Cystic fibrosis is a life-threatening disorder that causes the build up of thick mucus in the lungs, digestive tract, and other areas of the body. It is a hereditary autosomal-recessive disease caused by mutations of the CFTR gene. Cystic fibrosis eventually results in bronchiectasis which is defined as a permanent dilatation and obstruction of bronchi or bronchioles.

The operation aims to remove most of your large bowel including the blood supply and associated lymph glands and leave the rectum behind. It is most commonly recommended for inflammatory bowel disease like ulcerative colitis and Crohn’s disease.

It is also recommended for other bowel conditions like familial adenomatous polyposis, and when there is more than one bowel cancer.

In inflammatory bowel disease such as ulcerative colitis the small bowel is brought out to the skin of the tummy as an ileostomy and the remaining bowel (rectum) is closed off and left inside. In other conditions where possible, the two ends of the remaining healthy bowel (small bowel to rectum) are re-joined (an anastomosis). Most
people therefore do not require a stoma. However some people benefit from having a stoma made depending on circumstances regarding:
1) Their general state of health (heart disease, lung disease, diabetes, vascular disease, smoking, steroid medications, being undernourished);
2) Factors which cannot be seen until the surgeon can see inside your tummy (more extensive disease than originally thought, extensive pelvic scarring from previous surgery or other treatment, excessive bleeding).
Temporary stomas are made to divert faeces away from the join (de-functioning) to give the best chance to heal if there is concern it may be slow to heal.

Emergency indications for surgical intervention in severe UC include free perforation, haemorrhage or systemic instability. An urgent indication for colectomy is a severe attack that is unresponsive to medical therapy.

In the setting of severe UC, the procedure of choice is subtotal colectomy and ileostomy. The residual rectal disease is controllable in most patients. In general, there are advantages to the subtotal colectomy approach, including a lower morbidity if pelvic dissection is not performed, preservation of the rectum so that reconstructive procedures can be performed later, and allowing the definitive procedure to be deferred to an optimal situation when the patient is off immunosuppressive medications and has improved nutritional status. Usually, the staged reconstruction with IPAA or definitive total proctocolectomy is performed several months later.

Typical pilonidal sinus disease (PSD) occurs in the natal cleft i.e. sacrococcygeal region.
However, some occupation related pilonidal sinuses occurs in webs of fingers e.g. hairdresser, sheep shearer, dog groomer, slaughterman or milker.
Other locations where pilonidal sinuses may occur include penis shaft, axilla, intermammary area, groin, nose, neck, clitoris, suprapubic area, occiput, prepuce, chin, periungual region, breast, face and umbilicus.

Although the pilonidal disease may manifest as an abscess, a pilonidal sinus, a recurrent or chronic pilonidal sinus, or a perianal pilonidal sinus, the most common manifestation of pilonidal disease is a painful, fluctuant mass in the sacrococcygeal region.
Initially, 50% of patients first present with a pilonidal abscess that is cephalad to the hair follicle and sinus infection. Pain and purulent discharge from the sinus tract is present 70-80% of the time and are the two most frequently described symptoms. In the early stages preceding the development of an abscess, only cellulitis or folliculitis is present. The abscess is formed when a folliculitis expands into the subcutaneous tissue or when a pre-existing foreign body granuloma becomes infected.
The diagnosis of a pilonidal sinus can be made by identifying the epithelialized follicle opening, which can be palpated as an area of deep induration beneath the skin in the sacral region. These tracts most commonly run in the cephalad direction. When the tract runs in the caudal direction, perianal sepsis may be present.

The ideal treatment for a pilonidal sinus varies according to the clinical presentation of the disease. First, it is important to divide the pilonidal disease into the following three categories, which represent different stages of the clinical course:
– Acute pilonidal abscess
– Chronic pilonidal disease
– Complex or recurrent pilonidal disease

Acute pilonidal abscess:
A pilonidal abscess is managed by incision, drainage, and curettage of the abscess cavity to remove hair nests and skin debris. This can be accomplished in the surgical office or the emergency department, using local anaesthesia.
If possible, the drainage incision should be made laterally, away from the midline. Wounds heal poorly in the deep, intergluteal natal cleft, for two reasons. The first is the frictional motion of the deep cleft, which creates continuous irritation to the healing wound; the second is the midline nature of the wound, which is a product of constant lateral traction during sitting.

