✔ B

Superior mesenteric artery syndrome (SMA) (choice B) is the correct answer. This rare, but life-threatening condition must be considered in any patient with a history of rapid weight loss presenting with gastrointestinal symptoms. SMA syndrome describes the compression of the third portion of the duodenum by the abdominal aorta and the overlying superior mesenteric artery (see image below). Retroperitoneal fat and lymphatic tissue normally serve as a cushion for the duodenum, protecting it from compression by the SMA. This cushion effect is lost in cases of rapid weight loss. The resulting duodenal obstruction causes symptoms of epigastric pain (especially postprandial), nausea, vomiting, and early satiety. Abdominal CT scan is the modality of choice to make the diagnosis however signs of duodenal obstruction (gastric distension) may be present on radiographs.

⚠ Viral gastroenteritis (choice A) can have the described symptoms, but would typically be accompanied by lower GI tract symptoms as well (diarrhea). In addition, given this risk for SMA syndrome in this patient, a viral illness would be a diagnosis of exclusion.

⚠ While appendicitis (choice C) could be a possibility in any patient with nausea, vomiting and abdominal pain, the fact that she lacks fever and has a normal white count make it less likely. In addition, appendicitis would not explain the findings of stomach distension on the radiograph.  

⚠ Nutcracker syndrome (choice D) describes compression of the left renal vein between the aorta and the SMA. Symptoms are typically left flank pain and hematuria due to venous hypertension. This is an anatomical entrapment syndrome and, unlike SMA syndrome, is not related to sudden weight loss. 

⚠ Renal colic (choice E) typically presents with lower back/flank pain radiating to the groin. Most urinary calculi develop from the age of 20-40 (peak from 35-45 years), making it less likely in this adolescent.

🔖 Key point:

Superior mesenteric artery syndrome is rare, but life-threatening condition that must be considered in any patient with a history of rapid weight loss presenting with gastrointestinal symptoms. SMA syndrome describes the compression of the third portion of the duodenum by the abdominal aorta and the overlying superior mesenteric artery.

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The ratio of plasma aldosterone concentration to plasma renin activity is the preferred initial screening test for primary hyperaldosteronism.  Adrenal suppression testing can confirm the diagnosis, and positive tests require further adrenal imaging.  Adrenal venous sampling is the most sensitive test for differentiating adrenal adenoma and bilateral adrenal hyperplasia in patients without discrete unilateral adrenal mass on imaging.

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A 43-year-old man comes to the physician reporting acute-onset right knee pain.  He was diagnosed with diabetes mellitus a year ago and takes metformin.  He does not use tobacco, alcohol, or illicit drugs.  The patient is in a monogamous relationship.  His father also has diabetes.  His temperature is 37 C (98.6 F), blood pressure is 134/86 mm Hg, pulse is 86/min, and respirations are 16/min.  BMI is 26 kg/m2.  Physical examination shows a slightly swollen and tender right knee and mild hepatomegaly.  Right knee x-ray reveals chondrocalcinosis and a moderate effusion.  Appropriate analgesic is administered for joint pain.  Which of the following is the best next step in management of this patient?

A. Anticitrullinated peptide antibodies
B. Antismooth muscle antibodies
C. Liver biopsy
D. Serum iron studies
E. Serum uric acid level
F. Slit-lamp eye examination

Clinical manifestations of hereditary hemochromatosis

Skin

Hyperpigmentation (bronze diabetes)

Musculoskeletal

Arthralgia, arthropathy & chondrocalcinosis

Gastrointestinal

Elevated hepatic enzymes with hepatomegaly (early), cirrhosis (later) & increased risk of hepatocellular carcinoma

Endocrine

Diabetes mellitus, secondary hypogonadism & hypothyroidism

Cardiac

Restrictive or dilated cardiomyopathy & conduction abnormalities

Infections

Increased susceptibility to Listeria, Vibrio vulnificus & Yersinia enterocolitica

This patient has acute monoarticular arthritis with chondrocalcinosis (calcified articular cartilage on radiographs), diagnostic of calcium pyrophosphate dihydrate crystal deposition (CPPD) disease (pseudogout).  Patients with pseudogout should be evaluated for secondary causes such as hyperparathyroidism, hypothyroidism, and hemochromatosis.  Given this patient's recently diagnosed diabetes mellitus and hepatomegaly, hereditary hemochromatosis (HH) is highly likely.  HH-induced iron deposition in the synovial fluid appears to promote CPPD.  Diabetes in HH appears to be due primarily to loss of insulin secretion and often requires injectable insulin; however, mild or early disease is frequently managed with oral agents.

