Jaundice in children
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ifeoma Anaya, M.D.[2]
Synonyms and keywords: Jaundice in kids; hyperbilirubinemia
Overview
Historical Perspective
- The word 'Jaundice' was derived from the french word for yellow which is jaune.[1]
- Earliest known texts of Icterus neonatorum dates back to the medical records of the Providence Lying-in Hospital in the late 19th century. A phenomenon observed amongst several neonates in their first week of stay and attributed to breastfeeding which was the predominant way neonates were fed.
- A severe form termed Icterus gravis was better understood in the 1940s when advances in immunology and genetics led to the discovery of the Rh group of red cell antigens explaining its frequent recurrences in families after a first child becomes affected. These advances in hemolytic diseases birthed effective treatment modalities and screening methods that included maternal serology and amniocentesis in the perinatal period.
- The Baby Boom Years of the 1960s which was flanked by an increase in birth rates led to the development of the Rh-immune antiglobulin. This was as a result of a corresponding rise in the rate of neonatal jaundice. Thus, Rh erythroblastosis became very rare with screening and immunoglobulin prophylaxis during the antenatal period. [2]
- The principles behind Phototherapy were first discovered at Rochford General Hospital, Essex in the 1950s. Babies taken outside to the warm sunshine with the aim of taking a break from the confines of an incubator literarily became less yellow than before. Subsequently, lab results further confirmed this discovery. [3]
Classification
- Jaundice may be classified into two broad categories based on time of onset and cause of jaundice.
- Physiologic jaundice:
- Seen after the first 24 hours of life.
- Serum bilirubin levels never rise >5mg/dl daily and maximum concentration is about 12mg/dl and 14mg/dl in full terms and preterms respectively.
- Reaches the highest levels in the 4th-5th days and resolves within 2 weeks in both full-term and preterm infants.
- Infants usually appears well.
- Pathological jaundice:
- Appears anytime from the first few hours of life.
- Serum bilirubin levels rise at a rate of >5mg/dl per day or 0.5mg/dl per hour and maximum concentration is usually >12mg/dl and >14mg/dl in full terms and preterms respectively.
- Infants appear ill and pale with abnormal discoloration of urine and stool.[1]
- Physiologic jaundice:
Pathophysiology
- Jaundice is caused by hyperbilirubinemia which is high concentrations of bilirubin in the bloodstream.
- Hyperbilirubinemia can result from abnormalities in the metabolism of bilirubin which could occur at any stage from its production, hepatic metabolism, and its post hepatic transport.
- Hemoglobin released from the breakdown of old or defective red blood cells is composed of heme and globin. Globin is broken down into its component amino acids and recycled while heme is split into iron and biliverdin by the enzyme, heme oxygenase in the reticuloendothelial system. Iron is transferred to ferritin and used again to make hemoglobin while biliverdin is converted to bilirubin by biliverdin reductase. [1]
- Bilirubin, which is water-insoluble becomes coupled to albumin and transported into hepatic cells for conjugation.
- This albumin-bilirubin compound is broken down and the unconjugated bilirubin enters the cytosol of hepatocytes to be conjugated to glucuronic acid in the endoplasmic reticulum by the enzyme, Uridine diphosphate glucuronosyltransferase (UDPGT). [4]
- Conjugated bilirubin is secreted into bile and then into the small intestine after being stored in the gall bladder. It eventually gets to the colon where it is acted upon by bacterial flora and deconjugated to urobilinogen. Most of these are excreted into feces as the brown pigment, stercobilin, and the rest is reabsorbed into the blood, converted to yellow urobilin which is eventually excreted into the urine. [5]
- Conjugated or unconjugated hyperbilirubinemia gives a clue as to the defective mechanism/point in the system responsible for the metabolism of bilirubin.
Causes
Disease name] may be caused by [cause1], [cause2], or [cause3].
OR
Common causes of [disease] include [cause1], [cause2], and [cause3].
OR
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].
