Puberty is when a child’s body begins to develop and change as they become an adult.The average age for girls to begin puberty is 11, while for boys the average age is 12.It’s completely normal for puberty to begin at any point from the ages of 8 to 14. The process can take up to 4 years.

Neonatal hyperbilirubinemia is a common clinical condition and can be categorized as conjugated and unconjugated hyperbilirubinemia. Conjugated hyperbilirubinemia results from obstructive or hepatocellular causes mainly. The causes of conjugated hyperbilirubinemia include inborn errors of metabolism like galactosemia and aminoaciduria, obstruction from choledochal cysts, and biliary atresia. Long term TPN also leads to conjugated hyperbilirubinemia. Spherocytosis is a hereditary condition belonging to the group of haemolytic anaemias, resulting from plasma membrane protein deficiency. This defect of the RBC plasma membrane decreases their life span, making them osmotically fragile and prone to haemolysis. This leads to an increase in the unconjugated bilirubin levels, with a risk of developing kernicterus.

Group B beta-haemolytic Streptococcus (GBS) and Escherichia coli are the most common cause of early sepsis. Group B Streptococcus (GBS), also known as Streptococcus agalactiae, is best known as a cause of postpartum infection and as the most common cause of neonatal sepsis. The GBS organism colonizes the vagina, GI tract, and the upper respiratory tract of healthy humans. GBS infection is almost always associated with underlying abnormalities. In elderly persons aged 70 years or older, GBS infection is strongly linked with congestive heart failure and being bedridden.Penicillin remains the drug of choice for group B streptococcal infection but resistance may occur.

Polycythaemia is described as an abnormal increase in the red cell mass. As this influences hyper-viscosity, a peripheral venous sample of blood haematocrit can be used to determine the packed cell volume. Polycythaemia is present if the venous haematocrit is >65% or <22g/dl if converted into a haemoglobin value. Though it is the method of choice for screening, capillary blood samples obtained though heel pricks in new-born may be as much as 15% higher than venous samples and thus high values must be confirmed with a venous sample.

Answer: Curling’s ulcersCurling’s ulcer is an acute gastric erosion resulting as a complication from severe burns when reduced plasma volume leads to ischemia and cell necrosis (sloughing) of the gastric mucosa. The most common mode of presentation of stress ulcer is the onset of acute upper GI bleed like hematemesis or melena in a patient with the acute critical illness. A similar condition involving elevated intracranial pressure is known as Cushing’s ulcer. Cushing’s ulcer is a gastro-duodenal ulcer produced by elevated intracranial pressure caused by an intracranial tumour, head injury or other space-occupying lesions. The ulcer, usually single and deep, may involve the oesophagus, stomach, and duodenum. Increased intracranial pressure may affect different areas of the hypothalamic nuclei or brainstem leading to overstimulation of the vagus nerve or paralysis of the sympathetic system. Both of these circumstances increase secretion of gastric acid and the likelihood of ulceration of gastro-duodenal mucosa.Mallory-Weiss syndrome is characterized by upper gastrointestinal bleeding secondary to longitudinal mucosal lacerations (known as Mallory-Weiss tears) at the gastroesophageal junction or gastric cardia. However, Mallory-Weiss syndrome may occur after any event that provokes a sudden rise in the intragastric pressure or gastric prolapse into the oesophagus, including antecedent transoesophageal echocardiography. Precipitating factors include retching, vomiting, straining, hiccupping, coughing, primal scream therapy, blunt abdominal trauma, and cardiopulmonary resuscitation. In a few cases, no apparent precipitating factor can be identified. One study reported that 25% of patients had no identifiable risk factors.

Cranial nerve XI is also known as the accessory nerve. The accessory nerve innervates the trapezius muscle which retracts the scapula. The upper and lower fibres act together to also upwardly rotate it.

The most probable diagnosis in this patient is Noonan syndrome.Noonan syndrome is an autosomal dominant condition characterised by short stature, heart defects (mainly pulmonary stenosis) and learning difficulties. An affected foetus can present with a significant increase in the nuchal thickness or a cystic hygroma of the neck. The condition is variable, and an affected parent is often only diagnosed after the diagnosis in the child.

Haemophilia B (also known as Christmas disease) is due to a deficiency in factor IX. Haemophilia A is due to a deficiency in factor VIII.

The most appropriate next step would be to request for an EEG (electroencephalogram).The two primary differential diagnoses for the clinical scenario presented would be night terrors (hypnopompic hallucinations) due to underlying narcolepsy or frontal lobe epilepsy.Though occult airway obstruction and obstructive sleep apnoea can cause sleep disturbances, the clinical scenario presented is more suggestive of neurological disorders.The hyper-motor activity, along with arm posturing (often dystonic in appearance) and multiple occurrences per night would favour epilepsy. The daytime somnolence could be secondary to the nocturnal seizures.

