Pale stools suggest obstructive jaundice. Initially, the symptoms of biliary atresia are indistinguishable from those of neonatal jaundice, a usually harmless condition commonly seen in infants. However, infants with biliary atresia develop progressive conjugated jaundice, pale white stools and dark urine.

This is the clinical picture of dermatitis herpetiformis associated with gluten-sensitive enteropathy. To establish the diagnosis, you should measure the coeliac antibodies.
Darrier’s sign (where the skin urticates when it is stroked) is positive in urticaria pigmentosa.

Cardiac notch: is an indentation on the left lung of the heart.

Coronary sinus: a venous sinus on the surface of the heart (the posterior aspect) that receives blood from the smaller veins that drain the heart.

Coronary sulcus: a groove on the heart between the atria and ventricles.

Transverse pericardial sinus: located behind the aorta and pulmonary trunk and anterior to the superior vena cava.

Oblique pericardial sinus: located behind the left atrium. Accessed from the inferior side (or the apex) of the heart upwards.

Horizontal pericardial sinus: this is a made-up term.

Cyanotic congenital heart disease (CCHD) is a common cause of neonatal morbidity and mortality. They can be classified as CCHD due to:
– Right-to-left shunt, associated with the decreased pulmonary flow, e.g., tetralogy of Fallot (TOF), pulmonary atresia, right-sided hypoplastic heart,
– Right-to-left shunt, associated with the decreased aortic flow, e.g., left-sided hypoplastic heart, interrupted arch, severe coarctation;
– Bidirectional shunt, e.g., TGA, DORV, TA, etc.
Eisenmenger syndrome is not a neonatal CCHD; rather it develops later in young adulthood secondary to various CHD.

Hypopigmentation is a side-effect that is more likely to be due to eczema rather than topical steroids therapy.

All the options provided are potential side-effects of topical steroid therapy. They are observed when the potency of the steroid used is too high, or the amount of drug used is too much..
FTU or fingertip unit application is what is recommended, with caution, in delicate areas such as the face.

Hypopigmentation can occur in eczema and is a post-inflammatory response, in contrast to the total depigmentation which is seen in vitiligo.

Chronic eczema causes lichenification of skin, where the epidermis is thickened, and not skin atrophy.

Bone age is used to determine the maturation of a child’s bones’ and is used to detect pathological growth. This is done using the X-ray of the wrist. Several conditions can either advance or delay the bone age such that they may not match the child’s chronological age. Bone age is advanced in conditions where there are prolonged or elevated sex hormone levels such as precocious puberty, or in genetic overgrowth conditions such as Beckwith-Wiedemann syndrome. Bone age is delayed in constitutional growth delay, chronic ill health, endocrine disorders such as growth hormone deficiencies or hypothyroidism, genetic disorders such as Trisomy 21, Trisomy 18, and Turner’s syndrome. Obesity is unlikely to cause growth delay.

In the Hirschberg test, the corneal reflex should fall in the same place in both eyes. If there is a malalignment then the reflection of the light will appear in different places in the two eyes. The cover test is then used to differentiate between a latent deviation, or a manifest deviation.

In the Bruckner test, the direct ophthalmoscope is used to obtain a red reflex simultaneously in both eyes. If strabismus is present, the deviated eye will have a lighter and brighter reflex than the fixating eye.

The swinging flashlight test is a test of the pupils response to light, and to check for a relative afferent pupillary defect (RAPD).

The Angle Kappa test measures the angle between the line of sight and the corneal-pupillary axis. It is a monocular measurement.

The clinical presentation is typical of a slipped femoral epiphysis, which refers to a fracture through the growth plate (physis), resulting in slippage of the overlying end of the femur. It is the most common hip disorder in adolescence. SCFEs usually cause groin pain on the affected side, but sometimes cause knee or thigh pain. The range of motion in the hip is restricted in internal (medial) rotation, abduction, and flexion.

Noonan syndrome is one of the most common genetic diseases associated with congenital heart defects, being second in frequency only to Down syndrome.
Pulmonary stenosis and hypertrophic cardiomyopathy are generally the most common congenital heart defects found in Noonan syndrome.
Pulmonary stenosis is often associated with a thickened and dysplastic valve. It is usually difficult to obtain a satisfactory result using the transcatheter balloon dilatation of such dysplastic valves, so surgical intervention is more likely to be needed.
Hypertrophic cardiomyopathy involves predominantly the ventricular septum as asymmetric septal hypertrophy, but may also affect the ventricular free walls. Left ventricular outflow tract obstruction may occasionally be produced.

Among the treatment options provided, the most appropriate would be prednisolone 1-2 mg/kg.

Other options:
– Dexamethasone 0.15 mg per kg is the correct dose based on new trials and BNF guidance.
– Inhaled budesonide 2mg would be a more appropriate dose.
– Nebulised adrenaline may also be used.

Croup:
Laryngotracheobronchitis caused most commonly by parainfluenza virus. It most commonly affects children between 6 months to 3 years.
Seasonal peaks: Most prevalent in autumn and spring.
It starts as viral URTI then progresses to barking cough and a hoarse cry. Stridor and respiratory distress may occur. The illness can last from two days to 2 weeks.
Relevant differential diagnoses include epiglottitis (the child will be septic with high temp and drooling) and inhaled foreign body.
All children with moderate/severe croup should be given one dose of oral dexamethasone.
Further doses may be needed if children have rebound symptoms.

According to the NICE guidelines, the frequency of stool passage is considered constipation when two or more of the following findings are present:

Fewer than 3 stools a week, large hard stools, stools that resemble rabbit droppings; symptoms associated with defecation including distress, bleeding or straining; a history of previous episodes of a precious/current anal fissure.

The child in the scenario fails to meet the criteria, and has no evidence of impacted faeces on examination. The best course of action is to encourage the parents to give the child more water to prevent dehydration, and feed the child a fibre rich diet including fruits, vegetables and high fibre bread.

Hyperkalaemia is not to evaluate or detect AKI but rather the result of it. If one of the rest of the options is present, then AKI would be suspected.