Chronic pilonidal disease is the term applied when patients have undergone at least one pilonidal abscess drainage procedure and continue to have a pilonidal sinus tract. The term also refers to a pilonidal sinus that is associated with a chronic discharge without an acute abscess. Surgical options for management of a noncomplicated chronic pilonidal sinus include the following:
Excision and laying open of the sinus tract
Excision with primary closure
Wide and deep excision to the sacrum
Incision and marsupialization
Bascom procedure
Asymmetrical incisions
Skin flaps have also been described to cover a sacral defect after wide excision. Similarly, this keeps the scar off the midline and flattens the natal cleft.

Total thyroidectomy might sometimes result in inadvertent excision or damage of parathyroid glands, leading to hypoparathyroidism. Monitoring serum calcium levels in the post-operative period to detect hypocalcaemia is essential to diagnose and prevent this condition.

Interferon gamma is a soluble cytokine previously known as the macrophage activating factor. It is the only member belonging to the type II class of the interferons. It is secreted by a number of cells taking part in the immune reaction including: T-helper cells (CD-4), cells with immunological memory (CD45PA), killer cells (CD8), dendrite cells (CD23,35), natural killer cells (CD16) and B lymphocytes (CD22,CD23). It has both a defending as well as a pathological effect. It induces differentiation in the myeloid cell in the bone marrow. If macrophages are infected by parasites it activates the macrophages to destroy them. IFN-γ strengthens the anti-tumour activities of the cytotoxic lymphocytes. Together with CD4 or CD8 toxins, produced by lymphocytes, it suppresses the growth of the tumour cells. along with these functions it increases the non specific response of the natural killer cells, causing changes in the cell membrane surface to prevent adhesion and penetration of a virus. It can either increase or decrease B cell response and it activates osteoclasts which increases bone resorption.

The popliteal fossa is located at the back of the knee. It is bounded laterally by the biceps femoris above and the plantaris and lateral head of the gastrocnemius below and medially by the semitendinosus and semimembranosus above and by the medial head of the gastrocnemius below.

Containment of fistula output and skin protection should be instituted as soon as the diagnosis is made as it will decrease local skin excoriation and inflammation, pain and infection. While low output fistulas may be controlled with a simple absorbent dressing, complex fistulas often require advanced techniques including barrier creams, powders, and sealants to protect the skin from auto-digestion as well as bridging for fistula isolation, topographical enhancements, and complex pouching systems with or without sump drainage
Fistulas arising from the oesophagus, duodenal stump after gastric resection, pancreaticobiliary tract, and jejunum are more likely to close without operative intervention. Additionally, those with long tracts and small enteric wall defects are associated with higher spontaneous closure rates. Fistulas in the colon show favourable rates of spontaneous resolution.

Osteoarthritis is most common in older adults, predominating in women between the ages of 40 and 70; after this age, men and women are affected equally. It affects an entire joint, with disruption and potential loss of joint cartilage, along with other joint changes, including bone hypertrophy (osteophyte formation). The pain is usually gradual and is worse after activity, with occasional joint swelling. X-ray findings include marginal osteophytes, narrowing of the joint space, increased density of the subchondral bone, subchondral cyst formation, bony remodelling and joint effusions.

Postoperative hypertension, arrhythmias, and heart failure commonly occur in the first 2 days after surgery, but the risk of myocardial infarction persists for at least 5 or 6 days after surgery.
ECG should be done to exclude it.

ASA-V: A moribund patient who is not expected to survive without the operation. Examples include (but not limited to): ruptured abdominal/thoracic aneurysm, massive trauma, intracranial bleed with mass effect, ischemic bowel in the face of significant cardiac pathology or multiple organ/system dysfunction

ASA Grading
1 – No organic physiological, biochemical or psychiatric disturbance. The surgical pathology is localised and has not invoked systemic disturbance
2 – Mild or moderate systemic disruption caused either by the surgical disease process or though underlying pre-existing disease
3 – Severe systemic disruption caused either by the surgical pathology or pre-existing disease
4 – Patient has severe systemic disease that is a constant threat to life
5 – A patient who is moribund and will not survive without surgery

Secondary hyperaldosteronism in hypertension is either due to primary renin overproduction by the kidneys or renin overproduction secondary to decreased renal blood flow. The main stimulus for aldosterone release are adrenocorticotrophic hormone (ACTH), angiotensin II and high plasma K+ levels. Low plasma Na+ might also stimulate the adrenal cortex. Fluid overload will reduce aldosterone secretion. Atrial natriuretic peptide is secreted under conditions of expanded extracellular volume and will not lead to aldosterone secretion.

