Congenital adrenal hyperplasia (CAH) is a group of inherited disorders that are caused by autosomal recessive genes. The majority of affected patients, over 90%, have a deficiency of the enzyme 21-hydroxylase. This enzyme is encoded by the 21-hydroxylase gene, which is located on chromosome 6p21 within the HLA histocompatibility complex. The second most common cause of CAH is a deficiency of the enzyme 11-beta-hydroxylase. The condition is rare, with an incidence of approximately 1 in 500 births in the UK. It is more prevalent in the offspring of consanguineous marriages.

The deficiency of 21-hydroxylase leads to a deficiency of cortisol and/or aldosterone, as well as an excess of precursor steroids. As a result, there is an increased secretion of ACTH from the anterior pituitary, leading to adrenocortical hyperplasia.

The severity of CAH varies depending on the degree of 21-hydroxylase deficiency. Female infants often exhibit ambiguous genitalia, such as clitoral hypertrophy and labial fusion. Male infants may have an enlarged scrotum and/or scrotal pigmentation. Hirsutism, or excessive hair growth, occurs in 10% of cases.

Boys with CAH often experience a salt-losing adrenal crisis at around 1-3 weeks of age. This crisis is characterized by symptoms such as vomiting, weight loss, floppiness, and circulatory collapse.

The diagnosis of CAH can be made by detecting markedly elevated levels of the metabolic precursor 17-hydroxyprogesterone. Neonatal screening is possible, primarily through the identification of persistently elevated 17-hydroxyprogesterone levels.

In infants presenting with a salt-losing crisis, the following biochemical abnormalities are observed: hyponatremia (low sodium levels), hyperkalemia (high potassium levels), metabolic acidosis, and hypoglycemia.

Boys experiencing a salt-losing crisis will require fluid resuscitation, intravenous dextrose, and intravenous hydrocortisone.

Affected females will require corrective surgery for their external genitalia. However, they have an intact uterus and ovaries and are capable of having children.

The long-term management of both sexes involves lifelong replacement of hydrocortisone (to suppress ACTH levels).

The management of anaphylaxis involves several important steps. First and foremost, it is crucial to ensure proper airway management. Additionally, early administration of adrenaline is essential, preferably in the anterolateral aspect of the middle third of the thigh. Aggressive fluid resuscitation is also necessary. In severe cases, intubation may be required. However, it is important to note that the administration of chlorpheniramine and hydrocortisone should only be considered after early resuscitation has taken place.

Adrenaline is the most vital medication for treating anaphylactic reactions. It acts as an alpha-adrenergic receptor agonist, which helps reverse peripheral vasodilatation and reduce oedema. Furthermore, its beta-adrenergic effects aid in dilating the bronchial airways, increasing the force of myocardial contraction, and suppressing histamine and leukotriene release. Administering adrenaline as the first drug is crucial, and the intramuscular (IM) route is generally the most effective for most individuals.

The recommended doses of IM adrenaline for different age groups during anaphylaxis are as follows:

– Children under 6 years: 150 mcg (0.15 mL of 1:1000)
– Children aged 6-12 years: 300 mcg (0.3 mL of 1:1000)
– Children older than 12 years: 500 mcg (0.5 mL of 1:1000)
– Adults: 500 mcg (0.5 mL of 1:1000)

Calcium-channel blocker overdose is a serious condition that can be life-threatening. The most dangerous types of calcium channel blockers in overdose are verapamil and diltiazem. These medications work by binding to the alpha-1 subunit of L-type calcium channels, which prevents the entry of calcium into cells. These channels are important for the functioning of cardiac myocytes, vascular smooth muscle cells, and islet beta-cells.

When managing a patient with calcium-channel blocker overdose, it is crucial to follow the standard ABC approach for resuscitation. If there is a risk of life-threatening toxicity, early intubation and ventilation should be considered. Invasive blood pressure monitoring is also necessary if hypotension and shock are developing.

The specific treatments for calcium-channel blocker overdose primarily focus on supporting the cardiovascular system. These treatments include:

1. Fluid resuscitation: Administer up to 20 mL/kg of crystalloid solution.

2. Calcium administration: This can temporarily increase blood pressure and heart rate. Options include 10% calcium gluconate (60 mL IV) or 10% calcium chloride (20 mL IV) via central venous access. Repeat boluses can be given up to three times, and a calcium infusion may be necessary to maintain serum calcium levels above 2.0 mEq/L.

3. Atropine: Consider administering 0.6 mg every 2 minutes, up to a total of 1.8 mg. However, atropine is often ineffective in these cases.

4. High dose insulin – euglycemic therapy (HIET): The use of HIET in managing cardiovascular toxicity has evolved. It used to be a last-resort measure, but early administration is now increasingly recommended. This involves giving a bolus of short-acting insulin (1 U/kg) and 50 mL of 50% glucose IV (unless there is marked hyperglycemia). Therapy should be continued with a short-acting insulin/dextrose infusion. Glucose levels should be monitored frequently, and potassium should be replaced if levels drop below 2.5 mmol/L.

5. Vasoactive infusions: Catecholamines such as dopamine, adrenaline, and/or noradrenaline can be titrated to achieve the desired inotropic and chronotropic effects.

