Frequency and mortality associated with hyperglycemia in critically Ill children

Objective: To determine the frequency of hyperglycemia in critically ill children admitted in PICU of a tertiary care hospital of Karachi and to compare the mortality of critically ill children with and without hyperglycemia. Study Design: Cross-sectional study. Place and Duration of Study: Paediatrics Intensive Care Unit (PICU) of National Institute of Child Health (NICH), Karachi, from November 2011 to April 2012. Methodology: One hundred fifty critically ill children admitted to PICU were included. Patients who had fasting blood sugar levels more than 126 mg/dl within 48 hours of admission were included in the hyperglycemic group. The normoglycemic and hyperglycemic groups were followed till 10 days to determine the mortality associated with hyperglycemia. Results: Out of 150 patients, 82 (54.7%) had hyperglycemia. Mortality rate was 48.7% (n=73/150). However, mortality rate was significantly high 57.3% (n=47) in hyperglycemic patients than non-hyperglycemic patients (p=0.019). Conclusion: The presence of stress-induced hyperglycemia in critically ill patients is a well established marker of poor outcome, and a very high mortality rate. Normoglycemia was associated with favorable outcomes in terms of hospital stay and mortality

Safety of insulin tolerance test for the assessment of growth hormone deficiency in children

OBJECTIVE: To determine the safety of insulin tolerance test (ITT) for assessing growth hormone (GH) deficiency in children. METHODS: This hospital based study was conducted at the National Institute of Child Health, Karachi from 1st November 2008 till 30th October 2009. All children suspected of growth hormone deficiency, were included after excluding all other causes of short stature. Verbal informed consent was taken from the parents. Children less than 2 years of age, weighing less than 10 kg, untreated/inadequately treated hypothyroidism or Addison\u27s disease, epilepsy, having history of hypoglycaemic fits or cardiac disease were excluded. All children were subjected to the international standard protocol of ITT and their samples of growth hormone and blood sugars were drawn. Complications during the procedure like hypoglycaemia, hypothermia, loss of consciousness, fits, vomiting and failure to achieve hypoglycaemia were recorded. Insulin tolerance test was performed on a total of 168 subjects. The data was entered in SPSS version 17 for analysis. RESULTS: A total of 168 children were subjected to the ITT. Four of them were abandoned as they could not achieve hypoglycaemia despite repeating the dose of insulin. Results were analyzed on 164 children whose mean age was 10 +/- 3.5 years, There were 96 (58%) males and 68 (41%) females. Over all 79.8% children achieved hypoglycaemia. None of the subjects developed any complication (fits, loss of consciousness,) or required intravenous glucose during the test and it was completed in all children with close monitoring. The results showed that there was a significant effect of time after insulin administration on both the blood glucose level (BG) and growth hormone (GH) levels. The blood glucose level decreased rapidly after administration of insulin and was lowest 30 minutes after injection and showed an increasing trend in subsequent readings, becoming almost equal to the baseline value 120 min after injection. From the study group 111 (66%) children were diagnosed as having growth hormone deficiency, 52 (31.3%) were normal and 1 (0.6%) had growth hormone insensitivity. CONCLUSION: ITT in children was found to be a safe and reliable test but can be potentially dangerous and requires very close monitoring and supervision and should be performed in a center with experienced staff

Comparison of Body Composition Bio Electrical Impedance Analysis of Type-1 Diabetes vs. Non-Diabetes in Children and Adolescent

