Signs and symptoms of Sickle cell disease(SCD):
Acute and chronic pain: The most common clinical manifestation of SCD is vaso-occlusive crisis; pain crises are the most distinguishing clinical feature of SCD
Bone pain: Often seen in long bones of extremities, primarily due to bone marrow infarction
Anaemia: Universally present, chronic, and haemolytic in nature
Aplastic crisis: Serious complication due to infection with parvovirus B19 (B19V)
Splenic sequestration: Characterized by the onset of life-threatening anaemia with rapid enlargement of the spleen and high reticulocyte count
Infection: Organisms that pose the greatest danger include encapsulated respiratory bacteria, particularly Streptococcus pneumoniae; adult infections are predominantly with gram-negative organisms, especially Salmonella
Growth retardation, delayed sexual maturation, being underweight
Hand-foot syndrome: This is a dactylitis presenting as bilateral painful and swollen hands and/or feet in children
Acute chest syndrome: Young children present with chest pain, fever, cough, tachypnoea, leucocytosis, and pulmonary infiltrates in the upper lobes; adults are usually afebrile, dyspnoeic with severe chest pain, with multilobar/lower lobe disease
Pulmonary hypertension: Increasingly recognized as a serious complication of SCD
Avascular necrosis of the femoral or humeral head: Due to vascular occlusion
Central nervous system (CNS) involvement: Most severe manifestation is stroke
Ophthalmologic involvement: Ptosis, retinal vascular changes, proliferative retinitis
Cardiac involvement: Dilation of both ventricles and the left atrium
Gastrointestinal involvement: Cholelithiasis is common in children; liver may become involved
Genitourinary involvement: Kidneys lose concentrating capacity; priapism is a well-recognized complication of SCD
Dermatologic involvement: Leg ulcers are a chronic painful problem

Inferior medial pontine syndrome, also known as Foville syndrome, is one of the brainstem stroke syndromes which occurs when there is infarction of the medial inferior aspect of the pons due to occlusion of the paramedian branches of the basilar artery.

It is characterized by ipsilateral sixth nerve palsy, facial palsy, contralateral hemiparesis, contralateral loss of proprioception and vibration, ipsilateral ataxia, ipsilateral facial weakness, and lateral gaze paralysis and diplopia.

Approximately 400 red blood cell group antigens have been described. The ABO group antigens are unusual in that naturally occurring antibodies occur in the plasma of subjects who lack the corresponding antigen, even if they have not been exposed to that antigen previously. The most important of these natural antibodies are anti-A and anti-B, which are usually IgM. Anti-D antibodies don’t occur naturally, and are therefore immune antibodies that result from previous transfusions or pregnancy. Only IgG antibodies are capable of transplacental passage and the most important immune antibody is the Rh antibody, anti-D.

Nitrous oxide should not be utilized in patients with an air-containing confined area because it diffuses into these spaces, causing a rise in pressure. This includes circumstances like pneumothorax, intracranial air after a head injury, imprisoned air from a recent undersea dive, a recent intraocular gas injection, or intestinal blockage. Nitrous oxide increases cerebral blood flow and should be avoided in individuals who have or are at risk of having high intracranial pressure.

Pericardiocentesis is a procedure that removes excess fluid from the pericardium. As a result, it’s used in cases of cardiac tamponade, which occurs when there’s too much fluid in the space around the heart.

During the procedure, a needle and a small catheter are inserted 1 to 2 cm inferior and to the left of the xiphochondral junction into the pericardial cavity.

Phenytoin is in the anticonvulsants class of drugs and is used in the management and treatment of the following:
1. epilepsy
2. generalized tonic-clonic seizures
3. complex partial seizures
4. status epilepticus.

It works by inactivating the voltage-gated sodium channels responsible for increasing the action potential. It is non-specific and targets almost all voltage-gated sodium channel subtypes. More specifically, phenytoin prevents seizures by inhibiting the positive feedback loop that results in neuronal propagation of high-frequency action potentials.

C. difficile is normally found in the gut flora but its growth is normally suppressed by more dominant anaerobes. It has exotoxin-mediated effects causing profuse diarrhoea. Oral clindamycin (a broad spectrum antibiotic) is commonly implicated in precipitating C. difficile colitis; first line treatment is with oral metronidazole. Tetanolysin is a toxin produced by Clostridium tetani bacteria.