6. Sodium bicarbonate: Consider using sodium bicarbonate in cases where a severe metabolic acidosis develops.

The Apgar score is a straightforward way to evaluate the well-being of a newborn baby right after birth. It consists of five criteria, each assigned a score ranging from zero to two. Typically, the assessment is conducted at one and five minutes after delivery, with the possibility of repeating it later if the score remains low. A score of 7 or higher is considered normal, while a score of 4-6 is considered fairly low, and a score of 3 or below is regarded as critically low. To remember the five criteria, you can use the acronym APGAR:

Appearance
Pulse rate
Grimace
Activity
Respiratory effort

The Apgar score criteria are as follows:

Score of 0:
Appearance (skin color): Blue or pale all over
Pulse rate: Absent
Reflex irritability (grimace): No response to stimulation
Activity: None
Respiratory effort: Absent

Score of 1:
Appearance (skin color): Blue at extremities (acrocyanosis)
Pulse rate: Less than 100 per minute
Reflex irritability (grimace): Grimace on suction or aggressive stimulation
Activity: Some flexion
Respiratory effort: Weak, irregular, gasping

Score of 2:
Appearance (skin color): No cyanosis, body and extremities pink
Pulse rate: More than 100 per minute
Reflex irritability (grimace): Cry on stimulation
Activity: Flexed arms and legs that resist extension
Respiratory effort: Strong, robust cry

In this scenario, a 48-year-old patient presents to the emergency department with severe headache, excessive sweating, and episodes of blurred vision. The patient’s triage observations reveal a significantly elevated blood pressure of 234/138 mmHg. The patient also mentions that they are awaiting the results of a 24-hour urine collection for hypertension investigation, specifically for catecholamines, metanephrines, and normetanephrines, as there is suspicion of phaeochromocytoma.

Phaeochromocytoma is a rare tumor that arises from the adrenal glands and can cause excessive release of catecholamines, such as adrenaline and noradrenaline. This leads to symptoms like severe hypertension, headache, sweating, and palpitations.

Given the patient’s presentation and suspicion of phaeochromocytoma, the most appropriate medication choice to lower the blood pressure would be phentolamine. Phentolamine is an alpha-adrenergic antagonist that blocks the effects of catecholamines on blood vessels, resulting in vasodilation and a decrease in blood pressure.

Hydralazine, magnesium sulfate, and glyceryl trinitrate are not the most appropriate choices in this scenario. Hydralazine is a direct vasodilator that acts on smooth muscle to relax blood vessels, but it does not specifically target the effects of catecholamines. Magnesium sulfate is commonly used for conditions like preeclampsia and eclampsia, but it does not directly address the underlying cause of hypertension in phaeochromocytoma. Glyceryl trinitrate, also known as nitroglycerin, is primarily used for the management of angina and does not specifically target the effects of catecholamines.

Diazepam is a benzodiazepine that has sedative and anxiolytic properties but does not directly lower blood pressure or address the underlying cause of hypertension in phaeochromocytoma.

Further Reading:

A hypertensive emergency is characterized by a significant increase in blood pressure accompanied by acute or progressive damage to organs. While there is no specific blood pressure value that defines a hypertensive emergency, systolic blood pressure is typically above 180 mmHg and/or diastolic blood pressure is above 120 mmHg. The most common presentations of hypertensive emergencies include cerebral infarction, pulmonary edema, encephalopathy, and congestive cardiac failure. Less common presentations include intracranial hemorrhage, aortic dissection, and pre-eclampsia/eclampsia.

The signs and symptoms of hypertensive emergencies can vary widely due to the potential dysfunction of every physiological system. Some common signs and symptoms include headache, nausea and/or vomiting, chest pain, arrhythmia, proteinuria, signs of acute kidney failure, epistaxis, dyspnea, dizziness, anxiety, confusion, paraesthesia or anesthesia, and blurred vision. Clinical assessment focuses on detecting acute or progressive damage to the cardiovascular, renal, and central nervous systems.

Investigations that are essential in evaluating hypertensive emergencies include U&Es (electrolyte levels), urinalysis, ECG, and CXR. Additional investigations may be considered depending on the suspected underlying cause, such as a CT head for encephalopathy or new onset confusion, CT thorax for suspected aortic dissection, and CT abdomen for suspected phaeochromocytoma. Plasma free metanephrines, urine total catecholamines, vanillylmandelic acid (VMA), and metanephrine may be tested if phaeochromocytoma is suspected. Urine screening for cocaine and/or amphetamines may be appropriate in certain cases, as well as an endocrine screen for Cushing’s syndrome.

The management of hypertensive emergencies involves cautious reduction of blood pressure to avoid precipitating renal, cerebral, or coronary ischemia. Staged blood pressure reduction is typically the goal, with an initial reduction in mean arterial pressure (MAP) by no more than 25% in the first hour. Further gradual reduction to a systolic blood pressure of 160 mmHg and diastolic blood pressure of 100 mmHg over the next 2 to 6 hours is recommended. Initial management involves treatment with intravenous antihypertensive agents in an intensive care setting with appropriate monitoring.

Potassium-sparing diuretics, like spironolactone and amiloride, can raise the chances of developing hyperkalemia when taken alongside ACE inhibitors, such as ramipril, and angiotensin-II receptor antagonists, like losartan.

For more information, you can refer to the BNF section on ramipril interactions.

Patients typically initiate dialysis when their glomerular filtration rate (GFR) drops to 10 ml/min. However, if the patient has diabetes, dialysis may be recommended when their GFR reaches 15 ml/min. The GFR is a measure of kidney function and indicates how well the kidneys are able to filter waste products from the blood. Dialysis is a medical procedure that helps perform the function of the kidneys by removing waste and excess fluid from the body.

