Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

Okada, Tadao; Honda, Susumu; Miyagi, Hisayuki; Matsushima, Masashi; Taketomi, Akinobu

doi:10.4254/wjh.v4.i10.284

Cited by 8 publications

(6 citation statements)

References 7 publications

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“…However, several studies have identified impairment of bone mass and mineral development in children with CLD, particularly those with biliary atresia and Alagille syndrome. 8,[13][14][15] The risk for fractures is higher in pediatric patients post-LT than the general pediatric population. 16 Bone fracture prevalence after LT ranges between 12% and 38%.…”

Section: Discussionmentioning

confidence: 99%

“…6,7 Patients with CLD, including biliary atresia, familial cholestasis, primary sclerosing cholangitis, and Alagille syndrome, often develop hepatic osteodystrophy. 8,9 Risk factors for bone loss in cholestatic disease include cirrhosis, poor nutrition, prolonged corticosteroid use, concomitant unconjugated bilirubin, 25-OH vitamin D (VitD) deficiency, malabsorption, and physical inactivity. 10 Skeletal abnormalities in patients with CLD can include osteopenia, rickets, and fractures.…”

mentioning

confidence: 99%

“…Several of these disorders may be distinguished from nonaccidental trauma by physical and radiologic findings, as well as clinical history 6,7 . Patients with CLD, including biliary atresia, familial cholestasis, primary sclerosing cholangitis, and Alagille syndrome, often develop hepatic osteodystrophy 8,9 . Risk factors for bone loss in cholestatic disease include cirrhosis, poor nutrition, prolonged corticosteroid use, concomitant unconjugated bilirubin, 25-OH vitamin D (VitD) deficiency, malabsorption, and physical inactivity 10 …”

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confidence: 99%

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Bone Fractures in Children With Cholestatic Liver Disease May Mimic Those Seen in Child Abuse

et al. 2019

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Certain fractures in children are highly specific for child abuse.Metabolic bone disease frequently develops in patients with cholestatic liver disease (CLD); this can result in weakened bones and a predisposition to pathologic fractures. Fractures that occur in patients with rickets and osteopenia may mimic a bone response to inflicted injury, which in children raise the concern of child abuse. Here we report a series of 15 patients with CLD who developed pathologic fractures in the setting of metabolic bone disease. During initial evaluation, the caretakers of 5 of these 15 patients were reported to child protective services and investigated for child abuse. Pediatricians should be aware that children with CLD have an increased incidence of pathologic fractures, even after the cholestasis has resolved.

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confidence: 99%

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Bone Fractures in Children With Cholestatic Liver Disease May Mimic Those Seen in Child Abuse

et al. 2019

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“…In individuals surviving with native livers after HPE, long term follow up is necessary to ascertain the health status and to detect complications of biliary cirrhosis[ 7 - 9 ]. Malnutrition[ 10 , 11 ], cholangitis[ 12 , 13 ], and fractures are common medical complications[ 14 , 15 ]. Ng et al[ 7 ] reported that cholangitis and bone fractures are the two major complications in long-term survivors of children with BA living with native livers.…”

Section: Introductionmentioning

confidence: 99%

Chronic liver disease is universal in children with biliary atresia living with native liver

Lee

Ong

Foo

et al. 2017

WJG

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AIMTo examine the medical status of children with biliary atresia (BA) surviving with native livers.METHODSIn this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices.RESULTSFifty-two children [females = 32, 62%; median age 7.4 years, n = 35 (67%) older than 5 years] with BA (median age at surgery 60 d, range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension (40%, n = 21; 2 younger than 5 years), cholangitis (36%) and bleeding varices (25%, n = 13; 1 younger than 5 years). Fifteen (29%) had no clinical complications of CLD and three (6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient (2%).CONCLUSIONClinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age, underscoring the importance of medical surveillance for complications of BA starting at a young age.

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“…Metabolic bone disease is a common disorder that can be found in patients with hepatic osteodystrophy, particularly those affected by chronic cholestasis[ 6 , 7 ]. Its etiology is complex and multifactorial and presents as osteopenia and osteoporosis which should be investigated and diagnosed early in patients with chronic liver disease in order to minimize the risk of fractures and improve their quality of life[ 8 , 9 ]. The purpose of this study was to determine bone mineral density (BMD) from postKasai BA children and to investigate the association of BMD and outcome parameters in postoperative BA patients.…”

Section: Introductionmentioning

confidence: 99%

Low bone mineral density and the severity of cholestasis in biliary atresia

Homchan

Chaiwatanarat

Udomsinprasert

et al. 2017

WJH

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AIMTo investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia (BA) children and the association of bone mineral density (BMD) and biochemical parameters in postKasai BA subjects.METHODSA total of 70 patients with postKasai BA were enrolled in this prospective study. The patients were classified into two groups according to their jaundice status. BMD of the lumbar spine was analyzed using dual energy X-ray absorptiometry.RESULTSThe prevalence of low bone mass (osteopenia and osteoporosis) in BA patients were 51.4% (36 out of 70). Ten patients (35.7%) in the jaundice group and 8 patients (19.0%) in the non-jaundice group had osteopenia. Sixteen patients (57.1%) in the jaundice group and 2 patients (4.8%) in the no jaundice group had osteoporosis. In addition, lumbar spine BMD Z-score was substantially lower in the jaundice BA patients compared with non-jaundice patients. BA subjects with persistent jaundice had significantly lower serum 25-hydroxyvitamin D than those without jaundice. Further analysis revealed that lumbar spine BMD was correlated with age (r = 0.774, P < 0.001), serum albumin (r = 0.333, P = 0.005), total bilirubin (r = -0.476, P < 0.001), aspartate aminotransferase (r = -0.583, P < 0.001), alanine aminotransferase (r = -0.428, P < 0.001), and alkaline phosphatase(r = -0.456, P < 0.001).CONCLUSIONLow BMD was associated with biochemical parameters reflecting the severity of cholestasis in postKasai BA patients.

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Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

Cited by 8 publications

References 7 publications

Bone Fractures in Children With Cholestatic Liver Disease May Mimic Those Seen in Child Abuse

Bone Fractures in Children With Cholestatic Liver Disease May Mimic Those Seen in Child Abuse

Chronic liver disease is universal in children with biliary atresia living with native liver

Low bone mineral density and the severity of cholestasis in biliary atresia

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