The initial evaluation of HH includes serum iron studies, which will show increased levels of serum iron, ferritin, and transferrin saturation.  The diagnosis can be confirmed with genetic testing for hemochromatosis-associated mutations (eg, HFE).  Liver biopsy is not required but may be useful to stage the extent of liver involvement (eg, in patients with significant liver function test abnormalities) or to confirm the diagnosis in patients who have iron studies indicating iron overload but negative results on the classic HFE gene markers (Choice C).  Long-term management of hemochromatosis involves serial phlebotomy to deplete excess iron stores.

(Choices A and E)  Chondrocalcinosis is not a typical feature of gout or rheumatoid arthritis (which is associated with anticitrullinated peptide antibodies).

(Choice B)  Antismooth muscle antibodies are seen in autoimmune hepatitis, which can be associated with other autoimmune disorders including type 1 diabetes mellitus, thyroid disease, and rheumatoid arthritis.  Patients may develop a subacute, symmetric polyarthritis involving the small joints, but acute monoarthritis with chondrocalcinosis would not be seen.

(Choice F)  Slit-lamp eye examination is helpful in identifying the Kayser-Fleischer rings of Wilson disease.  Wilson disease can cause hepatomegaly; however, it typically presents with neuropsychiatric manifestations, and almost all patients are diagnosed before age 35.

Educational objective:

Hereditary hemochromatosis is commonly associated with calcium pyrophosphate dihydrate crystal deposition in joints, leading to chondrocalcinosis, pseudogout, and chronic arthropathy.  Patients commonly also have diabetes and liver disease.  Diagnosis is suggested by iron overload on serum iron studies and can be confirmed by genetic tests

A 76-year-old woman comes to the office for evaluation of leukopenia.  The patient came to the United States to visit her son a month ago and was seen at an urgent care clinic 2 weeks ago for acute sinusitis, where she was found to have leukopenia.  She was prescribed an oral antibiotic, and a repeat blood test was advised.  The patient has had 2 episodes of skin infections over the past 3 months.  She also has had pain and swelling in the joints of her hands and knees for the past several years.  The patient does not use tobacco, alcohol, or illicit drugs.  Temperature is 37.2 C (98.9 F), blood pressure is 130/80 mm Hg, and pulse is 74/min.  Physical examination shows normal heart and lung sounds, moderate splenomegaly, generalized lymphadenopathy, no sinus tenderness, and well-healing lower extremity ulcerations.  The small joints of the hands are swollen and deformed.  Laboratory results are as follows:

Hemoglobin 11.8 g/dL
Mean corpuscular volume 92 µm3
Platelets 280,000/mm3
Leukocytes 1,800/mm3 (20% neutrophils)
Creatinine 0.8 mg/dL
Calcium 9.2 mg/dL
Erythrocyte sedimentation rate 68 mm/h
HIV serology negative
Additional evaluation of this patient is most likely to reveal which of the following?

A. Anticitrullinated peptide antibodies
B. Decreased vitamin B12 level
C. Hypocellular bone marrow
D. Philadelphia (Ph1) chromosome
E. Serum monoclonal proteins

Felty syndrome

⚪Clinical features

Rheumatoid arthritis
Severe erosive joint disease & deformity
Rheumatoid nodules
Vasculitis (mononeuritis multiplex, necrotizing skin lesions)
Neutropenia (ANC <2000/µL)
Splenomegaly

⚪Diagnosis

Anti-CCP & RF are positive in >90% of patients
Markedly elevated ESR, often >85 mm/hr
Peripheral smear & bone marrow biopsy to rule out other causes of neutropenia

ANC = absolute neutrophil count; anti-CCP = anticyclic citrullinated peptide; ESR = erythrocyte sedimentation rate; RF = rheumatoid factor.

This patient with swollen, deformed hand joints and an elevated erythrocyte sedimentation rate likely has untreated inflammatory polyarthritis.  The presence of concurrent neutropenia and splenomegaly raise strong suspicion for Felty syndrome, an uncommon but serious complication of long-standing, erosive rheumatoid arthritis (RA).

