Nail clubbing

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Clubbing
SynonymsDrumstick fingers, digital clubbing, watch-glass nails [1]
Acopaquia.jpg
Clubbing
Specialty Pulmonology

Nail clubbing, also known as digital clubbing, is a deformity of the finger or toe nails associated with a number of diseases, mostly of the heart and lungs. [2] [3] Clubbing for no obvious reason can also occur, but is rare. [4] [5] Hippocrates was the first to formally document clubbing as a sign of disease, and the phenomenon is therefore occasionally called "Hippocratic fingers."

Nail (anatomy) hard projection of digit

A nail is a horn-like envelope covering the tips of the fingers and toes in most primates and a few other mammals. Nails are similar to claws in other animals. Fingernails and toenails are made of a tough protective protein called alpha-keratin. This protein is also found in the hooves and horns of different animals.

Hippocrates ancient Greek physician

Hippocrates of Kos, also known as Hippocrates II, was a Greek physician of the Age of Pericles, who is considered one of the most outstanding figures in the history of medicine. He is often referred to as the "Father of Medicine" in recognition of his lasting contributions to the field as the founder of the Hippocratic School of Medicine. This intellectual school revolutionized medicine in ancient Greece, establishing it as a discipline distinct from other fields with which it had traditionally been associated, thus establishing medicine as a profession.

Contents

Causes

Isolated clubbing

Clubbing is associated with:

Lung cancer cancer in the lung

Lung cancer, also known as lung carcinoma, is a malignant lung tumor characterized by uncontrolled cell growth in tissues of the lung. This growth can spread beyond the lung by the process of metastasis into nearby tissue or other parts of the body. Most cancers that start in the lung, known as primary lung cancers, are carcinomas. The two main types are small-cell lung carcinoma (SCLC) and non-small-cell lung carcinoma (NSCLC). The most common symptoms are coughing, weight loss, shortness of breath, and chest pains.

Interstitial lung disease group of lung diseases affecting the interstitium (the tissue and space around the air sacs of the lungs)

Interstitial lung disease (ILD), or diffuse parenchymal lung disease (DPLD), is a group of lung diseases affecting the interstitium. It concerns alveolar epithelium, pulmonary capillary endothelium, basement membrane, and perivascular and perilymphatic tissues. It may occur when an injury to the lungs triggers an abnormal healing response. Ordinarily, the body generates just the right amount of tissue to repair damage, but in interstitial lung disease, the repair process goes awry and the tissue around the air sacs (alveoli) becomes scarred and thickened. This makes it more difficult for oxygen to pass into the bloodstream. The term ILD is used to distinguish these diseases from obstructive airways diseases.

Idiopathic pulmonary fibrosis chronic, irreversible and ultimately fatal disease characterized by a progressive decline in lung function

Idiopathic pulmonary fibrosis (IPF) is a type of chronic lung disease characterized by a progressive and irreversible decline in lung function. Symptoms typically include gradual onset of shortness of breath and a dry cough. Other changes may include feeling tired and nail clubbing. Complications may include pulmonary hypertension, heart failure, pneumonia, or pulmonary embolism.

Nail clubbing is not specific to chronic obstructive pulmonary disease (COPD). Therefore, in patients with COPD and significant degrees of clubbing, a search for signs of bronchogenic carcinoma (or other causes of clubbing) might still be indicated. [10]

A congenital form has also been recognized. [11]

HPOA

Bone scan of a patient with HPOA Marie-Bamberger2.jpg
Bone scan of a patient with HPOA

A special form of clubbing is hypertrophic pulmonary osteoarthropathy, known in continental Europe as Pierre Marie-Bamberger syndrome. This is the combination of clubbing and thickening of periosteum (connective tissue lining of the bones) and synovium (lining of joints), and is often initially diagnosed as arthritis. It is commonly associated with lung cancer.[ citation needed ]

Primary HPOA

Primary hypertrophic osteoarthropathy is HPOA without signs of pulmonary disease. This form has a hereditary component, although subtle cardiac abnormalities can occasionally be found. It is known eponymously as the Touraine–Solente–Golé syndrome. This condition has been linked to mutations in the gene on the fourth chromosome (4q33-q34) coding for the enzyme 15-hydroxyprostaglandin dehydrogenase (HPGD); this leads to decreased breakdown of prostaglandin E2 and elevated levels of this substance. [12]

