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 (\u3c 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

A three-year review of antimicrobial resistance of Salmonella enterica serovars Typhi and Paratyphi A in Pakistan

Introduction: Enteric fever is among the most common bacteraemic illnesses in South Asia. Multidrug resistance as well as fluoroquinolone resistance has severely limited therapeutic options in high disease burden countries such as Pakistan. This review was conducted to determine the frequency of drug-resistant Salmonella enterica serovar Typhi (S.Typhi) and Salmonella enterica serovar Paratyphi A (S. Paratyphi A) between 2009 and 2011.METHODOLOGY: This study was a review of laboratory data. The antibiotic susceptibility of typhoidal Salmonellae isolated from blood cultures submitted to the Aga Khan University Hospital\u27s laboratory from all over Pakistan between January 2009 and December 2011 were reviewed.Results: The sensitivity data of 4,323 positive isolates of S. Typhi and S. Paratyphi A isolated during the three-year period were reviewed. The majority of isolates were S. Typhi (59.6%).Over three years, the incidence of multidrug-resistant (MDR) S.Typhi remained high, ranging from 64.8%-66.0%, while MDR S. Paratyphi A decreased from 4.2% to 0.6%. Fluoroquinolone resistance increased for S. Typhi from 84.7% to 91.7%. Cefixime- and ceftriaxone-resistant S. Typhi were isolated in two children.CONCLUSIONS: Our results show high rates of multidrug and fluoroquinolone resistance among S. Typhi and S. Paratyphi. The occurrence of two cases of ceftriaxone resistance is alarming

Sentinel hospital-based surveillance for assessment of burden of rotavirus gastroenteritis in children in Pakistan

Objectives: To determine the burden and molecular epidemiology of rotavirus gastroenteritis in children hospitalized with severe acute watery diarrhea in Pakistan prior to introduction of rotavirus vaccine.Methods: A cross-sectional study was carried out over a period of two years from 2006 - 2008 at five sentinel hospitals in the cities of Karachi, Lahore, Rawalpindi, and Peshawar. Stool samples collected from children under five years of age hospitalized with severe acute watery diarrhea were tested for rotavirus antigen via enzyme immunoassay (EIA) (IDEA REF K6020 Oxoid Ltd (Ely), Cambridge, United Kingdom). A subset of EIA positive stool samples were further processed for genotyping.Results: 6679 children were enrolled and stool specimens of 2039 (30.5%) were positive for rotavirus. Rotavirus positivity ranged from 16.3% to 39.4% in the 5 hospitals with highest positivity in Lahore. 1241 (61%) of all rotavirus cases were in infants under one year of age. Among the strains examined for G-serotypes, the occurrence of G1, G2, G9 and G4 strains was found to be 28%, 24%, 14% and 13%, respectively. Among P-types, the most commonly occurring strains were P6 (31.5%) followed by P8 (20%) and P4 (12%). Prevalent rotavirus genotype in hospitalized children of severe diarrhea were G1P[8] 11.6% (69/593), followed by G2P[4] 10.4% (62/593), and G4P[6] 10.1% (60/593).CONCLUSIONS: Approximately one third of children hospitalized with severe gastroenteritis in urban centers in Pakistan have rotavirus. Introduction of rotavirus vaccine in Pakistan\u27s national immunization program could prevent many severe episodes and diarrheal deaths

Clinical manifestations and treatment outcomes in HIV-1-infected children receiving antiretroviral therapy in Karachi, Pakistan

Introduction: The impact of antiretroviral (ARV) therapy on immunological and growth parameters in HIV-positive children in Pakistan has not been reported to date.METHODOLOGY: A retrospective chart review of children diagnosed with HIV at the Sindh AIDS Control Proigramme (SACP) and registered at the Aga Khan University, Karachi, between January 2005 and 2013 was conducted, evaluating clinical and laboratory profiles of HIV+ ARV+ children for ARV impact (serial height and weight CD4 and viral counts).Results: Twenty-four children were diagnosed and registered as HIV positive over five years, and 20 were started on ARV. Six were excluded from analysis (ARV duration \u3c 6 months). Nine (64.3%) of 14 fulfilled WHO criteria for treatment failure at a median duration of 25 weeks (IQR 18-32) on ARV and underwent resistance genotyping. All nine had NNRTI resistance, two had high-grade NRTI resistance (≥ 4 thymidine analog mutations). Median age at start of ARV was 71.5 weeks (IQR 37.5-119). Median baseline weight for age (WAZ) and height for age (HAZ) z-scores changed from -1.94 to 1.69 and -1.99 to -1.59, respectively, after six months of therapy. Median CD4 percentage and viral load at baseline changed from 13.8 to 17.8, while viral load changed from 285 * 104 copies to zero at six months.CONCLUSIONS: ARV improved absolute CD4 and viral counts. Weight and height did not improve significantly, highlighting the need for aggressive nutritional rehabilitation. Early development of ARV resistance in these children requires formal assessment

Cumulative age distribution of children less than 5 years of age admitted with severe diarrhea at five sentinel hospitals in Pakistan, 2006–2008.

<p>Bars represent number of children with rotavirus- positive diarrhea. Points represent cumulative rate (%) of rotavirus- positive gastroenteritis.</p

Distribution of rotavirus positive gastroenteritis by age in months at five sentinel hospitals in Pakistan from 2006–2008.

<p>Distribution of rotavirus positive gastroenteritis by age in months at five sentinel hospitals in Pakistan from 2006–2008.</p

Geographic distribution of the five sentinel sites participating in rotavirus surveillance in Pakistan from 2006–2008.

<p>Geographic distribution of the five sentinel sites participating in rotavirus surveillance in Pakistan from 2006–2008.</p