These cardiovascular findings are suggestive of coarctation of the aorta, which is common among patients with Turner’s syndrome. Physical features of Turner’s syndrome include a short neck with a webbed appearance, a low hairline at the back of the head, low-set ears, and narrow fingernails and toenails that are turned upward.
Atrioventricular septal defect is the most common cardiac anomaly in Down’s syndrome.
Structural heart defects at birth (i.e., ventricular septal defect, atrial septal defect, patent ductus arteriosus) are common in Edward’s syndrome.
Pulmonary stenosis with or without dysplastic pulmonary valve and hypertrophic cardiomyopathy are common in Noonan’s syndrome.
Supravalvar aortic stenosis and peripheral pulmonary stenosis are found in William’s syndrome.

Gastrointestinal polyps are common in children and may result in intussusception due to polyp traction. Treatment is usually surgical with enterotomy and removal of the polyp or of a segment of the bowel.

The most probable cause for the patient’s presenting symptoms is encephalitis secondary to measles infection.

Encephalitis:
Encephalitis occurs in 1 per 1000 measles cases. It usually presents within 1-14 days of the rash, usually day 5. It may present with fever, headache, vomiting, stiff neck, meningeal irritation, drowsiness, seizures, reduced consciousness; 15% will have neurological sequelae; 10% mortality.

Other options:
– Acute disseminated encephalomyelitis occurs in 1 per 1000 measles cases. It is thought to be a postinfectious immune response. Clinical manifestations include fever, headache, neck stiffness, seizures and mental status changes. It could fit this presentation although it tends to present in the recovery phase of measles infection, typically two weeks after the exanthem. In contrast, encephalitis tends to occur within a few days of the rash.

– The history is not that of a typical febrile seizure as it lasted >15 minutes. He has also not recovered consciousness at 4 hours. The diazepam should have been eliminated within 4 hours and so should no longer be contributing to his reduced level of consciousness. Also, the history states the recent diagnosis of measles, and so complications of measles should be considered.

– Intracranial bleed secondary to thrombocytopenia: Measles is not known to cause thrombocytopenia.

– Subacute sclerosing panencephalitis (also known as Dawson’s encephalitis): This is an extremely rare complication of measles. It is a progressive degenerative disease of the central nervous system that occurs 7-10 years after a measles infection.
The treatment for SSPE is mainly intraventricular interferon therapy through an Ommaya reservoir. The prognosis is poor, and it is usually fatal.

The property described in the question is heterogeneity.

Heterogeneity is when a single phenotype or genetic disorder can be caused by multiple numbers of genetic mutations.

Other options:
– Pleiotropy occurs when a single gene influences multiple phenotypic traits. The underlying mechanism is that the gene codes for a product that is used by various cells. A classic example of pleiotropy is the human disease PKU (phenylketonuria).
– The penetrance of a disease-causing mutation is the proportion of individuals with the mutation who exhibit clinical symptoms. For example, the gene responsible for a particular autosomal dominant disorder has 70% penetrance, then 70% of those with the mutation will develop the disease, while 30% will not.

The external auditory canal (EAC) extends from the auricle to the tympanic membrane. Its lateral portion is fibrocartilaginous and its medial portion is bony. Its bony portion is formed by the tympanic portion of the temporal bone.

Turner’s syndrome is characterised by a webbed neck, short stature, primary amenorrhea and cardiac, renal and muscular defects. Gene analysis shows a single X chromosome in the patients. Downs syndrome has certain cognitive and physical abnormalities, whereas in Klinefelter syndrome there are widely spaced nipples, long arms and infertility and it only occurs in men.

Henoch-Schonlein purpura (HSP) rashes are commonly found on the legs, feet, and buttocks while Immune thrombocytopenia (ITP) rashes manifest predominantly on the lower legs. HSP happens following a sore throat while ITP usually happens following an URTI or Flu. HSP is an inflammation of a blood vessel (vasculitis) while ITP is immune mediated insufficiency of platelets.

Androgen insensitivity syndrome (AIS), previously referred to as testicular feminization, is an X-linked disorder in which the patients are genotypically male (possessing and X and Y chromosome) and phenotypically female. This disorder is rare, with reported incidences from 1 in 20,000 to 1 in 60,000 live male births, and is the result of a missing gene on the X chromosome that is responsible for the cytoplasmic or nuclear testosterone receptor. In its absence, the gonad, which is a testis, produces normal amounts of testosterone; however, the end tissues are unable to respond due to the deficient receptors leading to the external genitalia developing in a female fashion. Anti-Mullerian hormone, which is produced by the testis, is normal in these patients, leading to regression of the Mullerian duct. Wolffian duct development, which depends on testosterone, does not occur as the ducts lack the receptors.
The cumulative effect is a genotypic male with normal external female genitalia (without pubic or axillary hair), no menses, normal breast development, short or absent vagina, no internal sex organs, and the presence of testis. Frequently, these patients have bilateral inguinal hernias in childhood, and their presence should arouse suspicion of the diagnosis.

Endoderm derivatives include the epithelium of the following: gastrointestinal tract and its glands, glandular cells of the liver and pancreases, urachus and urinary bladder, pharynx, trachea and alveoli, part of the tonsils, thyroid and parathyroid, tympanic cavity and thymus and part of the anterior pituitary gland.

HbA1c stands for glycated haemoglobin. In patients with haemolytic diseases associated with a shortened RBC survival, HbA1c levels can be unusually low despite controlled diabetes mellitus.
These haemolytic diseases can include glucose-6-phosphate dehydrogenase deficiency and sickle cell anaemia.

HbA1c:
It is a measure of non-enzymatic glycation which occurs due to haemoglobin’s exposure to plasma glucose. As plasma glucose increases so does HbA1c.
The level of HbA1c quantitively assesses the control of diabetes mellitus over the last 120 days (as this is the life span of a red blood cell).

Haemoglobin type A is separated on cation exchange chromatography
Other subsections include HbA1O, HbA1a, HbA1b.

According to the updated NICE recommendations (2015):
The target level of HbA1c in children with T1DM is 48 mmol/mol (6.5%) or lower. It is monitored every three months. Better control is associated with fewer long term complications

The Chi-squared test would have been a useful test to compare the proportions in the scenario. However, due to the small sample size, Fisher’s exact test can be applied to analyse the significance of the difference. Adequate information is not given to determine what sample sizes were used to test the efficacy of the treatment, and to tell whether the treatment should be discontinued without further analysis on the data.

Among the options provided, zidovudine causes lipoatrophy as a side effect.

