PREVALENCE OF GLUCOSE-6-PHOSPHATE DEHYDROGENASE DEFICIENCY AMONG HOSPITALIZED CHILDREN AT TERTIARY CARE HOSPITAL IN EASTERN PART OF NEPAL

of this study is to ﬁnd out the hospital prevalence of G6PD deﬁciency in hospitalized children at Birat Medical College teaching Hospital. This is a hospital based cross seconal study carried out in the Department of Paediatric, Birat Medical College Teaching Hospital from 30th November 2020 to 30th May 2021. Three hundred children upto to ten years of age admied in department of pediatrics were included in this study. This study was performed on hospitalized children who were screened for G6PD deﬁciency. The test was carried out using the Randox G6PD quantave in vitro test to determine the prevalence of G6PD deﬁciency among the admied children upto 10 years of age. Data was analysed using SPSS version 16. This study was performed on 300 children, in which male babies (n=192; 64%) outnumbered the female babies (n=108; 36%). The majority of children were in the age group of < 1 years (n=131; 43.7%).The overall prevalence of G6PD deﬁciency was 9.3% of which 96.4% were moderately deﬁcient while 3.6% was severely deﬁcient. The highest proporon was noted in the age group of 1 to 5 years. The frequency of this disorder in males and females were 13.5% and 1.9% respecvely which was stascally signiﬁcant (p=0.001). The present study conﬁrms the high prevalence of G6PD deﬁciency in eastern region of Nepal. Therefore, we need to establish roune screening and educaonal programs in order to prevent grave complicaons in future. ABSTRACT


Introduc on
Glucose 6 phosphate dehydrogenase (G6PD) deficiency is the most significant enzyme defect. It is an X linked inherited disorder that affects males and females are rarely affected by lyoniza on. Severe jaundice, anemia and hemoly c crisis following inges on of fava beans and certain drugs are known to occur in children with G6PD deficiency. Therefore, rou ne neonatal and child screening programs to facilitate the iden fica on and effec ve management of children with G6PD deficiency is paramount.

Objec ve
The main objec ve of this study is to find out the hospital prevalence of G6PD deficiency in hospitalized children at Birat Medical College teaching Hospital.

Methodology
This is a hospital based cross sec onal study carried out in the Department of Paediatric, Birat Medical College Teaching Hospital from 30th November 2020 to 30th May 2021. Three hundred children upto to ten years of age admi ed in department of pediatrics were included in this study. This study was performed on hospitalized children who were screened for G6PD deficiency. The test was carried out using the Randox G6PD quan ta ve in vitro test to determine the prevalence of G6PD deficiency among the admi ed children upto 10 years of age. Data was analysed using SPSS version 16.

Result
This study was performed on 300 children, in which male babies (n=192; 64%) outnumbered the female babies (n=108; 36%). The majority of children were in the age group of < 1 years (n=131; 43.7%).The overall prevalence of G6PD deficiency was 9.3% of which 96.4% were moderately deficient while 3.6% was severely deficient. The highest propor on was noted in the age group of 1 to 5 years. The frequency of this disorder in males and females were 13.5% and 1.9% respec vely which was sta s cally significant (p=0.001).

Conclusion
The present study confirms the high prevalence of G6PD deficiency in eastern region of Nepal. Therefore, we need to establish rou ne screening and educa onal programs in order to prevent grave complica ons in future.

