This post has been written in response to the comment on this article.

The original question was

I am one of medical student in ETHIOPIAN university and I am confused how clubbing is happen? i.e the pathophysiology of clubbing.

Widely speaking the specific  mechanism of digital clubbing is still not known.

Increased interstitial edema is the earliest change. As clubbing progresses, the volume of the terminal portion of the digit may increase because of an increase in the vascular connective tissue and change in quality of  the tissue.

It is postulated that different pathological diseases may cause different pathways to a common end point.

Most researchers agree that this results from an increase in distal digital vasodilation is the major factor. But what causes the vasodilation remains unclear. Also unknown is the exact mechanism by which increased blood flow results in changes in the vascular connective tissue under the nail bed.  A circulating or local vasodilator, neural mechanism, response to hypoxemia, genetic predisposition, or a combination of these are thought as possible factors/

An increased incidence of digital clubbing has been associated with the vagally innervated organs and therefore a neural mechanism has also been purposed.

Genetic inheritance and predisposition has also been proposed.

Recent studies have suggested platelet-derived growth factor as the possible mediator of the digital clubbing. This factor has been shown to have general growth-promoting activity and causes increased capillary permeability and connective tissue hypertrophy.

The bleeding in hemophilia has been divided into serious and life threatening.

Serious Bleeding

• Joints (hemarthrosis)
• Muscle/soft tissue
• Mouth/gums/nose
• Hematuria

Life-threatening

• Central nervous system
• Gastrointestinal
• Neck/throat

Incidence Of Bleeding In Different Sites

• Hemarthrosis- 70%-80%
• Muscle/soft tissue-10%-20%
• Other major bleeds- 5%-10%
• Central nervous system (CNS) bleeds: < 5%

Incidence of Bleeding In Different Joints

• Knee: 45%
• Elbow: 30%
• Ankle: 15%
• Shoulder: 3%
• Wrist: 3%
• Hip: 2%
• Other: 2%

Addisonian crisis is also called adrenal crisis. It  is a constellation of symptoms that indicate severe adrenal insufficiency.

It may occur  in either previously undiagnosed Addison’s disease due to  a disease process suddenly affecting adrenal function. It may  also due to an intercurrent problem like  infection or  trauma in the setting of known Addison’s disease.

The  situation may also develop in those on long-term oral glucocorticoids who have suddenly stopped taking their medication.

Addisonian crisis is also a concern in the setting of myxedema coma. Thyroxine given in that setting without glucocorticoids may precipitate a crisis.

Untreated, an Addisonian crisis can be fatal.

It is a medical emergency, usually requiring hospitalization.

Following symptoms would suggest Addisonian crisis

• Sudden penetrating pain in the legs, lower back or abdomen
• Severe vomiting and diarrhea, resulting in dehydration
• Low blood pressure
• Syncope (loss of consciousness)
• Hypoglycemia
• Confusion, psychosis, slurred speech
• Severe lethargy
• Hyperkalemia
• Hypercalcaemia
• Convulsions
• Fever

Prevention

It is recommended that a person persn with adrenal insufficiency should always carry identification stating their condition in case of an emergency.

The card should alert emergency personnel about the need to inject 100 mg of cortisol if its bearer is found severely injured or unable to answer questions. The card should also include the doctor’s name and telephone number and the name and telephone number of the nearest relative to be notified.

When traveling, a needle, syringe, and an injectable form of cortisol should be carried for emergencies. A person with Addison’s disease also should know how to increase medication during periods of stress or mild upper respiratory infections. Immediate medical attention is needed when severe infections, vomiting, or diarrhea occur, as these conditions can precipitate an Addisonian crisis.

A patient who is vomiting may require injections of hydrocortisone, since oral hydrocortisone supplements cannot be adequately metabolised.

Addison’s disease is also called as chronic adrenal insufficiency, hypocortisolism or hypocorticism. It is a rare disorder where the adrenal glands are not able to produce glucocorticoids and less often mineralocorticoids in sufficient quantity. The condition was first described by Dr Thomas Addison, the British physician who first described the condition.

Causes of Addison disease

Adrenal dysgenesis

This means adrenal glands which produce glucocorticoid are absent.

Impaired Formation of Hormone

To form cortisol, the adrenal gland requires cholesterol, which is then converted biochemically into steroid hormones. Interruptions in the delivery of cholesterol include Smith-Lemli-Opitz syndrome and abetalipoproteinemia.

