scholarly journals Liver Disease in Hemophilia

1977 ◽  
Author(s):  
Joel A. Spero ◽  
Jessica H. Lewis ◽  
Ute Hasiba

Abnormal liver function tests (LFTs) in patients with Hemophilia A and B who have been treated with commercial factor concentrates have been observed since 1973. One hundred and seventeen of these had had LFTs performed at least twice and at least six months apart. Of these, 13 were chronically HbsAg positive and the rest anti-Hbs positive. Forty percent of the anti-Hbs group had persistently abnormal LFTs for greater than 6 months, the majority for more than two years. Owing to the uncertain relationship of this “transaminitis” with true liver pathology, especially in the anti-body positive patients, seven closed liver biopsies were performed without incident under high coverage with factor VIII replacement. All patients were asymptomatic at the time of biopsy. One had a prior episode of illness probably related to subclinical hepatitis. An eighth patient underwent an open liver biopsy as part of a staging laparotomy for Hodgkins Disease. Six biopsies showed varying degrees of chronic persistent hepatitis (CPH). One was not evaluable owing to changes related to a retrospectively obtained history of ETOH intake. Finally, the individual with previous clinical liver disease had post-necrotic cirrhosis. Fluorescent studies with anti-Hbs and-anti-Hbc as well as electron microscopy were done on the seven closed biopsy specimens. The biopsy results suggest that the “transaminitis” found in transfused hemophiliacs represents histologic liver disease. In the majority of patients studied, CPH, a relatively benign entity, was present; and this does not justify withdrawal of concentrate therapy at this time.

2021 ◽  
Vol 10 (6) ◽  
pp. 1161
Author(s):  
Raluca Pais ◽  
Thomas Maurel

The epidemiology and the current burden of chronic liver disease are changing globally, with non-alcoholic fatty liver disease (NAFLD) becoming the most frequent cause of liver disease in close relationship with the global epidemics of obesity, type 2 diabetes and metabolic syndrome. The clinical phenotypes of NAFLD are very heterogeneous in relationship with multiple pathways involved in the disease progression. In the absence of a specific treatment for non-alcoholic steatohepatitis (NASH), it is important to understand the natural history of the disease, to identify and to optimize the control of factors that are involved in disease progression. In this paper we propose a critical analysis of factors that are involved in the progression of the liver damage and the occurrence of extra-hepatic complications (cardiovascular diseases, extra hepatic cancer) in patients with NAFLD. We also briefly discuss the impact of the heterogeneity of the clinical phenotype of NAFLD on the clinical practice globally and at the individual level.


Diagnosis ◽  
2015 ◽  
Vol 2 (2) ◽  
pp. 137-140 ◽  
Author(s):  
Wycliffe Mbagaya ◽  
Joanne Foo ◽  
Ahai Luvai ◽  
Claire King ◽  
Sarah Mapplebeck ◽  
...  

AbstractMacrocomplexes between immunoglobins and aspartate aminotransferase (macro-AST) may result in persistently increased AST concentration. The presence of macro-AST in patients has been implicated in unnecessary investigations of abnormal liver function tests. We report the case of a 44-year-old female who presented to the rheumatology clinic with a 12-months’ history of constant widespread pain affecting her limbs and was found to have an elevated AST concentration. Further information from her GP revealed a 14-years’ history of elevated AST with otherwise normal liver function. Previous abdominal ultrasound and two liver biopsies carried out 2 years apart were normal. This prompted further analytical investigation by the biochemistry department which identified macro-AST as the cause. This case illustrates that persistently raised isolated AST concentration with no other abnormal indices may warrant macroenzyme analysis potentially avoiding unnecessary invasive investigations.


2006 ◽  
Vol 5 (3) ◽  
pp. 93-95
Author(s):  
Ajay Kiran Muddu ◽  
◽  
Mark Wright ◽  
Nick Sheron ◽  
◽  
...  

