Clinical profile and outcome of cyanotic congenital heart disease in neonates

OBJECTIVE: To determine the clinical profile and assess the outcome of all neonates diagnosed with cyanotic congenital heart disease. STUDY DESIGN: A case series. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital from January 1998 to December 2000. PATIENTS AND METHODS: Neonates admitted with diagnosis of cyanotic congenital heart disease were evaluated for clinical diagnosis, survival and mortality. RESULTS: Forty four neonates met the inclusion criteria. Eleven babies (25%) had Tetralogy of Fallot or its variants. Other malformations were d-transposition of great arteries, tricuspid valve anomalies (tricuspid atresia and Ebstein\u27s anomaly), hypoplastic left heart syndrome, truncus arteriosus, total anomalous pulmonary venous return and complex congenital heart disease like single ventricle. Twenty eight (63.6%) neonates survived and 16 (36.4%) expired during hospital stay. Cause of death was surgical in 2 cases and medical problems in 14 babies. CONCLUSION: Tetralogy of Fallot or variants was the commonest cyanotic heart disease in neonates with frequency of 27.27%. Majority of neonates with congenital cyanotic heart disease showed survival with appropriate management

Clinical screening for congenital heart disease in newborns at a tertiary care hospital of a developing country

Objective: To screen all newborns admitted to a tertiary care hospital to rule out congenital heart disease before discharge and to find out the utility of pulse oximetry to detect congenital heart disease. Methodology: This prospective study was done at Aga Khan University Hospital from January 2014 to December 2014 in 1,650 newborns over a period of 12 months. Pulse oximetry and clinical examination were done. Persistent oxygen saturation less than 95% was considered as positive pulse oximetry. Newborns who had positive pulse oximetry or abnormal clinical examinations findings were subjected to echocardiography. Result: Pulse oximetry was performed on 1,650 newborns, out of which 25 (1.5%) had congenital heart disease. Positive pulse oximetry cases were 16 (0.97%), out of which 10 had only positive pulse oximetry (negative clinical examination). Positive clinical examination cases were 45 (2.7%), out of which 39 cases had only positive clinical examinations (negative pulse oximetry). Six newborns had both positive pulse oximetry and positive clinical examination. Out of the 25 diagnosed cases of congenital heart disease, ventricular septal defect (VSD) was the most common congenital heart disease, followed by patent ductus arteriosus (PDA). The sensitivity, specificity, positive predictive value, and negative predictive value of pulse oximetry were 32%, 99.5%, 50%, and 98.9% respectively. Conclusion: The community setting of a developing country, a combination of pulse oximetry screening and clinical examination are better at detecting congenital heart defects than either test alon

Neonatal outcomes and congenital anomalies in pregnancies affected by hypothyroidism

Background: Maternal hypothyroidism has been reported to have concerns over neonatal outcomes, not only in the context of neurocognitive development but also in the short term as birth weight and neonatal jaundice.Patients and methods: We conducted a cross-sectional retrospective study on 638 cases who delivered live births in the Aga Khan University Hospital after ethical approval. Data were collected on hypothyroid pregnant females who were diagnosed before conception or during their antenatal visits during the year 2008-2016. Neonatal outcomes were noted for birth weight, maturity, and neonatal jaundice, neonatal hypothyroidism, neonatal respiratory distress syndrome, sepsis, hypocalcaemia, congenital anomalies, need for intensive care admission, and neonatal death. Subgroup analysis was performed on the timing of diagnosis of maternal hypothyroidism. Data analysis was performed on Statistical Package for the Social Sciences version 20.0.Results: Neonatal jaundice was the most common neonatal outcome (37.6%) in our cohort of 662 live births. Nearly 15% required intensive care unit admission, however, neonatal death was very rare. The most common clinically significant congenital anomalies were cardiovascular defects, whereas Mongolian spots were the commonest congenital condition to report. There is a statistically significant association between low birth weight (OR 1.86, 95% CI 1.0-3.2, p ≤ 0.05) and congenital anomalies (OR 2.39, 95% CI 1.4-4.0, p ≤ 0.05) with women diagnosed with hypothyroidism before pregnancy.Conclusion: We report the neonatal outcomes and spectrum of congenital anomalies of hypothyroid pregnancies diagnosed before and during conception for the first time from the region of Pakistan.Key Message: Overall, none of the neonates of hypothyroid pregnancies developed congenital hypothyroidism.Cardiovascular defects in these neonates imply extensive screening and monitoring during pregnancy.Low birth weight and congenital anomalies are associated with the timings of diagnosis of hypothyroidism in pregnancy

Needs assessment for curricular reform of a post graduate paediatric training programme at a private university hospital in Karachi, Pakistan