Zidovudine: Although both hypertrophy and atrophy are described related to HIV medications, nucleoside reverse transcriptase inhibitor (NRTIs) such as zidovudine and stavudine are closely associated with fat loss.

Other options:
– Enfuvirtide is an HIV-fusion inhibitor. Lipoatrophy is not commonly associated with this drug.
– Efavirenz is an NNRTI, which is not associated with lipoatrophy. Common side effects include neuropsychiatric effects, rash and nausea.
– Ganciclovir is not an anti-HIV medication and is used for cytomegalovirus (CMV) infections.
– Raltegravir is an integrase inhibitor and is associated with fat gain.

Oesophageal atresia refers to a congenitally interrupted oesophagus. One or more fistulae may be present between the malformed oesophagus and the trachea. The lack of oesophageal patency prevents swallowing. In addition to preventing normal feeding, this problem may cause infants to aspirate and literally drown in their own saliva, which quickly overflows the upper pouch of the obstructed oesophagus. If a tracheoesophageal fistula (TEF) is present, fluid (either saliva from above or gastric secretions from below) may flow directly into the tracheobronchial tree.
The complete absence of gas in the GI tract denotes the absence of a distal tracheoesophageal fistula (TEF); however, distal fistulae simply occluded by mucous plugs have been rarely reported.

If no distal TEF is present, a gastrostomy may be created. In such cases, the stomach is small, and laparotomy is required. In all cases of oesophageal atresia in which a gastrostomy is created, care should be taken to place it near the lesser curvature to avoid damaging the greater curvature, which can be used in the formation of an oesophageal substitute. When a baby is ventilated with high pressures, the gastrostomy may offer a route of decreased resistance, causing the ventilation gases to flow through the distal fistula and out the gastrostomy site. This condition may complicate the use of ventilation.

The most likely organism is enterohemorrhagic verotoxin-producing E.coli. It usually causes haemolytic uremic syndrome.
Crohn’s disease rarely manifests in an acute manner.
Polio and giardiasis usually manifest with non-bloody diarrhoea.

Bulimia nervosa is a condition in which a person is involved in binge eating and then purging. This patient has swollen parotid glands. The glands swell in order to increase saliva production so that the saliva lost in the vomiting is compensated. This patient also has thickened calluses on the back of her hand. This is known as Russell’s sign. This occurs because of putting fingers in the mouth again and again to induce the gag reflex and vomit. The knuckles get inflamed in the process after coming in contact with the teeth multiple times.

Teenagers may have poor eating habits and neglect to take their vitamin supplements. They are less likely than older women to be of adequate pre-pregnancy weight or to gain an adequate amount of weight during pregnancy. Low weight gain increases the risk of having a low birthweight baby. Weight gain is maximal during the 2nd trimester.

aPTT and gallbladder emptying is decreased during pregnancy whereas d-dimers is increased.

Protein energy malnutrition often presents in two common forms, Kwashiorkor and Marasmus. Kwashiorkor is characterised by a protein deficiency with an additional inadequate calorie intake. As a result, affected children present with oedema, muscular atrophy, and their weight for age is 60-80% of the expected weight. Their cutaneous tissue is however preserved. Marasmus on the other hand is characterised by a severe calorie deficiency leading to atrophy of the muscles and adipose tissue, with weight loss being less than 60% of the normal. In both cases, if the child is not promptly rehabilitated, the malnutrition could cause irreversible damage, such as hepatic, cardiac and renal impairments.

The child has Chronic suppurative otitis media (CSOM) which is defined as a perforated tympanic membrane with persistent drainage from the middle ear for more than 2-6 weeks.
CSOM differs from chronic serous otitis media in that chronic serous otitis media may be defined as a middle ear effusion without perforation that is reported to persist for more than 1-3 months.
Patients with chronic suppurative otitis media (CSOM) present with a draining ear of some duration and a premorbid history of recurrent acute otitis media, traumatic perforation, or the placement of ventilation tubes. Typically, they deny pain or discomfort. A common presenting symptom is hearing loss in the affected ear. Reports of fever, vertigo, and pain should raise concerns about infratemporal or intracranial complications. A history of persistent CSOM after appropriate medical treatment should alert the physician to consider cholesteatoma.
The external auditory canal may or may not be oedematous and is not typically tender. The discharge varies from fetid, purulent, and cheese like to clear and serous. Granulation tissue is often seen in the medial canal or middle ear space. The middle ear mucosa visualized through the perforation may be oedematous or even polypoid, pale, or erythematous.

The trigeminal nerve is responsible for the motor innervation of the muscles of mastication and well as providing sensory input for most of the head. In testing the function of this nerve, the jaw jerk reflex may be brisk in cases of upper motor neuron lesions, but is however absent in nuclear or infra nuclear lesions involving the trigeminal nerve. Other nerve reflexes include the gag reflex of the glossopharyngeal nerve, and the pupillary light reflex of the optic nerve.

Consuming iron in its Fe2+ (ferrous form) form can improve the ability to absorb dietary iron in patients with iron deficiency anaemia (IDA).

Clinical features of IDA include:
– Koilonychia
– Atrophic glossitis
– Post-cricoid webs
– Angular stomatitis

The peripheral blood smear shows the following abnormal RBC morphologies:
– Target cells
– Pencil poikilocytes
– Microcytic-hypochromic cells

Recurrent bacterial infections may be due to lack of B-cell function, consequently resulting in a lack of gamma globulins production. Once the maternal antibodies have depleted, the disease manifests with greater severity and is called x-linked agammaglobulinemia also known as ‘X-linked hypogammaglobulinemia’, ‘XLA’ or ‘Bruton-type agammaglobulinemia. it is a rare x linked genetic disorder that compromises the bodies ability to fight infections.

Acute leukaemia causes immunodeficiency but not so specific.

DiGeorge syndrome is due to lack of T cell function.

Aplastic anaemia and EBV infection does not cause immunodeficiency.

Common neurological symptoms of Wilson disease that may appear and progress with time include tremor, involuntary movements, difficulty swallowing (dysphagia), difficulty speaking and poor articulation (dysarthria), lack of coordination, spasticity, dystonic postures, and muscle rigidity.

