Adrenal Crisis in Pediatric Emergency

An adrenal crisis is a physiological event caused by an acute relative insufficiency of adrenal hormones. It may be precipitated by physiological stress, such as infection or surgery in a susceptible patient. It should be considered in patients with:

• congenital adrenal hyperplasia

• hypopituitarism with replacement therapy

• previous or current prolonged steroid therapy

• a history of central nervous system pathology or neurosurgical procedures.

Assessment

Signs of both glucocorticoid deficiency and mineralocorticoid deficiency should be looked for if adrenal insufficiency is suspected. In the history and examination, you should look for the following:

• glucocorticoid deficiency presenting with weakness, anorexia, nausea and/or vomiting, hypoglycemia, hypotension (particularly postural) and shock

• mineralocorticoid deficiency presenting with dehydration, hyperkalemia, hyponatremia, acidosis and prerenal renal failure.

Investigations

Prompt treatment is essential and should be based on the following investigations:

• immediate blood glucose using a Dextrostix

• serum glucose, urea, sodium and potassium

• arterial or capillary acid base

Where the underlying diagnosis of adrenal insufficiency is suspected, you should collect at least 2 ml of clotted blood for later analysis (cortisol and 17-hydroxyprogesterone). The results are unlikely to be available to help in the emergency management of the patient.

Management

Susceptible patients who present with vomiting

Susceptible patients who present with vomiting but who are not other-wise unwell should be considered to have incipient adrenal crisis. To prevent this from possibly developing further:

• Administer i.v. or i.m. hydrocortisone 2 mg /kg weight

• Give oral fluids and observe for 4–6 hours before considering discharge.

• Discuss with appropriate consultant.

For all other children

For all other children where adrenal insufficiency is suspected, the various abnormalities should be considered, namely, fluid requirements, hydrocortisone requirements, hypoglycemia, hyperkalemia and precipitating causes such as sepsis.

1. Give intravenous fluids

The level of dehydration should be assessed and intravenous fluids given accordingly. For management of various levels of dehydration,

Management of dehydration in adrenal insufficiency

a. For shock and severe dehydration

• Normal saline 20 ml/kg weight i.v. bolus. Repeat until circulation is restored.
• Administer remaining deficit plus maintenance fluid volume as normal saline in 5% dextrose evenly over 24 hours.
• Check electrolytes and glucose frequently.
• After the first few hours, if serum sodium is >130 mmol/liter, change to half normal saline.
• 10% dextrose may be needed to maintain normoglycemia.

b. For moderate dehydration in adrenal insufficiency

• Normal saline 10 ml/kg i.v. bolus. Repeat until circulation is restored.
• Administer remaining deficit plus maintenance fluid volume as normal saline in 5% dextrose evenly over 24 hours.

c. For mild dehydration in adrenal insufficiency

• No bolus.
• 1.5 times maintenance fluid volume administered evenly over 24 hours.

2. Give hydrocortisone

Hydrocortisone must be given intravenously. If intravenous access is difficult, hydrocortisone should be given intramuscularly while you are establishing the intravenous line, in the following doses:

Neonate: hydrocortisone 25 mg stat and then 50 mg/m² per 24 hours by continuous infusion

• 1 month–1 year: hydrocortisone 25 mg stat, then 50 mg/m² body surface per 24 hours by continuous infusion
• Toddlers (1–3 years): hydrocortisone 25–50 mg stat then 50 mg/m² per 24 hours by continuous infusion
• Children (4–12years): hydrocortisone 50–75 mg stat, then 50 mg/m² per 24 hours by continuous infusion
• Adolescents and adults: hydrocortisone 100–150 mg stat, then 50 mg/m² per 24 hours by continuous infusion

When the patient is stable, you should reduce the intravenous hydrocortisone dose, and then switch to triple dose oral hydrocortisone therapy, gradually reducing to maintenance levels (10–15 mg/m² per day).

In patients with mineralocorticoid deficiency, start fludrocortisone at maintenance doses (usually 0.1mg daily) as soon as the patient is able to tolerate oral fluids.

