Cont.

Neurogenic shock is due to loss of sympathetic vascular tone and happens only after a significant proportion of the sympathetic nervous system has been damaged - as can occur with lesions above the 6-th thoracic vertebra. In a quadriplegic patient, blood pressure normally ranges between 80/40mmHgto 100/60mmHg and pulse rate is down to 50bpm. The first measure to consider in patients with neurogenic shock is close attention to airway, breathing and circulation (ABC). Hypoperfusion to an injured spine can be associated with poor outcomes. Oxygenation should be monitored carefully, and oxygen be given. It is recommended that patients with traumatic spinal injury receive 15L/m oxygen via a non-rebreathing mask.

Hypotension and bradycardia are common features of neurogenic shock. According to guidelines by Neurosurgical Society of Australia, the first measure to consider is placement of the patient in Trendelenburg position. In neurogenic shock, the main mechanism of hypotension is pooling of blood in the peripheral venules and small vein ; therefore, such maneuver can correct hypotension by increased venous return to the heart.

This patient has hypoxia evident by an oxygen saturation of 88% (normal >95%); therefore, supplemental oxygen should begin. In patients with neurogenic shock, systolic blood pressure should be maintained above 90 mmHg. Placement of the patient in Trendelenburg position (if there are no contraindication e.g. head trauma) should be considered next.

⚠ (Option B) Isotonic fluids (not colloids) should come next after oxygenation and placement in Trendelenburg position. Careful monitoring for volume overload is a 'must'.

⚠ (Option C) Blood transfusion is considered in hemorrhagic patients if there is minimal response to adequate fluid resuscitation using crystalloids (e.g. normal saline). Patients with neurogenic shock as their sole underlying cause of their hypotension do not require blood transfusion.

⚠ (Option D) Vasopressors or inotropes such as dopamine, nor-adrenaline (norepinephrine) or phenylephrine are considered for patients with hypotension resistant to position or adequate volume resuscitation. Urinary output can be an appropriate guide (urinary output of <0.5cc/kg/hour). Adrenaline is not used for such a purpose.

⚠ (Option E) Atropine is reserved for patients with bradycardia of <50bpm. As mentioned earlier, it is quite common for patients with neurogenic shock to have bradycardia down to 50bpm. Atropine is only indicated if there is severe bradycardia (<40bpm), or when there is significant hemodynamic instability caused by it. Atropine should also be considered in patients who are undergoing maneuvers that can induce bradycardia by vagal stimulation e.g. nasopharyngeal suction or intubation.

⏳ Case-based MCQ | #Case_354
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A 50-year-old hypertensive patient presents with light-heodedness of eight hours duration associated with intermittent palpitations, described as very fast and irregular beats. The radial pulse rote is 90/min and irregular, BP is 118/60 mmHg, and there is evidence of biventriculor cardiac failure. The jugular venous pressure (JVP) is elevated 4cm and shows a single waveform consistent with 'v' waves.

⏳ Case-based MCQ | #Case_354 | #answer
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✅ E

This scenario is typical of complete heart block, which frequently presents as episodic loss of consciousness manifest as falls in an elderly patient, and as illustrated in Rhythm strip E (E is correct). Cannon 'a'-waves can be a feature of heart block.

⏳ Case-based MCQ | #Case_355
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A 56-year-old woman presents with a swelling in front of his right ear. He also complains of dry itchy eyes and dry mouth. On examination, the swelling is mobile, and there is xerostomia. Fine needle aspiration cytology (FNAC) is performed which is significant for lymphocytes without any clues to malignancy.

⏳ Case-based MCQ | #Case_355 | #answer
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✅ A

Sjogren syndrome is a chronic inflammatory disease characterized by lymphocytic infiltration and fibrosis of exocrine glands, especially lacrimal and salivary glands. The disease is much more prevalent in women (female/male - 9:1), with the usual age of onset being in the 4 and 5 decades of life. Sjogren syndrome for which no underlying etiology can be identified is termed primary, whereas when it is secondary to other connective tissue disorders, it is called secondary Sjogren syndrome.

🔎 Autoimmune diseases associated with Sjogren syndrome include:
▫Rheumatoid arthritis
▫Systemic lupus erythematosus
▫Scleroderma

Considering the overlap of Sjogren syndrome with many other rheumatic disorders,it is sometimes difficult to determine whether a clinical manifestation is solely a result of Sjogren syndrome or is due to one of its overlapping disorders.
Clinical features of the disease include the following:
• Dry eyes and keratoconjunctivitis sicca due to
decreased tear production- the patient may complain of feeling as sand under the eyelid or itchy eyes.
• Dry mouth (xerostomia) due to decreased salivation - dry mouth can lead to following manifestations:
▪Red smooth and dry tongue
▪Severe and progressive dental caries
▪Cracks at the corners of the mouth
▪Chronic oral Candidiasis
▪Parotid gland swelling

🔔 NOTE - Dry eyes and mouth are the most common presenting features in adults, whereas, children often present with parotid gland enlargement.

