RESUMO
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 [
Results: Median age was 16 days, Majority of them [n=23, 58%] were severely cyanosed with SpO[2] of 41.4 +/- 3.4% and underwent emergency BAS and remaining underwent elective procedure. An increase in SpO[2]% 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
RESUMO
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. Descriptive, analytical study. Department of Surgery, The Aga Khan University Hospital, Karachi, from January 1996 to December 2010. 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. 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. 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
RESUMO
To determine the clinico-demographic features and laboratory parameters of children with severe combined immunodeficiency [SCID]. Case series. Department of Paediatrics and Child Health, the Aga Khan University, Karachi, from July 2006 to July 2011. Thirteen infants who were discharged with a diagnosis of SCID were inducted in the study. Their clinico-demographic features and laboratory parameters were determined. Descriptive statistics has been used for computing frequency and percentage. 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. SCID should be considered in infants presenting with severe and recurrent infections. Low ALC [< 2500/mm[3]], is a reliable diagnostic feature of SCID. These infants should be promptly referred to a facility where stem cell transplant can be done
RESUMO
The term "Ambiguous genitalia" applies to confusing appearance of the external genitalia. Sex assignment becomes essential for the parent's peace of mind, and in turn depends on anatomy and functional endocrinology rather than karyotype. Two cases with all different genetic sex, gonadal sex and phenotypic sex are described. First case is that of congenital adrenal hyperplasia [CAH] in a month old baby whose genotype was female with laboratory investigations exposing her diagnosis. She is doing well with oral hydrocortisone and fludrocortisone. Second case is that of probable 5-alpha reductase deficiency who would probably need future surgery