FEBRUARY CASE OF THE MONTH!!

WELCOME BACK FOR OUR NEXT CASE!!

For those who joined us yesterday for our conference, we hope you had an awesome time – weren’t the speakers so inspirational?!

Today, we have another case to help you get your thinking cap on – we hope you enjoy!

THE CASE

You are a junior doctor working in the emergency department. You are asked to review a 14 year old girl, Emily Davis, who has presented with her parents. She is complaining of feeling generally unwell with worsening fever and abdominal pain for the past six days.

Come up with a list of differentials for this stage

Given the relatively non-specific nature of the symptoms, there are several differentials that I would be considering.

Sepsis
Bacterial or viral infection e.g. gastroenteritis
Kawasaki disease, due to the prolonged fever
PIMS-TS
Staphylococcal or streptococcal toxic shock syndrome
Systemic lupus erythematous, or other vasculitic disease
Hyperinflammatory syndromes e.g. haemophagocytic lymphocytic histiocytosis; macrophage activation syndrome, a life-threatening complication of rheumatic fever
Surgical conditions e.g. appendicitis due to abdominal pain

What questions do you want to ask?

I’d like to ask Emily about the nature of her symptoms, including the onset, time course and severity. How have her symptoms changed over the past few days? Was there anything which preceded them?

For the abdominal pain, I would take a detailed history using SOCRATES as a framework so to ensure I collect all the information. For the fever, I would like to know when it began, how it has changed over the past few days, whether it is there all the time or is there a temporal nature e.g. only present at night. Once I had collected sufficient information regarding Emily’s presenting complaints of abdominal pain and fever, I would complete my history by enquiring about other features, a systems review, past medical history, family history, drug history and the patient’s ideas, concerns and expectations.

Emily and her parents report that she has been getting worse over the past six days, and is feeling really unwell and has had to be off school. 3 weeks ago she tested positive for COVID-19, and was unwell with a mild dry cough and fever, but has since been back to school and was feeling much better. Her fever began six days ago and her parents measured it last night as 38.7 degrees celsius. The fever is not settling with paracetamol No one else in the family is feeling unwell currently and there is no recent travel history.

Regarding her abdominal pain, you use SOCRATES and find out that it is a dull ache across her whole abdomen which began four days ago. It doesn’t move anywhere, and nothing is making it better or worse; it feels quite similar to her period pains but she isn’t due on for a couple of weeks. She would score it a 4/10.

Upon completing a thorough systems review, you find Emily has also had a two day history of headaches, which feels like a tight band across her forehead, and are getting worse. She hasn’t had any vomiting but hasn’t eaten for the past day. There are no rashes. She reports no respiratory symptoms but she is breathing quite fast, with no cough and no stridor or wheezes.

Past medical history: Nil

Family history: High blood pressure paternal side

Drug history: Nil and no known allergies

Social history: Lives at home with parents, attends school but has been off recently.

Ideas, concerns and expectations: Worried as she’s not getting any better and feels a bit different to pains she has experienced before.

How would you assess emily?

Firstly, I would perform an A to E assessment, given Emily is acutely unwell. I would assess her fluid status, and ensure her obs were in normal range. I would ensure to look for any rashes during exposure, and any features of other pathologies, such as meningism.

Assuming all was normal, and given the presenting fever of abdominal pain, I would perform an abdominal examination, looking for any tenderness on palpation, organomegaly and any other signs of abdominal disease. I would also probably perform a neurological assessment given that Emily mentioned a two day history of headaches. Given the relatively non-specific symptoms, and depending on the clinical indications following the measurement of Emily’s vital signs such as blood pressure, oxygen sats and heart rate, I would likely also examine Emily’s cardiovascular and respiratory symptoms to look for any signs here which may indicate the complex pathology behind her symptoms.