Felty syndrome is marked by the formation of autoantibodies against neutrophil components and granulocyte colony-stimulating factor, leading to neutropenia (ie, absolute neutrophil count <2,000/mm3) and an increased risk of recurrent bacterial infection (particularly of the skin and sinuses).  Neutrophils coated with antibodies are also trapped in the spleen, which usually results in splenomegaly.  Most patients also have extraarticular manifestations of RA such as lymphadenopathy, rheumatoid nodules, and/or necrotizing skin lesions.

The diagnosis is made based on clinical features but is supported by the presence of high-titer rheumatoid factor and anticitrullinated peptide antibodies (both of which are usually elevated in RA).  Most patients are also HLA-DR4 positive (indicating a genetic susceptibility).  Other causes of neutropenia should be ruled out with bone marrow biopsy and peripheral smear prior to establishing the diagnosis.  Symptoms generally improve with treatment of the underlying RA.

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🧩 Medical Mnemonics


Causes of delirium

🇮‌  ‌🇼‌‌🇦‌‌🇹‌‌🇨‌‌🇭‌    ‌🇩‌‌🇪‌‌🇦‌‌🇹‌‌🇭‌ ❗👽


🪧 🅘nfectious (encephalitis, meningitis, UTI)

🪧 🅦ithdrawal (alcohol, benzodiazepines)
🪧 🅐cute metabolic disorder (electrolyte imbalance)
🪧 🅣rauma (head injury, postoperative)
🪧 🅒NS pathology (stroke, hemorrhage, tumor)
🪧 🅗ypoxia (anemia, cardiac failure)

🪧 🅓eficiencies (vitamin B12, folic acid, thiamine)
🪧 🅔ndocrinopathies (thyroid, glucose)
🪧 🅐cute vascular (shock, vasculitis,
hypertension)
🪧 🅣oxins, substance use, medications
🪧 🅗eavy metals (arsenic, lead, mercury)

#psychiatry 
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🧩 Medical Mnemonics

Modified Alvarado score for diagnosis of appendicitis


🌎 🇨🇦 ‌🇲‌‌🇴‌‌🇳‌‌🇹‌‌🇷‌‌🇪‌‌🇦‌‌🇱‌


❕𝗠igration 𝗢f pain to right lower quadrant (1 point)

❕𝗡ausea and vomiting (1 point)

❕𝗧enderness in right lower quadrant (2 points)

❕𝗥ebound tenderness (1 point)

❕𝗘levated temperature (1 point)

❕𝗔norexia (1 point)

❕𝗟eukocytosis >10 × 10⁹/liter (2 points)



- Score of ≥4 indicates that the patient should be further evaluated for appendicitis.


#surgery
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🧩 Medical Mnemonics


Hypokalemia causes


Eid Mubarak 🌺🌙 🎆🕋

⚡Enteric losses ( diarrhea)
⚡Insulin excess, Increase of Glucocorticoid (Cushing's, exogenous steroids, ectopic ACTH), Increased sweat losses
⚡Dialysis, Decrease of body temperature (hypothermia)

⚡Magnesium depletion
⚡Urinary losses(Diuretics)
⚡β-adrenergic activity, Bartter's or Gitelman's syndrome
⚡Alkalosis, Amphotericin B
⚡RTA types I and II
⚡Aldosteronism
⚡Ketoacidosis

#nephrology
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🧩 Medical Mnemonics

Differential diagnosis of elevated serum aminotransferases


🌠 ‌🇦‌‌🇧‌‌🇨‌‌🇩‌‌🇪‌‌🇫‌‌🇬‌‌🇭‌‌🇮‌


🪶 𝗔utoimmune hepatitis, 𝗔drenal insufficiency, 𝗔norexia nervosa
🪶 Hepatitis 𝗕
🪶 Hepatitis 𝗖, 𝗖eliac disease
🪶 𝗗rugs or toxins
🪶 𝗘thanol
🪶 𝗙atty liver
🪶 𝗚enetic disorders (Wilson disease, Hemochromatosis, Alpha-1 antitrypsin deficiency)
🪶 𝗛emodynamic disorders (CHF and MI)
🪶 𝗜nfiltration of the liver by malignancy, 𝗜njury to muscle (strenuous exercise, myopathy)

#gastroenterology
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