Adrenal, Renal, and Electrolyte EmergenciesAnisha Kshetrapal, MD, MSEdMay 6, 2020

Conflicts of Interest/Disclosures• None

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Objectives• Let’s come back to this at the end!

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Case 1• 12 yo healthy female presenting with vomiting x 2 days• No fevers or diarrhea, but she has only peed once today• Vague abdominal pain for the last few weeks, just not feeling well• Keeps telling Mom she isn’t very hungry, Mom has caught her

looking in the mirror at her belly and worries she has an eating disorder• VS T 36.6 C, HR 98, RR 18, BP 98/65• Looks pale and tired, but is appropriately interactive• You don’t note any signs of an eating disorder on your exam, or

when you complete your HEADDSSS exam• What do you do next?

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5

130 97 14119

6.1 21 0.7

• You give her a NS bolus for her dehydration• She is admitted to the general pediatrics floor• The next morning, her labs look like this:

136 101 1197

5.5 24 0.6

What’s the diagnosis?

Primary adrenal insufficiency• Remember that adrenal insufficiency can have primary or secondary

causes• Signs and symptoms of primary adrenal insufficiency vary depending

on what class(es) of hormone are deficient and the severity• What kinds of deficiencies can you have?– Glucocorticoid: hypoglycemia, hyperpigmentation, muscle weakness, morning

headache– Mineralocorticoid: dizziness, weight loss, anorexia, dehydration, hypotension

• Which one causes the syndrome of adrenal crisis?

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Treatment & Clinical Pearls• Hydrocortisone!• The PICU will tell you 50/m2, but we are ED docs! We have easy

rules for crises:• 25 mg for 0-3 years of age• 50 mg for 3-12 years of age• 100 mg for > 12 years

• What’s the treatment for hypoglycemia?• Rule of 50– If you have D10, give 5 ml/kg– If you have D5, give 10 ml/kg

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• Which kids are susceptible to adrenal insufficiency?– Leukemia (dexamethasone is a mainstay of treatment)– Severe asthma on chronic steroids– Short gut (pred often used for gut inflammation)– Suspect in sepsis that is fluid refractory– Suspect in anyone with another endocrine problem (T1DM)

• Suspect if you see the labs• Call a friendly endocrinologist for help!

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Case 2• 2 week old ex full term baby girl coming in for poor feeding• Started vomiting yesterday. It’s COVID times so they were afraid to

go to the pediatrician and did a telehealth visit instead• Pediatrician told them to keep hydrated with ORS and to go to the

ED if she had less than 4 wet diapers• Today, she won’t even take the bottle, seems very sleepy• Mom says her pregnancy and delivery were unremarkable• Nurse in triage sees that the baby looks limp and gray and rushes

her back to the trauma bay• You get her on the monitor. Rectal temp 35.9, HR 191, RR 12, SpO2

not picking up yet• You start bagging her• A paramedic is able to get an IV and pull off just enough for an iSTAT

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• 7.02/58/62/11/-12• Na 123, K 7.1, glu 41• What now?• Oh yeah, Mom just told the resident taking the history that the baby

was born at home….and when you look in her diaper, you see clitoromegaly

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Treatment and Clinical Pearls• Hyperkalemia and shock can be the first presentation of CAH in a

neonate• This is called a salt-losing crisis 2/2 no mineralocorticoid• What’s the first and most important treatment here?– NS bolus, glucose, Ca gluconate, and steroids!– How much and which steroid are you going to give?– We are VERY careful with Ca administration since if it infiltrates, it can cause

severe morbidity including contractures– Hyperkalemia typically improves promptly with fluids and hydrocortisone

therapy & rarely requires administration of glucose and insulin

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Clinical Pearls• You might get a kid sent in from the pediatrician’s office based on

the newborn metabolic screen– What is this screen? When does it come back?• 217-785-8101 between 8:30 a.m.- 5 p.m– What labs do you want?

