scholarly journals Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome—Case Series and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Beidi Zhu ◽  
Zunguo Du ◽  
Zhengxin Wang ◽  
Yang Li ◽  
Jiming Zhang ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Literature Review ◽  
Hepatic Fibrosis ◽  
Clinical Laboratory ◽  
Clinical Manifestations ◽  
Case Series ◽  
Congenital Hepatic Fibrosis ◽  
Multidisciplinary Cooperation ◽  
Central Nervous Systems

Background. Congenital hepatic fibrosis is a hereditary fibropolycystic disease caused by ductal plate malformation. It is characterized by portal hypertension, but the manifestations, management, and outcome vary in children and adults. To raise awareness of medical staff, we have comprehensively compared the clinical features of congenital hepatic fibrosis between children and adults. Methods. We retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at the Huashan Hospital from August 2015 to August 2017 and analyzed their familial, clinical, laboratory, imaging, treatment, and follow-up data in detail. In addition, we reviewed cases with congenital hepatic fibrosis reported in the past 20 years in China and analyzed them according to the patients’ age. Results. A total of eight patients were diagnosed with congenital hepatic fibrosis in the study, including four children and four adults. The onset age of the children, who suffered from severe complications of portal hypertension and needed liver transplantation, ranged from 1 to 15 years old. The disorder developed in adults aged 26 to 60 years old. Three adults complained of recurrent abnormal liver function at the onset of illness, and they mainly received conservative treatments. The literature review included 30 children and 33 adults. In comparison, hepatomegaly was more common in children than in adults (57% vs. 21%, p=0.004). Malformation of kidneys and bile duct abnormalities were common, and multisystem involvement included eyes, other digestive organs, and genital and central nervous systems. Conclusions. Serious complications of portal hypertension developed in children requiring liver transplantation, while adults often had mild-to-moderate liver injuries upon onset. Adults with CHF varied a lot in clinical manifestations. Multiorgan involvement and unusual course are helpful to make a diagnosis. Timely histological assessment by liver biopsy and multidisciplinary cooperation are crucial for definitive diagnosis and early intervention.

Cirrhosis and Complications of Portal Hypertension

2018 ◽  
Author(s):  
Andres Cardenas ◽  
Isabel Graupera ◽  
Elsa Sola ◽  
Pere Ginès
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Variceal Bleeding ◽  
Bacterial Infections ◽  
Hepatorenal Syndrome ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Diagnostic Methods ◽  
Decompensated Cirrhosis ◽  
Ultrasound Images

Cirrhosis is the most advanced stage of all the different types of chronic liver diseases. It is defined as a diffuse disorganization of normal hepatic structure by extensive fibrosis associated with regenerative nodules. Hepatic fibrosis is potentially reversible if the causative agent is removed. However, advanced cirrhosis leads to major alterations in the hepatic vascular bed and is usually irreversible. Cirrhosis is a progressive and severe clinical condition associated with considerable morbidity and high mortality. It leads to a wide spectrum of characteristic clinical manifestations, mainly attributable to hepatic insufficiency and portal hypertension. Major complications of portal hypertension include ascites, gastrointestinal (GI) variceal bleeding, hepatic encephalopathy (HE), renal failure, and bacterial infections. In recent years, major advances in the understanding of the natural history and pathophysiology of cirrhosis and the treatment of its complications have led to improved management, quality of life, and life expectancy of patients with this disease. Cirrhosis is also a risk factor for developing hepatocellular carcinoma (HCC). Decompensated cirrhosis carries a poor short-term prognosis; thus, orthotopic liver transplantation (OLT) should always be considered in suitable candidates. This chapter describes the epidemiology, etiology and genetic factors, pathogenesis, diagnosis, general management, and treatment of cirrhosis. Complications of cirrhosis are discussed, including ascites, spontaneous bacterial peritonitis, dilutional hyponatremia, hepatorenal syndrome, variceal bleeding, hepatopulmonary syndrome and postpulmonary hypertension, HE, and HCC. Indications and contraindications for liver transplantation are described. Figures show liver biopsy results and ultrasound images in cirrhosis from hepatitis C, a patient with tense ascites, transjugular intrahepatic portosystemic shunting (TIPS), large esophageal varices with red spots, and HCC. Tables outline the main causes of cirrhosis and the diagnostic methods for identifying them, the Child-Pugh score, diagnostic criteria for hepatorenal syndrome, grades of HE, and indications for liver transplantation.This chapter contains 6 highly rendered figures, 8 tables, 73 references.

scholarly journals Its Congenital Hepatic Fibrosis; Not Cirrhosis At All

2018 ◽  
Vol 1 (2) ◽  
pp. 124-126
Author(s):  
Ananta Shrestha ◽  
Mamun Al-Mahtab ◽  
Salimur Rahman ◽  
Jahangir Sarkar ◽  
Thupten K Lama
Keyword(s):  
Portal Hypertension ◽  
Hepatic Fibrosis ◽  
Autosomal Recessive ◽  
Rare Condition ◽  
Recessive Trait ◽  
Congenital Hepatic Fibrosis ◽  
Life Threatening ◽  
Hepatic Histology ◽  
Upper Abdomen ◽  
Medical University