Congenital adrenal hyperplasia may also cause impaired synthesis

Medications

Medicationslike ketoconazole  interfere with  enzymes that synthesize the hormone, while others like rifampicin, phenytoin increase hormonal breakdown.

Adrenal destruction

• Autoimmune adrenalitis
• Adrenoleukodystrophy (ALD)
• Metastasis (
• Hemorrhage (Waterhouse-Friderichsen syndrome or antiphospholipid syndrome)
• Infection
• Amyloidosis.

What Are Symptoms and signs Of Addison Disease

The disease symptoms are insidious in onset and per se the symptoms are quite non specific. Following symptoms can be noted with this condtion.

• Fatigue
• Lightheadedness upon standing or while upright
• Muscle weakness
• Weight loss
• Anxiety
• Nausea, vomiting, diarrhea
• Headache, sweating
• Changes in mood and personality
• Vague joint and muscle pains.
• Marked cravings for salt or salty foods [Due to loss of salt in urine]
• Darkening of the skin [Due to hypepigmentation]
• Orthostatic hypotension [Low blood pressure that falls further when standing]

Routine Lab Investigations may show

• Hypercalcemia or low calcium level
• Hypoglycemia or low blood sugar level
• Hyponatraemia  or low blood sodium levels
• Hyperkalemia  or raised blood potassium levels
• Eosinophilia and lymphocytosis
• Metabolic acidosis (increased blood acidity), also due to loss of the hormone aldosterone

Special Tests

ACTH stimulation test

Low adrenal hormone levels even after appropriate stimulation with synthetic pituitary ACTH hormone tetracosactide

Renin and adrenocorticotropic hormone levels,  ultrasound, computed tomography or magnetic resonance imaging are other tests used in making a diagnosis.

Treatment

Treatmentaims at replacing the missing cortisol. Hydrocortisone  or prednisone tablets  are commonly used and are continued for life. In addition, many patients require fludrocortisone as replacement for the missing aldosterone.

When the umbilicus is stretched transversely as in ascites, it is called smil9ng umbilicus.

An umbilicus that exudes fluid e.g. ascetic fluid in massive ascites or feculent material in enteric fistulae, or clear fluid in patent urachus is called crying umbilicus.

Swellings in the Abdominal Wall

Cold Abscess

1. Fluctuant swelling with no signs of inflammation
2. Swelling becomes prominent when the abdominal muscles contract
3. Irregularity in the affected rib or deformity of the spine

Intra-abdominal Swellings

Hepatic

1. It moves with respiration but is not mobile sideways
2. The swelling is continuous with the liver dullness without a band of colonic resonance

Gall Bladder

1. Oval smooth swelling, the size of an egg
2. Moves with respiration, can be moved sideways but cannot be pushed down into the loin (like kidney swelling)

Chronic Cholecystitis and Cholelithiasis

1. Pain over the right rectus muscle radiating to the inferior angle of scapula, aggravated after fatty meals. Often the patient makes an attempt to get relief by frequent belching or vomiting but relief is seldom complete.
2. Gall bladder may be palpable.
3. Murphy’s sign is positive: i.e. Tenderness under the right costal margin at the lateral border of the rectus muscle when the patient takes a deep breath. This occurs due to the descent of the inflamed gallbladder which touches the examiner’s fingers. If a stone is present in the common bile duct there is a triad of intermittent colic, intermittent jaundice and fever with chills and rigors. By Courvoisier’s law, gall bladder is not palpable.

Sub-Phrenic Abscess

1. Pain in the right hypochondrial region referred to the shoulders
2. Diffuse tender swelling in the right hypochondrial region
3. Signs of septicemia: High fever with rigors, sweating and marked tachycardia
4. Screening: Raised and fixed diaphragm with gas under it
5. Features of the causative condition e.g. perforated peptic ulcer, liver abscess

Stomach and Duodenum

Carcinoma of Pylorus:

1. There is irregular firm lump which moves on respiration
2. Patient is usually elderly and has anorexia and weight loss
3. Barium meal would show filling defect

Sub-Acute Perforation of a Peptic Ulcer

1. Localized, tender, inflammatory mass may be present with a central abscess
2. History of peptic ulcer
3. Barium meal would reveal the ulcer

Hepatic Flexure of Colon

Hypertrophic Tuberculosis

This usually causes a lump in the right iliac fossa which may be drawn towards the right hypochondriac region by fibrosis.