In order to make and sustain changes in the curriculum, needs assessment is the key. We conducted a need assessment survey using a questionnaire based on a 7 point Likert Scale by residents and alumni. Over all response rate in the study was 77.5%. Fifty eight percent participants were females. Highest level of agreement (mean score 5.7) was found between the alumni and residents regarding the need to revise the content, teaching and assessment strategies in the programme. Twenty seven alumni (n= 38, 71%) had cleared their FCPS exams. The alumni thought that the existing teaching and assessment strategies do not facilitate in passing professional certificate exams (mean score 3.6). The mean attempts required by the alumni for clearing this exam was 3.3, and 84.2% considered their weak clinical skills as the reason for their recurrent failure

Frequency and outcome of meconium aspiration syndrome in babies born with meconium-stained liquor at secondary care hospital in Pakistan: A case series study

Background: Meconium aspiration syndrome (MAS) in the newborn is characterized by hypoxia, hypercapnia, and acidosis. MAS is a leading cause of morbidity and mortality in neonates. The primary objective of this study was to estimate the frequency of meconium aspiration syndrome (MAP) in babies born with meconium-stained liquor. The secondary outcome was to estimate the meconium aspiration syndrome; in terms of hospital stay, complications, and mortality.Methods: The study was done at Aga Khan Maternal and Child Care Centre, Hyderabad, Pakistan. Study design was case series and the duration of the study was of 6 months. All patients who fulfilled the inclusion criteria were included in the study after taking informed written consent. A brief history was taken, clinical examination was done and laboratory investigations were sent to the institutional laboratory. Study outcomes were measured from this data i.e. MAS, its complications, mortality, and a number of days in the hospital.Results: A total of 87 babies born with meconiumstained liquor at secondary care hospital were included. A total of 45 patients (52%) were males and 42 patients (48%) were females with a mean gestational age of babies 38.896 ± 1.210 weeks. The mean Apgar score at 5 minutes was 8.896 ± 0.404. MAS was present in 13 patients (14.9%). Complications were seen only one patient (1.1%) and there was no neonatal death reported.Discussion: MAS was present in 14.9% of babies; the complication of subacute bacterial infection was low seen in just 1.1% cases with no neonatal mortality

Newborn screening for congenital hypothyroidism: Improvement in short-term follow-up by audit and monitoring

Objective: Newborn screening for congenital hypothyroidism (CH) at our hospital during this study was by measurement of thyroid stimulating hormone (TSH) in serum samples. This audit was conducted over a 2 year period, to determine the compliance of reporting of results greater than the screening cutoffs for serum TSH. Gaps of non-compliance were identified, and re-audit was undertaken after the corrective actions were taken.Results: The critical limit was defined as serum TSH (≥ 20 µIU/ml) following consultation with a pediatric endocrinologist. All results above this limit were reported urgently to physicians. During the audit period, 27,407 tests were performed, 0.7% had a value of ≥ 20 µIU/ml, of those only 62% were reported to the general paediatrician or neonatologist. Reasons for not reporting results included non-availability of contact information, lack of policy awareness by technologists, critical results not highlighted on the computer display, and absence of regular monitoring. Corrective measures were taken, and re-audit was done. During the re-audit period, a total of 22,985 tests was performed, 0.6% had a value of ≥ 20 µIU/ml. Of these, 77% were reported to the general paediatrician or neonatologist. Critical result reporting was improved after the audit, and further enhanced the laboratory service of CH screening

Establishing reference interval for thyroid-stimulating hormone in children below two-year ages in Pakistani population

Introduction: Reference intervals (RIs) of thyroid-stimulating hormone (TSH) and free thyroxine (FT4) are age, assay and population specific. Currently, the age and assay-specific RIs for TSH are not available for children under two years of age. This study aimed to establish reference intervals for serum concentrations of TSH and FT4 in healthy children aged 1-24 months as per CLSI C28-A3 guidelines.Methods: This prospective cross-sectional study was conducted in children from 1 to 24 months visiting the clinical laboratory for serum vitamin D testing but without any recent illness, hospitalization, medication and history of maternal thyroid diseases from August 2018 to March 2019 were invited to participate in the study.Serum TSH and FT4 were measured on ADVIA Centaur (Siemens Diagnostics, US), using chemiluminescence immunoassay. Kolmogorov-Smirnov test assessed normality of the data and RIs based on central 95% of the population were established using the non-parametric approach.Results: After excluding one subject with confirmed congenital hypothyroidism, a total of 131 children were included in the study. The median (IQR) age of the study subjects was 12 months (11), and majority 78 (59.5%) were boys. The RIs were established using non-parametric approach as the data was not normally distributed. Reference interval for TSH was 0.73-4.94 μIU/mL and for FT4 was 0.81-1.51 ng/dl.Conclusion: We established assay-specific RIs for serum TSH and FT4 in children aged 1-24 months in our population. The RIs were slightly lower from RIs developed on other platforms in different population

Lifestyle changes and glycemic control in type 1 diabetes mellitus: A trial protocol with factorial design approach

