Development, linguistic and clinimetric validation of the WOMAC VA3.01 Bangla for Bangladesh Index.

The aim of this study was to develop and to validate a Bengali version of the Western Ontario and McMaster Osteoarthritis (WOMAC) index in Bangladesh. The WOMAC was translated into the local language of Bangladesh (Bengali) and adapted in the local sociocultural context, following the standard guidelines by Beaton et al. Content validity of the preliminary Bengali version was assessed by using the index of content validity (ICV) and floor and ceiling effects. Patients were assessed at the Department of Rheumatology of Bangabandhu Sheikh Mujib Medical University and were diagnosed to have knee OA by American College of Rheumatology criteria and recruited according to the requirements of the validation study. Convergent and divergent validity were measured by comparing with Health Assessment Questionnaire (HAQ) and the Short Form-36 (SF-36), and internal consistency was assessed using Cronbach's alpha coefficient. The questionnaire was readministered to 40 patients within a week for assessing reliability by using intra-class correlation coefficient (ICC) and Spearman's rank correlation coefficient. In addition, factor analysis of Bengali WOMAC questionnaire was performed to examine the number of factors influencing a common set of items. A Bengali version was developed with changes in three items to suit local practices. The ICV of the content validity was 1 for all items. The Bengali WOMAC had similar construct validity when compared to the HAQ (ρ 0.74, n = 70) and SF-36 bodily pain and physical functioning. It had dissimilar construct validity to SF-36 mental health domain except WOMAC pain. Factor analysis revealed five factors with eigenvalues of more than 1.0. Cronbach's alpha and ICC exceeded 0.7 in all domains. In the test-retest reliability testing, Spearman's ρ for all items exceeded 0.4 (n = 40). This study has demonstrated that the Bengali version of WOMAC is a valid tool for assessing quality of life of patients with knee osteoarthritis in Bangladesh and is reliable.

Scleredema Diabeticorum - A Case Report.

Buschke Scleredema is a rare connective tissue disorder of unknown aetiology and characterized by thickening of the dermis. It resembles systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. It may be classified into three clinical groups, each has a different history, course, and prognosis. Histology of skin is characterized by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histological peculiarity. No therapy is effective. In this communication we have presented a 54 year old man with scleredema successfully treated by PUVA and methotrexate. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment.

Wegener's granulomatosis - a case report.

Wegener's granulomatosis is an uncommon multi-systemic disease characterized by necrotizing granulomatous inflammation of the upper and lower respiratory tracts and general focal necrotizing vasculitis (commonly known as Wegener's triad). The diagnosis of Wegener's granulomatosis is suggested from the clinical and laboratory findings and from the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA) although the absence of ANCA does not exclude the diagnosis. We described a case of 27-year-old man with pansinusitis, mild azotemia and initial respiratory tract symptoms such as foul smelling nasal discharge and epistaxis. Later he developed fever, poly-arthritis, abdominal pain and haematuria. There were multiple painful oral ulcers and skin showed multiple palpable purpuric rash. C-ANCA was positive. He was treated with IV methyl prednisolone 1gm daily for 3 days followed by oral prednisolone 1mg/kg body weight and oral cyclophosphamide. His condition improved dramatically and on follow up after 3 months he was reasonably well. In this report, we wanted to emphasize that Wegener's granulomatosis, although rare, should be considered in the above clinical scenario and treatment should be initiated as soon as possible.

Cough in systemic lupus erythematosus.

This observational study was conducted in the lupus clinic of Department of Medicine, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from January 2007 to July 2007. Thirty-six consecutive SLE patients fulfilled American College of Rheumatology (ACR) diagnostic criteria and having cough for any duration was enrolled. Equal number of age and sex matched controls (The patient attending in the out patient department in the Medicine having cough without SLE) was taken after having informed consent. Severely disabled cases and cases unwilling to participate with a cough were excluded. Mean age of study population was 29.1±8.7 years and 29.8±9.9 years in controls. Duration of cough was less than 3 weeks in 17% of SLE patients and 8% of control group and was more than three weeks in 83% and 92% respectively. In SLE patient commonest causes of cough was bronchial asthma 47% and was 33% in controls. Post nasal drip syndrome was the next common causes and frequently due to sinusitis and allergic rhinitis. Its frequency was 14% and 16% in SLE and control groups respectively. Other causes were pneumonia (11%, 14%), Diffuse Parenchymal Disease lung (DPLD) (5.6% & 2.8%), and tuberculosis (5.6% & 8%) in SLE & control groups respectively. Gastro esophageal reflux disease (GERD) was 2.6% in SLE group but was not found in control group. Cough is common in SLE patients. Frequency distribution though similar to that of causes of cough in community but warns the possibility of serious underlying diseases in SLE cases and attentive evaluation.