Pathophysiology

The exact cause for sporadic clubbing is unknown, with numerous theories as to its cause. Vasodilation (i.e., distended blood vessels), secretion of growth factors (e.g., platelet-derived growth factor and hepatocyte growth factor) from the lungs, and other mechanisms have been proposed. The discovery of disorders in the prostaglandin metabolism in primary osteoarthropathy has led to suggestions that overproduction of PGE2 by other tissues may be the causative factor for clubbing. [12]

Another mechanism by which clubbing is thought to arise from is due to increased entry of megakaryocytes into the systemic circulation. Under normal circumstances in healthy individuals, megakaryocytes that arise from the bone marrow are trapped in the pulmonary capillary bed and broken down before it enters the systemic circulation. It is thought that in disorders where there is right-to-left shunting or lung malignancy, the megakaryocytes can bypass the breakdown within the pulmonary circulation and enter the systemic circulation. They are then trapped within the capillary beds within the extremities, such as the digits, and release platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF). PDGF and VEGF have growth promoting properties and causes connective tissue hypertrophy and capillary permeability. [13]

Diagnosis

Clubbing of the fingernail: The red line shows the outline of a clubbed nail. Clubbing.svg
Clubbing of the fingernail: The red line shows the outline of a clubbed nail.

When clubbing is encountered in patients, doctors will seek to identify its cause and exclude pseudoclubbing before making the diagnosis. They usually accomplish this by obtaining a detailed medical history—particular attention is paid to lung, heart, and gastrointestinal conditions—and conducting a thorough clinical examination, which may disclose associated features relevant to the underlying diagnosis. Additional studies such as a chest X-ray and a chest CT-scan may also be performed. [10]

Detection

Clubbing may be present in one of five stages: [10]

Schamroth's test or Schamroth's window test (originally demonstrated by South African cardiologist Leo Schamroth on himself) [14] is a popular test for clubbing. When the distal phalanges (bones nearest the fingertips) of corresponding fingers of opposite hands are directly opposed (place fingernails of same finger on opposite hands against each other, nail to nail), a small diamond-shaped "window" is normally apparent between the nailbeds. If this window is obliterated, the test is positive and clubbing is present.

Epidemiology

The exact frequency of clubbing in the population is not known. A 2008 study found clubbing in 1%, or 15 patients, of 1511 patients admitted to a department of internal medicine in Belgium. Of these, 40%, or 6 patients, turned out to have significant underlying disease of various causes, while 60%, or 9 patients, had no medical problems on further investigations and remained well over the subsequent year. [5]

See also

Related Research Articles

Cardiology branch of medicine dealing with disorders of the heart as well as parts of the circulatory system

Cardiology is a branch of medicine dealing with disorders of the heart as well as parts of the circulatory system. The field includes medical diagnosis and treatment of congenital heart defects, coronary artery disease, heart failure, valvular heart disease and electrophysiology. Physicians who specialize in this field of medicine are called cardiologists, a specialty of internal medicine. Pediatric cardiologists are pediatricians who specialize in cardiology. Physicians who specialize in cardiac surgery are called cardiothoracic surgeons or cardiac surgeons, a specialty of general surgery.

Pleural cavity thin fluid-filled space between the two pulmonary pleurae (visceral and parietal) of each lung

The pleural cavity is the thin fluid-filled space between the two pulmonary pleurae of each lung. A pleura is a serous membrane which folds back onto itself to form a two-layered membranous pleural sac. The outer pleura is attached to the chest wall, but is separated from it by the endothoracic fascia. The inner pleura covers the lungs and adjoining structures, including blood vessels, bronchi and nerves. The pleural cavity can be viewed as a potential space because the two pleurae adhere to each other under all normal conditions. Parietal pleura projects up to 2.5 cm above the junction of the middle and medial third of the clavicle

Pleural effusion accumulation of excess fluid in the pleural cavity

A pleural effusion is excess fluid that accumulates in the pleural cavity, the fluid-filled space that surrounds the lungs. This excess fluid can impair breathing by limiting the expansion of the lungs. Various kinds of pleural effusion, depending on the nature of the fluid and what caused its entry into the pleural space, are hydrothorax, hemothorax (blood), urinothorax (urine), chylothorax (chyle), or pyothorax (pus). A pneumothorax is the accumulation of air in the pleural space, and is commonly called a "collapsed lung".

