Noncirrhotic Portal Hypertension in HIV-Infected Patients: A Case Control Evaluation and Review of the Literature

Nodular regenerative hyperplasia (NRH) is a rare disease that is characterized by benign transformation of the hepatic parenchyma into small nodules with little to no fibrosis. Nodular regenerative hyperplasia is a cause of noncirrhotic portal hypertension. Symptoms can range from asymptomatic disease to more serious complications of portal hypertension such as esophageal varices and ascites. Nodular regenerative hyperplasia has been described in association with a variety of different rheumatologic, hematologic, and oncologic diseases, as well as in immune deficiency states and with exposures to certain toxins. Diagnosis is made by histology, and the treatment involves addressing the underlying disease. The first description of this rare disease was actually described in a patient with rheumatoid arthritis, neutropenia, and splenomegaly (Felty’s Syndrome). We describe 2 cases of NRH associated with underlying rheumatic disorders, in one of which NRH was actually the presenting feature of the patient’s underlying autoimmune condition. Subsequently, we provide a brief review of the literature of NRH in autoimmune disease with respect to epidemiology, cause, clinical manifestations, diagnosis, and treatment.

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Sa1011 Noncirrhotic Portal Hypertension (NCPH) in HIV-Infected Patients: A Case Control Study

Gastroenterology ◽

10.1016/s0016-5085(12)63664-6 ◽

2012 ◽

Vol 142 (5) ◽

pp. S-945-S-946 ◽

Cited By ~ 1

Author(s):

Neil Parikh ◽

Tatyana Kushner ◽

Valerie Martel-Laferriere ◽

Kate Childs ◽

Deepti Dronamraju ◽

...

Keyword(s):

Portal Hypertension ◽

Case Control Study ◽

Case Control ◽

Noncirrhotic Portal Hypertension ◽

Control Study

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A young man with bleeding esophageal varices

Thai Journal of Hepatology ◽

10.30856/th.jhep2018vol1iss1_002 ◽

2018 ◽

Vol 1 (1) ◽

pp. 14-16

Author(s):

Soonthorn Chonprasertsuk

Keyword(s):

Risk Factors ◽

Portal Hypertension ◽

Liver Function ◽

Physical Examination ◽

Esophageal Varices ◽

Noncirrhotic Portal Hypertension ◽

Liver Histopathology ◽

Bleeding Esophageal Varices

The noncirrhotic portal hypertension is an uncommon cause of bleeding esophagealvarices. This condition must be suspected in patients with preserved liver function. We reporta 25-year old man with SLE disease who presented with hematemesis. He had no historyor risk factors for an underlying liver condition. A huge splenomegaly was detectedby physical examination. The EGD found three large varices with red wale sign, whereas liverfunction tests were unremarkable. The noncirrhotic portal hypertension was diagnosedand confirmed by liver histopathology. Figure 1 แสดงผลการส่องกล้องทางเดินอาหารส่วนบนพบ F3 varices with red wale sign

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Risk factors and outcome of HIV-associated idiopathic noncirrhotic portal hypertension

Alimentary Pharmacology & Therapeutics ◽

10.1111/apt.12049 ◽

2012 ◽

Vol 36 (9) ◽

pp. 875-885 ◽

Cited By ~ 26

Author(s):

J. N. L. Schouten ◽

M. E. Van der Ende ◽

T. Koëter ◽

H. H. M. Rossing ◽

M. Komuta ◽

...

Keyword(s):

Risk Factors ◽

Portal Hypertension ◽

Noncirrhotic Portal Hypertension

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Hematological Indices in Portal Hypertension: Cirrhosis versus Noncirrhotic Portal Hypertension

Journal of Clinical Medicine ◽

10.3390/jcm7080196 ◽

2018 ◽

Vol 7 (8) ◽

pp. 196 ◽

Cited By ~ 1

Author(s):

Abdurrahman Sahin ◽

Hakan Artas ◽

Nurettin Tunc ◽

Mehmet Yalniz ◽

Ibrahim Bahcecioglu

Keyword(s):

Portal Hypertension ◽

Retrospective Study ◽

Radiological Findings ◽

Hematological Indices ◽

Noncirrhotic Portal Hypertension ◽

End Stage Liver Disease ◽

Cirrhotic Patients ◽

Hematological Abnormalities ◽

End Stage ◽

Cirrhotic Group

Portal hypertension (PHT) leads to several alterations on hematological indices (HI). The aim of the study is to investigate the differences in HI between cirrhotic subjects and subjects who have noncirrhotic PHT (NCPHT). This retrospective study included 328 patients with PHT (239 cirrhosis and 89 NCPHT). Demographic and clinical features, endoscopic and radiological findings, and HI including neutrophil to lymphocyte ratio (NLR) at the time of PHT diagnosis were recorded. Severity of cirrhosis was assessed according to the Child–Turcotte–Pugh (CTP) classification and Model for End-Stage Liver Disease (MELD) scores. Hematological abnormalities were found in 92.5% of cirrhotic patients and in 55.1% of patients with NCPHT (p < 0.001). While thrombocytopenia was the most common HI in patients with cirrhosis, anemia was the most prevalent HI in NCPHT group. In the cirrhotic group, the NLR was the only parameter to differentiate each CTP group from two others. The NLR value increased with the severity of cirrhosis (2.28 ± 0.14 in CTP-A, 2.85 ± 0.19 in CTP-B and 3.26 ± 0.37 in CTP-C). The AUROC of NLR was 0.692 for differentiating compensated cirrhotic patients from decompensated. Hematological abnormalities are more prevalent and more severe in cirrhotic patients compared to patients with NCPHT. NLR may be used to assess the severity of cirrhosis.

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