Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis

Kewcharoen, Jakrin; Mekraksakit, Poemlarp; Limpruttidham, Nath; Kanitsoraphan, Chanavuth; Charoenpoonsiri, Nattawat; Poonsombudlert, Kittika; Pattison, Robert J.; Rattanawong, Pattara

doi:10.1177/2150135119872196

Cited by 13 publications

(6 citation statements)

References 32 publications

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“…A recent meta-analysis showed that it is effective in ameliorating serum albumin levels in this setting. 9 Long-term complications of GD which were not observed before the availability of ERT are becoming more apparent with increased life expectancy due to ERT. All five reported GD cases with PLE belong to post-ERT era.…”

Section: Discussionmentioning

confidence: 99%

A very rare cause of protein losing enteropathy: Gaucher disease

Göktaş¹,

Gümüş²,

Demır³

et al. 2021

Turk J Pediatr

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Gaucher disease (GD) is a rare, autosomal recessive genetic disorder caused by deficiency of lysosomal enzyme, glucocerebrosidase, which results in the accumulation of glucocerebrosideladen macrophages (Gaucher cells) in the reticuloendothelial system. 1 Mesenteric lymphadenopathy is a rare manifestation of GD in children and can be accompanied by protein losing enteropathy (PLE). PLE is a difficult-to-treat complication of GD. To date, only a few pediatric GD cases with PLE and massive mesenteric lymphadenopathies have been reported. [1][2][3][4] Here, we report a girl with chronic neuronopathic GD whose disease course was complicated by massive mesenteric lymphadenopathies with resultant protein losing enteropathy despite a regular and appropriate enzyme replacement therapy (ERT). Case ReportThe patient was the first child of nonconsanguineous parents and the diagnosis of GD was made at the age of 18 months. At the time of diagnosis, she had severe hepatosplenomegaly

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Section: Discussionmentioning

confidence: 99%

A very rare cause of protein losing enteropathy: Gaucher disease

Göktaş¹,

Gümüş²,

Demır³

et al. 2021

Turk J Pediatr

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“…Because long-term systemic steroid administration may lead to side effects such as osteo- who have undergone the Fontan procedure for congenital heart disease. 13,14 Inhaled budesonide-formoterol is a combination of a corticosteroid and long-acting beta2-adrenergic agonist. No reports have shown the efficacy and long-term improvements induced by inhaled budesonide-formoterol treatment.…”

Section: E T T E R T O T H E E D I T O R Regression Of Kaposiform Lym...mentioning

confidence: 99%

Regression of kaposiform lymphangiomatosis and chronic disseminated intravascular coagulation after inhaled budesonide‐formoterol treatment

Imamura,

Shin,

Ozeki

et al. 2024

Pediatric Blood & Cancer

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“…It is hypothesized that midodrine works by its effect on tone in the smooth muscle cells in the lymphatic vessels, though additional work is needed to confirm this hypothesis ( 113 , 114 ). Additional medical treatments for patients with PLE include oral budesonide and octreotide, but use may not reduce the need for transplant over the long term ( 65 , 115 , 116 ).…”

Section: Treatmentmentioning

confidence: 99%

Management of circulatory failure after Fontan surgery

2022

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With improvement in survival after Fontan surgery resulting in an increasing number of older survivors, there are more patients with a Fontan circulation experiencing circulatory failure each year. Fontan circulatory failure may have a number of underlying etiologies. Once Fontan failure manifests, prognosis is poor, with patient freedom from death or transplant at 10 years of only about 40%. Medical treatments used include traditional heart failure medications such as renin-angiotensin-aldosterone system blockers and beta-blockers, diuretics for symptomatic management, antiarrhythmics for rhythm control, and phosphodiesterase-5 inhibitors to decrease PVR and improve preload. These oral medical therapies are typically not very effective and have little data demonstrating benefit; if there are no surgical or catheter-based interventions to improve the Fontan circulation, patients with severe symptoms often require inotropic medications or mechanical circulatory support. Mechanical circulatory support benefits patients with ventricular dysfunction but may not be as useful in patients with other forms of Fontan failure. Transplant remains the definitive treatment for circulatory failure after Fontan, but patients with a Fontan circulation face many challenges both before and after transplant. There remains significant room and urgent need for improvement in the management and outcomes of patients with circulatory failure after Fontan surgery.

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Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis

Cited by 13 publications

References 32 publications

A very rare cause of protein losing enteropathy: Gaucher disease

A very rare cause of protein losing enteropathy: Gaucher disease

Regression of kaposiform lymphangiomatosis and chronic disseminated intravascular coagulation after inhaled budesonide‐formoterol treatment

Management of circulatory failure after Fontan surgery

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