Answer: Achalasia

Achalasia is a primary oesophageal motility disorder characterized by the absence of oesophageal peristalsis and impaired relaxation of the lower oesophageal sphincter (LES) in response to swallowing. The LES is hypertensive in about 50% of patients. These abnormalities cause a functional obstruction at the gastroesophageal junction (GEJ).
Symptoms of achalasia include the following:

Dysphagia (most common)

Regurgitation

Chest pain (behind the sternum)

Heartburn

Weight loss

Physical examination is non-contributory.

Treatment recommendations are as follows:

Initial therapy should be either graded pneumatic dilation (PD) or laparoscopic surgical myotomy with a partial fundoplication in patients fit to undergo surgery

Procedures should be performed in high-volume centres of excellence

Initial therapy choice should be based on patient age, sex, preference, and local institutional expertise

Botulinum toxin therapy is recommended for patients not suited to PD or surgery

Pharmacologic therapy can be used for patients not undergoing PD or myotomy and who have failed botulinum toxin therapy (nitrates and calcium channel blockers most common).

The invasion of the oesophageal neural plexus by the tumour can cause nonrelaxation of the LES, thus mimicking achalasia. This condition is known as malignant pseudo achalasia. Since contrast radiography and endoscopy frequently fail to differentiate these 2 entities, patients with a presumed diagnosis of achalasia but who have a shorter duration of symptoms, greater weight loss, and a more advanced age and who are referred for minimally invasive surgery should undergo additional imaging studies, including endoscopic ultrasound and computed tomography with fine cuts of the gastroesophageal junction, to rule out cancer.

Effort rupture of the oesophagus, or Boerhaave syndrome, is a spontaneous perforation of the oesophagus that results from a sudden increase in intraoesophageally pressure combined with negative intrathoracic pressure (e.g., severe straining or vomiting). The classic clinical presentation of Boerhaave syndrome usually consists of repeated episodes of retching and vomiting, typically in a middle-aged man with recent excessive dietary and alcohol intake.

These repeated episodes of retching and vomiting are followed by a sudden onset of severe chest pain in the lower thorax and the upper abdomen. The pain may radiate to the back or to the left shoulder. Swallowing often aggravates the pain.

Typically, hematemesis is not seen after oesophageal rupture, which helps to distinguish it from the more common Mallory-Weiss tear.

Swallowing may precipitate coughing because of the communication between the oesophagus and the pleural cavity. Shortness of breath is a common complaint and is due to pleuritic pain or pleural effusion.

A pulmonary embolism is a blood clot that occurs in the lungs.

It can damage part of the lung due to restricted blood flow, decrease oxygen levels in the blood, and affect other organs as well. Large or multiple blood clots can be fatal.
The most common symptom of a pulmonary embolism is shortness of breath. This may be gradual or sudden.

Other symptoms of a pulmonary embolism include:
anxiety
clammy or bluish skin
chest pain that may extend into your arm, jaw, neck, and shoulder
fainting
irregular heartbeat
light-headedness
rapid breathing
rapid heartbeat
restlessness
spitting up blood
weak pulse

The left colic flexure is the bend of the transverse colon as it continues to form the descending colon on the left upper quadrant. The spleen is located on the superior aspect of the left colic flexure. It is commonly referred to as the splenic flexure because of its relation o the spleen superiorly.

Acute aortic dissection is a serious condition in which the inner layer of the aorta tears and the blood flows in between the inner and middle layers of the aorta causing their separation (dissection). Aortic dissection can lead to rupture or decreased blood flow to organs. Clinical manifestations most often include the sudden onset of severe, tearing or ripping chest pain that can radiate to the shoulder, back or neck; syncope; altered mental status; dyspnoea; pale skin; stroke symptoms etc. The diagnosis of acute aortic dissection is based on clinical findings, imaging studies, electrocardiography and laboratory analysis.

Answer: Perthes disease

Perthes’ disease is a condition affecting the hip joint in children. It is rare (1 in 9,000 children are affected) and we do not clearly understand why it occurs.

Part or all of the femoral head (top of the thigh bone: the ball part of the ball-and-socket hip joint) loses its blood supply and may become misshapen. This may lead to arthritis of the hip in later years.

