CH WK 3 CASE 2

You are working on the Paediatric Assessment Unit. Jack is a three month old boy who presents with a three day history of poor feeding and high temperature.

Please give 2 questions you may ask mum and why they may assist your diagnosis:

1. How has he been behaving?

· In young babies with fever, quiet sleepy behavior or unsettled behavior on handling support a diagnosis of significant sepsis.

· Questions regarding how he is feeding also provides information regarding his alertness

2. How high was his fever?

· In older children, the height of the fever bears little relationship to the likelihood of the cause being bacterial or viral

· In infants less than 3 months a fever greater than 38.0 indicates a greater likelihood of bacterial sepsis.

Mother is concerned today because he is more sleepy and quiet than usual. He had had a temperature of 38.7 measured at home.

What else would you ask about his current presentation?

Why could Jack’s past history be important?

Questions regarding current presentation:

· Try to identify a focus for his fever, e.g. a history of cough, coryza and difficulty in breathing point to a respiratory cause of his fever

· Poor urine output may be nonspecific. There can be smelly urine in urinary tract infection

Antenatal history:

· A history of antenatal abnormalities such as renal pelvis dilatation, cysts, or poor production of amniotic fluid (oligohydramnios) may indicate antenatal renal abnormalities predisposing to infection.

· Prematurity may indicate an increased overall infection risk

Past history may be suggestive of an underlying problem requiring further investigation

Family History:

Some disorders of the renal tract such as Vesico-ureteric Reflux can run in families (although are not in general single gene defects), Screening may be indicated

Jack was born at term, Mother’s antenatal scan at 20 weeks gestation was reported as normal. Jack does not have any medical problems and is not taking any regular medication. There is no significant family history.

He has been taking approximately half the amount of his milk feeds that he would usually take and is nappies have been less wet than before, she thought his urine was a bit stronger than usual.

What aspects of Jack’s Examination findings will help you decide how unwell Jack is?

· Capillary refill time

· Blood pressure

· Alertness

· Heart rate

· O2 saturations

Capillary refill time

.Correct answer.

· Blood pressure

.Correct answer.

Note: Abnormalities of blood pressure are a late sign in serious illness.

· Alertness

.Correct answer.

· Heart rate

.Correct answer.

· O2 saturations

.Correct answer.

On examination, Jack’s temperature is 38.5°C, heart rate 167 bpm, capillary refill time <2 sec, respiratory rate 48/min, SaO2 97% in air, BP 78/48mmHg. He appears well and is responsive. Examination of his chest, heart, abdomen and neurology does not reveal any significant abnormalities.

Calculate Jack's PEWS score using the chart below.

Paediatric Early Warning Score (PEWS) Chart

Respiratory rate: 48 = 1

Oxygen requirement: 97% in air = 0

Pulse rate: 167 = 1

Blood pressure: 78/48 = 0

Capillary refill time: <2 seconds = 0

Conscious level: Responds to voice = 2

TOTAL SCORE = 4

Should you be concerned about Jack? If so, why?

Yes: Jack is a young baby with a high fever and Tachycardia, although he does not have signs of shock, this may indicate early signs of serious illness in this age-group.

What is your differential diagnosis?

Sepsis; Urinary tract infection needs to be excluded. It is also important to consider septicaemia and meningitis, although these are less likely since Jack is well with normal examination findings. Viral infection is a possibility.

Note: That an infant under 3 months of age would need to have consideration of a full septic screen.

Which investigations would you carry out at this point?

· Urine microscopy and culture

· Full blood count

· CRP

· Blood culture

· Chest x-ray

Urine microscopy and culture

.Correct answer.

· Full blood count

.Correct answer.

· CRP

.Correct answer.

· Blood culture

.Correct answer.