Cyanosis medical diagnosis

Cyanosis is the bluish or purplish discolouration of the skin or mucous membranes due to the tissues near the skin surface having low oxygen saturation. Based on Lundsgaard and Van Slyke's work, it is classically described as occurring if 5.0 g/dL of deoxyhemoglobin or greater is present. This was based on an estimate of capillary saturation based on a mean of arterial versus peripheral venous blood gas measurements. Since estimation of hypoxia is usually now based either on arterial blood gas measurement or pulse oximetry, this is probably an overestimate, with evidence that levels of 2.0 g/dL of deoxyhemoglobin may reliably produce cyanosis. Since, however, the presence of cyanosis is dependent upon there being an absolute quantity of deoxyhemoglobin, the bluish color is more readily apparent in those with high hemoglobin counts than it is with those with anemia. Also, the bluer the color, the more difficult it is to detect on deeply pigmented skin. When signs of cyanosis first appear, such as on the lips or fingers, intervention should be made within 3–5 minutes because a severe hypoxia or severe circulatory failure may have induced the cyanosis.

Polycythemia Human disease

Polycythemia is a disease state in which the hematocrit is elevated.

Megakaryocyte

A megakaryocyte is a large bone marrow cell with a lobated nucleus responsible for the production of blood thrombocytes (platelets), which are necessary for normal blood clotting. Megakaryocytes usually account for 1 out of 10,000 bone marrow cells in normal people, but can increase in number nearly 10-fold during the course of certain diseases. Owing to variations in combining forms and spelling, synonyms include megalokaryocyte and megacaryocyte.

Pulmonary hypertension hypertension characterized by an increase of blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries

Pulmonary hypertension is a condition of increased blood pressure within the arteries of the lungs. Symptoms include shortness of breath, syncope, tiredness, chest pain, swelling of the legs, and a fast heartbeat. The condition may make it difficult to exercise. Onset is typically gradual.

Cyanotic heart defect is a group-type of congenital heart defect (CHD) that occurs due to deoxygenated blood bypassing the lungs and entering the systemic circulation or a mixture of oxygenated and unoxygenated blood entering the systemic circulation. It is caused by structural defects of the heart, or any condition which increases pulmonary vascular resistance. The result being the development of collateral circulation.

Eisenmengers syndrome fetal heart defect

Eisenmenger's syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt. Because of the advent of fetal screening with echocardiography early in life, the incidence of heart defects progressing to Eisenmenger's has decreased.

Noonan syndrome syndrome that is characterized by mildly unusual facial features, short stature, heart defects, bleeding problems, skeletal malformations, and many other signs and symptoms

Noonan syndrome (NS) is a genetic disorder that may present with mildly unusual facial features, short height, congenital heart disease, bleeding problems, and skeletal malformations. Facial features include widely spaced eyes, light colored eyes, low set ears, a short neck, and a small lower jaw. Heart problems may include pulmonary valve stenosis. The breast bone may be either protruding or be sunk while the spine may be abnormally curved. Intelligence is often normal. Complications of NS may include leukemia.

CREST syndrome syndrome characterized by calcinosis, Raynauds phenomeno, esophageal dysmotility, sclerodactyly and telangiectasia

CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc), is a multisystem connective tissue disorder. The acronym "CREST" refers to the five main features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

Respiratory disease disease of the respiratory system

Respiratory disease is a medical term that encompasses pathological conditions affecting the organs and tissues that make gas exchange possible in higher organisms, and includes conditions of the upper respiratory tract, trachea, bronchi, bronchioles, alveoli, pleura and pleural cavity, and the nerves and muscles of breathing. Respiratory diseases range from mild and self-limiting, such as the common cold, to life-threatening entities like bacterial pneumonia, pulmonary embolism, acute asthma and lung cancer.

Pachydermoperiostosis (PDP) or primary hypertrophic osteoarthropathy (PHO) is a rare genetic disorder that affects both bones and skin. Other names are idiopathic hypertrophic osteoarthropathy or Touraine-Solente-Golé syndrome. It is mainly characterized by pachydermia, periostosis and finger clubbing.

Hypertrophic osteopathy is a bone disease secondary to cancer in the lungs.