A not immunized patient with a clean wound requires an immediate vaccination against diphtheria, tetanus and polio, according to the Green Book of Immunisation against infectious disease. (The Green Book has the latest information on vaccines and vaccination procedures, for vaccine preventable infectious diseases in the UK.)

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.

Diabetes insipidus (DI) is defined as the passage of large volumes (>3 L/24 hr) of dilute urine (< 300 mOsm/kg). It has the following 2 major forms:Central (neurogenic, pituitary, or neurohypophyseal) DI, characterized by decreased secretion of antidiuretic hormone (ADH| also referred to as arginine vasopressin [AVP])Nephrogenic DI, characterized by decreased ability to concentrate urine because of resistance to ADH action in the kidney.The boy most probably has nephrogenic diabetes insidious (DI) not central DI so he is not responding to the ADH treatment.

Colonic Polyposis and neoplasia are often seen in Cowden Syndrome which is a hamartomatous polyposis syndrome. Patients with Cowden syndrome have an increased risk for colorectal cancer.

Non Epileptic attack disorder, also known as psychogenic non epileptic seizures, is a condition characterised by episodes of abnormal movement and behaviours that resemble seizures , but are not caused by electrical activity changes in the brain. It is believed that the disorder is caused by the brain’s response to overwhelming or stressful situations. In Jamila’s case, triggering conditions for her episodes may have been the absence of her mother, and the stress of her upcoming examinations. A diagnosis of generalised epilepsy is unlikely because this would require abnormal movement in all 4 limbs during an episode and a longer period of semi consciousness after. Cataplexy can also be ruled out as it does not involve a loss of consciousness which we see in Jamila’s case. Vasovagal syncope does not correspond with the history as there was no evidence of simultaneous collapse and responsiveness coupled with colour change and near spontaneous recovery when supine. A complex partial seizure may be possible in Jamila’s case, however the history of long duration, her eyes being tightly shut and the differences in presentation between the two episodes makes it less likely.

The external aspect of the tympanic membrane is lined by stratified squamous epithelium. Clinical correlation: Following middle ear infections, this type of epithelium can migrate into the middle ear through a perforated tympanic membrane.The ear is composed of three anatomically distinct regions: – External ear: Auricle is composed of elastic cartilage covered by skin. The lobule has no cartilage and contains fat and fibrous tissue. External auditory meatus is variable in length, measuring approximately 2.5cm long in fully grown children. Lateral third of the external auditory meatus is cartilaginous, and the medial two-thirds is bony. The greater auricular nerve innervates the region. The auriculotemporal branch of the trigeminal nerve supplies most of the external auditory meatus and the lateral surface of the auricle. – Middle ear: It is the space between the tympanic membrane and cochlea. The aditus leads to the mastoid air cells is the route through which middle ear infections may cause mastoiditis. Anteriorly the eustachian tube connects the middle ear to the nasopharynx. The tympanic membrane consists of an outer layer of stratified squamous epithelium, a middle layer of fibrous tissue and an inner layer of mucous membrane continuous with the middle ear. The chorda tympani nerve passes on the medial side of the pars flaccida. The glossopharyngeal nerve and pain innervate the middle ear. Thus, pain may radiate to the middle ear following tonsillectomy. – Ossicles:Malleus attaches to the tympanic membrane (the Umbo). Malleus articulates with the incus (synovial joint). Incus attaches to stapes (another synovial joint). – Internal ear:It consists of the cochlea, semicircular canals, and vestibule. Organ of Corti is the sense organ of hearing and is located on the inside of the cochlear duct on the basilar membrane. Vestibule accommodates the utricle and the saccule. These structures contain endolymph and are surrounded by perilymph within the vestibule. The semicircular canals lie at various angles to the petrous temporal bone. All share a common opening into the vestibule.

Hypogonadism is found to be a feature in gigantism also known as acromegaly, a condition caused by growth hormone abnormalities. The most common cause of gigantism is a pituitary adenoma. Hypogonadism exists among other endocrinopathies found in gigantism. Other causes of hypogonadism include Kleinfelter’s syndrome, which is characterized by premature testicular failure. The other options listed, primary hypothyroidism, teratoma, tuberous sclerosis and hepatoblastoma are all associated with precocious puberty.

Howell-Jolly bodies are often seen in post-splenectomy cases, together with Pappenheimer bodies, target cells and irregular contracted red blood cells. The loss of splenic tissue results in the inability to readily remove immature or abnormal red blood cells from the circulation.