Background: Analyzing the body composition of children and adolescents with diabetes is becoming more and more popular. For managing weight changes that may emerge from treatment and evaluating treatment response, it is essential to comprehend the body composition of diabetic children. Objective: To compare body composition by Bioelectrical Impedance Analysis (BIA) among T1DM versus healthy children and adolescents in Pakistan. Methods: This comparative cross-sectional study was performed during Jan-March, 2023. Type 1 diabetic children and adolescents were enrolled from the pediatric endocrinology outpatient clinic of the National Institute of Child Health whereas healthy children and adolescents were enrolled from a nearby school to the hospital location. Evaluation of anthropometry indices and bioelectrical impedance analysis was performed after obtaining the consent of parents. Results: A total of 100 subjects were enrolled in the study, 50 each in the diabetic and healthy group. The mean age of participants was 10.4 ± 2.4 years. The majority of study subjects were females (61%). The mean duration of T1DM was 4.7 ± 0.8 years. Height (127.4 ± 11.7 versus 139.5 ± 15, p<0.001), Waist-hip ratio (0.8 ± 0.1 versus 0.7± 0.3, p=0.037) and resistance (686.2 ± 90.7 versus 651.1 ± 96.6, p=0.002) were significantly higher among T1DM group than healthy group. Percentage of muscle mass (45.1 ± 8.2 versus 50.3 ± 7.6, p=0.008), body cell mass (47.1 ± 3.8 versus 50.6 ± 4.5, p=0.045), reactance (56.3 ± 9.8 versus 62.4 ± 4.2, p=0.017) and phase angle (4.3 ± 0.9 versus 5.7 ± 0.6, p=0.012) were significantly lower in T1DM patients than healthy individuals. Conclusion: BIA analysis showed that body composition parameters and body functional status were lower among T1DM children and adolescents than in the healthy group in terms of resistance, reactance, and phase angle

Bone Mineral Density in Children with Congenital Adrenal Hyperplasia

Background: Treatment in all forms of Congenital Adrenal Hyperplasia (CAH) includes lifelong replacement of steroids. Steroids have an impact on bone health in multiple ways and are known to cause osteoporosis when given in high doses or for a longer duration. Objective: To evaluate bone mineral density (BMD), using dual-energy X-ray absorptiometry (DEXA) scan in children with CAH taking long-term steroids presenting in the pediatric endocrinology ward of National Institute of Child Health, (NICH) Karachi, Pakistan. Materials and Methods: This cross-sectional study was performed at the Department of Pediatric Endocrinology, National Institute of Child Health, Karachi from October 2021 to July 2022. A total of 47 diagnosed cases of CAH taking steroids for more than 5 years were enrolled. Assessment of BMD was done using a DEXA scan. Lumbar spine BMD was done and Z-score was modified for height for age z-score. The dose of steroids and duration was calculated. Results: Out of 47 patients, low BMD was observed in 8 (17.02%) patients. Individuals with low BMD had significantly higher median duration, (p=0.017), dose (p=0.003), and median alkaline phosphate level (p=0.036),) but low median BMD value (p=0.009) and z score (p<0.001) than normal BMD individuals. Although median bone age (p=0.009) was appropriate for chronologic age in low BMD patients. A moderate negative significant correlation was observed between z score and age (rho=-0.319, p=0.029), z-score and duration of steroid treatment (rho=-0.364, p=0.012), z score and alkaline phosphate (rho=-0.466, p=0.001), z score and bone age (rho=-0.378, p=0.009). Conclusion: Low BMD was observed in 17% of children on the DEXA scan. Moreover, these individuals had significantly higher median average duration and dose of hydrocortisone

Growth Characteristics in Children with Congenital Adrenal Hyperplasia Visiting Tertiary Care Hospital, Karachi

Objective: To determine the growth characteristics in children with congenital adrenal hyperplasia visiting tertiary care hospital Karachi. Study Design: Cross-Sectional study. Place and Duration of Study: Paediatrics Department, National Institute of Child Health, Karachi Pakistan, from Nov 2018 to May 2019. Methodology: All patients who visited the OPD were included in the study. Growth characteristics like underweight, normal BMI, overweight, obese and short stature were recorded. This information, along with the age of diagnosis, family history of CAH, parental consanguinity, family history of CAH or any abortion or prenatal death, were noted. Results: The mean age of the patients was 7.40±2.34 years. Out of 69 congenital adrenal hyperplasia patients, 41(59.4%) were males, and 28(40.6%) were females. The short suture was noted in 53(76.8%) patients. 19(27.5%) were underweight, 6(8.7%) were normal weight, 10(14.5%) were overweight, and 34(49.3%) patients w ere obese. Conclusion: A significant effect of congenital adrenal hyperplasia was documented in the children

