Erythema nodosum (EN) is an acute, nodular, erythematous eruption that usually is limited to the extensor aspects of the lower legs. It is a delayed hypersensitivity reaction to a variety of antigens| circulating immune complexes have not been found in idiopathic or uncomplicated cases but may be demonstrated in patients with inflammatory bowel disease.Currently, the most common cause of erythema nodosum is a streptococcal infection in children and streptococcal infection and sarcoidosis in adultsIn most patients, erythema nodosum is a self-limited disease and requires only symptomatic relief using nonsteroidal anti-inflammatory drugs (NSAIDs), cool wet compresses, elevation, and bed rest.

Answer: The burn area is extremely painful until skin graftedThird-degree burns destroy the epidermis and dermis. Third-degree burns may also damage the underlying bones, muscles, and tendons. The burn site appears white or charred. There is no sensation in the area since the nerve endings are destroyed. These are not normally painful until after skin grafting is done since the nerve endings have been destroyed.

In haemophilia C, The severity of the deficiency is based on plasma factor XIC (clotting) activity. Major factor XI deficiency is present when the activity of factor XI in plasma is less than 1-15 IU/dL.In major deficiency factor XI, bleeding is related to injury, especially when trauma involves tissues rich in fibrinolytic activators, such as the oral mucosa, the nose, and the urinary tract. Unlike patients with severe haemophilia A or B, patients with major factor XI deficiency do not spontaneously bleed.The aPTT is usually prolonged in factor XI deficiency (but depends on the sensitivity of the reagent and test system–partial deficiency can be missed), whereas the PT and TT are normal.

Undescended testis (UDT) is a common abnormality, affecting about 1/20 males at birth. Half of these have delayed testicular descent, with the testis in the scrotum by 10-12 weeks after term. Beyond this spontaneous descent is rare. Current treatment recommendations are that UDT beyond 3 months need surgery between 6-12 months of age. Some children have scrotal testes in infancy but develop UDT later in childhood because the spermatic cord does not elongate with age, leaving the testis behind as the scrotum moves further from the groin. The maldescended testis suffers heat stress when not at the lower scrotal temperature (33 degrees Celsius), interfering with testicular physiology and development of germ cells into spermatogonia. Recent evidence suggests orchidopexy between 6-12 months improves germ cell development, with early reports of improved fertility, but no evidence yet for changes in malignancy prognosis.Hypospadias is also a common abnormality in new-born males, affecting about 1/150 boys. Androgens control masculinization of the genital tubercle into penis between 8-12 weeks’ gestation, with tabularization of the urethra from the perineum to the tip of the glans. If this process is disrupted hypospadias occurs, with a variable proximal urethral meatus, failed ventral preputial development producing a dorsal hood, and discrepancy in the ventral versus dorsal penile length, causing a ventral bend in the penis, known as chordee. Surgery to correct hypospadias is recommended between 6-18 months.

The most probable cause for the child’s presenting symptoms and the findings in ultrasound would be the presence of an abnormal posterior urethral valve. A posterior urethral valve is a developmental anomaly that usually affects male infants (incidence 1 in 8000) leading to obstructive uropathy. Diagnostic features include bladder wall hypertrophy, hydronephrosis and bladder diverticula.Note:Posterior urethral valves are the most common cause of infra-vesical outflow obstruction in males. They can be diagnosed on antenatal ultrasonography. Due to the necessity of the fetal bladder to develop high emptying pressures in utero secondary to this anomaly, the child may develop renal parenchymal damage. This leads to renal impairment noted in 70% of boys at the time of presentation. Management:The immediate treatment would be to place a bladder catheter to relieve the acutely retained urine. The definitive treatment of choice would be an endoscopic valvotomy with a cystoscopic and renal follow up.

Injuries to the peroneal nerve can cause numbness, tingling, pain, weakness and foot drop. The branches of the common peroneal nerve innervate and control the muscles in the legs that lift the ankle and toes upward (dorsi flexion).

