Juvenile polyps are benign hamartomas with neoplastic potential that are the most frequent gastrointestinal polyp of childhood, with the peak incidence between 3 and 5 years of age.. The presence of multiple juvenile polyps may indicate a premalignant condition commonly named juvenile polyposis coli or juvenile polyposis syndrome (JPS). In contrast, single or solitary juvenile polyps generally are considered benign sporadic lesions that confer little to no future risk of malignancy. Most frequent presentation is painless rectal bleeding. Other features include a prolapsing rectal mass and abdominal pain.

Capillaries are the smallest of the body’s blood vessels, measuring 5–10 μm and they help to enable the exchange of water, oxygen, carbon dioxide, and many other nutrients and waste substances between the blood and the tissues surrounding them. The walls of capillaries are composed of only a single layer of cells, the endothelium. Ion channels are pore-forming proteins that help to establish and control the small voltage gradient that exists across the plasma membrane of all living cells by allowing the flow of ions down their electrochemical gradient. An ion channel is an integral membrane protein or more typically an assembly of several proteins. The archetypal channel pore is just one or two atoms wide at its narrowest point. It conducts a specific ion such as sodium or potassium and conveys them through the membrane in single file.

Jejunostomy feeding (enteral feeding) is now the standard of care in most feeding protocols after esophagectomy. The feeding regimen consists of a gradually increasing volume of feeds in the first five to seven days. Patients should resume oral intake as soon as possible after surgery. In hospital, all forms of enteral access appear to be safe. Out of hospital, the ability to provide home feeding by feeding jejunostomy is likely where meaningful nutritional improvements can be made. Improving nutrition and related quality of life in the early months might improve the long-term outcome

The spinothalamic tract is a sensory pathway originating in the spinal cord that transmits information to the thalamus. There are two main parts of the spinothalamic tract: the lateral spinothalamic tract transmits pain and temperature and the anterior spinothalamic tract transmits touch (crude touch). The decussation of this pathway occurs at the level of the spinal cord. Hence, a unilateral lesion of the lateral spinothalamic tract causes contralateral loss of pain and temperature.

Patients with burns of more than 20% – 25% of their body surface should be managed with aggressive IV fluid resuscitation to prevent “burn shock.
Rule of 9’s for Adults: 9% for each arm, 18% for each leg, 9% for head,18% for front torso, 18% for back torso.
A variety of formulas exist, like Brooke, Galveston, Rule of Ten, but the most common formula is the Parkland Formula. This formula estimates the amount of fluid given in the first 24 hours, starting from the time of the burn.
Four mL lactated ringers solution × percentage total body surface area (%TBSA) burned × patient’s weight in kilograms = total amount of fluid given in the first 24 hours.
One-half of this fluid should be given in the first eight hours.
For example, a 75 kg patient with 55% total body surface area burn would need; 4 mL LR × 75kg × 55% TBSA = 16,500 mL in the first 24 hours, with 8,250 mL in the first eight hours or approximately 1 litre/hr for the first eight hours.
For paediatric patients, the Parkland Formula can be used plus the addition of normal maintenance fluids added to the total.
Whichever formula is used, the important point to remember is the fluid amount calculated is just a guideline. Patient’s vital signs, mental status, capillary refill and urine output must be monitored and fluid rates adjusted accordingly. Urine output of 0.5 mL/kg or about 30 – 50 mL/hr in adults and 0.5-1.0 mL/kg/hr in children less than 30kg is a good target for adequate fluid resuscitation.
Other management for severe burns includes nasal gastric tube placement as most patients will develop ileus. Foley catheters should be placed to monitor urine output. Cardiac and pulse oximetry monitoring is indicated. Pain control is best managed with IV medication. Finally, burns are considered tetanus-prone wounds and tetanus prophylaxis are indicated if not given in the past five years. In any severe flame burn, you should always consider possible associated inhalation injury, carbon monoxide or cyanide poisoning.

