Understanding Vesicoureteric Reflux

Vesicoureteric reflux (VUR) is a condition where urine flows back from the bladder into the ureter and kidney. This is a common urinary tract abnormality in children and can lead to urinary tract infections (UTIs). In fact, around 30% of children who present with a UTI have VUR. It is important to investigate for VUR in children following a UTI as around 35% of children develop renal scarring.

The pathophysiology of VUR involves the ureters being displaced laterally, which causes a shortened intramural course of the ureter. This means that the vesicoureteric junction cannot function properly. VUR can present in different ways, such as hydronephrosis on ultrasound during the antenatal period, recurrent childhood UTIs, and reflux nephropathy, which is chronic pyelonephritis secondary to VUR. Renal scarring can also produce increased quantities of renin, which can cause hypertension.

To diagnose VUR, a micturating cystourethrogram is usually performed. A DMSA scan may also be done to check for renal scarring. VUR is graded based on the severity of the condition, with Grade I being the mildest and Grade V being the most severe.

Overall, understanding VUR is important in preventing complications such as UTIs and renal scarring. Early diagnosis and management can help improve outcomes for children with this condition.

It is recommended that all children who experience an acute exacerbation of asthma receive a short course of oral steroids, such as 3-5 days of prednisolone, along with a salbutamol inhaler. This approach should be taken regardless of whether the child is typically on an inhaled corticosteroid. It is important to ensure that patients have an adequate supply of their salbutamol inhaler and understand how to use it. Prescribing antibiotics is not necessary unless there is an indication of an underlying bacterial chest infection. Beclomethasone may be useful for long-term prophylactic management of asthma, but it is not typically used in short courses after acute exacerbations. A course of 10 days of prednisolone is longer than recommended and may not be warranted in all cases. A salbutamol inhaler alone would not meet the recommended treatment guidelines for acute asthma.

Managing Acute Asthma Attacks in Children

When it comes to managing acute asthma attacks in children, it is important to assess the severity of the attack and take appropriate action. For children between the ages of 2 and 5, those with severe or life-threatening asthma should be immediately transferred to the hospital. For moderate attacks, children should have a SpO2 level above 92% and no clinical features of severe asthma. However, for severe attacks, children may have a SpO2 level below 92%, be too breathless to talk or feed, have a heart rate above 140/min, and use accessory neck muscles. For life-threatening attacks, children may have a SpO2 level below 92%, a silent chest, poor respiratory effort, agitation, altered consciousness, and cyanosis.

For children over the age of 5, it is recommended to attempt to measure PEF in all cases. For moderate attacks, children should have a SpO2 level above 92%, a PEF level above 50% best or predicted, and no clinical features of severe asthma. For severe attacks, children may have a SpO2 level below 92%, a PEF level between 33-50% best or predicted, and be unable to complete sentences in one breath or too breathless to talk or feed. For life-threatening attacks, children may have a SpO2 level below 92%, a PEF level below 33% best or predicted, a silent chest, poor respiratory effort, altered consciousness, and cyanosis.

For children with mild to moderate acute asthma, bronchodilator therapy should be given via a beta-2 agonist and spacer (or close-fitting mask for children under 3 years old). One puff should be given every 30-60 seconds up to a maximum of 10 puffs. If symptoms are not controlled, the beta-2 agonist should be repeated and the child should be referred to the hospital. Steroid therapy should also be given to all children with an asthma exacerbation for 3-5 days, with the usual prednisolone dose varying based on age and weight.

Uncommon Clinical Features of Cardiac Failure in Infancy

Ascites, or the accumulation of fluid in the abdomen, is a rare occurrence in infants with cardiac failure. Additionally, there are several other uncommon clinical features that may be observed in these cases. Bibasal crackles, which are abnormal sounds heard during breathing, are not commonly present. Raised jugular venous pressure, which is an indication of increased pressure in the heart, is also not frequently seen. A third heart sound, which is an extra sound heard during a heartbeat, and pulsus alternans, which is a regular alternation of strong and weak pulses, are also uncommon in infants with cardiac failure. These features may be helpful in distinguishing cardiac failure from other conditions in infants.

The appropriate test for school entry hearing screening in most areas of the UK is pure tone audiometry. This test is suitable for individuals over the age of 3 and involves playing sounds at varying frequencies and volumes, with the patient indicating when they can no longer hear the sound. This test helps to identify the presence of hearing loss, the affected frequencies, and the severity of the condition.

The auditory brainstem response test, distraction test, and otoacoustic emission test are not suitable for school entry hearing screening. The auditory brainstem response test is typically performed on newborns and infants with abnormal otoacoustic emission test results. The distraction test is used for children aged 6-9 months to assess their ability to hear and locate sounds. The otoacoustic emission test is part of the newborn hearing screening program and is used to determine the health of the cochlea.

