# A nurse is caring for a 1-month-old infant diagnosed with salt-wasting congenital adrenal hyperplasia (CAH) who was started on hormone replacement therapy 3 days ago. Which assessment finding requires the most immediate nursing intervention?

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## 문제

A nurse is caring for a 1-month-old infant diagnosed with salt-wasting congenital adrenal hyperplasia (CAH) who was started on hormone replacement therapy 3 days ago. Which assessment finding requires the most immediate nursing intervention?

The nurse is monitoring a newborn with salt-wasting congenital adrenal hyperplasia who was started on hormone replacement therapy 3 days ago.

## 보기

1. Serum sodium level of 118 mEq/L (118 mmol/L) **✔ 정답**
2. Weight loss of 200 grams since yesterday
3. Irritability and poor feeding for 6 hours
4. Ambiguous genitalia noted on physical examination

**정답: 1**

## 해설

Severe hyponatremia (sodium

## 심화 해설

Understanding the Clinical Priority in Salt-Wasting CAH

When caring for an infant with salt-wasting congenital adrenal hyperplasia (CAH), the most critical threat to life is an adrenal crisis, which is driven by severe electrolyte imbalances and hypovolemia. This condition represents the most severe form of CAH, and affected infants are at constant risk for rapid decompensation due to the lack of mineralocorticoid and glucocorticoid hormones [1]. While hormone replacement therapy is the cornerstone of management, the early days of treatment are a period of high vulnerability where fluid and electrolyte status can shift dramatically.

Analysis of Assessment Findings

To determine the priority intervention, we must apply the ABC (Airway, Breathing, Circulation) and safety framework, focusing on the finding that indicates the most immediate threat to cerebral and cardiac function.

| Assessment Finding | Pathophysiological Rationale & Clinical Significance |
| --- | --- |
| 1. Serum sodium level of 118 mEq/L (118 mmol/L) | This is a critical finding. In salt-wasting CAH, a deficiency of aldosterone leads to profound renal sodium loss and water loss, causing severe hyponatremia and hypovolemia [1,3]. A sodium level of 118 mEq/L represents severe, life-threatening hyponatremia. This rapid osmotic shift can cause cerebral edema, seizures, coma, and cardiac arrhythmias. This directly threatens the "Circulation" and neurological components of the primary survey and requires the most immediate intervention, such as a rapid fluid bolus and possible hypertonic saline administration, to prevent irreversible neurological injury. |
| 2. Weight loss of 200 grams since yesterday | An acute weight loss of this magnitude in a 1-month-old infant is highly significant and indicates severe fluid volume deficit (dehydration), a hallmark of an impending adrenal crisis [3]. While this is a critical finding that demands urgent fluid resuscitation, the immediate, life-threatening consequence of that fluid loss is the electrolyte disturbance and circulatory collapse. The sodium level provides a more direct measure of the imminent neurological danger. Weight loss confirms the hypovolemic state but is a secondary indicator to the direct measurement of the life-threatening electrolyte abnormality. |
| 3. Irritability and poor feeding for 6 hours | These are early, non-specific signs of an adrenal crisis in an infant, often accompanied by vomiting and lethargy [1,3]. They represent a significant change in neurological status and a failure to maintain adequate intake, which will rapidly worsen the hypovolemia and hyponatremia. These symptoms are the clinical manifestation of the underlying metabolic crisis. The priority is to treat the root cause—the severe hyponatremia and hypovolemia—which is most precisely identified by the laboratory value in option 1. |
| 4. Ambiguous genitalia noted on physical examination | Ambiguous genitalia is a classic finding in female infants with classical CAH due to in-utero androgen excess [1]. While this requires sensitive care, genetic counseling, and a planned diagnostic workup, it is a structural finding that does not pose an immediate threat to the infant's airway, breathing, or circulation. This is a chronic management issue, not an acute priority in the context of a potential salt-wasting crisis. |

Synthesizing the Evidence for the Priority Decision

The case reports on salt-wasting CAH consistently describe the rapid progression from non-specific symptoms like poor feeding and vomiting to a full-blown adrenal crisis characterized by severe dehydration, electrolyte imbalances, and cardiovascular collapse [1,2,3]. The underlying mechanism is the deficiency of cortisol and aldosterone, which are critical for maintaining vascular tone, glucose homeostasis, and sodium-potassium-water balance. When hormone replacement is newly initiated, the infant is still stabilizing, and any stressor or dosing inadequacy can precipitate a crisis. The finding of a serum sodium of 118 mEq/L is not just a number; it is a direct marker of the failed compensatory mechanisms and indicates that the infant is already in a state of severe, life-threatening hyponatremia. This cerebral and cardiac irritability demands an immediate nursing intervention—notifying the provider urgently and preparing to administer prescribed emergency fluids and sodium—to prevent progression to seizures, respiratory arrest, or cardiac arrhythmias. While the weight loss and clinical symptoms are deeply concerning and validate the crisis, the specific, quantifiable severity of the hyponatremia makes it the most immediate trigger for a life-saving intervention.References (research sources)

