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.
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| 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:
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| Premature Contraction |
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.
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| 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.
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| Fahr's disease (syndrome) |
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| Prader-Labhart-Willi syndrome |
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7-year-old boy with obesity, short stature, characteristic shape of the mouth, acromicria, genua valgum, and hypogonadism has Prader-Labhart-Willi syndrome. |
Kahler’s disease (Multiple myeloma), the most common bone malignancy, is occurring with increasing frequency in older persons. Typical symptoms are bone pain, malaise, anemia, renal insufficiency, and hypercalcemia. Incidental discovery on comprehensive laboratory panels is common. The disease is diagnosed with serum or urine protein electrophoresis or immunofixation and bone marrow aspirate analysis.
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| Kahler’s disease |
On the picture: Kahler’s disease. Kahler’s disease cells are abnormal plasma cells (a type of white blood cell) that build up in the bone marrow and form tumors in many bones of the body. Normal plasma cells make antibodies to help the body fight infection and disease. As the number of Kahler’s disease cells increases, more antibodies are made. This can cause the blood to thicken and keep the bone marrow from making enough healthy blood cells. Kahler’s disease cells also damage and weaken the bone.
Skeletal radiographs are important in staging Kahler’s disease and revealing lytic lesions, vertebral compression fractures, and osteoporosis. Magnetic resonance imaging and positron emission tomography or computed tomography are emerging as useful tools in the evaluation of patients with myeloma; magnetic resonance imaging is preferred for evaluating acute spinal compression. Nuclear bone scans and dual energy x-ray absorptiometry have no role in the diagnosis and staging of myeloma. The differential diagnosis of monoclonal gammopathies includes monoclonal gammopathy of uncertain significance, smoldering (asymptomatic) and symptomatic Kahler’s disease, amyloidosis, B-cell non-
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| Characteristic osteolytic lesions in the skull of a patient with myeloma |
Hodgkin lymphoma, Waldenström macroglobulinemia, and rare plasma cell leukemia and heavy chain diseases. Patients with monoclonal gammopathy of uncertain significance or smoldering Kahler’s disease should be followed closely, but not treated.
The antiglobulin, or Coombs test is part of the compatibility tests that any patient who will receive a red blood cell transfusion must undergo. This test is also essential in the diagnostic work of patients with anemia whose origin is not easily determined and when the etiology must be identified precisely.
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| Direct Coombs test |
In 1945, Robin Coombs, Arthur Mourant and Rob Race described a test to detect anti-Rho (anti-D) non-agglutinant antibodies. Originally, the test was devised by Robin Coombs as part of his postgraduate studies at Race and Mourant's laboratory in Cambridge, England in 1945. His goal was to study the characteristics of the antibodies involved in the context of what was known as fetal erythroblastosis, which is now known as hemolytic disease of the newborn (HDN), caused most frequently by the incompatibility between an Rh-negative mother sensitized during a previous pregnancy, who produces IgG anti-D antibodies able to pass the placenta barrier due to their small size that then cover the fetal red blood cells.
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| Indirect Coombs test |
These are later phagocytosed in the spleen and liver, organs which, in addition to their other functions, maintain extramedullary hematopoiesis in the fetus to compensate for anemia resulting from hemolysis. Later, the Coombs test was used to demonstrate the presence of incomplete antibodies which covered the erythrocytes in vivo, such as is seen in cases of autoimmune hemolytic anemia (AHA). The description of the method, and its application in various hematological diseases, was published in The Lancet and The British Journal of Experimental Pathology in 1945 and 1946, respectively.
Marchiafava-Bignami disease (MBD) is a very rare disorder of demyelination/necrosis of the corpus callosum and the near subcortical white matter that is especially predominant in ill-fed alcoholics. It was discovered in 1903 by Italian pathologists Ettore Marchiafava and Amico Bignami. They described men with alcohol use disorder who died of seizures and coma that presented necrosis of their corpus callosum on autopsy. However, few cases have been described in non-alcoholic patients, suggesting that alcohol is not the sole responsible for these lesions.
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| Marchiafava-Bignami disease |
A, Parasagittal MR T1-weighted image shows multiple areas of decreased signal intensity involving the genu and the anterior aspect of the corpus callosum.
B, MR T2-weighted image corresponding to A shows the lesions to be hyperintense and also some mild atrophy of the involved portions of the corpus callosum.
C, MR Axial T2-weighted image shows a mild degree of periventricular white matter hyperintensity, particularly in the region of the frontal horns of the lateral ventricle.
D, Axial T2-weighted image at the level of the centra semiovale shows a mild degree of periventricular white matter hyperintensities.
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The structure of the Pacinian corpuscle was described by Pacini (1835). It is widely distributed in mammals and is similar to the Herbst corpuscles found in birds. The Pacinian corpuscle is an ovoid structure about 1 mm in length and is easily seen by the naked eye in a number of locations such as the mesentery.
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| Pacini's corpuscles |
On microscopic examination, the lamellar structure of the corpuscle is evident, the lamellae giving an appearance which has been likened to a section through an onion. The corpuscle is innerv!lted by a myelinated sensory axon of medium diameter which terminates within the center of the corpuscle.
