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Optimal grain-appearance quality is largely determined by grain size. To date, dozens of grain size-related genes have been identified. However, the regulatory mechanism of slender grain formation is not fully clear. We identified the OsSG34 gene by map-based cloning. A 9-bp deletion on 5'-untranslated region of OsSG34, which resulted in the expression difference between the wild-type and sg34 mutant, led to the slender grains and good transparency in sg34 mutant. OsSG34 as an α/ß fold triacylglycerol lipase affected the triglyceride content directly, and the components of cell wall indirectly, especially the lignin between the inner and outer lemmas in rice grains, which could affect the change in grain size by altering cell proliferation and expansion, while the change in starch content and starch granule arrangement in endosperm could affect the grain-appearance quality. Moreover, the OsERF71 was identified to directly bind to cis-element on the mutant site, thereby regulating the OsSG34 expression. Knockout of three OsSG34 homologous genes resulted in slender grains as well. The study demonstrated OsSG34, involved in lipid metabolism, affected grain size and quality. Our findings suggest that the OsSG34 gene could be used in rice breeding for high yield and good grain-appearance quality via marker-assisted selection and gene-editing approaches.
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Oryza , Oryza/genética , Oryza/metabolismo , Proteínas de Plantas/genética , Proteínas de Plantas/metabolismo , Melhoramento Vegetal , Endosperma/genética , Endosperma/metabolismo , Grão Comestível/genética , Grão Comestível/metabolismo , Amido/metabolismoRESUMO
Objectives: To compare the efficacy of mycophenolate mofetil with intravenous cyclophosphamideas induction therapy in lupus nephritis. METHODS: The observational, prospecrive, cohort study was conducted at the Rheumatology Department of Fatima Memorial Hospital, Lahore, Pakistan, from July 2016 to June 2019, and comprised lupus nephritis patients. For induction therapy, the patients were assigned at the discretion of the treating rheumatologist to mycophenolate mofetil group MMF, and intravenous cyclophosphamide group CYC. The latter group was further divided into NIH subgroup that received the therapy as per the protocol of the National Institutes of Health, and ELNT subgroup which recived the therapy as per the Euro Lupus Nephritis Trial protocol. Maintenance therapy in all groups was mycophenolate mofetil. Tacrolimus was added in case of non-response. The outcome was the achievement of complete renal response at 6, 12 and 24 months. Data was analysed using SPSS 26. RESULTS: Of the 131 patients, 126(96.2%) were females. The overall mean age was 27±7.7 years. There were 58(44.2%) patients in group MMF and 73(55.7%) in group CYC, which had subgroup NIH 46(63%) and subgrpup ELNT 27(37%). The complete renal response rates at 6, 12, and 24 months were 22 (43.1%), 35 (71.4%), and 40(83.3%) for group MMF; 5(12.5%), 9(22%) and 24 (58.5%) for subgroup NIH, and 6(26.1%), 8(36.4%) and 14(63.6%) for subgroup ELNT. Group MMF outcomes were significantly better than the rest (p<0.05). CONCLUSIONS: Mycophenolate mofetil induction therapy was more effective than intraveenous cyclophosphamide in terms of achieving remission at 6, 12 and 24 months.
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Ciclofosfamida , Imunossupressores , Nefrite Lúpica , Ácido Micofenólico , Centros de Atenção Terciária , Humanos , Nefrite Lúpica/tratamento farmacológico , Ácido Micofenólico/uso terapêutico , Ciclofosfamida/uso terapêutico , Feminino , Adulto , Paquistão , Masculino , Imunossupressores/uso terapêutico , Adulto Jovem , Resultado do Tratamento , Estudos de Coortes , Tacrolimo/uso terapêutico , Quimioterapia de Indução/métodos , Indução de Remissão/métodosRESUMO
Pachydermodactyly (PDD) is a rare benign condition characterized by painless soft tissue swelling of small joints of hands. The most common presentation is bilateral Symmetrical swelling of proximal interphalangeal and metacarpophalangeal joint similar to Rheumatoid arthritis. The etiology of this disease is unknown, and it sometimes can coexist with other diseases. We present here a case of PDD coexisting with Tuberous Sclerosis, an autosomal dominant genetic disorder characterized by of formation of multiple benign multisystem tumors.
