Hoarseness is a symptom that can be caused by hypothyroidism.

When a patient presents with hoarseness, it can be difficult to determine the underlying cause. However, if the hoarseness is accompanied by other symptoms commonly associated with hypothyroidism, it can help narrow down the diagnosis.

The reason for the voice change in hypothyroidism is due to the thickening of the vocal cords caused by the accumulation of mucopolysaccharide. This substance, also known as glycosaminoglycans, is found throughout the body in mucus and joint fluid. When it builds up in the vocal cords, it can lower the pitch of the voice. The thyroid hormone plays a role in preventing this buildup.

Hoarseness can be caused by various factors such as overusing the voice, smoking, viral infections, hypothyroidism, gastro-oesophageal reflux, laryngeal cancer, and lung cancer. It is important to investigate the underlying cause of hoarseness, and a chest x-ray may be necessary to rule out any apical lung lesions.

If laryngeal cancer is suspected, it is recommended to refer the patient to an ENT specialist through a suspected cancer pathway. This referral should be considered for individuals who are 45 years old and above and have persistent unexplained hoarseness or an unexplained lump in the neck. Early detection and treatment of laryngeal cancer can significantly improve the patient’s prognosis.

Chronic bronchitis is characterized by a persistent cough with sputum production for a minimum of 3 months in two consecutive years, after excluding other causes of chronic cough. Emphysema, on the other hand, is defined by the enlargement of air spaces beyond the terminal bronchioles. None of the remaining options are considered as definitions of COPD.

COPD, or chronic obstructive pulmonary disease, can be caused by a variety of factors. The most common cause is smoking, which can lead to inflammation and damage in the lungs over time. Another potential cause is alpha-1 antitrypsin deficiency, a genetic condition that can result in lung damage. Additionally, exposure to certain substances such as cadmium (used in smelting), coal, cotton, cement, and grain can also contribute to the development of COPD. It is important to identify and address these underlying causes in order to effectively manage and treat COPD.

Rinne’s and Weber’s Test for Differentiating Conductive and Sensorineural Deafness

Rinne’s and Weber’s tests are used to differentiate between conductive and sensorineural deafness. Rinne’s test involves placing a tuning fork over the mastoid process until the sound is no longer heard, then repositioning it just over the external acoustic meatus. A positive test indicates that air conduction (AC) is better than bone conduction (BC), while a negative test indicates that BC is better than AC, suggesting conductive deafness.

Weber’s test involves placing a tuning fork in the middle of the forehead equidistant from the patient’s ears and asking the patient which side is loudest. In unilateral sensorineural deafness, sound is localized to the unaffected side, while in unilateral conductive deafness, sound is localized to the affected side.

The table below summarizes the interpretation of Rinne and Weber tests. A normal result indicates that AC is greater than BC bilaterally and the sound is midline. Conductive hearing loss is indicated by BC being greater than AC in the affected ear and AC being greater than BC in the unaffected ear, with the sound lateralizing to the affected ear. Sensorineural hearing loss is indicated by AC being greater than BC bilaterally, with the sound lateralizing to the unaffected ear.

Overall, Rinne’s and Weber’s tests are useful tools for differentiating between conductive and sensorineural deafness, allowing for appropriate management and treatment.

Surgical resection is the preferred treatment for pleomorphic adenoma, a benign tumor of the parotid gland that may undergo malignant transformation. Chemotherapy and radiotherapy are not effective in managing this condition. Additionally, salivary stone removal is not relevant to the treatment of pleomorphic adenoma.

Understanding Pleomorphic Adenoma

Pleomorphic adenoma, also known as a benign mixed tumour, is a non-cancerous growth that commonly affects the parotid gland. This type of tumour usually develops in individuals aged 40 to 60 years old. The condition is characterized by the proliferation of epithelial and myoepithelial cells of the ducts, as well as an increase in stromal components. The tumour is slow-growing, lobular, and not well encapsulated.