Echocardiographic assessment of cardiac involvement in systemic lupus erythematosus patients.

This study was designed to assess cardiac abnormalities in patients with systemic lupus erythematosus (SLE) by echocardiography. It was an analytic type of cross sectional study, conducted in lupus clinic, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from July 2008 to June 2009. Fifty lupus patients, diagnosed on the basis of ACR criteria, without cardiovascular symptoms, were enrolled in the study and were evaluated by standard echocardiography with color Doppler. SLEDAI was applied for assessment of disease activity. Out of 50 patients 80% had abnormal echocardiographic findings. Pericardial thickening was found in 38% patients, pericardial effusion 20%, diastolic dysfunction 72%, hypokinesia of ventricular wall 8%, overall valvular abnormalities 20%, commonest being aortic regurgitation (12%), followed by mitral regurgitation (8%), and 6% had pulmonary hypertension. Males (100%) were more vulnerable to cardiac involvement than females (68.2%) and later age of disease onset (31-40 years) was associated with higher (87.5%) chance of echo abnormalities. The differences, however, were not statistically significant (p>0.05). There was significant relationship between disease duration and cardiac abnormalities (p<0.01). Active disease (80.08%) was associated with higher frequency of cardiac involvement than disease in remission (62.50%) but the result was not statistically significant (p=0.151). Cardiac abnormalities are very common in lupus patients even when clinically asymptomatic from cardiac aspect. Echocardiography is an excellent non-invasive tool for cardiac evaluation. These observations emphasize a need for further assessment of early intervention to reduce subsequent cardiac morbidity and mortality among the lupus patients.

Comparative efficacy of subcutaneous versus oral methotrexate in active rheumatoid arthritis.

This prospective study was conducted in rheumatology clinic under the department of medicine of Bangabandhu Sheikh Mujib Medical University from December 2004 to December 2005 to asses the efficacy, safety and compliance of subcutaneous methotrexate (MTX) in active rheumatoid arthritis (RA) patients. A total of 92 active rheumatoid arthritis patients according to American College of Rheumatology (ACR) criteria were recruited for the trial for six months. Among them 46 cases belonged to injectable MTX group and 46 cases belonged to oral MTX group. Mean±SD age of patients was 45.54±12.42 vs. 44.63±13.99 years in subcutaneous group and oral group respectively. In the subcutaneous group 41 were female and 5 male; in the oral group 34 were female and 12 male. Mean duration of the disease was 49.74 months in subcutaneous group and 49 months in oral group. RA test was positive in 35 cases in both groups whereas Rose Waaler test was positive in 19 patients in subcutaneous group and 14 patients in oral group. At 24 week, response rate of ACR 20 was significantly higher in subcutaneous MTX than oral MTX group (93% vs. 80%, p=0.02). Similarly ACR 50 response was significantly higher in subcutaneous MTX than in oral group (89% vs. 72%, p=0.03). ACR 70 response was not significantly higher in SCMTX group then oral group (11% vs. 9 %, p=0.72). Adverse effects were relatively less in subcutaneous MTX and most common side effects were nausea (37% vs. 63%), vomiting (11% vs. 30%), dyspepsia (29% vs. 48%), dizziness (4l% vs. 52%) and alopecia (72% vs. 85%). The results of the study demonstrated that subcutaneous MTX was significantly more effective than oral MTX at the same dosage in active Rheumatoid arthritis patients with no increase in side effects.

Estimation of 10-Year Fracture Risk with and without Bone Mineral Density in Patients with Rheumatoid Arthritis.

