Febrile Seizures

Simple Febrile Seizures 

Generalized tonic-clonic seizure that last <15 minutes with fever 38°C in a child 6 months to 5 years of age that occurs only once in a 24-hour period. Simple febrile seizures tend to occur within the rst 24 hours of a febrileillness. If the seizure occurs > 24 hrs after the onset of fever as in this case, the index of suspicion for a serious bacterial illness should be heightened. Anticonvulsant therapy is not recommended for simple febrile seizures since side effects outweigh the minor risks of seizure recurrence. No dedicated seizure workup is required for simple febrile seizures. The workup should focus on looking for the source of the fever, and the approach should be the same as if the child had only the fever with no seizure. However, lumbar puncture should be strongly considered in children who presnet with signs or symptoms suggestive of intracranial infection or those who are unimmunized. 

The height of fever does

not correlate with seizures; however, the rapidity of the rise in temperature is thought to correlate with the occurrence of seizures.

Complex febrile seizures 

Seizures with fever that last >15 minutes, that recur within a 24-hour period, are focal or generalised, or occur in children <6 months or >5 years of age without any signs of serious infection. The distinction between simple versus complex febrile seizures is important, as complex febrile seizures may indicate a more serious underlying disease process and warrant a workup. Meningitis should always be on thedi erential diagnosis in a child with complex febrile seizures. 

Having a febrile seizure does not mean that a child will develop epilepsy. Risk of epilepsy is approximately 2% after a simple febrile seizure and 5% after a complex febrile seizure (compared with 1% in the general population)

When working up, routine tests include FBC, CRP, Blood Cultures, Urine Analysis, Electrolytes and LP should be considered if:

• Post-ictal >1 hr
• Signs of meningitis
• Irritable
• ALready on Antibiotics
• Unvaccinated
• Complex Partial Seizures

Although antipyretics are indicated in children with fever, there is no evidence that antipyretics can prevent subsequent febrile seizures.

Admission and discharge depends on parental comfort, well/sick looking child but consider admission for the following groups: 

• Complex febrile seizures 
• A child with a febrile fit who is less than 18 months of age 
• The child with no serious clinical findings but who is recently/currently taking antibiotics 
• Children who do not have a clear focus of infection 

References:

• Provisional Committee on Quality Improvement, Subcommittee on Febrile Seizures:practice parameter: the neurodiagnostic evaluation of the child with a first simple febrile seizure. Pediatrics 97: 769, 1996.
• Subcommitte on Febrile Seizures, American Academy of Pediatrics: Neurodiagnostic evaluation of the child with a simple febrile seizure. Pediatrics 127: 389, 2011.
• Kimia AA, Capraro AJ, Hummel D, et al: Utility of lumbar puncture for first simple febrile seizure among children 6 to 18 months of age. Pediatrics 123: 6, 2009. [PMID: 19117854]
• SteeringCommitteeonQualityImprovementandManagement,SubcommitteeonFebrile Seizures: Febrile seizures: clinical practice guideline for the long-term management of the child with simple febrile seizures. Pediatrics 121: 1281, 2008.
• https://cks.nice.org.uk/febrile-seizure#!scenario

Posted by:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic

FRCEM Final SAQ - The Holy Grail

I took the FRCEM Final SAQ exam lately and in this post, I am going to share the way I prepared for this exam. EM Trainees take this exam anytime between ST4-6 years of training (EM training lasts for 6 years in the UK). This way of preparation worked well for me and this is just one of the ways to prep for the exam. If you have any further thoughts, please feel free to comment below. 

Exam pattern: 60 questions with 1-3 stems in each question. Each question carries 3 marks and you score out of a total of 180. To pass the exam, you need to get about 120-130 questions correct. This exam basically tests your EM knowledge and management skills. It is a good idea to familiarize yourself with the basic structure of NHS. However, it is not must to have UK work experience prior to taking this exam. 

Study time: I spent around 2 weeks to actively prepare for the exam. Since I work in a busy A&E, I was in constant touch with Emergency Medicine. I believe 1 month is a decent amount of time to prep for this exam. 

