low gestational age, sepsis and respiratory distress syndrome were independent predictors for the development of ROP. CONCLUSION: The frequency of ROP in our hospital was on the higher side of the range reported in neighbouring developing countries. Low gestational age, sepsis and respiratory distress syndrome were independent predictors of ROP development. Our results suggest that ROP is an important emerging cause of preventable childhood blindness in urban areas of Pakistan like Karachi.


Subject(s)
Retinopathy of Prematurity/epidemiology , Female , Humans , Incidence , Infant, Newborn , Infant, Premature , Intensive Care Units, Neonatal/statistics & numerical data , Logistic Models , Male , Pakistan/epidemiology , Retrospective Studies , Risk Factors
20.
J Pak Med Assoc ; 58(4): 214-7, 2008 Apr.
Article in English | MEDLINE | ID: mdl-18655436

ABSTRACT

OBJECTIVE: To determine the prevalence, attitude and knowledge of self-medication amongst university students of Karachi, Pakistan. METHODS: This cross-sectional, study was conducted from Jan-Feb 2007. A convenience sample was taken from 2 medical and 2 non-medical universities of the city of Karachi, Pakistan. Data was analyzed using SPSS v 14 and associations were tested using the Chi square test. RESULTS: Of the 572 participants (mean age=21 +/- 1.8 years, Male: Female ratio=1:1.5), 295 were medical and 277 were non-medical students. The prevalence of self-medication was 76%. Forty three percent students stated that they alter the regimen of prescribed medicines while 61.9% stated that they stop taking a prescribed medicine without consulting a doctor. The most common reason for self-medication was previous experience (50.1%) and the most common symptoms were headache (72.4%), flu (65.5%), and fever (55.2%). Commonly used medicines were analgesics (88.3%), antipyretics (65.1%) and antibiotics (35.2%). Eighty seven percent of students thought self-medication could be harmful and 82.5% students thought that it was necessary to consult a doctor before taking a new medicine. There was no significant difference between the self medication practices of medical and non medical students (p=0.8) CONCLUSION: Prevalence of self-medication is high in the educated youth, despite majority being aware of its harmful effects. There is a need to educate the youth to ensure safe practices. Strict policies need to be implemented on the advertising and selling of medications to prevent this problem from escalating.


Subject(s)
Health Knowledge, Attitudes, Practice , Self Medication/statistics & numerical data , Students/statistics & numerical data , Universities/statistics & numerical data , Adult , Cross-Sectional Studies , Female , Humans , Male , Pakistan , Surveys and Questionnaires
SELECTION OF CITATIONS
SEARCH DETAIL
...