The earliest sign of Legg-Calvé-Perthes disease (LCPD) is an intermittent limp (abductor lurch), especially after exertion, with mild or intermittent pain in the anterior part of the thigh. LCPD is the most common cause of a limp in the 4- to 10-year-old age group, and the classic presentation has been described as a painless limp.
The patient may present with limited range of motion of the affected extremity. The most common symptom is persistent pain.

Hip pain may develop and is a result of necrosis of the involved bone. This pain may be referred to the medial aspect of the ipsilateral knee or to the lateral thigh. The quadriceps muscles and adjacent thigh soft tissues may atrophy, and the hip may develop adduction flexion contracture. The patient may have an antalgic gait with limited hip motion.
Early radiographic changes may reveal only a nonspecific effusion of the joint associated with slight widening of the joint space, metaphyseal demineralization (decreased bone density around the joint), and periarticular swelling (bulging capsule). This is the acute phase, and it may last 1-2 weeks. Decreasing bone density in and around the joint is noted after a few weeks. Eventually, the disease may progress to collapse of the femoral head, increase in the width of the neck, and demineralization of the femoral head. The final shape of this area depends on the extent of necrosis and the degree of collapse.

Chest pain, hypoxia, and clear chest on auscultation in pregnancy should lead to a high suspicion of pulmonary embolism (PE).

PE is one of the leading causes of mortality in pregnancy. Evaluation with low-dose perfusion scintigraphy may be preferable to computed tomographic pulmonary angiography (CTPA).
PE is treated with LMWH throughout pregnancy and for 4–6 weeks after childbirth. Warfarin is contraindicated in pregnancy (though may be continued in women with mechanical heart valves due to a significant risk of thromboembolism).

Diphtheria is an infection caused by the bacterium Corynebacterium diphtheriae. Signs and symptoms may vary from mild to severe. They usually start two to five days after exposure. Symptoms often come on fairly gradually, beginning with a sore throat and fever. In severe cases, a grey or white patch develops in the throat. This can block the airway and create a barking cough as in croup. The neck may swell in part due to enlarged lymph nodes.

The disease may remain manageable, but in more severe cases, lymph nodes in the neck may swell, and breathing and swallowing are more difficult. People in this stage should seek immediate medical attention, as obstruction in the throat may require intubation or a tracheotomy. Abnormal cardiac rhythms can occur early in the course of the illness or weeks later, and can lead to heart failure. Diphtheria can also cause paralysis in the eye, neck, throat, or respiratory muscles. Patients with severe cases are put in a hospital intensive care unit and given a diphtheria antitoxin (consisting of antibodies isolated from the serum of horses that have been challenged with diphtheria toxin). Since antitoxin does not neutralize toxin that is already bound to tissues, delaying its administration increases risk of death. Therefore, the decision to administer diphtheria antitoxin is based on clinical diagnosis, and should not await laboratory confirmation.
Antibiotics are used in patients or carriers to eradicate C. diphtheriae and prevent its transmission to others. The Centres for Disease Control and Prevention recommends either:
Metronidazole
Erythromycin is given (orally or by injection) for 14 days (40 mg/kg per day with a maximum of 2 g/d), or
Procaine penicillin G is given intramuscularly for 14 days (300,000 U/d for patients weighing <10 kg and 600,000 U/d for those weighing >10 kg); patients with allergies to penicillin G or erythromycin can use Rifampicin or clindamycin.

Pancreatitis is inflammation of the pancreas with variable involvement of regional tissues or remote organ systems. Acute pancreatitis (AP) is characterized by severe pain in the upper abdomen and elevation of pancreatic enzymes in the blood. In the majority of patients,
Biliary pancreatitis should always be treated eventually with a cholecystectomy after the process has subsided.
Feeding should be introduced enterally as the patient’s anorexia and pain resolves.
The use of nasogastric aspiration offers no clear advantage in patients with mild AP, but is beneficial in patients with profound pain, severe disease, paralytic ileus, and intractable vomiting.
AP is a mild, self-limiting disease that resolves spontaneously without complications. Patients can be initiated on a low-fat diet initially and need not invariably start their dietary advancement using a clear liquid diet. Systematic reviews and meta-analyses have shown that administration of enteral nutrition may reduce mortality and infectious complications compared with parenteral nutrition. Although the ideal timing to initiate enteral feeding remains undetermined, administration within 48 hours appears to be safe and tolerated.