The clinical features of pleomorphic adenoma include a gradual onset of painless unilateral swelling of the parotid gland. The swelling is typically movable on examination rather than fixed. The management of pleomorphic adenoma involves surgical excision. The prognosis is generally good, with a recurrence rate of 1-5% with appropriate excision (parotidectomy). However, recurrence may occur due to capsular disruption during surgery. If left untreated, pleomorphic adenoma may undergo malignant transformation, occurring in 2-10% of adenomas observed for long periods. Carcinoma ex-pleomorphic adenoma is the most common type of malignant transformation, occurring most frequently as adenocarcinoma.

Identify the life-threatening features of an asthma attack.

Assessing the severity of asthma attacks in children is crucial for effective management. The 2016 BTS/SIGN guidelines provide criteria for assessing the severity of asthma in general practice. These criteria include measuring SpO2 levels, PEF (peak expiratory flow) rates, heart rate, respiratory rate, use of accessory neck muscles, and other symptoms such as breathlessness, agitation, altered consciousness, and cyanosis.

A severe asthma attack is characterized by a SpO2 level below 92%, PEF rates between 33-50% of the best or predicted, being too breathless to talk or feed, and a high heart and respiratory rate. On the other hand, a life-threatening asthma attack is indicated by a SpO2 level below 92%, PEF rates below 33% of the best or predicted, a silent chest, poor respiratory effort, use of accessory neck muscles, agitation, altered consciousness, and cyanosis.

It is important for healthcare professionals to be familiar with these criteria to ensure prompt and appropriate management of asthma attacks in children. Early recognition of the severity of an asthma attack can help prevent complications and reduce the risk of hospitalization or death.

Paranasal Air Sinuses and Carotid Sinus

The paranasal air sinuses are air-filled spaces found in the bones of the skull. They are named after the bone in which they are located and all communicate with the nasal cavity. The four paired paranasal air sinuses are the frontal sinuses, maxillary sinuses, ethmoid air cells, and sphenoid sinuses. The frontal sinuses are located above each eye on the forehead, while the maxillary sinuses are the largest and found in the maxillary bone below the orbit. The ethmoidal air cells are a collection of smaller air cells located lateral to the anterior superior nasal cavity, while the sphenoid sinuses are found in the posterior portion of the roof of the nasal cavity.

On the other hand, the carotid sinus is not a paranasal air sinus. It is a dilatation of the internal carotid artery, located just beyond the bifurcation of the common carotid artery. It contains baroreceptors that enable it to detect changes in arterial pressure.

Overall, understanding the location and function of these sinuses and the carotid sinus is important in various medical procedures and conditions.

The presence of adult-onset asthma, eosinophilia, and a positive pANCA test strongly suggests a diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) in this patient.

Although GPA can cause epistaxis, the absence of other characteristic symptoms such as saddle-shaped nose deformity, haemoptysis, renal failure, and positive cANCA make EGPA a more likely diagnosis.

Polyarteritis Nodosa, Temporal Arteritis, and Toxic Epidermal Necrolysis have distinct clinical presentations that do not match the symptoms exhibited by this patient.

Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome)

Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg-Strauss syndrome, is a type of small-medium vessel vasculitis that is associated with ANCA. It is characterized by asthma, blood eosinophilia (more than 10%), paranasal sinusitis, mononeuritis multiplex, and pANCA positivity in 60% of cases.

Compared to granulomatosis with polyangiitis, EGPA is more likely to have blood eosinophilia and asthma as prominent features. Additionally, leukotriene receptor antagonists may trigger the onset of the disease.

Overall, EGPA is a rare but serious condition that requires prompt diagnosis and treatment to prevent complications.

The patient’s history of severe gastro-oesophageal reflux disease (GORD) suggests that she may have aspiration pneumonia, particularly as she had not received appropriate treatment for it. Aspiration of gastric contents is likely to occur in the right lung due to the steep angle of the right bronchus. Klebsiella pneumoniae is a common cause of aspiration pneumonia and is known to produce ‘red-currant jelly’ sputum.

Mycoplasma pneumoniae is a cause of atypical pneumonia, which typically presents with a non-productive cough and clear lung sounds on auscultation. It is more common in younger individuals.

