In caring for an infant after circumcision, the nurse observes continued bleeding from the site and suspects hemophilia. Which hematological components are insufficient causing bleeding if hemophilia is present?

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a progressive neurodegenerative disorder affecting motor neurons in the brain and spinal cord. Understanding the pathophysiological process of ALS is crucial for providing accurate information about the disease prognosis to the client. Here's why option B is the correct choice:

A) It occurs as a complication of a spinal cord injury:

This statement is incorrect. ALS is not a complication of a spinal cord injury. While both conditions involve motor neuron dysfunction, they have different etiologies and pathophysiological processes. ALS is characterized by the degeneration of motor neurons in the brain and spinal cord, leading to muscle weakness and atrophy, whereas spinal cord injury results from trauma to the spinal cord.

B) Muscle weakness is progressive, degenerative, and fatal:

Correct. ALS is characterized by progressive degeneration of motor neurons, leading to muscle weakness, atrophy, and eventual paralysis. The disease is relentless and fatal, typically within 2 to 5 years of diagnosis, although survival can vary widely among individuals. As motor neurons degenerate, voluntary muscle control is lost, eventually affecting the ability to speak, swallow, breathe, and move. Respiratory failure is the most common cause of death in ALS patients.

C) Mental status changes occur late in the disease:

While cognitive and behavioral changes can occur in some individuals with ALS, particularly in the later stages of the disease, they are not universal. ALS primarily affects motor neurons, leading to progressive muscle weakness and paralysis. However, some individuals may experience frontotemporal dementia (FTD), a type of cognitive impairment characterized by changes in behavior, personality, and language.

D) Autonomic nervous system and sensory changes occur:

ALS primarily affects motor neurons rather than sensory neurons or the autonomic nervous system. Sensory symptoms such as numbness, tingling, or loss of sensation are not typical features of ALS. Autonomic dysfunction, including changes in heart rate, blood pressure, or bowel and bladder function, is not a prominent feature of ALS.

Polycystic kidney disease (PKD) is a genetic disorder characterized by the formation of multiple fluid-filled cysts in the kidneys. One of the complications associated with PKD is hypertension, which often occurs due to activation of the renin-angiotensin-aldosterone system (RAAS). Here's how the pathophysiological process of the RAAS contributes to the client's elevated blood pressure:

A) Intravascular fluid deficit:

In polycystic kidney disease, the development of multiple cysts in the kidneys can impair renal function and lead to decreased filtration and reabsorption capacity. However, this impairment typically leads to fluid retention rather than intravascular fluid deficit, contributing to hypertension rather than hypotension.

B) Renin angiotensin mechanism:

Correct. In PKD, the cysts disrupt normal kidney architecture and function, leading to activation of the renin-angiotensin-aldosterone system (RAAS). Reduced renal blood flow and glomerular filtration rate (GFR) stimulate the release of renin from the juxtaglomerular cells of the kidneys. Renin acts on angiotensinogen to convert it into angiotensin I, which is then converted to angiotensin II by angiotensin-converting enzyme (ACE). Angiotensin II is a potent vasoconstrictor that increases peripheral vascular resistance, leading to elevated blood pressure. Additionally, angiotensin II stimulates the secretion of aldosterone, which promotes sodium and water retention, further contributing to hypertension.

C) Inflammatory process of bladder mucosa:

This option is not directly related to the pathophysiological process of hypertension in polycystic kidney disease. Flank pain and hematuria in PKD are often associated with cyst rupture or hemorrhage within the cysts rather than an inflammatory process of the bladder mucosa.

D) Mineral precipitation in urine:

Mineral precipitation in urine, such as the formation of kidney stones, can occur in polycystic kidney disease but is not directly associated with hypertension. Kidney stones may contribute to flank pain and hematuria but do not typically cause systemic hypertension.