Generally speaking, if a child shows clinical signs of dehydration but does not exhibit shock, it can be assumed that they are 5% dehydrated. On the other hand, if shock is also present, it can be assumed that the child is 10% dehydrated or more. To put it in simpler terms, 5% dehydration means that the body has lost 5 grams of fluid per 100 grams of body weight, which is equivalent to 50 milliliters per kilogram of fluid. Similarly, 10% dehydration implies a loss of 100 milliliters per kilogram of fluid.

The clinical features of dehydration are summarized below:

Dehydration (5%):
– The child appears unwell
– The heart rate may be normal or increased (tachycardia)
– The respiratory rate may be normal or increased (tachypnea)
– Peripheral pulses are normal
– Capillary refill time (CRT) is normal or slightly prolonged
– Blood pressure is normal
– Extremities feel warm
– Decreased urine output
– Reduced skin turgor
– Sunken eyes
– Depressed fontanelle
– Dry mucous membranes

Clinical shock (10%):
– The child appears pale, lethargic, and mottled
– Tachycardia (increased heart rate)
– Tachypnea (increased respiratory rate)
– Weak peripheral pulses
– Prolonged CRT
– Hypotension (low blood pressure)
– Extremities feel cold
– Decreased urine output
– Decreased level of consciousness

Pelvic inflammatory disease (PID) is an infection that affects the upper female reproductive tract, including the uterus, fallopian tubes, and ovaries. It is typically caused by an ascending infection from the cervix. The most common culprits are sexually transmitted diseases like chlamydia and gonorrhea, with chlamydia being the most prevalent infection seen in UK genitourinary medicine clinics.

PID can often be asymptomatic, but when symptoms do occur, they may include lower abdominal pain and tenderness, fever, painful urination, painful intercourse, purulent vaginal discharge, abnormal vaginal bleeding, and tenderness in the cervix and adnexa. It’s important to note that symptoms of ectopic pregnancy can be mistaken for PID, so a pregnancy test should always be conducted in patients with suspicious symptoms.

To investigate a potential case of PID, endocervical swabs are taken to test for chlamydia and gonorrhea using nucleic acid amplification tests. It is recommended to start empirical antibiotic treatment as soon as a presumptive diagnosis of PID is made, without waiting for swab results.

Mild to moderate cases of PID can usually be managed in primary care or outpatient settings. However, patients with severe disease should be admitted to the hospital for intravenous antibiotics. Signs of severe disease include a fever above 38°C, signs of a tubo-ovarian abscess, signs of pelvic peritonitis, or concurrent pregnancy.

The current recommended treatment for outpatient cases of PID is a single intramuscular dose of ceftriaxone 500 mg, followed by oral doxycycline 100 mg twice daily and oral metronidazole 400 mg twice daily for 14 days. An alternative regimen is oral ofloxacin 400 mg twice daily and oral metronidazole 400 mg twice daily for 14 days.

For severely ill patients in the inpatient setting, initial treatment consists of intravenous doxycycline, a single-dose of intravenous ceftriaxone, and intravenous metronidazole. Afterward, the treatment is switched to oral doxycycline and metronidazole to complete a 14-day course.

If a patient fails to respond to treatment, laparoscopy is necessary to confirm the diagnosis or consider alternative diagnoses.

According to the 2011 recommendations from the World Health Organization (WHO), the following criteria are used to diagnose diabetes mellitus:

– A random venous plasma glucose concentration that exceeds 11.1 mmol/l.
– A fasting plasma glucose concentration that is higher than 7.0 mmol/l.
– A two-hour plasma glucose concentration that exceeds 11.1 mmol/l, measured two hours after consuming 75g of anhydrous glucose during an oral glucose tolerance test (OGTT).
– An HbA1c level that is greater than 48 mmol/mol (equivalent to 6.5%).

These guidelines provide specific thresholds for diagnosing diabetes mellitus based on various glucose measurements and HbA1c levels. It is important for healthcare professionals to consider these criteria when evaluating individuals for diabetes mellitus.

Erythema ab igne is a condition that is frequently observed in older individuals. It typically occurs when they spend extended periods of time near a fire or utilize a hot water bottle in an attempt to relieve pain symptoms. This condition arises due to the harmful effects of heat on the skin, resulting in the appearance of reddened and pigmented areas. Fortunately, erythema ab igne tends to resolve on its own without any specific treatment.

Blood transfusion is a crucial medical treatment that can save lives, but it also comes with various risks and potential problems. These include immunological complications, administration errors, infections, and immune dilution. While there have been improvements in safety procedures and a reduction in transfusion usage, errors and adverse reactions still occur.

One serious complication is acute haemolytic transfusion reactions, which happen when incompatible red cells are transfused and react with the patient’s own antibodies. This usually occurs due to human error, such as mislabelling sample tubes or request forms. Symptoms of this reaction include a feeling of impending doom, fever, chills, pain and warmth at the transfusion site, nausea, vomiting, and back, joint, and chest pain. Immediate action should be taken to stop the transfusion, replace the donor blood with normal saline or another suitable crystalloid, and check the blood to confirm the intended recipient. IV diuretics may be administered to increase renal blood flow, and urine output should be maintained.

Another common complication is febrile transfusion reaction, which presents with a 1-degree rise in temperature from baseline, along with chills and malaise. This reaction is usually caused by cytokines from leukocytes in the transfused blood components. Supportive treatment is typically sufficient, and paracetamol can be helpful.