Burkholderia pseudomallei is the causative organism for melioidosis, a condition that is transmitted through exposure to contaminated water or soil, and is more commonly found in Southeast Asia. However, given the patient’s sedentary lifestyle and lack of travel history, it is unlikely to be the cause of her symptoms.

Streptococcus pneumoniae is the most common cause of pneumonia, but it typically produces yellowish-green sputum rather than the red-currant jelly sputum seen in Klebsiella pneumoniae infections. It also presents with fever, productive cough, and crackles on auscultation.

Understanding Klebsiella Pneumoniae

Klebsiella pneumoniae is a type of bacteria that is commonly found in the gut flora of humans. However, it can also cause various infections such as pneumonia and urinary tract infections. It is more prevalent in individuals who have alcoholism or diabetes. Aspiration is a common cause of pneumonia caused by Klebsiella pneumoniae. One of the distinct features of this type of pneumonia is the production of red-currant jelly sputum. It usually affects the upper lobes of the lungs.

The prognosis for Klebsiella pneumoniae infections is not good. It often leads to the formation of lung abscesses and empyema, which can be fatal. The mortality rate for this type of infection is between 30-50%.

The correct answer is low 2,3-DPG. The professor’s description refers to a left shift in the oxygen dissociation curve, which indicates that haemoglobin has a high affinity for oxygen and is less likely to release it to the tissues. Factors that cause a left shift include low temperature, high pH, low PCO2, and low 2,3-DPG. 2,3-DPG is a substance that helps release oxygen from haemoglobin, so low levels of it result in less oxygen being released, causing a left shift in the oxygen dissociation curve.

The answer high temperature is incorrect because it causes a right shift in the oxygen dissociation curve, promoting oxygen delivery to the tissues. Hypercapnoea also causes a right shift in the curve, promoting oxygen delivery. Hyperglycaemia has no effect on haemoglobin’s ability to release oxygen, so it is also incorrect.

Understanding the Oxygen Dissociation Curve

The oxygen dissociation curve is a graphical representation of the relationship between the percentage of saturated haemoglobin and the partial pressure of oxygen in the blood. It is not influenced by the concentration of haemoglobin. The curve can shift to the left or right, indicating changes in oxygen delivery to tissues. When the curve shifts to the left, there is increased saturation of haemoglobin with oxygen, resulting in decreased oxygen delivery to tissues. Conversely, when the curve shifts to the right, there is reduced saturation of haemoglobin with oxygen, leading to enhanced oxygen delivery to tissues.

The L rule is a helpful mnemonic to remember the factors that cause a shift to the left, resulting in lower oxygen delivery. These factors include low levels of hydrogen ions (alkali), low partial pressure of carbon dioxide, low levels of 2,3-diphosphoglycerate, and low temperature. On the other hand, the mnemonic ‘CADET, face Right!’ can be used to remember the factors that cause a shift to the right, leading to raised oxygen delivery. These factors include carbon dioxide, acid, 2,3-diphosphoglycerate, exercise, and temperature.

Understanding the oxygen dissociation curve is crucial in assessing the oxygen-carrying capacity of the blood and the delivery of oxygen to tissues. By knowing the factors that can shift the curve to the left or right, healthcare professionals can make informed decisions in managing patients with respiratory and cardiovascular diseases.

The patient’s oxygen dissociation curve has shifted to the left, indicating respiratory alkalosis. This is likely due to the patient experiencing a panic attack and hyperventilating, leading to a decrease in carbon dioxide levels and an increase in the affinity of haemoglobin for oxygen. Respiratory acidosis, hypercapnia, and a right shift of the curve are not appropriate explanations for this patient’s condition.

Understanding the Oxygen Dissociation Curve

The oxygen dissociation curve is a graphical representation of the relationship between the percentage of saturated haemoglobin and the partial pressure of oxygen in the blood. It is not influenced by the concentration of haemoglobin. The curve can shift to the left or right, indicating changes in oxygen delivery to tissues. When the curve shifts to the left, there is increased saturation of haemoglobin with oxygen, resulting in decreased oxygen delivery to tissues. Conversely, when the curve shifts to the right, there is reduced saturation of haemoglobin with oxygen, leading to enhanced oxygen delivery to tissues.

