To confirm the diagnosis, especially in patients with negative anti-Ro or anti-La antibodies, labial gland biopsy is done. In performing a labial biopsy, the surgeon typically makes a shallow 1/2 inch wide incision on either side of the inner lip after numbing the area with a local anaesthetic. Schirmer’s test determines whether the eye produces enough tears to keep it moist. This test can be done for ocular symptoms of Sjogren syndrome but is of no diagnostic importance as it can be positive with many other diseases.

The Hyperlipidaemia Effect: Pseudohyponatremia in Pancreatic Cancer; Patients who have disorders of cholestasis commonly present with volume depletion due to vomiting and poor oral intake, which, in turn, often leads to hypovolemic hyponatremia. It is less well known that disorders of cholestasis, including tumours of the hepatobiliary system, can be accompanied by hyperlipidaemia.

Diclofenac sodium is a non-selective reversible and competitive inhibitor of cyclooxygenase (COX), subsequently blocking the conversion of arachidonic acid into prostaglandin precursors. This leads to an inhibition of the formation of prostaglandins that are involved in pain, inflammation and fever. Clinical trials of several COX-2 selective and non-selective NSAIDs of up to three years duration have shown an increased risk of serious cardiovascular (CV) thrombotic events, including myocardial infarction (MI), and stroke, which can be fatal.

Cyclizine and prochlorperazine are both used for the treatment of Meniere’s disease.

The radial nerve supplies the following: brachioradialis, extensor carpi radialis longus, and the extensor muscles of the forearm (which is via the posterior interosseous nerve). The sensory branches supply the following: dorsum of the thumb, dorsum of the fingers up to the PIPJ (proximal interphalangeal joint), and the middle of the ring finger. The loss of muscle power depends on the extent of the nerve damage – the usual posture is a wrist drop, coupled with thumb adduction and hand pronation. Sensation in this male patient is impaired in this area. The thoracic nerve supplies the serratus anterior muscle, and thus, damage causes winging of the scapula. A median nerve palsy causes the following: the inability to abduct and oppose the thumb; weakness in the forearm, pronation and finger flexion; and sensory loss of the thumb. A T1 nerve root lesion causes Klimke’s palsy – this is a loss of finger flexion, abduction and adduction, and reduced sensation in the medial upper limb. The ulnar nerve is usually compromised at the elbow which causes numbness in the 5th finger, the medial aspect of the ring finger, and the dorsum of the hand (over the 5th finger). A clawed hand can develop (the little and ring fingers curl in towards the palm) if the weakness of the small muscles of the hand is allowed to progress.

From the given answers Fluticasone propionate is the inhaled glucocorticoid. It is not important as a reliever medication but important in maintenance therapy.

Varicella zoster virus (VZV) presents in this way and the rash transforms from macules to vesicles. Pneumonitis is a common complication of VZV infection.

Idiopathic hypercalciuria presents with excess calcium in the urine without an apparent cause. Dietary modification is the first step in addressing this condition, however, because hypercalciuria increases the risk of developing renal stones and bone demineralisation, thiazide diuretics should be prescribed to increase calcium reabsorption when these symptoms are also present.

High blood pressure and a low level of potassium in the blood indicates hyperaldosteronism. Therefore, aldosterone should be tested immediately to establish the diagnosis. Hyperaldosteronism, is a medical condition wherein too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood (hypokalaemia) and increased hydrogen ion excretion (alkalosis).

This case presents with sensorimotor neuropathy secondary to his DM. The progression of the neuropathy, known dying-back neuropathy, is a distal axonopathy or axonal degeneration as where the sensorimotor loss begins distally and travels proximally.

Myasthenia gravis (MG) is an organ-specific autoimmune disease caused by an antibody-mediated assault on the muscle nicotinic acetylcholine receptor (AChR) at the neuromuscular junction. Binding of antibodies to the AChR leads to loss of functional AChRs and impairs the neuromuscular signal transmission, resulting in muscular weakness.

Troponin T is a 37 ku protein that binds to tropomyosin, thereby attaching the troponin complex to the thin filament.

Ventricular tachycardia is not linked with Wolff-Parkinson-White syndrome. The other options are linked to WPW syndrome.

A Coomb’s test should be performed to test for autoimmune haemolytic anaemia. Spherocytes and reticulocytes in the blood film are indications for haemolytic anaemia.

High Altitude Cerebral Oedema (HACE) is a severe and potentially fatal manifestation of high altitude illness and is often characterized by ataxia, fatigue, and altered mental status. HACE is often thought of as an extreme form/end-stage of Acute Mountain Sickness (AMS). Although HACE represents the least common form of altitude illness, it may progress rapidly to coma and death as a result of brain herniation within 24 hours, if not promptly diagnosed and treated.

