Soru

Zorluk: OrtaPediatric Endocrine Disorders

An 88-month-old boy is evaluated in the pediatric emergency department for irritability, poor feeding, and passing copious amounts of dilute-appearing urine over the past three days. His parents report that he frequently demands breast milk or water but often vomits shortly afterward. His birth and prenatal history were unremarkable. He is currently at the 55 th percentile for weight and 2525 th percentile for length, having dropped from the 5050 th percentile for weight at his 44-month visit. Temperature is 37.1∘C37.1^\circ\text{C} (98.8∘F98.8^\circ\text{F}), blood pressure is 85/50 mm Hg85/50 \text{ mm Hg}, and pulse is 160/min160/\text{min}. Physical examination shows dry mucous membranes, a sunken anterior fontanelle, and poor skin turgor. Initial laboratory studies show:

Serum sodium: 154 mEq/L154 \text{ mEq/L}
Serum potassium: 4.0 mEq/L4.0 \text{ mEq/L}
Serum osmolality: 318 mOsm/kg H2O318 \text{ mOsm/kg H}_2\text{O}
Urine osmolality: 95 mOsm/kg H2O95 \text{ mOsm/kg H}_2\text{O}

The patient is admitted, and subcutaneous desmopressin is administered. Two hours later, a repeat urine osmolality is 105 mOsm/kg H2O105 \text{ mOsm/kg H}_2\text{O}. Which of the following is the most appropriate long-term pharmacotherapy to reduce polyuria in this patient?

  1. HydrochlorothiazideCevap
  2. B
    Desmopressin
  3. C
    Tolvaptan
  4. D
    Fludrocortisone
  5. E
    Somatropin

Cevap

The most appropriate long-term pharmacotherapy for this patient is Hydrochlorothiazide.
The correct answer is hydrochlorothiazide. This infant's presentation of hypernatremia, serum hyperosmolality, and inappropriately dilute urine confirms diabetes insipidus. The lack of a significant increase in urine osmolality following the administration of exogenous desmopressin (an ADH analog) indicates renal resistance to ADH, definitively diagnosing nephrogenic diabetes insipidus (NDI). The paradoxical first-line pharmacologic treatment for NDI is a thiazide diuretic (often combined with a low-solute diet or NSAIDs like indomethacin). Thiazides induce a mild state of hypovolemia, which stimulates the proximal tubule to increase the reabsorption of sodium and water. This physiological compensation decreases the volume of filtrate delivered to the distal nephron and ADH-resistant collecting ducts, ultimately reducing total polyuria.

Adım Adım Çözüm

1
Analyze the initial clinical and laboratory presentation.
The infant presents with severe hypernatremic dehydration (154 mEq/L154 \text{ mEq/L}) and hyperosmolality (318 mOsm/kg H2O318 \text{ mOsm/kg H}_2\text{O}) alongside inappropriately dilute urine (95 mOsm/kg H2O95 \text{ mOsm/kg H}_2\text{O}), establishing a diagnosis of diabetes insipidus (DI).
High serum sodium and osmolality with low urine osmolality defines the inability to concentrate urine, characteristic of DI.
2
Interpret the result of the desmopressin administration.
The urine osmolality remains highly dilute (105 mOsm/kg H2O105 \text{ mOsm/kg H}_2\text{O}) after exogenous desmopressin, indicating an absent renal response.
Failure to concentrate urine by at least 50%50\% following ADH analog administration distinguishes nephrogenic DI from central DI.
3
Determine the optimal pharmacologic management for the established diagnosis.
The patient requires therapy for nephrogenic DI, which involves a low-solute diet and a thiazide diuretic such as hydrochlorothiazide.
Thiazides paradoxically reduce urine output in NDI by causing mild hypovolemia, which enhances proximal tubule reabsorption of sodium and water, reducing delivery to the ADH-resistant collecting ducts.

Anahtar Kavram

Nephrogenic diabetes insipidus diagnosis and paradoxical treatment with thiazide diuretics.
Bu soruyu puanla