The L rule is a helpful mnemonic to remember the factors that cause a shift to the left, resulting in lower oxygen delivery. These factors include low levels of hydrogen ions (alkali), low partial pressure of carbon dioxide, low levels of 2,3-diphosphoglycerate, and low temperature. On the other hand, the mnemonic ‘CADET, face Right!’ can be used to remember the factors that cause a shift to the right, leading to raised oxygen delivery. These factors include carbon dioxide, acid, 2,3-diphosphoglycerate, exercise, and temperature.

Understanding the oxygen dissociation curve is crucial in assessing the oxygen-carrying capacity of the blood and the delivery of oxygen to tissues. By knowing the factors that can shift the curve to the left or right, healthcare professionals can make informed decisions in managing patients with respiratory and cardiovascular diseases.

Due to his profession as a builder, this individual is at risk of being exposed to asbestos. Given the 30-year latent period and the presence of a complex effusion, it is highly probable that the underlying cause is mesothelioma.

Understanding Mesothelioma

Mesothelioma is a type of cancer that affects the mesothelial layer of the pleural cavity, which is commonly linked to asbestos exposure. Although it is rare, other mesothelial layers in the abdomen may also be affected. Symptoms of mesothelioma include dyspnoea, weight loss, chest wall pain, and clubbing. In some cases, patients may present with painless pleural effusion. It is important to note that only 20% of patients have pre-existing asbestosis, but 85-90% have a history of asbestos exposure, with a latent period of 30-40 years.

Diagnosis of mesothelioma is typically made through a chest x-ray, which may show pleural effusion or pleural thickening. A pleural CT is then performed to confirm the diagnosis. If a pleural effusion is present, fluid is sent for MC&S, biochemistry, and cytology. However, cytology is only helpful in 20-30% of cases. Local anaesthetic thoracoscopy is increasingly used to investigate cytology negative exudative effusions as it has a high diagnostic yield of around 95%. If an area of pleural nodularity is seen on CT, an image-guided pleural biopsy may be used.

Management of mesothelioma is mainly symptomatic, with industrial compensation available for those who have been exposed to asbestos. Chemotherapy and surgery may be options for those who are operable. Unfortunately, the prognosis for mesothelioma is poor, with a median survival of only 12 months.

Pleural Effusion and its Investigation

Pleural effusion is a condition where there is an abnormal accumulation of fluid in the pleural space, which is the space between the lungs and the chest wall. This can be caused by various factors such as post-infection, carcinoma, or emboli. To determine the cause of the pleural effusion, a pleural tap is the most appropriate investigation. The sample obtained from the pleural tap is sent for cytology, protein concentration, and culture.

A normal pleural tap would have clear appearance, pH of 7.60-7.64, protein concentration of less than 2%, white blood cells count of less than 1000/mm³, glucose level similar to that of plasma, LDH level of less than 50% of plasma concentration, amylase level of 30-110 U/L, triglycerides level of less than 2 mmol/l, and cholesterol level of 3.5-6.5 mmol/l.

A transudative tap is associated with conditions such as congestive heart failure, liver cirrhosis, severe hypoalbuminemia, and nephrotic syndrome. On the other hand, an exudative tap is associated with malignancy, infection (such as empyema due to bacterial pneumonia), trauma, pulmonary infarction, and pulmonary embolism.

In summary, pleural effusion can be caused by various factors and a pleural tap is the most appropriate investigation to determine the cause. The results of the pleural tap can help differentiate between transudative and exudative effusions, which can provide important information for diagnosis and treatment.