HACE generally occurs after 2 days above 4000m but can occur at lower elevations (2500m) and with faster onset. Some, but not all, individuals will suffer from symptoms of AMS such as headache, insomnia, anorexia, nausea prior to transitioning to HACE. Some may also have concomitant High Altitude Pulmonary Oedema (HAPE). HACE in isolation is rare, but the absence of concomitant HAPE or symptoms of AMS prior to deterioration does not rule-out the presence of HACE.

Most cases develop as a progression of AMS and will include a history of recent ascent to altitude and prior complaints/findings of AMS including a headache, fatigue, nausea, insomnia, and/or light-headedness. Some may also have signs/symptoms of HAPE. Transition to HACE is heralded by signs of encephalopathy including ataxia (usually the earliest clinical finding) and altered mentation which may range from mild to severe. Other symptoms may include a more severe headache, difficulty speaking, lassitude, a decline in the level of consciousness, and/or focal neurological deficits or seizures.

The mainstay of treatment is the immediate descent of at least 1000m or until symptoms improve. If descent is not an option, one may use a portable hyperbaric chamber and/or supplemental oxygen to temporize illness, but this should never replace or delay evaluation/descent when possible. If available, dexamethasone 8mg for one dose, followed by 4mg every 6 hours should be given to adults via PO, IM, or IV routes.
Acetazolamide has proven to be beneficial in only a single clinical study. The suggested dosing regimen for Acetazolamide is 250 mg PO, given twice daily. Though effective in alleviating or temporizing symptoms, none of the adjunct treatment modalities are definitive or a replacement for an immediate descent.

Friedreich’s ataxia is an autosomal recessive disorder that usually begins before the end of the teens. It has an estimated prevalence in Europe of 1 in 50,000 and life expectancy is around 40-50 years. Neurological features include a progressive ataxia, cerebellar dysarthria, lower limb areflexia, decreased vibratory sensation and proprioception, and pyramidal weakness. Pes cavus and scoliosis are also both seen. Cardiomyopathy occurs in over 70% of cases. Less common features include optic atrophic, diabetes mellitus, and deafness.

Acute exacerbations of chronic obstructive pulmonary disease (COPD) are immediately treated with inhaled beta2 agonists and inhaled anticholinergics, followed by antibiotics (if indicated) and systemic corticosteroids. Methylxanthine therapy may be considered in patients who do not respond to other bronchodilators.
High flow oxygen would worsen his symptoms. Usually titrated oxygen (88 to 92 %) is given in such patients to avoid the risk of hyperoxic hypercarbia in which increasing oxygen saturation in a chronic carbon dioxide retainer can inadvertently lead to respiratory acidosis and death.

Rifampicin is the only antibiotic that has been reported to reduce plasma oestrogen concentrations. When taking Rifampicin, oral contraceptives cannot be relied upon and a second method of contraception is mandatory. Amoxicillin, ampicillin, griseofulvin, metronidazole and tetracycline have been rarely associated with contraceptive failure. When these agents are used, the clinician should discuss the available data with the patient and suggest a second form of birth control. Other antibiotics are most likely safe to use concomitantly with oral contraceptives.
The danger with COCP is enzyme inducers which can lower the levels of the hormone in he blood, azithromycin is not an enzyme inducer. No additional precautions are required to maintain contraceptive efficacy when using antibiotics that are not enzyme inducers with combined hormonal methods for durations of 3 weeks or less. The only proviso would be that if the antibiotics (and/or the illness) caused vomiting or diarrhoea, then the usual additional precautions relating to these conditions should be observed.

Inducers: RASAG
– Rifampicin
– Anticonvulsants, particularly phenytoin, carbamazepine, phenobarbitone and primidone
– Spironolactone, St Johns wort
– Alcohol, long term
– Griseofulvin

Long-term oxygen therapy (LTOT) is the cornerstone mode of treatment in patients with severe chronic obstructive pulmonary disease (COPD) associated with resting hypoxaemia. When appropriately prescribed and correctly used, LTOT has clearly been shown to improve survival in hypoxemic COPD patients. Requirements to proceed to LTOT is the patient should be stable and on appropriate optimum therapy (as in given case) and having stopped smoking tobacco. The patient should be shown to have a PaO2 of less than 7.3 kPa and/or a PaCO2 of greater than 6 kPa on two occasions at least 3 weeks apart. FEV1 should be less than 1.5 litres, and there should be a less than 15% improvement in FEV1 after bronchodilators. Patients with a PaO2 between 7.3 and 8 kPa who have polycythaemia, right heart failure or pulmonary hypertension may benefit from LTOT.