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OBJECTIVE: To analyze the clinical manifestation of patients with Behcet's disease, and performance of different classification criteria of Behcet's disease in our population. METHODS: It was a retrospective analysis of all Behcet's disease patients attending Department of Rheumatology at Fatima Memorial Hospital, Lahore, Pakistan from April 2019 to July 2019. We performed a comprehensive clinical evaluation of patients with Behcet's disease, with focus on patients' age, gender and different clinical manifestations. RESULTS: A consecutive cohort of 20 patients was studied. All patients met the International Criteria of Behcet's Disease criteria, and 18 out of 20 patients also met International Study Group criteria. Mean age of the cohort was 33.5±10.4 years and 45% was female (male to female ratio of 1:1.2). Around 90% of cohort had recurrent oral and genital ulcers. Ocular involvement was present in 80% patients, while Joint manifestations were present in 75% of patients. Cutaneous, neurological (both central and peripheral nervous system involvement), and GIT symptoms were present in 50%, 30%, and 15% of patients, consecutively. Joint pain and eye symptoms were major initial symptoms in males, while eye symptoms and neurological symptoms were more common in female patients at disease onset. Reaching border line significance, Cutaneous(p-value=0.479), ocular(p-value=0.61), and GIT involvement(p-value=0.59) were more prevalent in males while neurological involvement (p-value=0.336) in females. CONCLUSION: Behcet's disease occurs commonly in middle age population with equal male to female ratio, with mucocutaneus aphthosis, ocular disease and joint pains being common manifestation. Gastrointestinal symptoms are more common in males while neurological symptoms in females.
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PURPOSE: Previous studies in the western world have reported the possible association of obstructive sleep apnea (OSA) with anxiety and cardiovascular diseases. However, the data from developing countries such as Pakistan remains scarce in this regard. The main aim of this study was to investigate the prevalence of coronary artery disease (CAD) patients who are at high risk of OSA and to determine the association between the risk of OSA and levels of anxiety. METHODS: The sample population consisted of 400 participants including 200 patients, who had been previously diagnosed with CAD, and 200 healthy controls. The 200 CAD patients were approached for inclusion in the study on their follow-ups after their acute symptoms had subsided. The patients were interviewed after a mean time period of 13 weeks post event. All patients with lung disease or respiratory infection were excluded from the study. The risk of OSA was determined using Berlin questionnaire, while the levels of anxiety were measured by Beck anxiety inventory scale (BAI). RESULTS: More than half (n = 104) of the CAD patients were at high risk of OSA while majority (n = 168) of the healthy controls were at low risk. The high risk of OSA was significantly (P < 0.0001) more prevalent in CAD patients compared with controls. It was also observed that the patients who were at high risk of OSA, among both cases and controls, had significantly (P values <0.001) higher levels of anxiety. CONCLUSION: A significant proportion of CAD patients are at high risk of OSA in our region. Moreover, OSA is also associated with greater levels of anxiety in both healthy people and CAD patients.
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Transtornos de Ansiedade/epidemiologia , Doença da Artéria Coronariana/epidemiologia , Países em Desenvolvimento , Medição de Risco , Apneia Obstrutiva do Sono/epidemiologia , Adulto , Idoso , Transtornos de Ansiedade/diagnóstico , Estudos de Casos e Controles , Doença da Artéria Coronariana/diagnóstico , Estudos Transversais , Feminino , Humanos , Masculino , Programas de Rastreamento , Pessoa de Meia-Idade , Paquistão , Apneia Obstrutiva do Sono/diagnóstico , Estatística como AssuntoRESUMO
Compliance to dietary recommendations among hypertensive people is a major health care issue. Non-compliance can nullify the effects of even the most scientific and optimum treatment plan. The main aim of this study was to determine the frequency and predictors of non-adherence in our region. We also investigated the possible factors based on patient opinions that could increase compliance. A sample of 400 adult patients, diagnosed with hypertension for at least 3 years, who visited Civil Hospital during the time period April-June 2013 were included in this cross sectional study. Patient data and opinions were collected by two trained interviewers using a pre-coded questionnaire. Compliance was assessed based on patients self report. More than three quarters (n = 310, 77.5%) of the hypertensive patients were non-compliant. More than one social gathering in a week, peer-influence, no friends to follow the recommended diet plan and lack of believe regarding diet as an effective measure to control blood pressure were found to be the significant predictors of non-compliance (P values <0.0001). Counseling was thought to be the most effective way by the patients to increase adherence. Non-compliance to dietary recommendations is a major problem prevalent especially in Pakistan. There is an urgent need for doctors and nurses to counsel their patients effectively to prevent future morbidities and mortalities because of non-compliance.