INTRODUCTION
G6PD deficiency is a gene c disorder involving more than 1 400 million popula on worldwide. It is an X linked inherited disorder and males are more commonly affected than the 2 females. The main source of energy for the red cell is glucose, which is metabolized by two major routes; the hexose monophosphate 1 (HMP) shunt and the glycoly c pathway. G6PD is a cytoplasmic enzyme in the pentose monophosphate pathway and catalyzes the conversion of nico namideadenine dinucleo de phosphate (NADP) to its reduced form, NADPH which helps to protect the red blood cells (RBC) from oxida ve 3 damage. Therefore, in pa ents who are deficient in G6PD, the RBCs are damaged and undergo lysis, leading to acute hemolysis. The term favism is used to describe hemolysis triggered by inges on of fava beans in G6PD deficient 4 individuals and it runs in families. Based on the percentage of G6PD enzyme ac vity, the deficiency is categorized as moderate (<30% ac vity) or severe (<10% ac vity). There are certain triggers in the form of foods (Fava beans), pollen inhala on, drugs (primaquine, chloramphenicol and aspirin) or chemicals (Henna, Naphthalene) or infec ons which lead to severe haemolysis in such a situa on and urgent blood transfusion may be 2 required. The enzyme G6PD deficiency contributes to neonatal jaundice which is accompanied by hyperbilirubinemia and leads infants at risk for kernicterus within the first few days of life. Many children with this enzyme deficiency are healthy during childhood and becomes symptoma c a er exposure to a pro-oxidant medica ons such as an -malarial drugs which lead to hemolysis and resultant severe anemia, 5 heart failure, and even death if not recognized early. Therefore, screening and detec on of G6PD deficiency helps in reducing such episodes through appropriate selec on of treatment, pa ent counseling and abs nence from disease precipita ng drugs. In Asia, the prevalence of deficiency ranges from 6.0% to 6 15.8%. WHO recommends popula on screening in regions where the prevalence of G6PD deficiency is 3-5% or more, but this has yet to become rou ne prac ce in Nepal. The barriers to screening include cost, under es ma on of the public health impact of G6PD deficiency by the medical community, lack of awareness of G6PD deficiency among people and a paucity of guidelines regarding which high risk groups should be preferen ally screened when general 5 popula on screening is notpossible. There is a paucity of data on G6PD deficiency among children in the Koshi zone of Nepal. This study was specifically aimed at determining the prevalence of G6PD deficiency in children visi ng the Pediatric unit of Birat Medical College and Teaching Hospital for pediatric related care. The research findings may be useful for the pediatrician to make comprehensive management plan at an early stage while dealing with children with G6PD deficiency. The main objec ve of this study is to determine the prevalence rate of G6PD deficiency in children so that the complica ons can be prevented.

METHODOLOGY
This is a hospital based cross sec onal study carried out th th from 30 November 2020 to 30 May 2021 in the department of Pediatric, Birat Medical College and Teaching Hospital. Ethical clearance was obtained from the Ins tu onal Review Commi ee (IRC) of the ins tute to carry out the study. The study was conducted on 300 children out of which 192 (64%) were males and 108 (36%) were females. The informed consent was taken from their parents. The parents of children unwilling to give consent, children older than 10 years of age and samples with incomplete data were excluded. In this study all the children admi ed in pediatrics and neonatal ward upto the age of ten years were included. The ethylene diaminetetraace c acid (EDTA)-an coagulated blood was used for the screening of the subjects for G6PD deficiency using the Randox G6PD quan ta ve in vitro test. Red blood cell G6PD value of ≥2.9 U/gHb was regarded as normal. Children with red blood cell G6PD value of <2.9 U/gHb were regarded as deficient, while those with red blood cell G6PD value of <1.6 U/gHb were regarded as severely 6 deficient. The data was recorded in a pre designed proforma and data analysis was done using sta s cal package of social science (SPSS) version 16. Numerical variables were reported in terms of mean and standard devia on. Categorical variables were reported in terms of numbers and percentages.

RESULTS
The present study cons tuted a total of 300 cases, in which male children (n=192; 64%) outnumbered the female children (n=108; 36%). The majority of children were in the age group of < 1 years (n=131; 43.7%) followed by 115 ( 38.3%) cases in the age group of 1 to 5 years and 54 ( 18% ) cases in the age group of 6 to 10 years. ( Table 1)    Chi square value 11.16 p value= 0.001 Figure 1 shows the percentage of children with G6PD level. Of the 300 children tested, 28(9.3%) were G6PD deficient and 272 ( 90.7%) cases had normal G6PD level. Out of 28 G6PD deficient children, twenty seven (96.4%) were moderately deficient while one (3.6%) was severely deficient.
The overall hospital prevalence of G6PD deficiency in this period was 9.3%, with a prevalence of 13.5% among males and 1.9% among females. The p value was 0.001 which was sta s cally significant (Table 2) The highest prevalence of G6PD deficiency occurred among children in the 1-5 years age group which was 53.6 % followed by 39.3% cases in the age group of less than 1 year and 7.1% cases in the age group of 6-10 years . (Table 3)

