Idiopathic thrombocytopenic purpura in children: a 10 years experience at tertiary care hospital.

OBJECTIVE: To evaluate presenting features, treatment modalities and response to therapy in children with idiopathic thrombocytopenic purpura. METHODS: The retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised clinical data, presenting demographics, clinical spectrum, management and outcome of children admitted with idiopathic thrombocytopenic purpura from 2001 to 2010. SPSS 20 was used for statistical analysis. RESULTS: Records of 95 children between 0-15 years in the period 2001 to 2010 were reviewed. The overall mean age at the time of presentation was 6.1±3.8 years. There were 45(47.3%) male and 50(52.7%) female cases. A total of 34(35.8%) patients had history of preceding illness. Regarding clinical presentations, bruises 81(85.3%), petechial rash 75(79%), epistaxis 23(24%) were common. Median platelet count at the time of presentation was 5,000 (Inter-quartile range: 4000-13,000). Spontaneous recovery was seen in 7(7.4%) children. Overall, 24(25.2%) patients received intravenous immunoglobulin G and only 19(20%) showed complete response. Besides, a total of 32(33.7%) patients did not respond and only 5(5.3%) developed chronic ailment. CONCLUSION: Bruises, petechial rash and epistaxis were the common presentations. Overall prognosis was good.

Malignant mediastinal mass in children: a single institutional experience from a developing country.

OBJECTIVE: To determine the clinical spectrum and management outcomes of paediatric patients with modiastinal mass in a Karachi hospital. METHODS: Medical records of all cases of mediastinal masses in children diagnosed and treated between January 2005 and December 2011 were retrospectively reviewed to evaluate the mode of presentation, histopathological diagnosis, radiologic findings and management outcomes at Aga Khan University Hospital, Karachi, Pakistan. SPSS 19 was used for data analysis. RESULTS: A total of 37 patients of mediastinal masses were identified, and malignancy was found in 32 (86%) cases. The median age at diagnosis was 9 years (interquartile range: 4.7 years). Lymphoma 23 (72%) and leukaemia 8 (25%) were the most common causes of mediastinal mass. Nonspecific symptoms such as fever 26 (81%), cough 15 (47%) and dyspnoea 12 (37%) constituted the most commonly presenting complaints. Overall, 22 (68.7%) patients underwent surgical procedures (complete/partial resection of mass); local lymph node biopsy was performed in 5 (15.6%) cases; and computed tomography or ultrasound-guided biopsy was done in 2 (5.4%) patients. Besides, 27 (84.4%) patients were admitted to paediatric intensive care unit for supportive care, and assisted ventilation was required in 20 (62.5%) patients. The mean length of hospital stay was 9.3 +/- 6 days. None of the patients died due to complications related to mediastinal mass or diagnostic procedure. CONCLUSIONS: Although mortality rate has reduced significantly with refinements in the management protocols, but a high index of suspicion and comprehensive multidisciplinary approach is crucial to improve the morbidity and mortality.

Ovarian haemorrhage: a rare presentation and diagnostic dilemma in factor XIII deficiency.

Factor XIII (FXIII) deficiency is a rare (autosomal recessive) genetic disorder which is associated with delayed bleeding symptoms that occur hour or days after trauma. Spontaneous rupture of visceral organs due to FXIII deficiency is a rare cause of abdominal pain with grave consequences and can be easily confused with other abdominal pathologies because of normal standard coagulation profile in these patients. We report a 15-year-old girl with spontaneous ovarian haemorrhage and splenic haematoma with FXIII deficiency. She had normal coagulation screen along with normal FXIII screen initially on the 5M urea testing which lead to delay in her diagnosis.

Clinical Profile and Short-Term Outcome of Pediatric Hyperleukocytic Acute Leukemia from a Developing Country.

This study was conducted to determine the frequency, clinical profile, and short-term outcome of children with hyperleukocytosis at two pediatric oncology centers in Karachi. Of a total 1,045 patients, 13.97% (n=146) patients had hyperleukocytosis. Majority (61.7%, n=90) were under 10 years of age and 76% (n=146) were male. The symptom duration before diagnosis was more than 30 days in 49.3% (n=72). The median WBC count was 181 x109/L(IQR=130.45298.3) and extreme hyperleukocytosis (>200 x109/L) was observed in 44.5% (n=65) patients. Majority (94.5%, n=138) of patients were diagnosed with acute lymphoblastic leukemia. One or more complications developed in 78% (n=114) of cases. Clinical and laboratory tumor lysis syndrome (TLS) was observed in 17.1% (n=25) and 39% (n=57) patients, respectively. Pulmonary and neurological complications related to leukostasis were noted in 9.5% (n=14) and 27.3% (n=40) of cases, respectively. Infectious complications occurred in 23.2% (n=34) patients. The case-specific mortality was 20.5% (n=30). No mortality was related to early complications of hyperleukocytosis.