· Chest x-ray

.Consider chest X ray if red risk factors are present OR there are respiratory signs OR fever is greater than 39 0C and WBC greater than 20 x 10 9/L

See Fever in under 5s: assessment and initial management, NICE guidance (link) to review red, amber and green risk factors:

Recommendations – Fever in under 5s: assessment and initial management – Clinical guideline [CG160]

Task

See children of different ages presenting to the assessment unit or ward with signs and symptoms of infection. Ask when a urine specimen should be requested. What is a full septic screen?

How you would obtain a urine sample from an infant?

Clean catch sample is the recommended method for urine collection. This can be very time consuming. If this is unobtainable, urine collection pads can be used but not cotton wool balls or gauze.

When it is not possible to use non-invasive methods, a catheter sample or suprapubic aspiration (SPA) should be used. Ultrasound guidance prior to SPA is advisable to demonstrate the presence of urine.

Urine dipstick is a useful screening test for urinary tract infection in children of all ages

Leucocyte esterase sensitivity 83%, specificity 78%

Nitrite sensitivity 53%, specificity 93%

Jack’s urine dipstick results are as follows:

· Nitrite positive

· Leucocyte esterase positive

Microscopy results

What does this result indicate?

Urinary tract infection is likely – send a clean catch samples or a catheter / Supra pubic aspirate (SPA) sample for microscopy, culture and sensitivity (M,C&S) and start antibiotics whilst awaiting urine culture result.

The below tables are useful guides. Urine culture result will tend to guide care.

Dipstix results

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Microscopy results

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What other aspects are looked at in the urine dipstick?

Watch this short presentation on proteinuria and nephrotic syndrome by Dr Rachel Lennon:

Video Player http://www.taibahumbbs.com/wp-content/uploads/2017/09/proteinuria-and-nephrotic-syndrome-shortened-version-dr-rachel-lennon_hd.mp4?_=1

Proteinuria and Nephrotic Syndrome (Shortened Version) – Dr Rachel Lennon

In Renal disease, what are the significant levels of proteinuria?

>20 mmol/ml - This may be significant, and may indicate tubular disease

>200 mmol/mg - This is nephrotic range

Which of the following indicate a diagnosis of nephrotic syndrome?

· Proteinuria >200 mg/mmol

· Low serum albumin <25mmol/l

· Pallor

. Swelling

· Hypertension

Proteinuria >200 mg/mmol

.Correct answer.

· Low serum albumin <25mmol/l

.Correct answer.

· Pallor

.· Swelling

.Correct answer.

· Hypertension

Further feedback for question above:

Nephrotic syndrome is defined as a triad of the above findings.

Which complications of minimal change nephrotic syndrome are recognised in childhood?

· Recurrent disease

· Haemolysis

.· End stage renal failure is common

.· Infection with streptococci

· Spontaneous peritonitis

Recurrent disease

.Correct answer.

· Haemolysis

.· End stage renal failure is common

.· Infection with streptococci

.Correct answer.

· Spontaneous peritonitis

.Correct answer.

Further feedback for question above:

Infection with encapsulated organisms are common and can be fulminant. Penicillin prophylaxis is used, as ascites is common, spontaneous peritonitis can occur and careful assessment of fever in children in this group is essential. Most children make a full recovery, and renal impairment is unusual, but relapse is not uncommon.

Which of the following make a diagnosis of Minimal Change Nephrotic Syndrome less likely (meaning further investigations would be indicated)?

· Haematuria

· Good intial response to steroids

.· Hypertension

· Abormal renal function

· Very low serum albumin

Haematuria

.Correct answer.

· Good intial response to steroids

.· Hypertension

.Correct answer.

· Abormal renal function

.Correct answer.

· Very low serum albumin

Further feedback for question above:

The above factors would be indications that further investigations may be necessary.