Carcinoma of Colon

1. This commonly occurs in men above the age 40 years
2. There is alternate diarrhea and constipation.
3. The lump is irregular, firm and moves poorly on respiration
4. Occult blood may be present in stools
5. Filling defect may be seen on barium enema.

Intussusception

1. There is sudden intermittent abdominal pain with vomiting.
2. Absolute constipation may be replaced later by passage of blood and mucus (red current jelly) per anum without fecal odour.
3. There may be curved, sausage shaped lump in the line of the colon with its concavity towards the umbilicus. The lump may harden under examining fingers synchronously with an attack of screaming.
4. Barium enema would show typical pincer shaped ending of the radio-opaque material.

It is also known as Arterial spider, Spider telangiectasis, Spider angioma

Definition:

An arterial spider is a central arteriole, from which numerous small vessels radiate resembling a spider’s legs.

Sites:

Arterial spiders are found in the territory of the superior vena cava. They are commonly seen on the face, neck, forearm and shoulder.

Appearance:

They range in size from 3 to 15 mm in diameter. They are pulsatile and blanch on pressure. When the skin is stretched or compressed they fill from the centre to the periphery.

Causes:
1. Cirrhosis
2. In alcoholics
3. Viral hepatitis
4. Normal persons especially children
5. Pregnancy
6. Rheumatoid arthritis
7. Thyrotoxicosis

Mechanism

The selective distribution of spider nevi is not understood.
1. Exposure of upper parts of the body to certain elements may damage the skin so that it is susceptible to develop spider nevi when an appropriate internal stimulus exists.
2. Estrogen excess may be responsible. It forms arterioles of the endometrium in uterus during pregnancy which resembles spider nevi.

Significance

Appearance of fresh spiders suggest progression of liver damage.

Spider nevi disappears:
1. If liver function improves
2. If blood-pressure falls due to shock or hemorrhage

Examination of the abdomen begins with inspection. The shape of the abdomen in most normal persons with normal musculature is  boat-shaped i.e. the abdominal wall sinks slightly within the bony margins of the abdominal surface.

In a very muscular person, the lateral margin of the rectus muscle is visible in the center. Usually the medial edges of both the recti are contiguous. However they may be separated as a congenital defect, after pregnancy or with obesity and ascites. This is called divarication of recti.

Scaphoid or sunken abdomen is seen with starvation and malignancy especially of stomach and esophagus.

Umbilicus

Normal umbilicus is usually inverted and situated centrally in the mid-abdomen. The distance between the xiphisternum and the umbilicus is equal to the distance between the umbilicus and symphysis pubis.

In ascites, the distance between xiphisternum and umbilicus is greater than that between umbilicus and symphysis pubis, whereas in ovarian tumour the distance between xiphisternum and umbilicus is less than that between umbilicus and symphysis pubis.

In ascites, te umbilicus is transversely stretched (smiling) or flattened or everted whereas in obesity, the umbilical cleft is deeper than norma.

Everted umbilicus may occur with herniation of bowel or fat into the widened umbilical ring. Sometimes, umbilicus may exude fluid e.g. ascetic fluid in massive ascites or feculent material in enteric fistulae, or clear fluid in patent urachus (crying umbilicus).

Bluish discoloration or periumbilical region (Cullen sign) occurs in acute haemorrhagic pancreatitis or ruptured ectopic pregnancy.

Cherry-red swelling of the umbilicus suggests inflamed Meckel’s diverticulum.

Abdominal Movements

Normally the abdominal wall bulges during inspiration and falls during expiration. In diaphragmatic paralysis the abdomen bulges during expiration. In peritonitis, the abdominal movements are absent.

Pulsations

Normally pulsations are not visible over the abdomen. They may be visible in the following conditions:

• Aortic pulsations are visible in the nervous, anaemic individual.
• Aortic aneurysm produces expansile pulsations in any position.
• Transmitted pulsations from a tumour overlying the aorta disappear in knee-elbow position because the tumour falls away from the aorta in that position. This is not so if the tumour is adherent to the aorta.
• Right ventricular pulsations are seen only in the epigastrium and correspond with the apex beat.
• Congested liver, in addition, produces pulsations posteriorly.