Double inferior vena cava is a vein abnormality that is present from birth (congenital). Individuals with this anomaly have two inferior vena cavas instead of one. The inferior vena cava carries oxygen-poor blood from the lower parts of the body into the heart. Double inferior vena cava does not cause any symptoms. It is usually diagnosed when an imaging test, such as CT or MRI, is performed for other medical reasons. The reported incidence of double inferior vena cava ranges from 0.2 percent to 3 percent
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| Double inferior vena cava |
A double or duplicated inferior vena cava results from lack of atrophy in part of the left supracardinal vein, resulting in a duplicate structure to the left of the aorta. The common form is a partial paired inferior vena cava that connects the left common iliac and left renal veins. When caval interruption, such as placement of a filter, is planned, these alternate pathways must be considered. As an alternative, the inferior vena cava may not develop. The most common alternate route for blood flow is through the azygous vein, which enlarges to compensate. If a venous stenosis is present at the communication of iliac veins and azygous vein, back pressure can result in insufficiency, stasis, or thrombosis.
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| Fabry-Anderson disease |
Biceps reflex is a reflex test that examines the function of the C5 reflex arc and the C6 reflex arc. The test is performed by using a tendon hammer to quickly depress the biceps brachii tendon as it passes through the cubital fossa. Specifically, the test activates the stretch receptors inside the biceps brachii muscle which communicates mainly with the C5 spinal nerve and partially with the C6 spinal nerve to induce a reflex contraction of the biceps muscle and jerk of the forearm.
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| Galant's biceps reflex |
A strong contraction indicates a 'brisk' reflex, and a weak or absent reflex is known as 'diminished'. Brisk or absent reflexes are used as clues to the location of neurological disease. Typically brisk reflexes are found in lesions of upper motor neurones, and absent or reduced reflexes are found in lower motor neurone lesions.
A change to the biceps reflex indicates pathology at the level of musculocutaneous nerve, segment C5/6 or at some point above it in the spinal cord or brain.
Left superior vena cava (LSVC) is the most common congenital malformation of the thoracic venous return and is present in 0.3 to 0.5% of individuals in the general population with a normal heart, and 4.5% in individuals with congenital heart diseases. A LSVC co-occurs with the right superior vena cava in 80 to 90% of cases, 2 and may also be accompanied by other heart abnormalities, such as anomalous connections of the pulmonary veins, aortic coarctation, tetralogy of Fallot, transposition of the great vessels as well as dextroversion. Moreover, cardiac rhythm disturbances concerning impulse formation and conduction have been observed.
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| Left superior vena cava |
The LSVC
usually drains into the right atrium (in 80–92%) through a dilated coronary
sinus (CS),5,6 but in approximately 10 to 20% of cases, it is associated with
left atrial (LA) drainage. The LSVC may drain directly through the left atrium
or via the unroofed CS, which is a cause of right-to-left cardiac shunt. The
majority of patients with LSVC are asymptomatic. In general, only patients with
unusual drainage and right-to-left shunting are of clinical significance.
Anomalous venous return via the LSVC may be the cause of cardiac arrhythmias,
decreased exercise tolerance, progressive fatigue, chest discomfort,
palpitations, syncope or cyanosis.
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| The left superior vena cava drains into the coronary sinus in this heart specimen. CS, coronary sinus; GCV, great cardiac vein; PLSVC, persistent left superior vena cava. |
The implications of existing LSVC could be important for clinicians who are involved in placement of central venous-access devices.
The Spinal Galant Reflex provides the opportunity to experience a change from whole body movements to same side (homolateral) movements. It emerges at 20 weeks in utero, is actively present at birth, and should be integrated by the time the baby is 3 to 9 months of age.
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| A deep abdominal reflex in newborn. There is a contraction of the abdominal muscles on tapping the anterior superior iliac spine or touching the skin along spine from shoulder to hip. In infancy it is physiologically present until about the sixth month. At higher ages it is always pathological. The Spinal Galant Reflex should disappear by nine months, and retention beyond this point results in problems such as bed-wetting, fidgeting and the inability to sit still, short-term memory loss or an inability to concentrate. |
The Spinal Galant Reflex is present when a baby is placed in a prone position (one their stomach) and stimulation is placed to one side of the spine. If the reflex is present, the hip on the side of the stimulus will flex (curl toward that side). This reflex helps the baby to work it’s way down the birth canal during the birthing process, and also allows the fetus to hear and feel the sound vibrations in the womb.
Each day the pancreas secretes approximately 1 L of alkaline isosmotic pancreatic juice that originates from the pancreatic acinar cells and pancreatic ducts. The colorless, bicarbonate-rich, and protein-rich pancreatic juice plays key roles in duodenal alkalinization and food digestion. The acinar cells secrete the enzymes required for the digestion of the three main food types: amylase for carbohydrate (starch) digestion, proteases (e.g., trypsin) for protein digestion, and lipases for fat digestion. The acinar cells are pyramidal in shape with the apices facing the lumen of the acinus, where the enzyme-containing zymogen granules fuse with the apical cell membrane for release. Acinar cells, unlike the endocrine cells of the pancreas, are not specialized and produce all three types of pancreatic enzymes from the same cell type.