The following short presentation by Professor Adrian Woolf will give you an overview on congenital abnormalities of the renal tract:

https://youtu.be/-XHgOFkteI8

https://youtu.be/6KQUIEQNnfU

Congenital abnormalities of kidney and urinary tract (Shortened Version) – Professor Adrian Woolf

This link summarises common urogenital anomalies in childhood, which also commonly occur as part of genetic syndromes:

· Congenital Urogenital Malformations

Please answer the following questions relevant to the above resources:

When in gestation do the kidneys begin to form?

5 weeks with glomeruli still forming until 34 weeks

What is the commonest congenital renal anomaly?

Renal Hypoplasia

Give 2 reasons why is it important to understand that there may be a genetic basis to an abnormality seen in renal tract development.

1. There are important associations with abnormalities in other systems for example the need for screening children with particular abnormalities of the ear for renal abnormalities giving the opportunity for monitoring and treating infections and hypertension early to preserve renal function.

2. The screening of first degree relatives for example for vesicoureteruc reflux may help prevent uncontrolled infection and therefore scarring in this group.

Some key points:

· Inguinal hernias in infancy may easily become incarcerated and therefore require intervention.

· Undescended testes are common but if persistent need treatment to preserve fertility.

In hypospadias it is crucial to advise against circumcision prior to surgery as the foreskin is used in the repair process.

Task

Interpret a urine dipstick result on the ward or assessment unit during your placement.

The urine microscopy and culture result is as follows:

>100 white cells, 0 red cells
Pure growth of E coli >10 8/L

Blood culture was negative

You have confirmed the diagnosis of urinary tract infection.

Urinary tract infection is a common condition in childhood with a prevalence of 12% in girls and 4% in boys by age 16 years.

The highest incidence is in the first year of life and declines thereafter. Under 1 year of age, it is more common in boys and over 1 year it is more common in girls. E coli is the commonest pathogen. Mixed growth is usually contamination. If in doubt, repeat the sample.

Which of the following factors would influence your choice of antibiotics?

· Age of the child

· Does the child have an upper or lower urinary tract infection?

· Was the child already on a prophylactic antibiotic?

· How unwell is the child?

· Is the child unable to tolerate oral antibiotics due to vomiting?

Age of the child

.Correct answer.

· Does the child have an upper or lower urinary tract infection?

.Correct answer.

· Was the child already on a prophylactic antibiotic?

.Correct answer.

· How unwell is the child?

.Correct answer.

· Is the child unable to tolerate oral antibiotics due to vomiting?

.Correct answer.

Further feedback for question above:

These are all factors you should consider when prescribing antibiotics. Also, remember to ask about drug allergies.

Task

Look up your Local Antibiotic Policy and practice prescribing intravenous and oral antibiotics for different conditions during your placement.

Now review the summary guidance on UTI in Childhood:

· Acute management of urinary tract infection in children – NICE Clinical Pathway

· Urinary tract infection: Children – NICE Clinical Knowledge Summary

List 5 possible risk factors for UTI and serious underlying pathology

Follow the link below for further guidance:

· Urinary tract infection in children and young people - Quality standard [QS36]

· poor urine flow

· history suggesting previous UTI or confirmed previous UTI

· recurrent fever of uncertain origin

· antenatally-diagnosed renal abnormality

· family history of vesicoureteric reflux (VUR) or renal disease

· constipation

· dysfunctional voiding

· enlarged bladder

· abdominal mass

· spinal lesion

· poor growth

· high blood pressure.

In reviewing the NICE guidance, note that it is important to identify when a UTI is atypical or recurrent as this will determine further investigations.

Fever without a focus – Further Case Information

In case Fever without a focus

Jack responds promptly to oral antibiotics and his parents are keen to take him home.

Which investigation do you need to arrange?

· Renal ultrasound scan before discharge

. Renal ultrasound scan within 6 weeks

· DMSA

. MCUG

. Abdominal x-ray

Renal ultrasound scan within 6 weeks

A 1 month old baby boy is admitted with pyrexia 38.5 C and urine culture grows Enterococcus > 10^5 organisms.

Which of the following investigations should be arranged according to NICE Guidelines?