• The other cause of salt-losing crisis in a neonate can be caused by secondary aldosterone resistance due to renal disease.– Get a urinalysis, looking for obstructive uropathy, pyelonephritis, or

tubulointerstitial nephritis

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Case 3• 8 week old M with vomiting after every feed that started a few

days ago and has gotten worse• The parents say it is “projectile” but it wasn’t when it started• Emesis is NBNB, looks like breastmilk• No fevers or diarrhea; has had two wet diapers today• Again, it’s COVID times so the parents didn’t go to the pediatrician• You walk in and see a fussy baby who is rooting• The nurse puts him on the monitor where you note a heart rate in

the 170s, you count a RR of 40, SpO2 100% on RA• You get an ultrasound and a BMP

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• You call surgery, and they say there is “NO WAY” they are operating on this patient. Help??• Pyloric stenosis is a medical emergency, not a surgical emergency.• Treat the AKI first!• Need sodium >130 mEq/L, potassium at least 3 mEq/L, chloride >85

mEq/L, and a urine output of at least 1 to 2 mL/kg/hr. • How do you resuscitate? When do you add K?

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130 85 1071

2.9 31 0.55

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Case 4• 5 yo F with vomiting and diarrhea that started 5 days ago• Today, her diarrhea turned dark so mom brought her in, and she has

only peed once• Looks well on exam other than pallor• T 37.9, HR 91, RR 18, BP 118/76• The remainder of your exam is normal

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145 100 20111

4.9 21 0.95

6.97.9 58

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Hemolytic-Uremic Syndrome• What is the triad that defines HUS?– microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury

• There various etiologies of HUS that result in differences in presentation, management, and outcome, but the most common is Shiga-toxin-related• Thrombotic microangiopathy describes a specific pathologic lesion

in which abnormalities in the vessel wall of arterioles and capillaries lead to microvascular thrombosis• Dialysis is required in 60% of patients, and neurologic symptoms,

principally seizures and somnolence, are seen in 25%

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Clinical Pearls• Check a BMP and CBC on anyone with bloody diarrhea– A decrease in platelets is often the first laboratory manifestation of HUS

• Admit these kids!• Resuscitate early and aggressively: the AKI is both intrinsic and

prerenal• In two prospective cohort studies of children with HUS, volume

expansion was associated with lower rates of anuria• Obtain repeat CBCs and BMPs q8• What about antibiotics?– In a meta-analysis of mainly observational studies that included over 1800

patients with Shiga-toxin E. coli infection, there was a nonsignificant trend overall towards a higher risk of HUS with antibiotic use

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Case 5• 9 yo M brought in by mom because she is just frustrated• His pediatrician has recommended multiple behavioral

interventions, but he still hasn’t achieved daytime or nighttime dryness• Mom is upset about all the dirty sheets she has to change• He is developmentally normal and seems quite embarrassed by all

of this• He has also had recent headaches for which mom has been giving

him ibuprofen• He tells you that in the past few weeks, he “can’t pee” like his

friends, that it just dribbles out• You have completely normal vitals and exam, but Mom thinks he

looks puffy

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• What is his renal injury from?• Postrenal kidney injury from posterior urethral valves complicated

by NSAID use

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140 102 14101

3.9 25 2.2

• Chronic kidney disease (CKD) is commonly associated with PUV because many patients have renal dysplasia and/or acquired renal injury due to infection or ongoing issues with poor bladder function.• About 15 to 20 percent of patients with PUV progress to end-stage

renal disease (ESRD) • The relationship between the development of renal dysplasia and

PUV is unknown. Proposed mechanisms include:– Common developmental insult resulting in renal dysplasia and PUV.– Causal relationship between back pressure from bladder outlet obstruction of

PUV and defective nephron development.

• Recurrent infection resulting in renal scarring has been thought to be an important risk factor for CKD. However, in one study of 119 boys, recurrent urinary infection occurred in about one-half of the group and was not associated with an increased risk of developing ESRD

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What is the differential for AKI in kids?• Prerenal AKI– True volume depletion – Effective renal hypoperfusion as a result of decreased arterial pressure (due to

decreased cardiac output, sepsis, or cirrhosis) – An important compensatory mechanism is the production of renal

prostaglandins– Nonsteroidal anti-inflammatory drugs inhibit this response and can therefore

precipitate AKI even when following appropriate dosing– Two common scenarios in which this occurs are the use of indomethacin for

PDA closure and the use of ibuprofen in febrile children with hypovolemia due to gastroenteritis.