Rheumatoid arthritis (RA) is the most common inflammatory arthritis affecting 0.5-1.0% of the general population worldwide and although RA is properly considered a disease of the joints, it can cause a variety of extra-articular manifestations. This study was performed to find out any discrepancy in fracture risk estimates with and without bone mineral density (BMD) in rheumatoid arthritis (RA) patients. This observational cross-sectional study was carried out in the Department of Rheumatology, Bangabandhu Sheikh Mujib Medical University (BSMMU), Bangladesh from July 2013 to July 2015. Total 65 consecutive patients with RA fulfilling ACR/EULAR criteria aged 40-90 year were recruited. Ten year fracture risk of these patients was evaluated by the FRAX score with and without BMD and differences were observed. FRAX score without BMD revealed that major fracture risk was low in 58(89.2%) patients, moderate in 7(10.8%) patients but re-estimation with BMD revealed that 55(84.6%) patients remained in low risk group, 8(12.3%) patients in moderate risk group and 2(3.1%) patients went to the high risk group. In case of hip fracture risk without BMD, risk was low in 58(89.2%) patients, high in 7(10.8%) patients; but with BMD, 50(76.9%) patients remained in low risk group but risk of 15(23.1%) patients became high. Almost all the high risk patients (93.3%) were ≥55 years of age. Increasing age, female sex, disease duration and use of steroid were positively correlated with increased FRAX score where as high BMI and high BMD were associated with low FRAX score. But in multivariate analysis it was found that only relation with age was at statistically significant level. Significant numbers of patients with rheumatoid arthritis have high risk of fracture especially hip fracture. The mean of FRAX score increased in both major & hip osteoporotic fracture risk after adding BMD. More than half of the patients above fifty five years or more had high risk of fracture. So, BMD should be done in patients aged more than fifty five.

Tuberculosis of knee joint and tubercular pyomyositis of gastrocnemius muscle.

Extra-spinal musculoskeletal tuberculosis (TB) is rare and tubercular pyomyositis is extremely rare. Tuberculosis of the knee-joint is a haematogenous infection secondary to a focus of active disease elsewhere in the body which may not be found. Tubercular pyomyositis usually caused by invasion from the adjacent structures rather than a secondary spread. Here we describe a 40 years old male patient who presented to us with pain in the right knee joint for one and half years and pain and swelling of right calf muscle for fifteen days. He was eventually diagnosed as a case of tuberculosis of the right knee joint and tubercular pyomyositis of right gastrocnemius muscle on the basis of fine needle aspiration from the right calf that showed caseation necrosis with clusters of epithelial cells despite absence of systemic symptoms, the absence of other foci of active tuberculosis and a normal chest radiograph.

In silico analysis of Naegleria fowleri cathepsin B paralogs: important drug targets.

Naegleria fowleri is a deadly human pathogen that causes primary amoebic meningoencephalitis (PAM). In this study, in silico investigations of two important N. fowleri cathepsin B paralogs, i.e., copies of genes resulting from a gene duplication event, were carried out using comparative modeling and molecular dynamics (MD) simulations. Comparative models of both paralogs showed significant architectural similarity with their template, i.e., rat cathepsin B. However, in N. fowleri cathepsin B (UniProt ID: X5D761) and putative cathepsin B (UniProt ID: M1HE19) enzymes, eleven and fifteen residues in the occluding loop regions were deleted, respectively, suggesting that these enzymes have a short occluding loop. Thus, it is concluded that N. fowleri cathepsin B and putative cathepsin B enzymes lack exopeptidase activity but possess enhanced endopeptidase activity and an affinity for macromolecular inhibitors. MD simulations further confirmed that prosegments (macromolecular inhibitors) bond more tightly with both enzymes than with wild-type cathepsin B. Additionally, a mutation was identified at an important N-glycosylation site; this mutation is believed to affect cathepsin B targeting inside the cell and make cathepsin B available in the extracellular environment. Due to this important N-glycosylation site mutation, these enzymes are secreted in the extracellular environment via an alternative, still unknown, posttranslational processing strategy. The present study is the first to predict the three-dimensional folds of N. fowleri cathepsin B paralogous enzymes, including a detailed description of the active site architecture and information about propeptide binding mode. This information can contribute to the discovery of novel and selective treatments that are effective against N. fowleri.

