MEN IIb, IIa = pheochromocytoma, medullary cancer thoryid?
MEN III = mucosa neuroma
pneumothorax = air in pleural space
hemothorax = blood in thoraxic cavity
tension pneumothorax => knife wound chest
(-) pressure
paraseptal emphysema
emphysema - pneumothorax
=> bleb rupture
cardiac tamponade
"beck's triad"
Adrenal gland
modified radical masectomy
Hasselbach's triangle
nerves around the region
cremastetic nerve - innervates testicle
genitofemoral nerve
ilioinguinal nerve
pudental nerve
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Multiple endocrine neoplasia (MEN) (or "multiple endocrine adenomas", or "multiple endocrine adenomatosis" -- "MEA") consists of three syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. The presence of any one tumor type does not automatically have a patient labelled as MEN, but a search of the other at-risk areas is usually undertaken, especially when there are suggestive clinical signs.
MEN syndromes are inherited as autosomal dominant disorders. Medullary carcinoma of the thyroid may occur as an autosomal dominant in the absence of other features.
------------------------------------------------------------------------| ICD-10 | D44.8 |
|---|---|
| ICD-9 | 258.0 |
| ICD-O: | 8360/1 |
| OMIM | 131100 |
| DiseasesDB | 7971 |
| MedlinePlus | 000398 |
| eMedicine | med/2404 |
Type 1 is also known as Wermer's syndrome after Dr Paul Wermer, who described it in 1954:
- Parathyroid hyperplasia/tumour causing hyperparathyroidism.
- Pancreatic islet cell tumours causing hypoglycaemia (insulinoma) and Zollinger-Ellison syndrome (gastrinoma).
- Pituitary adenoma which may cause pituitary hormone excess.
The causative mutation is in the menin gene which encodes a nuclear protein that is believed to act as a tumor suppressor. Most cases of multiple endocrine neoplasia type 1 are inherited in an autosomal dominant pattern.
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MEN type 2
MEN syndrome types 2 and 3 have their basis in molecular genetics. Individuals can be tested for this genetic disorder reliably even when asymptomatic. The mutation is in the RET oncogene. Most cases of multiple endocrine neoplasia types 2 and 3 are inherited in an autosomal dominant pattern.
| ICD-10 | D44.8 |
|---|---|
| ICD-9 | 193 |
| OMIM | 171400 |
| DiseasesDB | 7984 |
| MedlinePlus | 000399 |
| eMedicine | med/1520 |
Type 2 is also known as Sipple syndrome (after the American Dr John H. Sipple, who described it in 1961) and used to be called type 2A:
- Medullary carcinoma of the thyroid which is associated with increased calcitonin secretion. A test for elevated calcitonin should be done after pentagastrin injection and/or calcium infusion, to ensure that all affected patients are detected.
- Pheochromocytoma
- Parathyroid hyperplasia/tumour causing hyperparathyroidism.
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MEN type 3
| ICD-10 | D44.8 |
|---|---|
| OMIM | 162300 |
| DiseasesDB | 7991 |
| eMedicine | med/1520 |
This syndrome has no eponym; it was described by Schimke et al in 1968. Originally thought to be a third MEN, then considered a variant of II (especially after linkage to RET was confirmed), it is now considered its own syndrome.
- Pheochromocytoma
- Medullary carcinoma of thyroid which is associated with increased calcitonin secretion. A test for elevated calcitonin should be done after pentagastrin injection and/or calcium infusion, to ensure that all affected patients are detected.
- Mucosal neuromas which are usually situated in the gastrointestinal tract.
- Marfanoid habitus
A ZES(Gastrinoma) is a tumor that secretes gastrin.
It is frequently the source of the gastrin in Zollinger-Ellison syndrome.
It is most commonly found in the pancreas and duodenum.
Treatment can involve surgery or proton pump inhibitors.
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Pronunciation: n(y)
-
r
-m
Function: noun
Inflected Form(s): plural -mas also -ma·ta /-m
t-/1 : a tumor or mass growing from a nerve and usually consisting of nerve fibers
2 : a mass of nerve tissue in an amputation stump resulting from abnormal regrowth of the stumps of severed nerves -- called also amputation neuroma
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In medicine (pulmonology), a pneumothorax or collapsed lung is a medical emergency caused by the collapse of the lung within the pleural cavity.
