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1.
Cureus ; 13(7): e16558, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34430162

RESUMO

Introduction Hepatitis B virus (HBV) is the leading cause of cirrhosis in the developing world. Despite the development of effective vaccine(s) and direct-acting antivirals (DAAs) such as tenofovir and entecavir, the eradication of chronic HBV remains a distant dream in endemic regions. Factors such as treatment naivety, longer duration of disease, late diagnosis, family history of liver disorders and hepatocellular carcinoma, fatty liver disease, multiple comorbidities, alcoholism, use of tobacco products, noncompliance to drugs, and loss to follow-up all contribute to disease progression and development of complications. In order to promote a better understanding of the treatment initiation, duration, and eventual outcomes, the European Association for the Study of the Liver (EASL) updated its guidelines in 2017 and introduced a new staging system for chronic HBV. Since these guidelines are relatively recent, data regarding the new staging is virtually absent in local/regional settings. Moreover, it has been observed that patients presenting to secondary care setups at major urban centers have disease characteristics quite distinct from those of their rural counterparts or patients presenting to tertiary care setups, even in the same cities. Additionally, there is scarce published data with regard to this aspect. With this study, we hope to make progress on all of those fronts. Objective We aimed to evaluate the demographics, biochemical characteristics, and phases of chronic HBV at the secondary care level. Methods This was a retrospective observational cohort conducted at the Sindh Government Hospital, Liaquatabad from January to December 2020. Patients of either gender who were aged ≥12 years, and diagnosed as a case of chronic HBV were eligible to be enrolled in the study. Only treatment-naive patients were enrolled in the study. Demographics, biochemical characteristics, and phases of HBV (in light of the updated guidelines issued by EASL in 2017 with respect to HBV) were recorded and analyzed. Patient confidentiality was ensured at all times. Results A total of 102 patients were enrolled in the study. The mean age of our cohort was 34.31 ±12.42 years, and the male-to-female ratio was 3:2. All referrals were made from within the city district. Housewives and laborers made up the majority of our patients. The mean alanine transaminase (ALT) levels were 39.83 ±21.33 IU/L; otherwise, the biochemical profile of the patients was unremarkable. Phase III was the most frequently recorded stage of chronic HBV; 41 (40.19%) of the patients were diagnosed with it. However, nearly half of the patients had chronic active hepatitis (phases II and IV). Only a single case each of hepatitis Delta virus and decompensated liver disease (DCLD) was recorded. Conclusions All patients of chronic HBV presenting at the secondary care level were referred locally, were relatively older, and exhibited a higher frequency of chronic active hepatitis (phases II and IV). This corresponded to borderline elevations of ALT. But the remainder of the biochemical profile was largely unremarkable due to the very low prevalence of cirrhosis among these patients.

2.
Clin Lymphoma Myeloma Leuk ; 21(10): 659-666, 2021 10.
Artigo em Inglês | MEDLINE | ID: mdl-34275772

RESUMO

BACKGROUND: Hairy cell leukemia (HCL) responds well to purine analogs with an overall median relapse free survival of 11-16 years. Most patients can be retreated with the same or a different purine analog however a subset of patients will become resistant or develop cumulative toxicities. Novel agents such as Vemurafenib (BRAF kinase inhibitor), Bendamustine/Rituximab (BR), Moxetumomab pasudotox (anti CD-22 recombinant immunotoxin) and Ibrutinib have emerging roles in patients with relapsed HCL. METHODS: Five databases (PubMed, Embase, Cochrane Library, Web of Science and ClinicalTrials.gov) were searched using the following search terms: "hairy cell leukemia" or "leukemia, hairy cell" AND "relapse" or "recurrence". We included only prospective clinical trials with outcome data. RESULTS: Vemurafenib monotherapy was evaluated in two separate arms of a phase 2 trial. In the US arm (n=24), the ORR was 100% (CR 42%; PR 58%). In the Italian arm (n=26), the ORR was 96% (CR 35%; PR 62%). In a phase 2 study (n=25), the combination of vemurafenib and rituximab showed CR of 100%. The combination of BR achieved an ORR of 100% whereas CR was 50% and 67% at a bendamustine dose of 70mg/m2 (n=6) and 90 mg/m2 (n=6) respectively. In a phase 3 trial, moxetumomab pasudotox (n=80) had an ORR of 75% (CR 41%). Single agent Ibrutinib (n=37) had an ORR of 54%. Therapies were generally well tolerated. CONCLUSION: Novel agents have good efficacy in HCL in patients with multiple relapses.