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• Intrinsic AKI– Vascular disease — like HUS, as we discussed above– Glomerular disease — The principal pediatric glomerular cause of AKI is acute

glomerulonephritis, which is most commonly poststreptococcal, and an important etiology in developing countries– Tubular and interstitial disease — Prolonged prerenal AKI with reduction in renal

perfusion, and tubular nephrotoxins are important causes of intrinsic AKI. Common drugs associated with tubulointerstitial disease include nonsteroidal antiinflammatory agents, aminoglycosides, amphotericin B, calcineurin inhibitors, and cisplatin. – Release of endogenous nephrotoxins such as myoglobinuria due to

rhabdomyolysis

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• Postrenal AKI —– Due to bilateral urinary tract obstruction – Or obstruction of the urinary tract of a solitary kidney– Causes: include renal calculi, clots, neurogenic bladder, and medications that

cause urinary retention– Children with chronic kidney disease from uncorrected congenital obstructive

uropathies remain at significant risk of AKI from ischemic, septic, and nephrotoxic insults, as discussed above

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Objectives• By the end of the presentation, participants will be able to…– Identify the causes and clinical symptoms and signs of acute adrenal

insufficiency– Execute appropriate resuscitation for adrenal insufficiency– Identify the natural history and physical findings of congenital adrenal

hyperplasia– Stabilize an infant with congenital adrenal hyperplasia– Recognize AKI in children and provide appropriate resuscitation– Recognize various prerenal, intrinsic, and postrenal causes of AKI– Recognize hypovolemia and administer appropriate resuscitation– Recognize complications of hyperkalemia and administer treatment– Recognize NSAID use as a complicating factor in renal injury even with

appropriate dosages

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Contact me!• akshetrapal@luriechildrens.org

• Resources• https://www.acmg.net/ACMG/Medical-Genetics-Practice-

Resources/ACT_Sheets_and_Algorithms.aspx

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retrospective Corticus cohort study. Crit Care Med 2007; 35: 1012-1018• Urban MD, Kogut MD. Adrenocortical insufficiency in the child. Curr Ther

Endocrinol Metab 1994; 5:131-135• Joint LWPES/ESPE CAH Working Group. Consensus statement on 21-hydroxylase

deficiency from the Lawson Wilkins Pediatric Endocrine Society and the European Society for Paediatric Endocrinology. J Clin Endocrinol Metab 2002; 87:4048-4053

• Hickey CA, Beattie TJ, Cowieson J, et al. Early volume expansion during diarrhea and relative nephroprotection during subsequent hemolytic uremic syndrome. Arch Pediatr Adolesc Med 2011; 165:884.

• Freedman SB, Xie J, Neufeld MS, et al. Shiga Toxin-Producing Escherichia coli Infection, Antibiotics, and Risk of Developing Hemolytic Uremic Syndrome: A Meta-analysis. Clin Infect Dis 2016; 62:1251.

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• Devarajan P. Update on mechanisms of ischemic acute kidney injury. J Am SocNephrol 2006; 17:1503.

• Devarajan P. Emerging urinary biomarkers in the diagnosis of acute kidney injury. Expert Opin Med Diagn 2008; 2:387.

• Askenazi D. Evaluation and management of critically ill children with acute kidney injury. Curr Opin Pediatr 2011; 23:201.

• Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the era of sonography. AJR Am J Roentgenol 1987; 148:959.

• Thakkar D, Deshpande AV, Kennedy SE. Epidemiology and demography of recently diagnosed cases of posterior urethral valves. Pediatr Res 2014; 76:560.

• Sarhan O, Zaccaria I, Macher MA, et al. Long-term outcome of prenatally detected posterior urethral valves: single center study of 65 cases managed by primary valve ablation. J Urol 2008; 179:307.

• DeFoor W, Clark C, Jackson E, et al. Risk factors for end stage renal disease in children with posterior urethral valves. J Urol 2008; 180:1705.

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