Perceptions of health professionals regarding minimally invasive tissue sampling (MITS) to identify the cause of death in stillbirths and neonates: Results from a qualitative study

Background: Pakistan is considered to be one of the riskiest places in the world for childbirth as measured by its high stillbirth and neonatal mortality rates. Complete diagnostic autopsy remains the gold standard to determine the cause of death (CoD); however, it is not routinely implemented due to religious objections, sociocultural beliefs, limited resources and low demand from physicians and families. Recently, minimally invasive tissue sampling (MITS) using needle biopsies of multiple tissues to obtain tissue for histological examination and organism identification with PCR has been developed and promoted to determine CoD in low-resource areas. To ensure successful implementation of MITS, it is important to understand health professionals\u27 attitudes and perceptions related to MITS.Methods: A qualitative study was conducted at the National Institute of Child Health (NICH), Karachi, Pakistan. Focus group discussions (FGDs) and Key-informant interviews (KIIs) were conducted with health professionals including doctors, nurses, trainees, clinicians, bioethics experts and public health experts to explore their perceptions and views on acceptability of MITS. Data were analyzed using NVivo 10 software.Results: A total of 12 interviews (FGDs = 4; KIIs = 8) were conducted. Four overarching themes were identified: (I) acceptability of MITS; (II) perceived benefits of the MITS procedure; (III) factors facilitating the implementation of MITS; and (IV) health system requirements for implementing the MITS procedure. Generally, MITS was considered as a positive development for the health system. Diagnostic accuracy and identification of less common causes of death were highlighted as two main benefits of the MITS procedure. The study highlighted a number of facilitators for the acceptability of MITS including effective counseling, building trust with parents, fast procedure time, and approaching families within a few hours of death. In addition, lack of skilled staff, poorly equipped healthcare facilities and the potential high cost to conduct MITS were identified as challenges for the implementation of MITS.Conclusions: This formative research provided a unique opportunity to explore health professionals\u27 views and attitudes towards the MITS procedure. Such insights are crucial to ensure successful implementation and integration of a new technique into the existing health system. The research identified the factors influencing the acceptability of MITS among health professionals in Pakistan. The study also informed factors that could help facilitate the implementation of the MITS procedures in the context of Pakistan and similar settings

Perceptions of parents and religious leaders regarding minimal invasive tissue sampling to identify the cause of death in stillbirths and neonates: Results from a qualitative study

Background: Recently, the minimal invasive tissue sampling (MITS) procedure has been developed to support determination of the cause of death as an alternate to conventional autopsy, especially in countries where complete diagnostic autopsy is not routine. To assess the feasibility of implementation of the MITS procedure for a study to determine cause of death in premature births and stillbirths in south Asia, we explored the views and perceptions of parents and religious leaders on the acceptability of MITS.Methods: A qualitative study was conducted at the National Institute of Child Health (NICH) hospital of Karachi, Pakistan. Focus group discussions (FGDs) were conducted with parents of newborns who visited well-baby clinics of the NICH hospital for post-natal check-ups. Key-informant interviews (KIIs) were conducted with religious leaders. Data were analyzed using NVivo 10 software.Results: A total of 13 interviews (FGDs = 8; KIIs = 5) were conducted. Three overarching themes were identified: (I) acceptability of MITS; (II) concerns affecting the implementation of MITS; and (III) religious and cultural perspectives. Participants\u27 acceptance of MITS was based on personal, religious, cultural and social beliefs. Parents widely recognized the need for this procedure in cases where the couple had experienced multiple stillbirths, neonatal deaths and miscarriages. Counseling of parents was considered vital to address emotional concerns of the parents and the family. Religious leaders indicated acceptability of the MITS procedure from a religious perspective and advised that respect for the deceased and consent of the guardians is mandatory when performing MITS.Conclusions: This qualitative study provided a unique opportunity to understand the views of parents and religious leaders towards the use of MITS. Generally, MITS appears to be an acceptable method for identifying the cause of death in neonates and stillbirths, provided that the deceased is respected and buried as soon as possible without any delays and parents are counseled appropriately. Findings from this research are essential in approaching families for consent for MITS

