E4

Question 1

Adrenal crisis pathophysiology and management?

Answer 1

Pathophysiology
Decreased corticotropin-releasing hormone (CRH) is caused by prolonged iatrogenic suppression of the hypothalamic-pituitary-adrenal axis from chronic glucocorticoid use.
Decreased production and release of CRH leads to decreased endogenous ACTH production and, in turn, insufficient serum cortisol levels in the absence of exogenous glucocorticoids.

Treatment includes administration of high doses of hydrocortisone, fluid replacement, and correction of hypoglycemia and electrolyte imbalances, often with intensive care monitoring.

Question 2

Normal serum osmolality?

Answer 2

275 - 295 mOsm/kg water

Question 3

low urine osmolarity?

Answer 3

depend on serum osmolarity

Question 4

recommendation for screening with dual-energy x-ray absorptiometry ?

Answer 4

for all women ≥ 65 years and in younger individuals if additional risk factors are present (e.g., prolonged glucocorticoid use, low BMI (< 21 kg/m2) or weight < 127 lb, alcohol use, smoking, amenorrhea).

Question 5

precocious puberty cause?

Answer 5

Central: is caused by increased gonadotropin-releasing hormone (GnRH) secretion. This excess secretion is typically idiopathic but can be caused by several tumors (e.g., hamartomas, astrocytomas, pinealomas).
Peripheral precocious puberty is caused by GnRH-independent exposure to sex hormones, which can be caused by granulosa cell tumors, Leydig cell tumors, congenital adrenal hyperplasia, and McCune-Albright syndrome.

Question 6

Type 1 renal tubular acidosis?

Answer 6

is associated with decreased activity of the H+/K+ ATPase antiporter on the apical surface of intercalated cells, which reabsorbs K+ and secretes H+ into the lumen of the tubule. This subsequently results in hypokalemia and hyperchloremic metabolic acidosis with urine that cannot be acidified to a pH < 5.5.
The most likely cause of type 1 RTA in an infant is an inherited gene defect. However, an ultrasound is usually performed to rule out other causes such as nephrocalcinosis, medullary sponge kidney, and reflux nephropathy.
Treatment of type 1 RTA consists of alkalization therapy with orally administered sodium bicarbonate or sodium citrate.

Question 7

Pinealoma hormonal complication?

Answer 7

The most common pinealoma in children is a germinoma, which often secretes β-hCG. Increased β-hCG stimulates the Leydig cells of the testes to produce excessive testosterone, which causes precocious puberty in boys. Other sequelae that may develop include hydrocephalus (due to obstruction of cerebrospinal fluid outflow pathways) and ataxia (due to cerebellar involvement). Treatment of pinealomas consists of focal radiation or chemotherapy.

Question 8

Cushing syndrome ECG?

Answer 8

ECG is a sign of hypokalemia(like T wave flatting) , which is a common finding in patients with

Question 9

a generalized tonic-clonic seizure. Normal blood tests, a negative toxicology screen, and the absence of findings on cranial imaging what to do next?

Answer 9

In patients with a new-onset seizure, electroencephalography is indicated if an epileptic disorder is suspected. Characteristic EEG findings – bursts of abnormal, rhythmic discharges featuring spikes and/or sharp waves with an abrupt onset and termination – establish the diagnosis of epilepsy.

Question 10

Primary hypercortisolism managment?

Answer 10

which is typically caused by autonomous overproduction of cortisol by the adrenal gland (most commonly due to adrenal adenoma).
Excess cortisol production suppresses pituitary secretion of ACTH, leading to low ACTH levels and ACTH-independent Cushing syndrome, as seen here. The treatment of choice for patients with adrenocortical tumors is laparoscopic or open adrenalectomy.
After surgical therapy, patients who develop adrenal insufficiency require lifelong glucocorticoid replacement therapy.

Question 11

Dysdiadochokinesia?

Answer 11

intention tremors, and a wide-based gait indicate a cerebellar cause for this patient’s ataxia and vertigo. Degeneration of the superior cerebellar vermis is the most common cause of subacute or chronic ataxia in patients with alcohol use disorder (seen in 1% of cases). Cerebellar degeneration in this patient group occurs due to the direct toxic effects of alcohol or as a result of thiamine deficiency.

