A 6-Day-Old Male Infant with Severe Hyperbilirubinemia Diagnosed with Hereditary Spherocytosis at a Tertiary Hospital in East Java, Indonesia: A Diagnostic and Management Challenge in a Developing Country.

BACKGROUND Hereditary spherocytosis (HS) is an autosomal dominant inherited disorder that causes severe hyperbilirubinemia in neonates. There is no factual data about the prevalence in Indonesia. It is common that neonates with suspected hereditary spherocytosis are not diagnosed or treated adequately in developing countries such as Indonesia. CASE REPORT A 6-day-old baby was referred from a secondary public hospital to our tertiary hospital in Malang, East Java with severe hyperbilirubinemia unresponsive to the 2 days of conventional phototherapy. Initial laboratory examination showed total serum bilirubin level 28.83 mg/dL and indirect bilirubin level 25 mg/dL. Complete blood count showed hemoglobin level of 10.3 g/dL with high MCHC 36.9 g/dL and increased RDW 18.7%. The HS ratio (MCHC per MCV) was 0.41. The blood smear showed spherocytes with positive family history from the mother and grandmother. There were no specific tests such as EMA binding, cryohemolysis, or analysis of erythrocyte membrane protein available in our hospital. The patient was then treated with 2 sessions of intensive phototherapy with phototherapy unit bilisphere 360 LED. The total serum bilirubin level dropped to 12.19 mg/dL. In this case, we decided to perform intensive phototherapy first, not only because of facility-based constraints to do timely exchange transfusion, but also due to the low socio-economic and educational background of the parents. CONCLUSIONS There are some challenges in diagnosing and treating HS adequately in Indonesia. Limitations of specific tests, inadequacy of conventional phototherapy, lack of awareness of and adherence to guidelines, and facility-based inability to perform timely exchange transfusion all can contribute to severe hyperbilirubinemia and its sequelae.

Corrigendum to "The knowledge of Indonesian pediatric residents on hyperbilirubinemia management" [Heliyon 7 (4) (2021) e06661].

[This corrects the article DOI: 10.1016/j.heliyon.2021.e06661.].

The knowledge of Indonesian pediatric residents on hyperbilirubinemia management.

Hyperbilirubinemia in the newborn occurs more frequently in Indonesia. Therefore, it is important that pediatric residents in Indonesia acquire adequate knowledge of hyperbilirubinemia management. This study aims to determine the pediatric residents' knowledge on hyperbilirubinemia management, whether they follow recommended guidelines, and whether differences exist between five large Indonesian teaching hospitals. We handed out a 25-question questionnaire on hyperbilirubinemia management to pediatric residents at five teaching hospitals. A total of 250 questionnaires were filled in completely, ranging from 14 to 113 respondents per hospital. Approximately 76% of the respondents used the Kramer score to recognize neonatal jaundice. Twenty-four percent correctly plotted the total serum bilirubin levels (TSB) on the phototherapy (PT) nomograms provided by the American Academy of Pediatrics (AAP) and the National Institute for Health and Care Excellence (NICE) for full-term and nearly full-term infants. Regarding preterm infants <35 weeks' gestational age, 66% of the respondents plotted TSB levels on the AAP nomogram, although this nomogram doesn't apply to this category of infants. Seventy percent of residents knew when to perform an exchange transfusion whereas 27% used a fixed bilirubin cut-off value of 20 mg/dL. Besides PT, 25% reported using additional pharmaceutical treatments, included albumin, phenobarbitone, ursodeoxycholic acid and immunoglobulins, while 47% of the respondents used sunlight therapy, as alternative treatment. The limited knowledge of the pediatric residents could be one factor for the higher incidence of severe hyperbilirubinemia and its sequelae. The limited knowledge of the residents raises doubts about the knowledge of the supervisors and the training of the residents since pediatric residents receive training from their supervisors.

Multicentre survey of retinopathy of prematurity in Indonesia.

Background: The incidence of retinopathy of prematurity (ROP) is higher in Indonesia than in high-income countries. In order to reduce the incidence of the disease, a protocol on preventing, screening and treating ROP was published in Indonesia in 2010. To assist the practical implementation of the protocol, meetings were held in all Indonesia regions, calling attention to the high incidence of ROP and the methods to reduce it. In addition, national health insurance was introduced in 2014, making ROP screening and treatment accessible to more infants. Objective: To evaluate whether the introduction of both the guideline drawing attention to the high incidence of ROP and national health insurance may have influenced the incidence of the disease in Indonesia. Setting: Data were collected from 34 hospitals with different levels of care: national referral centres, university-based hospitals, and public and private hospitals. Methods: A survey was administered with questions on admission numbers, mortality rates, ROP incidence, and its stages for 2016-2017 in relation to gestational age and birth weight. Results: We identified 12 115 eligible infants with a gestational age of less than 34 weeks. Mortality was 24% and any stage ROP 6.7%. The mortality in infants aged less than 28 weeks was 67%, the incidence of all-stage ROP 18% and severe ROP 4%. In the group aged 28-32 weeks, the mortality was 24%, all-stage ROP 7% and severe ROP 4%-5%. Both mortality and the incidence of ROP were highest in university-based hospitals. Conclusions: In the 2016-2017 period, the infant mortality rate before 32 weeks of age was higher in Indonesia than in high-income countries, but the incidence of ROP was comparable. This incidence is likely an underestimation due to the high mortality rate. The ROP incidence in 2016-2017 is lower than in surveys conducted before 2015. This decline is likely due to a higher practitioner awareness about ROP and national health insurance implementation in Indonesia.