Factors associated with poor outcomes after congenital heart surgery in low-resource setting in Pakistan: insight from the IQIC Registry - a descriptive analysis.

OBJECTIVE: This study aimed to assess the International Quality Improvement Collaborative single-site data from a developing country to identify trends in outcomes and factors associated with poor outcomes. DESIGN: Retrospective descriptive study. SETTING: The National Institute of Cardiovascular Diseases, Karachi, Pakistan. PARTICIPANTS: Patients undergoing surgery for congenital heart disease (CHD). OUTCOME MEASURE: Key factors were examined, including preoperative, procedural and demographic data, as well as surgical complications and outcomes. We identified risk factors for mortality, bacterial sepsis and 30-day mortality using multivariable logistic regression. RESULTS: A total of 3367 CHD surgical cases were evaluated; of these, 59.4% (2001) were male and 82.8% (2787) were between the ages of 1 and 17 years. Only 0.2% (n=6) were infants (≤30 days) and 2.3% (n=77) were adults (≥18 years). The in-hospital mortality rate was 6.7% (n=224), and 4.4% (n=147) and 0.8% (n=27) had bacterial sepsis and surgical site infections, respectively. The 30-day status was known for 90.8% (n=3058) of the patients, of whom 91.6% (n=2800) were alive. On multivariable analysis, the adjusted OR for in-hospital mortality was 0.40 (0.29-0.56) for teenagers compared with infancy/childhood and 1.95 (1.45-2.61) for patients with oxygen saturation <85%. Compared with Risk Adjustment for Congenital Heart Surgery (RACHS-1) risk category 1, the adjusted OR for in-hospital mortality was 1.78 (1.1-2.87) for RACHS-1 risk category 3 and 2.92 (1.03-8.31) for categories 4-6. The adjusted OR for 30-day mortality was 0.40 (0.30-0.55) for teenagers and 1.52 (1.16-1.98) for patients with oxygen saturation <85%. The 30-day mortality rate was significantly higher in RACHS-1 risk category 3 compared with category 1, with an adjusted OR of 1.64 (1.06-2.55). CONCLUSIONS: We observed a high prevalence of postoperative infections and mortality, especially for high-risk procedures, according to RACHS-1 risk category, in infancy/childhood, in children with genetic syndrome or those with low oxygen saturation (<85%).

Incidence, clinical profile and short term outcome of cerebral abscess in cyanotic congenital heart diseases.

Background: Brain abscess is a serious infection of brain parenchyma in patients with cyanotic congenital heart disease (CCHD) and around 25-46 % of unrepaired CCHD patients develop brain abscess. Aim of this study was to determine the incidence, clinical features, microbiology and factors associated with early and short term outcome of cerebral abscess in CCHD. Method: ology: This is a retrospective study, conducted at Pediatric cardiology department, National Institute of Cardiovascular Diseases (NICVD) Karachi. The data was collected from January 2019 to December 2021. All CCHD patients between 1 and 25 years of age were included. Data of patients with cerebral abscess was reviewed. Results: Among the 544 pediatric patients hospitalized in the last two years, brain abscesses were identified in 51 (9.3 %). Polycythemia (31.4 %) was the most significant contributing factor, especially in patients aged above 10 years. The most frequently seen CCHD was tetralogy of fallot (TOF) 60.8 %. Majority of the patients (84.3 %) had a single abscess while 15.7 % had multiple abscesses. E coli (9.7 %) was the most common isolated pathogen. Immediate complication identified was cerebral edema in 22 (43.1 %). Four patients (7.8 %) died, 47 (92.2 %) patients completed treatment course. 45.1 % had complete recovery however, 17 (33.3 %) had neurological deficits, 8 (15.7 %) had seizures and 2 (3.9 %) patients had residual abscess. Prolonged hospitalization was observed in patients of age group <10 years. Conclusion: In patients with underlying CCHD,early referrals and intervention are key to mitigating the severe consequences of cerebral abscesses and can drastically improve patient outcomes.

Clinical and epidemiological features of pediatric population hospitalized with COVID-19: a multicenter longitudinal study (March 2020-December 2021) from Pakistan.

