Juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is an important subtype of dermatomyositis characterized by inflammation of muscle, skin and gastrointestinal tract. A 14-year-old girl, with a history of fever, joint pain, easy fatigability and a rash since the age of 3 years is described. Physical examination, laboratory evaluation, electromyography (EMG) and muscle biopsy were suggestive of a chronic inflammatory process involving the muscles, most likely dermatomyositis. The report highlights the importance of a muscle biopsy as the gold standard for diagnosing dermatomyositis

Clinical and biochemical characteristics of children with juvenile idiopathic arthritis

Objective: To determine the clinical and biochemical characteristics of children with Juvenile Idiopathic Arthritis (JIA) at a tertiary care centre in Karachi, Pakistan. Study Design: A descriptive study. Place and Duration of Study: Paediatric Rheumatology Clinic of The Aga Khan University Hospital (AKUH), Karachi, from January 2008 to December 2011. Methodology: Clinical and laboratory profile and outcome of children less than 15 years of age attending the Paediatric Rheumatology Clinic of the Aga Khan University, Karachi with the diagnosis of Juvenile Idiopathic Arthritis according to International League against Rheumatism were studied. These children were classified into different types of JIA; their clinical and laboratory characteristics, response to therapy and outcome was evaluated. Results: Sixty eight patients satisfying the criteria of International League against Rheumatism (ILAR) for Juvenile Idiopathic Arthritis were enrolled during the study period of four consecutive years, their age ranged from 9 months to 15 years. Mean age at onset was 6.45 ± 4.03 years while mean age at diagnosis was 7.60 ± 3.93 years. Polyarticular was the most predominant subtype with 37 (54%) patients, out of these, 9 (24%) were rheumatoid factor positive. An almost equal gender predisposition was observed. Fever and arthritis were the most common presenting symptoms, with only 2 patients presenting with uveitis. Conclusion: The clinico-biochemical characteristics of JIA at the study centre showed a pattern distinct with early onset of disease, high frequency of polyarticular type and a higher rheumatoid factor (QRA) and ANA positivity in girls

A study protocol : using demand-side financing to meet the birth spacing needs of the underserved in Punjab Province in Pakistan

Background: High fertility rates, unwanted pregnancies, low modern contraceptive prevalence and a huge unmet need for contraception adversely affect women's health in Pakistan and this problem is compounded by limited access to reliable information and quality services regarding birth spacing especially in rural and underserved areas. This paper presents a study protocol that describes an evaluation of a demand-side financing (DSF) voucher approach which aims to increase the uptake of modern contraception among women of the lowest two wealth quintiles in Punjab Province, Pakistan. Methods/Design: This study will use quasi-experimental design with control arm and be implemented in: six government clinics from the Population Welfare Department; 24 social franchise facilities branded as `Suraj' (Sun), led by Marie Stopes Society (a local non-governmental organization); and 12 private sector clinics in Chakwal, Mianwali and Bhakkar districts. The study respondents will be interviewed at baseline and endline subject to voluntary acceptance and medical eligibility. In addition, health service data will record each client visit during the study period. Discussion: The study will examine the impact of vouchers in terms of increasing the uptake of modern contraception by engaging private and public sector service providers (mid-level and medical doctors). If found effective, this approach can be a viable solution to satisfying the current demand and meeting the unmet need for contraception, particularly among the poorest socio-economic group

Frequency of Carpal Tunnel Syndrome and Related Functional Status in Computer Operators

Objective: To find out the frequency of Carpal Tunnel Syndrome (CTS) and associated functional limitations in computer operators.   Materials &Methods: This cross-sectional study was conducted in the premises of DUHS, Ojha campus, Karachi. The duration of study is from 1st Dec 2019 to 29th February 2020.Boston Carpal Tunnel Syndrome Questionnaire (BCTQ) was used to evaluate the frequency of CTS in computer users, total sample size taken was 95, data was collected in the premises of DUHS, Ojha campus including both males and females employs between age group of 25 to 50 years that were working on the current job for more than 2 years, members that were <25 or >50 years were excluded from the study. Results: The study indicated possible association of Symptom severity and functional limitations with respect to age. The frequency of CTS symptoms with moderate to severe severity was 17.9% and 35.8% respectively whereas 46.3% reported mild symptoms. According to functional status scale 55.8% had mild functional limitations whereas moderate to severe difficulty in performing day to day activities was found 21.1% and 23.0% respectively.   Conclusion: This study survey indicated that computer using is associated with the development of CTS symptoms. It also results in the increasing the severity of the symptoms, causing prominent functional limitations that interferes with proper working of employs.   Keywords: carpal tunnel syndrome, frequency, computer users, functional limitations.   &nbsp

Sclerosing Mesenteritis as a Cause of Abdominal Mass and Discomfort in an Elderly Patient: A Case Report and Literature Review

Sclerosing mesenteritis is a rare benign process that involves inflammation, fat necrosis, and fibrosis of the mesentery. The disease poses great diagnostic challenge due to its nonspecific clinical and diagnostic findings. We report the case of a 75-year-old man who presented with vague abdominal discomfort associated with an intra-abdominal mass. With suspicion of a bowel carcinoid tumor on computed tomography scans, the patient underwent diagnostic laparoscopy. A diagnosis of sclerosing mesenteritis was made on histological examination. The patient's symptoms responded to a combination of immunosuppressive drugs, with no interval change in the size of the mass on radiological examination after fifteen months

Bilateral Moyamoya Disease in a 2-Year-Old Pakistani Male Treated with Bilateral Encephaloduroarteriosynangiosis: A Positive Outcome.