Created by the small intestinal cells, chylomicrons are large lipoprotein molecules which transport lipids to the liver, adipose, cardiac and skeletal tissue. Chylomicrons are mainly composed of triglycerides (,85%) along with some cholesterol and cholesteryl esters. Apo B-48 is the main apolipoprotein content.

The lymphatics from the anus, skin of the perineum and the scrotum end in the superficial inguinal nodes. In case of a malignant growth of the anus, the superficial inguinal lymph nodes would most likely be enlarge.

Acute mastitis results due to bacterial infection of the breast and results in signs of inflammation. It commonly occurs 2-3 weeks postpartum and common causative microorganisms are Staphylococcus aureus, Streptococcus species, and Escherichia coli. Complications like an abscess can be avoided by prompt treatment, which includes antibiotics and rest along with continued lactation.

A mitochondria is a membrane bound organelle found in eukaryotic cells. They are called the powerhouse of the cell and are the place where ATP is formed from energy generated through metabolism. They are capable of replication as well as repair and regeneration.

In patients with suspected gallstone complications, blood tests should include a complete blood cell (CBC) count with differential, liver function panel, and amylase and lipase. Up to 24% of women and 12% of men may have gallstones. Of these up to 30% may develop local infection and cholecystitis.

Acute cholecystitis is associated with polymorphonuclear leucocytosis. However, up to one third of the patients with cholecystitis may not manifest leucocytosis. In severe cases, mild elevations of liver enzymes may be caused by inflammatory injury of the adjacent liver.

Patients with cholangitis and pancreatitis have abnormal laboratory test values. Importantly, a single abnormal laboratory value does not confirm the diagnosis of choledocholithiasis, cholangitis, or pancreatitis; rather, a coherent set of laboratory studies leads to the correct diagnosis.

Choledocholithiasis with acute common bile duct (CBD) obstruction initially produces an acute increase in the level of liver transaminases (alanine and aspartate aminotransferases), followed within hours by a rising serum bilirubin level. The higher the bilirubin level, the greater the predictive value for CBD obstruction. CBD stones are present in approximately 60% of patients with serum bilirubin levels greater than 3 mg/dL.

If obstruction persists, a progressive decline in the level of transaminases with rising alkaline phosphatase and bilirubin levels may be noted over several days. Prothrombin time may be elevated in patients with prolonged CBD obstruction, secondary to depletion of vitamin K (the absorption of which is bile-dependent). Concurrent obstruction of the pancreatic duct by a stone in the ampulla of Vater may be accompanied by increases in serum lipase and amylase levels.

Repeated testing over hours to days may be useful in evaluating patients with gallstone complications. Improvement of the levels of bilirubin and liver enzymes may indicate spontaneous passage of an obstructing stone. Conversely, rising levels of bilirubin and transaminases with progression of leucocytosis in the face of antibiotic therapy may indicate ascending cholangitis with the need for urgent intervention. Blood culture results are positive in 30%-60% of patients with cholangitis.

The AJCC uses the TNM, Gleason score and PSA levels to determine the overall stage of prostatic cancer. This staging is as follows:
Stage I: T1, N0, M0, Gleason score 6 or less, PSA less than 10; or T2a, N0, M0, Gleason score 6 or less, PSA less than 10
Stage IIa: T1, N0, M0, Gleason score of 7, PSA less than 20; or T1, N0, M0, Gleason score of 6 or less, PSA at least 10 but less than 20; or T2a or T2b, N0, M0, Gleason score of 7 or less, PSA less than 20
Stage IIb: T2c, N0, M0, any Gleason score, any PSA; or T1 or T2, N0, M0, any Gleason score PSA of 20 or more; or T1 or T2, N0, M0, Gleason score of 8 or higher, any PSA
Stage III: T3, N0, M0, any Gleason score, any PSA Stage IV: T4, N0, M0,any Gleason score, any PSA; or any T, N1, M0,any Gleason score, any PSA; or Any T, any N, M1, any Gleason score, any PSA.
The patient in this case has a T2 N0 M0 G2 tumour, meaning it belongs in stage II

Cations: sodium, magnesium, calcium and potassium
Anions: chloride, phosphate, bicarbonate, lactate, sulphate and albumin

Osteomyelitis is most commonly caused by S. aureus in all age groups.