Answer: Do not operate and review the patient several weeks later

Air weapon injuries in children should be managed in the same way as any low velocity gun shot injury. Subcutaneous pellets are best removed. Urgent specialist referral is indicated for cranial, ocular, chest, abdominal, or vascular injuries as they may require emergency surgery. Cardiac injuries may be rapidly fatal. Penetrating abdominal injuries involving hollow viscera or major blood vessels need prompt exploration and repair. Intracranial air weapon pellets should be removed if possible. A pellet in lung parenchyma or muscle may be safely left in situ but there is a risk of infection. A pellet that has penetrated a joint or is associated with a fracture requires skilled orthopaedic management. A pellet lodged near a major blood vessel or nerve should ideally be removed. The possibility of intravascular embolism must be considered if the pellet is absent from a suspected entry site and there is no exit wound; numerous examples of arterial and venous embolism of an air weapon pellet in children have been described.

In this case, the child seems well so there is no need to operate. He should be reviewed several weeks later.

Answer: 13

Eye Opening Response
Spontaneous–open with blinking at baseline – 4 points
Opens to verbal command, speech, or shout – 3 points
Opens to pain, not applied to face – 2 point
None – 1 point

Verbal Response
Oriented – 5 points
Confused conversation, but able to answer questions – 4 points
Inappropriate responses, words discernible – 3 points
Incomprehensible speech – 2 points
None – 1 point

Motor Response
Obeys commands for movement – 6 points
Purposeful movement to painful stimulus – 5 points
Withdraws from pain – 4 points
Abnormal (spastic) flexion, decorticate posture – 3 points
Extensor (rigid) response, decerebrate posture – 2 points
None – 1 point

He is seen to be disorientated despite opening his eyes in response to speech and being able to talk. He is also able to obey motor commands. His score is therefore 13: 3 for eye opening response, 4 for verbal response and 6 for motor response.

Brown–Séquard syndrome results due to lateral hemisection of the spinal cord and results in a loss of motricity (paralysis and ataxia) and sensation. The hemisection of the cord results in a lesion of each of the three main neural systems: the principal upper motor neurone pathway of the corticospinal tract, one or both dorsal columns and the spinothalamic tract. As a result of the injury to these three main brain pathways the patient will present with three lesions. The corticospinal lesion produces spastic paralysis on the same side of the body (the loss of moderation by the upper motor neurons). The lesion to fasciculus gracilis or fasciculus cuneatus results in ipsilateral loss of vibration and proprioception (position sense). The loss of the spinothalamic tract leads to pain and temperature sensation being lost from the contralateral side beginning one or two segments below the lesion. At the lesion site, all sensory modalities are lost on the same side, and an ipsilateral flaccid paralysis.

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.

Rejection is related primarily to activation of T cells, which, in turn, stimulate specific antibodies against the graft. Various clinical syndromes of rejection can be correlated with the length of time after transplantation.

Hyperacute rejection
Hyperacute rejection of the renal allograft happens in the operating room within hours of the transplant, when the graft becomes mottled and cyanotic. This type of rejection is due to unrecognized compatibility of blood groups A, AB, B, and O (ABO) or to a positive T-cell crossmatch (class I human leukocyte antigen [HLA] incompatibility).
It is thought that IgG antibodies from the host bind to HLA-1 antigen of the donated organ.
No treatment exists, and nephrectomy is indicated.

Acute rejection
Acute rejection appears within the first 6 months after transplantation and affects approximately 15% of transplanted kidneys. Rejection is secondary to prior sensitization to donor alloantigens (occult T-cell crossmatch) or a positive B-cell crossmatch.
Acute tubular interstitial cellular rejection is the most common type of rejection reaction, with an incidence of approximately 20-25%. Typically, it occurs between 1 and 3 months after transplantation. It is T-cell mediated, and injury is directed to the renal tubules. The standard for diagnosis is renal allograft biopsy. Mild rejections may be successfully reversed with corticosteroids alone, whereas moderate or severe rejections may require the use of anti–T-cell antibodies, either polyclonal or monoclonal.
Late acute rejection is strongly correlated with the scheduled withdrawal of immunosuppressive therapy 6 months after transplantation.

Chronic rejection
Chronic rejection occurs more than 1 year after transplantation and is a major cause of allograft loss. It is a slow and progressive deterioration in renal function characterized by histologic changes involving the renal tubules, capillaries, and interstitium. Its precise mechanism is poorly defined and is an area of intense study. Diagnosis is by renal biopsy, and treatment depends on the identified cause if any. Application of conventional antirejection agents (e.g., corticosteroids or anti–T-cell antibodies) does not appear to alter the progressive course.

