Spectrum of joint deformities in children with juvenile idiopathic arthritis

Objective: To determine the frequency and types of joint deformities in children with juvenile idiopathic arthritis and their association with clinical parameters and rheumatoid factor

Study Design: Cross-sectional study

Place and Duration of Study: Rheumatology Outpatient Clinic, the Children's Hospital and the Institute of Child Health, Lahore, from September 2014 to February 2015

Methodology: All patients of both genders of less than 16 years of age, who fulfilled the International League of Association for Rheumatology [ILAR] criteria for Juvenile Idiopathic Arthritis [JIA], were enrolled in this study. Their demographic data, duration of disease at the time of presentation, types of JIA, various joint deformities and rheumatoid factor [RF] were documented. Statistical analysis of data was done on SPSS version 16. Chi-square test was applied to determine the association of clinical deformity with age of patients, disease duration at presentation, types of JIA and RF

Results: Out of 70 patients enrolled during the study period, 51.4% were boys with mean age at presentation being 9.44 +/-3.89 years [2-7 years] and median duration of disease being 24 months [interquartile range 42 months]. Forty patients [57.1%] had joint deformities. Most common joints involved were hand [50%], wrist [50%], and knee [35.7%]. The common types of joint deformities were boutonniere deformity [28.6%], ulnar deviation of wrist [28.6%], fixed flexion deformity of wrist [22.9%], and knee [31.4%]. The most common type of JIA was polyarthritis RF negative with or without deformity. There was a strong association of deformities with older age of patients at presentation [p=0.036], longer duration of disease at presentation [p=0.028], polyarthritis [RF seronegative / seropositive] [p=0.013], and seropositivity [p=0.04]

Conclusion: More than 50% patients with JIA have joint deformities. Joint deformities are more likely to be seen in children with long-standing disease, those with polyarthritis JIA and seropositive patients

Psittacosis: rare respiratory problem in children

Psittacosis is a rare disease particularly in children with usual presentation of respiratory and constitutional symptoms. The cases may remain undiagnosed or diagnosis may be delayed because of lack of awareness among the paediatricians and physicians. Early diagnosis is very important as this is potentially curable and preventable disease. An interesting case of psittacosis is being reported here, which has been treated successfully with azithromycin

Fibrodysplasia ossificans progressiva

Fibrodysplasia Ossificans Progressiva [FOP] is a rare autosomal dominant disorder characterized by postnatal progressive heterotopic ossification of connective tissue and congenital malformation of big toes. We report a 3-year male toddler with clinical and radiological features of FOP. He was born with bilateral hallux valgus and at the age of 3 years presented with hard swellings over back, scapular region and forehead that were initially inflammatory and then became bony hard. There is also tilting of neck towards the left due to calcification in neck region. The radiographs showed heterotopic ossification in thoracic region, neck, spine and region of hip joint

Juvenile rheumatoid arthritis

To determine the spectrum of clinical presentation, laboratory parameters and drug therapy in patients with Juvenile Rheumatoid Arthritis [JRA]. Case series. The Children's Hospital and The Institute of Child Health, Lahore, from October 2008 to October 2011. All patients who fulfilled the American College of Rheumatology criteria for JRA were enrolled. Their clinical features, investigations done and treatment received for JRA were noted. Statistical analysis of data was done on SPSS version 16.0 for obtaining descriptive statistics. Out of 185 patients, 50.3% [n = 93] were females; 54% [n = 100] were between 10 - 15 years of age. Polyarthritis was found in 71.9% [n = 133] followed by oligoarthritis [22.7%, n = 42] and systemic onset disease [5.4%, n = 10]. Morning stiffness [78%] and fever [68%] were the most common clinical presentations. All patients with systemic onset disease had fever [n = 10] followed by skin rash, hepatosplenomegaly and lymphadenopathy. Uveitis was found in 2 patients, and both belonged to the oligoarticular group. Rheumatoid factor was found in 10.27% [n = 19] of all patients. All patients were given non-steroidal anti-inflammatory drugs [NSAIDs]. Disease modifying agents [methotrexate] were given to 43.8% [n = 81]. Steroids were used in 61% [n = 113] of patients either with NSAIDs alone or NSAIDs plus methotrexate. Disease profile of JRA at the study centre showed that polyarthritis is the commonest type. Recognition of subtypes will help in planning the management of these patients

Pleural empyema in children: prolonged clinical course

Objective: To assess the hypothesis that empyema thoracis is a problem which is often not optimally treated and to analyze evolving experience in clinical presentation, management and outcome in thoracic empyema

Design: Prospective observational descriptive study

Place and Duration of study: Department of Pediatric Medicine of Children's Hospital and the ICH Lahore from February 2008 to October 2008

Patients and Methods: Out of these 50 children 29 [58%] were males and 21 [42%] were females. Age range was from 06 months to 14 years. The mean duration of illness prior to hospital admission was 18 days. Ultrasonography was utilized in the diagnosis of all patients. Chest CT scan was done in 16 [32%] patients. Pleural fluid culture was positive in only 6 [12%] patients. Staph aureus was the predominating organism 4 [8%]. All patients were treated with intra venous antibiotics and tube thoracostomy. Antituberculous medication was given to 6 [12%] of patients. 29 [58%] patients developed complications in the form of localized collection 12[24%], septations 8 [16%], bronchopleural fistula 7 [14%], pneumothorax 1 [2%] and hydropneumothorax 1 [2%]. 12 [24%] patients were shifted to surgery department for decortication. In this study, duration of illness before visiting hospital was significantly associated with complications [p=0.04]

Conclusion: Empyema associated with community-acquired pneumonia is an important cause of morbidity in children. Para pneumonic effusion should be treated aggressively by physicians. Early drainage and proper antibiotics are recommended to prevent progression to late stage and serious complications, which in turn subjects the patient to a major surgery

Scurvy: presenting as musculoskeletal pain

The incidence of scurvy in the paediatric population is very low. This report describes a case of scurvy in toddler girl who was admitted with severe pain in legs and difficulty in walking along with bleeding from gums. After extensive investigation, she was found to have vitamin C deficiency resulting from restrictive eating pattern. There was significant improvement in presenting symptoms and general health after introduction of vitamin C and fresh fruits and vegetables to her diet. Nutrition-based diseases can still occur in any socioeconomic setting. Nutritional screening remains an important part in every child's general health care