Allergic reactions can also occur, usually due to foreign plasma proteins or anti-IgA. These reactions often present with urticaria, pruritus, and hives, and in severe cases, laryngeal edema or bronchospasm may occur. Symptomatic treatment with antihistamines is usually enough, and there is usually no need to stop the transfusion. However, if anaphylaxis occurs, the transfusion should be stopped, and the patient should be administered adrenaline and treated according to the ALS protocol.

Transfusion-related acute lung injury (TRALI) is a severe complication characterized by non-cardiogenic pulmonary edema within 6 hours of transfusion. It is associated with antibodies in the donor blood reacting with recipient leukocyte antigens. This is the most common cause of death related to transfusion reactions. Treatment involves stopping the transfusion, administering oxygen, and providing aggressive respiratory support in approximately 75% of patients. Diuretic usage should be avoided.

The nerve agents, also known as nerve gases, are a group of highly toxic chemical warfare agents that were initially developed just before and during World War II.

The first compounds to be created are referred to as the G agents (with G representing German, as they were discovered and synthesized by German scientists). These include Tabun (GA), Sarin (GB), and Soman (GD).

In the 1950s, the V agents (with V standing for venomous) were synthesized, and they are approximately 10 times more poisonous than sarin. These include Venomous agent X (VX), Venomous agent E (VE), Venomous agent G (VG), and Venomous agent M (VM).

One of the most well-known incidents involving the use of a nerve agent was the March 1995 Tokyo subway sarin attack. During this attack, Sarin was released into the Tokyo subway system during rush hour. As a result, over 5,000 people sought medical attention. Among them, 984 were moderately poisoned, 54 were severely poisoned, and 12 lost their lives.

The nerve agents are organophosphorus esters that are chemically related to organophosphorus insecticides. They work by inhibiting acetylcholinesterase (AChE), an enzyme that breaks down the neurotransmitter acetylcholine (ACh). This leads to an accumulation of ACh at both muscarinic and nicotinic cholinergic receptors.

Nerve agents can be absorbed through any body surface. When dispersed as a spray or aerosol, they can be absorbed through the skin, eyes, and respiratory tract. When dispersed as a vapor, they are primarily absorbed through the respiratory tract and eyes. If a sufficient amount of agent is absorbed, local effects are followed by generalized systemic effects.

Wernicke’s encephalopathy is a condition that is commonly associated with alcohol abuse and other causes of thiamine deficiency. It is characterized by a triad of symptoms, including acute confusion, ophthalmoplegia (paralysis or weakness of the eye muscles), and ataxia (loss of coordination). Additional features may include papilloedema (swelling of the optic disc), hearing loss, apathy, dysphagia (difficulty swallowing), memory impairment, and hypothermia. Most cases also involve peripheral neuropathy, which typically affects the legs.

The condition is caused by capillary hemorrhages, astrocytosis (abnormal increase in astrocytes, a type of brain cell), and neuronal death in the upper brainstem and diencephalon. These changes can be visualized using MRI scanning, although CT scanning is not very useful for diagnosis.

If left untreated, most patients with Wernicke’s encephalopathy will develop Korsakoff psychosis. This condition is characterized by retrograde amnesia (loss of memory for events that occurred before the onset of amnesia), an inability to form new memories, disordered time perception, and confabulation (fabrication of false memories).

Patients suspected of having Wernicke’s encephalopathy should receive parenteral thiamine (such as Pabrinex) for at least 5 days. Oral thiamine should be administered after the parenteral therapy.

It is important to note that in patients with chronic thiamine deficiency, the infusion of glucose-containing intravenous fluids without thiamine can trigger the development of Wernicke’s encephalopathy.

The most probable diagnosis in this case is a subarachnoid haemorrhage (SAH).

When assessing patients who present with an SAH, there may be focal neurological signs that can indicate the potential location of the aneurysm. Common sites for aneurysms include the bifurcation of the middle cerebral artery, the junction of the anterior communicating cerebral artery, and the junction of the posterior communicating artery with the internal carotid artery. If there is complete or partial palsy of the oculomotor nerve, it suggests the rupture of a posterior communicating artery aneurysm.

While hypertension is a risk factor for SAH, a significant increase in blood pressure may occur as a reflex response following the haemorrhage.

The first-line investigation for SAH is a CT head scan, which can detect over 95% of cases if performed within the first 24 hours. The sensitivity of the scan increases to nearly 100% if done within 6 hours of symptom onset. If the CT head scan is negative and there are no contraindications, a lumbar puncture (LP) should be performed to diagnose SAH. It is recommended to perform the LP at least 12 hours after the onset of headache. It is important to note that approximately 3% of patients with a negative CT scan will be confirmed to have had an SAH after undergoing an LP.

Femoral shaft fractures are quite common among children and have a significant impact on both the child and their family. It is important to carefully examine children with these fractures for any associated injuries, such as soft-tissue injury, head trauma, or additional fractures. In fact, up to 40% of children who experience a femoral shaft fracture due to high-energy trauma may have these associated injuries. Additionally, a thorough neurovascular examination should be conducted.

Rapidly immobilizing the limb is crucial for managing pain and limiting further blood loss from the fracture. For distal femoral shaft fractures, well-padded long leg splints with split plaster casts can be applied. However, for more proximal shaft fractures, long leg splints alone may not provide adequate control. In these cases, skin traction is a better option. Skin traction involves attaching a large foam pad to the patient’s lower leg using spray adhesive. A weight, approximately 10% of the child’s body weight, is then applied to the foam pad and allowed to hang over the foot of the bed. This constant longitudinal traction helps keep the bone fragments aligned.

