Focal nodular hyperplasia of the liver in a child with sickle cell anemia

Markowitz, RI; Ht, Harcke; Wg, Ritchie; Huff, DS

doi:10.2214/ajr.134.3.594

Cited by 14 publications

(6 citation statements)

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“…111,115 Few other hepatic comorbidities have been described in patients with SCD. These include hepatic infarction, 116 pyogenic liver abscess, Budd-Chiari syndrome, 117,118 AIH, 119,120 focal nodular hyperplasia, 121,122 malignant histocytosis, 123 primary sclerosing cholangitis, and mesenteric thrombosis. 124…”

Section: Miscellaneous Conditions Associated With Scdmentioning

confidence: 99%

Sickle Hepatopathy

Praharaj

Anand

2021

Journal of Clinical and Experimental Hepatology

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Sickle hepatopathy is an umbrella term describing various pattern of liver injury seen in patients with sickle cell disease. The disease is not uncommon in India; in terms of prevalence, India is second only to Sub-Saharan Africa where sickle cell disease is most prevalent. Hepatic involvement in sickle cell disease is not uncommon. Liver disease may result from viral hepatitis and iron overload due to multiple transfusions of blood products or due to disease activity causing varying changes in vasculature. The clinical spectrum of disease ranges from ischemic injury due to sickling of red blood cells in hepatic sinusoids, pigment gall stones, and acute/chronic sequestration syndromes. The sequestration syndromes are usually episodic and self-limiting requiring conservative management such as antibiotics and intravenous fluids or packed red cell transfusions. However, rarely these episodes may present with coagulopathy and encephalopathy like acute liver failure, which are life-threatening, requiring exchange transfusions or even liver transplantation. However, evidence for their benefits, optimal indications, and threshold to start exchange transfusion is limited. Similarly, there is paucity of the literature regarding the end point of exchange transfusion in this scenario. Liver transplantation may also be beneficial in end-stage liver disease. Hydroxyurea, the antitumor agent, which is popularly used to prevent life-threatening complications such as acute chest syndrome or stroke in these patients, has been used only sparingly in hepatic sequestrations. The purpose of this review is to provide insights into epidemiology of sickle cell disease in India and pathogenesis and classification of hepatobiliary involvement in sickle cell disease. Finally, various management options including exchange transfusion, liver transplantation, and hydroxyurea in hepatic sequestration syndromes will be discussed in brief. (

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Section: Miscellaneous Conditions Associated With Scdmentioning

confidence: 99%

Sickle Hepatopathy

Praharaj

Anand

2021

Journal of Clinical and Experimental Hepatology

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“…Although the etiology is unclear, it is suspected that SCD patients with an underlying vascular anomaly such as an arteriovenous malformation in combination with vaso-occlusion are at risk for focal hyperplasia of the liver as the ischemic tissue regenerates [44]. These patients present with distended abdomens and tender hepatomegaly.…”

Section: Focal Nodular Hyperplasiamentioning

confidence: 99%

“…If the mass is providing symptomatic pressure on internal organs and there is internal bleeding caused by abdominal trauma or significant discomfort, surgical resection is an appropriate treatment. Uncontrollable bleeding and blood loss resulting in death is a risk of this intervention [44].…”

Section: Focal Nodular Hyperplasiamentioning

confidence: 99%

Care of the Critically Ill Pediatric Sickle Cell Patient

Rosanwo

McArthur

Archer

2019

Critical Care of the Pediatric Immunocompromised Hematology/Oncology Patient

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“…FNH has been observed in patients with sinusoidal obstruction syndrome due to hematopoietic stem cell transplantation (Sudour et al 2009). Two reports document children with sickle cell anemia (sickle cell disease, SCD) who developed FNH, possibly caused by the impairment of blood flow by sickling erythrocytes (erythrostasis) and the tendency for small vessel occlusion (Markowitz et al 1980;Heaton et al 1991). In fact, sickling in the sinusoidal vascular bed, making part of sickle cell hepatopathy, causes a nonuniform blood distribution.…”

Section: Hepatocyte Hyperplasia In Disorders Of Sinusoidal Perfusionmentioning

confidence: 99%

Focal Nodular Hyperplasia (FNH) of the Liver

Zimmermann¹

2016

Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Focal nodular hyperplasia (FNH) of the liver is a reactive mass-forming regenerative hyperplastic response of hepatocytes, secondary to localized vascular and circulatory abnormalities. FNH has a predilection for young females and represents, after hemangioma, the second most common benign hepatic tumorous lesion of the liver in adults. Many FNH are asymptomatic and detected by chance, but a minority of FNH are associated with various symptoms mainly related to large size or atypical location. Most FNH are solitary lesions that are predominantly located in the right liver lobe. In a minority of patients, multiple lesions, sometimes exceeding 30, are present. Macroscopically, FNH are well-circumscribed nodular, yellowish to tan masses with a characteristic nodular texture, radiating septa, and a central stellate scar-like fibrotic structure. Part of the lesions protrude from the liver and can become pedunculated masses. The diameter of FNH may exceed 20 cm. Histologically, FNH is characterized by nodules that consist of normal-looking hepatocytes forming thin plates. These nodules are separated from each other by fibrous septa and interspersed portal tract-like structures. The latter lack interlobular bile ducts but contain ductular proliferations. The fibrous tissue areas contain abnormal thick-walled blood vessels.

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Focal nodular hyperplasia of the liver in a child with sickle cell anemia

Cited by 14 publications

References 0 publications

Sickle Hepatopathy

Sickle Hepatopathy

Care of the Critically Ill Pediatric Sickle Cell Patient

Focal Nodular Hyperplasia (FNH) of the Liver

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