Histiocytic Osteolysis Secondary to Hyperbilirubinaemia: A Case Report

Nakamura, Tomoki; Kusuzaki, Katsuyuki; Matsubara, Takehiro; Satonaka, Haruhiko; Shintani, Ken; Wakabayashi, Tetsushi; M.D., and Akihiko Matsumine; Uchida, Atsushi

doi:10.1177/230949900801600229

Cited by 3 publications

(5 citation statements)

References 8 publications

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“…Histiocytic osteolysis can be associated with hyperbilirubinemia, 12 which was present in our patient; the lack of inclusion bodies or pigments in the histiocytes, though, suggests a distinct process in our patient. With the multiple unique features, our case could represent a novel syndrome.…”

Section: Discussioncontrasting

confidence: 42%

Novel syndrome of four-limb proximal fragility fractures associated with HIV infection, cholestatic liver failure, and histiocytic infiltration of bone marrow

Nissen

Balzer

et al. 2012

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We report a syndrome of four-limb proximal fragility fractures associated with HIV infection, cholestatic liver failure, and histiocytic infiltration of bone marrow in a 40-year-old African American man. the patient presented with multiple fractures in the proximal humeri and femurs without osteopenia in the vertebrae. His right humerus appeared normal on chest X-ray film 3 years before presentation when he was first diagnosed with HIV infection and abnormal liver functions. At presentation, the patient had vitamin D deficiency, hypogonadism, and low IGF-1 levels, but did not have hyperparathyroidism. bone biopsy showed diffuse foamy histiocytic infiltration of bone marrow at all fracture sites without evidence of infectious or neoplastic processes. Exhaustive search did not identify any similar cases in the English literature. Our case likely represents a novel syndrome, the etiology of which is probably multifactorial and includes HIV infection, cholestatic liver failure, immobility, and endocrine abnormalities. the case further calls for the need for monitoring of bone health in patients with HIV infection or liver disease.

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Section: Discussioncontrasting

confidence: 42%

Novel syndrome of four-limb proximal fragility fractures associated with HIV infection, cholestatic liver failure, and histiocytic infiltration of bone marrow

Nissen

Balzer

et al. 2012

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“…However, some patients experience significant morbidity as a consequence of bone fractures. Case studies document multiple recurrent fractures in some patients (10,11), and poor healing and/or marked postfractural deformities in others (12,13). Indeed, 1 study of 10-year outcomes in an AGS cohort reported that 6 of 8 patients referred for liver transplantation experienced recurrent and/or poorly healing bone fractures (11).…”

mentioning

confidence: 99%

Pathologic Lower Extremity Fractures in Children With Alagille Syndrome

Bales

Kamath

Muñoz

et al. 2010

J. pediatr. gastroenterol. nutr.

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Objectives In this retrospective study, we aimed to determine the incidence and distribution of fractures in patients with Alagille syndrome, one of the leading inherited causes of pediatric cholestatic liver disease. Methods Surveys regarding growth, nutrition, and organ involvement were distributed to patient families in the Alagille Syndrome Alliance or The Children’s Hospital of Philadelphia research database. Patients with a history of fracture were identified by their response to one question, and details characterizing each patient’s medical, growth, and fracture history were obtained through chart review and telephone contact. Results Twelve of 42 patients (28%) reported a total of 27 fractures. Patients experienced fractures at a mean age of 5 years, which contrasts with healthy children, in whom fracture incidence peaks in adolescence. Fractures occurred primarily in the lower extremity long bones (70%) and with little or no trauma (84%). Estimated incidence rate calculations yielded 399.6 total fractures/10,000 person years (95% CI = 206.5, 698.0) and 127.6 femur fractures/10,000 person-years (95% CI = 42.4, 297.7). There were no differences in gender, age distribution or organ system involvement between the fracture and no-fracture groups. Conclusions Children with Alagille syndrome may be at risk for pathologic fractures, which manifest at an early age and in a unique distribution favoring the lower extremity long bones. While this preliminary study is limited by small sample size and potential ascertainment bias, the data suggest that larger studies are warranted to further characterize fracture risk and to explore factors contributing to bone fragility in these children.

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“…Exposure to excessive levels of bilirubin inhibits the proliferation of osteoblasts in cell cultures. Despite these findings, studies investigating the association between bilirubin levels and osteoclast function are lacking [8]. In the present patient, long-term hyperbilirubinemia was associated with systemic osteoporosis.…”

Section: Discussionmentioning

confidence: 66%

“…The Alagille gene has been identified in the 20pl2 region. This syndrome manifests as a multisystem disorder involving the liver, heart, eyes, face, and skeleton [1,8]. One of the manifestations of Alagille syndrome is hyperbilirubinemia caused by cholestasis secondary to a paucity of interlobular bile ducts.…”

Section: Discussionmentioning

confidence: 99%

“…There are few reports of pathological fractures of the femur in Alagille syndrome. Case studies have documented multiple recurrent fractures in some patients [6] and poor healing and/or marked post-fractural deformities in others [7,8]. We report a case of a 9-year-old girl with marked hyperbilirubinemia from birth who presented with a pathological fracture of the femoral shaft.…”

Section: Introductionmentioning

confidence: 99%

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Pathological fracture of the femur in Alagille syndrome that was treated with low-intensity pulsed ultrasound stimulation and an Ilizarov ring fixator: a case report

Nozaka

Shimada

Miyakoshi

et al. 2014

BMC Musculoskelet Disord

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BackgroundAlagille syndrome is a multisystem disorder, which is characterized by hypoplasia of the intrahepatic bile ducts, malformations of the cardiovascular system, eyes, and vertebral column, and abnormal facies. Several of the characteristics of Alagille syndrome may result in an especially high risk of fracture. The majority of patients suffer from chronic cholestasis, which can have a variety of adverse effects on bone metabolism. In Alagille syndrome, fractures primarily occur in the lower limb long bones in the absence of significant trauma.Case presentationA 9-year-old Japanese girl with Alagille syndrome was admitted to our institution with marked hyperbilirubinemia and a pathological fracture of the femur. She had been diagnosed with biliary atresia at the age of 1 month and treated with surgical bile duct reconstruction, vitamins D and K, and ursodeoxycholic acid. However, her liver dysfunction and hyperbilirubinemia worsened. The pathological fracture of the femur was treated with low-intensity pulsed ultrasound stimulation (LIPUS) and an Ilizarov ring fixator. Seventy-four days after surgery, the patient had anatomically and functionally recovered. There was no leg-length discrepancy and no angular malalignment of the lower extremities as measured clinically and radiographically. The range of motion of the hip, knee, and ankle of the patient’s operative leg matched the range of motion in the nonoperative leg.ConclusionTo the best of our knowledge, there are no reports on use of the Ilizarov frame and LIPUS in diaphyseal femoral fractures in Alagille syndrome. This case report provides evidence that this procedure is successful for managing such diaphyseal fractures in Alagille syndrome.

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Histiocytic Osteolysis Secondary to Hyperbilirubinaemia: A Case Report

Cited by 3 publications

References 8 publications

Novel syndrome of four-limb proximal fragility fractures associated with HIV infection, cholestatic liver failure, and histiocytic infiltration of bone marrow

Novel syndrome of four-limb proximal fragility fractures associated with HIV infection, cholestatic liver failure, and histiocytic infiltration of bone marrow

Pathologic Lower Extremity Fractures in Children With Alagille Syndrome

Pathological fracture of the femur in Alagille syndrome that was treated with low-intensity pulsed ultrasound stimulation and an Ilizarov ring fixator: a case report

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