It can result from:
- A penetrating chest wound
- Barotrauma to the lungs
- Spontaneously (most commonly in tall, slim young males)
- Chronic lung pathologies including emphysema, asthma
- Acute infections
- Chronic infections, such as tuberculosis
- Cancer
- Catamenial pneumothorax (due to endometriosis in the chest cavity)
Pneumothoraces are divided into tension and non-tension pneumathoraces. A tension pneumathorax is a medical emergency as air accumulates in the pleural space with each breath. The remorseless increase in intrathoracic pressure results in massive shifts of the mediastinum away from the affected lung compressing intrathoracic vessels. A non-tension pneumothorax by contrast is a less severe pathology because the air in the pneumothorax is able to escape.
The accumulation of blood in the thoracic cavity (hemothorax) exacerbates the problem, creating a pneumohemothorax.
Clinical treatment
Small Pneumothoraces require no treatment other than repeat observation via Chest X-rays.
Larger Pneumothoraces may require tube thoracostomy, also known as chest tube placement. A tube is inserted into the chest wall outside the lung and air is extracted using a simple one way valve or vacuum and a water valve device, depending on severity. This allows the lung to re-expand within the chest cavity. The pneumothorax is followed up with repeated X-rays. If the air pocket has become small enough, the vacuum drain can be clamped temporarily or removed.
In case of penetrating wounds, these require attention, but generally only after the airway has been secured and a chest drain inserted. Supportive therapy may include mechanical ventilation.
Recurrent pneumothorax may require further corrective and/or preventative measures such as pleurodesis. If the pneumothorax is the result of bullae, then bullaectomy (the removal or stapling of bullae or other faults in the lung) is preferred. Pleurodesis is the injection of a chemical irritant that triggers an inflammatory reaction, leading to adhesion of the lung to the parietal pleura. Substances used for pleurodesis include talc, blood and bleomycin.
------------------------------------------------------------------------A hemothorax (or haemothorax) is a condition that results from blood accumulating in the pleural cavity. Its cause is usually traumatic, from a blunt or penetrating injury to the thorax, resulting in a rupture of either of the serous membrane lining the thorax and covering the lungs. This rupture allows blood to spill into the pleural space, equalizing the pressures between it and the lungs. Blood loss may be massive in people with these conditions, as each side of the thorax can hold 30%-40% of a person's blood volume. If left untreated, the condition can progress to a point where the blood accumulation begins to put pressure on the mediastinum and the trachea, effectively limiting the amount of diastolic filling of the ventricles and deviating the trachea to the unaffected side.
[edit] Signs and symptoms
- Tachypnea.
- Dyspnea.
- Cyanosis.
- Decreased or absent breath sounds on affected side.
- Tracheal deviation.
- Dull resonance on percussion.
- Unequal chest rise.
- Tachycardia.
- Hypotension.
- Pale, cool, clammy skin.
- Possibly subcutaneous air.
- Narrowing pulse pressure.
[edit] Management
A hemothorax is managed by removing the source of bleeding and by draining the blood already in the thoracic cavity. Blood in the cavity can be removed by inserting a drain (chest tube) in a procedure called a tube thoracostomy. Patients should recover swiftly after this. However, if the cause is rupture of the aorta in high energy trauma, the intervention by a thoracic surgeon is mandatory.
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A tension pneumothorax is a life-threatening condition that results from a progressive deterioration and worsening of a simple pneumothorax, associated with the formation of a one-way valve at the point of rupture.[1]
Upon inspiration, when the pressure inside the chest and pleural cavity lessens as a result of the respiratory muscles increasing chest dimensions, air is sucked in through this one way valve, into the pleural space. Because expiration is a passive process, there is an insignificant amount of pressure created to force the air back out of the pleural cavity. This condition over time results in a gradual accumulation of air to the degree that it begins to put pressure on the mediastinum, compressing the heart and decreasing cardiac output due to the reduced amount of diastolic filling of the ventricles, and also putting pressure against the trachea, deviating it from the midline. Because of the increased thoracic pressure, venous return to the heart is decreased, causing a backup of blood into the venous system, as is evidence by distended jugular veins.[1]
Signs and symptoms
- Decreased or absent breath sounds on the affected side
- Jugular venous distension
- Tracheal deviation towards unaffected side[1]
- Hyperesonance on percussion[1]
- Unequal chest rise[1]
- Dyspnea
- Tachypnea[1]
- Tachycardia[1]
- Hypotension[1]
- Hypoxia[1]
- Pale, cool, clammy skin
- Subcutaneous air
- Cyanosis
[edit] Differentiation
A tension pneumothorax is a condition whose signs and symptoms resemble very closely those of a condition called pericardial tamponade. A chest x-ray will distinguish the two. On physical exam, the differentiating factors are:
- Pericardial tamponade
- Breath sounds: Equal on both sides
- Trachea: Midline
- Percussion: Normal resonance
- Pulse: Affected by breathing, called pulsus paradoxus, or simply paradoxical pulse.