Pepsin is a protease that is released from the gastric chief cells and acts to degrade proteins into peptides. Released as pepsinogen, it is activated by hydrochloric acid and into pepsin itself. Gastrin and the vagus nerve trigger the release of pepsinogen and HCl when a meal is ingested. Pepsin functions optimally in an acidic environment, especially at a pH of 2.

Answer: Excision biopsy of a lymph node

Hodgkin lymphoma is an uncommon cancer that develops in the lymphatic system, which is a network of vessels and glands spread throughout your body. In Hodgkin lymphoma, B-lymphocytes (a particular type of lymphocyte) start to multiply in an abnormal way and begin to collect in certain parts of the lymphatic system, such as the lymph nodes (glands). The affected lymphocytes lose their infection-fighting properties, making you more vulnerable to infection. The most common symptom of Hodgkin lymphoma is a painless swelling in a lymph node, usually in the neck, armpit or groin.
A histologic diagnosis of Hodgkin lymphoma is always required. An excisional lymph node biopsy is recommended because the lymph node architecture is important for histologic classification.

Features of Hodgkin lymphoma include the following:

Asymptomatic lymphadenopathy may be present (above the diaphragm in 80% of patients)

Constitutional symptoms (unexplained weight loss [>10% of total body weight] within the past 6 months, unexplained fever >38º C, or drenching night sweats) are present in 40% of patients; collectively, these are known as B symptoms

Intermittent fever is observed in approximately 35% of cases; infrequently, the classic Pel-Ebstein fever is observed (high fever for 1-2 week, followed by an afebrile period of 1-2 week)

Chest pain, cough, shortness of breath, or a combination of those may be present due to a large mediastinal mass or lung involvement; rarely, haemoptysis occurs

Pruritus may be present

Pain at sites of nodal disease, precipitated by drinking alcohol, occurs in fewer than 10% of patients but is specific for Hodgkin lymphoma

Back or bone pain may rarely occur

A family history is also helpful; in particular, nodular sclerosis Hodgkin lymphoma (NSHL) has a strong genetic component and has often previously been diagnosed in the family.

Spherocytes are small rounded RBCs. It is due to an inherited defect of the RBC cytoskeleton membrane tethering proteins. Membrane blebs form that are lost over time and cells become round instead of biconcave. As it is a normochromic anaemia, the MCV is normal. it is diagnosed by osmotic fragility test which reveals increased fragility in a hypotonic solution.

During the generation of an action potential, the membrane gets depolarized which cause the voltage gated sodium channels to open and sodium diffuses inside the neuron, resulting in the membrane potential moving towards a positive value. This positive potential will then open the voltage gated potassium channels and cause more K+ to move out decreasing the membrane potential and restoring the membrane potential to its resting value.

Torsade de pointes is an uncommon and distinctive form of polymorphic ventricular tachycardia (VT) characterized by a gradual change in the amplitude and twisting of the QRS complexes around the isoelectric line. Torsade de pointes, often referred to as torsade, is associated with a prolonged QT interval, which may be congenital or acquired. Torsade usually terminates spontaneously but frequently recurs and may degenerate into ventricular fibrillation. This woman is likely to have hypokalaemia and hypomagnesaemia as a result of vomiting. In addition to this, the erythromycin will predispose her to torsades de pointes. The patient should be given Magnesium 2g over 10 minutes. Knowledge of the management of this peri arrest diagnosis is hence important in surgical practice.

The evaluation of a patient in whom hyperaldosteronism is first to determine that hyperaldosteronism is present (serum aldosterone) and, if it is present, to differentiate primary from secondary causes of hyperaldosteronism. The aldosterone-to-renin ratio (ARR) is the most sensitive means of differentiating primary from secondary causes of hyperaldosteronism as it is abnormally increased in primary hyperaldosteronism, and decreased or normal but with high renin levels in secondary hyperaldosteronism.