DISCUSSION
Glucose-6-phosphate dehydrogenase is the most common 7 human enzyme deficiency worldwide. The clinical expression of G6PD deficiency encompasses a spectrum of disease severity related to the ability of red cells to generate 8 NADPH. were 24.1%, 22.1% and 13.79% respec vely. However, in a study conducted by al-Abdulkareem et al, the female popula on had a higher rate of G6PD deficiency (13.5%). 10 This further reconfirms the natural history of G6PD deficiency of being an X linked recessive disorder, as well as the fact that only male hemizygotes and female homozygotes are the individuals most o en affected. However, females can also be clinically affected because of the skewed lyoniza on of the X chromosomes.
In this study, a significant number (n=15;53.6%) of children were in the 1-5 years age group. This finding is consistent with earlier studies conducted by Albagshi et al and Hassan et al where the highest prevalence occurred among the children in the 2-5 years age group with the percentage being 24.7% and 43.4% respec vely which emphasizes the need to set up neonatal and child screening programs to facilitate the iden fica on and effec ve management of 4,9 children with G6PD deficiency. In this study, the overall prevalence of G6PD deficiency was 9.3%. Our finding is consistent with earlier study conducted in Nepal by Gautam  WHO recommends popula on screening in regions where the prevalence of G6PD deficiency is 3-5% or more, but this has yet to become rou ne prac ce in many parts of Nepal. The barriers to screening include cost, underes ma on of the public health impact of G6PD deficiency by the medical community, lack of awareness of G6PD deficiency among people and a paucity of guidelines regarding which high risk groups should be preferen ally screened when general 5 popula on screening is not possible.
As a significant propor on of G6PD deficient people were reported in our study and as G6PD deficient children are highly vulnerable to life threatening hemolysis, screening tests implementa on and educa onal programs are warranted. Newborn screening (NBS) program needs to be implemented to determine the true prevalence prospec vely. This program will, not only determine the prevalence of G6PD deficiency, but will also plan for future monitoring for jaundice, to prevent acute encephalopathy from hyper bilirubinemia encephalopathy, and consequent observa on for future hemoly c episodes. The management of G6PD enzyme deficiency should include avoidance of drugs and foods that predispose to hemolysis, provision of safe red cell transfusion to manage acute hemolysis in acutely affected children, and facility of dialysis services to treat acute renal failure.

CONCLUSION
The prevalence of G6PD deficiency was higher among childrens in the age group of 1 to 5 years with male preponderance. WHO recommends popula on screening in regions where the prevalence of G6PD deficiency is 3-5% or more, but this has yet to become rou ne prac ce in Nepal. The barriers to screening include cost, under es ma on of the public health impact of G6PD deficiency by the medical community, lack of awareness of G6PD deficiency among people and a paucity of guidelines regarding which high risk groups should be preferen ally screened when general popula on screening is not possible. There is a need for the rou ne screening and educa onal programs for G6PD deficiency in our se ng to prevent grave complica ons in future. Further analysis of risk factors of drugs, food and family history should be done.

RECOMMENDATION
In the view of higher prevalence of G6PD deficiency in the children of 1 to 5 years age group, rou ne screening and educa on program on a regular basis needs to be considered.

LIMITATIONS OF THE STUDY
An important limita on to our study is the small number of pa ents and shorter dura on. Our study reflects data from one center only and may not represent that of other centers across the country. Hence, mul center trials would be necessary to determine the prevalence of G6PD deficiency in children. Also gene c analysis was not done in our study.