Atorvastatin ameliorates viral burden and neural stem/ progenitor cell (NSPC) death in an experimental model of Japanese encephalitis

Japanese encephalitis virus, a neurotropic flavivirus, causes sporadic encephalitis with nearly 25% fatal casereports. JEV infects neural stem/progenitor cells (NSPCs) and decreases their proliferation. Statin, a commonlyused class of cholesterol lowering drug, has been shown to possess potent anti-inflammatory and neuroprotective effects in acute brain injury and chronic neurodegenerative conditions. Here, we aimed to check theefficacy of atorvastatin in alleviating the symptoms of Japanese encephalitis (JE). Using BALB/c mouse modelof JEV infection, we observed that atorvastatin effectively reduces viral load in the subventricular zone (SVZ)of infected pups and decreases the resultant cell death. Furthermore, atorvastatin abrogates microglial activation and production of proinflammatory cyto/chemokine production post JEV infection in vivo. It alsoreduced interferon-b response in the neurogenic environs. The neuroprotective role of atorvastatin is againevident from the rescued neurosphere size and decreased cell death in vitro. It has also been observed that uponatorvastatin administration, cell cycle regulatory proteins and cell survival proteins are also restored to theirrespective expression level as observed in uninfected animals. Thus the antiviral, immunomodulatory andneuroprotective roles of atorvastatin reflect in our experimental observations. Therefore, this drug broadens apath for future therapeutic measures against JEV infection.

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

Changing survival rate of infants born before 26 gestational weeks: single-centre study

This study aimed to evaluate the changing survival rate and morbidities among infants born before 26 gestational weeks at the Sultan Qaboos University Hospital [SQUH] in Muscat, Oman. This retrospective study assessed the mortality and morbidities of all premature infants born alive at 23-26 gestational weeks at SQUH between June 2006 and May 2013. Infants referred to SQUH within 72 hours of birth during this period were also included. Electronic records were reviewed for gestational age, gender, birth weight, maternal age, mode and place of delivery, antenatal steroid administration, morbidity and outcome. The survival rate was calculated and findings were then compared with those of a previous study conducted in the same hospital from 1991 to 1998. Rates of major morbidities were also calculated. A total of 81 infants between 23-26 gestational weeks were admitted to the neonatal unit during the study period. Of these, 58.0% were male and 42.0% were female. Median gestational age was 25 weeks and mean birth weight was 770 +/- 150 g. Of the 81 infants, 49 survived. The overall survival rate was 60.5% compared to 41% reported in the previous study. Respiratory distress syndrome [100.0%], retinopathy of prematurity [51.9%], bronchopulmonary dysplasia [34.6%], intraventricular haemorrhage [30.9%] and patent ductus arteriosus [28.4%] were the most common morbidities. The overall survival rate of infants between 23-26 gestational weeks during the study period had significantly improved in comparison to that found at the same hospital from 1991 to 1998. There is a need for the long-term neurodevelopmental follow-up of premature infants

Outcome of dimon-hughston osteotomy in unstable intertrochanteric fractures

To Evaluate effectiveness of Dimon-Hughston osteotomy using a dynamic hip screw in unstable intertrochanteric fractures in term of union. Descriptive case series This was a multicenteric study conducted at Lahore General Hospital, Surriya Azeem Hospital Lahore and Civil Hospital Quetta for the period of 36 months [Between January 2012-December 2014]. Through non-probability purposive sampling, 50 cases of unstable intertrochanteric fractures were included. All fifty patients were managed by Dimon Hughston osteotomy with a 135[degree] dynamic hip screw. During the course of follow up radiographically data for union, nonunion and implant position and clinically data regarding infection was recorded. Data was analyzed by SPSS version 20. The mean age of all patients was 59.76 years [range 38-80years]. There were 28 [56%] male and 22 [44%] female patients. All patients included in study were AO type A2 fractures. Union occurred in 34 patients 68%, mean union time was 19.88 weeks [Range 18-26 weeks] nonunion and pulling out of plate occurred in 16 patients 32% during course of follow-up. Only 2 [4%] patients developed superficial infection later on which settled down with antibiotics. Dimon-Hughston osteotomy for unstable pertrochanteric fractures may provided immediate stability for early weight-bearing in some patients, but has got high failure rate