The clinical presentation of the boy suggests the involvement of the right frontal lobe.Psychiatric or behavioural disturbances secondary to frontal lobe lesions exhibit lateralisation. The lesions of the left hemisphere are associated with depression, especially if the lesion involves the dorsolateral portion of the prefrontal cortex. Whereas, lesions of the right hemisphere are associated with impulsivity, disinhibition, and aggression, as it is the case with the child in question.Presenting symptoms of lobar dysfunctions:- Frontal lobe: Contralateral hemiplegia, impaired problem solving, disinhibition, and lack of initiative. Broca’s aphasia and agraphia suggest the involvement of the dominant hemisphere.- Temporal lobe: Wernicke’s aphasia (dominant hemisphere involvement), homonymous upper quadrantanopia and auditory agnosia (non-dominant hemisphere involvement).- Parietal lobe: Anosognosia, dressing apraxia, spatial neglect and constructional apraxia are observed when the non-dominant hemisphere is involved. Gerstmann’s syndrome is observed when the dominant hemisphere is involved.- Occipital lobe: Visual agnosia, visual illusions and contralateral homonymous hemianopia.

Topical benzoyl peroxide is used for the treatment of mild to moderate acne vulgaris. It is actually a peeling agent and it clears the pores and reduces the bacterial cell count.

The average amount of energy required by a well preterm baby in a thermo-neutral environment is 50/kcal/kg to maintain essential body functions while it takes about 70 kcal/kg/day for optimal growth. It is important to ensure that enough proteins are given per calorie to limit catabolism and promote cell building. Caloric need usually increases when the baby is ill.

Gilbert’s syndrome is an autosomal recessive condition that results from defective bilirubin conjugation due to a deficiency of UDP glucuronyl transferase – 1. The prevalence is approximately 1-2% in the general population.The clinical presentation of Gilbert’s syndrome include:- Unconjugated hyperbilinaemia- Jaundice can occur during an intercurrent illnessManagement:Blood investigations usually reveal a rise in bilirubin following prolonged fasting or intravenous nicotinic acid.No treatment required for these patients.Other options:- CYP2C9 deficiency causes reduced warfarin metabolism, and subsequent enhanced drug effects. – Pancreatic disease, cholestasis, excessive alcohol consumption and certain drugs are common causes of raised GGT levels- Defective hepatocyte excretion of conjugated bilirubin is related to the pathophysiology of Dubin-Johnson syndrome- Reduced CYP2C19 levels can lead to the disordered metabolism of clopidogrel and other drugs such as proton-pump inhibitors, anticonvulsants and sedatives.

Unconjugated bilirubin is conjugated to glucuronic acid in the hepatocyte. Conjugated bilirubin passes into the enterohepatic circulation and the bilirubin which evades this system is metabolised by bacteria, primarily in the large intestine, to urobilinogen, then stercobilinogen and eventually oxidised to stercobilin. Stercobilin gives faeces its brown colour.

Among the provided options, we would expect a normally developing 18-month-old child to be able to carry toys while walking.At 18 months, a normally developing child will show the following gross motor abilities:- walks with the feet slightly apart- runs carefully with the head held high- pushes and pulls objects around the floor- walks upstairs if the hand is held- kneels on a flat surface without support- carry toys while walking.Other options:- Intentionally kicking a ball is a skill typically acquired by 2.5 years, not 18 months.- Jumping with two feet together from a small step is usually acquired by 2.5 years, not 18 months.- Propelling a tricycle forwards by pushing with the feet on the floor is a milestone generally acquired by 2 years, not 18 months.- Throwing a small ball overhand without falling is a milestone for children aged 2 years, not 18 months.

Chédiak–Higashi syndrome is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

The sensitivity of a screening test can be described in a variety of ways, typically such as sensitivity being the ability of a screening test to detect a true positive, being based on the true positive rate, reflecting a test’s ability to correctly identify all people who have a condition, or, if 100%, identifying all people with a condition of interest by those people testing positive on the test.The specificity of a test is defined in a variety of ways, typically such as specificity is the ability of a screening test to detect a true negative, being based on the true negative rate, correctly identifying people who do not have a condition, or, if 100%, identifying all patients who do not have the condition of interest by those people testing negative on the test.