Congenital hepatic fibrosis is a rare condition characterized by extensive fibrosis of liver but with preserved normal lobular architecture inherited as autosomal recessive trait. We report a 19 year-old-female admitted to Bangabandhu Sheikh Mujib Medical University with the complaints of lump in upper abdomen since last 13 years and episodes of fever and abdominal pain for same duration. She was diagnosed with hepatic TB on hepatic histology. Congenital hepatic fibrosis is a rare cause of portal hypertension that presents during childhood. Prognosis of congenital hepatic fibrosis is good. Life threatening events in these patients are related with variceal bleeding and episodes of cholangitis. Owing to relatively good liver function these patients tolerate portosystemic shunt surgeries quite well.Though rare, congenital hepatic fibrosis should be included in the differential diagnosis of portal hypertension in early life.

Case Series Regarding Parastomal Variceal Bleeding: Presentation and Management

2018 ◽  
Vol 17 (5) ◽  
pp. 0-10
Author(s):  
John Romano ◽  
Charles V. Welden ◽  
Jordan Orr ◽  
Brendan McGuire ◽  
Mohamed Shoreibah
Keyword(s):  
Portal Hypertension ◽  
High Risk ◽  
Literature Review ◽  
Variceal Bleeding ◽  
Coil Embolization ◽  
Case Series ◽  
Treatment Modalities ◽  
Post Procedure ◽  
Lost To Follow Up

Parastomal variceal bleeding (PVB) is a serious complication occurring in up to 27% of patients with an ostomy and concurrent cirrhosis and portal hypertension. The management of PVB is difficult and there are no clear guidelines on this matter. TIPS, sclerotherapy, and /or coil embolization are all therapies that have been shown to successful manage PVB. We present a case series with five different patients who had a PVB at our institution. The aim of this case series is to report our experience on the management of this infrequently reported but serious condition. We also conducted a systemic literature review focusing on the treatment modalities of 163 patients with parastomal variceal bleeds. In our series, patient 1 had embolization and sclerotherapy without control of bleed and expired on the day of intervention due to hemorrhagic shock. Patient 2 had TIPS in conjunction with embolization and sclerotherapy and had no instance of rebleed 441 days after therapy. Patient 3 did not undergo any intervention due to high risk for morbidity and mortality, the bleed self-resolved and there was no further rebleed, this same patient died of sepsis 73 days later. Patient 4 had embolization and sclerotherapy and had no instance of rebleed 290 days after therapy. Patient 5 had TIPS procedure and was discharged five days post procedure without rebleed, patient has since been lost to follow-up.

scholarly journals Liver transplantation in patients with neuroendocrine tumors: a case series and literature review

2021 ◽  
Vol 62 (1) ◽  
pp. 44-51
Author(s):  
Diana Ilić ◽  
Nino Kunac ◽  
Tina Borčić ◽  
Petra Dinjar ◽  
Kujundžić ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Literature Review ◽  
Neuroendocrine Tumors ◽  
Case Series

scholarly journals Clinical characteristics of COVID-19 in children compared with adults in Shandong, China

2020 ◽  
Author(s):  
Wenjun Du ◽  
Jinhong Yu ◽  
Hui Wang ◽  
Xiaoguo Zhang ◽  
Shouwei Zhang ◽  
...  
Keyword(s):  
Clinical Laboratory ◽  
Health Workers ◽  
Clinical Symptoms ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Case Series ◽  
Signs And Symptoms ◽  
Asymptomatic Patients ◽  
Positive Indicators ◽  
Clinical Signs And Symptoms

Abstract Aims & Background: The COVID-19 outbreak spread in China and is a threat to the world. We reported on the epidemiological, clinical, laboratory, and radiological characteristics of children cases to help health workers better understand and provide timely diagnosis and treatment.Methods: Retrospectively, two research centers’ case series of 67 consecutive hospitalized cases including 14 children cases with COVID-19 between 23 Jan 2020 to 15 Feb 2020 from Jinan and Rizhao were enrolled in this study. Epidemiological, clinical, laboratory, and radiological characteristics of children and adults were analyzed and compared.Results: Most cases in children were mild(21.4%) and conventional cases(78.6%), with mild clinical signs and symptoms, and all cases were of family clusters. Fever (35.7%) and dry cough(21.4%) were described as clinical manifestations in children cases. Dry cough and phlegm were not the most common symptoms in children compared with adults(p=0.03). In the early stages of the disease, lymphocyte counts did not significantly decline but neutrophils counts did in children compared with adults(p=0.00).There was an elevated level of LDH(p=0.01) and a lower level of CRP(p=0.00)and IL-6(p=0.01) in children compared with adults. There were 8 (57.1%)asymptomatic cases and 6 (42.9%)symptomatic cases among the 14 children cases. The age of asymptomatic patients was younger than that of symptomatic patients(p=0.03). Even among asymptomatic patients, 5(62.5%)cases had pneumonia including 3 (60%) cases with bilateral pneumonia, which was not different compared with that of asymptomatic cases(p=0.58, p=0.74).Conclusions: The clinical symptoms of children are mild, and the positive indicators of laboratory tests are rare, which may easily cause clinical misdiagnoses.

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