Cirrhosis clinical presentation

Clinical Presentation of Cirrhosis and Complications of Portal Hypertension... 

Cirrhosis and Portal Hypertension CHAPTER 21 I Clinical Presentation of Cirrhosis... 

Should cirrhosis be present at the time of initial diagnosis in the wake of confirmed pure autoimmune hepatitis (i. e. without HBV or HCV replication markers), it is advisable to implement combined immunosuppression - which is often successful in relatively low but sufficient dosage. Even if the morphological end-stage has already been reached, inflammatory activity can be repressed and clinical improvement achieved. The question of whether (and when) a liver transplantation should be planned always has to be considered. 

Manifest cirrhosis Manifest cirrhosis is characterized by subjective complaints and clinical findings. Flepatic haemodynamics are significantly altered, and other organs and organ systems as well as hormonal and humoral functions are affected. Liver cirrhosis orchestrates a complex array of altered biochemical and physiological processes. Manifest cirrhosis can present in two forms ... 

In a South African study 20 children were identified as suffering from hepatic veno-occlusive disease thought to be caused by the administration of traditional remedies (3). The predominant clinical presentation was ascites and hepatomegaly. Nine children died. The surviving patients progressed to cirrhosis and portal hypertension. In four cases early urine specimens were available, and in all of these the presence of pyrrolizidine alkaloids was confirmed. 

Drug-induced liver disease occurs as several different clinical presentations idiosyncratic reactions, allergic hepatitis, toxic hepatitis, chronic active toxic hepatitis, toxic cirrhosis, and liver vascular disorders. 

Most patients with Wilson disease, whatever their clinical presentation, have some degree of liver disease. Chronic liver disease (if undiagnosed and untreated) may precede manifestation of neurological symptoms for more than 10 years. Patients can present with liver disease at any age. The most common age of hepatic manifestation is between 8 and 18 years, but cirrhosis may already be present in children below the age of 5. On the other hand, Wilson disease is diagnosed also in patients presenting with advanced chronic liver disease in their 50s or 60s, without neurological symptoms and without Kayser-Fleischer rings. 

In the course of studies on other pathological amino acidurias, the accompanying peptiduria has also been observed by many authors. Rapp de Eston et al. (R2) observed a marked increase in the excretion of both free amino acids and peptides in patients with diffuse hepatic necrosis. Using a simplified chromatographic method adapted to clinical purposes and suitable for analysis of amino acids excreted with urine, Skarzynski et al. (S5) demonstrated a raised level of a certain peptide which is always present in normal urine in smaller quantities, as well as the appearance of some new peptides in cases of jaundice and liver cirrhosis. Some abnormal peptide spots were also detected on the chromatograms in cases of progressive muscular dystrophy (K4) and in patients irradiated with X-rays (S2). 

This point of view overlooks the fact that every well and normal individual is potentially an ill individual, and the roots of disease may be present in his make-up years before there is any overt disease. A dozen young men used as normal controls may each have metabolic peculiarities that point toward a different metabolic derangement gout, multiple sclerosis, diabetes, anemia, atherosclerosis, hypertension, nephrosis, hypothyroidism, rheumatoid arthritis, rheumatic heart disease, liver cirrhosis, and myasthenia gravis, for example, and yet at the time of their use as controls these young men may show no symptoms of the disease which is to appear later in life. It seems far from safe to assume that because an individual on clinical examination seems well, all of his blood values, for example, are normal and meaningless so far as disease susceptibilities are concerned. 

A neonatal form of hepatic failure has a rapidly fatal course and frequently includes severe hypotonia, myoclonus epilepsy, and psychomotor retardation [39]. Another type (found in children aged 2-18 months) has a milder clinical course with infrequent fatal outcome [11]. Abnormal histology (steatosis, micro and macro-nodular cirrhosis) and elevated plasma or cerebrospinal fluid (CSF) lactate are consistent features of the disease, regardless of the clinical subtype. The brain is often involved in these presentations, but other organs could also be involved. Dwarfism... 

This patient illustrates a complicated clinical course of oq-antitrypsin deficiency. Our patient had liver disease that presented during infancy and developed into hepatic cirrhosis. He exhibited most of the complications of cirrhosis, including portal hypertension with ascites, hyperammonemia, malnutrition, and variceal hemorrhage. These complications of cirrhosis are not unique to a,-antitrypsin deficiency, but it is important to note the potential severity of the liver disease associated with this condition. 

The synthetic and metabolic capacity of this patient s liver is unlikely to be affected by the isolated rise in ALT. Drug handling is unlikely to be altered. It is important to ensure that the patient has no signs of cirrhosis, as many diseases that present with this clinical picture can become cirrhotic despite near normal laboratory tests. 

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