Background: Type 1 diabetes (T1D) has been increasing globally over the past three decades. Self-monitoring of blood glucose is a challenge in both developed as well as developing countries. Self-management guidelines include maintaining logbooks for blood glucose, physical activity, and dietary intake that affect glycated hemoglobin (HbA1c) and a multitude of life-threatening acute complications. Innovative, cost-effective interventions along with beneficial lifestyle modifications can improve home-based self-monitoring of blood glucose in T1D patients. The overall objective of this study is to evaluate the relationship between maintaining log books for blood glucose levels, reinforcement by e-messages, and/or daily step count and changes in HbA1c.Methods/design: A randomized controlled trial will enroll participants aged 15 years and above in four groups. Each group of 30 participants will be working with a newly designed standard log book for documenting their blood glucose. The first group will be entirely on routine clinical care, the second group will be on routine care and will receive an additional e-device for recording step count (fit bit), the third group will receive routine care and daily motivational e-messages to maintain the log book, and the fourth group along with routine care will receive an e-device for measuring step count (fit bit) and e-messages about maintaining the log book. Patients will be enrolled from pediatric and endocrine clinics of a tertiary care hospital in Karachi. All groups will be followed up for a period of 6 months to evaluate outcomes. Log book data will be obtained every 3 months electronically or during a patient\u27s clinic visit. HbA1c as a main outcome will be measured at baseline and will be evaluated twice every 3 months. A baseline questionnaire will determine the socio-demographic, nutritional, and physical activity profile of patients. Clinical information for T1D and other co-morbidities for age of onset, duration, complications, hospitalizations, habits for managing T1D, and other lifestyle characteristics will be ascertained. Behavioral modifications for maintaining daily log books as a routine, following e-messages alone, fit bit alone, or e-messages plus using fit bit will be assessed for changes in HbA1c using a generalized estimated equation.Discussion: The proposed interventions will help identify whether maintaining log books for blood glucose, motivational e-messages, and/or daily step count will reduce HbA1c levels

Evaluation the effectiveness of abridged IMNCI (7-Day) course v standard (11-Day) course in Pakistan

Background: The conventional IMCI training for healthcare providers is delivered in 11 days, which can be expensive and disruptive to the normal clinical routines of the providers. An equally effective, shorter training course may address these challenges.Methods: We conducted a quasi-experimental study in two provinces (Sindh and Punjab) of Pakistan. 104 healthcare providers were conveniently selected to receive either the abridged (7-day) or the standard (11-day) training. Knowledge and clinical skills of the participants were assessed before, immediately on conclusion of, and six months after the training.Results: The improvement in mean knowledge scores of the 7-day and 11-day training groups was 31.6 (95% CI 24.3, 38.8) and 29.4 (95% CI 23.9, 34.9) respectively, p = 0.630 while the improvement in mean clinical skills scores of the 7-day and 11-day training groups was 23.8 (95% CI: 19.3, 28.2) and 23.0 (95% CI 18.9, 27.0) respectively, p = 0.784. The decline in mean knowledge scores six months after the training was - 12.4 (95% CI - 18.5, - 6.4) and - 6.4 (95% CI - 10.5, - 2.3) in the 7-day and 11-day groups respectively, p = 0.094. The decline in mean clinical skills scores six months after the training was - 6.3 (95% CI - 11.3, - 1.3) in the 7-day training group and - 9.1 (95% CI - 11.5, - 6.6) in the 11-day group, p = 0.308.Conclusion: An abridged IMNCI training is equally effective as the standard training. However, training for certain illnesses may be better delivered by the standard course

Nash-wo-Numa (childhood growth & development) study protocol: Factors that impact linear growth in children 9 to 15 years of age in Matiari, Pakistan

Introduction: Adolescence is a time of significant physical and emotional change, and there is emerging concern that adolescents living in low- and middle-income countries (LMIC) may face substantial challenges in relation to linear growth and mental health. Data on the global burden of stunting after 5 years of age are limited, but estimates suggest up to 50 per cent of all adolescents in some LMIC are stunted. Additionally, many LMIC lack robust mental health care delivery systems. Pakistan has one of the world\u27s largest populations of adolescents (10 to 19 years) at approximately 40 million. The Nash-wo-Numa study\u27s primary objective is to assess the prevalence and risk factors for stunting among early adolescents in rural Pakistan. The study also aims to determine the prevalence of poor mental health and identify factors associated with common mental health concerns during the childhood to adulthood transition.Methods: This cross-sectional study will include girls (n= 738) 9.0 to 14.9 years of age and boys (n=687) 10.0 to 15.9 years of age who live in the rural district of Matiari, Pakistan. Participants will be assessed for anthropometrical measures, puberty development, nutritional biomarkers as well as symptoms of depression, anxiety and trauma using validated scales.Ethics and Dissemination: The proposed study aims to complete the picture of child and adolescent health concerning linear growth and mental health by including puberty indicators. Ethics approval has been granted by the Ethics Review Committee at the Aga Khan University, Karachi, Pakistan, #5251-WCH-ERC-18 and Research Ethics Board at SickKids Hospital, Toronto, Canada, #:1000060684. Study results will be presented at relevant conferences and published in peer-reviewed journals