Due to its cholestatic properties, Flucloxacillin can block bile flow through the liver, leading to the accumulation of bilirubin in the blood, giving rise to jaundice. Dark urine is the result of excessive bilirubin in the blood being filtered by the kidney. As the bile doesn’t move into the small intestine, stools are pale.

The T wave represents ventricular repolarization.

Other options:
– P wave:
Depolarization that spreads from the SA node throughout the atria. The wave lasts 0.08 to 0.1 seconds (80-100 ms).
The isoelectric period after the P wave represents the time in which the impulse is travelling within the AV node.
– P-R interval:
Time from the onset of the P wave to the beginning of the QRS complex. The wave ranges from 0.12 to 0.20 seconds in duration.
Represents the time between the onset of atrial depolarization and the onset of ventricular depolarization
– QRS complex:
It represents ventricular depolarization. The duration of the QRS complex is normally 0.06 to 0.1 seconds.
– ST-segment:
The isoelectric period following the QRS. It represents the period in which the entire ventricle is depolarized and roughly corresponds to the plateau phase of the ventricular action potential
– U wave:
It is a small positive wave which may follow the T wave. It represents the last remnants of ventricular repolarization.
– Q-T interval
It represents the time for both ventricular depolarization and repolarization to occur, and therefore roughly estimates the duration of an average ventricular action potential.
The interval ranges from 0.2 to 0.4 seconds depending upon heart rate.
At high heart rates, ventricular action potentials shorten in duration, which decreases the Q-T interval. Therefore the Q-T interval is expressed as a corrected Q-T (QTc) by taking the Q-T interval and dividing it by the square root of the R-R interval (interval between ventricular depolarizations). This allows an assessment of the Q-T interval that is independent of heart rate.

The normal corrected Q-Tc interval is less than 0.44 seconds.

Burkitt lymphoma is associated with the c-myc gene translocation, usually t(8;14).

Burkitt lymphoma is a rare high-grade non-Hodgkin lymphoma endemic to west Africa and the mosquito belt. It has a close association with the contraction of Epstein-Barr virus (EBV). Burkitt lymphoma often presents with symmetrical painless lymphadenopathy, systemic B symptoms (fever, sweats, and weight loss), central nervous system involvement, and bone marrow infiltration. Classically in the textbooks, the patient also develops a large jaw tumour.

Other aforementioned options are ruled out because:
1. t(9;22)-Chronic myeloid leukaemia
2. t(15;17)-Acute promyelocytic leukaemia
3. t(14;18)-Follicular Lymphoma
4. t(11;14)-Mantle Cell Lymphoma

Insufficient surfactant production in premature infants leads to respiratory distress syndrome or surfactant deficient lung disease (SDLD), characterized by structurally immature lungs. There are many risk factors of this disease, some of them include male gender, caesarean section, infants of diabetic mothers, being the second born of the premature twins, perinatal asphyxia, sepsis, and hypothermia. Maternal hypertension is not a recognized risk factor for respiratory distress syndrome; instead, pregnancy-induced hypertension and chronic maternal hypertension are the protective factors against this disease.

Children are very prone to dehydration during an episode of gastroenteritis. Dehydration is detected early by increased capillary filling time.

Erythema nodosum is the most common type of inflammation of the subcutaneous fat tissue, referred to as panniculitis. Erythema nodosum appears as painful, erythematous round lumps, usually symmetrically on the anterior aspect of lower extremities. These lesions are self-limiting and tend to resolve within 2-8 weeks without undergoing necrosis or ulceration. There are various causes of erythema nodosum, but the most important among children is the streptococcal throat infection. Other causes include autoimmune conditions like sarcoidosis, inflammatory bowel disease, mycobacterial infection (TB), drugs like penicillin and sulphonamides, and malignancies like some leukemias and lymphomas.

Infantile spasm belongs to the spectrum of seizure disorders. Infantile spasm manifests itself with loss of consciousness and jerky movements. The peak age of onset is between 4 and 6 months. Approximately 90% of infantile spasms begin before 12 months of age. It is rare for infantile spasms to begin during the first 2 weeks of life or after 18 months.

Munchausen syndrome by proxy is a mental illness and a form of child abuse. The caretaker of a child, most often a mother or a father, either makes up fake symptoms or causes real symptoms to make it look like the child is sick. The person with MSP gains attention by seeking medical help for exaggerated or made-up symptoms of a child in his or her care. As health care providers strive to identify what’s causing the child’s symptoms, the deliberate actions of the mother or caretaker can often make the symptoms worse.
The person with MSP does not seem to be motivated by a desire for any type of material gain. People with MSP may create or exaggerate a child’s symptoms in several ways. They may simply lie about symptoms, alter tests (such as contaminating a urine sample), falsify medical records, or they may actually induce symptoms through various means, such as poisoning, suffocating, starving, and causing infection.

An odds ratio (OR) is a measure of the association between an exposure and an outcome. The OR represents the odds that an outcome will occur given a particular exposure, compared to the odds of the outcome occurring in the absence of that exposure. Odds ratios are most commonly used in case-control studies, however they can also be used in cross-sectional and cohort study designs as well (with some modifications and/or assumptions). Where

a = Number of exposed cases

b = Number of exposed non-cases

c = Number of unexposed cases

d = Number of unexposed non-cases

OR=(a/c) / (b/d) = ad/bc

The mnemonic STUMPED is helpful for remembering the differential diagnosis for congenital corneal opacities:
– Sclerocornea
– Tears in Descemet membrane (usually due to forceps trauma or congenital glaucoma)
– Ulcers (e.g. infection – rubella)
– Metabolic (e.g., mucopolysaccharidosis)
– Peters anomaly
– oEdema
– Dermoids (e.g. Goldenhar’s syndrome)

In the first trimester of pregnancy, amniotic fluid is produced from maternal plasma where there is a rapid bi-directional diffusion across the foetal skin. At 10 weeks the volume is about 25ml. By 16th weeks of gestation the foetal kidneys make urine that contributes to the increasing volume of amniotic fluid, along with nasal secretions. At 20 weeks, the level is 400ml and increases to 800ml at 28weeks, after which it reduces to 600ml at term.

There are four types of IVH. These are called grades and are based on the degree of bleeding.