Allergy: A history of a prior allergic-like reaction to contrast media is associated with an up to a 5-fold increase in the likelihood of experiencing a subsequent reaction. Also, any patient with a predilection to allergic reactions may predispose them to a reaction after the administration of contrast media. Given the increased risk of severe life-threatening anaphylaxis related to the administration of contrast media in the setting of the history of atopy, the risk versus benefits should be discussed before following through with the procedure. A premedication regimen may be used to reduce the risk of anaphylaxis.Asthma: A history of asthma may be indicative of a higher likelihood of developing a contrast reaction. Cardiac status: Attention must be turned to patients with significant cardiac disease (congestive heart failure, aortic stenosis, severe cardiomyopathy, and/or pulmonary hypertension), as higher volumes and osmolality of contrast material may result in an increased risk for a contrast reaction.Renal insufficiency: Contrast nephrotoxicity is defined as the rapid deterioration of renal function after the administration of contrast media when no other aetiology can be determined from the clinical records. The major predisposing risk factors include pre-existing renal insufficiency (defined as serum creatinine level >1.5 mg/dL) and diabetes. Other risk factors include dehydration, cardiovascular disease, the use of diuretics, advanced age (>70 years old), hypertension, and hyperuricemia. Obtaining multiple contrast-enhanced studies within 24 hours is also thought to increase the risk for contrast-induced nephrotoxicity. Miscellaneous: Relative contraindications to the use of high osmolality iodinated contrast media (HOCM) in patients with pheochromocytoma, sickle cell disease, and multiple myeloma have been reported. Although the administration of low osmolality or iso-osmotic contrast media may be beneficial in patients with pheochromocytoma and sickle cell disease, little evidence suggests that these agents mitigate the risks associated with multiple myeloma.

History is suggestive of a possible hearing impairment. As the baby is vocalizing and has normal motor development, an isolated hearing problem is possible. A hearing test will confirm the diagnosis.

Certain CHDs may present with a differential cyanosis, in which the preductal part of the body (upper part of the body) is pinkish but the post ductal part of the body (lower part of the body) is cyanotic, or vice versa (reverse differential cyanosis). The prerequisite for this unique situation is the presence of a right-to-left shunt through the PDA and severe coarctation of the aorta or aortic arch interruption or severe pulmonary hypertension. In patients with severe coarctation of the aorta or interruption of the aortic arch with normally related great arteries, the preductal part of the body is supplied by highly oxygenated pulmonary venous blood via the LA and LV, whereas the post ductal part is supplied by deoxygenated systemic venous blood via the RA, RV, main pulmonary artery (MPA) and the PDA. In the new-born with structurally normal heart, a differential cyanosis may be associated with persistent pulmonary hypertension of the new-born. In the cases of TGA with coarctation of the aorta or aortic arch interruption, the upper body is mostly supplied by systemic venous blood via the RA, RV, and ascending aorta, whereas the lower body is supplied by highly oxygenated pulmonary venous blood via the LA, LV, MPA, and then the PDA. For accurate detection of differential cyanosis, oxygen saturation should be measured in both preductal (right finger) and post ductal (feet) parts of the body.

Precocious puberty is the development of secondary sexual characteristics before the age of 8 in females, and 9 in males. The first sign of early puberty in females is breast enlargement, followed by the appearance of pubic and axillary hair, and finally menarche, 2-3 years after the onset of thelarche. Precocious puberty is caused by the premature activation of the hypothalamic-pituitary-gonadal axis where FSH and LH levels are raised. Less common is the gonadotrophin independent form, which is due to excess sex hormones, but low FSH and LH levels.

Given the presentation of short stature, primary amenorrhea, puffy arms, and a webbed neck in a 5-year-old girl, the most likely diagnosis is: Turner’s syndrome

Reasoning:

• Short Stature: Common in Turner syndrome.
• Primary Amenorrhea: Typical in Turner syndrome due to ovarian dysgenesis.
• Puffy Arms: Lymphedema is common in Turner syndrome, particularly in infancy.
• Webbed Neck: A classic feature of Turner syndrome.

Other Conditions:

• Down’s Syndrome: Typically presents with intellectual disability, characteristic facial features, and sometimes congenital heart defects, but not usually with primary amenorrhea or a webbed neck.
• Klinefelter’s Syndrome: Affects males and presents with hypogonadism and infertility, not applicable to a 5-year-old girl.
• Fragile X Syndrome: Primarily presents with intellectual disability and physical features such as an elongated face and large ears, not with a webbed neck or amenorrhea.

PDA is particularly common in premature babies and it is managed by indomethacin administration. However, if PDA is not the only defect, prostaglandin E1 can be administered in order to keep the ductus open until the surgery takes place.

Ideally, the test is done in the first 4 to 5 weeks, but it can be done at up to 3 months of age.The identification of all new-borns with hearing loss before age 6 months has now become an attainable realistic goal. In the past, parents and paediatricians often did not suspect a hearing loss until the age of 2-3 years, after important speech and language milestones have not been met. By the time these milestones are missed, the hearing-impaired child has already experienced irreversible delays in speech and language development.