Dilated Veins

Suggest venous obstruction. When dilated veins are present, the direction of the blood flow can be found by emptying (milking) a section of the vein and pressing each end of the emptied part with a finger. One finger is released and the filling of the vein is noted. Similarly, the other finger is released and filling of the vein is noted. Blood enters more rapidly and fills the veins from the direction of the blood flow.

Inferior vena cava obstruction

There will be dilated veins on the sides with flow of blood from below upwards. This occurs because the blood bypasses the inferior vena cava and travels from the lower limbs to the thorax via the veins of the abdominal wall. These veins are anastomotic channels between the superficial epigastric vein and circumflexiliac veins below and the lateral thoracic vein above conveying the diverted blood from the long saphenous vein to the axillary vein.

Portal vein obstruction

The engorged veins are centrally placed and may form a cluster around the umbilicus (caput medusa). The blood in these veins flows in all directions away from the umbilicus. They represent opening of anastomosis between portal and systemic veins.

Peristalsis

Peristalsis is best elicited by patiently observing the abdomen of the patient for some time. If it is not visible, an attempt to visualize it should be made either by making the patient swallow fluids or by applying sharp tap with the finger over the abdominal wall.

Peristaltic wave of the stomach is seen in pyloric stenosis in the epigastrium and left hypochondriac region, moving from left to right. Peristaltic wave of the large intestine (transverse colon) is seen in the same region but moving from right to left. Peristaltic wave of small intestine is seen in a ladder pattern down the centre of the abdomen.

Hernia

The hernial sites in the groin should be seen for any swelling. If there is no swelling, the patient should be asked to stand up, turn his head to one side and cough. If there is an impulse on coughing it suggests hernia.

Skin

Smooth and glossy skin indicates abdominal distension whereas wrinkled skin suggests old distension which has been relieved.

Abdominal striae (stretch marks) represent the rupture of subepidermal connective tissue as a result of recent or past abdominal distension. It is seen commonly following pregnancy, in obesity, in massive ascites and following corticosteroid therapy. When they first form, the striae are reddish or pink. If the state of distension stabilizes or the cause regresses, the colour fades to white.

In a patient with an abdominal disorder, the following points must be asked whilst taking the history and noted on examination.

History

History is of paramount importance in clinical examination. It enables you to narrow your examination to a region and also guides you about the likelihood of particular disorder. Following points should be asked in patient of abdominal complaints. These points cover whole abdomen in general and can be individualized depending upon the complaint.

• Anorexia, nausea, vomiting
• Dysphagia, flatulence, eructation
• Retrosternal burning, water brash
• Diarrhea, constipation, clay stools, worms in stools, mucus in stools
• Abdominal pain, abdominal lump, abdominal distension
• Hematemesis, melena, epistaxis, bleeding per rectum
• Jaundice, gynecomastia, libido, loss of hair
• Fever, weight loss
• Alcohol, smoking
• Past history of tuberculosis, malaria, kala-azar, leukemia, hemolytic crisis (sudden pallor and dyspnea) and drugs

General Examination

• Built and nutrition
• Nails and conjunctiva: Pallor, clubbing, cyanosis, icterus, etc.
• Edema of feet, lymphadenopathy, JVP
• Blood pressure
• Signs of liver cell failure: Scanty hair, palmarerythema, spider nevi, parotid swelling, gynecomastia, testicular atrophy, Dupuytren’s contractures.
• Miscellaneous: Bony tenderness, petechiae, genitals

An overview of Alimentary System Examination

Oral cavity

Teeth, Tonsils, Tongue, Oropharynx

Abdomen

Inspection:

Shape of abdomen, Umbilicus, Abdominal movements, Pulsations, Dilated veins, Peristalsis, Hernial orifices, Scars and sinuses, Skin.

Palpation:

1. Tenderness, guarding and rigidity
2. Liver, spleen, kidney, gall-bladder, colon, or any other lump (Its size, surface, borders, tenderness and bruit)
3. Fluid thrill

Percussion:

1. Horse-shoe and shifting dullness
2. Dullness over any lump, if palpable.

Auscultation:

Peristalsis, Rub, Bruit

Miscellaneous:

Abdominal girth, PR, Proctoscopy

Normal Jugular Venous Pulse (JVP):

The normal JVP consists of three positive pulse waves a, cand v and two negative pulse waves x and y.