Background: We aimed to explore the epidemiological, clinical, and phenotypic parameters of pediatric patients hospitalized with COVID-19 in Pakistan. Methods: This longitudinal cohort study was conducted in five tertiary care hospitals in Pakistan from March 2020 to December 2021. Data on various epidemiological and clinical variables were collected using Case Report Forms (CRFs) adapted from the WHO COVID-19 clinical data platform at baseline and at monthly follow-ups for 3 months. Findings: A total of 1090 children were included. The median age was 5 years (Interquartile range 1-10), and the majority presented due to new signs/symptoms associated with COVID-19 (57.8%; n = 631), the most common being general and respiratory symptoms. Comorbidities were present in 417 (38.3%) children. Acute COVID-19 alone was found in 932 (85.5%) children, 81 (7.4%) had multisystem inflammatory syndrome (MIS-C), 77 (7.0%) had overlapping features of acute COVID-19 and MIS-C, and severe disease was found in 775/1086 (71.4%). Steroids were given to 351 (32.2%) patients while 77 (7.1%) children received intravenous immunoglobulins. Intensive care unit (ICU) care was required in 334 (31.6%) patients, and 203 (18.3%) deaths were reported during the study period. The largest spike in cases and mortality was from July to September 2021 when the Delta variant first emerged. During the first and second follow-ups, 37 and 10 children expired respectively, and medical care after discharge was required in 204 (25.4%), 94 (16.6%), and 70 (13.7%) children respectively during each monthly follow-up. Interpretation: Our study highlights that acute COVID-19 was the major phenotype associated with high severity and mortality in children in Pakistan in contrast to what has been observed globally. Funding: The study was supported by the World Health Organization (WHO), which was involved in the study design but played no role in its analysis, writeup, or publication.

Effectiveness of oral sildenafil for neonates with persistent pulmonary hypertension of newborn (PPHN): a prospective study in a tertiary care hospital.

INTRODUCTION: The prevalence of persistent pulmonary hypertension of newborn (PPHN) has been estimated 1.9/1000 live births. Although the efficacy of inhaled nitric oxide and extracorporeal membrane oxygenation in PPHN is well established but it is difficult to administer and monitor in resource limited countries. Owing to this, other treatment options need to be evaluated. METHOD: This is a prospective observational study conducted in the Pediatric Cardiology Department, NICVD, Karachi, from February 2020 to October 2020 after the approval from the Institutional Ethical Review Committee. All the neonates referred to our Unit were screened by echocardiography (echo) and those who fulfilled the inclusion criteria were included. Echo were done before starting sildenafil and after 72 h to assess the pressure gradient across tricuspid valve and right to left or bidirectional shunt across patent ductus arteriosus , patent foramen ovale , or both. Sildenafil was started with a dose of 1 mg/kg/dose thrice a day and increased to 2 mg/kg/dose after 48 h if partial pressure of oxygen (PO1] did not increase. In neonates who did not respond to increased dose of Sildenafil were added on oral Bosentan 1 mg/kg/dose twice a day. RESULTS: Total 82 newborns were enrolled. Fifty-two patients improved after 48 h so were continued on same treatment. Sildenafil dose was increased in 30 (37.9%) patients whose PO2 did not increase to at least 10% from baseline after 48 h of starting treatment. Three patients expired within 48-72 h. Out of 27 remaining patients, only four responded whereas 23 patients did not show any improvement. In these patients, Bosentan was supplemented along with sildenafil. CONCLUSION: The results of our study show effectiveness of oral Sildenafil in treating PPHN. The overall improvement observed in the patients was overwhelming. Combination of Sildenafil with Bosentan is beneficial in patients who did not respond on Sildenafil alone.

Early and Intermediate-Term Outcome of Balloon Aortic Valvuloplasty in Children With Aortic Stenosis and Left Ventricular Dysfunction at Tertiary Care Hospital.