· US scan Mag3 and MCUG

· US scan, DMSA and MCUG

· US scan and DMSA scan

· Repeat urine culture and no additonal investigations

· US scan of kidneys 6 weeks after discharge

US scan, DMSA and MCUG

Why do we carry out investigations in children with proven urinary infections?

Research looking at adults with end stage renal disease has found that large numbers of those affected have associated anatomical renal tract abnormalities and a history of infection. The aim is to detect these children early and prevent renal damage by prompt treatment of urinary infections. Up to to 50% of infants with UTI may have a structural abnormality such as vesicoureteric reflux, hydronephrosis, duplex system or obstructive lesions. Pyelonephritis may lead to renal scarring, predisposing to hypertension and chronic kidney disease. It is standard practice also to use antibiotic prophylaxis daily in theory to prevent infections.

3 investigations are used selectively depending on the age of the child and presence of atypical or recurrent UTI symptoms: Ultrasound scan, MCUG and DMSA.

Renal ultrasound scan to look at the shape, size and location of the kidneys. US will also identify any hydronephrosis (renal pelvic dilatation) which is usually either due to obstruction or more commonly due to vesicoureteric reflux. US is also useful to assess bladder emptying in older children.

Indications:

· Infants less than 6 months with confirmed UTI (acutely if infant has atypical or recurrent UTI)

· Children more than 6 months old only in the presence of atypical UTI

Micturating cystogram (MCUG) to identify any vesicoureteric reflux (VUR), bladder abnormalities and posterior urethral valves within a few weeks after treatment of UTI The technique consists of catheterizing the child in order to fill the bladder with a radio-contrast agent then taking x-rays as the infant voids urine..

Indications:

· Infants younger than 6 months with atypical or recurrent UTI

· Consider in children older than 6 months if dilatation on ultrasound, poor urine flow, non-E coli infection or family history VUR

DMSA in 4-6 months. This is a radionucleotide scan used to assess renal function and identify any scarring of the kidneys due to the UTI. Healthy renal tissue takes up the isotope. Unhealthy or scarred tissue doesn’t take up the isotope and appears as a filing defect on DMSA scan. DMSA may be inaccurate if performed shortly after an infection.

ndications:

· All children with recurrent UTI

· Children under 3 years with atypical UTI

Is follow up required and if so why?

Follow up is not routinely required after a single UTI if the investigations are normal. If there is VUR, then antibiotic prophylaxis to prevent UTI can be considered. Low grade VUR (No dilatation of ureter or renal pelvis) usually spontaneously resolves by the age of 3-4 years whereas prognosis for high grade reflux is less good and affected children are at risk of recurrent UTI and if untreated, damage to the kidneys. If the DMSA shows scarring of the kidney, there is about 10% risk of developing hypertension in the longer term. The child will therefore require annual assessment of BP.

What advice would you give to parents in case of future similar episodes?

In the event of any febrile illness without an obvious focus, urine should be collected for M, C&S and antibiotics commenced if required, pending the result of the urine culture. This approach in theory should minimize scarring occurring. Antibiotic treatment should be tailored to sensitivities of the organism detected where possible as resistance is common. The evidence for long term preventative antibiotic treatment is less clear, but in those children at high risk this is used routinely in the UK according to the NICE guidance. The advice given to parents is very important, including the nature and reasons for investigations.

Task

During your placement: See children with urinary tract problems in outpatients. Check their blood pressure, weight and height and urine dipstick.

How else can urinary infections present in children?

· Dysuria

· Constipation

· Abdominal pain

· Bedwetting/enuresis

· Febrile convulsion

· Haematuria

Dysuria

.Correct answer.

· Constipation

.Correct answer.

· Abdominal pain

.Correct answer.

· Bedwetting/enuresis

.Correct answer.

· Febrile convulsion

.Correct answer.

· Haematuria

.Correct answer.