🔻Other clinical features of Sjogren disease include:
➕ Nasal dryness - can result in discomfort and bleeding
➕ Vaginal dryness - can result in dyspareunia, vaginitis and pruritus
➕ Myalgia and fatigue
➕ Arthralgia or arthritis - similar to that of SLE with symmetrical
involvement of small joints, arthritis is of non-erosive nature
➕ Raynaud's phenomenon
➕ Recurrent miscarriages or stillbirths in women and history of venous or arterial thrombosis related to the presence of antiphospholipid antibodies (e.g. lupus anticoagulant or anticardiolipin antibodies)
➕ Leukopenia, anemia
➕ Lymphadenopathy
➕ Non-Hodgkin lymphoma

Currently, minor salivary gland biopsy is the best single test to establish a diagnosis of Sjogren syndrome. In this procedure, an incision is made on the inner lip, and some minor salivary glands are removed for examination.

In patients with a possible diagnosis of this disease but with severe extraglandular symptoms, a lip biopsy is often performed to firmly establish the diagnosis of Sjogren syndrome.

While this is the most definitive test,performing it is not absolutely necessary from a clinical standpoint. Patients with Sjogren syndrome are essentially treated symptomatically and are observed for the development of other rheumatic disorders or lymphoma. This can be initiated without performing a biopsy. If, however, the diagnosis is in doubt or if a definitive diagnosis is needed, biopsy is the best test.

⚠ (Option B) Schirmer test shows decreased tear production. A strip of filter paper is put under the lower eye lid, and the distance along the paper that tears are absorbed is measured. Less than 5mm in 5 minutes is considered positive. It is one of the tests routinely performed when Sjogren disease suspected but it is not diagnostic.

⚠ (Options C and D) Imaging studies are not diagnostic; however they may be sometimes needed, particularly with extraglandular manifestations of the disease.

⚠ (Option E) ANA, particularly anti-Ro (SSA) and anti-La (SSB) may be positive, but again these could be elevated in other conditions as well.

⏳ Case-based MCQ | #Case_356
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A 28-year-old man is brought to the Emergency Department after he had an accident while driving a motorcycle and had his right ankle injured. On examination, his vital signs are stable. The right ankle joint is laterally displaced and there is a laceration over the joint.

⏳ Case-based MCQ | #Case_356 | #answer
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✅ D

Open fractures and/or dislocations are defined as the bone and/or joint being exposed to the external environment, or when the fracture or dislocation is caused by blunt or penetrating forces sufficient to disrupt or penetrate skin, subcutaneous tissue, muscle fascia, muscle, and/ or the bone or joint. Open fractures are often contaminated by foreign material (e g., clothing, grass, dirt, gravel), dead or devitalized tissue, and bacteria.

Always follow the following rules in open fractures/ dislocations:
• Remove any gross contamination off the wound. No provisional irrigation or debridement is performed at this stage.
• Take photographs of the wound and dress it with wet sterile cover (the photographs are taken, so that other treating physicians do not need to uncover the wound to see it)
• Give patient analgesic (preferably intravenously to both control the pain and prepare for a reduction in the emergency department). Morphine is a good option.
• By gentle traction, reduce the fracture and correct misalignments as much as possible.
• Start the patient on intravenous prophylactic antibiotics.
• Give the patient tetanus prophylaxis If indicated
• Obtain X-rays
• Urgently arrange for transferring the patient to the operating room for surgical wound debridement and definite treatment of the fracture and dislocation

NOTE • Initial irrigation or debridement in the emergency department is not recommended and should be avoided. In this scenario the most appropriate initial management would be reduction of the dislocation by gentle traction after non-surgical removal of any gross contamination, and analgesia. The patient then should be started on intravenous antibiotics and receive tetanus prophylaxis.

⚠ X-ray of the joint (option E) is required pre-operatively to visualize the anatomical disruption. Wound debridement (option A) is crucial but should be performed in the operating room ideally with 1-2 hours of presentation.

NOTE - In open fractures, wound treatment may sometimes be more time-consuming and require more work than the treatment of the fracture itself.

Debridement may be carried out several times before and after definitive fracture treatment is performed.

⏳ Case-based MCQ | #Case_357
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During physical examination of a 3 kg full-term male newborn, you notice that the baby's oxygen saturation decreases when he is laid. The fall in oxygen saturation is sometimes associated with mild cyanosis. However, when the baby starts crying the oxygen saturation increases again.