Background Left ventricular (LV) dysfunction in patients with aortic valve stenosis (AVS) is seen in two scenarios: in neonates and in elderly patients. Neonatal AVS may present as a congestive cardiac failure (CCF), while older children rarely present with CCF if they have not been diagnosed early. Only a few reports of LV dysfunction with AVS have been described in the literature. However, there is a paucity of data regarding the safety and effectiveness of balloon aortic valvuloplasty (BAV) in children with AVS with LV dysfunction. Therefore, the aim of this study was to evaluate outcomes to establish the safety and effectiveness of BAV in children with AVS and LV dysfunction in improving LV function and survival. Methods A total of 160 BAVs were performed from 2004 to 2017; of these, 41 (25.6%) patients had LV dysfunction. We reviewed these cases, and data were obtained on clinical features, echocardiographic parameters including LV ejection fraction (LVEF) and LV dimensions, LV posterior wall, interventricular septal thickness, pressure gradient across the valve, aortic valve morphology and annulus and aortic insufficiency (AI), and angiographic parameters such as aortic and LV pressures, AI and annulus size, and balloon size. Echocardiography was done before the procedure, one day after intervention, at three months, at six months, and on regular follow-up. Mortality during and after the procedure and at follow-up was reported. Results Children who had undergone BAV for AVS and LV dysfunction within the age range of six to 192 months showed a significant reduction in peak-to-peak pressure gradient (PPG) from 73.5 ± 30 mmHg to 26.7 ± 6.7 mmHg and improvement in LVEF from 32.8 ± 11% to 54.3 ± 12.7% after 24 hours. Instantaneous gradient on echocardiography after three months showed PPG was 29.8 ± 7.7 mmHg and mean LVEF was 63 ± 8.6%. Mean LV end-diastolic pressure was 20.8 ± 4.7 mmHg and decreased to 13 ± 2.4 mmHg. Four patients died, all of whom had severe LV dysfunction - one died during the procedure and three died within six to 20 hours after successful BAV. On average follow-up of 6.4 ± 3.8 years, with a range of three months to 13 years, there was no mortality, pressure gradient increased to 40 ± 16.3 mmHg (range, 20 to 90 mmHg), and three had BAV after one, four, and six years, respectively. There was an increase in AI from mild to moderate in five patients, but they did not require any intervention. Four patients had aortic valve replacement (AVR) with two patients having an increase in pressure gradient and AI after eight and 13 years, respectively. One patient had AI (+3) after BAV had AVR after three years, and one patient who had a very thick and dysplastic aortic valve with LVEF of 20% and pulmonary hypertension (PH) had AVR after six months. Conclusion Patients with AVS who develop LV dysfunction deteriorate and die soon without treatment. Our data suggest that BAV in children with aortic stenosis and LV dysfunction is safe and effective in the normalization of LV function.

Correlation between Echocardiography and Cardiac Catheterization for the Assessment of Pulmonary Hypertension in Pediatric Patients.

Introduction Cardiac catheterization is widely considered the "gold standard" for the diagnosis of pulmonary hypertension. However, its routine use is limited due to its invasive nature. Therefore, the aim of this study was to evaluate the correlation between pulmonary artery pressures obtained by various parameters of transthoracic echocardiography and cardiac catheterization. Methods This study includes 50 consecutive patients with intracardiac shunt lesions diagnosed with severe pulmonary hypertension on echocardiography and admitted for cardiac catheterization at the National Institute of Cardiovascular Diseases (NICVD) in Karachi, Pakistan. Cardiac catheterization and transthoracic echocardiography were performed in all patients simultaneously and systolic (sPAP) and mean pulmonary artery pressure (mPAP) were assessed with both modalities. Correlations and agreement, in terms of Bland-Altman plot, were computed between both modalities for sPAP and mPAP. Results Out of 50 patients, 46% (23) were male and mean age was 7.49 ± 4.45 years. On cardiac catheterization, sPAP was 93.92 ± 17.91 mmHg and mPAP was 67.0 ± 14.28 mmHg. Correlation between cardiac catheterization and echocardiography for the assessment of sPAP was 0.917 (p<0.001), and mPAP was 0.832 (p<0.001) for mean gradient of tricuspid regurgitation (PGTRmean), 0.749 (p<0.001) for peak gradient of pulmonary regurgitation (PGPRpeak), 0.691 (p<0.001) for Acceleration time across right ventricular outflow tract (RVOT), and 0.752 (p<0.001) for end gradient of pulmonary regurgitation (PGPRend). Bland-Altman plot showed moderate agreement between two modalities. Conclusion A positive but modest correlation was observed between hemodynamic parameters of transthoracic echocardiography and cardiac catheterization for assessment of pulmonary artery pressures. Transthoracic echocardiography can reliably be used as an initial non-invasive modality for the assessment of pulmonary artery hypertension and can obviate the need of right heart catheterization in some patient especially with mild pulmonary hypertension.