Further feedback for question above:

The majority of younger children present with non-specific signs of unexplained fever with or without unsettled behavior.Older children may have symptoms similar to adults of frequency and dysuria.

Secondary nocturnal enuresis (when a child has already been dry for 6 months occurs commonly (with the onset of Diabetes, and emotional distress being differential diagnoses).

Constipation increases the risk of urinary infection due to inefficiency of bladder emptying and ascending infection.

Children may also present with acute abdominal pain mimicking appendicitis.

Please see the flow chart below for use in Primary Care (please concentrate on the flow chart giving guidance for children):

· Diagnosis of UTI – Quick Reference Guide for Primary Care – British Infection Association (BIA)

The following summarises the current thinking regarding enuresis:

· Management of nocturnal enuresis – BMJ (Institutional log-in required)

Regarding enuresis, which of the following statements are TRUE?

· Associated neurological disease is an important but uncommon cause of primary enuresis

· Disturbed sleep / wake cycle contributes to late acquisition of nocturnal continence

· Diabetes mellitus can lead to secondary enuresis

· Medication is the first line treatment for nocturnal enuresis

. Child abuse and neglect may lead to daytime and nighttime wetting

· A urine dipstick is a useful investigation in the evaluation of children with enuresis

Associated neurological disease is an important but uncommon cause of primary enuresis

.Correct answer.

· Disturbed sleep / wake cycle contributes to late acquisition of nocturnal continence

.Correct answer.

· Diabetes mellitus can lead to secondary enuresis

.Correct answer.

· Medication is the first line treatment for nocturnal enuresis

. Child abuse and neglect may lead to daytime and nighttime wetting

.Correct answer.

· A urine dipstick is a useful investigation in the evaluation of children with enuresis

.Correct answer.

Fever without a focus – Background Science

In case Fever without a focus

The burden of renal pathology in childhood is in general due to infection, however, both Acute and Chronic kidney disease can occur in childhood.

Which substances are excreted via the kidney?

· Waste products of metabolism; urea / creatinine

· Water

· Carbohydrates

. Electrolytes

· Medications

Waste products of metabolism; urea / creatinine

.Correct answer.

· Water

.Correct answer.

· Carbohydrates

. Electrolytes

.Correct answer.

· Medications

.Correct answer.

Further feedback for question above:

The kidneys are crucial in the regulation of fluids and electrolytes and the excretion of multiple water soluble metabolites of organic waste and drugs. In renal impairment dose adjustment of medication is therefore often needed.

Which of the following statements are TRUE regarding renal tract anatomy and physiology?

· Hormonal regulation of reabsorption in the distal tubule allows homeostasis of fluid and sodium and calcium.

· Countercurrent multiplication is the use of the osmotic gradient in the loop of Henle (surrounded by a capillary network) to absorb water to concentrate the urine.

· Passive transport uses receptors to move solute against a concentration gradient.

. The ureters enter the muscular bladder wall anteriorly

. Only the external sphincter of the urethra is under voluntary control

· The high surface area ‘brush border’ in the proximal tubule is responsible for the majority of reabsorption of glucose, salts and water.

Hormonal regulation of reabsorption in the distal tubule allows homeostasis of fluid and sodium and calcium.

.Correct answer.

· Countercurrent multiplication is the use of the osmotic gradient in the loop of Henle (surrounded by a capillary network) to absorb water to concentrate the urine.

.Correct answer.

· Passive transport uses receptors to move solute against a concentration gradient.

. The ureters enter the muscular bladder wall anteriorly

. Only the external sphincter of the urethra is under voluntary control

.Correct answer.

· The high surface area ‘brush border’ in the proximal tubule is responsible for the majority of reabsorption of glucose, salts and water.

.Correct answer.

The following review article discusses polycystic kidney disease:

Autosomal dominant polycystic kidney disease in children – BMJ (Institutional log-in required)

Please list 2 discussion points regarding the ethics of screening for polycystic kidney disease in childhood.

