Idiopathic hyperammonemia after solid organ transplantation: Primarily a lung problem? A single-center experience and systematic review

Krutsinger, Dustin C.; Pezzulo, Alejandro A.; Blevins, Amy; Reed, Robert M.; Voigt, Michael; Eberlein, Michael

doi:10.1111/ctr.12957

Cited by 37 publications

(46 citation statements)

References 26 publications

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“…Idiopathic hyperammonemia has been reported to occur in 0.5%‐4.1% of lung transplant recipients . It has been defined as elevated plasma ammonia concentrations, >200 μmol/L, in the absence of significant liver dysfunction .…”

Section: Discussionmentioning

confidence: 99%

“…Patients typically present with mental status changes. Among solid organ transplants, this syndrome most commonly affects lung transplant recipients, although has been reported in heart, combined heart‐lung, and renal transplant recipients . Total parenteral nutrition, gastrointestinal complications and lung transplantation for primary pulmonary hypertension have been associated with increased risk for this syndrome in lung transplant patients .…”

Section: Discussionmentioning

confidence: 99%

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Successful treatment of Ureaplasma‐induced hyperammonemia syndrome post‐lung transplant

Matson

Sonetti

2018

Transplant Infectious Dis

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Hyperammonemia, in the absence of significant liver dysfunction, is an uncommon but often fatal occurrence following orthotopic lung transplant. Prior reports have provided evidence to support Ureaplasma species as an etiology for this syndrome. This case report describes an individual post‐lung transplant, treated emperically with doxycycline along with other measures to lower ammonia levels, at the time hyperammonemia with encephalopathy was recognized. The patient clinically improved. Ureaplasma species were subsequently identified using 16S ribosomal RNA gene PCR/sequencing of pleural fluid, and by culture of bronchoalveolar (BAL) fluid. This case provides further support for empiric treatment of Ureaplasma species upon recognition of hyperammonemia syndrome post‐lung transplant.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Successful treatment of Ureaplasma‐induced hyperammonemia syndrome post‐lung transplant

Matson

Sonetti

2018

Transplant Infectious Dis

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“…Post‐transplant hyperammonemia syndrome is a rare condition most commonly observed in the lung transplant population 2‐4,9,12,13 . It is characterized by elevated ammonia levels in the absence of synthetic liver dysfunction, with altered mental status and often cerebral edema 3 .…”

Section: Discussionmentioning

confidence: 99%

“…Hyperammonemia syndrome (HS), characterized by markedly elevated serum ammonia levels and acute, progressive neurologic dysfunction, was discovered as a potential complication of hematopoietic cell transplant as early as 1991 1 . Since that time, several reports have described a similar phenomenon after solid organ transplantation, primarily following lung transplant, 2‐4 but also after renal transplant 5,6 and rarely heart transplant 2,7 . In the past, this syndrome has been difficult to treat, with high mortality rates 3 .…”

Section: Introductionmentioning

confidence: 99%

Hyperammonemia syndrome due to Ureaplasma infection after liver‐kidney transplant

Cannon

Corcorran

Shaw

et al. 2020

Transplant Infectious Dis

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Hyperammonemia syndrome, with high levels of ammonia and neurologic dysfunction, is a syndrome with historically high mortality that may occur after solid organ transplantation. Recently, this has been associated with infection due to Ureaplasma, mostly following lung transplantation. We describe the first case of hyperammonemia syndrome due to Ureaplasma infection after liver‐kidney transplantation. Our patient rapidly recovered after specific antibiotic treatment. It is important to consider these infections in the differential diagnosis for encephalopathy post‐transplant, as these organisms often do not grow using routine culture methods and polymerase chain reaction testing is typically required for their detection. This is particularly critical after liver transplantation, where a number of other etiologies may be considered as a cause of hyperammonemia syndrome.

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“…In this population, a rare but severe disease named hyperammonemia syndrome has been described since the ’90s 10 . Hyperammonemia syndrome is characterized by elevated ammonia plasma levels in the early transplant period, complicated by neurologic symptoms, which can be fatal 11 . In 2015, Bharat et al .…”

Section: Donor-derived Infectionsmentioning

confidence: 99%

Recent advances in understanding and managing infectious diseases in solid organ transplant recipients

2018

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Background: Undergoing solid organ transplantation (SOT) exposes the recipient to various infectious risks, including possible transmission of pathogen by the transplanted organ, post-surgical infections, reactivation of latent pathogens, or novel infections. Recent advances: In the last few years, the emergence of Zika virus has raised concerns in the transplant community. Few cases have been described in SOT patients, and these were associated mainly with moderate disease and favorable outcome; the notable exception is a recent case of fatal meningo-encephalopathy in a heart transplant recipient. Because of the advances in treating hepatitis C, several teams recently started to use organs from hepatitis C-positive donors. The worldwide increasing incidence of multidrug-resistant pathogens, as well as the increasing incidence of Clostridioides difficile infection, is of particular concern in SOT patients. In the field of mycology, the main recent therapeutic advance is the availability of isavuconazole for the treatment of invasive aspergillosis and mucormycosis. This drug has the advantage of minimal interaction with calcineurin inhibitors. Regarding the viral reactivations occurring after transplant, cytomegalovirus (CMV) infection is still a significant issue in SOT patients. The management of resistant CMV remains particularly difficult. The approval of letermovir, albeit in bone marrow transplantation, and the therapeutic trial of maribavir bring a ray of hope. Another advancement in management of post-transplant infections is the development of in vitro tests evaluating pathogen-specific immune response, such as immunodiagnostics for CMV and, more recently, tests for monitoring immunity against BK virus. Conclusion: The increasing number of organ transplantations, the use of newer immunosuppressive drugs, and high-risk donors continue to define the landscape of transplant infectious diseases in the current era.

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Idiopathic hyperammonemia after solid organ transplantation: Primarily a lung problem? A single-center experience and systematic review

Abstract: IHS is a rare but often fatal complication that primarily affects lung transplant recipients within the first 30 days.

Cited by 37 publications

References 26 publications

Successful treatment of Ureaplasma‐induced hyperammonemia syndrome post‐lung transplant

Successful treatment of Ureaplasma‐induced hyperammonemia syndrome post‐lung transplant

Hyperammonemia syndrome due to Ureaplasma infection after liver‐kidney transplant

Recent advances in understanding and managing infectious diseases in solid organ transplant recipients

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