Background. We present a rare case of bilateral moyamoya disease presenting as multiple strokes and neurological deficits, treated with the neurosurgical procedure, encephaloduroarteriosynangiosis (EDAS), in a 2-year-old male Pakistani minor. A positive outcome was achieved and the patient recovered fully. Case Summary. Our patient presented with a history of seizures and multiple episodes of hemiparesis (on and off weakness) at the age of 2 years. He had a delayed speech development and could not speak more than a few words. He had a slight slurring of speech too. He was diagnosed with bilateral moyamoya disease on Computed Tomography Angiography (CTA). Bilateral EDAS was done in the same year, after which his symptoms improved and patient had moderate functional recovery. Conclusion. A rare disease, moyamoya has been left unexplored in Pakistan; physicians and surgeons when dealing with cases in the pediatric population presenting with symptoms of stroke, signs of generalized weakness, and seizures should consider moyamoya disease as a possibility. Furthermore, this case demonstrates the effectiveness of EDAS procedure for the treatment of moyamoya disease

Determinants of method switching among social franchise clients who discontinued the use of intrauterine contraceptive device

Introduction: Women who do not switch to alternatemethods after contraceptive discontinuation, for reasons other than the desire to get pregnant or not needing it, are at obvious risk for unplanned pregnancies or unwanted births. This paper examines the factors that influence women to switch from Intrauterine Contraceptive Device (IUCD) to other methods instead of terminating contraceptive usage altogether. Methods: The data used for this study comes from a larger cross-sectional survey conducted in nine (9) randomly selected districts of Sindh and Punjab provinces of Pakistan, during January 2011. Using Stata 11.2, we analyzed data on 333 women, who reported the removal of IUCDs due to reasons other than the desire to get pregnant. Results: We found that 39.9% of the women do not switch to another method of contraception within one month after IUCD discontinuation. Use of contraception before IUCD insertion increases the odds for method switching by 2.26 times after removal. Similarly, postremoval follow-up by community health worker doubles (OR = 2.0) the chances of method switching. Compared with women who received free IUCD service (via voucher scheme), the method switching is 2.01 times higher among women who had paid for IUCD insertion. Conclusion: To increase the likelihood of method switching among IUCD discontinuers this study emphasizes the need for postremoval client counseling, follow-up by healthcare provider, improved choices to a wider range of contraceptives for poor clients, and user satisfaction

Childhood-Onset systemic lupus erythematosus: a cohort study

Objective: To determine the clinical and immunological characteristics and short-term outcome of children with systemic lupus erythematosus (SLE).Study Design: A descriptive cohort study.Place and Duration of Study: Paediatric Rheumatology Clinic, The Aga Khan University Hospital, Karachi, from January 2011 to December 2015.Methodology: Clinical and immunological profile and short-term outcome of children less than 16 years of age admitted in the paediatric ward, with the diagnosis of SLE was studied. Demographic data, clinical presentation, laboratory findings, immunological profile and treatment regimens of these children were evaluated.Results: Thirty-two children, satisfying the criteria of American College of Rheumatology (ACR) for SLE, were enrolled during the study period of five consecutive years. A female predominance was observed with 28 (87.5%) patients being female (F:M 7:1). Mean age at symptom onset was 10.5 +2.7 years; and 8.8 +2.1 years in females and males, respectively. The mean age at diagnosis was 11.3 +2.8 years in females and 9.4 +1.9 years in males. Prolonged fever was the most common non-specific symptom found in 27 (84%), followed by pallor in 13 (41%) patients. Twenty-two (69%) children were found to be anemic and 18 patients (56%) having signs of arthritis at presentation. Renal involvement was observed in 15 (47%) patients. The most common laboratory finding was anemia, found in 22 (69%) of cases. The most common immunological markers were serum anti-neutrophil antibodies (ANA), positive in 28 (88%) patients, followed by anti double-stranded DNA antibodies, raised in 26 (81%) of cases. Out of 32, 12 patients were lost to follow-up. Of the remaining 20 children who were followed for four years, ten (50%) went into remission.Conclusion: Childhood-onset SLE encompasses a wide variety of manifestations with a female preponderance. Fever, arthralgia and pallor are the most frequent clinical manifestations among the children. Hemolytic anemia (HA) is the most common laboratory abnormality, with ANA and anti ds-DNA antibodies positivity in the majority of padiatric patients