Polyarthritis is a Rare Manifestation of Pachydermoperiostosis: A Case Report.

Pachydermoperiostosis or primary hypertrophic osteoarthropathy, also known as Touraine-Solente-Gole syndrome, is a rare process, frequently inherited. In its complete form it is characterized by pachydermia (thickening of the skin), skeletal changes (periostosis) and acropachia (digital clubbing). Diagnosis can be made considering the typical clinical features and the histological feature. We report a patient of 25-year old man presented with joint pain involving in multiple joints for last 7 years and progressive enlargement of his hands and feet with profuse sweating of palms and soles for last 4 years. Physical examination revealed thickened skin with excessive furrowing of his forehead, dropping of both eyelids, clubbing of all fingers, toes and enlargement of his hands and feet with pamoplantar hyperhidrosis. Laboratory investigation shows raised CRP, X-ray feet lateral view showed normal heel pad thickness, new bone formation and periosteal elevation in lower end of tibia and fibula and skin biopsy showed dense fibrocollagenous tissue in dermis and subcutis and mild acanthosis. With this scenario he was diagnosed as primary hypertrophic osteoarthropathy (Pachydermoperiostosis). After diagnosis he was treated with cholchicine (0.6mg) twice daily, naproxen (500mg) twice daily, and risedronate (150mg) monthly. After one year his skin texture became less thickened, joint pain improved, there was no further enlargement of acral part of fore arm. In this report we review the characteristic features of this syndrome. We highlight the importance of ruling out secondary forms of hypertrophic osteoarthropathy and of a close follow-up of these patients because of complications that might develop on long-term. Although no treatment was satisfactory, we wanted to emphasize that NSAIDs, Cholchicine and risedronate could be an effective treatment option.

Placebo controlled trial of methotrexate in systemic sclerosis.

Eighteen adult patients of systemic sclerosis were included in this prospective study from Rheumatology Clinic of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka during the period of July 1997 to December 1999 to observe the effectiveness of treatment with methotrexate (MTX) versus placebo. Among the eighteen patients 9 patients were randomly assigned to MTX and 9 to placebo therapy. Nine patients were dropped out (6 in placebo and 3 in the MTX group), because of toxicity and non-compliance. Clinical improvement following treatment was observed in 33.33% of the patient in MTX group but none in placebo group, but this difference was not statistically significant. Anorexia, nausea and occasional vomiting were common side effects in MTX group and subsided in most cases with the passage of time despite the continuation of therapy.

Efficacy of ciprofloxacin in enteric fever: comparison of treatment duration in sensitive and multidrug-resistant Salmonella.

The efficacy of two regimens of ciprofloxacin was compared in a randomized study conducted on 69 patients with enteric fever, 52.2% of whom had infection with multidrug-resistant (MDR) strains of Salmonella typhi or S. paratyphi. Patients were randomly assigned to two regimens (10 days versus 14 days) of ciprofloxacin (500 mg twice a day). The mean +/- SD time required for defervescence was similar for both regimens (4.2 +/- 1.9 days in the 10-day group and 4.9 +/- 2.6 days in the 14-day group). A 100% cure was observed in each treatment group and no serious side effects were observed. Relapse occurred in two patients (14-day regimen). Only one patient (14-day regimen) had growth of S. typhi in stool culture at the time of the first follow-up three days after completion of therapy. Follow-up studies on available patients on two, six, and 12 months after completion of therapy revealed that all patients had negative stool cultures for S. typhi and S. paratyphi. This study indicates that ciprofloxacin may be recommended as an initial therapy for enteric fever for adult men and nonpregnant and nonlactating women in areas where MDR strains of S. typhi and S. paratyphi are prevalent, and that 500 mg twice a day of the drug given for 10 days is as effective as 14 days at the same dosage.

Preliminary experience with continuous ambulatory peritoneal dialysis.