Assuntos
Antineoplásicos/uso terapêutico , Leucemia de Células Pilosas/tratamento farmacológico , Adolescente , Antineoplásicos/farmacologia , Criança , Feminino , Humanos , Masculino
3.
Cureus ; 13(5): e14828, 2021 May 03.
Artigo em Inglês | MEDLINE | ID: mdl-34104581

RESUMO

Introduction Asthma is a clinical syndrome characterized by reversible and recurrent airway obstruction leading to the symptoms of wheezing, cough, shortness of breath, fatigue, and respiratory distress. It is one of the most common lung pathologies worldwide. Its incidence is on the rise in Pakistan, which may be due to overt environmental pollution or improving screening protocols. Irrespective more and more patients are now being diagnosed as cases of asthma and this has led to renewed interest in research for the subject locally. Vitamin D plays a key component of the immune system and its deficiency has been associated with diseases, such as inflammatory bowel disease, celiac disease, rheumatoid arthritis, depression, sepsis, and coronavirus disease 2019 (COVID-19) pneumonia. The role of vitamin D in exacerbation, prevention, causation, and treatment of asthma is still up for debate. However, as more data emerges, it is becoming evident that vitamin D in one way or another is linked to outcomes in asthma. Especially the deficiency of vitamin D in asthma and its supplementation has garnered great attention in scientific trials. Our research is just one small step in this direction. This study aims to evaluate the frequency of vitamin D deficiency in patients with asthma. Methods This was a retrospective cross-sectional cohort conducted at the Department of Medicine of Abbasi Shaheed Hospital Karachi (ASHK) during March 2019 to August 2019. Patients of either gender aged ≥18 years that were diagnosed with cases of asthma were eligible to be included in the study. Asthma was defined in accordance with the latest guidelines issued by the Pakistan Chest Society. A vitamin D level of <20 ng/dl was considered deficient. Patient confidentiality was made certain.  Results A total of 97 (62.98%) of the 154 patients included in the study had <20 ng/ml of vitamin D level. The mean vitamin D level recorded in this study was 15.34±4.21 ng/dl. The male-to-female ratio was 1:5. The mean age of our cohort was 42.78±4.56 years. The mean duration of disease, i.e., asthma was 6.7±3.68 years. Both the age of the patients and duration of the disease were found to be statistically significant with respect to vitamin D levels in asthmatics. Conclusions The frequency of vitamin D deficiency is very high in patients with asthma. These suboptimal levels are significantly influenced by the age of the patient and the duration of the disease.

4.
Bone Marrow Transplant ; 56(4): 863-872, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33184452

RESUMO

Management of rare hematological disorders pose unique diagnostic and therapeutic challenges due to unusual occurrence and limited treatment options. We retrospectively identified 45 patients receiving matched related donor transplant for rare hematological disorders from 2006 to 2019. Patients were divided into two groups (1) malignant and (2) non malignant. The malignant disorder group included four patients while the nonmalignant group included 41 patients divided into immune dysregulation (n = 23), bone marrow failure (n = 10), metabolic (n = 5), and bleeding diathesis (n = 3). Twenty-six (57.8%) patients received myeloablative conditioning (MAC) and 16 (35.6%) received reduced intensity conditioning (RIC), while 3 (6.6%) patients with severe combined immunodeficiency received stem cell infusion alone without conditioning. The cumulative incidence (CI) of grade II-IV acute GVHD (aGVHD) was 39.1% (n = 18) and chronic GVHD (cGVHD) 15.2% (n = 7). There was no primary graft failure while CI of secondary graft failure was 9%. Overall survival (OS) and disease-free survival (DFS) was 82.2% and 77.8% respectively. Group wise OS was 75% in the malignant group, 82.6% in the immune dysregulation group, 80% in patients with metabolic disorders and bone marrow failure, while 100% in patients with bleeding diathesis. This retrospective analysis shows that hematopoietic stem cell transplant can be a feasible treatment option for rare hematological disorders.


Assuntos
Doença Enxerto-Hospedeiro , Transplante de Células-Tronco Hematopoéticas , Humanos , Paquistão , Estudos Retrospectivos , Condicionamento Pré-Transplante
5.
Cureus ; 11(8): e5302, 2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-31579641

RESUMO

Introduction Osteoarthritis (OA) is the single-most common cause of physical disability among adults. In view of its promising management modalities, an analysis of the level of awareness among the suspected individuals and their attitudes is crucial to assess the level of their implementation. This study aimed to do that among the female population in Karachi, Pakistan. Methods This cross-sectional study was conducted among 316 elderly females (≥60 years) in Karachi in 2018. Data were collected via a structured questionnaire, which included sections titled: demographics, knowledge, attitudes, and practices. Data were analyzed using the Statistical Package for Social Sciences (version 20.20, IBM, Armonk, New York, US), and chi-square tests were used to assess the connection between OA care and socioeconomic statuses. Mean and standard deviation were calculated for quantitative variables. Results A considerable portion (48%, n = 152) of the participants were from a lower socioeconomic background, and 51% of them had a history of joint pain. Significantly, 63% of the participants (n = 199) attributed their joint pain to age, while nearly half attributed it to their diet and exercise habits. A major segment (73%, n = 230) of the participants, irrespective of their socioeconomic backgrounds, had visited a doctor for their joint complaints. Around 65% of the participants said they would never undergo a knee-joint replacement, regardless of how worse their symptoms might get. Additionally, 36% of the participants were unsatisfied with their current treatment, while more than half of the participants said that medication would improve their condition. Conclusions Our results demonstrate a connection between the lack of awareness about OA and the factors negatively affecting its management. They also point towards areas that require focused efforts for better awareness.

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