Macrophage activating syndrome is associated with lobular hepatitis and severe bile duct injury with cholestasis

Bihl, Florian; Emmenegger, Urban; Reichen, Jürg; Neftel, Klaus; Zimmermann, Arthur; Cerny, Andreas

doi:10.1016/j.jhep.2006.03.003

Cited by 20 publications

(5 citation statements)

References 18 publications

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“…Patients with iPALF have a liver injury pattern similar to observations in familial and sporadic HLH and MAS . However, iPALF patients do not manifest the full spectrum of criteria required for diagnosis of HLH.…”

Section: Inflammatory Milieu In Palfmentioning

confidence: 99%

“…Patients with iPALF have a liver injury pattern similar to observations in familial and sporadic HLH and MAS. (19)(20)(21) However, iPALF patients do not manifest the full spectrum of criteria required for diagnosis of HLH. Like patients with HLH, some patients with iPALF present with elevated ferritin and soluble IL-2 receptor levels, low fibrinogen, and numerous infiltrating CD8 1 T cells on liver biopsy.…”

Section: Inflammatory Milieu In Palfmentioning

confidence: 99%

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Pediatric acute liver failure of undetermined cause: A research workshop

et al. 2017

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Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials. The clinical phenotype of indeterminate PALF shares important similarities to the hyperinflammatory state characteristic of Hemophagocytic lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS). A failure of cytotoxic T cells to limit or contract inflammatory responses may propagate injury and lead to a local and systemic milieu that does not support normal hepatic regeneration. Evidence was presented that bone marrow (BM) derived Sinusoidal endothelial cell PROgenitor Cells (sprocs) play a vital role in hepatic regeneration. Overwhelming systemic inflammatory responses may suppress mobilization of BM sprocs and dampen hepatic recovery. Conclusion: Thus, experience gained through treatment trials of HLH and MAS in childhood may inform study design for therapy of PALF. Successful approaches to limiting neuro-inflammation through reduction of systemic inflammation and standardized neuroprotection protocols that limit glial injury could significantly improve intact survival. Finally, since PALF is a rare disease, investigative efforts must include broad multi-center collaboration and careful stewardship of biorepository specimens.

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Section: Inflammatory Milieu In Palfmentioning

confidence: 99%

Section: Inflammatory Milieu In Palfmentioning

confidence: 99%

Pediatric acute liver failure of undetermined cause: A research workshop

et al. 2017

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“…Pediatric acute liver failure study group has recently published a review focusing not only on improving diagnostic approaches and defining the phenotype of iPALF but also on this emerging concept of this patient subgroup. Moreover, recently published studies have suggested that iPALF is associated with exaggerated systemic inflammatory responses, as evident by elevated serum soluble interleukin (IL)-2 receptor, whereas other reports have associated iPALF with hemophagocytic lymphohistiocytosis (HLH) because these patients exhibit similar liver injury patterns to familial and sporadic HLH without fulfilling all of the diagnostic criteria [45,46]. Supportive care continues to be the treatment of choice till recovery or liver transplant awaiting more evidence [26].…”

Section: Discussionmentioning

confidence: 99%

Pediatric acute liver failure in Saudi Arabia: prognostic indicators, outcomes and the role of genetic testing

Alhadab¹,

AlShihabi²,

Mohamed³

et al. 2022

European Journal of Gastroenterology &Amp; Hepatology

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Objective The objective of this study was to determine the etiologies, outcomes, prognostic indicators and the role of genetic testing in children with acute liver failure (ALF). Methods This retrospective study included 46 patients with pediatric acute liver failure (PALF) according to the PALF study group definition, admitted to King Fahad Specialist Hospital-Dammam, Saudi Arabia, between January 2014 and December 2021. Patients who survived with supportive therapy were designated as the recovery group, whereas those who died or underwent liver transplantation were designated as the death/transplant group. Results There were 26 (56.5%) patients in the recovery group and 20 (43.5%) patients in the death/transplant group. Four patients (8.7%) underwent liver transplantation. After indeterminate causes (45.6%), genetic-metabolic diseases and drug-induced liver injury (DILI) were the most common cause with 15.2 and 13%, respectively. Genetic testing had a high yield of (6/31) in identifying monogenic disease associated with ALF. Younger age, lower Glasgow Coma Scale and higher international normalized ratio (INR) on admission were predictors for poor prognosis. The death/transplant group had longer intensive care unit stay (P < 0.001), and on admission they had more advanced hepatic encephalopathy (P < 0.005), more prolonged prothrombin time (P < 0.001), higher lactate (P < 0.006), higher total and direct bilirubin (P < 0.008) and (P < 0.001), respectively. Conclusion Genetic, metabolic and DILI causes constituted the most common cause of PALF after indeterminate causes. The use of genetic testing can improve diagnostic rates in special cases, but we could not assess the effect of genetic testing on prognosis. The overall survival rate in our study was 65.2%. Younger age, higher admission INR and lower Glasgow coma scale were indicators of poor prognosis.

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“…Ein MAS konnte in Verbindung mit einer autoimmunen Hepatitis [12], sowie in mehreren Fällen mit einer akuten Hepatitis A [13] und chronischen Hepatitis B [14] diagnostiziert werden. Eine Arbeit beschreibt eine lobuläre Hepatitis und schwere Gallengangsschädigung mit Cholestase [15]. Bei unserer Patientin fanden sich zunächst führende Transaminasen mit im Verlauf steigenden Cholestaseparametern (▶ Abb.…”

Section: Diskussionunclassified

Hepatische Manifestation eines Makrophagen-Aktivierungs-Syndroms (MAS)

Nagel

Schwarting

Straub³

et al. 2017

Z Gastroenterol

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Elevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure. In addition to genetic causes, MAS is commonly associated with infections and rheumatic diseases. We report the case of a 26-year-old female patient suffering from MAS as a rare cause of elevated liver enzymes. Patient characteristics, laboratory values, liver histology, bone marrow and radiological imaging were documented and analyzed. After an ordinary upper airway infection with bronchitis, a rheumatic arthritis appeared and was treated with leflunomide und methotrexate. In the further course of the disease, the patient developed an acute hepatitis with fever, pancytopenia and massive hyperferritinemia. Immunohistochemistry of the liver biopsy revealed hemophagocytosis and activation of CD68-positive macrophages. In the radiological and histological diagnostics of the liver and bone marrow, an MAS was diagnosed as underlying disease of the acute hepatitis. Under therapy with prednisolone, the fever disappeared and transaminases and ferritin rapidly normalized. Aside from the frequent causes of elevated liver values in younger patients, such as nutritive toxic, drug induced liver injury, viral or autoimmune hepatitis, especially in case of massive hyperferritinemia, a MAS should be considered as a rare cause of acute liver disease.

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Macrophage activating syndrome is associated with lobular hepatitis and severe bile duct injury with cholestasis

Cited by 20 publications

References 18 publications

Pediatric acute liver failure of undetermined cause: A research workshop

Pediatric acute liver failure of undetermined cause: A research workshop

Pediatric acute liver failure in Saudi Arabia: prognostic indicators, outcomes and the role of genetic testing

Hepatische Manifestation eines Makrophagen-Aktivierungs-Syndroms (MAS)

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