Echocardiography guided bed side balloon atrial septostomy in dextro transposed great arteries (dTGA) with intact ventricular septum (IVS): A resource limited country experience.

OBJECTIVE: To determine effectiveness and safety of echocardiography guided bed side Balloon Atrial Septostomy (BAS) in dextro transposition of great Arteries (dTGA) with intact ventricular septum (IVS) at a public sector tertiary care hospital Karachi, Pakistan. METHODS: This case series include 40 patients with echocardiographic findings of dTGA with IVS and restricted PFO (≤ 2mm) who underwent bedside BAS at department of pediatric cardiology NICVD, Karachi, Pakistan. We recorded pre and post BAS diameter of PFO/Atrial Septal Defect (ASD), oxygen saturation (SpO2 %), and post procedure complications and outcome. RESULTS: Median age was 16 days, Majority of them (n=23, 58%) were severely cyanosed with SpO2 of 41.4±3.4% and underwent emergency BAS and remaining underwent elective procedure. An increase in SpO2% from 46.0±6% to 81.0±3.0% (p=<0.001) and ASD size from 1.4±2.8mm to 5.45±0.4mm was observed (p=<0.001). No complication was observed in most of cases (n=28, 70%). Mean hospital stay was 3.4±1 days. Success rate was 97.5% however, one neonate died due to neonatal sepsis. CONCLUSION: Our study provides sufficient evidence that bed side balloon atrial septostomy is a safer technique, save a lot of time and resources which were required otherwise in transporting these patients to catheterization laboratory.

Phosphine induced acute cardiotoxicity in children: A need for health awareness.

Aluminum Phosphate (AP) is a cheap and highly effective pesticide. Phosphine is the active pesticidal component which is highly toxic. Mortality is high and ranges between 40-70%. There is a desperate need for creating awareness. We report a case series with a mortality of 40% and emphasize the need for public awareness.

Clinicodemographic features and outcome of acute myocarditis in children admitted at tertiary care hospital.

BACKGROUND: Myocarditis is an inflammatory disorder of myocardium associated with high mortality and morbidity. It is often difficult to diagnose because it can either be asymptomatic or mimic other illnesses. This study is conducted to evaluate clinicodemographic features and outcome of acute myocarditis in children admitted at a tertiary care hospital. METHODS: Medical records of all children aged 1month to 16years who were admitted between 2005 and 2013 at Aga Khan University Hospital, Karachi and discharged with a diagnosis of myocarditis were reviewed retrospectively. Clinical and demographic features, management and outcome were recorded. RESULTS: Records of a total of 62 patients with the diagnosis of myocarditis were reviewed retrospectively between 2005 and 2013. Median age of patients was 12.5months with 28 (45%) females and 34 (55%) males. The main presenting complaint observed was irritability (73%) and least common symptom was abdominal pain (23%) while the most frequently occurring examination finding was tachycardia (90%). Length of the hospital stay was 8±4.7days with 21 (34%) admissions in the ward and 41 (66%) in intensive care unit. CONCLUSION: The most frequent presentation in our study was irritability, followed by difficulty in breathing while the least common symptom was abdominal pain. Tachycardia was the commonest clinical sign observed so it is important to look for heart rate and rhythm in a sick child with irritability.

Frequency, causes and outcome of neonates with respiratory distress admitted to Neonatal Intensive Care Unit, National Institute of Child Health, Karachi.

