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Despite a variable appearance which raises a broad differential diagnosis, this tumor is considered benign with only a rare risk of recurrence. Clinical Features Pleomorphic lipoma, considered a variant of spindle cell lipoma, typically presents as a dermal or subcutaneous solitary, circumscribed nodule or mass upon the upper back/posterior neck of middle-aged to elderly men. Foci of more myxoid stromal change with associated elongate spindled cells and scattered, enlarged floret type giant cells are also seen. Scattered adipocytes, either singly or in small clusters, are observed typically at the lesion periphery. Despite the enlarged floret cells and some measure of atypia, mi to ses are not identified. Pleomorphic lipoma lacking mature fat component in extensive myxoid stroma: a great diagnostic challenge. Diagnostically challenging spindle cell lipomas: a report of 34 "low-fat" and "fat-free" variants. Porokera to tic variant of Grover disease Answer: Grover disease, porokera to tic variant Grover disease, or transient acantholytic dyskera to sis, usually presents as asymp to matic or pruritic pink papules on the trunk of middle-aged men. Exacerbating fac to rs include heat and immobility, as may occur in the post-operative period. The most frequently observed feature is focal acantholytic dyskera to sis, with changes resembling those seen in Darier disease. Three other classical patterns include the spongiotic, pemphigus-like, and Hailey-Hailey disease-like changes. More recently reported microscopic variants include: porokera to tic, lentiginous, vesicular, lichenoid, dysmaturative, and epidermolytic hyperkera to sis variants. This particular case showed focal acantholytic dyskera to sis with numerous cornoid lamellae which, in combination with clinical findings, supported a diagnosis of Grover disease, porokera to tic variant. Grover Disease: A Reappraisal of His to pathological Diagnostic Criteria in 120 Cases. Squamous cell carcinoma Answer: Hypertrophic lichen planus Lichen planus has many clinical and microscopic variants. Hypertrophic lichen planus usually presents as multiple hyperkera to tic nodules on the lower extremities. Microscopic features include lichenoid lymphohistiocytic inflammation with epidermal hyperplasia and hypergranulosis. The lack of mucin deposition, basement membrane thickening, and superficial and deep perivascular lymphocytic inflammation may aid differentiation from hypertrophic lupus erythema to sus. Direct immunofluorescence testing is optional but can show scattered and clumped cy to id bodies with multiple immune conjugates, as well as shaggy fibrinogen deposition along the basement membrane zone. A challenging clinical scenario arises when patients develop atypical, proliferative lesions in the setting of lichen planus, since squamous cell carcinoma may arise in this context. One method of improving diagnostic accuracy in this scenario is for clinicians to treat hyperkera to tic lesions with high-potency to pical corticosteroids under occlusion for a few weeks before pursuing biopsy. If the lesion regresses considerably with this treatment, continued observation may be reasonable.
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It does this at lower concentrations than its metabolite ace to l, indicating greater anticonvulsant potency. The ke to genic diet does not work in all patients, and it seems desirable to increase its efiectiveness. A possible strategy to achieve this might be to combine the diet with inhibi to rs of metabolic breakdown of ace to ne [61]. In broad outline, fatty acid synthesis is a reversal of fi-oxidation: two carbon a to ms at a time are added to a growing fatty acid molecule, and the new fi carbon is then reduced to the alkane level. The bulk of the work in fatty acid synthesis is accomplished by a single enzyme, fatty acid synthase, which is quite an amazing molecule: it combines six active sites with eight distinct catalytic activities on a single polypeptide chain. As stated before, fatty acids vary in their chain lengths and degree of bond saturation. In animals, this is a large molecule with multiple active sites located on a single polypeptide chain. Attachment is made through a phosphopantetheine group, which functions as a fiexible tether, allowing the acyl chain to travel and visit the various active sites on the enzyme in turn. In both the substrate and the product, the acetyl group is bound as a thioester, which makes this an easy reaction. Inside the ke to acyl synthase active site, the malonyl group is decarboxylated, and the carbanion that forms in the process attacks the acetyl group that had been acquired earlier. Malonyl-acetyltransferase acquires the next malonyl residue from malonyl-CoA, which is again supplied by acetyl-CoA carboxylase. The cycle executes seven times overall, which results in the formation of palmitate; the palmi to yl residue is then released as free palmitate by the thioesterase activity of fatty acid synthase. Since acetyl-CoA is formed in the mi to chondrion by pyruvate dehydrogenase, it needs to get out of the mi to chondrion and in to the cy to sol. In that case, the transport of acyl-CoA to the mi to chondria is accomplished by the carnitine carrier system (slide 10. Since all reactions in the latter system are reversible, and acetyl-CoA is also an acyl-CoA, we might expect that the carnitine carrier system could help us out here as well. The latter is reduced by malate dehydrogenase, and malate is exchanged for citrate. The pathway has been demonstrated in rat livers [62, 63] and is also found in high activity in adipose tissue [64].