Study Material: 

• Oxford Handbook of EM
• Oxford Handbook of Acute Medicine
• Oxford Handbook of Clinical Specialities
• Victoria Stacey 
• RCEM guidance from RCEM website 

Tintinalli's Textbook or Rosen's is not typically followed in the UK as core EM Textbooks and are not must for this exam. But you will certainly have an extra edge over other candidates if you have read an EM textbook cover to cover. 

RCEM Curriculum, Images (Clinical Images, ECGs, X Rays)

Scroll through the college curriculum as you read OHEM and as you do the practice questions. Ensure you cover each bit of the curriculum, for instance, stuff that we don't see routinely (STDs, vaginal discharge, rheumatological diseases, sarcoid, spinal cord syndromes etc) should also be covered. Questions related to Lupus Psychosis, Ovarian Hyperstimulation Syndrome are some of the favourite topics. 

Questions with image interpretation are easy to score. Glance through X Rays of various differentials of limping child, C-Spine injuries, images of skin rashes in children and fundus findings for ophthalmological pathologies. Search "Google images" and look what pops up whenever you come across a question with a likely image interpretation scenario. 

Important ECG topics include 

• Posterior wall STEMI
• Types of Ventricular Tachycardia 
• ARVD
• Brugada
• Subtle ischemia (Wellens, de winters T waves, Early reciprocal changes in aVL)
• Torsades
• Trifascicular block
• AV Blocks
• Sgarbossa Criteria 
• ECG findings of PE
• Long/Short QTc syndromes 

How is FRCEM Final SAQ different from MRCEM SAQ?

While preparing for the exam, I always thought that this exam appears very similar to MRCEM SAQ. FRCEM Final SAQ focusses more on management aspects (RCEM Guidelines) in addition to core EM textbook knowledge. Please make sure that you go through the RCEM website and have a glance through the guidelines (absconding patient, frequent attenders, dealing with police, domestic violence, sexual assault, valid consent, confidentiality, handling complaints, adverse events, MH issues covering all the sections, common law and assessing capacity, dealing with intoxicated colleagues, major incident management, quality indicators, RCEM standards of care etc.)

Study Buddy: Choose to have 1 or 2 study buddies to prepare. I suggest to read the text on your own but do questions followed by discussions together. As you read OHEM, you will realize that it possible to frame a question from each sentence of this handbook. It is a good idea to think how a question can be framed on a particular topic as you read OHEM and then test your partner with that. 

Exam preparation courses: Surprisingly, there are only a few resources and question banks to prepare for the exam. Take your pick between a formal prep course or an online question bank. Going to a course (which is more expensive than the exam) without brushing up OHEM is a sheer waste of time. 

Exam taking strategy: With electronic health records in place, most of us are not used to swiftly scribble on a sheet of paper. In fact, one of my mentors once told me, if you attempt all the questions then you are highly likely to pass. Therefore, practice a few timed mock tests prior to the exam. Tips for the day of exam:

• You are given 3 hours to answers 60 questions. If you are not sure about an answer, keep moving forward and get back in the end. 
• Do NOT spend more than 2:00 - 2:30 minutes on one question 
• Image questions: Don't jump to conclusions and Don't jump to the stem after seeing the image. Even if you are 100% sure about the image interpretation, you MUST read the question completely before you write down the answer. 

Decision-making tools

You don't need to remember all of them but have an idea about few components of each decision tool. For instance, remember 4 things that makeup PERC criteria or 4 things that make up TIMI score or HEART score. Use mdcalc to check up to date scores. 

We have also covered 2 posts in the past about MRCEM SAQ. A lot of what has been mentioned in these posts holds relevant for Final FRCEM SAQ as well. I highly recommend you to go through them:

MRCEM part B by Lakshay Chanana
MRCEM Part B by Apoorva Chandra

Posted by:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic

Leading the shop floor - Unsolicited advices!