- Tension pneumothorax
- Breath sounds: Decreased or absent on affected side
- Trachea: Deviated to unaffected side
- Percussion: Hyper-resonance
- Pulse: Normal
[edit] Treatment
Initial treatment involves the insertion of a large bore cannula or needle into the second intercostal space on the mid-clavicular line (known as "needle thoracostomy", or more commonly, "needle decompression"), thereby releasing the pressure in the pleural cavity and converting the tension pneumothorax to a simple pneumothorax, which is then treated at the earliest opportunity by inserting a chest tube.[1]
Tension pneumothorax represents a medical emergency which cannot often accommodate the time spent waiting for the capture and interpretation of a chest radiograph. Consequently, the decision to proceed with needle decompression must be made clinically (i.e., "at the bedside") by observing the acute presentation and reviewing relevant history. There is some debate on the topic of needle thoracostomy. There are risks associated with the process such as lung laceration, especially if no tension pneumothorax condition is present, and that relieved tension may reaccumulating undetected if the needle thoracostomy becomes dislodged. There is also the possibility that the canula will not reach the pleural cavity due to a thick chest wall, especially in overweight individuals.[2][3]
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EmphysemaClinical:
Emphysema is characterized by parenchymal destruction distal to the terminal bronchioles, without fibrosis, and irreversible airflow obstruction [13]. Pulmonary function studies are relatively insensitive to the early detection of emphysema- up to 30% of the lung units can be destroyed without producing a functional abnormality .
Medical treatment for emphysema involves smoking cessation, bronchodilators, steroids, supplemental oxygen, and exercise training. Lung volume reduction surgery is an option for selected patients.
Four subtypes of emphysema have been described according to their locations in the secondary pulmonary lobule: Panlobular, centrilobular, distal lobular (paraseptal), and paracicatrical (associated with scar and fibrosis).
Centtribular emphysema:
Centrilobular emphysema is the most common form and is strongly associated with cigarette smoking. The specific pathophysiologic mechanism by which smoking causes centrilobular emphysema has not been definitively identified, but is likely related to an imbalance in protease-antiprotease activity. Cigarette smoke inactivates antiprotease protective enzymes in the lungs and causes macrophage activation within the respiratory bronchioles that release elastolytic enzymes. It tends to predominate in the upper portions of the individual lobes (apices in the upper lobes, superior segments of the lower lobes). There is central destruction of the secondary lobule- alveoli around the proximal respiratory bronchioles (first and second generation respiratory bronchioles) are destroyed, while more distal alveoli are spared. The emphysematous spaces can coalesce to form bulla (sharply demarcated areas of emphysema larger than 1 cm in diameter with invisible or pencil thin walls). Although there is a communication between the tracheobronchial tree and a bulla, gas exchange between them is slow. Development of a fluid level within a bulla or wall thickening suggests infection.
Panlobular emphysema
In panlobular emphysema, which predominates in the lower lungs, there is destruction of the entire secondary lobule back to the level of the terminal bronchiole (ie: There is destruction of lung distal to the terminal bronchiole. Since the acinus consists of all parenchymal tissue distal to the terminal bronchiole, panlobular emphysema affects the entire acinous). Panlobular emphysema is seen most commonly in alpha-1-antitrypsin (alpha-1-protease inhibitor) deficiency and Swyer-James syndrome. Alpha-1-antitrypsin is a protein that blocks the destructive action of neutrophil elastase on the lung elastin fibers. Smoking causes an earlier onset of symptoms in patients with homozygous alpha-1-antitrypsin deficiency. Heterozygous deficiency has not been associated with an increased risk for panlobular emphysema. Panlobular emphysema may also be seen in smokers without alpha-1-antitrypsin deficiency (although this is controversial) and the elderly. IV abuse of talc containing medications and methylphenidate (Ritalin) also have a tendency to cause predominantly lower lobe panacinar emphysematous changes.