Grades 1 and 2 involve a smaller amount of bleeding. Most of the time, there are no long-term problems as a result of the bleeding. Grade 1 is also referred to as germinal matrix haemorrhage (GMH).
Grades 3 and 4 involve more severe bleeding. The blood presses on (grade 3) or directly involves (grade 4) brain tissue. Grade 4 is also called an intraparenchymal haemorrhage. Blood clots can form and block the flow of cerebrospinal fluid. This can lead to increased fluid in the brain (hydrocephalus).

CMV infection is usually asymptomatic in adults. However, if the mother is infected for the first time during pregnancy then there is high chances of this infection passing on to the foetus. CMV infection can cause blindness, deafness, learning difficulties, restricted growth etc. Hepatitis B, herpes simplex, syphilis and HIV do not present with these classical signs of CMV infection in new-borns. It is estimated that 10 stillbirths occur in England and Wales every year due to CMV infection. The foetus is most at risk in early pregnancy. There is no effective prevention.

This patient has pulmonary renal syndrome which is most commonly due to an ANCA positive vasculitis. The history of recurrent epistaxis makes Wegener’s granulomatosis the most probable diagnosis. Wegener’s granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO).

Intrauterine growth restriction refers to the failure of the fetus to grow in accordance with the weeks of gestation. There are two types of growth restriction, symmetrical and asymmetrical. Causes include various genetic abnormalities, fetal infections, maternal health conditions, etc. Risk factors for the development of IUGR include fetal echogenic bowel, maternal age above 40 years, low PAPP-A levels, maternal smoking or cocaine use, etc. Fetal echogenic bowel implies a brighter than usual fetal intestines on ultrasonography. It is a marker associated with trisomy 21, which is a cause of IUGR.

Intussusception is a condition in which one segment of intestine telescopes inside of another, causing an intestinal obstruction. Although intussusception can occur anywhere in the gastrointestinal tract, it usually occurs at the junction of the small and large intestines. Most describe the symptoms of intussusception as a triad of colicky abdominal pain, bilious vomiting, and currant jelly stool. The primary symptom of intussusception is described as intermittent crampy abdominal pain.

Lung buds are formed by 4-5 weeks of gestation.

Development of the respiratory tract has five stages:
– Embryonic (at 4 – 5 weeks of gestation):
Formation of lung buds, trachea and mainstem bronchi occur. These structures are formed from a ventral outpouching of foregut pharynx. At this stage, the beginnings of the five lung lobes are present.
– Pseudoglandular (at 5 – 16 weeks of gestation):
Formation of terminal bronchioles, cartilage and smooth muscles occur in this stage. Adult numbers of airways proximal to acini are in place.
– Canalicular (at 16-24 weeks gestation):
Differentiation of type I and II epithelial cells can be done in this stage. There is also an increase in the size of proximal airways).
– Saccular (at 24 – 40 weeks of gestation): Terminal saccule formation occurs. Production of surfactant takes place at this stage with an increase in the number of goblet cells
Up to half the adult number of alveoli are in place by this stage.
– Alveolar (occurs between 32 weeks of gestation till the post-natal age 8): Formation of alveoli and septation occurs with the expansion of air spaces.

Bulimia nervosa is an eating disorder that involves frequent episodes of binge eating followed by inappropriate purging behaviour. The underlying reason for this behaviour is distorted self-image. Bulimia leads to several medical issues, including various laboratory derangements. Among these deranged laboratory parameters are the hormone cholecystokinin, which is found to be decreased in women having bulimia. It is hypothesized that the bingeing episodes observed in patients with bulimia are due to lower levels of CCK since normal levels of CCK are involved in maintaining satiety and controlling the urge to binge eat.

The post-ingestion plasma level, which is required in order to guide the treatment, reaches a peak at 4 hours. Levels requiring antidote (N-acetyl cysteine) include: 100 mcg per ml at 4 hours, 35 mcg per ml at 10 hours and 25 mcg per ml at 12 hours. These levels are in conjunction with the levels recorded and they should all be put down on a treatment nomogram.

Alopecia is a scalp condition characterized by either overall baldness or patches of hair loss over the head. It can be broadly classified as scarring alopecia and non-scarring alopecia. Non-scarring is the most common type and is seen in various conditions like nutritional deficiencies, alopecia areata, hypothyroidism, tinea capitis, and SLE. Scarring alopecia is commonly seen in cases of discoid lupus erythematosus, which is a common cause of widespread inflammatory and scarring lesions all over the body and scalp.

Nonspecific triggers of inflammation in patients with atopic dermatitis may include physical or chemical irritants. The following simple measures should be followed in daily life to reduce the frequency and severity of irritant-induced atopic dermatitis flares :
Skin care products that contain alcohol and astringents should be avoided.
New clothes should be laundered before use to remove formaldehyde and other chemicals.
Liquid detergents are preferred over powder detergents for laundering clothes, as liquids are easier to rinse out. A second rinse cycle may also improve removal of residual detergent.
Patients should shower immediately after swimming in chlorinated pools and should subsequently apply moisturizer.
Fragrance-free skin products that are hypoallergenic or made for sensitive skin may be less irritating than other kinds of skin products.
Prenatal and postnatal probiotic supplementation may be helpful in preventing the development of atopic dermatitis in young children. In a 2008 meta-analysis, the most commonly studied probiotic was Lactobacillus rhamnoses GG. Larger, randomized controlled studies are needed to confirm these initial findings.

Breastfeeding during the first 4 months of life may reduce the incidence and severity of childhood atopic disease but only modestly and only in those at high risk.

From the given scenario, the extraocular muscle affected in the child is superior oblique muscle.

Point to remember:
All of the extraocular muscles are supplied by the oculomotor nerve (3rd cranial nerve), except superior oblique (trochlear nerve/4th cranial nerve) and lateral rectus (abducens nerve/6th cranial nerve).

The superior oblique muscle causes the eye to move downwards and medially. The unopposed inferior oblique, in this case, causes the eye to deviate upwards and medially.
Children tend to tilt their head (torticollis) to the side opposite to the affected eye with their chin down toward the shoulder, and their face turned away from the affected side to decrease the diplopia.

Among the given options, lipoatrophy takes time to develop as it is a long-term complication of insulin therapy. Since it has only been two weeks since the initiation of insulin therapy, it is unlikely to occur so early.
The risk of lipoatrophy is reduced with newer insulins and also can be avoided by site rotation.
However, anaphylaxis, allergic reactions, infection and abscesses at the site of an insulin pump can occur in this patient as a complication of insulin therapy.