Night shifts tire out A&E doctors and lead to considerable amount of distress. Things can go worse due to lack of staffing, complicated patients (who always turn up during these hours), unfamiliar (or familiar) locum doctors around. Explicit details vary depending on the "local settings and culture of Emergency Medicine" but overall theme remains the same to manage a busy department overnight. By morning, we end up signing countless ECGs, ordering several medications (with minimal background info available) and listening to numerous SHO/F2 case presentations. Essentially, we walk on the very thin ice during night shifts and there are good chances of making errors. 

Is there a way to circumvent this or at least minimize this risk?
What can we do to manage flow better and avoid handing over an 8 hour waiting time?

1. Using checklists

• Procedural Sedation Checklist
• Resuscitation checklist 
• Post Cardiac Arrest Care checklist
• Reading a CXR checklist
• ECG checklist 
• Discharge Checklist
• 
  

These can reduce cognitive overloading, save time and minimize errors, especially during the busy hours.

2. Listening to FY/SHOs presentations 
Take time and set rapport with your night team. Get a sense of their background, previous experiences and what rotations they have been through so far. Allocate tasks keeping this in your mind. Early on, try and see (at least eyeball) every patient that they are allocated and once you understand their clinical acumen, go with that. 

Being approachable with junior doctors is they key for patient safety. Ask them if they are concerned about anything in particular or if they want you to examine the patient. 

3. Documentation and Risk Management 
Diagnostic uncertainty is a part of Emergency Medicine. it is almost impossible to come with a definite diagnosis within a span of few hours. Sometimes, it takes weeks and months to reach a definitive diagnosis after several out-patient visits. Our job is to think about life threats. We work on the basis of probability and likelihood. Explain this to a patient. Most of them will appreciate and just need some reassurance. Prepared scripts can be useful here. For instance, discharging a low-risk chest pain. Document a clinical decision rule such as HEART score and tell them that:

Based on our evaluation today, your blood reports, and serial ECGs, your risk of heart disease is extremely low. I think your symptoms are likely due to acid reflux. I am going to prescribe some medications and discharge you. With aging, we all develop some degree of blockage in blood vessels around the heart and you may have that as well but this does not mean that you are having a heart attack. You need to follow up with you General Practitioner as an out-patient for further evaluation, see response to reflux medications and a for a definite diagnosis. However, if things change anytime i.e you feel unwell, sweaty, dizzy, short of breath or concerned about anything else then please come back to A&E and we will be happy to assess you again. We are here 24x7. 

Document this conversation briefly in the chart briefly under "Medical Decision Making". Your chart should depict your thought process and rationale behind your decisions. Use scoring systems like Wells, PERC, HEART to manage risk. Occasionally, you will see a patient who asks for a 100% definite diagnosis. Ask about their concerns and set expectations as soon as you see them. If you are sending someone home who looks clinically very well but with a unclear diagnosis, safety net them and arrange further follow up as an out-patient. 

Documentation: It is best to finish documentation/clerking as you go rather than carrying a bunch of charts with you to finish after the morning handover. After the handover, you would want to leave the ED ASAP and this will certainly compromise the quality of your notes. Write explicit and legible notes. 

Patient info leaflets and discharge advice: If you think they are going home, hand over specific discharge leaflets to them beforehand. Tell them to have a glance through them and ask any questions. This keeps them busy and helps to understand their ailment better. Moreover, you are medicolegally safer since you hand over written red fags to them specifying when to return to ED.

4. Triage led blood sets (for minors) - to expedite decision making

• Young Chest Pain 
• Pleuritic Chest Pain 
• CP with risk factors
• Abdo pain female
• Abdo pain male 
• Shortness of Breath 
• Needle Stick Injury
• Febrile Illness
• Fever in Traveller 

Triage led to blood tests based on chief complaints improves the flow but these should be interpreted carefully. Sending troponins for every 20yo with CP will lead to nowhere and may lead to unnecessary further testing. I advocate that tests such as d-dimer and hs-troponin should only be ordered after discussion with a consultant or any senior physician whenever possible. Both these tests are very non-specific but if used judiciously, they can be very useful. Routine second troponins lead to unnecessary long waits and prolong the length of stay. False positive d-dimers subject patients to the unnecessary risk of anticoagulation and follow up. 