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Dieta/normas , Conhecimentos, Atitudes e Prática em Saúde , Hipertensão/dietoterapia , Cooperação do Paciente/psicologia , Fatores Etários , Estudos Transversais , Escolaridade , Feminino , Humanos , Hipertensão/complicações , Hipertensão/prevenção & controle , Entrevistas como Assunto , Masculino , Estado Civil , Pessoa de Meia-Idade , Paquistão , Cooperação do Paciente/estatística & dados numéricos , Fatores de Risco , Comportamento SocialRESUMO
BACKGROUND AND AIM: Benefits of the skeletonized internal thoracic artery (ITA) include increased graft flow, increased graft length, and reduced incidence of sternal complications. We conducted a randomized, double-blinded comparison of skeletonized versus pedicled ITA to assess the differences in pain intensity and extent of disability between the two types of harvesting procedures at one and three months follow-up. METHODS: A total of 50 patients were included in our study. Twenty-five patients had undergone skeletonized grafting while the other half had undergone pedicled grafting. The patients were evaluated for their pain at one and three months postoperatively. Extent of disability was measured via Pain Disability Index and intensity of pain was measured via Visual Analogue Scale (VAS). The patients were also questioned about the details of their pain using Short Form McGill Pain Questionnaire. RESULTS: In the first month, the mean pain intensity measured through VAS was 30.4 ± 4.0 and 55.0 ± 5.7 mm in skeletonized and pedicle group, respectively. The pedicled group had significantly higher scores measured by all three scales at both one- and three-month intervals (p-values < 0.0001). CONCLUSIONS: Our results indicate that skeletonization of ITA significantly reduces postcoronary artery bypass graft surgery pain at both one- and three-month intervals. Long-term clinical trials involving larger sample sizes should be conducted to fully confirm the benefits of the skeletonization technique.
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Ponte de Artéria Coronária/métodos , Dor Pós-Operatória/prevenção & controle , Coleta de Tecidos e Órgãos/métodos , Método Duplo-Cego , Feminino , Seguimentos , Humanos , Masculino , Artéria Torácica Interna/transplante , Medição da Dor/métodos , Dor Pós-Operatória/diagnóstico , Índice de Gravidade de Doença , Inquéritos e Questionários , Fatores de TempoRESUMO
Coronary artery bypass graft surgery relieves angina symptoms and reduces mortality among ischaemic heart disease patients. It remains the gold standard for the treatment of multi-vessel and left main coronary artery disease. It is a well-known fact that internal mammary artery conduits have excellent and long-lasting patency when used for coronary artery bypass grafting. Its supremacy is largely because it prevents atherosclerosis. The old-style internal mammary artery bypass grafting, classically known as pedicle grafting, includes a circular rim of tissue around the graft. Bilateral pedicled internal mammary arteries, especially among diabetic patients, have been reported to cause complications in the sternum like sternal osteomyelitis. In many studies it has been reported that dissection of pedicled internal mammary artery can lead to sternal devascularisation which can lead to higher incidence of infections. Considering the higher incidence of deep sternal infections in patients with double pedicled arterial grafts, dissection of internal mammary artery in skeletonised manner was proposed. In this review, we outline the advantages of skeletonised grafting with respect to incidence of sternal infection, patency rates, blood flow, post-coronary artery bypass graft pain and the length of the graft.