The thyroglossal duct cyst is the most common congenital anomaly of the central portion of the neck. The thyroglossal duct cyst is intimately related to the central portion of the hyoid bone and usually elevates along with the larynx during swallowing. Thyroglossal cysts can be defined as an irregular neck mass or a lump which develops from cells and tissues left over after the formation of the thyroid gland during the developmental stages.

The type of significance test used depends on whether the data is parametric (can be measured, usually normally distributed) or non-parametric.

Parametric tests:

Student’s t-test – paired or unpaired*

Pearson’s product-moment coefficient – correlation

Non-parametric tests:

Mann-Whitney U test – unpaired data

Wilcoxon signed-rank test – compares two sets of observations on a single sample

chi-squared test – used to compare proportions or percentages

Spearman, Kendall rank – correlation.

The outcome measured is not normally distributed, i.e. it is non-parametric. This excludes the Student’s t-tests. We are not comparing percentages/proportions so the chi-squared test is excluded. The Mann-Whitney U test is a nonparametric test of the null hypothesis that it is equally likely that a randomly selected value from one sample will be less than or greater than a randomly selected value from a second sample.

This test can be used to investigate whether two independent samples were selected from populations having the same distribution.

Ichthyosis vulgaris presents clinically with xerosis, hyperkeratosis, excess scaling, keratosis pilaris, and palmar and plantar hyperlinearity. It most commonly affects the extensor surfaces of the limbs and spares flexor surfaces, the face, and the neck.

Most cases of Prader-Willi syndrome are not inherited, particularly those caused by a deletion in the paternal chromosome 15 or by maternal uniparental disomy. These genetic changes occur as random events during the formation of reproductive cells (eggs and sperm) or in early embryonic development. Affected people typically have no history of the disorder in their family.
Rarely, a genetic change responsible for Prader-Willi syndrome can be inherited. For example, it is possible for a genetic change that abnormally inactivates genes on the paternal chromosome 15 to be passed from one generation to the next.

Prader-Willi syndrome is a complex genetic condition that affects many parts of the body. In infancy, this condition is characterized by weak muscle tone (hypotonia), feeding difficulties, poor growth, and delayed development. Beginning in childhood, affected individuals develop an insatiable appetite, which leads to chronic overeating (hyperphagia) and obesity. Some people with Prader-Willi syndrome, particularly those with obesity, also develop type 2 diabetes (the most common form of diabetes).

Case control studies are used to determine the relationship between exposure to a risk factor and the resultant outcomes. It finds patients in the general population who have a certain condition and retrospectively searches for past exposure to possible risk factors for the disease. Controls are people who do not have the disease found in the general population. This type of study is useful for rare diseases, and is less time consuming to conduct.

While cohort studies can be used to study rare diseases they measure the relative risk of developing the disease over time based on exposure.

Ecological or cross-sectional studies attempt take a snapshot of a whole population, and thus are inappropriate for rare diseases as larger sample numbers are needed. A randomised control trial is better suited to determine the effect of an intervention.

Cystic fibrosis is a progressive, genetic disease. A defective gene causes a thick, sticky build-up of mucus in the lungs, pancreas, and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage, and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients. Since the digestive enzymes are not being made, the food is not completely digested making them bulky, smelly, and hard to flush away. Finger clubbing is a prominent feature of cystic fibrosis on most patients.

An epidural hematoma occurs when there is bleeding between the dura mater (a tough fibrous layer of tissue between the brain and skull) and the skull bone. These occur when arteries are torn as a result of a blow to the head, and injury in the temple area is a common cause. Although the pattern of a lucid interval followed by later neurological symptoms is characteristic, only a minority of patients display this pattern of symptoms. Reported death rates from epidural hematoma vary widely, ranging from 5% to over 40%. Middle meningeal artery is frequently involved in such incidents.

Patients with HIV have a deficiency of CD4 lymphocytes which are also known as helper T cells (Th).
They are involved with antigen-specific responses as well as delayed-type hypersensitivity.
The risk of developing P. jiroveci pneumonia is greatest with a CD4 count of 200 x 109/l or below.

Epilepsy is a common paediatric neurologic disorder characterized by seizures of varying frequency and intensity. There are many childhood or infantile epilepsy syndromes that can be categorized as benign epilepsy syndromes as the child suffering from these usually outgrows them after reaching a certain age, and they do not lead to significant cognitive or physical impairment. Some of these may not need any treatment. Such benign epilepsy syndromes include benign rolandic epilepsy, Panayiotopoulos syndrome, Gastaut type-idiopathic childhood occipital epilepsy, and idiopathic photosensitive occipital lobe epilepsy.
West syndrome, also called infantile spasms, is a serious infantile epileptic encephalopathy, characterized by multiple clusters of myoclonic spasms, and regression of the previous normally attained milestones. It is associated with severe cognitive and physical impairment, often leading to life long disability.

History of recurrent infections and failure to thrive (probably due to pancreatic enzyme insufficiency) is highly suggestive of cystic fibrosis. Pseudomonas has been identified as an important respiratory pathogen in patients with cystic fibrosis.

Maternal smoking is one of the most significant risk factors for pyloric stenosis.

Remember the three Ps for Pyloric Stenosis:
P -palpable mass
P -peristalsis
P -projectile vomiting

History of recurrent infections and failure to thrive (probably due to pancreatic enzyme insufficiency) is highly suggestive of cystic fibrosis. Pseudomonas has been identified as an important respiratory pathogen in patients with cystic fibrosis.

Among the options provided, an 8-week-old full-term infant with normal developmental milestones will smile in response to appropriate stimuli.

Rationale:
Smiles in response to appropriate stimuli are seen from around 6 weeks of age. A social smile, where the infant has an awareness that a smile attracts attention, is not observed until around 3 months of age.

Other options:
– At 6 weeks an infant is able to follow objects in the horizontal plane through 90 deg; fixing and following an object through 180 deg in the horizontal plane is a milestone achieved at 3 months, as is pushing up onto forearms.
– Pushing up onto hands occurs by around 5 months.
– Infants of 3 months should startle to a 60 dB sound.

Lamotrigine does not significantly increase risk of birth defects during pregnancy.