When children experience severe pain, it is important to manage it aggressively yet safely. Immobilizing the fracture can provide significant relief. The Royal College of Emergency Medicine recommends other pain control measures for children, such as intranasal diamorphine (0.1 mg/kg in 0.2 ml sterile water), intravenous morphine (0.1-0.2 mg/kg), and oral analgesia (e.g., paracetamol 20 mg/kg, max 1 g, and ibuprofen 10 mg/kg, max 400 mg).

This individual has a typical history for a transient ischaemic attack (TIA). According to the NICE recommendations, it is advised to offer aspirin (300 mg daily) to individuals who have experienced a suspected TIA, unless there are contraindications. This treatment should be started immediately. It is also important to refer individuals who have had a suspected TIA for specialist assessment and investigation, with the aim of being seen within 24 hours of symptom onset. Scoring systems, such as ABCD2, should not be used to assess the risk of subsequent stroke or determine the urgency of referral for individuals with a suspected or confirmed TIA. Secondary prevention measures, in addition to aspirin, should be offered as soon as possible after the diagnosis of TIA is confirmed.

In terms of imaging, it is not recommended to offer CT brain scanning to individuals with a suspected TIA, unless there is clinical suspicion of an alternative diagnosis that CT could detect. After a specialist assessment in the TIA clinic, MRI (including diffusion-weighted and blood-sensitive sequences) may be considered to determine the area of ischaemia, detect haemorrhage, or identify alternative pathologies. If an MRI is performed, it should ideally be done on the same day as the assessment. Carotid imaging is also important for everyone with a TIA who is considered a candidate for carotid endarterectomy, and this should be done urgently.

to the NICE guidelines on stroke and transient ischaemic attack in individuals over 16 years old: diagnosis and initial management.

Le Fort fractures are complex fractures of the midface that involve the maxillary bone and surrounding structures. These fractures can occur in a horizontal, pyramidal, or transverse direction. The distinguishing feature of Le Fort fractures is the traumatic separation of the pterygomaxillary region. They make up approximately 10% to 20% of all facial fractures and can have severe consequences, both in terms of potential life-threatening injuries and disfigurement.

The Le Fort classification system categorizes midface fractures into three groups based on the plane of injury. As the classification level increases, the location of the maxillary fracture moves from inferior to superior within the maxilla.

Le Fort I fractures are horizontal fractures that occur across the lower aspect of the maxilla. These fractures cause the teeth to separate from the upper face and extend through the lower nasal septum, the lateral wall of the maxillary sinus, and into the palatine bones and pterygoid plates. They are sometimes referred to as a floating palate because they often result in the mobility of the hard palate from the midface. Common accompanying symptoms include facial swelling, loose teeth, dental fractures, and misalignment of the teeth.

Le Fort II fractures are pyramidal-shaped fractures, with the base of the pyramid located at the level of the teeth and the apex at the nasofrontal suture. The fracture line extends from the nasal bridge and passes through the superior wall of the maxilla, the lacrimal bones, the inferior orbital floor and rim, and the anterior wall of the maxillary sinus. These fractures are sometimes called a floating maxilla because they typically result in the mobility of the maxilla from the midface. Common symptoms include facial swelling, nosebleeds, subconjunctival hemorrhage, cerebrospinal fluid leakage from the nose, and widening and flattening of the nasal bridge.

Le Fort III fractures are transverse fractures of the midface. The fracture line passes through the nasofrontal suture, the maxillo frontal suture, the orbital wall, and the zygomatic arch and zygomaticofrontal suture. These fractures cause separation of all facial bones from the cranial base, earning them the nickname craniofacial disjunction or floating face fractures. They are the rarest and most severe type of Le Fort fracture. Common symptoms include significant facial swelling, bruising around the eyes, facial flattening, and the entire face can be shifted.

Intussusception occurs when a section of the bowel folds into another section, causing a blockage. This can be due to a specific underlying issue, like a Meckel’s diverticulum, or it can happen without any specific cause. The condition is most commonly seen in boys between the ages of 5 and 10 months. Symptoms include sudden vomiting and episodes of abdominal pain that come and go. The vomit quickly becomes greenish-yellow in color. Dance’s sign, which is the absence of bowel in the lower right part of the abdomen, may be observed. Redcurrant jelly-like stools are a late indication of the condition. It is believed that more than 90% of cases are caused by a non-specific underlying issue, often viral infections like rotavirus, adenovirus, and human herpesvirus 6.

When performing CPR on patients with a core temperature of 30-35°C, it is recommended to double the interval between IV drug doses compared to what is used for normothermic patients. However, if the core temperature is above 35°C, standard drug protocols should be followed.

Further Reading:

Cardiopulmonary arrest is a serious event with low survival rates. In non-traumatic cardiac arrest, only about 20% of patients who arrest as an in-patient survive to hospital discharge, while the survival rate for out-of-hospital cardiac arrest is approximately 8%. The Resus Council BLS/AED Algorithm for 2015 recommends chest compressions at a rate of 100-120 per minute with a compression depth of 5-6 cm. The ratio of chest compressions to rescue breaths is 30:2.

After a cardiac arrest, the goal of patient care is to minimize the impact of post cardiac arrest syndrome, which includes brain injury, myocardial dysfunction, the ischaemic/reperfusion response, and the underlying pathology that caused the arrest. The ABCDE approach is used for clinical assessment and general management. Intubation may be necessary if the airway cannot be maintained by simple measures or if it is immediately threatened. Controlled ventilation is aimed at maintaining oxygen saturation levels between 94-98% and normocarbia. Fluid status may be difficult to judge, but a target mean arterial pressure (MAP) between 65 and 100 mmHg is recommended. Inotropes may be administered to maintain blood pressure. Sedation should be adequate to gain control of ventilation, and short-acting sedating agents like propofol are preferred. Blood glucose levels should be maintained below 8 mmol/l. Pyrexia should be avoided, and there is some evidence for controlled mild hypothermia but no consensus on this.