Spontaneous fragmentation of DJ stent and migration to urinary bladder

Increased use of the DJ stent in urological problems has been associated with a marked increase in DJ related complications. Spontaneous fragmentation of DJ stent is a rare complication and removal of upper fragmented part is technically difficult and frustrating. We report a case of fragmentation of DJ stent and passage of upper coiled portion from renal pelvis to urinary bladder spontaneously

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

Multiple myeloma presenting at unusual sites

To report unusual presentations of multiple myeloma and to highlight that early detection of these unusual features will encourage early investigation, diagnosis and appropriate management and consequently better prognosis of this difficult disease. Three consecutive cases of plasmacytoma reported at histopathology department of Foundation University Medical College which were later on diagnosed as multiple myeloma are presented. The clinical features and lab investigations were extracted from the patients' files. The hematoxylin and eosin stained slides were retrieved and reviewed. Three cases comprising a surgically excised chest wall mass [presternal], trucut biopsy of a mediastinal mass and a surgically excised extra dural mass along D7-D8 vertebrae were received in histopathology lab. All the patients were females. The ages of the patients were 63, 55 and 47 years respectively. On initial view of slides the diagnosis of plasma cell tumor, plasmacytoma and non-Hodgkin's lymphoma [lymphoplasmacytic type] was made respectively although the differential of large cell lymphoma with plasmacytic differentiation was also kepi in mind. Surprisingly all three cases turned out to be multiple myeloma after bone marrow aspiration, serum protein electrophoresis and skeletal x- rays. Plasmacytoma with coexistent multiple myeloma must be kept in the differential of mediastinal and vertebral masses and further lab and radiologic assessment must be done before starting the treatment.

Ventilator-associated pneumonia in children

To determine the frequency of Ventilator-Associated Pneumonia [VAP] and to identify the associated factors, causative organisms and outcome of VAP in children admitted to ICU. Cross-sectional, observational study. Medical ICU [MICU] of the Children/'s Hospital and Institute of Child Health, Lahore, from August 2008 to March 2009. All children admitted to MICU and requiring ventilation during the study period were included and monitored for any features suggestive of VAP. Partial septic screen was done in all suspected cases. VAP was labelled when any patient on the ventilator for more than 48 hours had at least 2 of the following features of nosocomial infection - fever > 101'F, TLC < 4000 or > 15000 per mm[3], neutrophils > 85%, CRP > 48 mg/L or new findings on chest examination suggestive of pneumonia' and radiological evidence of new or progressive and persistent infiltrates. Percentages were compared using chi-square test with the significance at p-value less than 0.05. Of the 93 children requiring mechanical ventilation during the study period, 16 developed VAP [17%]. Almost half [46%] were younger than 1 year with male to female ratio of 1.2:1. Children developing VAP required ventilation for 13.5 [+ 10.1] days compared to 7.7 [+ 5.5] days in those who did not develop VAP. The common organisms isolated were Pseudomonas, Klebsiella and E. coli. Factors associated with increased frequency of VAP included age less than 1 year, unplanned emergency intubation and use of continuous intravenous sedation. Features that strongly suggested underlying VAP included purulent tracheal secretions compared to increased secretions alone, CRP > 48 mg/L, positive radiological findings and positive tracheal aspirate culture. Overall mortality was 23% among the ventilated cohort. Thirty two percent of them had VAP compared to only 13% among those who survived to discharge [p = 0.03]. The frequency of VAP was 17% in this series. Factors significantly associated with VAP were age less than 1 year, unplanned intubation and continuous sedation. The important predictors of VAP included purulent tracheal secretions, high CRP and persistent new radiological findings