Paraseptal emphysema:
Paraseptal emphysema involves the periphery of the pulmonary lobule, especially adjacent to connective tissue septa. It is most prominent in a subpleural location, typically within the apices. It is associated with spontaneous pneumothorax, but not with air-flow obstruction. Although paraseptal emphysema can be seen as an isolated abnormality, it is most often associated with centrilobular emphysema. Paraseptal emphysema has also been associated with idiopathic giant bullous emphysema.
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A bleb is a large blister filled with serous fluid. Blebs can form on a number of tissues due to different pathologies, including frostbitten tissues and damaged cell membranes. In ophthalmology, blebs may be formed intentionally in the treatment of glaucoma.
A bleb can also be a cytoplasmic extension present during apoptosis. The formation process is called blebbing.
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Cardiac tamponade is a medical emergency condition where liquid accumulates in the pericardium in a relatively short time. The elevated pericardial pressure prevents proper filling of heart cavities. Instead of reducing the filling of both ventricles equally, the septum of the heart will bend into either the left or right ventricle. The end result is low stroke volume, shock and often death.Causes
Cardiac tamponade can happen acutely, such as from a stab wound, from surgical complications, or from heart muscle rupture. Although heart rupture is uncommon, if it occurs, it will usually do so around the site of myocardial infarction. Chronic cardiac tamponade is a slower process in which up to two litres of fluid can enter the pericardial space over a period of time, and the pericardium stretches to accommodate the volume. Rapid onset of cardiac tamponade can occur with as little as 80 ml of fluid accumulation. Other conditions are constrictive pericarditis in which the pericardium shrinks and hardens.
[edit] Diagnosis
Signs and symptoms of cardiac tamponade can appear very similar to congestive heart failure. Usually, however, the differential diagnosis can be made via a history of sudden onset attributable to trauma, particularly in younger patients.
Identification of cardiac tamponade relies upon Beck's triad: hypotension, jugular vein distension, and muffled heart sounds resulting from accumulated fluid dampening sound transmission through the chest wall. In pre-hospital settings, identification of the quiet heart sounds can be difficult. It is important to note the baseline condition during the primary survey and recognize a downward trend.
Tension pneumothorax is the major differential diagnosis of cardiac tamponade. A tension pneumo will present with a deviated trachea and unequal breath sounds. Cardiac tamponade presents with a midline trachea and equal breath sounds, unless comorbid with either hemothorax or pneumothorax. A paradoxical pulse may also present in cardiac tamponade.
Clinical treatment
Pericardiocentesis, needle evacuation of the fluid and lowering of the pericardial pressure, and then treatment of the underlying cause, is life-saving. Often, a pericardial drain is left in situ to prevent short-term recurrence. Surgery to repair the damage to the heart is often required.
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Beck's triad is comprised of fall in the systolic pressure, rising jugular venous pressure and suppressed heart sounds. These findings are typical of cardiac tamponade.
1: The rising jugular venous pressure is evidenced by distended jugular veins while in a non-supine position. It is caused by reduced diastolic filling of the right ventricle, due to the outside pressure being exerted on it by the expanding pericardial sac. This results of a backup of fluid into the veins draining into the heart, most notably, the jugulars. In severe hypovolaemia, the neck veins may NOT be distended.
2: The fall in systolic pressure results when the fluid in the pericardial cavity accumulates to a degree that it impairs ventricular stretch, thus reducing stroke volume and cardiac output.
3: The suppressed heart sounds occur due to the muffling effects of the sounds passing through the fluid surrounding the heart.
Associated persons: Claude Schaeffer Beck
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Adrenal gland
In mammals, the adrenal glands (also known as suprarenal glands) are the triangle-shaped endocrine glands that sit atop the kidneys; their name indicates that position (ad, "near" or "at" + renes, "kidneys"). They are chiefly responsible for regulating the stress response through the synthesis of corticosteroids and catecholamines, including cortisol and adrenaline.