Congenital Factor VII Deficiency in Children at Tertiary Health Care Facility in Pakistan.

This study presents the demographics, clinical spectrum, and outcome of patients with congenital factor VII (FVII) deficiency at a tertiary care center over a period of 12 years. Of the 49 patients, 27 (55%) patients were males. Consanguinity was found in 92% of the patients. The median age of symptom onset was 2.4 (interquartile range [IQR]: 1.1-6.5) years with a median age of 5.8 (IQR: 3.1-10) years at diagnosis. Life-threatening complications like intracranial bleeding (ICB) and intra-abdominal bleeding (IAB) were observed in 8 (16.4%) patients. We found that 11 (55%) of the 20 patients with FVII coagulant activity (FVIIc) <1% were either asymptomatic or showed mild phenotype. In contrast, 9 (53%) of the 17 patients with FVIIc >5% were affected by severe symptoms. Age <1 year was the only identified risk factor associated with development of life-threatening bleeding episodes (P = .042; odds ratio 6.46). Overall, 4 (8.2%) died as a consequence of ICB (3 patients) and IAB (1 patient).

Risk factors for complicated varicella infection in pediatric oncology patients at a tertiary health care facility in Pakistan.

INTRODUCTION: Varicella zoster infection (VZI) is well recognized as a potential cause of morbidity and mortality in immunocompromised pediatric oncology patients (POP). The purpose of this study was to describe the clinical profile and risk factors for complications and outcomes of VZI in POP treated with acyclovir. METHODOLOGY: Medical records of all POP with a discharge diagnosis of VZI over a period of seven years (2005-2011) were reviewed. The demographic features, underlying malignancy, risk factors for VZI, complications, and outcomes were recorded. RESULTS: Thirty-six POP with VZI were identified. Leukemia was the most common underlying malignancy (n = 20, 58.8%), followed by lymphoma (n = 7, 20.6%) and solid organ tumors (n = 7, 20.6%). Most of the cases (41%) were observed in children under five. All patients were treated with acyclovir. Varicella-related complications developed in 10 (29%) patients. The most frequent complication was bloodstream infection (n = 3, 8.8%), followed by pneumonia (n = 2, 5.9%), skin infection (n = 2, 5.9%), hepatitis, renal failure, and encephalitis. Independent risk factors associated with complications were age < five years, weight for age < fifth percentile, delay in seeking care (> seven days after onset of symptoms) and severe neutropenia (ANC < 500/cm). One child died secondary to varicella encephalitis. CONCLUSION: Our data suggests that young age, poor health-seeking behavior, severe neutropenia, and being underweight are the major risk factors for the development of varicella-related complications in POP in developing countries. These complications could be favorably modified through active immunization of immunocompetent children.

Neonatal sepsis following prolonged rupture of membranes in a tertiary care hospital in Karachi, Pakistan.

INTRODUCTION: Prolonged rupture of membrane (PROM) is an important risk factor for early onset neonatal sepsis (EONS), which is associated with increased neonatal morbidity and mortality. We reported the incidence and associated risk factors of PROM for culture-proven EONS. METHODOLOGY: The medical records of all neonates born at Aga Khan University, Karachi over a period of five years (2007-2011) with PROM (> 18 hours) were reviewed. Data about maternal and neonatal risk factors for EONS was collected and adjusted logistic regression (AOR) analysis was applied. RESULTS: Incidence of PROM in this neonatal birth cohort was 27/1,000 live births. A total of 17 (4%) cases with blood-culture proven bacterial sepsis were identified within 72 hours of birth. Klebsiella pneumonia (n = 5; 29%) and Pseudomonas aeruginosa (n = 4; 24%) were the commonest isolates followed by group B Streptococcus (n = 3; 18%) and Escherichia coli (n = 2; 12%). Maternal fever (p = <0.001; AOR, 36.6), chorioamnionitis (p < 0.001; AOR, 4.1), PROM > 48 hr. (p < 0.001; AOR, 8.2), neonatal prematurity < 34 weeks (p < 0.001; AOR, 4.1) and low birth weight < 1,500 grams (p 0.001; AOR, 9.8) along with neonatal thrombocytopenia and raised CRP were found to be independent risk factors associated with culture-proven EONS in PROM. CONCLUSIONS: Preventive measures should focus on recognition of these high-risk infants with prompt laboratory screening for sepsis and early institution of empirical antibiotic based on local data. Such approaches would be a safe and cost-effective strategy, especially in developing countries.