In the case of injuries to the upper roots of the brachial plexus there is complete loss of abduction. The muscle performing this movement is the supraspinatus. This initiates the movement, followed by the deltoid muscle, which allows for complete abduction. Both these muscles are innervated by nerves originating from C5 and C6. The injury to these roots results in a condition named Erb-Duchenne’s palsy.

C5a is a strong chemoattractant as well as an anaphylotoxin and is involved in the recruitment of inflammatory cells such as neutrophils, eosinophils, monocytes, and T lymphocytes. It is also involved in activation of phagocytic cells, release of granule-based enzymes and generation of oxidants. All of which contribute to innate immune functions.

AV node damage may lead to an increase in the PR interval to as high as 0.25 – 0.40 s (normal = 0.12 – 0.20 s). In the case of severe impairment, there might be a complete failure of passage of impulses leading to complete block. In this case, the atria and ventricles will beat independently of each other.

The most likely diagnosis is aortic dissection.

Aortic dissection occurs following a tear in the aortic intima with subsequent separation of the tissue within the weakened media by the propagation of blood. There are four different classifications of aortic dissection and the commonest one used is the Stanford classification dividing them into type A and type B. A type A dissection involves the ascending aorta and/or the arch whilst type B dissection involves only the descending aorta and occurs distal to the origin of the left subclavian artery.

Aortic dissection in pregnancy occurs most commonly in the third trimester due to the hyperdynamic state and hormonal effect on vasculature. Other common predisposing factors for aortic dissection include Marfans syndrome, Ehlers-Danlos syndrome, and bicuspid aortic valve. Aortic dissection often presents with sudden severe, tearing chest pain, vomiting, and syncope, most often from acute pericardial tamponade. The patient may be hypertensive, clinically. The right coronary artery may become involved in the dissection, causing myocardial infarct in up to 2% of the cases (hence ST-segment elevation in the inferior leads). An aortic regurgitant murmur may be auscultated.

The management options during pregnancy include:
1. <28 weeks of gestation: aortic repair with the foetus kept in utero
2. 28–32 weeks of gestation: dependent on foetal condition
3. >32 weeks of gestation: caesarean section followed by aortic repair in the same operation

Hypertrophy of breast tissue in males is known as gynaecomastia. It is normally due to proliferation of stroma and not of mammary ducts. Physiological gynaecomastia can occur during puberty and is often transient, bilateral, symmetrical and possibly tender. Gynaecomastia can occur during old age too, but is usually unilateral. Other causes include hepatic or renal failure, endocrinological disorders, drugs (anabolic steroids, antineoplastic drugs, calcium channel blockers, cimetidine, digitalis, oestrogens, isoniazid, ketoconazole, methadone, metronidazole, reserpine, spironolactone, theophylline), and marijuana. It should not be confused with malignancy which is often hard, asymmetric and fixed to the dermis or fascia. Treatment if indicated, includes withdrawal of the causative factor or treatment of the underlying disorder.

Shock is a life-threatening condition that occurs when the organs and tissues of the body are not receiving a sufficient flow of blood. Lack of blood flow, oxygen and nutrients results in the inability to function properly and damage to many organs. Shock requires immediate treatment because, if left untreated the impaired tissue perfusion and cellular hypoxia can cause irreversible tissue injury, collapse, coma or even death. There are various types of physiological shock, including: cardiogenic (due to heart damage), hypovolaemic (due to low total volume of blood or plasma), neurogenic (due to nervous system damage), septic (due to infections) and anaphylactic shock (due to allergic reactions). Hypovolaemic shock can be caused by blood loss due to trauma, internal bleeding or other fluid loss due to severe burns, prolonged diarrhoea, vomiting and sweating.

Vestibular neuronitis or labyrinthitis causes a self-limited episode of vertigo, presumably due to inflammation of the vestibular division of cranial nerve VIII. Its causes are unknown, It may be due to a virus, but it can be related to a bacterial infection, head injury, stress, allergy, or as a reaction to medication. Symptoms can last up to 7-10 days.

Vincristine is an alkaloid chemotherapeutic agent. It is used to treat a number of types of cancer including acute lymphocytic leukaemia, acute myeloid leukaemia, Hodgkin’s disease, neuroblastoma, and small cell lung cancer among others. The main side-effects of vincristine are peripheral neuropathy and constipation.

The patient is in hypovolemic shock, he is transfused with blood, this fluid resuscitation will result in a decreased sympathetic discharge and adequate ventricular filling which will result in the decreases TPR with an increased CO and cardiac filling pressures