Hepatic manifestations of systemic disease: an imaging-based review

Cao, Joseph; Artunduaga, Maddy; Schooler, Gary R.

doi:10.1007/s00247-021-05222-5

Pediatr Radiol

2021

DOI: 10.1007/s00247-021-05222-5

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Hepatic manifestations of systemic disease: an imaging-based review

Joseph Cao

Maddy Artunduaga

Gary R. Schooler

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Cited by 3 publications

References 102 publications

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Relato De Caso De Uma Glomerulopatia Associado a Doenças Sistêmicas

Pimenta,

Pinho

2024

Rev. Contemp.

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Objetivo: Relatar um caso de glomerulopatia associada a doenças sistêmicas, destacando os desafios diagnósticos e terapêuticos apresentados pela coexistência de lúpus eritematoso sistêmico (LES) e doença mista do tecido conjuntivo (DMTC). Métodos: Estudo observacional de braço único, no qual foram coletadas informações clínicas e laboratoriais de um paciente do sexo masculino, 21 anos, com diagnóstico de síndrome nefrótica secundária ao LES e à DMTC. A abordagem terapêutica incluiu o uso de ciclofosfamida, prednisona e hidroxicloroquina, com acompanhamento ambulatorial. Detalhamento de Caso: O paciente apresentou sintomas como edema progressivo, febre intermitente e mialgias, sendo admitido para investigação. Exames laboratoriais revelaram FAN e anti-RNP positivos, além de hipocomplementemia, sugerindo uma doença autoimune subjacente. A biópsia renal revelou glomerulopatia membranosa com padrão "Full House", confirmando o diagnóstico de DMTC sobreposto ao LES. O tratamento inicial foi com metilprednisolona e ciclofosfamida, seguido de micofenolato para estabilização clínica. Considerações finais: O manejo de glomerulopatias autoimunes associadas ao LES e DMTC apresenta desafios diagnósticos e terapêuticos significativos. A abordagem multidisciplinar e personalizada, adaptada à resposta clínica, é essencial para melhorar os desfechos clínicos. Este relato reforça a importância de terapias inovadoras para condições autoimunes complexas.

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Relato De Caso De Uma Glomerulopatia Associado a Doenças Sistêmicas

Pimenta,

Pinho

2024

Rev. Contemp.

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Hepatic multiple hyperintense cystic lesions: a rare caroli disease.

Alsharif

Bakhit

Alarifi

et al. 2023

NAMJ

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Caroli’s disease is a rare malformation of the biliary tract as its incidence rate is one per million of people. Most cases of hepatic cystic lesions are asymptomatic and are discovered incidentally during abdominal ultrasonography. It is reported that Caroli’s disease is an autosomal-recessive disorder with multifocal segmental non-obstructive dilation features of intrahepatic bile ducts that may involve a segment, a lobe, or the whole liver. Several complications might be developed in some cases, such as cholangiocarcinoma. Therefore, early diagnosis is vital for proper management. There is a lack of literature discussing Caroli’s disease among Africans compared to Asians. Females are more affected than males. Moreover, most of Caroli’s disease cases were about 22 years old of age. This study describes the existing case’s novelty in that we discuss a rare Caroli’s disease case of an African male patient; moreover, the patient age in mid-adulthood. In addition, the severity of the current case is that both hepatic lobes showed severe multiple cystic lesions. The embryonic justification of the case is the large cranial portion of the hepatic diverticulum gives rise to the cords of hepatocytes and intrahepatic biliary networks. On the other hand, the small caudal portion forms the extrahepatic biliary tract. The hepatocytes as well as cholangiocytes, are differentiated from the hepatoblasts under the influence of typical hepatic genes, α-fetoprotein, liver-specific transcription factors, and albumin. Based on the above, Caroli disease is defined by biliary tract malformation, whereas Caroli syndrome is determined by the presence of associated congenital hepatic fibrosis.

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Hepatic multiple hyperintense cystic lesions: a rare caroli disease

Alsharif

Bakhit

Alarifi

et al. 2023

NAMJ

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Hepatic manifestations of systemic disease: an imaging-based review

Cited by 3 publications

References 102 publications

Relato De Caso De Uma Glomerulopatia Associado a Doenças Sistêmicas

Relato De Caso De Uma Glomerulopatia Associado a Doenças Sistêmicas

Hepatic multiple hyperintense cystic lesions: a rare caroli disease.

Hepatic multiple hyperintense cystic lesions: a rare caroli disease

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