Avoid "just to be sure" type of investigations. Do not start hunting for problems in a well-looking 90-year-old. Follow guidelines for imaging and deviate only if you feel strongly about something. If a blood test is not going to alter your management, then don't do it. Check out RCEM guidelines on redundant activities here. 


5. Situational Awareness and communication
While running the show as a registrar (equivalent to final year resident in the US), do less and allocate more. Having a good sense of what is going around with patients seen by junior doctors is crucial. Anticipate further care needs of every patient. For instance, eyeballing patients on arrival and organizing imaging such as X Rays and Head Scans can save a lot of time. Ask yourself:

• What can you do for this patient in ED?
• Who is well and who is sick? 
• Who needs admission and who can be discharged?

Seasoned clinicians almost always decide to admit v/s discharge after 2-3 minute conversation with a patient, occasionally just after eyeballing a patient! Verbalise a clear plan to junior doctors and if possible, write it on the chart. 

Keep a track of co-morbidities and social issues (carers, living alone, frail elderly demented, homeless, domestic violence) before discharging patients. Think twice before discharging an elderly during twilight hours. On a 10 hour shift, I typically spend just about 1 hour with patients and rest of my time goes in getting things done - requesting scans, discussion with Radiology and inpatient teams, adding blood tests, calling labs, difficult cannulas, and most-importantly documentation. When running the shop floor, it is important to delegate as much as you can. 

• IV Cannulation - Ask the nursing staff to keep the USG machine and equipment ready
• Joint reduction - Ask FY/SHO to prep everything - hook them to monitor and keep sedation drugs ready 
• FAST/AAA Scan - Move the patient to a room and keep the USG machine jelled  
• Wound Repair - Procedure nurse to prepare equipment 

If you are leading the department, then your time is precious. Everyone will be out there looking for you (nurses, junior docs, angry patients, in-patient teams, difficult referrals) and don't involve yourself in something time-taking procedures. 

I work in an enormous ED and it can take me up to 2 minutes to walk from one end to the other. Having a list of phone numbers of HDU, Minors, Resus, Assessment areas can be immensely useful. You can then just call and liaise with nurses rather than physically going to each of these areas. 



Communicate with nurses : Nurses are the backbone of your team and if they stand by your side, things go very smooth. Know them by their names and let them know that you are running the shop floor before you begin your shift. Assign them tasks like putting back slabs, getting medications quickly, patients who need monitoring and frequent observations. 

6. Calling for help
Knowing our limitations is crucial and we must know what we exactly want a specialist to do when we call for a consult. Whether it is ITU, Anesthetics, Trauma team - the theme remains the same. Whenever possible, know their names and with time set a rapport with them. Developing negotiation skills while conversing with a specialist is a skill that we all must learn. 

7. Safeguarding and other clerical work 
On a busy shift, the last thing we want is a doctor held up in sorting out safeguarding and social issues for a patient. Undoubtedly, these tasks are important our job should be to raise concerns about them. FIlling 6 page long referral forms and sending e-mails to a group of people can be very time-consuming and this can seriously halt the flow of the department. I strongly believe that these tasks would be best done by a clerk (non-medical) as this does not require a medical degree. 

If you have anymore thoughts on this, then please feel free to comment and share your wisdom.

Posted by:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic

Acute Otitis Media (AOM)

AOM refers to the acute onset of signs and symptoms of middle ear inflammation. Otits Media and URTIs are one of the commonest diagnosis in children. The peak incidence of AOM is between 6 and 18 months of age. Most common bugs causing AOM are Streptococcus pneumoniae (49%), Haemophilus influenzae (29%), and Moraxella catarrhalis (28%), picornaviruses such as rhinovirus and enterovirus, respiratory syncytial virus, and parainfluenza virus. RIsk factors for AOM are:

• Males
• Day care attendees
• Exposed to tobacco smoke
• Craniofacial anomalies
• Prone position sleepers
• Pacifier users
• Born with immunodeficiency syndromes

Breastfed infants have a lower incidence of AOM compared to infants who are formula-fed.

