Sadia sultana

At the end of case study learner will be able to Define Pseudohypoaldosteronism Understand the

Pathophysiology Sign and symptom Causes Riskfactor diagnostic test complication

Discuss the treatment regimen Discuss the nursing practice

Name :: xyz Age :: 4 months Body:: weight 6.50 kg Head circumference ::40 cm Chief complains:: vomiting 2_3 days, decrease feeding and lethargy.

Father work in ptcl company Other not significant.

K:: 8.2 Na :: 115 Hco3 :: 16 Bun :: 19 Urea :: 41 Cr :: 0.35 Glucose :: 71

Tab sodamint. Syp motilliulm Syp vidyalin. Tab flourinef Tab hydrocartizone K exallate(sachet)

PHA refers to a heterogeneous group of electrolyte metabolism characterize by an apparent state of renal tubular unresponsiveness or resistance to the action of aldosterone. Or A group of disorders involving an electrolyte imbalance due to kidney inability to response to aldosterone. Characterize by hyperkalemia , metabolic acidosis, and GRF.
McGraw-Hill Concise Dictionary of Modern Medicine. 2002 by The McGraw-Hill Companies, Inc.

One of the functions of aldosterone is to regulate salt levels in the body. Aldosterone stimulates Na+ and water reabsorption from the gut salivary and sweat glands in exchange for K+ Aldosterone stimulates uptake of K+ into cells.

Endocrine system condition Water electrolyte imbalance

Kidney condition
Urinary system condition

The most common causes of pseudohypoaldosteronism are

1. adrenal insufficiency (it is a condition in which the adrenal glands do not produce adequate amounts of steroid hormones primarily cortisol, but may also include impaired aldosterone production (a mineralcorticoid) which regulates K+, Na+ and H2o retention) 2. congenital adrenal hyperplasia (refers to any of several autosomal recessive diseases resulting from mutations of genes for enzymes mediating the biochemical steps of production of cortisol from cholesterol by adrenal glands) 3. medications (certain diuretics, NSAIDs, and ACE inhibitors

Occur due to Maturation disorder in no or function of aldosterone receptors. Other organs are involves such as sweat glands salivary glands and colon. Loss of function mutation in alpha or beta subunit of the epithelial sodium channel. Result in defective sodium transport in many organ including kidney lung colon sweat and salivary glands.

1. 2. 3. 4. 5.

6.
7. 8. 9.

10.
11.

Failure to weight gain. Metabolic acidosis Low blood pressure/high blood pressure. Renal salt retention/wasting. Low aldosterone level Hypovolemia. High blood potassium level Low blood sodium level Increased blood renin level Decrease blood renin level Normal blood renin volume

Measurement in blood
serum aldosterone cortisol Chem. 7 Routine labs Ecg

plasma renin activity (PRA)

Supportive management Severe mineralocorticoid insufficiency with significant hyperkalemia and metabolic acidosis can be managed with Florinef/fludrocortisone, 0.1 0.2 mg/d PO.

It is very important for patients to be educated about avoiding drugs that may aggravate the syndrome, such as ACE inhibitors, prostaglandin synthetase inhibitors (NSAID), beta blockers, and potassium-sparing diuretics. Proper follow up Avoid the diet that are rich in potassium. Take high sodium diet. Asses for complication and informed your doctor.

Possible are.

Kidney failure

Heart failure

Hypoaldosteronism, familial: A very rare genetic disorder where deficiency of a particularly chemical (aldosterone synthase) results in a deficiency of aldosterone. The condition can be severe enough to cause infant death unless the patient is diagnosed and treated.

Vital sign Monitor hydration status Monitor sign and symptom of anemia Monitor renal function test Maintain I/O chart Provide skin care Asses Neurologic status Monitor electrolyte imbalances