JAK2V617F mutation in chronic myeloid leukemia predicts early disease progression

To determine the association of JAK2V617F mutation along with BCR-ABL translocation or Philadelphia chromosome in chronic myeloid leukemia with early disease progression to advanced stages [accelerated phase or blast crisis] and poor outcome. Case series. National Institute of Blood Diseases and Bone Marrow Transplantation, Karachi, from February 2008 to August 2009. All the newly diagnosed cases of BCR-ABL or Philadelphia positive CML were tested for JAK2V617F mutation by Nested PCR. Demographic data, spleen size, hemoglobin levels, white blood cell and platelet counts were recorded. Independent sample t-test was used for age, haemoglobin level and spleen size. Fisher's exact test was applied to compare disease progression in JAK2V617F mutation positive and negative cases. Out of 45 newly diagnosed cases of CML, 40 were in chronic phase, 01 in accelerated phase and 04 in blast crisis. JAK2V617F mutation was detected in 12 [26.7%] patients; 09 [22.5%] in chronic phase, none in accelerated phase and 03 [75%] in blast crisis. During a mean follow-up of 8 months, 03 patients in chronic phase transformed in blast crisis and 02 into accelerated phase. Overall 08 out 0f 11 [73%] JAK2V617F positive patients either had advanced disease or showed disease progression. Only 2 of 20 [10%] available patients, negative for the mutation, showed disease progression by transforming into blast crisis [p < 0.001]. No statistically significant difference was seen in the age, spleen size, haemoglobin levels, white blood cells and platelets counts in JAK2V617F positive patients. JAK2V617F mutation was detected in 26.7% cases of chronic myeloid leukemia. A significant proportion of them showed early disease progression

neonate with Poland-Mobius syndrome

Poland-Mobius syndrome is a combination of two rare congenital syndromes characterized by congenital facial weakness, loss of abduction of eyes, limb defects, and absence or hypoplasia of the pectoral muscles. A patient with features of both the Mobius syndrome and the Poland syndrome is presented in this case report

Socioeconomic risk factors for asthma in child

The purpose of the study was to explore and explain different socioeconomic conditions and factors related to childhood asthma, Cross sectional descriptive study, This study was conducted at the Children's Hospital and the Institute of Child Health, Lahore for a period of three month. This is a descriptive study done at the asthma clinic of the Children's Hospital and the Institute of Child Health, Lahore. Total 110 patients were included in the study between 2-16 years, An objectively designed structured questionnaire was used and data was collected after taking consent from the parents. Out of 110 patients, the commonest age was 2-8 years for both males and females. The disease was most common in boys [63.6%] and urban area [57.3%] had higher frequency than patients from semi urban [24.5%] or rural areas [18.2%]. Asthma was more common in lower and middle class than upper. Different socioeconomic risk factors involved in the increased frequency of asthma were smoking, animals at home and environmental dust. Allergic reactions to food, change in weather and preceding upper respiratory tract infections also play an important role. Poor socioeconomic class, uneducated families, urban life and environmental allergen exposure are responsible for increase frequency of asthma and by investing resources in to community based education and support services we can provide a better control of asthma to our community

case of polyglandular autoimmune syndrome type I with unusual presentation

Eight years old girl presented with mucocutaneous candidiasis, nail dystrophy, twitching left half of face, progressively increasing generalized skin hyperpigmentation and hypopigmented patches over both shins. Her investigations revealed low intact PTH level, low serum cortisol, high ACTH, impaired glucose tolerance test and candidal onycomycosis. She was diagnosed as Polyglandular Autoimmune Syndrome [PGA] type I. She also developed idiopathic generalized epileptiform seizures and Alcaligenes faecalis septicemia not previously reported with PGA type I. The patient responded well to alphacalcidol, hydrocortisone, valproate sodium, topical antifungal and systemic antibiotic

child with roberts syndrome

Roberts syndrome Is a genetically determined rare birth defect causing, skeletal deformities, particularly symmetrical limb reduction and craniofacial anomalies. For any child with limb and craniofacial bony malformations, this syndrome should be considered in the differentials. Although this syndrome represents only a small proportion of the total number of individuals with limb deficiency, it is important to be identified in order to give accurate genetic counselling including recurrence risk in siblings and possible prenatal diagnosis. This is the case report of a 22 days old male infant who presented with defective development of all four extremities and craniofacial abnormalities. The overall clinical and radiological features were suggestive of Roberts syndrome