Gonadotropin independent sexual precocity in a Pakistani male infant from an activating mutation in LHCGR gene

A male child, aged seven months, visited the out patients clinic of the National Institute of Child Health, Karachi, in May 2020 with the features of iso-sexual puberty. After ruling out the more common causes of early puberty, like congenital adrenal hyperplasia and tumours secreting chorionic gonadotropin hormone, hormonal assessment indicated raised testosterone independent of gonadotropin. The volume of the testicles was symmetric and testicular ultrasonography revealed no mass. Genetic analysis for the LHCGR gene was performed for confirmation which revealed activating heterozygous missense pathogenic mutation in c.1732G>T (p.Asp578Tyr). This is the first reported case of testotoxicosis (FMPP) from Pakistan which was genetically confirmed. Keywords: Testosterone, Puberty, Gonadotrophins, Mutation, Genetic

Early suspicion can save lives; mucormycosis in two children with diabetic ketoacidosis in Pakistan.

Background: Mucormycosis is a cause of fulminant necrotizing fungal infection in children with underlying immunocompromising condition. Rhino-orbito-cerebral infection is the most common form of mucormycosis in children with uncontrolled diabetes mellitus or diabetic ketoacidosis. Once considered a rare entity is now increasingly being reported especially in the diabetic population. The initial presentation can mimic a bacterial infection, thus a high index of suspicion is needed for timely intervention to reduce morbidity and mortality. Case report: We present a case of rhino-orbito-cerebral mucormycosis in two patients with diabetic ketoacidosis for the first time from Pakistan. Both the patients couldn’t survive due to late and extensive disease on presentation. Keywords: mucormycosis; rhino-orbito-cerebral mucormycosis; diabetic ketoacidosis; diabetes mellitus, pediatric; childre

Perceptions of parents and healthcare professionals regarding minimal invasive tissue sampling to identify the cause of death in stillbirths and neonates: a qualitative study protocol

Abstract Background Globally, around 2.6 million neonatal deaths occur world-wide every year and the numbers of stillbirths is almost similar. Pakistan is ranked among the highest countries in the world for neonatal mortality. In 2016, for every 1000 babies born in Pakistan, 46 died before the end of the first month of life. Also, Pakistan had the highest rate of stillbirths (43.1/1000 births) in 2015. To meet sustainable development (SDG) targets of reducing neonatal mortality and stillbirths, it is essential to gain understanding about the causes of neonatal death and stillbirths. In Pakistan, full autopsies are conducted only in medico-legal cases and are very rarely performed to identify a definitive cause of death (CoD) and because of cost and insufficient staff are generally not feasible. Recently, minimally invasive tissue sampling (MITS) has been used to determine CoD in neonates and stillbirths as it addresses some of the socio-cultural and religious barriers to autopsy. However, it is not known how families and communities will perceive this procedure; therefore, exploring family and healthcare professionals’ perceptions regarding MITS is essential in determining acceptable and feasible approaches for Pakistan. Methods The study will employ an exploratory qualitative research design. The study will be conducted at the National Institute of Child Health (NICH) hospital of Karachi. The data collection method will consist of key-informant interviews (KIIs) and focus group discussions (FGDs). FGDs will be conducted with the families and relatives of newborns who are visiting the outpatient department (OPD) and well-baby clinics of NICH hospital. KIIs will be conducted with the NICH - medical director, healthcare providers, professionals involved in proceedings related to death and dying, religious leaders, health sector representatives from the government, public health experts, maternal and child health (MCH) specialists, obstetricians and neonatologists and experts from the bioethics committee. Study data will be analyzed using NVivo 10 software. Discussion The research will help explore specific cultural, religious and socio-behavioral factors that may increase or decrease the acceptability of MITS for identifying COD in neonates and stillbirths. The findings of the qualitative study will provide a better understanding of parents’ and healthcare professionals’ attitudes towards the use of MITS on neonatal deaths and stillborns