Hepatopulmonary syndrom

   Hepatopulmonary syndrome is defined as a clinical disorder (associated with advanced liver disease) due to disturbed pulmonary gas exchange leading to hypoxemia and widespread intrapulmonary vasodilation and shunting of blood in the absence of detectable primary cardiopulmonary disease.

Hepatopulmonary syndrom

  In chronic liver disease, pulmonary affection is common and may result in hypoxia and cyanosis. The pulmonary changes that may complicate chronic liver diseases are: 

Premature Contraction

Three common terms used to describe certain abnormal cardiac contractions are premature contractions (beats occurring early in time), ectopic beats (beats with sites of origin outside the sinus node), and extrasystoles (added beats). Only extrasystoles are truly added or additional beats, often interpolated or added between two normal beats without interfering with the basic rhythm.

Premature Contraction

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Jaccoud’s arthritis

Jaccoud’s arthritis or chronic postrheumatic-fever arthritis is a syndrome which associates a chronic relatively asymptomatic severe joint deformity with preceding episodes of endocarditis. Its etiology and pathophysiology remain unclear. Although the term arthritis implies an inflammatory process, cardinal signs of inflammation are not a part of this syndrome. Its hallmark is severe deformity without joint destruction. The term Jaccoud’s arthritis is a misnomer and should be replaced by the term Jaccoud’s arthropathy.

Jaccoud’s arthritis

Through the years, there has been an interest in the relationship of rheumatic fever, valvular heart disease and rheumatoid arthritis.  There appears to be a spectrum between pure rheumatic heart disease and pure rheumatoid arthritis. 

📖 Firestein & Kelley’s Textbook of Rheumatology

Fahr's disease (syndrome)

Fahr's Syndrome is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex. 

Fahr's disease (syndrome)

Symptoms of the disorder may include deterioration of motor function, dementia, seizures, headache, dysarthria (poorly articulated speech),spasticity (stiffness of the limbs) and spastic paralysis, eye impairments, and athetosis (involuntary, writhing movements). Fahr's Syndrome can also include symptoms characteristic of Parkinson's disease such as tremors, muscle rigidity, a mask-like facial appearance, shuffling gait, and a "pill-rolling" motion of the fingers. These symptoms generally occur later in the development of the disease. More common symptoms include dystonia (disordered muscle tone) and chorea (involuntary, rapid, jerky movements). Age of onset is typically in the 40s or 50s, although it can occur at any time in childhood or adolescence.

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Hemoglobin

Hemoglobin consists of four protein chains called globins. Two of these, the alpha (α) chains, are 141 amino acids long, and the other two, the beta (β) chains, are 146 amino acids long. Each chain is conjugated with a nonprotein moiety called the heme group, which binds oxygen to an iron atom (Fe) at its center. 

(A) The Structure of Hemoglobin. The hemoglobin molecule consists of two alpha proteins and two beta proteins, each conjugated to a nonprotein heme group.

(B) The Structure of Hemoglobin. Structure of the heme group. Oxygen binds to iron (Fe) at the center of the heme.

📖 Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management