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Anastomose de Artéria Torácica Interna-Coronária/métodos , Ponte de Artéria Coronária/métodos , Humanos , Anastomose de Artéria Torácica Interna-Coronária/tendências , Dor Pós-Operatória/epidemiologia , Artéria Radial/transplante , Esterno , Infecção da Ferida Cirúrgica/epidemiologia , Coleta de Tecidos e Órgãos , Resultado do Tratamento , Grau de Desobstrução VascularRESUMO
Patients with penetrating thoracic injuries should be managed appropriately and rapidly under the supervision of multi-disciplinary team. We present a case of a 9-year-old female student who survived a major road traffic accident (RTA), a leading contributor to accidental deaths in Pakistan. The girl was hit by a public transport bus impaled by a metallic rod through the right axilla, and subsequently dragged for a distance, resulting in avulsion of a broad front of the mid-anterior and left precordial part of the chest wall. Although both the lungs and heart were exposed, she did not suffer any myocardial injury. Despite massive hypovolaemic shock with bilateral open pneumothoraces, the girl made an uneventful recovery. She was discharged on the 7th post-operative day. The report highlights the value of proper management of hypovolaemic shock and optimally timed and executed surgical intervention.
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Acidentes de Trânsito , Traumatismos Torácicos/cirurgia , Ferimentos Penetrantes/cirurgia , Criança , Feminino , HumanosRESUMO
Natural resistance-associated macrophage proteins (NRAMPs) are a class of metal transporters found in plants that exhibit diverse functions across different species. Transporter proteins facilitate the absorption, distribution, and sequestration of metallic elements within various plant tissues. Despite the extensive identification of NRAMP family genes in various species, a full analysis of these genes in tree species is still necessary. Genome-wide identification and bioinformatics analysis were performed to understand the roles of NRAMP genes in copper (CuCl2) stress in Kandelia obovata (Ko). In Arachis hypogaea L., Populus trichocarpa, Vitis vinifera, Phaseolus vulgaris L., Camellia sinensis, Spirodela polyrhiza, Glycine max L. and Solanum lycopersicum, a genome-wide study of the NRAMP gene family was performed earlier. The domain and 3D structural variation, phylogenetic tree, chromosomal distributions, gene structure, motif analysis, subcellular localization, cis-regulatory elements, synteny and duplication analysis, and expression profiles in leaves and CuCl2 were all investigated in this research. In order to comprehend the notable functions of the NRAMP gene family in Kandelia obovata, a comprehensive investigation was conducted at the genomic level. This study successfully found five NRAMP genes, encompassing one gene pair resulting from whole-genome duplication and a gene that had undergone segmental duplication. The examination of chromosomal position revealed an unequal distribution of the KoNRAMP genes across chromosomes 1, 2, 5, 7, and 18. The KoNRAMPs can be classified into three subgroups (I, II, and SLC) based on phylogeny and synteny analyses, similar to Solanum lycopersicum. Examining cis-regulatory elements in the promoters revealed five hormone-correlated responsive elements and four stress-related responsive elements. The genomic architecture and properties of 10 highly conserved motifs are similar among members of the NRAMP gene family. The conducted investigations demonstrated that the expression levels of all five genes exhibited alterations in response to different levels of CuCl2 stress. The results of this study offer crucial insights into the roles of KoNRAMPs in the response of Kandelia obovata to CuCl2 stress.