Relationship of joint hypermobility and musculoskeletal problems and frequency of benign joint hypermobility syndrome in children

The majority of individuals with joint hypermobility remain asymptomatic. However, those associated with Benign Joint Hypermobility Syndrome [BJHS], develop a number of systemic manifestations. Our objective was to determine the relationship between joint hypermobility and musculoskeletal problems, and frequency of BJHS in children and adolescents. This crosssectional observational descriptive study was conducted at Outpatient Department, The Children's Hospital, Lahore, Pakistan. A total of 872 individuals [4-18 year] were examined for hypermobile joints using Beighton score >/= 4. A questionnaire was implied to get data regarding demographic profile, musculoskeletal and extra-articular complaints, family history of joint problems and daily activity. Brighton's criteria were implied for diagnosis of BJHS. The frequency of joint hypermobility was 37.0%; male 39.5%, and female 34.2% [p=0.1]. There was a gradual decline in mean Beighton score with age. The female population showed increase in mean Beighton score around 16-17 year age. Arthralgias and back pains 7.7% vs. 1.6%, [p<0.001], and hernias 2.5% [p=0.03] were significantly higher in individuals with joint hypermobility. History of joint problems in the family was also significantly higher in children with joint hypermobility [p=0.01]. BJHS was detected in 4.8% children [male 3.6% and female 6.3%, p=0.06]. Arthralgias [51.0%], hernias [16.3%], joint dislocations [8.2%] and varicose veins [8.2%] were the most common presentations. BJHS is common among children. Arthralgias, back pains and hernias are significantly higher in these individuals

case of granuloma faciale

A 60-year-old housewife reported with a raised red lesion on the left cheek since the last four months. Local examination revealed a well-defined erythematous indurated plaque 3x3 cm[2] with geographical borders on the left cheek close to the angle of mouth. Routine investigations were normal. Histopathology revealed loosely scattered granulomas in the dermis with lymphocytes, plasma cells, neutrophils and eosinophils. A disease free Grenz zone was seen. A diagnosis of granuloma faciale was made and the patient was put on intralesional steroid therapy. She continues to be on regular follow up

Johanson-blizzarci syndrome with diamond-blackfan anemia

Johanson Blizzard syndrome [JBS] is a rare multi-system disorder characterized by congenital aplasia or hypoplasia of alae nasi, exocrine pancreatic insufficiency, hypothyroidism, deafness, growth retardation, varying degree of mental retardation, alopecia, wide open fontanels, anti-mongoloid slant, cafe-au-lait spots and absent of permanent teeth. We report a 3 months old male child having Johanson Blizzard syndrome with classical clinical features, pancreatic insufficiency and Diamond-Blackfan anemia

Vasculopathy in patients with diabetic foot using doppler ultrasound

To determine the extent of vasculopathy in patients with diabetic foot using Doppler ultrasound scanning. This descriptive study was carried out in Surgical Unit III, Civil Hospital, Karachi from January 2004 to December 2005. All patients having diabetic foot complications including abscess formation, cellulitis, ulceration and gangrene were included in the study after taking informed consent. Peripheral pulses like posterior tibial, dorsalis pedis, and popliteal arteries were checked. The presence of vasculopathy was checked with Doppler scanning of dorsalis pedis artery, posterior tibial artery, popliteal artery and veins. The data was collected on a proforma that was specially designed for this study. Out of the total 30 patients included in this study, vasculopathy was detected in 19 [63.33%] patients. The pulse was clinically weak in 10 [33.33%] and absent in 7 [23.33%] patients, while the Doppler scan revealed that in 17 [56.66%] cases the dorsalis pedis artery and in 19 [63.33%] the posterior tibial artery was involved. These findings were asymmetrical i.e. they were mostly found in the affected limb, with a male preponderance. This study shows that the vasculopathy is strong independent risk factor in the development of diabetic foot lesions. The usual symptoms and signs of lower limb ischemia may not be present and indeed may be misleading in diabetic foot disease