⏳ Case-based MCQ | #Case_357 | #answer
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✅ E

The presentation is classic for choanal atresia. Choanal atresia is a congenital abnormality in which there is failure of canalization of the bucconasal membrane. This can be either unilateral or bilateral and is usually due to a combination of bone and soft tissue anomalies.
It occurs in 1:5000 to 1:8000 births. Unilateral atresia is more common, with a predilection for females.

🔆 CHARGE Syndrome (coloboma, heart defect, atresia choanae, retarded growth and development, genital abnormality and ear abnormality) or other congenital abnormalities are present in 50% of patients with bilateral choanal atresia.

Bilateral choanal atresia is a relatively rare anomaly of the upper airway. As neonates are obligatory nasal breathers, presentation may be with life-threatening respiratory distress, retractions and paradoxical cyanotic episodes which are relieved by crying as the infant begins to breathe via the mouth. Unilateral choanal atresia does not usually produce severe symptoms.

When choanal atresia is suspected the diagnosis can be confirmed with trying to pass a nasogastric tube. It is not possible to pass a nasogastric tube through the nares and choanae if there is choanal atresia.

⚠ In the presence of bilateral choanal atresia, as the history and physical suggest, oxygen through nasal cannula will be of no benefit.

➕ Other measures to consider as parts of management plan include:
• Oxygen
• Oropharyngeal airway
• Intubation and ventilation if there is life-threatening hypoxemia
• CT scan
• ENT consultation and referral for surgical treatment

Since the manifestation is typical of choanal atresia, chest X-ray is not indicated now, but it may be obtained to exclude other possible associated respiratory conditions or anomalies

⏳ Case-based MCQ | #Case_358
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A 37-year-old African American woman was diagnosed with diffuse type of scleroderma and was prescribed an antihistamine and prednisolone 5 mg/day to control the pruritus. Two months later, the patient came to you with severe headache, chest pain, and blurred vision. Her BP is 210/118 mmHg. Urinalysis showed proteinuria and microscopic hematuria. Blood urea nitrogen was 11 mmol/L (N 2.9-7.1 mmol/L), and serum creatinine was 124 pmol/L (N 50-90 pmol/L). Your patient was still taking prednisolone 5 mg/day prescribed earlier. You diagnosed your patient with sclerodermal renal crisis and decided to put her on an ACE inhibitor.

⏳ Case-based MCQ | #Case_358 | #answer
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✅ E

Systemic sclerosis (SS) is a chronic systemic disorder of unknown cause. It is characterized by the thickening of the skin and the involvement of multiple internal organs. Thickening of skin distinguishes this disease from other connective tissue diseases. The disease has two distinct phases: an inflammatory and fibrotic phase. Patients with disease can be classified into two major subsets according to the degree of clinically involved skin. Diffuse cutaneous systemic sclerosis is more severe with prominent internal organ involvement, while in the limited form, it is less severe but is more likely to be associated with pulmonary arterial hypertension. Systemic sclerosis affects 9-19 per 100,000 and is more common in women. African Americans are more susceptible to disease. The age of onset is between 30 and 50 years. Development of sclerodermal renal crisis has been associated with glucocorticoid use, and indeed, the use of these drugs should be avoided in scleroderma. They can, however, be used in small doses to control the early skin symptoms, for myositis associated with scleroderma and for cardiac involvement. Thus, in this patient, prednisolone should be stopped completely (choice E) because it is used for non-life-threatening skin lesions. Other glucocorticoids, including dexamethasone, should be avoided in this patient.

🔖 Key point: Development of sclerodermal renal crisis has been associated with glucocorticoid use, which is managed by glucocorticoid discontinuation.

⏳ Case-based MCQ | #Case_359
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A 52-year-old lady presents to your GP clinic for breast cancer screening. She has been on combined hormone replacement therapy (HRT) for the past year. Mammogram is the only available method of screening for breast cancer and because of HRT she is expected to have dense breast tissue.

⏳ Case-based MCQ | #Case_359 | #answer
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✅ C

About 10% of women on combined HRT will have extremely dense breasts; nonetheless, presently, there is not enough evidence to recommend that patients on combined hormone replacement therapy should stop HRT for any length of time before their mammography in an attempt to reduce breast density.

Therefore, this patient should continue HRT and have mammography based on current recommendations for other women. Women making decisions as to whether to commence combined HRT should be aware of the fact that HRT may lead to inaccurate mammography results. Furthermore, they should be informed about the increased risk of breast cancer if HRT extends beyond the recommended period (3 to 5 years).