OBJECTIVE: To determine the frequency, aetiology and outcome of respiratory distress in neonates in intensive care unit. METHODS: The descriptive cross-sectional study was conducted at the Neonatal Intensive Care Unit, National Institute of Child Health, Karachi, from October 2009 to March 2010. It comprised neonates aged day 0 to 28 who were admitted to Neonatal Intensive Care Unit. The neonates were screened first for respiratory distress and presence of one or more signs and symptoms. History, examination and investigations were carried out to find out various aetiologies of respiratory distress. Outcome was measured in terms of discharge and death. Data was analysed using SPSS12. RESULTS: Of the 205 neonates in the study, 120(58.6%) were boys and 85(41.4%) were girls The overall mean age was 70.58±110.02 hours and the mean gestational age was 36.32±2.72 weeks while the mean weight was 2.41±2.4kg. Respiratory rate >60/min was found in all (100%) the neonates. In terms of signs and symptoms, 125(60.9%) had grunting, 205(100%) had subcostal retractions and nasal flaring, and 81(40%) had cyanosis. The aetiologies observed were birth asphyxia, sepsis, transient tachypnoea of the newborn, pneumonia, meconium aspiration syndrome and respiratory distress syndrome in 22(10.75%), 37(18.05%), 29(14.1%), 36(17.6%), 34(16.7%) and 47(23.0%) neonates respectively. The incidence of neonates with respiratory distress was 68(33.3%). CONCLUSIONS: The frequency of respiratory distress among the neonates was high, while mortality was high in neonates with respiratory distress, especially in pre-term and low birthweight neonates. Early diagnosis and management is important for better outcome.

Empyema thoracis in children: clinical presentation, management and complications.

OBJECTIVE: To determine the etiology, clinical manifestation, management (medical and surgical) and complications of children with empyema thoracis in a tertiary care hospital from Karachi, Pakistan. STUDY DESIGN: Descriptive, analytical study. PLACE AND DURATION OF STUDY: Department of Surgery, The Aga Khan University Hospital, Karachi, from January 1996 to December 2010. METHODOLOGY: Medical records of admitted children aged > a month to 15 years with discharge diagnosis of empyema thoracis and data was collected on demographic features, clinical manifestation, management and complications. Children managed medically were compared with those managed surgically by using interquartile range and median comparison. Mann-Whitney U test was used to compare age in months, weight (kg) and length of stay in days and presenting complaint, duration of illness; chi-square test was used to compare thrombocytosis in between groups and p-value was calculated. RESULTS: Among the 112 patients, 59 (53%) were younger than 5 years of age. Males (n=83, 74%) were predominant. Fifty (45%) children were admitted in winter. Thirty (27%) children found unvaccinated and one fourth (n=27; 24%) were severely malnourished. Fever, cough, and dyspnea were the major presenting symptoms. Sixty-six (59%) were on some antibiotics prior to admission. Staphylococcus aureus (n=13) and Streptococcus pneumoniae (n=5) were the commonest organism isolated from blood and pleural fluid cultures. Majority of the children required some surgical intervention (n=86). Surgically managed children were younger (p=0.01); had less weight (p=0.01) and prolonged fever (p=0.02); and stayed longer in hospital (p < 0.001) as compared to medically managed children. Requiring readmission (n=8), subcutaneous emphysema (n=5) and recollection of pus (n=5) were the major complications. CONCLUSION: Staphylococcus aureus was the major organism associated with paediatric empyema thoracis. Early identification and empiric antibiotic as per local data is essential to prevent short and long-term complications. Younger, lower weight children with prolonged fever required surgical management.

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.

Outcome of infants with unrepaired heart disease admitted to the pediatric intensive care unit: single-center developing country perspective.

OBJECTIVE: Congenital heart disease (CHD) has an incidence of ∼0.8-1%. Outcome of previously diagnosed CHD patients awaiting surgery (either correction or palliation) in a developing country setting is unknown. We strive to determine the outcome of patients with CHD awaiting surgery who present to pediatric intensive care unit (PICU) setting with an acute illness. DESIGN: Retrospective cross-sectional chart review. SETTING: Pediatric intensive care unit of The Aga Khan University Hospital, Karachi, Pakistan. PATIENT: Medical records of infants (1-12 months) with CHD awaiting surgery presenting to the PICU with an acute illness between January 2009 and June 2012 were included. Newly diagnosed CHD patients, those not requiring PICU admission, and those transferred to another hospital were excluded. RESULTS: A total of 34 infants met the inclusion criteria. Median age at presentation was 5 months. Seventy-four percent of the infants had CHD lesion characterized by increased pulmonary blood flow (shunt lesions). Though none of the patients met the strict criteria for sepsis or pneumonia, 74% were admitted with a diagnosis of pneumonia or sepsis. Only 15% of patient had congestive heart failure as an admitting diagnosis. Oxygen therapy was given to 94% of these patients. Fifty-nine percent of these patients expired during the admission, 95% of those expired had multiorgan dysfunction. CONCLUSION: Patients with CHD awaiting surgery and who admitted to the PICU with acute illness are at high risk for mortality. Stringent criteria to diagnose pneumonia or sepsis should be used in these patients.