Answer: Sickle cell disease

An anal fissure is a painful linear tear or crack in the distal anal canal, which, in the short term, usually involves only the epithelium and, in the long term, involves the full thickness of the anal mucosa. Anal fissures develop with equal frequency in both sexes; they tend to occur in younger and middle-aged persons.

The exact aetiology of anal fissures is unknown, but the initiating factor is thought to be trauma from the passage of a particularly hard or painful bowel movement. Low-fibre diets (e.g., those lacking in raw fruits and vegetables) are associated with the development of anal fissures. No occupations are associated with a higher risk for the development of anal fissures. Prior anal surgery is a predisposing factor because scarring from the surgery may cause either stenosis or tethering of the anal canal, which makes it more susceptible to trauma from hard stool.

In rare cases, an anal fissure may develop due to:
-anal cancer
-HIV
-tuberculosis
-syphilis
-herpes

A study showed that a patient may exhibit severe, disabling, anorectal symptoms which are disproportionate to physical findings. Rarely leukaemia may be the cause, and the first signs of blood dyscrasia may appear in the anorectum. The diagnosis may not be suggested by the history, physical examination or routine laboratory data.

Crohn’s disease can also cause problems around the anus. These may include tiny but painful cracks in the skin known as anal fissures. Tunnelling sores called fistulas cause abnormal connections between the bowel and the skin; or an abscess, a pocket of inflamed or dead tissue that is usually very painful.

Diagnosis of meningitis can be carried out with examination of cerebrospinal fluid (CSF) with a lumbar puncture (LP). In a case of bacterial meningitis, the CSF analysis will show:
– Opening pressure: > 180 mmH2O
– White blood cell count: 10–10 000/μl with neutrophil predominance
– Glucose: < 40 mg/dl
– CSF glucose to serum glucose ratio: < 0.4
– Protein: > 4.5 mg/dl
– Gram stain: positive in > 60%
– Culture: positive in > 80%
– Latex agglutination: may be positive in meningitis due to Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae, Escherichia coli and group B streptococci
– Limulus, lysates: positive in Gram-negative meningitis

Indirect inguinal hernias have a higher risk of strangulation. The risk of strangulation and obstruction is lowest for direct inguinal hernias as they have a wide neck, which can often be monitored and managed conservatively. A study conducted showed that the cumulative probability of strangulation for inguinal hernias was 2.8 per cent, rising to 4.5 per cent after 2 years.

Perihepatic packing is a surgical procedure used in connection with surgery to the liver. In this procedure the liver is packed to stop non arterial bleeding, most often caused by liver injury.

During this surgery laparotomy pads are placed around the site of the bleeding. The main purpose of hepatic packing is to prevent the person from succumbing to the trauma triad of death. Under- or over-packing of the liver can cause adverse outcomes, and if the bleeding cannot be controlled through this surgical method, the Pringle manoeuvre is an alternate technique that can be utilized.

Rebleeding, constant decline of haemoglobin and increased transfusion requirement, as well as the failure of angioembolization of actively bleeding vessels are a few factors which indicate the need for laparotomy.

The operative approach has also evolved over the last two decades. Direct suture ligation of the parenchymal bleeding vessel, perihepatic packing, repair of venous injury under total vascular isolation and damage control surgery with utilization of preoperative and/or postoperative angioembolization are the preferred methods, compared to anatomical resection of the liver and use of the atriocaval shunt.

On transmission of a loud sound into the central nervous system, an attenuation reflex occurs after a latent period of 40-80 ms. This reflex contracts the two muscles that pull malleus and stapes closer, developing a high degree of rigidity in the entire ossicular chain. This reduces the ossicular conduction of low frequency sounds to the cochlea by 30-40 decibels. In this way, the cochlea is protected from damage due to loud sounds (these are low frequency sounds) when they develop slowly.

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.