Continuous ambulatory peritoneal dialysis (CAPD) was employed in seven patients of endstage renal failure (ESRF). All the patients had symptom-free ambulant life during CAPD. Blood pressure was well controlled and the haemoglobin level improved significantly. Blood urea and serum creatinine levels were maintained near normal levels. Metabolic acidosis was corrected. Recurrent peritonitis, heavy protein loss and catheter failure were the main complications of CAPD. It appears from these observations that CAPD may be helpful in patients with ESRF.

Sucralfate in the prevention of duodenal ulcer relapse and factors influencing the relapse rate.

Forty three subjects with DU confirmed at endoscopy and healed after eight weeks of sucralfate therapy were subjected to a randomized double-blind controlled trial for six months. Twenty one subjects received sucralfate (1 g one hour before breakfast and dinner). Twenty two subjects received placebo. Symptoms were evaluated every month. The subjects were endoscoped at the end of the trial or earlier in case symptoms recurred. Sucralfate was found to be significantly more effective than placebo (6/21 vs. 17/22, p < 0.005) in preventing DU relapse. Age at presentation, age at onset, duration of illness, sex, periodicity, smoking, gastric stasis like symptoms, associated irritable bowel syndrome, degree of deformity of the bulb and initial presence of duodenitis were the factors examined for their effect upon the relapse. In the placebo group relapsers had significantly shorter mean duration of illness indicating that DU may relapse more frequently in earlier part of its natural course. Other factors did not influence the relapse rate. In the sucralfate group, higher ages at onset and at presentation were associated with significantly higher relapse rate. Sucralfate may be less effective in preventing relapse in elderly and late onset DU patients.

Factors influencing development of bulbar deformity in duodenal ulcer disease.

Sixty subjects were carefully interrogated and repeatedly endoscoped with a view to studying the effect of sucralfate on healing and recurrence of duodenal ulcer disease. Degree of bulbar deformity was noted during each endoscopy. During the trial it appeared that deformity progressed more rapidly in late onset subjects (onset at or above 30 years of age). In eight subjects deformity became gross over a period of three years. Seven of them belonged to the late onset subgroup. Two subject had large ulcers and both belonged to the late onset subgroup, one had rapidly progressive disease. Out of six subjects with multiple ulcers four belonged to the late onset subgroup, two of the later had rapidly progressive disease.

Etiology and clinical profile of hepatocellular carcinoma in Bangladesh.

Sixty-four consecutive subjects with hepatocellular carcinoma were prospectively studied in the department of Hepatology, IPGMR, Dhaka. Their mean age was 50.11 years. Fifty-two were male and 12 female. Cirrhosis was present in 12 (18.75%) subjects. Thirty subjects (46.88%) had HBsAg in their sera. Seven (58.33% of females) patients gave history of use of oral contraceptives. Cirrhosis, HBV infection, male sex, middle age, and probably the use of oral contraceptives in females appeared to be important risk factors for development of HCC in Bangladesh. Majority of patients presented with upper abdominal pain, weight loss and anorexia. Hepatomegaly was invariably present. Alpha fetoprotein was significantly higher in cirrhotic HCC patients than in non-cirrhotic ones. Median survival was two months. None of the clinical or laboratory features predicted the prognosis.

Predictors of duodenal ulcer healing in sucralfate treated patients.

The objective of the study was to assess the efficacy of sucralfate in promoting duodenal ulcer healing and to assess the value of some variables in predicting outcome of such therapy. Following variables were tested for predicting the outcome: age at onset, age at presentation, duration of symptoms, sex, periodicity, smoking, nocturnal pain, relief by food, relief by antacid, gastric stasis like symptoms, associated irritable bowel syndrome, site, size and number of ulcers and degree of deformity of bulb. Sixty patients with uncomplicated DU confirmed at endoscopy were treated with sucralfate one gram before three major meals and 1 g at bedtime for two months. Endoscopy was repeated at the end of the trial. There were four drop-outs. Complete, partial and no healing occurred in 45 (80.36)%, 3 (5.36%) and 8 (14.28%) subjects. Ulcer healing rate was higher in those without gross bulbar deformity (41/46) than in those with gross deformity (4/10), (Odd's ratio 12.3, 95% ci 1.98 to 78.44). Other variables were not found to be associated with ulcer healing.