Lung metastases occur when a cancer started in another part of the body (primary site) spreads to the lungs. The lungs are among the most common site where cancer can spread due to its rich systemic venous drainage, almost every type of cancer can spread to the lung. The most common types of cancer that spread to the lung are breast, colorectal, kidney, testicular, bladder, prostate, head and neck cancers.

Atorvastatin is a member of the drug class of statins, used for lowering cholesterol. The mode of action of statins is inhibition of 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase. This enzyme is needed by the body to make cholesterol. The primary uses of atorvastatin is for the treatment of dyslipidaemia and the prevention of cardiovascular disease.

The most appropriate management option to consider, after GTN or other topical nitrates have failed, is botulinum toxin injection. In males, a lateral internal sphincterotomy would be an acceptable alternative. In a female who has yet to conceive, this may predispose to an increased risk of sphincter dysfunction. Moreover, division of the external sphincter would result in faecal incontinence and is not a justified treatment for anal fissure.

Anal fissures are a common cause of painful, bright red rectal bleeding. Most fissures are idiopathic and present as a painful mucocutaneous defect in the posterior midline (90% cases). Fissures are more likely to be anteriorly located in females, particularly if they are multiparous. Diseases associated with fissure-in-ano include:
1. Crohn’s disease
2. Tuberculosis
3. Internal rectal prolapse

Diagnostic options are as follows:
In most cases, the defect can be visualised as a posterior midline epithelial defect. Where symptoms are highly suggestive of the condition and examination findings are unclear, an examination under anaesthesia may be helpful. Atypical disease presentation should be investigated by colonoscopy and EUA, with biopsies of the area.

For management of anal fissure:
1. Stool softeners are important as hard stools may tear the epithelium and result in recurrent symptoms. The most effective first-line agents are topically applied GTN (0.2%) or Diltiazem (2%) paste.
2. Resistant cases may benefit from injection of botulinum toxin or lateral internal sphincterotomy. Advancement flaps may be used to treat resistant cases.

Tuberculosis is least likely to be associated with this condition. Anal pruritus affects up to 5% of the population. It is often persistent and the constant urge to scratch the area can cause great distress. Although usually caused by a combination of irritants, particularly faecal soiling and dietary factors, it can be a symptom of serious dermatosis, skin or generalised malignancy or systemic illness.

Driving pressure is the difference between inflow and outflow pressure. For the pulmonary circulation, this is the difference between pulmonary arterial (pa) and left atrial pressure (pLA). Normally, mean driving pressure is about 10 mmHg, computed by subtracting pLA (5 mmHg) from pA (15 mmHg). This is in contrast to a mean driving pressure of nearly 100 mmHg in the systemic circulation.

The abdomen is divided by theoretical anatomic lines into four quadrants. The median plane follows the linea alba and extends from the xiphoid process to the pubic symphysis and splits the abdomen in half. The transumbilical plane is a horizontal line that runs at the level of the umbilicus. This forms the upper right and left quadrants and the lower right and left quadrants. Structures in the right lower quadrant include: caecum, appendix, part of the small intestine, ascending colon, the right half of the female reproductive system, right ureter. Pain in this region is most commonly associated with appendicitis.

Steps involved in leukocyte arrival and function include:
1. margination: cells migrate from the centre to the periphery of the vessel.
2. rolling: selectins are upregulated on the vessel walls.
3. adhesion: upregulation of the adhesion molecules ICAM and VCAM on the endothelium interact with integrins on the leukocytes. Interaction of these results in adhesion.
4. diapedesis and chemotaxis: diapedesis is the transmigration of the leukocyte across the endothelium of the capillary and towards a chemotactic product.
5. phagocytosis: engulfing the offending substance/cell.

Medulloblastoma is the most common malignant brain tumour in children, accounting for 10-20% of primary CNS neoplasms. Most of the tumours originate in the cerebellar vermis.

Sodium nitroprusside is a potent peripheral vasodilator that affects both arterioles and venules. It is often administered intravenously to patients who are experiencing a hypertensive emergency. It reduces both total peripheral resistance as well as venous return, so decreasing both preload and afterload. For this reason it can be used in severe cardiogenic heart failure where this combination of effects can act to increase cardiac output. It is administered by intravenous infusion. Onset is typically immediate and effects last for up to ten minutes. The duration of treatment should not exceed 72 hours.