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| Exocrine functions of the pancreas |
Amylase is secreted in its active form and hydrolyzes starch and glycogen to the simple sugars of dextrins and maltose; maltose is then metabolized to glucose by intestinal maltase.
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The portal system is responsible for transporting blood from most of the gastrointestinal tract to the liver for metabolic processing before the blood returns to the heart. The portal system drains venous blood from the distal end of the esophagus, stomach, small and large intestines, proximal portion of the rectum, pancreas, and spleen. The portal system is the venous counterpart to areas supplied by the celiac trunk and the superior and inferior mesenteric arteries.
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| The portal venous system |
The liver is unique in that it receives both nutrient-rich deoxygenated blood (portal vein) and oxygenated blood (hepatic arteries). The portal vein branches as it enters the liver, where its blood percolates around hepatocytes in tiny vascular channels known as sinusoids. Hepatocytes detoxify the blood, metabolize fats, carbohydrates, and drugs, and produce bile. The sinusoids receive deoxygenated blood from the portal veins (provide blood for metabolism and detoxification) and oxygenated blood from the hepatic arteries (provide oxygen for hepatocytes). Blood exits the sinusoids into a central vein, which empties into the hepatic veins and ultimately into the inferior vena cava, which passes through the diaphragm before entering the right atrium of the heart.
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| Portal vein. |
Oral drugs travel throughout the gastrointestinal tract, where they are absorbed by the small intestine. These drugs then travel to the liver via the hepatic portal system, where they are metabolized before entering the systemic circulation.
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As a continuation of the axillary artery, it begins at the inferior border of the teres major tendon and it ends at the level of the neck of the radius about 1cm distal to the elbow joint. At this point, it divides into the ulnar and radial arteries. The brachial artery runs medial to the humerus proximally, before moving more anteriorly to lie between the epicondyles of the humerus.
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| Brachial artery |
Relationships of the brachial artery to other structures in the arm can be important in clinical practice. The brachial artery is a superficial vessel and is only covered by the layers of the skin, as well as the superficial and deep fasciae, with a few exceptions:
The first exception to this is at the cubital fossa, where the bicipital aponeurosis, which is the aponeurosis of the biceps brachii muscle, covers the artery, and separates it from the median cubital vein.
The second exception is when the median nerve crosses the brachial artery near the distal attachment of the coracobrachialis.
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| Brachial artery |
Posteriorly, the brachial artery is separated from the long head of the triceps brachii muscle by the profunda brachii artery and the radial nerve. The attachments of the coracobrachialis and the brachialis muscles, as well as the medial head of the triceps brachii muscle, also lie posterior to the brachial artery.
The median nerve and coracobrachialis muscle lie laterally to the brachial artery at its proximal aspect whereas the medial cutaneous nerve of the forearm and the ulnar nerve lie medially to the artery proximally.
Kyphoscoliosis has long been recognized as a cause of cardiorespiratory failure. Only in recent years, however, has the combination of clinical picture, physiologic measurements, and anatomic observations at autopsy clarified the natural history of the cardiorespiratory disorder.
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| Kyphoscoliosis |
Unless there is independent lung disease, such as bronchitis or emphysema, only patients with severe spinal deformities are candidates for cardiorespiratory failure. Subjects with mild deformities are consistently asymptomatic. In contrast, those with severe degrees of deformity, particularly if considerable dwarfing has occurred, are often restricted in their activities by dyspnea on exertion. They are most prone to cardiorespiratory failure if an upper respiratory infection should supervene. From the point of view of disability and the likelihood of cardiorespiratory failure, the nature of the deformity (i.e., kyphosis, scoliosis, or both) is unimportant when compared with the severity of the deformity and dwarfing.
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| Kyphoscoliosis in the lower thoracic and lumbar spine. 10-year-old female with rib head dislocation from dystrophic scoliosis. FINDINGS: 3-D reconstructed CT image of the thoracolumbar spine demonstrates a severe Sshaped scoliotic curve in the lower thoracic and lumbar spine. TECHNIQUE: Noncontrast axial CT (100 mAs, 120 kVp, Slice thickness: 2.5 mm). |
One approach to classifying individuals with kyphoscoliosis is on the basis of lung volumes. The more normal the total lung capacity, vital capacity, and tidal volume, the more the subject tends to remain asymptomatic. In those with severe reduction in lung volumes, the stage is set for cor pulmonale.
The available evidence suggests that the macula densa, located at the end of the thick ascending limb, senses tubular flow based on the concentrations of sodium and chloride in the local filtrate. The sensing apparatus appears to be apical Na+/K+/2Cl− (NKCC2) cotransporters.
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| Tubuloglomerular feedback and modulation of renin release |
When tubular fl ow rates are high, there is a slight decrease in solute reabsorption before the macula densa, and thus higher concentrations of sodium and chloride are present at this area. Increased activation of NKCC2 transporters ensues, which leads to constriction of the afferent arteriole and inhibition of renin release.