Morphological spectrum of chronic hepatitis [C] infection: experience at foundation university medical college and Fauji foundation hospital Rawalpindi

To determine the morphological patterns of HCV related liver disease in our set up. 164 routinely processed liver biopsies were assessed and scored for grading [necro-inflammatory changes] and staging [fibrosis] using modified Knodell HAI scoring system. Our patients were mostly females and maximum cases were in 4[th] and 5[th] decades. Mild portal inflammation was seen in 78% and moderate in 20% cases. Piecemeal necrosis was mild in 76% while moderate in 15% cases. HAI grades ranged from mild [161 cases] to moderate [03 cases]. Fibrous portal expansion/short fibrous septae were seen in 35[21.3%] cases. HCV related lived disease is mild in our set up and a long term follow up of these cases is required to assess the progression of disease

Management of periorbital defects with local flaps after resection of basal cell carcinoma eye lids

Basal cell carcinoma [BCC] is the most common malignant tumour of eyelids. In this study management of periorbital defects of patients treated for BCC is described. A prospective study. This study was done from June 2001 to October 2003 at CMH Quetta. This study included all the patients suffering from BCC who were treated for periorbital defects resulting from resection of BCC. Fifteen patients with periorbital BCC were included in this study. Eight were males and seven were females. Ages varied from forty to seventy years. These patients were treated by cheek flap, muscular flap, Glabellar flap and Tenzel flap. An early detection and timely management of eyelid BCC give excellent results and prevent disfigurement and morbidity

Comparison of three prognostic scores [PRISM, PELOD and PIM 2] at pediatric intensive care unit under Pakistani circumstances

To compare the performance of the Pediatric Risk of Mortality [PRISM], the Pediatric Index of Mortality 2 [PIM 2] and Pediatric Logistic Organ Dysfunction [PELOD] scores at general pediatric intensive care unit in a developing country setting, investigating the relation between observed and predicted mortality. A contemporary cohort study was undertaken at Pediatric Intensive Care Unit [PICU], Children's Hospital, Institute of Child Health, Lahore, Pakistan. 131 consecutive admissions fulfilling the inclusion criteria were enrolled in the study. PRISM, PIM 2 and PELOD calculations were performed as set out by original articles, using the published formulae. Statistical analysis included Standardized Mortality Rate [SMR], Hosmer Lemeshow goodness of fit test, receiver operating curve [ROC] characteristics and Spearman's correlation test. 139 patients were admitted to PICU. 38 presented exclusion criteria. 29 [28.7%] patients died. Estimated mortality was; PRISM: 19.7[19.5%], PIM: 21.01[20.5%] and PELOD: 18.4[18.3%]. SMR was 1.47 [SD +/- 0.19], 1.4 [SD +/- 0.19] and 1.57 [SD +/- 0.19], respectively. PRISM had better calibration [x[2] = 7.49, p = 0.49] followed by PIM 2 [x[2] = 9.65, p = 0.29]. PIM 2 showed best discrimination with area under ROC = 0.88 [0.81-0.94] followed by PRISM 0.78 [0.67-0.89] and PELOD 0.77 [0.68-0.87]. Spearman's correlation r between PRISM and PIM 2 returned 0.74 [p < 0.001]. PRISM as well as PIM 2 is validated for PICU setting in Pakistani circumstances. PELOD performed poorly. PIM 2 has advantages over PRISM for stratification of patients in clinical trials