The presentation is highly suggestive of a hypoglycaemic episode, which is more common amongst older patient with diabetes mellitus. The chance of hypoglycaemia is higher with insulin users. Blood sugar measurement is crucial in the management.

In a patient with abnormal LFTs and UC, think primary sclerosing cholangitis (PSC). MRCP and liver biopsy is the best answer. MRCP will show classically beads on a string – intra and extrahepatic stricturing and dilation. Remember this finding!! Liver biopsy is required for official diagnosis (need tissue!).

Poor prognostic factors for breast cancer include:
1. Young age (<40 years)
2. Premenopausal at the time of diagnosis
3. Increased tumour size
4. High-grade tumour
5. Oestrogen and progesterone receptor-negative tumour
6. Positive lymph node status

As the patient has early menopause, hormone replacement therapy (HT) is considered to be the first line of choice for prevention of bone loss and fracture in the early postmenopausal period for 5 years.

The Green Book guidelines report in an active infection (HBeAg+ and HBsAg+) to give the vaccine as well as immunoglobulin. If antibodies are present it is not active infection (anti-Hbe) and then in that case only the vaccine, and no immunoglobulin, would be given. The presence of HBeAg means a person is infectious and can transmit to other people.

Candida albicans is the most common cause of candidiasis and appears almost universally in low numbers on healthy skin, in the oropharyngeal cavity, and in the gastrointestinal and genitourinary tracts. In immunocompetent individuals, C. albicans usually causes minor localized infections, including thrush (affecting the oral cavity), vaginal yeast infections (if there is an underlying pH imbalance), and infections of the intertriginous areas of skin (e.g., the axillae or gluteal folds). More widespread and systemic infections may occur in immunocompromised individuals (e.g., neonates, diabetics, and HIV patients), with the oesophagus most commonly affected (candida esophagitis). Localized cutaneous candidiasis infections may be treated with topical antifungal agents (e.g., clotrimazole). More widespread and systemic infections require systemic therapy with fluconazole or caspofungin.

This presentation is known as ‘Saturday night palsy’. When someone falls asleep with a arm hanging over the arm rest of a chair, the radial nerve compresses and causes wrist drop and loss of sensation at the web of the thumb.

Hyperthyroidism is characterised by heat intolerance, loss of weight, increased sweating, increased bowel frequency and tachycardia. On GPE, there might be proptosis of eyes and tremors in the hands.

The history is suggestive of scleroderma renal crisis, where we would expect thrombotic microangiopathy process with vascular endothelium involvement manifesting as mucoid intimal thickening or onion skin effect.

This patient meets the diagnostic criteria for neutropenic sepsis, which is a relatively common complication of cancer therapy – usually chemotherapy occurring 7-14 days after. It is defined as a neutrophil count of <0.5 x 10^9/L in a patient undergoing anticancer treatment and who has either a temperature higher than 38°C or has other features consistent with clinically significant sepsis. Management approach is the same as mentioned in this case. However, if the patient still remains unwell, then an antifungal such as amphotericin B is started after risk-stratifying the patient and carrying out investigations (e.g. HRCT and Aspergillus PCR) to determine the likelihood of systemic fungal infection.

Both fibrates and nicotinic acid have been associated with myositis, especially when combined with a statin. However, the Committee on Safety of Medicines has produced guidance which specifically warns about the concomitant prescription of fibrates with statins concerning muscle toxicity.

Bezafibrate: It is a fibric acid derivative (fibrate) that has been used as a class of agents known to decrease triglyceride levels while substantially increasing HDL-C levels.
Pharmacological effects:
– Increases VLDL catabolism by increasing lipoprotein and hepatic triglyceride lipase.
– Decreases triglyceride synthesis by inhibiting acetyl-CoA reductase.
– Decreases cholesterol synthesis by inhibiting HMG-CoA reductase.

Side effects:
– Hypersensitivity
– Primary biliary cirrhosis
– Pre-existing gallbladder disease
– Concurrent use with HMG-CoA inhibitors (statins) can produce myopathy
– Hepatic/renal impairment in a patient warrants dose adjustment as this drug is primarily excreted via the renal mechanism.

Contraindications: Concurrent use of MAO inhibitors, hypersensitivity, pre-existing cholestasis, and pregnancy.

Use: It can be used to treat Barth syndrome (characterized by dilated cardiomyopathy, neutropenia (presenting with recurrent infections), skeletal myopathy and short stature)