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A 24-year-old woman presents to the hospital with symp to ms of nausea, vomiting, anorexia, and gross hematuria. A Foley catheter is inserted to relieve the obstruction, and 1500 cc of urine is emptied from his bladder. Which of the following urine electrolyte values is most likely in keeping with his diagnosis of post obstructive diuresisfi A 68-year-old woman uses large amounts of nonsteroidal anti-inflamma to ry analgesics for years to relieve symp to ms of back pain due to arthritis. She now has an elevated urea and creatinine consistent with chronic kidney disease. Which of the following changes in the kidney is most likely seen with her diagnosisfi A 64-year-old woman develops severe diarrhea 2 weeks after finishing antibiotics for pneumonia. The urinalysis is positive for heme-granular casts and the urine sodium is 42 mEq/L. A 64-year-old woman is seen in the clinic for routine assessment of her hypertension. On physical examination, the blood pressure is 130/78 mm Hg, heart rate 80/min, and the rest of the exam is normal. Routine electrolytes and renal function are checked and the only abnormal value is an elevated bicarbonate level of 34 mEq/L. A 32-year-old man with sickle cell anemia is seen in the clinic for routine follow-up. He feels well at the present time, but in the past he has had many sickle cell crises, which have resulted in kidney injury. The pain started suddenly the previous evening and there is no his to ry of any trauma. He mentions this pain occurs frequently over the past 8 years, and the symp to ms usually resolve with ibuprofen. Which of the following mechanisms is the most likely explanation for his renal impairmentfi A 67-year-old man presents to the emergency department with symp to ms of renal colic. Which of the following coexisting medical conditions increases the risk of contrast-induced nephropathyfi A 64-year-old man presents to the hospital with symp to ms of weight gain, shortness of breath, easy bruising, and leg swelling. A renal biopsy shows nodular deposits that have an apple-green birefringence under polarized light when stained with Congo red. A 74-year-old man presents to the clinic for assessment of symp to ms of fatigue, shortness of breath on exertion, and back and rib pain that is made worse with movement. Investigations reveal he is anemic, the calcium, urea, and creatinine are elevated.
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The partial female to male sex reversal in Wnt-4-deficient females involves induced expression of tes to sterone biosynthetic genes and tes to sterone production, and depends on androgen action. Bioinformatics enrichment to ols: paths to ward the comprehensive functional analysis of large gene lists. Fetal Leydig cells: cellular origin, morphology, life span, and special functional features. Changes in the concentration and size of testicular macrophages during development. The nuclear recep to r steroidogenic fac to r 1 is essential for the formation of the ventromedial hypothalamic nucleus. Developmental expression of mouse steroidogenic fac to r-1, an essential regula to r of the steroid hydroxylases. The nuclear recep to r steroidogenic fac to r 1 acts at multiple levels of the reproductive axis. Identification of a new gene mutated in Fraser syndrome and mouse myelencephalic blebs. Temporal transcriptional profiling of somatic and germ cells reveals biased lineage priming of sexual fate in the fetal mouse gonad. In situ hybridization analysis of anterior pituitary hormone gene expression during fetal mouse development. Wnt4 overexpression disrupts normal testicular vasculature and inhibits tes to sterone synthesis by repressing steroidogenic fac to r 1/beta-catenin synergy. Fetal programming of adult Leydig cell function by androgenic effects on stem/progeni to r cells. TopHat2: accurate alignment of transcrip to mes in the presence of insertions, deletions and gene fusions. Fibroblast growth fac to r recep to r 2 regulates proliferation and Ser to li differentiation during male sex determination. Fgf9 and Wnt4 act as antagonistic signals to regulate mammalian sex determination. Anti Mullerian hormone Bruxelles: a nonsense mutation associated with the persistent Mullerian duct syndrome. Expression of a candidate sex determining gene during mouse testis differentiation. Sex-specific timing of meiotic initiation is regulated by Cyp26b1 independent of retinoic acid signalling.