3. Treat hypoglycemia

Hypoglycemia is common in infants and small children. Treat with an intravenous bolus of 5 ml/kg weight 10% dextrose in a neonate or infant and 2 ml/kg weight of 25% dextrose in an older child or adolescent. Maintenance fluids should contain 5–10% dextrose.

4. Treat hyperkalemia

Hyperkalemia usually normalises with fluid and electrolyte replacement.

• If potassium is above 6 mmol/liter, perform an ECG and apply a cardiac monitor as arrhythmias and cardiac arrest can occur.
• If potassium is above 7 mmol/liter and hyperkalemic ECG changes are present (e.g. peaked T waves, wide QRS complex), give 10% calcium gluconate 0.5 ml/kg weight i.v. over 3–5 minutes. Commence an infusion of insulin 0.1 units/kg weight per hour i.v. together with an infusion of 50% dextrose 2 ml/kg weight per hour.
• If the potassium is above 7 mmol/liter with a normal ECG, give sodium bicarbonate 1–2 mmol/kg weight i.v. over 20 minutes, with an infusion of 10% dextrose at 5 ml/kg weight per hour.

5. Identify and treat potential precipitating causes such as sepsis.

6. Admit to appropriate inpatient facility.

Prevention

The prevention of a crisis is usually possible in susceptible individuals. Situations likely to precipitate a crisis should be anticipated and the patient given:

• triple normal oral maintenance steroid dose for 2–3 days during stress (e.g. fever, fracture, laceration requiring suture)

• intramuscular hydrocortisone when absorption of oral medication is doubtful (e.g. in vomiting or severe diarrhea)

• increased parenteral hydrocortisone (1–2 mg/kg weight) before anesthesia, with or without an increased dose postoperatively.

Read More »

Jaundice Icteric in Pediatric Emergency

Jaundice in early infancy

There are many causes of jaundice in early infancy. It is clinically defined as the yellow colouration of the skin and sclera. The best approach is to define it, if it is unconjugated or a conjugated jaundice, as this will dictate the approach to investigation and treatment.

Physiological jaundice

Jaundice is very commonly noted in the first 2 weeks of life. It is part of a normal physiological process and affects 50–70% of babies. Mild jaun-dice with onset after 24 hours of life and which is fading by 14 days needs no investigation or treatment.

Causes of neonatal jaundice

Unconjugated

• Breast milk jaundice: 3–5% of breastfed babies
• Prematurity: exaggerated physiological pattern; may last 4 weeks
• Bruising or cephalohematoma: breakdown of heme
• Hemolysis (Rhesus, ABO, G6PD or pyruvate kinase (PK) deficiency, spherocytosis): early onset for ABO, Rhesus
• Sepsis: rarely presents with jaundice alone (occasional for UTI); usually unwell
• Metabolic (e.g. hypothyroidism): prolonged jaundice, can be mixed conjugated/unconjugated
• Polycythemia: delayed cord clamping, twin-to-twin transfusion
• Gilbert or Crigler—Najjar: rare, usually presents as prolonged jaundice
• GI obstruction (e.g. pyloric stenosis)

Conjugated—pale stools/dark urine, raised conjugated bilirubin (>25% total or >25 µ mol/Liter)

• Biliary atresia
• Choledochal cyst
• Neonatal hepatitis (congenital infection, alpha-1 antitrypsin deficiency,often idiopathic)
• Metabolic (galactosemia, fructose intolerance—ask about sucrose/fructose in food/medication)
• Complication of total parenteral nutrition (TPN)

Breast milk jaundice

This is the most common cause of prolonged jaundice beyond 14 days but other causes should be eliminated before this diagnosis is made. A breastfed baby with prolonged unconjugated jaundice, normal stool and urine colour, normal FBC, blood film and Coombs test who is well and thriving probably has breast-milk jaundice. Do not stop breastfeeding. The child should have a review in a general pediatric clinic if not improving or if there are any changes—especially in stool colour.

Conjugated hyperbilirubinemia

This must be excluded as the causes of this pattern need urgent evaluation and treatment. Surgery for biliary atresia is most successful when the condition is diagnosed and treated early. Don’t forget to ask about the color of urine and stools. View a dirty nappy yourself if possible.