OR
The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.
Differentiating [disease name] from other Diseases
For further information about the differential diagnosis, click here.
Epidemiology and Demographics
- The prevalence of jaundice varies among patient populations.
- In infants born at term, 60% will develop jaundice in their first-week life. This rises to 80% in preterms. [1]
- 5-10% of neonates will require being admitted for the treatment of pathological jaundice. [6]
- Causes of jaundice also vary with age groups. In newborns, immature hepatic conjugation, hemolysis, and certain congenital disorders are top causes while Hepatitis A infection is a cause seen more in older children.
- Death rate is 0.28 per 1 million live births. [4]
Age
- Patients of all age groups may develop Jaundice.
- It is more commonly observed in newborns and the elderly populations. [4]
Gender
- Gender predilection can be observed in the etiology of jaundice.
- An example is the documented male preponderance of Glucose-6-Phosphate dehydrogenase (G6PD) deficiency with an incidence of 4.5% males to 0.5% in females. [7]
Race
- Racial predilection for Jaundice is observed in a cause of unconjugated hyperbilirubinemia, Gilbert syndrome.
- This is caused by a genetic mutation in the gene responsible for the production of the enzyme, UDPGT. It is a diagnosis of exclusion and symptoms are triggered by stressful situations like dehydration, illness.
- It has a prevalence of 5-10% in Caucasian and Asian populations. [7]
Risk Factors
- Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
Natural History, Complications and Prognosis
- The majority of patients with [disease name] remain asymptomatic for [duration/years].
- Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
- If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with [disease name] is approximately [#%].
Diagnosis
Diagnostic Criteria
- The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
- [criterion 1]
- [criterion 2]
- [criterion 3]
- [criterion 4]
Symptoms
- [Disease name] is usually asymptomatic.
- Symptoms of [disease name] may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with [disease name] usually appear [general appearance].
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
Laboratory Findings
- There are no specific laboratory findings associated with [disease name].
- A [positive/negative] [test name] is diagnostic of [disease name].
- An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
- Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
Electrocardiogram
There are no ECG findings associated with [disease name].
OR
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
X-ray
There are no x-ray findings associated with [disease name].
OR
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with [disease name].
OR
Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
CT scan
There are no CT scan findings associated with [disease name].
OR
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
MRI
There are no MRI findings associated with [disease name].
OR
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
OR
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
Other Imaging Findings
There are no other imaging findings associated with [disease name].
OR
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
Other Diagnostic Studies
- [Disease name] may also be diagnosed using [diagnostic study name].
- Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
Treatment
Medical Therapy
- There is no treatment for [disease name]; the mainstay of therapy is supportive care.
- The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
- [Medical therapy 1] acts by [mechanism of action 1].
- Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
Surgery
- Surgery is the mainstay of therapy for [disease name].
- [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
- [Surgical procedure] can only be performed for patients with [disease stage] [disease name].
Prevention
- There are no primary preventive measures available for [disease name].
- Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
- Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
References
- ↑ 1.0 1.1 1.2 1.3 "StatPearls". 2020. PMID 30422525.
- ↑ https://www.rimed.org/medhealthri/2010-05/2010-05-154.pdf
- ↑ https://www.viapath.co.uk/news-and-press/the-birth-of-phototherapy
- ↑ 4.0 4.1 4.2 "StatPearls". 2020. PMID 31334972.
- ↑ https://www.rahulgladwin.com/noteblog/gastroenterology/jaundice.php
- ↑ Mishra S, Agarwal R, Deorari AK, Paul VK (2008). "Jaundice in the newborns". Indian J Pediatr. 75 (2): 157–63. doi:10.1007/s12098-008-0024-7. PMID 18334797.
- ↑ 7.0 7.1 Chee YY, Chung PH, Wong RM, Wong KK (2018). "Jaundice in infants and children: causes, diagnosis, and management". Hong Kong Med J. 24 (3): 285–292. doi:10.12809/hkmj187245. PMID 29807950.