Neonatal Cholestasis

Feldman, Amy G.; Sokol, Ronald J.

doi:10.1542/neo.14-2-e63

Cited by 125 publications

(165 citation statements)

References 39 publications

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“…Cholestasis, defined by a direct bilirubin level >20% of the total serum bilirubin level and seen in this case, has been associated with hypopituitarism [5, 6]. Cholestasis typically represents an underlying pathologic condition, with a wide differential including infections, anatomic abnormalities of the biliary system, toxic exposures, oncologic processes, and endocrine and metabolic disorders [7].…”

Section: Discussionmentioning

confidence: 99%

Septooptic Dysplasia with an Associated Arachnoid Cyst

McLaurin‐Jiang

Wood

Crudo

2016

Case Reports in Pediatrics

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A 4-week-old male infant presented with hypothermia, hypoglycemia, and hyperbilirubinemia. His medical history was remarkable for hydrocephalus secondary to an arachnoid cyst, intermittent hypoglycemia, hypothermia, and poor feeding requiring nasogastric tube for nutrition. Physical exam revealed retrognathia, mild hypotonia, micropenis, and clinodactyly. Ophthalmologic exam demonstrated bilateral optic nerve hypoplasia (ONH). Laboratory data confirmed inadequate cortisol and growth hormone response to hypoglycemia, a low thyroxine level, and direct hyperbilirubinemia. Magnetic resonance imaging of the brain confirmed the known history of arachnoid cyst with hydrocephalus but also revealed anterior pituitary hypoplasia, absence of the posterior pituitary bright spot, a thin pituitary stalk, and bilateral optic nerve hypoplasia. A diagnosis of septooptic dysplasia (SOD) was made. Hormone replacement with hydrocortisone and levothyroxine was started with improvement in the infant's glycemic control, thermoregulation, feeding, and cholestasis. This case reinforces the importance of careful physical examination and laboratory review in a patient with known history of arachnoid cyst which has been previously described as an associated feature of optic nerve hypoplasia and hypopituitarism.

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Section: Discussionmentioning

confidence: 99%

Septooptic Dysplasia with an Associated Arachnoid Cyst

McLaurin‐Jiang

Wood

Crudo

2016

Case Reports in Pediatrics

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“…One possible hypothesis is that this is due to intravascular haemolysis secondary to accumulation of galactose-1-phosphate inside RBCs 16. Previous data suggest that of the total cases of conjugated hyperbilirubinemia, nearly one-fifth are due to metabolic liver disease, but the percentage contributed by galactosaemia is still not known 17. Worldwide prevalence of kernicterus following extreme hyperbilirubinemia or Rh haemolytic disease is 73/100 000 live births out of which south Asia contributes 37% of cases 18.…”

Section: Discussionmentioning

confidence: 99%

Galactosaemia: an unusual cause of chronic bilirubin encephalopathy

et al. 2015

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Galactosaemia is a disorder of galactose metabolism in which raised levels of galactose and galactose-1-phosphate damage various organs. Although galactosaemia is a common metabolic liver disease in childhood, it is a rare cause of neonatal hyperbilirubinemia requiring intervention. We report an unusual case of neonatal galactosaemia that at presentation had features of acute bilirubin encephalopathy requiring exchange transfusion and at discharge had features of chronic bilirubin encephalopathy. This case report emphasises the need for timely suspicion and diagnosis of this disease for prevention of chronic morbidity.

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“…Unlike indirect hyperbilirubinemia, direct hyperbilirubinemia is never normal or physiological, and early diagnosis and treatment are essential to achieving a better outcome (8,9). Therefore, it is important to differentiate phototherapy-related transient direct hyperbilirubinemia from direct hyperbilirubinemia due to other serious conditions.…”

Section: Introductionmentioning

confidence: 99%

Phototherapy-Related Transient Direct Hyperbilirubinemia

Kanburoğlu¹,

Cizmeci²,

Akelma³

et al. 2016

Turkish J Pediatr Dis

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Objective: We aimed to investigate the frequency and possible risk factors of phototherapy-related direct hyperbilirubinemia. Material and Methods:We scanned the medical records of 1301 patients who were admitted to the neonatology unit for indirect hyperbilirubinemia between 2005 and 2013. Infants with a clinical condition other than hyperbilirubinemia were excluded. Results:Following phototherapy both the direct bilirubin level and direct bilirubin/total bilirubin ratio increased (p<0.001 and p<0.001, respectively). The frequency of phototherapy-related direct hyperbilirubinemia was found to be 0.9% if the direct bilirubin/total bilirubin cut-off level was set to 20%. The elevated direct bilirubin level and direct bilirubin/total bilirubin ratio returned to normal within three days, although they were still significantly higher than the pre-phototherapy levels (p < 0.001; p < 0.001, respectively). The increase in direct bilirubin level and direct bilirubin/total bilirubin ratio were found to be brisker in patients with ABO incompatibility (p < 0.001 and p < 0.001, respectively). Conclusion:The direct bilirubin level and direct bilirubin/total bilirubin ratio may temporarily rise following phototherapy. This deviation is short term, and ABO incompatibility is the only identified risk factor.To prevent unnecessary investigations this phenomenon should kept in mind by the physicians caring for infants. Bulgular: Fototerapiyi takiben hem direkt bilirubin seviyesi hem de direkt bilirubin/total bilirubin oranı artmaktadır (sı-rasıyla p < 0.001 ve p < 0.001). Patolojik direkt bilirubin/total bilirubin sınırı %20 alındığında, fototerapi ile ilişkili direkt hiperbilirubinemi sıklığı %0.9 olarak bulundu. Artmış direkt bilirubin seviyeleri ve direkt bilirubin/total bilirubin oranı 3 gün içerisinde normale dönmekteydi, fakat bu değerler halen fototerapi öncesindeki değerlere göre anlamlı olarak yüksekti (sırasıyla p < 0.001 ve p < 0.001). ABO uygunsuzluğu olan hastalarda direkt bilirubindeki ve direkt bilirubin/total bilirubin değerindeki artışın daha hızlı olduğu saptandı (sırasıyla p < 0.001 ve p < 0.001).Sonuç: Fototerapi sonrasında direkt bilirubin düzeyi ve direkt bilirubin / total bilirubin oranı geçici olarak artabilmektedir. Bu durum kısa sürelidir ve saptanabilen tek risk faktörü ABO uygunsuzluğudur. Gereksiz ileri araştırmaların önüne geçil-mesi açısından yenidoğanların tedavisinde görev alan doktorların bu durumu akılda tutmaları yararlı olacaktır.

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Neonatal Cholestasis

Cited by 125 publications

References 39 publications

Septooptic Dysplasia with an Associated Arachnoid Cyst

Septooptic Dysplasia with an Associated Arachnoid Cyst

Galactosaemia: an unusual cause of chronic bilirubin encephalopathy

Phototherapy-Related Transient Direct Hyperbilirubinemia

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