Middle Ear Anatomy

In the healthy state, middle ear is aerated and contains the auditory bones, which transmit sound to the inner ear. Compared with adults, the eustachian tube in children is shorter and more horizontally oriented. This orientation is the anatomic rationale for the increased incidence of middle ear disease seen in children. An upper respiratory tract infection can obstruct the eustachian tube and disrupt its function of aerating the middle ear, creating conditions favorable to the development of sterile or purulent effusions. 

Clinical Features

The classic presnetation of AOM is fever with rapid-onset ear pain with preciding history of  rhinorrhea, congestion, cough is common because an URTI creates conditions favorable to the development of AOM. Children may pull or rub the ear or be fussy and irritable. Older children may complain of decreased hearing due to con- ductive hearing loss of middle ear effusion. Complications of AOM are tympanic membrane perforation, mastoid- itis, encephalitis, abscess, sinus thrombosis, otitis hydrocephalus, facial or abducens nerve palsy, and labyrinthitis/acquired sensorineural hearing due to changes in the inner ear.

Diagnosis

Erythema of the tympanic membrane alone is insufficient fto diagnose AOM because erythema can be caused by middle ear inflammation, crying, or fever. When examining, look specifically for discharge in the ear canal, tympanic membrane’s position, color, degree of translucency. 

Treatment 

Most cases of AOM resolve spontaneously and without complications. Tympanic membrane perforation typically heals spontaneously after AOM resolution, but persistent perforations need ENT consultation. Mainstay of treatment is pain relief. Symptoms can last for up to a week but most children get better within 3 days without antibiotics. Antibiotics make little difference to the rates of common complications. 

Pain Relief: Rx with NSAIDs. Topical otic analgesic drops may be used in combination as they have a rapid onset of action. Topical analgesics are contraindicated in patients with perforation.


Need for Antibiotics?

Initial observation option is a reasonable startegy for select children with AOM and this approach is supported by numerous studies. Remember, when observation is used,  ensure fa way to follow-up and start of antibiotics if the child worsens or fails to improve. A wait-and-see antibiotic prescription can be provided at the initial visit with instructions for the caregiver to initiate antibiotics if the child worsens or fails to improve within 3-4 days. Indications for Abx are:

• Bilateral AOM in <2 years
• AOM with perforation/ear discharge
• Recurrent AOM
• Craniofacial abnormalitites
• Immunocompromised
• Systemically unwell
• Infants <6/12 old

Amoxicillin for 5 to 10 days is the first-line treatment.  If amoxicillin fails, change to amoxicillin-clavulanate or ceftriaxone to provide coverage against β-lactamase–producing M. catarrhalis and nontypeable H. influenzae. If the second antibiotic regimen fails, treat with clindamycin and a third-general cephalosporin, or consult ear, nose, and throat for tympanocentesis and culture. For people who are allergic to penicillin, prescribe a 5-day course of erythromycin or clarithromycin.

References:

1. Tintinalli textbook of EM - 8th edition
2. https://cks.nice.org.uk/otitis-media-acute#!scenario

Posted by:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic

Spotting the sick neonate - ED Perspective

Emergency Medicine is knowing "something about everything" in contrast to specialists who endeavor to know "everything about something". Therefore, as frontline physicians, we must know about common neonatal problems as well. 