A twenty year old female was referred to hospital by her GP, after he received the results of blood tests taken earlier in the day. She had presented to him complaining of malaise, nausea and anorexia over a 3 day period. On the day of referral she had also become jaundiced with dark urine, but normal stool colour. There was no abdominal pain. She had no significant past medical history with no history of jaundice, liver disease or autoimmune conditions, and no apparent risk factors for blood-borne hepatitis infection. There was no relevant family history. She was taking no prescribed medication, had not taken any over the counter medication or herbal remedies. She denied excessive alcohol use or use of intravenous drugs in the past, although she was not specifically questioned on the use of other recreational drugs. She was a single mother and admitted to being under considerable stress recently. On examination she appeared well, apart from marked jaundice. There were no signs of hepatic encephalopathy or chronic liver disease. Abdominal examination revealed mild left upper quadrant tenderness, but no significant hepatomegaly. Liver function tests (LFTs) taken by her GP are shown in Table 1, revealing marked elevation of the Alanine Transaminase (ALT) (Table 1), with a relatively preserved albumin. Unfortunately her International Normalised Ratio (INR) had not been measured. An Ultrasound of the abdomen demonstrated a normal size liver with normal contour and texture with no other abnormality.


2017 ◽  
Vol 71 (5) ◽  
pp. 412-419 ◽  
Author(s):  
Kaushik Majumdar ◽  
Puja Sakhuja ◽  
Amarender Singh Puri ◽  
Kavita Gaur ◽  
Aiman Haider ◽  
...  

BackgroundCoeliac disease (CD) is a gluten-sensitive enteropathy diagnosed on the basis of ESPGHAN criteria and clinical response to gluten-free diet (GFD). Histological abnormalities on liver biopsy have been noted in CD but have seldom been described.AimsTo assess the histological spectrum of ‘coeliac hepatitis’ and possibility of reversal of such features after a GFD.MethodsTwenty-five patients with concomitant CD and hepatic derangement were analysed for clinical profile, laboratory investigations and duodenal and liver biopsy. A histological comparison of pre- and post-GFD duodenal and liver biopsies was carried out, wherever possible.ResultsFifteen patients presenting with CD subsequently developed abnormal liver function tests; 10 patients presenting with liver disease were found to have tissue positive transglutaminase in 70% and antigliadin antibodies in 60%. Serological markers for autoimmune liver disease (AILD) were positive in eight patients. Liver histology ranged from mild reactive hepatitis, chronic hepatitis, steatosis to cirrhosis. Liver biopsies after a GFD were available in six cases, of which five showed a decrease in steatosis, portal and lobular inflammation and fibrosis score.ConclusionCoeliac hepatitis could be a distinct entity and the patients may present with either CD or secondary hepatic derangement. Evaluation for the presence of CD is recommended for patients presenting with AILD, unexplained transaminasaemia or anaemia. This is one of the very few studies demonstrating the continuum of liver histological changes in ‘coeliac hepatitis’. Trial of a GFD may result in clinicopathological improvement of ‘coeliac hepatitis’.


1988 ◽  
Vol 66 (3) ◽  
pp. 903-921
Author(s):  
Rosemary A. Robbins

This paper is an account of studies of the linguistic transformation that took place in ancient Greece between the eighth and fourth centuries B.C., searching for factors which contribute to the shift in how humans perceived themselves. The group or force-field consciousness of the men of the Iliad and the linguistic factors which allowed “individuality” to emerge by the time of Plato is explored. The account relates the emergence of the notion of “madness” to the development of the individual and asks whether madness is an artifact of individuality and explores the relationship of these developments to our present underlying assumption of a duality in human nature composed of the rational and the irrational.


PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0256117
Author(s):  
George Marek ◽  
Amy Collinsworth ◽  
Chen Liu ◽  
Mark Brantly ◽  
Virginia Clark

Background Pathological mutations in Alpha-1 Antitrypsin (AAT) protein cause retention of toxic polymers in the hepatocyte endoplasmic reticulum. The risk for cirrhosis in AAT deficiency is likely directly related to retention of these polymers within the liver. Polymers are classically identified on liver biopsy as inclusion bodies by periodic acid schiff staining after diastase treatment and immunohistochemistry. However, characterization of the polymer burden within a biopsy sample is limited to a semi-quantitative scale as described by a pathologist. Better methods to quantify polymer are needed to advance our understanding of pathogenesis of disease. Therefore, we developed a method to quantify polymer aggregation from standard histologic specimens. In addition, we sought to understand the relationship of polymer burden and other histologic findings to the presence of liver fibrosis. Methods Liver samples from a well-categorized AATD cohort were used to develop histo-morphometric tools to measure protein aggregation. Results Whole-slide morphometry reliably quantifies aggregates in AATD individuals. Despite very low levels of inclusions present (0–0.41%), accumulation of globules is not linear and is associated with higher fibrosis stages. Immunohistochemistry demonstrates that fibrosis is associated with polymer accumulation and not total AAT. A proportion of patients were found to be “heavy accumulators” with a polymer burden above the upper 25% of normal distribution. Males had significantly more liver inclusions and polymer than females. These measurements also highlight interrelated phenotypes of hepatocellular degeneration and autophagy in AATD liver disease. Conclusion Quantitative inclusion analysis measures AAT accumulation in liver biopsy specimens. Quantification of polymer may identify individuals at risk for progressive disease and candidates for therapeutic interventions. Furthermore, these methods may be useful for evaluating efficacy of drugs targeting accumulation of AAT.


2020 ◽  
Vol 10 (3) ◽  
pp. 245-254 ◽  
Author(s):  
Yala K. Reddy ◽  
Hemnishil K. Marella ◽  
Yu Jiang ◽  
Surosree Ganguli ◽  
Peter Snell ◽  
...  

2016 ◽  
Vol 2016 ◽  
pp. 1-2
Author(s):  
Seth Shaffer ◽  
Mayur Brahmania ◽  
Hemant Shah

A 49-year-old Brazilian male presented to the emergency department with a five-day history of abdominal pain, dark stools, and syncope. Physical examination did not reveal any melena on digital rectal examination and there were no stigmata of chronic liver disease. Laboratory results showed hemoglobin of 47 g/L, MCV of 80 fL, and ferritin of 6 ng/mL. Liver enzymes and liver function tests were normal. Abdominal ultrasound showed a cirrhotic liver with splenomegaly and varices suggestive of portal hypertension. His past history was significant for cirrhosis based on a previous variceal bleed but a workup for chronic liver disease was negative and a liver biopsy did not show steatosis, fibrosis, or cirrhosis. A gastroscopy in this admission showed large esophageal varices without high-risk stigmata and no overt bleeding was seen. A colonoscopy was subsequently completed to the terminal ileum and was normal aside from a 5 mm sessile polyp in the descending colon.


Gut ◽  
2016 ◽  
Vol 66 (9) ◽  
pp. 1688-1696 ◽  
Author(s):  
Pierre Bedossa ◽  
Joan Tordjman ◽  
Judith Aron-Wisnewsky ◽  
Christine Poitou ◽  
Jean-Michel Oppert ◽  
...  

2002 ◽  
Vol 6 (1) ◽  
pp. 1-11
Author(s):  
Chris Powici

AbstractSigmund Freud's analysis of the childhood dream of the Wolf Man, in The History of an Infantile Neurosis, has come to be seen as one of the defining moments of psychoanalysis. Freud interpreted this dream in terms of the Oedipus complex, concluding that the wolves which threatened to devour his patient were, in effect, father-substitutes, the archaic trace in the unconscious of the individual of the threat posed by the tyrannical father of the 'original' human family. In this article I argue that this conclusion conceals a problematic reading, on Freud's part, of the human/animal border, which is evidenced, in The History of an Infantile Neurosis, as well as elsewhere in his writings, as an anxiety as to the ontological status of the human subject and the 'nature' of civilisation, and as a repressed acknowledgement of the animal as sublime presence. However, in trying to negotiate similar questions today, and despite this marked ambivalence toward the 'animal', I also argue that Freud's insight into the mechanisms of repression remains a valuable way of exploring the relationship of the human to the nonhuman.


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