Contents[hide] |
[edit] Overview
Above each human kidney is one of the two adrenal glands.
Anatomically, the adrenal glands are located in the abdomen, situated on the anteriosuperior aspect of the kidneys. In humans, the adrenal glands are found at the level of the 12th thoracic vertebra and receive their blood supply from the adrenal arteries.
It is separated into two distinct structures, the adrenal medulla and the adrenal cortex, both of which receive regulatory input from the nervous system. As its name suggests, the adrenal medulla is at the center of the adrenal gland surrounded by the adrenal cortex.
The adrenal medulla is the body's main source of the catecholamine hormones adrenaline (epinephrine) and noradrenaline (norepinephrine). By contrast, some cells of the adrenal cortex belong to the hypothalamic-pituitary-adrenal axis and are the source of cortisol synthesis. Other cortical cells produce androgens such as testosterone, while some regulate water and electrolyte concentrations by secreting aldosterone.
Adrenal medulla
From Wikipedia, the free encyclopedia
Composed mainly of hormone-producing chromaffin cells, the adrenal medulla is the principal site of the conversion of the amino acid tyrosine into the catecholamines adrenaline (epinephrine) and noradrenaline (norepinephrine). Medullary cells are derived from the embryonic neural crest and, as such, are simply modified neurons. In particular, they are modified postganglionic cells of the sympathetic nervous system that have lost their axons and dendrites, receiving innervation from corresponding preganglionic fibers. Moreover, as the synapses between pre- and postganglionic fibers are called ganglia, the adrenal medulla is actually a ganglion of the sympathetic nervous system.
In response to stressors such as exercise or imminent danger, medullary cells release catecholamines into the blood in a 70:30 ratio of adrenaline to noradrenaline. Notable effects of adrenaline and noradrenaline include increased heart rate, blood vessel constriction, bronchiole dilation, and increased metabolism, all of which are characteristic of the fight-or-flight response.
[edit] Pathology
- Pheochromocytoma is a catecholamine-secreting tumor of the adrenal medulla.
Modified Radical Mastectomy
Woman with modified radical mastectomy.
A pink highlighted area indicates tissue removed at mastectomy
B axillary lymph nodes: levels I
C axillary lymph nodes: levels II
D axillary lymph nodes: levels III
More on mastectomy, including frequently asked questions.
There are several different types of surgical procedures used to treat breast cancer. Depending on the location or surgeon who performs the procedure, different terms may be used.
Surgical procedures for breast cancer include:
- Simple or total mastectomy: removal of the breast, with its skin and nipple, but no lymph nodes. In some cases, a separate sentinel node biopsy is performed to remove only the first one to three axillary (armpit) lymph nodes.
- Modified radical mastectomy: removal of the entire breast, nipple/areolar region, and often the axillary lymph nodes. This is the most common form of mastectomy performed today.*
- Radical mastectomy: removal of the entire breast, nipple/areolar region, the pectoral (chest) major and minor muscles, and lymph nodes. This procedure is rarely performed today.*
- Quandrantectomy: removal of a quarter of the breast, including the skin and breast fascia (connective tissues). The surgeon may also perform a separate procedure to remove some or all of the axillary (armpit) lymph nodes, either an axillary node dissection or a sentinel node biopsy.
- Partial or segmental mastectomy: removal of a portion of the breast tissue and a margin of normal breast tissue. This procedure usually involves removing less tissue than a quandrantectomy but more than a lumpectomy or wide excision.
- Lumpectomy or wide excision: removal of the breast cancer tumor and a surrounding margin of normal breast tissue.
- Excisional biopsy also the removal of the breast tumor and a surrounding margin of normal breast tissue. Sometimes further surgery is not needed if an excisional biopsy successfully removes the entire breast cancer tumor. This is most likely to occur if the breast tumor is very small. An excisional biopsy may be performed with "needle" or "wire" localization.
*In the past, radical mastectomy was the frequently performed on women with breast cancer. However, experts have found that modified radical mastectomy is equally effective in most cases, and therefore, it has become the most common type procedure for removing the entire breast.
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| Radical Mastectomy. | Modified Radical Mastectomy. |
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| Simple (total) Mastectomy. | Partial Mastectomy |