- [1]Unveiling Salt-Wasting Congenital Adrenal Hyperplasia in an Infant: A Diagnostic Challenge.Research articleKummari S, Krishna Sravya M, R M. (2026) · DOI: 10.7759/cureus.103140

- [3]A Case of Salt-Wasting Congenital Adrenal Hyperplasia Caused by a Rare Intronic Variant in the <i>CYP21A2</i> Gene.Case reportAntysheva Z, Esibov A, Avsievich E, Petriaikina E, Yudin V, Keskinov A, Yudin S, Svetlichnyy D, Krupinova J, Ivashechkin A, Katsaran Y, Woroncow M, Skvortsova V, Bogdanov V, Volchkov P. (2025) · DOI: 10.3390/ijms26146648

## 임상 시나리오

Clinical Management of Salt-Wasting Crisis in CAH

A Practical Guide for Immediate Nursing Intervention

1. Recognizing the Emergency

A serum sodium level of 118 mEq/L in an infant with salt-wasting CAH constitutes a medical emergency. This severe hyponatremia is the hallmark of an impending or active adrenal crisis. The priority is to prevent cerebral edema, seizures, and cardiac arrhythmias. Early signs like irritability and poor feeding often precede rapid decompensation, making laboratory confirmation the definitive trigger for escalation.

2. Immediate Nursing Actions

- **Notify the Provider STAT:** Report the critical sodium value and any accompanying signs of shock (tachycardia, prolonged capillary refill, hypotension) immediately.

- **Prepare for Intravenous Access and Fluid Resuscitation:** Anticipate orders for an isotonic saline bolus (e.g., 0.9% NaCl at 10-20 mL/kg) to correct hypovolemia, followed by dextrose-containing fluids to prevent hypoglycemia once initial shock is addressed.

- **Administer Emergency Hormones:** Have injectable hydrocortisone (stress dose) available for immediate administration as prescribed. This is life-saving and addresses the underlying glucocorticoid deficiency.

- **Strict Monitoring:** Place the infant on continuous cardiorespiratory monitoring and pulse oximetry. Perform frequent neurological checks (level of consciousness, fontanelle status, pupillary response) to detect early signs of cerebral edema.

3. Avoiding Complications During Correction

- **Controlled Sodium Correction:** Rapid overcorrection of chronic hyponatremia can cause osmotic demyelination syndrome. The goal is typically a correction rate not exceeding 6-8 mEq/L in 24 hours, requiring frequent serum sodium checks (every 2-4 hours initially).

- **Monitor Electrolytes and Glucose:** Closely track potassium (risk of life-threatening hyperkalemia from aldosterone deficiency) and blood glucose (risk of hypoglycemia from cortisol deficiency).

4. Family-Centered Care

Explain the rationale for all interventions to the caregivers in clear, calm language. Emphasize that the crisis is a known complication of the condition, not a failure of their home management. Reinforce sick-day rules (stress dosing) for future prevention before discharge.

## 핵심 개념

- **Congenital Adrenal Hyperplasia** — Congenital adrenal hyperplasia. A genetic disorder caused by a deficiency of the enzyme needed for cortisol synthesis in the adrenal glands. The salt-wasting type causes life-threatening dehydration and hyponatremia/hyperkalemia due to aldosterone deficiency.
- **Salt-wasting crisis** — Salt-wasting crisis. An acute condition primarily occurring in 21-hydroxylase deficiency CAH. A medical emergency characterized by vomiting, dehydration, hyponatremia, hyperkalemia, and shock.
- **Hyponatremia** — Hyponatremia. A condition where serum sodium concentration is less than 135 mEq/L. Less than 120 mEq/L is considered severe, with a high risk of neurological symptoms (irritability, lethargy, seizures) and death.
- **Hormone Replacement Therapy (for CAH)** — (For CAH) Hormone replacement therapy. This treatment supplies the deficient hormones (mainly glucocorticoids (hydrocortisone) and often mineralocorticoids (fludrocortisone)) to prevent crises and normalize growth.
- **Ambiguous genitalia** — Ambiguous genitalia. A condition where the external genitalia are not clearly distinguishable as male or female. This is a diagnostic finding commonly seen in female-type CAH (21-hydroxylase deficiency).

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