Hearing Tests for Children

Hearing tests are important for children to ensure that they are developing normally. There are several tests that may be performed on children of different ages. For newborns, an otoacoustic emission test is typically done as part of the Newborn Hearing Screening Programme. This test involves playing a computer-generated click through a small earpiece and checking for the presence of a soft echo, which indicates a healthy cochlea. If the results of this test are abnormal, an Auditory Brainstem Response test may be done.

For infants between 6-9 months, a Distraction test may be performed by a health visitor with the help of two trained staff members. For children between 18 months to 2.5 years, a Recognition of familiar objects test may be used, which involves using familiar objects like a teddy or cup and asking the child simple questions like where is the teddy? For children over 2.5 years, Performance testing and Speech discrimination tests may be used, such as the Kendall Toy test or McCormick Toy Test. Pure tone audiometry is typically done at school entry in most areas of the UK for children over 3 years old.

In addition to these tests, there is also a questionnaire for parents in the Personal Child Health Records called Can your baby hear you? It is important for parents to be aware of these tests and to have their child’s hearing checked regularly to ensure proper development.

Pyloric stenosis is the probable diagnosis when a newborn experiences non-bilious vomiting during the first few weeks of life. This condition results in the loss of hydrochloric acid (HCl) from the stomach contents, leading to hypochloremia and potassium loss. The metabolic alkalosis is caused by the depletion of hydrogen ions due to the vomiting of stomach acid.

Understanding Pyloric Stenosis

Pyloric stenosis is a condition that usually occurs in infants between the second and fourth weeks of life. However, in rare cases, it may present later, up to four months. This condition is caused by the thickening of the circular muscles of the pylorus. Pyloric stenosis is more common in males, with an incidence of 4 per 1,000 live births. It is also more likely to affect first-borns and infants with a positive family history.

The most common symptom of pyloric stenosis is projectile vomiting, which usually occurs about 30 minutes after a feed. Other symptoms may include constipation, dehydration, and a palpable mass in the upper abdomen. Prolonged vomiting can lead to hypochloraemic, hypokalaemic alkalosis, which can be life-threatening.

Diagnosis of pyloric stenosis is typically made using ultrasound. Management of this condition involves a surgical procedure called Ramstedt pyloromyotomy. This procedure involves making a small incision in the pylorus to relieve the obstruction and allow for normal passage of food. With prompt diagnosis and treatment, infants with pyloric stenosis can make a full recovery.

Guidelines for Imaging in Atypical UTIs in Children

When a child presents with an atypical urinary tract infection (UTI), imaging is necessary to identify any structural abnormalities in the urinary tract. The National Institute for Health and Care Excellence (NICE) guidelines provide recommendations for imaging based on the age of the child and the severity of the infection.

For children under 6 months of age with an atypical UTI, an urgent ultrasound scan (USS) is required during the acute infection. Once the infection has resolved, a routine dimercaptosuccinic acid (DMSA) scan and a micturating cystourethrogram (MCUG) are performed 4-6 months later.

For children older than 6 months with recurrent UTIs, a routine USS and DMSA scan plus MCUG are recommended. However, for children aged 6 months to 3 years with an atypical UTI, an urgent USS followed by a routine DMSA is sufficient. An MCUG is only performed if there is any dilation identified on USS, poor urine flow, family history of vesico-ureteric reflux, or a non-E. coli infection.

It is important to follow these guidelines to ensure appropriate imaging and management of atypical UTIs in children.

Friedreich’s ataxia is inherited in an autosomal recessive manner. This is the most common type of hereditary ataxia and typically presents with symptoms before the age of 25, including ataxia, cardiomyopathy, motor weakness, pes cavus foot deformity, and scoliosis. It should be noted that Friedreich’s ataxia is not inherited in an autosomal dominant or X-linked recessive manner, nor is it caused by mitochondrial dysfunction.

Autosomal Recessive Conditions

Autosomal recessive conditions are genetic disorders that occur when an individual inherits two copies of a mutated gene, one from each parent. These conditions are often referred to as ‘metabolic’ as they affect the body’s metabolic processes. However, there are notable exceptions, such as X-linked recessive conditions like Hunter’s and G6PD, and autosomal dominant conditions like hyperlipidemia type II and hypokalemic periodic paralysis.

Some ‘structural’ conditions, like ataxia telangiectasia and Friedreich’s ataxia, are also autosomal recessive. The following conditions are examples of autosomal recessive disorders: albinism, congenital adrenal hyperplasia, cystic fibrosis, cystinuria, familial Mediterranean fever, Fanconi anemia, glycogen storage disease, haemochromatosis, homocystinuria, lipid storage disease (Tay-Sach’s, Gaucher, Niemann-Pick), mucopolysaccharidoses (Hurler’s), PKU, sickle cell anemia, thalassemias, and Wilson’s disease.

It is worth noting that Gilbert’s syndrome is still a matter of debate, and many textbooks list it as autosomal dominant. Nonetheless, understanding the inheritance patterns of these conditions is crucial for genetic counseling and management.