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OBJECTIVE: To evaluate the correlation of quantitative anti-dsDNA level with proteinuria levels in patients with lupus nephritis in a tertiary care hospital. STUDY DESIGN: In this prospective cross-sectional study, 76 patients of newly diagnosed SLE coming to Fatima Memorial Hospital were included in the study period between January 2020 to June 2020. Demographic data such as age, gender, lupus manifestations such as serositis, arthritis, mucocutaneous disease, and neuropsychiatric manifestations were recorded. Quantitative anti-dsDNA was measured by enzyme-linked immunosorbent assay and proteinuria was estimated by 24h urinary protein collection. Data was analyzed by SPSS 23. Association between categorical variables was assessed using chi-square test. For comparison of categorical independent and continuous dependent variable t-test or Mann-Whitney U test was applied. RESULTS: The median age of the cohort was 29 (with inter quartile range - IQR - of 13) years. The female gender comprised of 68 (89.4%) of the cohort population. The median anti-dsDNA level was 54.9 (183.6 IQR) IU, and baseline proteinuria of the cohort was 520mg/dL (1.49 IQR). There was a significant association of anti-dsDNA level with systemic features such as arthritis (p=<0.01), serositis (p=<0.01) and, Raynaud's phenomenon (p=<0.01). NPSLE and mucocutaneous features did not show statistically significant association (p=0.91 and 0.14 respectively). Baseline anti-dsDNA showed a statistically significant correlation with baseline proteinuria levels (p=<0.01). CONCLUSION: Quantitative anti-dsDNA is directly correlated with nephritis measured as proteinuria, and can be detected even before organ involvement. Hence, it can determine disease course and guide early treatment.
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Artrite , Lúpus Eritematoso Sistêmico , Serosite , Adolescente , Anticorpos Antinucleares , Estudos Transversais , DNA , Feminino , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Estudos Prospectivos , Proteinúria/etiologiaRESUMO
BACKGROUND: Takayasu arteritis (TKA) is a rare large vessel vasculitis occurring in young adults of less than 50 year of age. We analyse the clinical, radiological features, and treatment regimens in Pakistani patients presenting to a tertiary care center. METHODS: A retrospective cross-sectional analysis of TKA patients done at the Rheumatology department of Fatima Memorial Hospital. A comprehensive evaluation of clinical, laboratory, radiographic features and treatment regimens was carried out. RESULTS: A consecutive cohort of 18 patients, with 13 patients (72%) of female gender was studied. Mean age of the cohort was 35.94±2.7 years. A mean delay of 2.32±0.43 years between symptoms and final diagnosis was reported, attributed to alternate diagnosis in 57.1% and late presentation in 42.8% cases. Limb claudication (44.4%), absent pulses (38.9%), were the common initial manifestation. Hypertension (61.5%), blood pressure discrepancy between arms (88.9%) and bruit (72.2%) over major vessels were common systemic features. As per angiographic classification, Type V (44.4%), and Type I (33.3%), were most common pattern of disease in the cohort. Subclavian artery (72.2%), renal artery (33.3%), iliofemoral arteries (27.8%), and coronary artery involvement (16.7%) were the common lesions. Coronary artery lesion was higher in females (p=0.52) while renal artery lesion in males (p=0.27). There was no statistically significant difference in involvement of vessels according to gender (p >0.05). CONCLUSIONS: Type V and Type I are the common pattern of TKA. Limb claudication was the most common initial manifestation. Renal artery involvement was seen more commonly in males while coronary artery involvement more commonly in females.
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Arterite de Takayasu , Adulto , Angiografia , Estudos Transversais , Feminino , Humanos , Masculino , Paquistão/epidemiologia , Estudos Retrospectivos , Arterite de Takayasu/diagnóstico por imagem , Adulto JovemRESUMO
A 25-year-old female presented on the acute medical take with rapidly evolving ascending weakness, sensory loss, and areflexia after a prodromal diarrhoeal illness, ultimately critical care admission, tracheostomy, and intravenous immunoglobulin (IVIG) therapy. The patient had been diagnosed with Guillain-Barré Syndrome (GBS) six years previously, treated with intravenous Immunoglobulin, and discharged after a five-day in-patient stay without mechanical ventilation. On this occasion, a diagnosis of recurrent GBS was made, supported by cytoalbuminological dissociation in the cerebrospinal fluid (CSF). Investigations for infective precipitants were negative aside from a stool culture, positive for Cryptosporidium spp. DNA (deoxyribonucleic acid) two weeks earlier. There are no previously reported cases of GBS due to cryptosporidiosis on PubMed. The patient was treated with a course of IVIG and discharged from critical care after 66 days, requiring ongoing neurorehabilitation, which is likely to be prolonged.