While taking care of neonates, special attention needs to be paid to the adequacy of airway and breathing as they have limited compensatory reserves. As a rule, we should always consider early use of positive-pressure ventilation or endotracheal intubation for respiratory distress. Tachypnea in neonates can be very nonspecific as it may be due to underlying cardiovascular, respiratory failure or other systemic diseases. But as with everything else in EM, regardless of the cause, primary management remains similar i.e supporting ABCs. Here is a list of common life-threatening neonatal conditions:

Neonatal Sepsis (Pnemonia, Meningitis, Shock)

The height of the temperature does not distinguish a viral versus bacterial cause in neonates. Neonatal Sepsis is one of the most common causes of neonatal cardiorespiratory distress and most experts keep a low threshold for a full sepsis workup in neonates.  Fever in the first month of life is defined as rectal temperature ≥38°C, and hypothermia is rectal temperature <36.5°C (97.7°F). Early-onset sepsis (commonly septic shock) is seen in first few days and is usually associated with is usually associated with maternal or perinatal risk factors, such as maternal fever, prolonged rupture of membranes, and fetal distress. In contrast, late-onset sepsis (commonly meningitis) usually occurs after 7 days of birth and tends to develop gradually, with no predisposing risk factors. 

Clinical signs of early or late-onset sepsis in neonates are non-specific. They may demonstrate lethargy, irritability, seizures, tachypnea, grunting, hypo/hyperthermia, jaundice, rash, poor feeding, diarrhea, vomiting. Tachypnea and respiratory distress may be a sign of sepsis, meningitis, or urinary tract infection. Neck stiffness and other signs of meningismus may not be seen in neonates with meningitis. Common pathogens most frequently encountered in neonatal sepsis are gram-positive cocci (β-hemolytic streptococci) and enteric organisms (Escherichia coli and Klebsiella species, and Haemophilus influenzae). Other possible agents could be Listeria monocytogenes, viruses such as enteroviruses (coxsackievirus and echovirus), respiratory syncytial virus and influenza A virus. 

Work up includes FBC, CRP, Cultures, Urine Analysis, Renal Function, LFT and Coags and imaging as indicated. Admission and IV empiric IV antibiotics is a norm. The initial choice usually includes ampicillin (covers group B Streptococcus and Listeria) and an aminoglycoside (covers E. coli and other gram-negative organisms). Ceftriaxone is avoided in neonates as it can cause kernicterus. Use cefotaxime instead. Acyclovir is added for neonates with a maternal history of herpes or suspicious cerebrospinal fluid findings and all neonates who are ill appearing.


Congenital Heart Disease

Congenital heart defects that present with cyanosis include transposition of the great arteries, tetralogy of Fallot, tricuspid atresia, truncus arteriosus, and total anomalous pulmonary venous return. Acyanotic lesions include those that result in pulmonary overcirculation such as VSD, ASD, PDA and atrioventricular canal as well as those with restricted pulmonary or systemic blood flow such as pulmonary stenosis, aortic stenosis, and aortic coarctation.

Suspect congenital heart disease in any well-developed neonate who presents with unexplained cardiorespiratory collapse, cyanosis, and or tachypnea, especially without chest retractions or use of accessory muscles for breathing. Undiagnosed congenital heart disease may be first identified after discharge:

Day 7-14:  Lesions dependent on pulmonary or systemic blood flow through the ductus arteriosus (e.g., hypoplastic left heart syndrome, critical coarctation of the aorta) present with shock and acidosis as the duct begins to close. 
>Day 14: Lesions that involve left-to-right shunting of blood (ASD/VSD) typically present after the second week of life as pulmonary vascular resistance falls, allowing pulmonary overcirculation and the onset of congestive heart failure.

Neonates with cyanosis secondary to congenital heart disease rarely have respiratory symptoms other than tachypnea whereas neonates with lung disease producing cyanosis show respiratory distress, grunting, tachypnea, and retractions. The single most important therapeutic intervention for duct-dependent lesions is IV PGE1 to restore ductal patency. 

Bronchiolitis

Neonates with bronchiolitis present with nasal discharge and sneezing followed by diminished appetite, difficulty with feeds, cough, dyspnea, irritability, and, occasionally, periods of apnea. Respiratory symptoms include hypoxia, wheezing, retractions, and possibly palpable liver and spleen due to pulmonary hyperinflation. Factors associated with complications of bronchiolitis include prematurity, underlying pulmonary or congenital heart disease, initial oxygen saturation <92% and bronchiolitis caused by RSV.