Post ROSC investigations may include a chest X-ray, ECG monitoring, serial potassium and lactate measurements, and other imaging modalities like ultrasonography, echocardiography, CTPA, and CT head, depending on availability and skills in the local department. Treatment should be directed towards the underlying cause, and PCI or thrombolysis may be considered for acute coronary syndrome or suspected pulmonary embolism, respectively.

Patients who are comatose after ROSC without significant pre-arrest comorbidities should be transferred to the ICU for supportive care. Neurological outcome at 72 hours is the best prognostic indicator of outcome.

Boyle’s law states that when the temperature remains constant, the volume of a gas is inversely related to its pressure. This means that as the pressure of a gas increases, its volume decreases, and vice versa. Mathematically, this relationship can be expressed as P1V1 = P2V2.

Further Reading:

Decompression illness (DCI) is a term that encompasses both decompression sickness (DCS) and arterial gas embolism (AGE). When diving underwater, the increasing pressure causes gases to become more soluble and reduces the size of gas bubbles. As a diver ascends, nitrogen can come out of solution and form gas bubbles, leading to decompression sickness or the bends. Boyle’s and Henry’s gas laws help explain the changes in gases during changing pressure.

Henry’s law states that the amount of gas that dissolves in a liquid is proportional to the partial pressure of the gas. Divers often use atmospheres (ATM) as a measure of pressure, with 1 ATM being the pressure at sea level. Boyle’s law states that the volume of gas is inversely proportional to the pressure. As pressure increases, volume decreases.

Decompression sickness occurs when nitrogen comes out of solution as a diver ascends. The evolved gas can physically damage tissue by stretching or tearing it as bubbles expand, or by provoking an inflammatory response. Joints and spinal nervous tissue are commonly affected. Symptoms of primary damage usually appear immediately or soon after a dive, while secondary damage may present hours or days later.

Arterial gas embolism occurs when nitrogen bubbles escape into the arterial circulation and cause distal ischemia. The consequences depend on where the embolism lodges, ranging from tissue ischemia to stroke if it lodges in the cerebral arterial circulation. Mechanisms for distal embolism include pulmonary barotrauma, right to left shunt, and pulmonary filter overload.

Clinical features of decompression illness vary, but symptoms often appear within six hours of a dive. These can include joint pain, neurological symptoms, chest pain or breathing difficulties, rash, vestibular problems, and constitutional symptoms. Factors that increase the risk of DCI include diving at greater depth, longer duration, multiple dives close together, problems with ascent, closed rebreather circuits, flying shortly after diving, exercise shortly after diving, dehydration, and alcohol use.

Diagnosis of DCI is clinical, and investigations depend on the presentation. All patients should receive high flow oxygen, and a low threshold for ordering a chest X-ray should be maintained. Hydration is important, and IV fluids may be necessary. Definitive treatment is recompression therapy in a hyperbaric oxygen chamber, which should be arranged as soon as possible. Entonox should not be given, as it will increase the pressure effect in air spaces.

There are various specific remedies available for different types of poisons and overdoses. The following list provides an outline of some of these antidotes:

Poison: Benzodiazepines
Antidote: Flumazenil

Poison: Beta-blockers
Antidotes: Atropine, Glucagon, Insulin

Poison: Carbon monoxide
Antidote: Oxygen

Poison: Cyanide
Antidotes: Hydroxocobalamin, Sodium nitrite, Sodium thiosulphate

Poison: Ethylene glycol
Antidotes: Ethanol, Fomepizole

Poison: Heparin
Antidote: Protamine sulphate

Poison: Iron salts
Antidote: Desferrioxamine

Poison: Isoniazid
Antidote: Pyridoxine

Poison: Methanol
Antidotes: Ethanol, Fomepizole

Poison: Opioids
Antidote: Naloxone

Poison: Organophosphates
Antidotes: Atropine, Pralidoxime

Poison: Paracetamol
Antidotes: Acetylcysteine, Methionine

Poison: Sulphonylureas
Antidotes: Glucose, Octreotide

Poison: Thallium
Antidote: Prussian blue

Poison: Warfarin
Antidote: Vitamin K, Fresh frozen plasma (FFP)

By utilizing these specific antidotes, medical professionals can effectively counteract the harmful effects of various poisons and overdoses.

ATLS guidelines now suggest administering only 1 liter of crystalloid fluid during the initial assessment. If patients do not respond to the crystalloid, it is recommended to quickly transition to blood products. Studies have shown that infusing more than 1.5 liters of crystalloid fluid is associated with higher mortality rates in trauma cases. Therefore, it is advised to prioritize the early use of blood products and avoid large volumes of crystalloid fluid in trauma patients. In cases where it is necessary, massive transfusion should be considered, defined as the transfusion of more than 10 units of blood in 24 hours or more than 4 units of blood in one hour. For patients with evidence of Class III and IV hemorrhage, early resuscitation with blood and blood products in low ratios is recommended.

Based on the findings of significant trials, such as the CRASH-2 study, the use of tranexamic acid is now recommended within 3 hours. This involves administering a loading dose of 1 gram intravenously over 10 minutes, followed by an infusion of 1 gram over eight hours. In some regions, tranexamic acid is also being utilized in the prehospital setting.