Acute epiglottitis is a rare but serious infection caused by Haemophilus influenzae B (HiB) bacteria. It is characterized by a sudden onset of fever, stridor, and drooling due to inflammation of the epiglottis. It is important to keep the affected child calm and seek specialist input from anaesthetics and paediatrics. In the UK, the current vaccination against HiB has made epiglottitis uncommon. Bordetella pertussis, Streptococcus pneumoniae, and Parainfluenza virus are incorrect answers as they do not produce the same presentation as acute epiglottitis.

Acute epiglottitis is a rare but serious infection caused by Haemophilus influenzae type B. It is important to recognize and treat it promptly as it can lead to airway obstruction. Although it was once considered a disease of childhood, it is now more common in adults in the UK due to the immunization program. The incidence of epiglottitis has decreased since the introduction of the Hib vaccine. Symptoms include a rapid onset, high temperature, stridor, drooling of saliva, and a tripod position where the patient leans forward and extends their neck to breathe easier. Diagnosis is made by direct visualization, but x-rays may be done to rule out a foreign body.

Immediate senior involvement is necessary, including those who can provide emergency airway support such as anaesthetics or ENT. Endotracheal intubation may be necessary to protect the airway. It is important not to examine the throat if epiglottitis is suspected due to the risk of acute airway obstruction. The diagnosis is made by direct visualization, but only senior staff who are able to intubate if necessary should perform this. Treatment includes oxygen and intravenous antibiotics.

If the onset of cough is within the previous 21 days, the recommended first-line treatment for whooping cough is a course of oral clarithromycin or azithromycin. In this case, the correct answer is to start a course of oral clarithromycin as the patient’s history is consistent with whooping cough. Starting treatment within 21 days of onset of the cough can help to reduce the risk of spread. It is incorrect to not start any treatment as the patient has presented within the appropriate timeframe. Offering an immediate booster vaccination is also not indicated in the initial management of the index case, and starting a course of oral doxycycline is not the first-line treatment for whooping cough.

Whooping Cough: Causes, Symptoms, Diagnosis, and Management

Whooping cough, also known as pertussis, is a contagious disease caused by the bacterium Bordetella pertussis. It is commonly found in children, with around 1,000 cases reported annually in the UK. The disease is characterized by a persistent cough that can last up to 100 days, hence the name cough of 100 days.

Infants are particularly vulnerable to whooping cough, which is why routine immunization is recommended at 2, 3, 4 months, and 3-5 years. However, neither infection nor immunization provides lifelong protection, and adolescents and adults may still develop the disease.

Whooping cough has three phases: the catarrhal phase, the paroxysmal phase, and the convalescent phase. The catarrhal phase lasts around 1-2 weeks and presents symptoms similar to a viral upper respiratory tract infection. The paroxysmal phase is characterized by a severe cough that worsens at night and after feeding, and may be accompanied by vomiting and central cyanosis. The convalescent phase is when the cough subsides over weeks to months.

To diagnose whooping cough, a person must have an acute cough that has lasted for 14 days or more without another apparent cause, and have one or more of the following features: paroxysmal cough, inspiratory whoop, post-tussive vomiting, or undiagnosed apnoeic attacks in young infants. A nasal swab culture for Bordetella pertussis is used to confirm the diagnosis, although PCR and serology are increasingly used.

Infants under 6 months with suspected pertussis should be admitted, and in the UK, pertussis is a notifiable disease. An oral macrolide, such as clarithromycin, azithromycin, or erythromycin, is indicated if the onset of the cough is within the previous 21 days to eradicate the organism and reduce the spread. Household contacts should be offered antibiotic prophylaxis, although antibiotic therapy has not been shown to alter the course of the illness. School exclusion is recommended for 48 hours after commencing antibiotics or 21 days from onset of symptoms if no antibiotics are given.

Complications of whooping cough include subconjunctival haemorrhage, pneumonia, bronchiectasis, and

VSD and Heart Sounds

Ventricular septal defect (VSD) is a heart condition that usually becomes apparent after the first month of life and is characterized by pulmonary plethora. However, most cases of VSD resolve on their own. If central cyanosis is present, it indicates shunt reversal and pulmonary hypertension, which are associated with a poor prognosis and a low likelihood of responding to surgical repair of the VSD.

The second heart sound is typically split, which means that the aortic (A2) and pulmonary (P2) components of the sound are separated. This splitting is considered normal or physiological and only occurs during inspiration, when P2 comes after A2. During expiration, there is no splitting, and only a single S2 is heard.

Fixed splitting, on the other hand, is a feature of atrial septal defect (ASD), not VSD. This occurs when P2 is delayed and comes after A2 during both inspiration and expiration. Reversed splitting is associated with severe aortic stenosis and occurs when A2 comes after P2. these heart sounds and their associations with different heart conditions can aid in the diagnosis and management of VSD.