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Gastric volvulus is a rare surgical emergency which mostly presents in children and occasionally in adults. It results due to pathological malrotaion of the stomach along its longitudinal or short axis. The condition, if not treated promptly, can result in increased morbidity and caries a high risk of death. Very few cases have been reported in literature regards this important clinical condition. Early decompression and repair of anatomical defects are the corner stone of its management. There is a dire need to develop guidelines and algorithms for management and treatment of this rare condition to improve patient outcome, prevent recurrence and facilitate early diagnosis by practicing physicians and surgeons. We report three such cases which presented to our setup in emergency along with a brief description of how they were successfully managed. Keywords: Gastric Volvulus, Malrotation, Vomiting, Continuous...

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.

Background Nocturnal enuresis (NE) is a common symptom in children worldwide. International Children’s Continence Society (ICCS) defines enuresis as either mono-symptomatic, NE with lower urinary tract symptoms and NE with co-morbid conditions. The objectives of this study were to determine the frequencies and types of NE and associated symptoms and conditions in children aged 5 to 16 years based on ICCS criteria. Methods A multi-center cross sectional study was conducted between November 2012 and December 2013 in the primary care clinics of four hospitals in Karachi. Children aged five to fifteen years were included through consecutive sampling. Informed consent was obtained from the parents and a pre-coded semi-structured questionnaire was used to obtain the information. Data was entered on SPSS version 20.0 and multivariable logistic regression analysis was used for data analysis. Results Out of 429 children aged between five and sixteen years, 243(56.9%) were boys and the remaining 186(43.1%) were girls. One hundred and eighty three children (43%) had nocturnal enuresis (NE). Forty four (10.3%), had mono-symptomatic NE, 57(31.1%) had associated lower urinary tract symptoms (NE-LUTS), whereas 30 (16.3%) had NE with a co-morbid condition. Fifty two (28.4%) NE’s had at least one of both LUTS and a co-morbid condition. Out of the 246(57%) non-enuretic’s, 31(12.6%) had a LUTS, 95(38.6%) had a co-morbid condition and 57(23.2%) had at least one of both LUTS and a co-morbid condition. The remaining 63 (25.6%) were symptom free. Increased voiding frequency, urgency, dysuria, suprapubic pain and daytime incontinence were the LUTS significantly associated with NE. Co-morbid conditions significantly associated with NE included constipation, congenital defects, developmental delay, and learning and sleep problems. Conclusion Although NE can be an only symptom, it is often associated with lower urinary tract symptoms like dysuria, urgency, suprapubic pain, and daytime incontinence. Children presenting with NE often have co-morbid conditions like constipation, urinary tract infection, sleep disorders, and developmental delay. Many children presenting with these conditions as the primary complaint may also have NE. It should be addressed as unrecognized and untreated NE can cause additional morbidity and distress.

Introduction Sarcoidosis is an uncommon multi-system disorder with many possible complications. Arteriovenous malformations (AVMs) are a rare vascular complication of sarcoidosis. Case description A 15-year-old girl presented to the Pediatric Clinic at AKUH with pulmonary, hepatic, joint, and skin manifestations. Physical examination and investigations pointed toward sarcoidosis, including raised erythrocyte sedimentation rate, angiotensin converting enzyme (ACE), and alanine transaminase (ALT). An incidental finding of pulmonary arteriovenous malformation (PAVM) was noticed on echocardiography. She responded to oral corticosteroids, her ACE and ALT levels improved. There was lack of indication for pulmonary angio-embolization for her PAVM. On a 3-year follow-up, her condition improved and she is clinically well. Discussion Pulmonary arteriovenous malformation is an extremely rare complication of sarcoidosis, especially among the pediatric population. Hence, this is the first reported case of its kind. The relation between sarcoidosis and PAVM is difficult to establish; however, there are some theories. This condition may be treated depending on the symptoms. Since our patient did not have any significant symptoms of PAVM, she was treated for the underlying disease, i.e., sarcoidosis. Conclusion While dealing with patients having multi-system disorders like sarcoidosis, one must be very vigilant so as not to miss out on any complication. Regular follow-up visits should be scheduled to rule out new complications and to monitor the past ones.

Aim: Diabetes is an emerging health problem, both in developing and developed countries and has an enormous economic and social impact. The objective of our study was to find the impact of insulin pump on the quality of life of patients with type 2 diabetes (T2D) and compare it to the quality of life of patients with T2D using an insulin pen. Subjects and Methods: This is a case–control study which was conducted among patients with T2D presenting between November 2014 and November 2015. A total of 83 patients with T2D, using insulin pump were enrolled in the study as cases and 322 patients with T2D not using insulin pump but using insulin pens were enrolled as controls. Short form-36 quality of life questionnaire was used for data collection. Results: Mean age of patients using insulin pump was 52.49 ± 9.28 while the mean age of patients not using insulin pump was 54.72 ± 16.87. Mean score of all domains in the questionnaire was found to be higher in patients using insulin pump as compared to patients not using insulin pumps (P < 0.05). In 81.1% of the patients, the insulin pump decreased the frequency of hypoglycemic episodes. Conclusion: Insulin pump has significantly improved the quality of life of patients in terms of better self-esteem, decreased stress, and better mood. It has resulted in improved physical health, meal time flexibility, and ease of travel. It allows patient to have more active participation in social and recreational activities improving their personal and family life.