Cirrhosis in an Active Duty Soldier With Concomitant Isolated Polycystic Disease and H63D Homozygosity

Madigan, Cory; Wang, James

doi:10.7205/milmed-d-15-00455

Cited by 3 publications

(1 citation statement)

References 8 publications

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“…Secondary complications of PH result from severe hepatic fibrosis, including splenomegaly, ascites, variceal hemorrhage, and encephalopathy, but the development of liver cirrhosis is a rare event. [121][122][123][124][125] Compression of the inferior vena cava frequently occurs, but in most cases, there are no hemodynamic repercussions. 115,119,120 Conversely, compression of hepatic veins is much more common, leading to the formation of intraparenchymal and subcapsular veno-venous collaterals, a situation that may increase the risks of hemorrhagic events during surgical interventions.…”

Section: Clinical Presentationmentioning

confidence: 99%

Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Norcia¹,

Watanabe²,

Filho³

et al. 2022

HMER

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Polycystic liver disease (PLD) is a clinical condition characterized by the presence of more than 10 cysts in the liver. It is a rare disease Of genetic etiology that presents as an isolated disease or assoc\iated with polycystic kidney disease. Ductal plate malformation, ciliary dysfunction, and changes in cell signaling are the main factors involved in its pathogenesis. Most patients with PLD are asymptomatic, but in 2-5% of cases the disease has disabling symptoms and a significant reduction in quality of life. The diagnosis is based on family history of hepatic and/or renal polycystic disease, clinical manifestations, patient age, and polycystic liver phenotype shown on imaging examinations. PLD treatment has evolved considerably in the last decades. Somatostatin analogues hold promise in controlling disease progression, but liver transplantation remains a unique curative treatment modality.

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Section: Clinical Presentationmentioning

confidence: 99%

Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Norcia¹,

Watanabe²,

Filho³

et al. 2022

HMER

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Biliary Duct Hamartomas: A Systematic Review

Shekhar

Javed

et al. 2021

Preprint

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Biliary duct hamartomas are benign lesions of intrahepatic bile ducts. Oftentimes an incidental finding on imaging, these lesions can pose a diagnostic dilemma because of their overlapping features with malignant masses. We performed a structured systematic review of literature and identified 139 cases of biliary duct hamartomas. Patient demographics, clinical presentation with key laboratory and imaging findings, diagnostic modalities, management strategies, and outcomes were analyzed and systematically summarized. The systematic review is aimed to help with better understanding of biliary duct hamartomas and its principal features.

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Biliary Duct Hamartomas: A Systematic Review

Sheikh¹,

Nguyen²,

Leyba³

et al. 2022

Cureus

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Biliary duct hamartomas are benign intrahepatic bile duct lesions. Despite being primarily incidental findings on imaging, these lesions can provide a diagnostic conundrum due to their shared characteristics with malignant tumors. The goal of this systematic review is to offer a thorough clinical profile of biliary duct hamartomas. There were 139 cases of biliary duct hamartomas identified in a structured systematic review of the literature. Patient demographics, clinical presentation, significant laboratory and imaging data, diagnostic modalities, treatment choices, and outcomes were all studied and reported. Biliary duct hamartomas present with mild symptoms and laboratory abnormalities, and while being visible on imaging, the results are non-specific and may require biopsy in case of red flag signs such as weight loss and a progressive increase in the size of the lesion. Furthermore, there are currently no published guidelines for the treatment of biliary duct hamartomas, and many people have had surgery despite the clinically benign nature of these abnormalities. As per the findings of the study, individuals who exhibit signs of malignancy should be investigated further. Eyeballing for red flag symptoms, followed by a specialized imaging scan and invasive treatment, is the three-step approach to biliary duct hamartomas. Since our recommendations include a shift in strategy and do not contradict existing rules, there are likely to be few roadblocks to improvement; the key barriers being technological equipment and image quality. In this study, we intended to pave the way for future research in the field. In our opinion, the next decade will bring a better understanding of the characteristics of biliary hamartomas, disease symptoms, and better recognition of any suspicious features. These indications will aid in reducing the number of unneeded surgical or invasive operations. Finally, the findings of these future studies will allow the medical community to improve and provide the best care possible.

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Cirrhosis in an Active Duty Soldier With Concomitant Isolated Polycystic Disease and H63D Homozygosity

Cited by 3 publications

References 8 publications

Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Polycystic Liver Disease: Pathophysiology, Diagnosis and Treatment

Biliary Duct Hamartomas: A Systematic Review

Biliary Duct Hamartomas: A Systematic Review

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