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.

Severe combined immune deficiency syndrome.

OBJECTIVE: To determine the clinico-demographic features and laboratory parameters of children with severe combined immunodeficiency (SCID). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Department of Paediatrics and Child Health, the Aga Khan University, Karachi, from July 2006 to July 2011. METHODOLOGY: Thirteen infants who were discharged with a diagnosis of SCID were inducted in the study. Their clinicodemographic features and laboratory parameters were determined. Descriptive statistics has been used for computing frequency and percentage. RESULTS: The median age at diagnosis was five months; 5 infants presented within 3 months of life. Three-fourth (77%) were males. Most of the infants were severely malnourished (85%) at the time of presentation. More than two-thirds (69%) were products of consanguineous marriages. All subjects had severe lymphopenia {absolute lymphocyte count (ALC) ranging between 170 - 2280} and low T and B lymphocyte counts. CONCLUSION: SCID should be considered in infants presenting with severe and recurrent infections. Low ALC (< 2500/mm³), is a reliable diagnostic feature of SCID. These infants should be promptly referred to a facility where stem cell transplant can be done.

Midterm results of bovine jugular vein conduit for right ventricular outflow tract reconstruction.

OBJECTIVE: To evaluate the midterm results of Contegra conduit. METHODS: The retrospective study comprised patient record at Aga Khan University Hospital, Karachi, of conduits implanted between May 2007 and June 2012. Data collection was made from the clinical notes and from serial echocardiograms by a single cardiologist. The last followup echocardiography was done at the time of data collection in June 2012. SPSS 19 was used for statistical analysis. RESULTS: A total of 18 conduits had been implanted (16-22 mm) during the study period. Median age at the time of surgery was 9 years (range: 2.5-16 years). Early mortality was seen in 3 (16.66%) patients, but none was Contegra related. Of the remaining 15 patients, 2 (13.33%) with a diagnosis of Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries (PA), developed severe distal pressure gradient (50 mmHg) across Contegra over a median period of 18 months (range: 12-24 months), with resultant severe regurgitation and needed percutaneous intervention. There was no thrombosis, calcification, anuerysmal dilation or late deaths. CONCLUSION: At midterm followup, Contegra conduit was associated with low re-intervention rates with satisfactory haemodynamic results. However, long-term durability must be determined for this conduit, especially in patients with Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries.

Acinetobacter species meningitis in children: a case series from Karachi, Pakistan.

INTRODUCTION: Multidrug-resistant strains of Acinetobacter pose a serious therapeutic dilemma in hospital practice, particularly when they cause meningitis, as the few antimicrobial agents to which these isolates are susceptible have poor central nervous system (CNS) penetration. METHODOLOGY: We retrospectively reviewed the clinical course and outcome of eight consecutive cases of meningitis due to Acinetobacter spp. in children ages 15 years or less, seen in a tertiary care medical center in Karachi, Pakistan. RESULTS: Of the eight cases of Acinetobacter meningitis, isolates from five patients were pan-resistant, and two were multidrug-resistant. A neurosurgical procedure was performed in five of eight patients followed by external ventricular drain insertion prior to the development of infection. Seven received intravenous (IV) polymyxin (mean; 12.8 days), while 5/8 also received intrathecal (IT) polymyxin (mean; 12.0 days). The mean length of hospitalization was 38.7 ± 19 days. All patients achieved cerebrospinal fluid (CSF) culture negativity by the end of treatment (mean; 5.4 days). Two patients died: one with pan-resistant Acinetobacter, and the second with a multi-drug resistant isolate. CONCLUSION: Post-neurosurgical multidrug-resistant and pan-resistant Acinetobacter meningitis can be successfully treated if appropriate antimicrobial therapy is instituted early. The role of IT polymyxin B administration alone versus combination therapy (IV and IT) needs further study.