Haemostasis is your body’s defence against an injury that causes bleeding. It stops bleeding in three main steps:

1) Primary haemostasis – formation of a weak platelet plug
– The primary reaction of the body is to cause local vasoconstriction at the site of injury and decrease blood flow to the affected area
– the release of cytokines and inflammatory markers lead to adhesion of platelets and aggregation at the site of injury forming a platelet plug
– the injured vessel wall has exposed subendothelial collagen that releases von Willebrand factor

Any damage to the vessel wall causes the release of the Von Willebrand factor, which is necessary for platelet adhesion. Tissue Thromboplastin is also released, which activates the coagulation pathway, a component of secondary haemostasis. The coagulation cascade ultimately results in the conversion of fibrinogen to fibrin.

2) Secondary haemostasis
3) Fibrinolysis

Fibrin (factor Ia) is a long, thin protein with branches produced at the end of the coagulation cascade when fibrinogen (factor I) is converted to fibrin, which stabilizes the blood clot.

Lymphoma is a cancer of the lymphatic system, which is part of the body’s germ-fighting network. They are a group of diseases that are caused by malignant lymphocytes. These malignant cells accumulate in lymph nodes and other lymphoid tissue, giving rise to the characteristic clinical feature of lymphadenopathy.
They can be subdivided into Hodgkin lymphoma (HL) which are characterised by the presence of Reed-Sternberg cells, and non-Hodgkin lymphoma (NHL).
Characteristics of HL include:
1. can present at any age but is rare in children and has a peak incidence in young adults,
2. almost 2:1 male predominance.
3. presents with painless, asymmetrical, firm and discrete enlargement of superficial lymph nodes.
4. cervical node involvement in 60-70% of cases,
5. axillary node involvement in 10-15%
6. inguinal node involvement in 6-12%.
7. modest splenomegaly during the course of the disease in 50% of patients
8. may occasionally have liver enlargement
9. bone marrow failure involvement is unusual in early disease.
Approximately 85% of patients are cured, but the prognosis depends on age, stage and histology.
Two well‐known but rare symptoms in HL are alcohol‐induced pain and pruritus.

Extensor carpi ulnaris is a fusiform muscle in the posterior forearm. It spans between the elbow and base of the little finger. This muscle belongs to the superficial forearm extensor group, along with anconaeus, brachioradialis, extensor carpi radialis longus, extensor carpi radialis brevis, extensor digitorum and extensor digiti minimi muscles.

Like all the muscles of this compartment, extensor carpi ulnaris works as an extensor of the wrist. Moreover, due to its specific course, this muscle also acts to adduct the hand.

Extensor carpi ulnaris is innervated by the posterior interosseous nerve (C7, C8), a branch of the deep division of the radial nerve. The radial nerve stems from the posterior cord of the brachial plexus.

Blood supply to the extensor carpi ulnaris muscle is provided by branches of the radial recurrent and posterior interosseous arteries, which stem from the radial and ulnar arteries, respectively.

Inguinal hernias can present with an array of different symptoms. Most patients present with a bulge in the groin area, or pain in the groin. Some will describe the pain or bulge that gets worse with physical activity or coughing. Symptoms may include a burning or pinching sensation in the groin. These sensations can radiate into the scrotum or down the leg. It is important to perform a thorough physical and history to rule out other causes of groin pain. At times an inguinal hernia can present with severe pain or obstructive symptoms caused by incarceration or strangulation of the hernia sac contents. A proper physical exam is essential in the diagnosis of an inguinal hernia. Physical examination is the best way to diagnose a hernia. The exam is best performed with the patient standing. Visual inspection of the inguinal area is conducted first to rule out obvious bulges or asymmetry in groin or scrotum. Next, the examiner palpates over the groin and scrotum to detect the presence of a hernia. The palpation of the inguinal canal is completed last. The examiner palpates through the scrotum and towards the external inguinal ring. The patient is then instructed to cough or perform a Valsalva manoeuvre. If a hernia is present, the examiner will be able to palpate a bulge that moves in and out as the patient increases intra abdominal pressure through coughing or Valsalva.

Groin hernias are categorized into 2 main categories: inguinal and femoral.

Inguinal hernias are further subdivided into direct and indirect. An indirect hernia occurs when abdominal contents protrude through the internal inguinal ring and into the inguinal canal. This occurs lateral to the inferior epigastric vessels. The hernia contents may extend into the scrotum, and can be reduced superiorly then superolaterally. A direct inguinal hernia is protrusion of abdominal contents through the transversalis fascia within Hesselbach’s triangle. The borders of Hesselbach’s triangle are the inferior epigastric vessels superolaterally, the rectus sheath medially, and inguinal ligament inferiorly.