Value of clinical features in the diagnosis of enteric fever.

There is no objective data on the value of individual clinical symptoms or signs in the diagnosis of enteric fever in a febrile patient. The purpose of the study was to assess the value of some clinical and simple laboratory features in the diagnosis of enteric fever. One hundred & six patients with microbiologically confirmed enteric fever and 170 patients with other established febrile illnesses were included in the evaluation. History of stepladder pattern of rise of temperature, loose motions, relative bradycardia and coated tongue proved to be powerful markers of enteric fever with high specificity (100%, 94.71%, 94.71%, 94.12% respectively), positive and negative predictive values. Headache, hepatomegaly and splenomegaly were moderately powerful. ESR and WBC count appeared to have little value in the diagnosis of enteric fever. Pattern of onset and loose motions did not discriminate between typhoid and paratyphoid fever. Most of these patients had illness persisting beyond one week by which viral infections and infectious enterocolitides were largely excluded. Elucidation of power of these markers in distinguishing enteric fever from other febrile illnesses with the help of better designed prospective studies would lessen our dependence on expensive and time consuming laboratory investigations.

Efficacy of methotrexate in rheumatoid arthritis.

Rheumatoid arthritis is a common inflammatory articular disorder in Bangladesh. Methotrexate has proved to be an effective and relatively safe disease modifying drug for this disease. A quasiexperimental trial of the efficacy of methotrexate in rheumatoid arthritis was carried out in the Rheumatology Clinic, Institute of Postgraduate Medicine & Research, Dhaka during the period between July 1992 and September 1993. Thirty eight patients fulfilling the revised ARA criteria were given methotrexate in a total weekly dose of 7.5 to 15 mg. They were followed up at weekly intervals for one month and then monthly for a total duration of six months. Twenty three subjects eventually completed the trial. The trial showed significant differences in the disease activity indices at the end of six months. The decline of activity was noted at the end of one month. As a whole the response was complete in 4(17%), marked in 14(61%), moderate in 4(17%) and nil in 1(4%). Adverse effects occurred in 27 subjects. They were mild and transient in 22. Methotrexate appeared to be an acceptable DMARD for our rheumatoid arthritis population.

Efficacy of sulphasalazine plus methotrexate in rheumatoid arthritis.

Early intervention with slow acting anti-rheumatic drugs (SAARDs) has led to improvement in substantial proportion of rheumatoid arthritis (RA) patients. The present open, controlled study was designed to assess whether a combination of SAARDs offer any added benefit. Fifty-four adult RA patients were randomly allocated to methotrexate (MTX) (n = 27) and MTX plus sulphasalazine (SSZ) (n = 27) groups. The subjects were followed-up fortnightly for four weeks then monthly for six months. The disease activity was assessed with the help of 10 clinical and four laboratory indices. The improvement was graded as: minor, mild decreases in indices, non-steroidal anti-inflammatory drugs (NSAIDs) continued, physician's global assessment (PGA) decreased by one; marked, acceptable decreases in indices, NSAIDs being taken sparingly, PGA decreased by at least 2, and complete, all indices normalised and patients discontinued NSAIDs completely. The improvement was considered clinically important when marked or complete improvement occurred. Adverse drug reactions resulted in withdrawal of 4 subjects from the MTX + SSZ group and 1 from the control groups. Four and three subjects in the combined and MTX groups respectively were lost to follow-up. Subjects in both groups showed significant decline in all indices except hemoglobin and neutrophil count. The differences between the two groups in the pre-treatment and post-treatment values were insignificant. Complete, marked, minor and no improvement occurred in 4 (21%), 12 (63%), 3 (16%) & 0 in the MTX and in 11 (48%), 7 (30%), 4 (17%) & 1 (4%) in MTX + SSZ groups respectively. The differences in the rates of complete and clinically important improvement between the two groups were insignificant (P 0.1398 and 0.7092). The incidence of side effects was insignificantly higher in the MTX + SSZ group. Most of them were mild and transient. The combination of SAARDs offered little added advantage in RA. However, the higher rate of complete improvement in the combination group justifies trials including larger samples.