If a patient presents with raised serum ACE levels, sarcoidosis should be considered as a possible diagnosis. The combination of erythema nodosum and bilateral hilar lymphadenopathy on a chest x-ray is pathognomonic of sarcoidosis. Lung cancer is unlikely in a young patient without a significant smoking history, and tuberculosis would require recent foreign travel to a TB endemic country. Multiple myeloma would not cause the same symptoms as sarcoidosis. Exposure to organic material would not be a likely cause of raised serum ACE levels.

Understanding Sarcoidosis: A Multisystem Disorder

Sarcoidosis is a condition that affects multiple systems in the body and is characterized by the presence of non-caseating granulomas. The exact cause of this disorder is unknown, but it is more commonly seen in young adults and individuals of African descent.

The symptoms of sarcoidosis can vary depending on the severity of the condition. Acute symptoms may include erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, and polyarthralgia. On the other hand, insidious symptoms may include dyspnea, non-productive cough, malaise, and weight loss. Additionally, some individuals may develop skin symptoms such as lupus pernio, while others may experience hypercalcemia due to increased conversion of vitamin D to its active form.

Sarcoidosis is also associated with several syndromes, including Lofgren’s syndrome, Mikulicz syndrome, and Heerfordt’s syndrome. Lofgren’s syndrome is an acute form of the disease that typically presents with bilateral hilar lymphadenopathy, erythema nodosum, fever, and polyarthralgia. Mikulicz syndrome is characterized by enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis, or lymphoma. Finally, Heerfordt’s syndrome, also known as uveoparotid fever, presents with parotid enlargement, fever, and uveitis secondary to sarcoidosis.

In conclusion, sarcoidosis is a complex disorder that can affect multiple systems in the body. While the exact cause is unknown, early diagnosis and treatment can help manage symptoms and improve outcomes.

Cervical ribs are formed when the transverse process of the 7th cervical vertebrae becomes elongated, resulting in a fibrous band that connects to the first thoracic rib.

Cervical ribs are a rare anomaly that affects only 0.2-0.4% of the population. They are often associated with neurological symptoms and are caused by an anomalous fibrous band that originates from the seventh cervical vertebrae and may arc towards the sternum. While most cases are congenital and present around the third decade of life, some cases have been reported to occur following trauma. Bilateral cervical ribs are present in up to 70% of cases. Compression of the subclavian artery can lead to absent radial pulse and a positive Adsons test, which involves lateral flexion of the neck towards the symptomatic side and traction of the symptomatic arm. Treatment is usually only necessary when there is evidence of neurovascular compromise, and the traditional operative method for excision is a transaxillary approach.

When a patient is in an upright position, the functional residual capacity (FRC) can increase due to less pressure from the diaphragm and abdominal organs on the lung bases. This increase in FRC can also be caused by emphysema and asthma. On the other hand, factors such as abdominal swelling, pulmonary edema, reduced muscle tone of the diaphragm, and aging can lead to a decrease in FRC. Additionally, laparoscopic surgery, obesity, and muscle relaxants can also contribute to a reduction in FRC.

Understanding Lung Volumes in Respiratory Physiology

In respiratory physiology, lung volumes can be measured to determine the amount of air that moves in and out of the lungs during breathing. The diagram above shows the different lung volumes that can be measured.

Tidal volume (TV) refers to the amount of air that is inspired or expired with each breath at rest. In males, the TV is 500ml while in females, it is 350ml.

Inspiratory reserve volume (IRV) is the maximum volume of air that can be inspired at the end of a normal tidal inspiration. The inspiratory capacity is the sum of TV and IRV. On the other hand, expiratory reserve volume (ERV) is the maximum volume of air that can be expired at the end of a normal tidal expiration.

Residual volume (RV) is the volume of air that remains in the lungs after maximal expiration. It increases with age and can be calculated by subtracting ERV from FRC. Speaking of FRC, it is the volume in the lungs at the end-expiratory position and is equal to the sum of ERV and RV.

Vital capacity (VC) is the maximum volume of air that can be expired after a maximal inspiration. It decreases with age and can be calculated by adding inspiratory capacity and ERV. Lastly, total lung capacity (TLC) is the sum of vital capacity and residual volume.