Question 12

Test to do next in delayed menarche with secondary sexual characteristic?

Answer 12

An x-ray of the left hand and wrist is commonly used to determine bone age and should be performed in almost all children with short stature to determine whether their growth is delayed or accelerated (e.g., by precocious puberty). This patient was of normal length at birth and both parents are of normal size, which suggests a constitutional growth delay. Patients with constitutional growth delay typically have a delayed bone age and, after a brief downward shift in percentiles after birth, a constant growth rate at or below the 3rd percentile during childhood.

Question 13

Obesity in children risk?

Answer 13

is associated with an overproduction of leptin, which results in increased GnRH pulsatility.
This, in turn, triggers central precocious puberty (CPP). Central precocious sexual development usually follows the physiological order of sexual development (i.e. thelarche, pubarche, menarche), which is consistent with this girl’s symptoms.

Question 14

screening for type 2 diabetes mellitus?

Answer 14

Native Americans have the highest prevalence of type 2 diabetes mellitus among race groups in the United States, the reasons for which are a combination of genetic and socioeconomic factors. According to the American Diabetes Association (ADA) and USPSTF, patients with an elevated BMI who are between 40 and 70 years of age should undergo regular screening for type 2 diabetes mellitus. All patients with at least one additional risk factor for type 2 diabetes mellitus should undergo screening even before 40 years of age. While smoking, diet, and alcohol use are risk factors that are generally assessed in routine health maintenance examinations

Question 15

endometrial hyperplasia. Biopsy confirmed the absence of atypia, so the treatment?

Answer 15

choice in this patient is progestin therapy. Progestin opposes the stimulating effect of estrogen on the endometrium, thereby preventing hyperplasia and the occurrence of heavy vaginal bleeding. Good regression rates have been reported in > 98% of women after 3–6 months of progestin therapy. Follow-up with ultrasound after 3–6 months of conservative treatment is recommended to rule out progression to endometrial hyperplasia with aplasia or endometrial carcinoma.

Question 16

This patient with metastatic thyroid cancer can present with?

Answer 16

most likely developed lytic bone lesions with consequent hypercalcemia of malignancy. 
Patients with mild to moderate hypercalcemia are typically asymptomatic or develop symptoms such as constipation, fatigue, and polyuria. 
Severe hypercalcemia (hypercalcemic crisis), which is seen here, is a life-threatening condition that manifests with oliguria/anuria, dehydration, gastrointestinal symptoms, and an altered level of consciousness. 
The treatment for severe hypercalcemia includes immediate administration of normal saline to replenish volume loss and dilute serum calcium, as well as pharmacotherapy with calcitonin, bisphosphonates, or denosumab to inhibit bone resorption and calcium release. Hemodialysis can be considered in patients who do not respond to therapy or who cannot tolerate large amounts of intravenous saline because of the increased risk of fluid overload (e.g., due to acute kidney injury or heart failure).

Question 17

Type 4 renal tubular acidosis (RTA) is?

Answer 17

Caused by aldosterone deficiency and/or aldosterone resistance in the distal convoluted tubules and collecting duct. This patient with poorly regulated type 2 diabetes mellitus and elevated creatinine most likely has diabetic nephropathy, which can cause hyporeninemic hypoaldosteronism due to damage to the juxtaglomerular apparatus.
Angiotensin-converting enzyme inhibitors (e.g., enalapril) diminish aldosterone release by inhibiting the conversion of angiotensin I to angiotensin II and can thereby also cause type 4 RTA and worsen hyperkalemia.
In the healthy nephron, aldosterone increases sodium reabsorption in exchange for potassium and hydrogen excretion; the relative deficiency of aldosterone causes the accumulation of excess potassium and hydrogen ions in the serum (explaining the mild hyperkalemia and acidosis). The urine is appropriately acidic, as the nephron is attempting to compensate for the acidic serum by reabsorbing bicarbonate, another hallmark of type 4 RTA.
Treatment consists of furosemide to lower potassium and fludrocortisone as mineralocorticoid replacement.