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Objective To assess the frequency of early postoperative complications and surgical anatomy after ileostomy reversal among the population of Khyber Pakhtunkhwa, Pakistan. Materials and methods In the current study, a total of 241 patients were assessed. Sufficient urine output, usual serum electrolytes and urea were indicators of a sufficient recovery. All patients remained in the ward for a minimum of seven days after surgery to detect early postoperative complications like surgical site infection (SSI), wound dehiscence, small bowel obstruction, and anastomotic leak. Results In the present study, 113 (47%) were in age 18-40 years, while 128 (53%) patients were in age 41-60 years. The mean age was 40±10.05. One hundred twenty-three (51%) were male, and 118 (49%) patients were female. One hundred seventy-one (71%) had ileostomy closure in ≤3 months, 70 (29%) had ileostomy closure in >3 months. The mean duration of closure was 03±3.70 months. One hundred and six (44%) had enteric perforation, 87 (36%) had blunt trauma, 48 (20%) had tuberculous abdomen. Moreover, the frequency of early complications of ileostomy closure was analyzed as 19 (8%) had surgical site infection, 14 (6%) patients had wound dehiscence, 12 (5%) patients had small bowel obstruction, and three (1%) patients had anastomotic leakage. Conclusions Our study concluded that early postoperative complications and surgical anatomy after ileostomy reversal among the population of Khyber Pakhtunkhwa, Pakistan were surgical site infection (8%), wound dehiscence (6%), small bowel obstruction (5%), and anastomotic leak was (1%).
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BACKGROUND: Eye involvement is a common and potentially devastating complication of various immune related rheumatic diseases. We aimed to determine the spectrum, associations and the impact of ocular manifestations among well characterised autoimmune rheumatic disease patients presenting to ophthalmology and rheumatology clinics in a tertiary care hospital in Lahore, Pakistan. METHODS: Descriptive cross-sectional study performed in Rheumatology department of Fatima memorial hospital. Only those rheumatic disease patients were included who have been attending ophthalmology department for their ophthalmic conditions. The patients with ophthalmic symptoms who have not attended an ophthalmologist were not included in this study. Proforma was designed and the studied parameters were recorded prospectively from patient's interview and also by reviewing patient's medical and ophthalmologic medical records. Parameters assessed were demographics, symptoms and the diagnosis of eye disease, unilateral or bilateral presentation, duration of eye symptoms along with the duration of the primary rheumatologic disease, and the complications of the eye disease whether due to the eye diagnosis or its treatment. RESULTS: Eighty-three consecutive patients with mean age 33±11 years, 67.5% being female were recruited. Spondyloarthritis (SpA) comprised 38.6% (n=32) of patients followed by 21.7% (n=18) of Behcet's disease. Majority of patients (68.7%) had bilateral eye symptoms. In our cohort, 70% (n=58) of the patients had uveitis and almost all of these patients had either SpA or Behcet's disease as their primary rheumatologic diagnosis. Scleritis in 15.7% (n=13) patients followed by retinal vasculitis in 9.6% (n=8) patients, while glaucoma and keratopathy were present in 2.4% (n=2) patients each comprised other manifestations. Reassuringly our uveitis patients had no long-term eye related complications either due to the disease or its treatment. CONCLUSIONS: Uveitis represented the most common ophthalmologic manifestation associated with underlying rheumatologic diseases in our cohort, and it was associated with underlying Behcet's disease and SpA. Uveitis associated with connective tissue diseases has good prognosis with low-risk of significant long-term complications. Moreover, uveitis associated with SpA was noted to present much earlier in its disease course.