The femoral nerve is the main nerve supply for the thigh muscles including the pectineus, iliacus, sartorius, which flex the hip; and the quadriceps femoris made up of the rectus femoris, vests laterals, vastus medialis and vastus intermedius, which extend the knee. The nerve is derived from the L2, L3 and L4 nerve roots, and supplies cutaneous branches to the anteromedial thigh and the medial side of the leg via the saphenous nerve.

Tetralogy of Fallot (TOF), a congenital heart defect, includes the following: right ventricular hypertrophy, ventricular septal defect, abnormal position of the aorta and pulmonary valve stenosis. The O2 saturation in patients with TOF is typically lower than normal and the condition usually becomes symptomatic early in life. A feature of the disease with high diagnostic significance is squatting or crouching of the infant as a compensatory mechanism to increase the peripheral vascular resistance.

Orbital bone fracture: The child has sustained a head injury and subsequently describes a CSF rhinorrhoea (indicating a cerebro-spinal fluid leak). CSF rhinorrhoea can occur in skull or nasal bone fractures. Given the symptoms of loss of consciousness and headache, this child is more at risk of having suffered a skull fracture requiring emergency CT head investigation and specialist neurosurgical management. An ethmoid bone fracture may also present this way. A skull x ray would help to determine an air fluid level and indeed allow some visualisation of the nasal bones, though in children the nasal bones do not visualise well due to lack of fusion. Either way this child would need assessment in the nearest Emergency Department and the school would be expected to follow a ‘head injury’ protocol.

Based on the clinical scenario, the boy is going through normal pubertal changes.

In the 3 years before puberty, low pulsatile LH levels become detectable during sleep. LH and FSH are produced in the anterior pituitary and released due to pulsatile gonadotrophin-releasing hormone (GnRH) secreted by the hypothalamus.
There is an increase in the amplitude and frequency of LH secretion as puberty approaches, which causes enlargement of the gonads. In boys, the testicles produce testosterone, and in girls, the ovaries produce oestradiol and ovarian androgens, which, with the adrenal androgens, produce secondary sexual characteristics.

Note:
The average age at onset of puberty is 11 years in girls. The first sign is breast bud development, followed by the appearance of pubic hair 6-12 months later.
Menarche usually occurs 2-2.5 years after breast bud development. Peak height velocity in girls occurs at breast stage 2-3 and virtually always precedes menarche.
The onset of puberty in boys is at 11.5 years. The first sign is testicular enlargement (>3 ml) and thinning of the scrotum.
This is followed by the pigmentation of the scrotum and growth of the penis, and pubic hair follows.
Peak height velocity (growth spurt) is two years later in boys than in girls and occurs at testicular stage 4-5 (i.e. testicular volume 10-12 ml), which is around 13-14 years of age.
Breast enlargement occurs in 40-60% of boys (rarely, significant enough to cause social embarrassment in 10%) and is a result of oestradiol produced by the metabolism of testosterone.
It usually resolves within three years. During puberty, elongation of the eye often occurs, causing short-sightedness.

Standard deviation is defined as the measure of dispersion of a set of data from its mean. It measures the absolute variability of a distribution; the higher the dispersion or variability, the greater is the standard deviation and greater will be the magnitude of the deviation of the value from their mean.

Psoriasis is an autoimmune dermatological condition that causes cells of the epidermal layer to rapidly develop, leading to itchy scaly plaques. Phototherapy with UV radiation has emerged as a new treatment for difficult to treat psoriasis. The therapy works by inducing epidermal cell apoptosis by DNA damage through the formation of pyrimidine dimerization, disrupting the cell cycle. This slows the rapid proliferation of cells.

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

Mature human breast milk is nutrient and enzyme rich, optimal for human infants. The average calorie requirement for a one month old infant is about 100kcal/kg/day. Lactose, the most abundant carbohydrate, is beneficial for the baby’s developing gastro intestinal system. The proteins in mature milk are mostly whey (as compared to a high proportion of casein in cows milk), which digests easily. Other proteins found in breast milk include immunoglobulin, lysozyme, lactoferrin and lactalbumin. In terms of micronutrients, while Vitamin K levels are low in breast milk, Vitamin A, C and E are found in higher concentrations than in cow’s milk.

Doctors must perform an infection screen and treat with intravenous antibiotics for at least 48 hours pending results to prevent neonatal sepsis. Risk factors for neonatal sepsis include rupture of the membranes.
Risk factors for neonatal sepsis include rupture of membranes greater than 12-24 hours, intrapartum maternal pyrexia (> 38°C), fetal tachycardia, chorioamnionitis, pre-term birth and maternal colonisation with group B Streptococcus. In this case there are two risk factors: prolonged rupture of membranes, and pre-term birth.

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.

Persistent pulmonary hypertension of the new-born is secondary to the failure of normal circulatory transition at birth, leading to an abnormally high pulmonary vascular resistance. This elevated resistance causes right-to-left shunting of blood and hypoxemia. It can be caused by parenchymal lung diseases (meconium aspiration syndrome, pneumonia or ARDS), lung hypoplasia (like occurring in oligohydramnios or diaphragmatic hernia), or it can be idiopathic. Other possible causes include maternal indomethacin use, group B streptococcal septicaemia, and high-pressure ventilation. Duct dependent congenital heart disease does not lead to persistent pulmonary hypertension.

Cortisol is secreted in a circadian pattern.
The secretion of cortisol is regulated by the suprachiasmatic nucleus located in the hypothalamus.

Other options:
– FSH, LH, GH and prolactin are secreted in a pulsatile pattern, i.e. these hormones are secreted in an episodic manner rather than continuously.
– Thyroxine is secreted in a continuous pattern, not pulsatile.
– The secretion of ACTH is in response to stress.
Secondary to stress, the hypothalamus secretes corticotrophin releasing hormones, which are transported to the pituitary gland via the hypophyseal-portal system.
ACTH is then released by the pituitary gland and binds to its receptor on the adrenal gland, which releases cortisol.

Mongolian spots, otherwise called congenital dermal melanocytosis, are pigmented birthmarks. They are usually located on the buttocks or back and although they’re usually present at birth, they may appear soon after. They are flat and have a blue-grey colour (bruise-like). They are benign and present no health risk.

The clinical presentation and leucocytes on the urine dipstick is suggestive of a urinary tract infection. To confirm the diagnosis, urine should be sent for culture and sensitivity.

