The US FDA has approved efanesoctocog alfa (Altuviiio), a first-in-class, high-sustained factor VIII replacement therapy for adults and children with hemophilia A. The product is used once a week and is indicated for routine prophylaxis and on-demand treatment to control bleeding episodes, as well as to control bleeding during surgery (perioperative management).
History: A 24-year-old male presented to the hospital with a cough productive of reddish sputum and shortness of breath of 6 days duration. The exam showed a left testicular mass. What’s the finding in the chest x-ray?
Answer: Cannonball metastases refer to multiple large, well-circumscribed, round pulmonary metastases. The primary tumors for these lesions can be remembered with the help of this mnemonic:
CRESP
Mnemonic
C: choriocarcinoma
R: renal cell carcinoma
E: endometrial carcinoma
S: synovial sarcoma
P: prostate carcinoma
The US FDA approved a new indication for sacituzumab govitecan for patients with unresectable, locally advanced or metastatic hormone receptor (HR)-positive, HER2-negative breast cancer after endocrine-based therapy and at least two additional systemic therapies for metastatic disease.
Adverse events: decreased leukocyte count, decreased neutrophil count, decreased hemoglobin, decreased lymphocyte count, diarrhea, fatigue, nausea, alopecia, glucose elevation, constipation, and decreased albumin. Labeling for the agent carries a black box warning of severe or life-threatening neutropenia and severe diarrhea.
Dose: 10 mg/kg IV on days 1 and 8 of 21-day cycles until disease progression or unacceptable toxicity.
Sacituzumab govitecan was previously approved for unresectable, locally advanced or metastatic triple-negative breast cancer after two or more prior systemic therapies and locally advanced or metastatic urothelial cancer after platinum-based chemotherapy and either a PD-1 or PD-L1 inhibitor.
History: A 60 yr old male had developed livedo reticularis on the left side of his face after he underwent stenting and balloon dilation of the left common carotid artery. Shivering, sweating, and confusion developed immediately after artery dilation, along with a left gaze preference, dysarthria, and hemiparesis on the right side of his body. Angiography showed a patent left internal carotid artery and an occluded distal left facial artery. No IC bleed on NCCT head. What’s the diagnosis?
Answer: The patient was transferred to the ICU, where MRI angiography of the brain revealed infarctions in the territory of the left middle cerebral artery with patent vasculature. An acute embolic stroke and cholesterol embolization syndrome of the face was diagnosed. In cholesterol embolization syndrome, atherosclerotic plaque contents from large-caliber arteries embolize to smaller arteries and lead to vascular occlusion, inflammation, and end-organ damage. Livedo reticularis is one of the most common skin manifestations of the syndrome. The patient received supportive care in the ICU. The livedo reticularis resolved 1 week after the event, but the neurologic deficits persisted at the time of his discharge from the hospital.
History: A healthy 1-year-old boy with the unremarkable medical history presented to a private clinic for a routine examination. Flushing & gustatory sweating was noted on the child’s left cheek. What’s the diagnosis?
Answer: Auriculotemporal nerve syndrome (Frey’s syndrome, Baillarger’s syndrome, Dupuy’s syndrome, or Frey-Baillarger syndrome) usually manifests as immediate unilateral or bilateral flushing, sweating in the distribution of the auriculotemporal nerve, or both in response to gustatory or tactile stimuli. In adults, this syndrome is a well-recognized sequela of parotid surgery, trauma, or infection. It occurs rarely in children, most often noted after the introduction of solid food. The flushing is often attributed erroneously to food allergy. It typically begins at 2–6 months of age when solid foods, mostly fruit, are introduced. Occurring within a few seconds of eating, it has a peculiar distribution in a triangular area that extends from the tragus of the ear to the midpoint of the cheek. It is not associated with sweating and persists for 20–60 min. The flushing continues to occur for up to 5 years. In adults, gustatory sweating is the predominant feature of auriculotemporal nerve syndrome; flushing happens less often. One-half of pediatric patients with this symptom were delivered with forceps assistance, which possibly causes trauma to the nerve. The likely mechanism is a misdirection of parasympathetic fibers along sympathetic pathways during the nerve regeneration that follows trauma. This may account for erythema when eating. The emergence of symptoms several months after the proposed trauma (usually 3–6 months) is probably related to the time required for nerve regeneration, and it is possible that vigorous chewing causes intense stimulation of the parotid gland.