Respiratory alkalosis can be caused by hyperventilation, such as during periods of anxiety. It can also be a result of conditions like pulmonary embolism, CNS disorders (such as stroke or encephalitis), altitude, pregnancy, or the early stages of aspirin overdose.

Respiratory acidosis is often associated with chronic obstructive pulmonary disease (COPD) or life-threatening asthma. Other causes include pulmonary edema, sedative drug overdose (such as opiates or benzodiazepines), neuromuscular disease, obesity, or certain medications.

Metabolic alkalosis can occur due to vomiting, potassium depletion (often caused by diuretic usage), Cushing’s syndrome, or Conn’s syndrome.

Metabolic acidosis with a raised anion gap can be caused by conditions like lactic acidosis (which can result from hypoxemia, shock, sepsis, or infarction) or ketoacidosis (commonly seen in diabetes, starvation, or alcohol excess). Other causes include renal failure or poisoning (such as late stages of aspirin overdose, methanol, or ethylene glycol).

Metabolic acidosis with a normal anion gap can be attributed to conditions like renal tubular acidosis, diarrhea, ammonium chloride ingestion, or adrenal insufficiency.

Patients who are being treated with insulin infusion for diabetic ketoacidosis (DKA) should expect their plasma glucose levels to decrease by at least 3 mmol/L per hour. The purpose of the insulin infusion is to correct both hyperglycemia and ketoacidosis. It is important to regularly review and check the insulin infusion to ensure it is working effectively. If any of the following are observed, the infusion rate should be adjusted accordingly: capillary ketones are not decreasing by at least 0.5 mmol/L per hour, venous bicarbonate is not increasing by at least 3 mmol/L per hour, or plasma glucose is not decreasing by at least 3 mmol/L per hour.

Further Reading:

Diabetic ketoacidosis (DKA) is a serious complication of diabetes that occurs due to a lack of insulin in the body. It is most commonly seen in individuals with type 1 diabetes but can also occur in type 2 diabetes. DKA is characterized by hyperglycemia, acidosis, and ketonaemia.

The pathophysiology of DKA involves insulin deficiency, which leads to increased glucose production and decreased glucose uptake by cells. This results in hyperglycemia and osmotic diuresis, leading to dehydration. Insulin deficiency also leads to increased lipolysis and the production of ketone bodies, which are acidic. The body attempts to buffer the pH change through metabolic and respiratory compensation, resulting in metabolic acidosis.

DKA can be precipitated by factors such as infection, physiological stress, non-compliance with insulin therapy, acute medical conditions, and certain medications. The clinical features of DKA include polydipsia, polyuria, signs of dehydration, ketotic breath smell, tachypnea, confusion, headache, nausea, vomiting, lethargy, and abdominal pain.

The diagnosis of DKA is based on the presence of ketonaemia or ketonuria, blood glucose levels above 11 mmol/L or known diabetes mellitus, and a blood pH below 7.3 or bicarbonate levels below 15 mmol/L. Initial investigations include blood gas analysis, urine dipstick for glucose and ketones, blood glucose measurement, and electrolyte levels.

Management of DKA involves fluid replacement, electrolyte correction, insulin therapy, and treatment of any underlying cause. Fluid replacement is typically done with isotonic saline, and potassium may need to be added depending on the patient’s levels. Insulin therapy is initiated with an intravenous infusion, and the rate is adjusted based on blood glucose levels. Monitoring of blood glucose, ketones, bicarbonate, and electrolytes is essential, and the insulin infusion is discontinued once ketones are below 0.3 mmol/L, pH is above 7.3, and bicarbonate is above 18 mmol/L.

Complications of DKA and its treatment include gastric stasis, thromboembolism, electrolyte disturbances, cerebral edema, hypoglycemia, acute respiratory distress syndrome, and acute kidney injury. Prompt medical intervention is crucial in managing DKA to prevent potentially fatal outcomes.

Vascular dementia is the second most common form of dementia, accounting for approximately 25% of all cases. It occurs when the brain is damaged due to various factors, such as major strokes, multiple smaller strokes that go unnoticed (known as multi-infarct), or chronic changes in smaller blood vessels (referred to as subcortical dementia). The term vascular cognitive impairment (VCI) is increasingly used to encompass this range of diseases.

Unlike Alzheimer’s disease, which has a gradual and subtle onset, vascular dementia can occur suddenly and typically shows a series of stepwise increases in symptom severity. The presentation and progression of the disease can vary significantly.

There are certain features that suggest a vascular cause of dementia. These include a history of transient ischemic attacks (TIAs) or cardiovascular disease, the presence of focal neurological abnormalities, prominent memory impairment in the early stages of the disease, early onset of gait disturbance and unsteadiness, frequent unprovoked falls in the early stages, bladder symptoms (such as incontinence) without any identifiable urological condition in the early stages, and seizures.

For the treatment of pregnant women with lower urinary tract infections (UTIs), it is recommended to provide them with an immediate prescription for antibiotics. It is important to consider their previous urine culture and susceptibility results, as well as any prior use of antibiotics that may have contributed to the development of resistant bacteria. Before starting antibiotics, it is advised to obtain a midstream urine sample from pregnant women and send it for culture and susceptibility testing.

Once the microbiological results are available, it is necessary to review the choice of antibiotic. If the bacteria are found to be resistant, it is recommended to switch to a narrow-spectrum antibiotic whenever possible. The choice of antibiotics for pregnant women aged 12 years and over is summarized below:

First-choice:
– Nitrofurantoin 100 mg modified-release taken orally twice daily for 3 days, if the estimated glomerular filtration rate (eGFR) is above 45 ml/minute.