Various vagotonic manoeuvres (e.g. Valsalva manoeuvre, carotid sinus massage, pressure on eyeballs, ice-water facial immersion, swallowing of ice-cold water) result in increased parasympathetic tone through the vagus nerve which results in a decrease in heart rate. These manoeuvres may be clinically useful in terminating supraventricular arrhythmias.

Haemoglobin S is an abnormal type of haemoglobin seen in sickle cell anaemia. This allows for the haemoglobin to crystalize within the RBC upon exposure to low partial pressures of oxygen. This results in rupture of the RBCs as they pass through microcirculation, especially in the spleen. This can cause blockage of the vessel down stream and ischaemic death of tissues, accompanied by severe pain.

Management of Acute Pancreatitis revolves around supportive care, adequate nutrition, and intravenous hydration. The rationale for hydration is based on the need to resolve the hypovolemia that occurs secondary to vomiting, reduced oral intake, third space extravasation, respiratory losses and diaphoresis. Besides, early hydration provides macrocirculatory and microcirculatory support to prevent the cascade of events leading to pancreatic necrosis.
There is a lack of high level evidence to guide the choice of fluid in AP. Crystalloids are recommended by the American Gastroenterological Association, and colloids (packed red blood cells) are considered in cases of low haematocrit (< 25%) and low serum albumin (< 2 g/dL). Among the crystalloids, Ringer's lactate solution is preferred over Normal saline. However, there is an urgent need of studies on this issue.

Dextran 40 and 70 have a higher rate of causing anaphylaxis than either gelatins or starches.

Dextrans are branched polysaccharide molecules, with dextran 40 and 70 available. The high-molecular-weight dextran 70 may persist for up to eight hours. They inhibit platelet aggregation and leucocyte plugging in the microcirculation, thereby, improving flow through the microcirculation. They are primarily used in sepsis.
Unlike many other intravenous fluids, dextrans are a recognised cause of anaphylaxis.

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

Chronic obstructive pulmonary disease (COPD) is a type of obstructive lung disease characterized by long-term poor airflow. The main symptoms include shortness of breath and cough with sputum production. The best diagnostic test for evaluating patients with suspected chronic obstructive pulmonary disease (COPD) is lung function measured with spirometry. Key spirometrical measures may be obtained with a portable office spirometer and should include forced vital capacity (FVC) and the normal forced expiratory volume in the first second of expiration (FEV1). The ratio of FEV1 to forced vital capacity (FEV1/FVC) normally exceeds 0.75. Patients with COPD typically present with obstructive airflow. Complete pulmonary function testing may show increased total lung capacity, functional residual capacity and residual volume. A substantial loss of lung surface area available for effective oxygen exchange causes diminished carbon monoxide diffusion in the lung (DLco) in patients with emphysema. Tobacco smoking is the most common cause of COPD, with factors such as air pollution and genetics playing a smaller role.

H+/K+-ATPase or ‘proton pump’ located in the canalicular membrane plays a major role in acid secretion. The ATPase here is magnesium-dependent. Omeprazole is a proton pump inhibitor and blocks H+/K+- ATPase.

Normal pH with low p(CO2) and low standard bicarbonate could indicate either compensated respiratory alkalosis or a compensated metabolic acidosis. However, the history of hyperventilation for 5 days (pleurisy) favours compensated respiratory alkalosis. Compensated metabolic acidosis would have been likely in a diabetic patient with fever, vomiting and high glucose (diabetic ketoacidosis).

Unstable angina is a common cardiovascular condition associated with major adverse clinical events. Over the last 15 years, therapeutic advances have dramatically reduced the complication and mortality rates of this serious condition. The standard of therapy in patients with unstable angina now incorporates the combined use of a potent antithrombotic (aspirin, clopidogrel, heparin and glycoprotein IIb/IIIa receptor antagonists) and anti-anginal (β-blockade and intravenous nitrates) regimens complemented by the selective and judicious application of coronary revascularisation strategies.
Increasingly, these invasive and non-invasive therapeutic interventions are being guided not only by the clinical risk profile but also by the determination of serum cardiac and inflammatory markers.
Moreover, rapid and intensive management of associated risk factors, such as hypercholesterolaemia, would appear to have potentially substantial benefits even within the acute in-hospital phase of unstable angina. Aspirin 300mg should be given as soon as possible. If the patient has a moderate to high risk of myocardial infarction, then Clopidogrel should be given with a low molecular weight heparin. Thrombolysis or urgent percutaneous intervention should be given if there are significant ECG changes.