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Doenças Autoimunes , Doenças Reumáticas , Uveíte , Adulto , Estudos Transversais , Feminino , Humanos , Masculino , Paquistão/epidemiologia , Doenças Reumáticas/complicações , Doenças Reumáticas/epidemiologia , Atenção Terciária à Saúde , Uveíte/epidemiologia , Uveíte/etiologia , Adulto JovemRESUMO
Objectives: This study aims to evaluate the clinical and serological characteristics of systemic sclerosis (SSc) in Pakistani population. Patients and methods: This prospective, cross-sectional study included a total of 38 patients (6 males, 32 females; mean age: 34.5±1.5 years; range, 16 to 60 years) with SSc who were admitted to our rheumatology clinic between November 2019 and January 2020. We evaluated the clinical, serological, and radiological features of SSc patients. Results: Thirty-four (89.5%) patients developed Raynaud phenomenon at the time of disease onset, while sclerodactyly was found in 34 (89.5%), digital ulcers in 25 (65.8%), and tendon friction rub in 12 (31.6%) patients. Interstitial lung disease was present in 30 (78.9%) patients with a higher prevalence in diffuse scleroderma (100%) than in limited scleroderma (70%) (p=0.01). Pulmonary hypertension was present in 18 patients with a significantly higher prevalence in diffuse disease (57.1%) than limited disease (11.8%) (p<0.01). Thirty (78.9%) patients had impaired pulmonary function tests. Fibromyalgia was present in seven (18.4%) patients, and depression was present in 10 (26.3%) patients. Antinuclear antibody (ANA) was positive in 30 (78.9%) patients. Anti-Scl-70 antibodies were present in 24 (63.2%) patients with a significant association with diffuse disease (85% vs. 35.3%, respectively; p<0.01). The anti-centromere antibodies (ACA) were present in 20 (52.6%) patients with a significantly higher rate in limited disease (94.2% vs. 19.0%, respectively; p<0.01). Conclusion: Scleroderma has a female preponderance. Raynaud phenomenon is the most initial clinical feature followed by other manifestations of a variable course and disease severity.
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BACKGROUND: Pulmonary valve stenosis (PS) is common congenital heart disease in children and patient can present with cyanosis, chest pain, dyspnea and failure with severe form. The objective of this study was to enlighten the short outcome of balloon pulmonary valvuloplasty in children with severe pulmonary stenosis. METHODS: This cross sectional observational study was done in paediatric cardiology department of Lady Reading Hosptial, Peshawer form June 2019 to December 2020 over 1.5 years. Children aged 6 months to 16 years of either sex diagnosed as case of severe stenosis with doming pulmonary valve and having symptoms of chest pain and dysnea on exeration and pregradient pressure of 64 mm Hg or more on echocardiography were included. Patients were interviened with ballon-valvuloplasty. Outcome was taken as discharge from hospital with complications after procedure if any. Patients were followed up for 3 months. Data including age, sex, pre cath echocardiography, cath pulmonary valve annulus, post ballooning pulmonary valve (PV) gradient, PV gradient on echocardiography after intervention and follow up gradient on echocardiography at three months was documented. Data was analyzed by SPSS 20. Chi square test and paired T - Test was applied where required. Results were taken as significant with p value <0.05. RESULTS: There were 51 patients, 35 (68.6%) male and 16 (31.4%) female. Mean age was 8.35±4.93 years. Mean pre cath gradient across the pulmonary valve on echocardiography was 109.14±31.44 mm Hg. Post intervention mean pressure gradient across PV was 32.41±11.49 mm Hg. Pulmonary valve annulus on echocardiography before intervention ranged from 7 to 25 mm with mean of 14.67±3.79 mm. There was no complication in majority (82.4%) of patients. Mild PR was in 5 (9.8%) patients. There was significant relationship between pre and post intervention pressure gradient across PV valve with p value of <0.001. CONCLUSIONS: Balloon pulmonary valvuloplasty in one of the safest intervention for PS in children with few complications.