Cafe-au-lait spots is hyperpigmented lesions that vary in colour from light brown to dark brown, with borders that may be smooth or irregular. Causes include:

Neurofibromatosis type I

McCune-Albright syndrome

Legius syndrome

Tuberous sclerosis

Fanconi anaemia

Idiopathic

Ataxia-telangiectasia

Basal cell nevus syndrome

Benign congenital skin lesion

Bloom syndrome

Chediak-Higashi syndrome

Congenital nevus

Gaucher disease

Hunter syndrome

Maffucci syndrome

Multiple mucosal neuroma syndrome

Noonan syndrome

Pulmonary Stenosis

Silver-Russell syndrome

Watson syndrome

Wiskott-Aldrich syndrome

Familial adenomatous polyposis can have different inheritance patterns.

When familial adenomatous polyposis results from mutations in the APC gene, it is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In most cases, an affected person has one parent with the condition.

When familial adenomatous polyposis results from mutations in the MUTYH gene, it is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. Most often, the parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but do not show signs and symptoms of the condition.

Gitelman syndrome is a kidney disorder that causes an imbalance of charged atoms (ions) in the body, including ions of potassium, magnesium, and calcium.

The signs and symptoms of Gitelman syndrome usually appear in late childhood or adolescence. Common features of this condition include painful muscle spasms (tetany), muscle weakness or cramping, dizziness, and salt craving. Also common is a tingling or prickly sensation in the skin (paraesthesia), most often affecting the face. Some individuals with Gitelman syndrome experience excessive tiredness (fatigue), low blood pressure, and a painful joint condition called chondrocalcinosis. Studies suggest that Gitelman syndrome may also increase the risk of a potentially dangerous abnormal heart rhythm called ventricular arrhythmia.

DiGeorge syndrome is one of the most common microdeletion syndromes, resulting from 22q11 deletion. 10% of the cases can be inherited in an autosomal dominant fashion, while 90% are sporadic. The syndrome is characterized by a deficiency of both T and B-cell lines along with hearing loss, 20-fold increased lifetime chances of developing schizophrenia, renal abnormalities, congenital heart defects, and a borderline or low IQ. Distinctive facial features include micrognathia, long face, short philtrum, cleft palate, and small teeth.

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 illness

Management:
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.

Nephrogenic Diabetes Insipidus is an acquired or hereditary condition that affects the water balance. It presents with polyuria and polydipsia, leading commonly to dehydration.

In this particular case, the symptoms and signs are suggestive for congenital dislocation of the hip. There are several risk factors present including, a breech delivery, female gender (female:male = 6:1) and oligohydramnios. It is also more common in the left hip than the right.

This child is experiencing delayed speech. By the age of 2, a child should be able to combine at least 2 words to make a very simple sentence.

According to the Rumack-Matthew Normogram, accurate levels of paracetamol can be measured only between 4-15 hours. The levels measured before 4 hours post-ingestion would highly underestimate the level of paracetamol ingested.
The patient in question presents 2 hours after ingestion. Hence the most appropriate time to measure the levels of acetaminophen would be a further 2 hours later.

Other options:
– Note that the treatment with NAC can be delayed until the 4-hour level is obtained and compared to the nomogram.
– Furthermore, haemodialysis is only indicated if she develops hepato-renal syndrome that is likely to occur 72-96 hours post-ingestion.
– Indications for acute liver transplantation include persistent acidosis, hepatorenal syndrome and worsening coagulopathy.

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.

The trend in the management of splenic injury continues to favour nonoperative or conservative management.

In Paediatrics, Blunt splenic injuries with hemodynamic stability and absence of other internal injuries requiring surgery should undergo an initial attempt of Non-operative Management (NOM) irrespective of injury grade.
In hemodynamically stable children with isolated splenic injury, splenectomy should be avoided.
NOM is contraindicated in the presence of peritonitis, bowel evisceration, impalement or other indications to laparotomy.
The vast majority of paediatric patients do not require angiography/angioembolization (AG/AE) for CT blush or moderate to severe injuries.
AG/AE may be considered in patients undergone to NOM, hemodynamically stable with sings of persistent haemorrhage not amenable of NOM, regardless with the presence of CT blush once excluded extra-splenic source of bleeding.

During lumbar puncture, the first structures encountered by the needle are skin and subcutaneous tissue. But, since they are not provided in the options, the most superficial structure after them is the supraspinous ligament.

Lumbar puncture is a procedure performed to obtain cerebrospinal fluid. The procedure is best performed at the level of L3/L4 or L4/5 interspace. During the procedure, the needle pierces the following structures in order from superficial to deep:
Skin, subcutaneous tissue, supraspinous ligament, interspinous ligament, ligamentum flavum, the epidural space containing the internal vertebral venous plexus, dura, and arachnoid, finally entering the subarachnoid space.
The supraspinous ligament connects the tips of spinous processes and the interspinous ligaments between adjacent borders of spinous processes.

As the needle penetrates the ligamentum flavum, it causes a give. A second give is felt when the needle penetrates the dura mater and enters the subarachnoid space.
At this point, clear CSF flows through the needle and can be collected for diagnostic purposes.

Classic Kallmann syndrome (KS) is due to isolated hypogonadotropic hypogonadism. Most patients have gonadotropin-releasing hormone (GnRH) deficiency, as suggested by their response to pulsatile GnRH therapy. The hypothalamic-pituitary function is otherwise normal in most patients, and hypothalamic-pituitary imaging reveals no space-occupying lesions. By definition, either anosmia or severe hyposmia is present in patients with Kallmann syndrome.

Removal of foreign bodies from the ear is indicated whenever a well-visualized foreign body is identified in the external auditory canal. Insects are better extracted with suction than with forceps or hooks. From the given answers, irrigation with warm water is the most suitable answer.

The umbilical cord that connects the fetus to the placenta is about 50cm long. This tissue consists of the body stalk and vitelline duct. The former containing the allantoic diverticulum and the umbilical vessels. The latter contains the connection linking the digestive tube and the yolk sac. This cord is wrapped by stratum of ectoderm and gelatinous tissue or jelly of Wharton. The right umbilical vein plus the vitelline vessels and ducts disappear and this at birth the cord has three vessels which are the umbilical vein and two umbilical arteries.