Answer: Transposition of the great arteries

Transposition of the great arteries (TGA) is the most common cyanotic congenital heart lesion that presents in neonates. The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle.

Infants with transposition of the great arteries (TGA) are usually born at term, with cyanosis apparent within hours of birth.

The clinical course and manifestations depend on the extent of intercirculatory mixing and the presence of associated anatomic lesions. Note the following:

Transposition of the great arteries with intact ventricular septum: Prominent and progressive cyanosis within the first 24 hours of life is the usual finding in infants if no significant mixing at the atrial level is evident.

Transposition of the great arteries with large ventricular septal defect: Infants may not initially manifest symptoms of heart disease, although mild cyanosis (particularly when crying) is often noted. Signs of congestive heart failure (tachypnoea, tachycardia, diaphoresis, and failure to gain weight) may become evident over the first 3-6 weeks as pulmonary blood flow increases.

Transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction: Infants often present with extreme cyanosis at birth, proportional to the degree of left ventricular (pulmonary) outflow tract obstruction. The clinical history may be similar to that of an infant with tetralogy of Fallot.

Transposition of the great arteries with ventricular septal defect and pulmonary vascular obstructive disease: Progressively advancing pulmonary vascular obstructive disease can prevent this rare subgroup of patients from developing symptoms of congestive heart failure, despite a large ventricular septal defect. Most often, patients present with progressive cyanosis, despite an early successful palliative procedure.

Lynch syndrome, also known as hereditary non-polyposis colorectal cancer (HNPCC), is a type of inherited cancer syndrome associated with a genetic predisposition to different cancer types. People who have Lynch syndrome have a significantly increased risk of developing colorectal cancer. There is also an increased risk of developing other types of cancers, such as endometrial (uterine), stomach, breast, ovarian, small bowel (intestinal), pancreatic, prostate, urinary tract, liver, kidney, and bile duct cancers. Alterations in several genes involved in DNA mismatch repair that have been linked to Lynch syndrome. They include the genes of MLH1, MSH2, MSH6, PMS2, and EPCAM. A mutation (alteration) in any of these genes gives a person an increased lifetime risk of developing colorectal cancer and other related cancers.

Women also have an increased risk of developing endometrial and ovarian cancers. The safest operative strategy is a pancolectomy and end ileostomy.

The atmospheric pressure is lower at high altitudes as compared with sea level. This leads to a decrease in the partial pressure of oxygen. Once 2100 m (7000 feet) of altitude is reached, there is a drop in saturation of oxyhaemoglobin. The oxygen saturation of haemoglobin determines the oxygen content in the blood. The body physiological tries to adapt to high altitude by acclimatization. Immediate effects include hyperventilation, fluid loss (due to a decreased thirst drive), increase in heart rate and slightly lowered stroke volume. Long term effects include lower lactate production, compensatory alkali loss in urine, decrease in plasma volume, increased erythropoietin release and red cell mass, increased haematocrit, higher concentration of capillaries in striated muscle tissue, increase in myoglobin, increase in mitochondria, increase in aerobic enzyme concentration such as 2,3-DPG and pulmonary vasoconstriction.

Acute transfusion reactions present as adverse signs or symptoms during or within 24 hours of a blood transfusion. The most frequent reactions are fever, chills, pruritus, or urticaria, which typically resolve promptly without specific treatment or complications.

Glucose is reabsorbed almost 100% via sodium–glucose transport proteins (apical) and GLUT (basolateral) in the proximal convoluted tubule. Glycosuria or glucosuria is a condition of osmotic diuresis typical in those suffering from diabetes mellitus. Due to a lack of insulin, plasma glucose levels are above normal. This leads to saturation of receptors in the kidneys and glycosuria usually at plasma glucose levels above 11 mmol/l. Rarely, glycosuria is due to an intrinsic problem with glucose reabsorption within the kidneys (such as Fanconi syndrome), producing a condition termed renal glycosuria.