Upon examination, you find the following notable features:

Warm peripheries
CRT 4s
RIF tenderness, no lymphadenopathy, no organomegaly, no palpable masses
Respiratory system – bilateral symmetrical chest expansion, tachypnoeic at 30 breaths per minute, SATs 98% on air
Cardiovascular – S1 and S2, no additional sounds, tachycardia at 129bpm, blood pressure 75/40mmHg
No systemic signs of infection or meningism. No rash seen. Temperature now 39.3

Are there any results here which are concerning? Is there any management you might initiate

Given the low blood pressure, I would be concerned about the possibility of shock. I would prescribe a fluid bolus (likely 10ml/kg NaCl) and see if the shock is fluid responsive.Given Emily’s fever and suspicion of sepsis, empiric antibiotics should be commenced as per local sepsis protocols with blood cultures.

I would likely contact PICU retrieval teams early for advice, give the high possibility of Emily deteriorating further and her worrying features, and consider escalation to the high dependency unit. I would put Emily on cardiorespiratory monitoring, including continuous oxygen saturation measurements, blood pressure and ECG. I would do an hourly PEWS score and update the PICU in case of any deterioration before retrieval.

You send off some bloods which showed the following abnormal results:

FBC- neutrophilia and lymphopaenia
Inflammatory markers – raised CRP, fibrinogen and ferritin
Cardiac markers – troponin, pro-BNP and d-dimer all significantly elevated
Clotting screen – prolonged APTT and PT
Raised creatine kinase and hypoalbuminaemia

What other investigations might you consider?

Based on the raised BNP and troponin, I would like to carry out an echocardiogram to track cardiac dysfunction and evolution of coronary artery abnormalities.

Given Emily is presenting with abdominal pain, I would like to image the abdomen.

Given that Emily is presenting with a fever, I would like to order a SARS-CoV-2 PCR test.

I would also like to look for any microbial cause, such as bacterial sepsis, toxic shock syndromes and infections associated with myocarditis. As a result, I would order blood cultures, a urine dip and a urine MC&S.

Given the COVID-19 infection from 3 weeks ago, I would probably order a chest x-ray to see if there had been any resolution of this..

From your investigations you find the following:

Echocardiography shows a reduction in left ventricular function, but no coronary artery abnormalities or valvular dysfunction
Abdominal CT shows free fluid and lymphadenopathy
Chest X-Ray shows small bilateral pleural effusions
SARS-CoV-2 PCR negative
Blood cultures and urine MC&S showed no growth.
Urine dip microscopic haematuria and protein

How do these findings affect your differential diagnosis?

Given the findings above, Emily’s deteriorating condition, previous COVID-19 infection and prolonged resistant fever, my primary differential would be PIMSTS – Paediatric Inflammatory Multisystem Syndrome Temporally Associated with SARS-CoV-2.

What management would Emily likely be given?

I would escalate Emily’s care given her deterioration. She would likely be given supportive care, in addition to the following medications:

IV Immunoglobulin – to reduce the risk of coronary artery aneurysm
Aspirin or low molecular weight heparin due to risk of venous thrombosis – discuss with cardiologist
Steroids – usually IV methylprednisolone
Consider immunotherapy as per MDT advice

Aspirin is not usually given to children, except in rare cases such as this and in other hyperinflammatory states. Why is this?

There is a concern that giving aspirin to children may cause Reye’s syndrome. Reye’s sydnrome usually begins a few days after a viral infection, and children experience nausea, vomiting, fatigue and shortness of breath, which can progress to seizures and loss of consciousness.

Learning Points

Most children infected with SARS-CoV-2 exhibit mild symptoms or are asymptomatic, however a minority develop a severe hyperinflammatory state following exposure to SARS-CoV-2. This is known as PIMS-TS. The presentation is similar to Kawasaki disease, with features such as prolonged fever, rash, conjunctivitis and peripheral oedema; as well as features such as abdominal pain and gastrointestinal upset which are less commonly seen in Kawasaki disease.
The principle management for PIMS-TS is high quality general paediatric intensive care, and patients should be transferred to a unit where this is possible. The condition must be promptly recognised due to the high risk of complications, with cardiac involvement in ~50% and acute kidney injuries in ~70%.
SARS-CoV-2 PCR testing may be positive or negative, however most children are usually positive for the antibody. The peak in PIMS-TS appears to be a month behind the COVID-19 peak in the general population, suggesting the illness is mediated by the development of acquired immunity rather than by direct viral injury.
RCPCH have released the following infographic aiming to help aid clinicians in their diagnosis of PIMS-TS.