Imo, this answer choice is wrong, there is no problem in the process of collagen "synthesis" per se. The issue is with excessive synthesis and disorganized deposition. Not an 'abnormal synthetic process' - as would be in EDS, MF, Menkes, etc.
BBs work by decreasing cAMP and Ca2+ thereby slowing SAN & AVN activity. This prolongs phase 4 of depolarization. Therefore, they are known to increase the duration of diastole (predominantly) causing both a rise in heart coronary perfusion and reduction in heart rate.
Did anyone else wonder WHAT "PULMONARY SYMPTOMS" is the question referring to?? There is literally not a single symptom mentioned in the whole vignette. No "crackles heard over both lung fields" are not symptoms. They are signs found by the physician.
Seriously doubting the whole NBME board test writers right now. Do they adequately revise their work? This is not the first technical mistake I realize on the new forms.
WTF is "weakness of plantar dorsiflexion" ????? it's like saying "extension flexion" This is not the only obvious technical mistake in the new NBMEs ...
Am I the only one who thought, my whole life, that it actually originates from the thyroid but just physically connected to the tongue
I think metastasis was the best option here because there are multiple malignant neoplasms... primary cancers tend to start as a single mass in the tissue of origin. In the lung, metastases are more common than primary neoplasms.
I think metastasis was the best option here because there are multiple malignant neoplasms... primary cancers tend to start as a single mass in the tissue of origin. In the lung, metastases are more common than primary neoplasms.
Sounds like a case of Li-Fraumeni syndrome - since p53 is a tumor suppressor for a bunch of cell types, mutations in this gene (as in LFS) result in a myriad of familial tumor types.
I believe this is Caplan Syndrome (bronchogenic carcinoma + rheumatoid arthritis). Only flaw to that is that the pulmonary findings don't perfectly represent pneumoconioses.
Syringomyelia normally presents with bi-lateral loss of pain/temp in a cape like distribution due to damage of the anterior white commisures of the Spino-Thalamic Tract.
This person also has wasting of the small muscles of her hand, which is due to Syrinx expansion causing damage to LMNs of the anterior horn (from CNS Pathoma)
From Faid 2019 new figure: IGF-1 mainly functions as an anabolic hormone on muscles and bones (pretty much like insulin-> decreases serum glucose). GH acts separately by promoting insulin resistance (increasing serum glucose). Therefore, IGF-1 is not the answer. If GH was among the answers it would have got really confusing.
With chronic vomiting, you lose electrolytes and a lot of acid. It triggers metabolic alkalosis which is why all the serum values are low (or on the lower end of the normal range) except for bicarbonate.
Most of the pts values were normal. Drinking wasn't outrageous, LDL was mild, BMI has fine. He did have HTN though. The biggest risk factors are the fact that he had suffered an MI and started suffering severe depression (weight loss/anxiety). Thus, he is more at risk for suicide.
Analysis of the elastin in the question showed a decreased number of desmosine cross-links. Desmosine is made up of four lysine residues. Therefore abnormal elastin is likely missing lysine necessary for the formation of these desmosine cross-links. Wikipedia article on Desmosine.
pKa is pH at which any drug is at its 50% ionized state.
Now we are alk urine i.e inc pH. when pH>pKa it will have two diff path for acidic drug & basic drug.
Acidic drug will inc its elimination (inc ionized form), basic drug will be more absorbed. so we need to know the drug is basic /acidic.
Now if u alk urine its elimination inc. so it have to be acidic. or u can know its a sodi salt of drug with CNS property i.e most like Phenobarbital (Weak acid)
so if pKa of drug is 6---at pH 7 we will start eliminating
but if pKa is 0 we need to raise pH of urine at 11 to start eliminating.at that point prev drug (pKa=6) would be totally out of system.
thats why A is the right ans (pKa = 6)
"Thyroglossal duct cysts most often present with a palpable asymptomatic midline neck mass usually below [65% of the time] the level of the hyoid bone."
"The thyroglossal tract arises from the foramen cecum at the junction of the anterior two-thirds and posterior one-third of the tongue."
https://en.wikipedia.org/wiki/Thyroglossal_cyst
Androgen Insensitivity Syndrome - Defect in androgen receptor resulting in normal-appearing female (46,XY DSD). Functioning testes causes increased testosterone at puberty, which is converted to estrogen peripherally, giving female secondary sexual characteristics (female external genitalia). Lack of androgen receptor function leads to absent or scant axillary and pubic hair. Patients have rudimentary vagina, but uterus and fallopian tubes absent.
Androgen insensitivity syndrome is the answer but you might have considered Mรผllerian agenesis (Mayer-Rokitansky- Kรผster-Hauser syndrome).
Mullerian agenesis will have normal hormone levels and may present as 1ยฐ amenorrhea (due to a lack of uterine development) in females with fully developed 2ยฐ sexual characteristics (functional ovaries). Hair development is normal as well. Patients also have normal height.
Seems like this question did not give us much to distinguish besides height and tanner stage 1 pubic/axillary hair.
APOCRINE vs. ECCRINE
Your skin has two types of sweat glands: eccrine and apocrine. Eccrine glands occur over most of your body and open directly onto the surface of your skin. Apocrine glands open into the hair follicle, leading to the surface of the skin. Apocrine glands develop in areas abundant in hair follicles, such as on your scalp, armpits and groin.
Ref: https://www.mayoclinic.org/diseases-conditions/hyperhidrosis/multimedia/sweat-glands/img-20007980