The US FDA approved pirtobrutinib (Jaypirca) for relapsed or refractory mantle cell lymphoma (MCL) after at least two lines of systemic therapy, including a Bruton’s tyrosine kinase (BTK) inhibitor. Pirtobrutinib is the first and only non-covalent BTK inhibitor.
Dose: 200 mg once-daily
Adverse reactions: Fatigue, musculoskeletal pain, diarrhea, edema, dyspnea, pneumonia, bruising, decreased neutrophil counts, lymphocyte counts, and platelet counts.
History: A 12-year-old boy presents with a sore throat, difficulty swallowing, inability to fully open his mouth, trismus, drooling, and a “hot-potato” voice. What is the diagnosis?
Answer: Peritonsillar abscess, aka quinsy is an accumulation of pus due to an infection behind the tonsil.
Symptoms: Fever, throat pain, trouble opening the mouth, and a change to the voice. Pain is usually worse on one side.
Complications: Blockage of the airway or aspiration pneumonitis, retropharyngeal abscess.
Cause: Commonly involved aerobic pathogens include Streptococcus, Staphylococcus, and Haemophilus. The most common anaerobic species include Fusobacterium necrophorum, Peptostreptococcus, Prevotella species, and Bacteroides.
Diagnosis: Medical imaging may include CT scan, MRI, or ultrasound.
Treatment: Antibiotics, volume repletion with fluids, and pain medication, in cases where airway obstruction or systemic sepsis occurs, surgical drainage may be necessary.
Two classes of drugs may be more effective than others for the treatment of gastroparesis, though the overall quality of evidence remains low to moderate and additional data are needed, according to a new report. Oral dopamine antagonists and tachykinin-1 antagonists appear superior to placebo. Only one drug, the dopamine antagonist metoclopramide, has US Food and Drug Administration approval for the treatment of gastroparesis (Gastroparesis is a chronic disorder which means delayed stomach emptying without a blockage).
History: A 16-year-old male patient who is known to have HBSS disease, presented with a history of chest and back pain for a few days prior to admission. He also complained of a nonproductive cough for 2 days but denied fever. On examination, his HR 110, RR 25, Temp – 103F. On chest exam, he had left basal crackles with decreased air entry. His chest x-ray showed left retrocardiac and left lower lung zone opacity suggesting left lower lobe consolidation. What is the likely diagnosis?
Answer: Acute chest syndrome is a vaso-occlusive crisis of the pulmonary vasculature commonly seen in people with sickle cell anemia. This condition commonly manifests with a new opacification of the lung(s) on a chest x-ray.
Signs and symptoms:
ACS is defined by a new pulmonary density on chest imaging involving at least one complete lung segment and at least one of the following [68]:
Investigations: CBC, CXR, Blood culture, LFT, KFT, ABG
Treatment: Bronchodilators, Antibiotics, Supplemental oxygen, Non-invasive/ invasive ventilation.
History: A 65 year old male with HTN presented to the ED with a 1-week history of progressive SOB. On physical examination, BP 140/62 mm Hg, HR 120 bpm, RR 30, and SP02 92% on 3L of O2 by nasal cannula. The cardiac examination was notable for a crescendo–decrescendo systolic murmur and a decrescendo diastolic murmur. The ophthalmologic examination revealed dilation and constriction of the pupils, synchronized with the patient’s heartbeat. What’s the diagnosis?
Answer: Landolfi’s sign is seen in patients with severe aortic regurgitation and is a manifestation of wide pulse pressure and large stroke volume in the iridial vessels, which causes systolic constriction and diastolic dilation of the pupils. Transthoracic echocardiography revealed severe aortic regurgitation with dilatation of the ascending aorta and a dissection flap. CT scan of the aorta showed a Stanford type A aortic dissection. The patient underwent the replacement of his ascending aorta and aortic valve (Bentall procedure). On discharge 10 days later, Landolfi’s sign was no longer present.