A femoral hernia is a protrusion into the femoral ring. The borders of the femoral ring are the femoral vein laterally, Cooper’s ligament posteriorly, the iliopubic tract/inguinal ligament anteriorly and lacunar ligament medially.

If the complement sequence is completed, an active phospholipase (the membrane attack complex, MAC) is produced, which punches holes in the cell membrane and causes cell lysis. Because the MAC appears to be the sole means to destroy the Neisseria family of bacteria, C5 – C9 deficiency increases susceptibility to Neisseria infections.

Damage to the deep fibular nerve results in loss of dorsiflexion of the ankle, with resultant foot drop with high-stepping gait, loss of toe extension, weakness of foot inversion and loss of sensation over the webspace between the 1st and 2nd toes.

Synthesis of platelets occurs in the bone marrow by fragmentation of megakaryocytes cytoplasm, derived from the common myeloid progenitor cell. The average time for differentiation of the human stem cell to the production of platelets is about 10 days. The major regulator of platelet formation is thrombopoietin and 95% of this is produced by the liver. Normal platelet count is 150 – 450 x 109/L and the normal lifespan of a platelet is about 10 days. Usually about one-third of the marrow output of platelets may be trapped at any one time in the normal spleen.

Lithium is the drug of choice for bipolar disorders but is commonly associated with side effects and toxicity.

Fine hand tremor is very commonly seen and reported in as many as 50% of patients during the first week of therapy with Lithium. The tremor tends to reduce with time and is only present in around 5% of patients taking the medication two years or longer. Lithium tremors are more common with older age, presumably due to the additive effects of age-related essential tremors.

Option The normal therapeutic range is 2.0-2.5 mmol/l: Lithium should be carefully monitored as it has a very low therapeutic index. The normal therapeutic range is 0.4-0.8 mmol/l. Levels should be checked one week after starting therapy and one week after every change in dosage. (Option Levels should be checked one month after starting therapy)

Option It can induce hyperthyroidism: Lithium has a known effect on thyroid function. Lithium decreases the production of T4 and T3 and commonly causes hypothyroidism. More rarely, lithium causes hyperthyroidism due to thyroiditis.

Option It can induce diabetes mellitus: Lithium can induce nephrogenic diabetes insipidus but not diabetes mellitus.

Of the filtered sodium, about 65% is reabsorbed in the proximal tubule.

Treponema pallidum is an obligate pathogen. Obligate pathogens are almost always associated with disease and usually cannot survive outside of the body for long periods of time. Examples include and HIV.

Staphylococcus aureus and Bacteroides fragilis are conditional pathogens. These are pathogens that usually cause disease only if certain conditions are met.

Pneumocystis jiroveci and Pseudomonas aeruginosa, are opportunistic pathogens. These are pathogens that can only cause disease in an immunocompromised host or under unusual circumstances.

A low pH in the stomach inhibits gastrin secretion, therefore when the stomach is empty or when acid has been secreted for some time after food has entered it, there is inhibition of acid secretion. However, when food first enters the stomach, the pH rises, and this leads to release of the inhibition and causes a maximum secretion of gastrin. Thus gastric acid secretion is self-regulating.

Rodents and domestic animals are the primary reservoirs for the Leptospira spp, although other animals, including cows, horses, mongooses, and frogs, can also harbour the leptospires. Humans may be directly infected from animal urine or indirectly by contact with soil or water that is contaminated with urine from infected animals. Infected humans can shed leptospires in urine for up to 11 months, infected cows for 3.5 months, infected dogs for 4 years, and infected rodents possibly for their entire lifetime.

The organisms enter the host through mucous membranes or abraded skin. The incubation period ranges from 5 to 14 days.

Phyllocontin continus contains aminophylline (a mixture of theophylline and ethylenediamine), a bronchodilator used in the management of COPD and asthma.
This patient is exhibiting symptoms of theophylline toxicity, which may have been triggered by the prescription of the antibiotic. Quinolone antibiotics, such as ciprofloxacin and levofloxacin, and macrolide antibiotics, such as erythromycin, increase the plasma concentration of theophyllines and can lead to toxicity.
The drugs that commonly affect the half-life and the plasma concentration of theophylline are summarised in the table below:
Drugs increasing plasma concentration of theophylline
Drugs decreasing plasma concentration of theophylline
Calcium channel blockers, e.g. Verapamil
Cimetidine
Fluconazole
Macrolides, e.g. erythromycin
Quinolones, e.g. ciprofloxacin
Methotrexate
Barbiturates
Carbamazepine
Phenobarbitol
Phenytoin (and fosphenytoin)
Rifampicin
St. John’s wort