Anthracycline-induced cardiotoxicity: prospective cohort study from Pakistan.

OBJECTIVES: To identify anthracycline-induced acute (within 1 month) and early-onset chronic progressive (within 1 year) cardiotoxicity in children younger than 16 years of age with childhood malignancies at a tertiary care centre of Pakistan. DESIGN: Prospective cohort study. SETTING: Aga Khan University, Karachi, Pakistan. PARTICIPANTS: 110 children (aged 1 month-16 years). INTERVENTION: Anthracycline (doxorubicin and/or daunorubicin). OUTCOME MEASUREMENTS: All children who received anthracycline as chemotherapy and three echocardiographic evaluations (baseline, 1 month and 1 year) between July 2010 and June 2012 were prospectively analysed for cardiac dysfunction. Statistical analysis including systolic and diastolic functions at baseline, 1 month and 1 year was carried out by repeated measures analysis of variance. RESULTS: Mean age was 74±44 months and 75 (68.2%) were males. Acute lymphoblastic leukaemia was seen in 70 (64%) patients. Doxorubicin alone was used in 59 (54%) and combination therapy was used in 35 (32%). A cumulative dose of anthracycline <300 mg/m(2) was used in 95 (86%). Fifteen (14%) children developed cardiac dysfunction within a month and 28 (25%) children within a year. Of these 10/15 (66.6%) and 12/28 (43%) had isolated diastolic dysfunction, respectively, while 5/15 (33.3%) and 16/28 (57%) had combined systolic and diastolic dysfunction. Seven (6.4%) patients expired due to severe cardiac dysfunction. Eight of 59 (13.5%) children showed dose-related cardiotoxicity (p=<0.001). Cardiotoxicity was also high when the combination of doxorubicin and daunorubicin was used (p=0.004). CONCLUSIONS: Incidence of anthracycline-induced cardiotoxicity is high. Long-term follow-up is essential to diagnose its late manifestations.

Cardiac involvement in Kawasaki disease in Pakistani children.

INTRODUCTION: Coronary artery involvement is the most dreaded long-term complication of Kawasaki disease. Our aim was to look at the pattern of cardiovascular involvement in Pakistani children admitted with Kawasaki disease. METHODS: This study included children admitted with Kawasaki disease at the Aga Khan University Hospital Karachi over a period of 14 years from January 1997 to December 2010. Information gathered included patient demographics, clinical features, investigations, echocardiographic findings, treatment and follow-up. Those with coronary artery involvement on initial echocardiogram remained on long-term follow-up with clinical examination and echocardiogram. RESULTS: A total of 56 patients were admitted. (Mean age at diagnosis 33 ± 30 months, age range 2 months to 9 years). 18% of patients had incomplete features. Twenty-five percent (14/56) patients presented after 10 days of fever. Cardiac examination was normal except for tachycardia. Abnormal coronary arteries were seen in 23 patients (41%) - left main coronary artery in 23 (41%), left anterior descending and right main coronary artery in 20 (36%), circumflex branch in 17 (30%). Risk factors for cardiac involvement were male sex, fever >10 days duration at the time of initial presentation and neutrophil percentage >75% in the initial white blood cell counts. Fifty four of 56 cases received intravenous immunoglobulin (IVIG), Seventy-five percent of the patients received IVIG within 10 days of illness. Mean duration of follow-up was 2.5 years. Eight percent of the patients still continue to have abnormal coronaries. There was no mortality. CONCLUSIONS: A higher incidence of coronary artery involvement was found in our study. Presentation after 10 days of illness increases the risk of coronary artery involvement. High index of suspicion among the general pediatricians about the disease can possibly be helpful for early referral and treatment.