GATA1 protein-coding gene in the species Homo sapiens

GATA-binding factor 1 or GATA-1 is the founding member of the GATA family of transcription factors. This protein is widely expressed throughout vertebrate species. In humans and mice, it is encoded by the GATA1 and Gata1 genes, respectively. These genes are located on the X chromosome in both species.

In medicine, hepatopulmonary syndrome is a syndrome of shortness of breath and hypoxemia caused by vasodilation in the lungs of patients with liver disease. Dyspnea and hypoxemia are worse in the upright position.

Hypertrophic osteoarthropathy medical condition combining clubbing and periostitis of the small hand joints

Hypertrophic osteoarthropathy is a medical condition combining clubbing and periostitis of the small hand joints, especially the distal interphalangeal joints and the metacarpophalangeal joints. Distal expansion of the long bones as well as painful, swollen joints and synovial villous proliferation are often seen. The condition may occur alone (primary), or it may be secondary to diseases like lung cancer. It is especially associated with non-small cell lung carcinoma. These patients often get clubbing and increased bone deposition on long bones. Their presenting symptoms are sometimes only clubbing and painful ankles.

Pulmonary capillary hemangiomatosis human disease

Pulmonary capillary hemangiomatosis (PCH) is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. It is a rare cause of pulmonary hypertension, and occurs predominantly in young adults. Together with pulmonary veno-occlusive disease, PCH comprises WHO Group I' causes for pulmonary hypertension. Indeed, there is some evidence to suggest that PCH and pulmonary veno-occlusive disease are different forms of a similar disease process.

References

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  2. Rutherford, JD (14 May 2013). "Digital clubbing". Circulation. 127 (19): 1997–9. doi:10.1161/circulationaha.112.000163. PMID   23671180.
  3. Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN   0-07-138076-0. :656
  4. Schwatz, RA. "Clubbing of the Nails". Medscape. Medscape. Retrieved 14 August 2014.
  5. 1 2 Vandemergel X, Renneboog B (July 2008). "Prevalence, aetiologies and significance of clubbing in a department of general internal medicine". Eur. J. Intern. Med. 19 (5): 325–9. doi:10.1016/j.ejim.2007.05.015. PMID   18549933.
  6. Sridhar KS, Lobo CF, Altman RD (1998). "Digital clubbing and lung cancer". Chest. 114 (6): 1535–37. doi:10.1378/chest.114.6.1535. PMID   9872183. Archived from the original (PDF) on 2003-11-01.
  7. Epstein O, Dick R, Sherlock S (1981). "Prospective study of periostitis and finger clubbing in primary biliary cirrhosis and other forms of chronic liver disease". Gut. 22 (3): 203–6. doi:10.1136/gut.22.3.203. PMC   1419499 . PMID   7227854.
  8. Naeije R (March 2003). "Hepatopulmonary syndrome and portopulmonary hypertension". Swiss Med Wkly. 133 (11–12): 163–9. PMID   12715285.
  9. "acropachy" . GPnotebook.
  10. 1 2 3 Myers KA, Farquhar DR (2001). "The rational clinical examination: does this patient have clubbing?". JAMA. 286 (3): 341–7. doi:10.1001/jama.286.3.341. PMID   11466101.
  11. Shah K, Ferrara TM, Jan A, Umair M, Irfanullah, Khan S, Ahmad W, Spritz RA (August 2017). "Homozygous SLCO2A1 translation initiation codon mutation in a Pakistani family with recessive isolated congenital nail clubbing". Br. J. Dermatol. 177 (2): 546–548. doi:10.1111/bjd.15094. PMID   27681482.
  12. 1 2 Uppal S, Diggle CP, Carr IM, et al. (June 2008). "Mutations in 15-hydroxyprostaglandin dehydrogenase cause primary hypertrophic osteoarthropathy". Nat. Genet. 40 (6): 789–93. doi:10.1038/ng.153. PMID   18500342.
  13. Dickinson, CJ; Martin, JF (19 December 1987). "Megakaryocytes and platelet clumps as the cause of finger clubbing". Lancet. 2 (8573): 1434–5. PMID   2891996.
  14. Schamroth L (February 1976). "Personal experience". S. Afr. Med. J. 50 (9): 297–300. PMID   1265563.
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