Second-choice (if there is no improvement in lower UTI symptoms with the first-choice antibiotic for at least 48 hours, or if the first-choice is not suitable):
– Amoxicillin 500 mg taken orally three times daily for 7 days (only if culture results are available and show susceptibility).
– Cefalexin 500 mg taken twice daily for 7 days.

For alternative second-choice antibiotics, it is recommended to consult a local microbiologist and choose the appropriate antibiotics based on the culture and sensitivity results.

A central venous catheter (CVC) is a type of catheter that is inserted into a large vein in the body, typically in the neck, chest, or groin. It has several important uses, including CVP monitoring, pulmonary artery pressure monitoring, repeated blood sampling, IV access for large volumes of fluids or drugs, TPN administration, dialysis, pacing, and other procedures such as placement of IVC filters or venous stents.

When inserting a central line, it is ideal to use ultrasound guidance to ensure accurate placement. However, there are certain contraindications to central line insertion, including infection or injury to the planned access site, coagulopathy, thrombosis or stenosis of the intended vein, a combative patient, or raised intracranial pressure for jugular venous lines.

The most common approaches for central line insertion are the internal jugular, subclavian, femoral, and PICC (peripherally inserted central catheter) veins. The internal jugular vein is often chosen due to its proximity to the carotid artery, but variations in anatomy can occur. Ultrasound can be used to identify the vessels and guide catheter placement, with the IJV typically lying superficial and lateral to the carotid artery. Compression and Valsalva maneuvers can help distinguish between arterial and venous structures, and doppler color flow can highlight the direction of flow.

In terms of choosing a side for central line insertion, the right side is usually preferred to avoid the risk of injury to the thoracic duct and potential chylothorax. However, the left side can also be used depending on the clinical situation.

Femoral central lines are another option for central venous access, with the catheter being inserted into the femoral vein in the groin. Local anesthesia is typically used to establish a field block, with lidocaine being the most commonly used agent. Lidocaine works by blocking sodium channels and preventing the propagation of action potentials.

In summary, central venous catheters have various important uses and should ideally be inserted using ultrasound guidance. There are contraindications to their insertion, and different approaches can be used depending on the clinical situation. Local anesthesia is commonly used for central line insertion, with lidocaine being the preferred agent.

Harmful drinking is when a person consumes at least 35 units of alcohol per week if they are a woman, or at least 50 units per week if they are a man. This level of drinking can lead to negative consequences for their mental and physical health.

Hazardous drinking, also known as increasing risk drinking, refers to a pattern of alcohol consumption that raises the likelihood of harm. For women, this means drinking more than 14 units but less than 35 units per week, while for men it means drinking more than 14 units but less than 50 units per week.

High-risk drinking, or harmful drinking, is a pattern of alcohol consumption that causes mental or physical damage. This occurs when a woman drinks 35 units or more per week, or when a man drinks 50 units or more per week.

Further Reading:

Alcoholic liver disease (ALD) is a spectrum of disease that ranges from fatty liver at one end to alcoholic cirrhosis at the other. Fatty liver is generally benign and reversible with alcohol abstinence, while alcoholic cirrhosis is a more advanced and irreversible form of the disease. Alcoholic hepatitis, which involves inflammation of the liver, can lead to the development of fibrotic tissue and cirrhosis.

Several factors can increase the risk of progression of ALD, including female sex, genetics, advanced age, induction of liver enzymes by drugs, and co-existent viral hepatitis, especially hepatitis C.

The development of ALD is multifactorial and involves the metabolism of alcohol in the liver. Alcohol is metabolized to acetaldehyde and then acetate, which can result in the production of damaging reactive oxygen species. Genetic polymorphisms and co-existing hepatitis C infection can enhance the pathological effects of alcohol metabolism.

Patients with ALD may be asymptomatic or present with non-specific symptoms such as abdominal discomfort, vomiting, or anxiety. Those with alcoholic hepatitis may have fever, anorexia, and deranged liver function tests. Advanced liver disease can manifest with signs of portal hypertension and cirrhosis, such as ascites, varices, jaundice, and encephalopathy.

Screening tools such as the AUDIT questionnaire can be used to assess alcohol consumption and identify hazardous or harmful drinking patterns. Liver function tests, FBC, and imaging studies such as ultrasound or liver biopsy may be performed to evaluate liver damage.

Management of ALD involves providing advice on reducing alcohol intake, administering thiamine to prevent Wernicke’s encephalopathy, and addressing withdrawal symptoms with benzodiazepines. Complications of ALD, such as intoxication, encephalopathy, variceal bleeding, ascites, hypoglycemia, and coagulopathy, require specialized interventions.

Heavy alcohol use can also lead to thiamine deficiency and the development of Wernicke Korsakoff’s syndrome, characterized by confusion, ataxia, hypothermia, hypotension, nystagmus, and vomiting. Prompt treatment is necessary to prevent progression to Korsakoff’s psychosis.

In summary, alcoholic liver disease is a spectrum of disease that can range from benign fatty liver to irreversible cirrhosis. Risk factors for progression include female sex, genetics, advanced age, drug-induced liver enzyme induction, and co-existing liver conditions.

If a case is not deemed urgent, it is necessary to inform the appropriate officer within a period of three days. However, if the case is suspected to be urgent, it is crucial to verbally notify the proper officer within a timeframe of 24 hours.