Management remains supportive as in infants and toddlers. Antibiotics have no role in the management of bronchiolitis. CXR is done to rule out other causes of breathlessness. Bronchodilators may be tried but there is limited evidence to support their routine use. Adrenaline nebs may also be administered. There is conflicting evidence regarding the use of steroids. Full sepsis evaluation is done only if they appear critcially ill.

Airway Issues
Most of these anomalies are identified prior to discharge. 
Upper respiratory tract problems: choanal atresia, laryngomalacia, tracheomalacia, micrognathia, macroglossia, tracheoesophageal fistula, vascular slings
Lower respiratory tract: congenital lobar emphysema, sequestration, cystic adenomatous malformation, congenital diaphragmatic hernia

All these diagnoses should be considered in any infant with respiratory distress. Admission and work up is needed for diagnosis.

Neuromuscular Disorders

Muscle weakness in neonates may be associated with a compensatory increase in respiratory rate. Possible etiologies include Infantile botulism, Down’s syndrome; Hypoxic-ischemic encephalopathy,  Myelomeningocele, Spinal muscular atrophy, Myasthenia gravis, metabolic disorders and myotonic dystrophy.

Inborn Errors of Metabolism (IEM)

Since most metabolic toxins cross the placenta and are cleared by maternal enzymes, most newborns in-uterus are asymptomatic and present after enteral feeding begins. Sudden deterioration of a well neonate should always prompt consideration of metabolic diseases. Vomiting, altered mental status, hypoglycemia, ketonuria, poor feeding and cardiorespiratory distress are the most common clinical features of metabolic emergencies.  Hypoglycemia with negative urinary ketones suggests a fatty acid oxidation defect, and hypoglycemia with positive urinary ketones suggests organic acidemias. Reaching a precise diagnosis is often not possible in ED and management is limited to maintaining normoglycemia, supplementing fluids and electrolytes, blood gas analysis, checking serum ammonia levels and capillary ketone levels. Administer dextrose containing IV fluids for a suspected metabolic disease until specialty consultation can be obtained.  

Empiric therapy with arginine and sodium benzoate can reduce ammonia levels drastically. Early aggressive treatment may eliminate the need for dialysis.

Congenital Adrenal Hyperplasia

Congenital adrenal hyperplasia results from a deficiency in one of the five enzymes involved in the production of cortisol. It is one of the life-threatening endocrine emergencies that present in the neonatal period. Despite screening for this disorder, neonates may present in shock before screening results are known, typically in the first or second week of life. On examination, look for virilization, ambiguous genitalia, and hyperpigmentation. Hyponatremia and hyperkalemia occur in the salt-wasting form of congenital adrenal hyperplasia. ED management includes fluid resus with 10-20 mL/kg of normal saline, correction of hypoglycemia and administering steroids i.e. hydrocortisone 25mg IV/IM/IO, and urgent management of hyperkalemia. 

Image taken from http://what-when-how.com/acp-medicine/the-adrenal-part-2/

Avoid giving for treating hyperkalemia in an infant as this may result in profound hypoglycemia.

Intracranial and Intra-abdominal Hemorrhage

Birth trauma, NAI, and Vitamin K deficiency predispose to Intracranial bleed.
Neonates with abdominal distention, dehydration, bilious vomiting may have underlying congenital malrotation leading to midgut volvulus and bowel infarction. Necrotizing enterocolitis is classically seen in premature infants with poor feeding; abdominal distension, tenderness, and discoloration; lethargy or irritability; vomiting or diarrhea; temperature instability; apnea; and circulatory collapse. Other possibilities include testicular torsion and NAI.



References:
Tintinalli textbook of EM - 8th edition


Posted by:

              

     Lakshay Chanana

     

     Speciality Doctor

     Northwick Park Hospital

     Department of Emergency Medicine

     England

     @EMDidactic