The ‘A’ Wave: The ‘a’ wave is produced by retrograde transmission of the pressure pulse produced by right atrial contraction. In normal subjects the ‘a’ wave is often the largest positive wave visible, coinciding with the fourth heart sound.

1. ‘a’ wave is absent in atrial fibrillation

2. ‘a’ wave is diminished in

• Tachycardia
• Prolonged PR interval

3. Large or giant ‘a’ waves are present in

• Tricuspid stenosis
• Tricuspid atresia
• Right atrial myxoma
• Pulmonary stenosis
• Pulmonary hypertension

4. Cannon ‘a’ waves occur in

• Complete heart block when the right atrium and right ventricle contract simultaneously with a closed tricuspid valve.
• Ventricular tachycardia
• Ectopic beats

The ‘C’ Wave: the ‘c’ wave is produced by two events:

1. Impact of the carotid artery adjacent to the jugular vein.
2. Retrograde transmission of a positive wave in the right atrium produced by the right ventricular systole and the bulging of the tricuspid valve into the right atrium.

It normally begins at the end of the first heart sound and reaches its peak shortly after the first heart sound. The ‘c’ wave is not often seen clinically.

The ‘X’ Wave: ‘x’ descent is produced by:

1. The downward displacement of the tricuspid valve during ventricular systole and resultant fall in right atrial pressure.
2. Continued atrial relaxation.

Abnormalities of ‘x’ wave

1. The ‘x’ descent is obliterated or may be replaced by a positive wave (‘s’ wave) in tricuspid regurgitation. This ‘s’ wave may fuse with ‘c’ and ‘v’ waves to produce a giant ‘v’ wave.
2. The ‘x’ wave may sometimes be prominent in constrictive pericarditis.

The ‘V’ Wave: The ‘v’ wave occurs because of right atrial filling with the tricuspid valve closed during ventricular systole.

Abnormalities of ‘v’ wave

Giant ‘v’ waves, as discussed earlier, appear in tricuspid regurgitation:

The ‘Y’ Wave: The ‘y’ descent is produced by opening of the tricuspid valve and subsequent rapid inflow of blood from the right atrium to the right ventricle leading to a sudden fall of pressure in the right atrium which is reflected in the jugular veins. It corresponds with the third heart sound.

The ascending limb of the ‘y’ wave is due to continuous diastolic inflow of blood into the great veins, right atrium and ventricle which are all in free communication during diastole.

1. Rapid ‘y’ descent occurs in

• Constructive pericarditis (Friedreich’s sign).
• Severe heart failure.
• Tricuspid regurgitation.

2. A short ‘y’ descent occurs in tricuspid stenosis

Jugular Venous Pressure

Normal: 3-4 cm of water.

Procedure: The patient is given a back rest to keep him at 45 degree. In this position, normally, the jugular vein is just seen above the clavicles. The upper level of the vein is noted and a ruler is kept at that level, parallel to the ground.

Another rule is put perpendicular to the first ruler upto the angle of Louis. The distance from the angle of Louis. The distance from the angle of Louis to the first ruler gives the jugular pressure. In the supine position the jugular pressure may falsely appear elevated whilst in the upright position it is falsely lowered.

Significance

The jugular veins are in direct continuity with the superior vena cava and the right atrium.

Elevated venous pressure occurs in:

1. Right ventricular failure
2. Cardiac tamponade
3. Tricuspid stenosis
4. Superior vena cava obstruction
5. Hyperkinetic circulator state
6. Increased blood volume
7. Pulmonary diseases like asthma, emphysema

Kussmaul’s sign

Normally inspiration lowers the jugular venous pressure giving an inspiratory collapse, because intrathoracic pressure falls and there is increased blood flow into the thorax. In contrast, when the intrapericardial pressure is raised as in constrictive pericarditis there is a paradoximal increase in jugular venous pressure on inspiration. This is called Kussmaul’s sign.

Hepatojugular Reflex

Normally, when pressure is applied over the abdomen for 30 seconds, initially there is a rise in jugular venous pressure (due to increased venous return), followed by a fall (due to the capacity of normal myocardium to accommodate the extra venous return).

However, in early cardiac failure, even before jugular pressure is elevated, there is a sustained elevated pressure in the jugular veins (for more than a minute) on pressure over the abdomen because the failing heart cannot compensate for the extra venous return. This is positive hepatojugular reflux.

Decreased venous pressure in seen in

1. Shock
2. Dehydration