Physiological dead space (VD) is calculated by multiplying tidal volume by the difference between arterial carbon dioxide pressure (PaCO2) and end-tidal carbon dioxide pressure (PeCO2) and then dividing the result by PaCO2.

The right pulmonary artery originates from the proximal portion of the sixth pharyngeal arch on the right side, while the distal portion of the same arch gives rise to the left pulmonary artery and the ductus arteriosus.

The Development and Contributions of Pharyngeal Arches

During the fourth week of embryonic growth, a series of mesodermal outpouchings develop from the pharynx, forming the pharyngeal arches. These arches fuse in the ventral midline, while pharyngeal pouches form on the endodermal side between the arches. There are six pharyngeal arches, with the fifth arch not contributing any useful structures and often fusing with the sixth arch.

Each pharyngeal arch has its own set of muscular and skeletal contributions, as well as an associated endocrine gland, artery, and nerve. The first arch contributes muscles of mastication, the maxilla, Meckel’s cartilage, and the incus and malleus bones. The second arch contributes muscles of facial expression, the stapes bone, and the styloid process and hyoid bone. The third arch contributes the stylopharyngeus muscle, the greater horn and lower part of the hyoid bone, and the thymus gland. The fourth arch contributes the cricothyroid muscle, all intrinsic muscles of the soft palate, the thyroid and epiglottic cartilages, and the superior parathyroids. The sixth arch contributes all intrinsic muscles of the larynx (except the cricothyroid muscle), the cricoid, arytenoid, and corniculate cartilages, and is associated with the pulmonary artery and recurrent laryngeal nerve.

Overall, the development and contributions of pharyngeal arches play a crucial role in the formation of various structures in the head and neck region.

Reduced lung compliance is a common consequence of pulmonary edema, which occurs when fluid accumulates in the alveoli and exerts mechanical stress on the air-filled alveoli. This can happen in patients with acute decompensation of congestive cardiac failure, often triggered by an infection. On the other hand, emphysema can increase compliance due to long-term damage that reduces the elastic recoil of the lungs. Additionally, lung surfactant produced by type II pneumocytes can increase lung compliance. Finally, aging can also lead to increased compliance as the loss of lung connective tissue can reduce elastic recoil.

Understanding Lung Compliance in Respiratory Physiology

Lung compliance refers to the extent of change in lung volume in response to a change in airway pressure. An increase in lung compliance can be caused by factors such as aging and emphysema, which is characterized by the loss of alveolar walls and associated elastic tissue. On the other hand, a decrease in lung compliance can be attributed to conditions such as pulmonary edema, pulmonary fibrosis, pneumonectomy, and kyphosis. These conditions can affect the elasticity of the lungs and make it more difficult for them to expand and contract properly. Understanding lung compliance is important in respiratory physiology as it can help diagnose and manage various respiratory conditions. Proper management of lung compliance can improve lung function and overall respiratory health.

Emphysema causes an increase in residual volume, leading to a decrease in vital capacity. This is due to damage to the alveolar walls, which results in the formation of large air sacs called bullae. The lungs lose their compliance, making it difficult to fully exhale and causing air to become trapped in the bullae. As a result, the total volume that can be exhaled is reduced, leading to a decrease in vital capacity.

Understanding Lung Volumes in Respiratory Physiology

In respiratory physiology, lung volumes can be measured to determine the amount of air that moves in and out of the lungs during breathing. The diagram above shows the different lung volumes that can be measured.

Tidal volume (TV) refers to the amount of air that is inspired or expired with each breath at rest. In males, the TV is 500ml while in females, it is 350ml.

Inspiratory reserve volume (IRV) is the maximum volume of air that can be inspired at the end of a normal tidal inspiration. The inspiratory capacity is the sum of TV and IRV. On the other hand, expiratory reserve volume (ERV) is the maximum volume of air that can be expired at the end of a normal tidal expiration.

Residual volume (RV) is the volume of air that remains in the lungs after maximal expiration. It increases with age and can be calculated by subtracting ERV from FRC. Speaking of FRC, it is the volume in the lungs at the end-expiratory position and is equal to the sum of ERV and RV.