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Valvuloplastia com Balão , Estenose da Valva Pulmonar , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Feminino , Seguimentos , Humanos , Lactente , Masculino , Estenose da Valva Pulmonar/cirurgia , Resultado do TratamentoRESUMO
Understanding how multidrug-resistant Enterobacterales (MDRE) are transmitted in low- and middle-income countries (LMICs) is critical for implementing robust policies to curb the increasing burden of antimicrobial resistance (AMR). Here, we analysed samples from surgical site infections (SSIs), hospital surfaces (HSs) and arthropods (summer and winter 2016) to investigate the incidence and transmission of MDRE in a public hospital in Pakistan. We investigated Enterobacterales containing resistance genes (blaCTX-M-15, blaNDM and blaOXA-48-like) for identification, antimicrobial susceptibility testing and whole-genome sequencing. Genotypes, phylogenetic relationships and transmission events for isolates from different sources were investigated using single-nucleotide polymorphism (SNP) analysis with a cut-off of ≤20 SNPs. Escherichia coli (14.3%), Klebsiella pneumoniae (10.9%) and Enterobacter cloacae (16.3%) were the main MDRE species isolated. The carbapenemase gene blaNDM was most commonly detected, with 15.5%, 15.1% and 13.3% of samples positive in SSIs, HSs and arthropods, respectively. SNP (≤20) and spatiotemporal analysis revealed linkages in bacteria between SSIs, HSs and arthropods supporting the One Health approach to underpin infection control policies across LMICs and control AMR.
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Vetores Artrópodes/microbiologia , Farmacorresistência Bacteriana Múltipla , Infecções por Enterobacteriaceae/microbiologia , Enterobacteriaceae/isolamento & purificação , Infecção da Ferida Cirúrgica/microbiologia , Animais , Antibacterianos/farmacologia , Vetores Artrópodes/classificação , Proteínas de Bactérias/genética , Farmacorresistência Bacteriana Múltipla/efeitos dos fármacos , Farmacorresistência Bacteriana Múltipla/genética , Enterobacteriaceae/classificação , Enterobacteriaceae/efeitos dos fármacos , Enterobacteriaceae/genética , Infecções por Enterobacteriaceae/epidemiologia , Infecções por Enterobacteriaceae/transmissão , Microbiologia Ambiental , Variação Genética , Hospitais , Humanos , Testes de Sensibilidade Microbiana , Paquistão/epidemiologia , Filogenia , Plasmídeos/genética , Prevalência , Estações do Ano , Infecção da Ferida Cirúrgica/epidemiologia , Infecção da Ferida Cirúrgica/transmissão , beta-Lactamases/genéticaRESUMO
In Europe and North America, cardiac surgery can be done successfully on octogenarians, but differences in patient selection, surgical and bypass techniques prevent us from concluding similarly in Pakistan. This study investigated the short and long-term outcomes of cardiac surgery in octogenarians operated over a 5 years period at The Aga Khan University Hospital, from January 2001 to December 2006. Seventeen octogenarians, (mean age 81.7 ± 2.3) underwent coronary artery bypass graft (CABG). Thirty-days hospital mortality was reported in only 1 patient. Surgical complication of any kind was reported in 13 (76.5%) patients with pleural effusion being the commonest in hospital complication seen in 11 (64.7%) patients. About 13 (92.9%) patients were satisfied with the results of the surgery and reported post-surgery improvement in quality of their life. Cardiac surgery has an important role in the management of elderly patients with medically refractory cardiac symptoms, but the implications of widespread cardiac surgery in the elderly need to be addressed.
Assuntos
Ponte de Artéria Coronária , Idoso de 80 Anos ou mais , Ponte de Artéria Coronária/efeitos adversos , Ponte de Artéria Coronária/métodos , Ponte de Artéria Coronária/mortalidade , Feminino , Humanos , Paquistão , Fatores de RiscoRESUMO
Vestibular fistula represents the most common type of anorectal defect seen in girls with anorectal malformation. Adult presentation of this malformation is rare. The following is a case of adult presentation of anorectal malformation with a vestibular fistula in a 23 years old female, who came because of cosmetic and fertility concerns before her marriage. The patient complained of having an anorectal malformation since birth and passage of fecal matter through the fistula. The patient was continent for feces, but had leakage of flatus. She had no other associated anomaly. The patient subsequently underwent a surgical procedure where anal transposition was done with an intact skin bridge. Post-operatively, these developed an anal stenosis which was successfully treated through anoplasty, and the patient is now continent both for feces and flatus for the last 6 months.