Children are able to use a palmar grasp by the age of 6 months and use a pincer grasp by the age of 9 to 10 months. They can sit with support by 6 months and sit without support by 7 to 9 months. They can vocalize by 3 months. Stranger fear appears from 7 to 10 months. So this baby’s development is normal.

Breslow thickness is measured from the top of the granular layer of the epidermis (or, if the surface is ulcerated, from the base of the ulcer) to the deepest invasive cell across the broad base of the tumour (dermal/subcutaneous) as described by Breslow.Margins of excision-Related to Breslow thicknessLesions 0-1mm thick – 1cmLesions 1-2mm thick – 1- 2cm (Depending upon site and pathological features)Lesions 2-4mm thick – 2-3 cm (Depending upon site and pathological features)Lesions >4 mm thick – 3cmMarsden J et al Revised UK guidelines for management of Melanoma. Br J Dermatol 2010 163:238-256.

Randomization is one of the ethical protocols for research work, involving unbiased sample selection. Consent is required before undertaking any research and is taken for the complete research work at once. No separate consent is required for the process of randomization. The consent should explain each and every aspect of the study, including details about the process of randomization. Only particular study designs, like Zelen design, permit the avoidance of consent to randomization but these studies have their setbacks.

Immunity against mumps develops over a long time. There is nothing to be done except to proceed with the usual vaccination schedule.

The most probable diagnosis for this patient is Maple syrup urine disease (MSUD).Note:- Healthy new-borns have blood sugars between 3.3 and 5 mmol/L- Neonatal hypoglycaemia is glucose < 2.2 mmol/L if measured in the first 3 days of life.- Neonatal hypoglycaemia is glucose < 2.5 mmol/L after the first 3 days of life.Causes of persistent hypoglycaemia include:- Preterm- IUGR- SGA- Hyperinsulinism due to maternal diabetes- Beckwith-Wiedemann syndrome- Hypoxia at birth- Sepsis- Cardiopulmonary disease- Inborn errors of metabolism- Hepatic enzyme deficiencies- Glycogen storage disease

To get more information regarding hearing loss, a physician should perform both Rinne and Weber tests. Weber test alone cannot tell which side is affected.Rinne test – a vibrating tuning fork (typically 512 Hz) is placed initially on the mastoid process behind each ear until sound is no longer heard. Then, without re-striking the fork, the fork is then quickly placed just outside the ear with the patient asked to report when the sound caused by the vibration is no longer heard. A normal or positive Rinne test is when sound is still heard when the tuning fork is moved to air near the ear (air conduction or AC), indicating that AC is equal or greater than (bone conduction or BC).Weber’s test – tuning fork is placed in the middle of the forehead equidistant from the patient’s ears. The patient is then asked which side is loudest. In unilateral sensorineural deafness, sound is localised to the unaffected side however in unilateral conductive deafness, sound is localised to the affected side.

Urine culture and sensitivity is used to diagnose a urinary tract infection (UTI). A mid-stream clean catch urine sample is the most common type of sample collected. It is important to follow the clean catch process to have accurate results from an uncontaminated sample. Urine cultures can also check for infections of the bladder or kidney.

Diazepam given rectally may be helpful in treating prolonged convulsions outside and within hospital when intravenous injection is not possible. A rectal dose of 0.5 mg/kg (maximum 10 mg) of injectable diazepam, undiluted or diluted with a 50% propylene glycol solution, can stop seizures in up to 80% of children. There are few adverse reactions. Seizures in children usually cease spontaneously within 5-10 minutes and are rarely associated with significant sequelae. The chance of a seizure stopping spontaneously decreases significantly after 10-15 minutes. Similarly, the efficacy of anticonvulsant medication decreases after 10-15 minutes of fitting and the risk of adverse effects increases. Convulsive seizures lasting longer than 30 minutes constitute status epilepticus and may be complicated by cardio respiratory depression and brain injury. Diazepam or clonazepam, given intravenously, is generally the drug of choice for the emergency treatment of convulsive status epilepticus. Intravenous diazepam may be difficult to administer to the young convulsing child and, because of the need for intravenous access, is not ideal for rapid treatment in the community by non-medical carers.

A surgeon will observe the patient and will decide which procedure he needs.