NICE does not recommend the use of antibiotics or tonsillectomy in this case. Paracetamol/Ibuprofen are indicated as they provide antipyretic action and analgesia. Indications for tonsillectomy are 5 or more episodes of acute sore throat/year, symptoms present for at least 2 years, symptoms are severe enough to disrupt a child’s social life. Indications for antibiotics are features of marked systemic upset secondary to the acute sore throat, unilateral peritonsillitis, history of rheumatic fever, an increased risk from acute infection, acute tonsillitis with 3 or more of the following: history of fever, tonsillar exudates, no cough and tender anterior cervical lymphadenopathy.

Hematopoietic stem cells (HSCs) are a rare population of cells residing in the bone marrow (BM) and continuously replenish all mature blood cells throughout their life span.

The rational part of a teen’s brain isn’t fully developed until the age of 25 or so.In fact, recent research has found that adult and teen brains work differently. Adults think with the prefrontal cortex, the brain’s rational part. This is the part of the brain that responds to situations with good judgment and an awareness of long-term consequences. Teens process information with the amygdala. This is the emotional part.

The given presentation is highly suggestive of withdrawal effect of ketamine.Ketamine is known for its unique property of inducing dissociative anaesthesia. Emergence phenomenon (as seen in the child in question) is an adverse effect that occurs during recovery from dissociative anaesthesia. The components of emergence phenomenon include a euphoric state associated with hallucinations.

Findings described are suggestive of Waterhouse-Friderichsen syndrome which develops secondary to meningococcaemia. The reported incidence of Addison’s disease is 4 in 100,000. It affects both sexes equally and is seen in all age groups. It tends to show clinical symptoms at the time of metabolic stress or trauma. The symptoms are precipitated by acute infections, trauma, surgery or sodium loss due to excessive perspiration.

Neonatal hypoglycaemia is a common yet serious condition characterized by blood sugar levels less than 2.2mmol/L during the first 3 days of life and less than 2.5mmol/L later on. There are multiple aetiologies and various risk factors that lead to hypoglycaemia in new-borns like prematurity, sepsis, inborn errors of metabolism, and maternal diabetes- induced hyperinsulinism in the new-born. Among the inborn errors of metabolism, fatty acid oxidation defects can lead to persistent hypoglycaemia in new-borns. One such defect is the medium- chain acyl CoA dehydrogenase deficiency, which is the enzyme needed for the breakdown of medium- chain fatty acids.

The azygos lobe is usually well seen on the chest radiograph, where it is limited by the azygos fissure, a fine, convex (relative to the mediastinum) line that crosses the apex of the right lung.

Precocious puberty is the attainment of normal pubertal biochemical and physical features at an abnormally early age. The age cut-offs commonly used to define precocious puberty are 8 years for females and 9 years for males. Precocious puberty may be central (true) or peripheral (false) based on the aetiology. Central precocious puberty is due to the premature activation of the hypothalamic-pituitary-gonadal axis, which in turn leads to the development of secondary sexual characteristics at an earlier than usual age. Central precocious puberty is mostly idiopathic, but other causes include congenital or prolonged, untreated hypothyroidism, congenital or acquired central nervous system pathologies, and several genetic syndromes like Silver-Russell syndrome. The other mentioned choices cause peripheral or false precocious puberty.

Eczema herpeticum is a form of Kaposi varicelliform eruption, characterized by extensive vesicular skin eruptions that arise from a pre-existing skin condition, usually atopic dermatitis. The most common pathogen is the herpes simplex virus type 1, which has a higher propensity of attacking the epidermis already damaged by atopic dermatitis. The incubation period of the illness is 5-14 days. The eruption is initially small, monomorphic, dome-shaped papulovesicles that rupture to form tiny punched-out ulcers. It is diagnosed by taking viral swabs for culture.

Endothoracic fascia: the connective tissue (fascia) that is between the costal parietal pleura and the inner wall of the chest wall.
Costomediastinal recess: the point where the costal pleura becomes mediastinal pleura.
Costodiaphragmatic recess: is the lowest point of the pleural sac where the costal pleura becomes diaphragmatic pleura.
Cupola: the part of the parietal pleura that extends above the first rib level into the root of the neck.
Costocervical recess: this is a made-up term.
Peritracheal fascia: a layer of connective tissue that invests the trachea.

The symptoms of a partial generalized seizure are consistent with this patient.

There are two lateral rotators of the arm, the infraspinatus and the teres minor muscles. The infraspinatus muscle receives nerve supply from C5 and C6 via the suprascapular nerve, whilst the teres minor is supplied by C5 via the axillary nerve.

The general target of weight gain in the neonatal intensive care unit is to replicate the intrauterine growth in the third trimester, which equates to the daily weight gain of nearly 15 g/kg/day with infants receiving 120 kcal/kg/day