1. The disease course of polycystic kidney disease in general cannot be altered therefore early detection does not have a role in childhood, when the psychological burden of chronic disease may be significant.

2. Due to the complexity of the disease process is it possible for a child to give adequate consent for such screening?

Adult Polycystic Kidney Disease is Autosomal Dominantly inherited.

Further feedback for question above:

Autosomally inherited conditions are on areas of the genome separate from the sex chromosomes and therefore have equal prevalence in males and females. Spontanoeous mutations (such as in >60% achondroplasia diagnoses) and variable penetrance are common (such as in neurofibromatosis). In X linked recessive conditions such as Duchenne muscular dystrophy fathers cannot pass the condition to their sons as they give their unaffected Y chromosome to their son (making him a boy).

Acute Kidney Injury in Children

Watch this short video on the physiology of micturition:

https://youtu.be/JwaeWXhklio

CASE COMPONENT

Fever without a focus – Case Conclusion

In case Fever without a focus

This is a case of 3 month Jack presenting with nonspecific symptoms and pyrexia with no clear focus. Dipstick of a clean catch urine sample was suggestive of urinary tract infection and this was confirmed with urine M,C&S. The infant was treated with oral antibiotics for presumed acute pyelonephritis and responded within 48 hours. There were no features to suggest serious underlying pathology or atypical UTI. The baby was discharged home to complete a 7 day course of oral antibiotics and an USS renal tract was arranged as an outpatient to identify any associated renal tract anomalies.

Fever without a focus – Formative Assessment

Which of the following are 'red risk' factors in a child with fever?

· Not smiling

· Rigors

· Skin looks pale/mottled/ashen/blue

· Continuous cry

· Skin looks pale/mottled/ashen/blue

.Correct answer.

· Continuous cry

.Correct answer.

In a child with suspected urinary tract infection, which of the following statements are TRUE?

· White blood cells in the urine are a reliable guide to infection

· Nitrite on dipstick testing has low sensitivity for infection

· Antibiotics should be commenced while waiting the results of the urine culture

· Urine sample is routinely obtained using a catheter

· Nitrite on dipstick testing has low sensitivity for infection

.Correct answer.

· Antibiotics should be commenced while waiting the results of the urine culture

.Correct answer.

Which of the following are risk factors for UTI and serious underlying pathology?

· High blood pressure

· Antenatal hydronephrosis

· Family history of vesicoureteric reflux

· Poor urine flow

· High blood pressure

.Correct answer.

· Antenatal hydronephrosis

.Correct answer.

· Family history of vesicoureteric reflux

.Correct answer.

· Poor urine flow

.Correct answer.

In a child with bacteriuria, which of the following statements are TRUE?

· E coli is the commonest pathogen

· Loin pain suggests cystitis

· Antibiotics are not required if blood cultures are negative

· Pyrexia of 38.5oC suggests pyelonephritis

· E coli is the commonest pathogen

.Correct answer.

Pyrexia of 38.5oC suggests pyelonephritis

.Correct answer.

In the investigation of a child with UTI, which of the following statements are TRUE?

· Ultrasound is a good test to diagnose scarring in the kidney.

· DMSA scan is useful to obtain split function of each kidney.

· All children with UTI require an ultrasound scan of the renal tract.

· Micturating cystogram is the gold standard test to diagnose vesicoureteric reflux.

DMSA scan is useful to obtain split function of each kidney.

Micturating cystogram is the gold standard test to diagnose vesicoureteric reflux.

The characteristic features of childhood steroid sensitive nephrotic syndrome are:

· Oedema, haematuria and hypertension

· Oedema, heavy proteinuria and macroscopic haematuria

· Oedema, oliguria and hypertension

· Oedema, severe hypoalbuminemia and heavy proteinuria

Oedema, severe hypoalbuminemia and heavy proteinuria

.Correct answer.

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