Phototherapy or rarely exchange transfusion

This may be necessary in a baby with severe unconjugated jaundice associated with prematurity, hemolytic disease, or rare disorders such as Crigler—Najjar. Outside of these conditions, unconjugated jaundice is unlikely to lead to CNS or hearing problems, and no treatment is usually necessary

Read More »

Diarrhea and Vomiting in Pediatric Emergency

Most diarrhea and vomiting in children is due to infective gastroenteritis ) but not all. It may be the presentation of a urinary tract infection (UTI), meningitis, appendicitis, intussusception or systemic illness. In the unwell child, especially if anuric, consider hemolytic uremic syndrome (hemolytic anemia, acute renal failure and thrombocytopenia).

Assessment

There are essentially two aspects to the assessment of a child with gastroenteritis:

• Diagnosis: Does the child have a cause other than infective gastroenteritis?

• Degree of dehydration: Does the child need admission to hospital? Is there any reason why oral rehydration is not appropriate?

An initial weight measurement should form part of the initial assessment of any child who presents with diarrhea and vomiting. Any child who is toxic, vomiting blood or bile or has severe abdominal pain or abdominal signs needs immediate consultant referral. Be especially careful of those children with chronic illnesses and poor growth, and of the very young.

You must assess degree of dehydration on clinical signs and change in body weight (if recent weight available).

• With mild dehydration (<4%) there are no clinical signs.

• Moderate dehydration (4–6%) tends to be accompanied by dry mucous membranes and sunken eyes.

• With increasing severity to 7–9%, the signs of dehydration are more pronounced with cool peripheries, decreased skin turgor, impaired peripheral circulation and acidotic breathing.

• A shocked child needs immediate intravenous resuscitation.

Fecal samples should be collected for microbiological culture if the child has significant associated abdominal pain or blood in the motions, as a bacterial cause of gastroenteritis is more likely.

Management

The child with diarrhea should continue to be fed unless severely dehydrated. Most children can be rehydrated with oral or nasogastric feeds unless shocked. Do not give medications to reduce the vomiting or diarrhea. They do not work and may be harmful.

Oral rehydration

There is no evidence that milk, or other foods, should be diluted or excluded during the diarrheal illness, unless there is documented lactose intolerance, although a short period without food will not harm children. Breastfeeding should continue.

For mildly dehydrated children, increased frequency of normal drinks, giving small amounts often, will be adequate. Do not give fizzy drinks or fruit juice as their osmolarity is too high. For moderately dehydrated children, who are still drinking, small frequent amounts of fluids should be given according to the volumes given on the charts below. The fluids should be oral rehydrating fluids.

Commercially available preparations usually have 35–50 mmol sodium/Liter. Examples include Diorylate or Rehydrat. Clear instructions and a written information sheet should be given to the parents together with an indication of when to seek review. Indications for admission are:

• refusal to take fluid

• severe pain

• bilious vomiting

• abdominal distension

• blood in the motions

• altered level of consciousness

• increasing dehydration

• parents coping poorly

Nasogastric rehydration

Nasogastric rehydration is a safe and effective way of rehydrating most children with moderate to severe dehydration, even if the child is vomiting. There are a number of possible regimens. Current practice is to replace deficit over 6 hours and then give daily maintenance over the next 18 hours using oral rehydration solution. To calculate hourly rates see Table 7.4. 

However, recent recommendations from the American Academy of Pediatrics say that rapid nasogastric rehydration over a 4-hour period is safe and effective. Using 100 ml/kg weight over 4 hours for a moderately dehydrated child or for more severe even 150 ml/kg weight over 4 hours is an appropriate replacement rate. In most cases it is not necessary to check electrolytes.

Intravenous rehydration

Any child with severe dehydration requires immediate boluses of 20 ml/kg weight normal saline until circulation restored (i.v. or i.o.). Urgent U&Es, glucose, FBC, blood gas and urinalysis. Consider whether a septic work-up and parenteral antibiotics are needed or a surgical consultation.