Vital capacity (VC) is the maximum volume of air that can be expired after a maximal inspiration. It decreases with age and can be calculated by adding inspiratory capacity and ERV. Lastly, total lung capacity (TLC) is the sum of vital capacity and residual volume.

Physiological dead space (VD) is calculated by multiplying tidal volume by the difference between arterial carbon dioxide pressure (PaCO2) and end-tidal carbon dioxide pressure (PeCO2) and then dividing the result by PaCO2.

During a median sternotomy, the interclavicular ligament is typically cut to allow access. However, it is important to avoid intentionally cutting the pleural reflections, as this can lead to the accumulation of fluid in the pleural cavity and require the insertion of a chest drain. The pectoralis major muscles may also be encountered, but if the incision is made in the midline, they should not need to be formally divided. It is crucial to be mindful of the proximity of the brachiocephalic vein and avoid injuring it, as this can result in significant bleeding.

Sternotomy Procedure

A sternotomy is a surgical procedure that involves making an incision in the sternum to access the heart and great vessels. The most common type of sternotomy is a median sternotomy, which involves making a midline incision from the interclavicular fossa to the xiphoid process. The fat and subcutaneous tissues are then divided to the level of the sternum, and the periosteum may be gently mobilized off the midline. However, it is important to avoid vigorous periosteal stripping. A bone saw is used to divide the bone itself, and bleeding from the bony edges of the cut sternum is stopped using roller ball diathermy or bone wax.

Posteriorly, the reflections of the parietal pleura should be identified and avoided, unless surgery to the lung is planned. The fibrous pericardium is then incised, and the heart is brought into view. It is important to avoid the left brachiocephalic vein, which is an important posterior relation at the superior aspect of the sternotomy incision. More inferiorly, the thymic remnants may be identified. At the inferior aspect of the incision, the abdominal cavity may be entered, although this is seldom troublesome.

Overall, a sternotomy is a complex surgical procedure that requires careful attention to detail and a thorough understanding of the anatomy of the chest and heart. By following the proper techniques and precautions, surgeons can safely access the heart and great vessels to perform a variety of life-saving procedures.

The patient’s medical history suggests that they may be suffering from alpha-1 antitrypsin deficiency.

When there is a shortage of alpha-1 antitrypsin, neutrophil elastase is not inhibited and can break down proteins in the lung interstitium. Although neutrophil elastase is a crucial part of the innate immune system, its unregulated activity can lead to excessive breakdown of extracellular proteins like elastin, collagen, fibronectin, and fibrin. This results in reduced pulmonary elasticity, which can cause emphysema and COPD.

Alpha-1 antitrypsin (A1AT) deficiency is a genetic condition that occurs when the liver does not produce enough of a protein called protease inhibitor (Pi). This protein is responsible for protecting cells from enzymes like neutrophil elastase. A1AT deficiency is inherited in an autosomal recessive or co-dominant manner and is located on chromosome 14. The alleles are classified by their electrophoretic mobility, with M being normal, S being slow, and Z being very slow. The normal genotype is PiMM, while heterozygous individuals have PiMZ. Homozygous PiSS individuals have 50% normal A1AT levels, while homozygous PiZZ individuals have only 10% normal A1AT levels.

A1AT deficiency is most commonly associated with panacinar emphysema, which is a type of chronic obstructive pulmonary disease (COPD). This is especially true for patients with the PiZZ genotype. Emphysema is more likely to occur in non-smokers with A1AT deficiency, but they may still pass on the gene to their children. In addition to lung problems, A1AT deficiency can also cause liver issues such as cirrhosis and hepatocellular carcinoma in adults, and cholestasis in children.

Diagnosis of A1AT deficiency involves measuring A1AT concentrations and performing spirometry to assess lung function. Management of the condition includes avoiding smoking and receiving supportive care such as bronchodilators and physiotherapy. Intravenous alpha1-antitrypsin protein concentrates may also be used. In severe cases, lung volume reduction surgery or lung transplantation may be necessary.