Always remember the ABC approach to a sick child. If serum Na+ is between 130 mmol/liter and 150 mmol/Liter, aim to replace deficit and maintenance over 24 hours after the circulation has been restored by fluid boluses. Reassess clinically and reweigh on the ward at 6 and 12 hours.

Contact a gastroenterology consultant if the child is hemodynamically unstable, has a past history of gut surgery or other significant disease. If serum Na+ is less than 130 mmol/Liter or more than 150 mmol/Liter, aim to replace deficit carefully over 48–72 hours, and consult with senior staff.

Read More »

Constipation in Pediatric Emergency

Constipation is defined as hard stools that are difficult to pass. Defecation may be painful and may be less frequent than normal. There is a wide range of normal stool frequency: normal breastfed infants may have a stool following each feed or only one every 7–10 days; bottle-fed infants and older children will usually have a bowel action at least every 2–3 days. Constipation occurs more commonly in older children when they have been switched from breast milk or formula to cow’s milk. Constipation may be associated with abdominal pain, reduced appetite and irritability. Vomiting is rarely a sign of constipation alone.

Children may develop constipation as a result of:

• a natural tendency related to reduced gut motility

• a poor diet

• a toddler behavior pattern

• inadequate fluid intake (sometimes after acute illness)

• reduced activity

• painful anal conditions, e.g. fissures

• sometimes following sexual abuse.

Organic causes are rare after early infancy.

Assessment

All infants under 3 months should be discussed with the registrar or consultant. They may require referral for exclusion of Hirschsprung disease. Suggestive symptoms and signs include delayed passage of meconium, vomiting, failure to thrive, abdominal distension and a positive family history.

Do not perform a rectal examination—it is rarely helpful and usually traumatic to the child. An inspection of the anus is important to exclude painful conditions. Acute anal fissures are generally posterior and may occur after passage of a large stool or may complicate inflammation of the perianal skin, e.g. in pinworm (or threadworm) infestation. Perianal cellulitis is caused by group A streptococcal infection and is characterised by induration and marked erythema of the perianal skin with mucopurulent exudate. An abdominal X-ray is not helpful in the initial assessment and should not be ordered. Remember that urine infections are more common in constipated children and should be sought if symptoms are suggestive.

Assesment Constipation in Pediatric Emergency

Management

Carers need to be reassured about the safety of laxatives in children. Rectal medications should be avoided in the first instance and not prescribed without discussion with a consultant. Management can be divided into three main steps

Step 1

Initially give a high fibre diet with adequate fluid intake, adequate exercise and regular toileting.

Step 2

The child will need laxatives, including a stool softener and an aperient.

A suggested regime is: Parachoc or Lactulose; plus sennakot

• Liquid paraffin (<5 years: 10 ml daily; 6–12 years: 15 ml daily). Parachoc should be avoided if there is an increased risk of aspiration, e.g. in cerebral

palsy with bulbar involvement. Children may be given up to 25 ml of Parachoc per dose.

• Lactulose (<5 years: 5 ml b.i.d.; >5 years: 10 ml b.i.d.). Children may be given up to 25 ml of Lactulose per dose.

• Senna granules: (2–6 years: 1/2 teaspoon nocte; 6–12 years: 1 teaspoon nocte). The maximum daily dose of Sennakot granules should be 3 teaspoons per day

Abdominal pain may occur as a side effect. Increased doses may be required in some children with chronic constipation. The dose should be titrated to achieve at least one soft stool per day.

If overflow incontinence is present, warn families that it may worsen initially with treatment.

Step 3

Rarely patients may require admission to achieve bowel emptying. Treatment will involve a bowel preparation agent either Picoprep (Picolax in the UK) sachets (sodium picosulphate 10 mg, magnesium oxide 3.5 mg, citric acid 12.0 g, aspartame 36 mg): <2 years—1/4 sachet; 2–4 years—1/2 sachet; 4–9 years—3/4 sachet, repeating in 6 hours if no response; or colonic lavage solution (e.g. Golytely: orally or via a nasogastric tube).

Disposition

All patients should be referred for follow up in a general pediatric clinic within 7 to 10 days.

Read More »