Answer: B. Formation of γ₄ tetramers with markedly increased oxygen affinity and poor tissue oxygen release
Diagnosis
This newborn has Hb Bart's hydrops fetalis syndrome, the most severe form of α-thalassemia, caused by deletion of all four α-globin genes (--/--).
Why?
In fetal life, the major non-α chain is γ (gamma). When no α chains are available, excess γ chains combine to form:
Hb Bart's = γ₄ tetramers
These tetramers:
Have extremely high oxygen affinity
Bind oxygen tightly
Fail to release oxygen effectively to tissues
The result is profound fetal tissue hypoxia despite oxygen being present in the blood.
Consequences
Severe anemia
High-output cardiac failure
Generalized edema (hydrops fetalis)
Ascites
Pleural and pericardial effusions
Usually intrauterine death or death shortly after birth without intervention
Why the other options are wrong
A. β₄ tetramers → HbH disease (three α-gene deletions), not hydrops fetalis.
C. Reduced β-chain synthesis with excess α chains → β-thalassemia.
D. HbS polymerization → Sickle cell disease.
E. Defective heme synthesis with ring sideroblasts → Sideroblastic anemia.
Exam Pearl
4 α-gene deletions → Hb Bart's (γ₄) → Hydrops fetalis
3 α-gene deletions → HbH disease (β₄)
2 deletions → α-thalassemia trait
1 deletion → Silent carrier
✅ Correct answer: B.
The location and appearance are highly suggestive of an infraorbital dermoid cyst.
🔍 Why this fits best:
Congenital lesion presenting in infancy
Slowly progressive, painless swelling
Well-circumscribed subcutaneous mass
Typical location along embryonic fusion lines near the orbit
📌 Important differentials:
Deep infantile hemangioma → usually more compressible/bluish with Doppler vascularity
Dacryocystocele → more medial near the medial canthus
Rhabdomyosarcoma → rapid aggressive progression with proptosis/inflammation
🧬 Dermoid cysts are choristomas formed by trapped ectodermal tissue during embryologic closure.
\text{Embryonic fusion defect} \rightarrow \text{Ectodermal inclusion cyst (Dermoid cyst)}
📷 Best initial investigation:
Ultrasound with Doppler
CT/MRI if deep orbital extension is suspected
💡 High-yield pearl: Periorbital dermoid cysts classically occur at the frontozygomatic suture (superolateral brow), but infraorbital variants can occur along the maxillary-zygomatic fusion plane.
A man spends 50 years teaching at MIT.
He knows his time is running out.
So he records one last lecture — everything he knows, distilled into a single hour.
He died 5 months later.
This is that lecture.
The most important hour you'll watch this week. 👇
Bookmark it for later
#May2026#OutNow
Lots of clinically relevant EM content this month covering 🚁 prehospital care, 🧠 diagnostics, 📚 education, and ⚡ systems redesign.
Well worth a read for anyone working in emergency care 👇
https://t.co/LnhpEelCGL
#EmergencyMedicine#FOAMed#EMJ
Jamaican Olympian Josh Kirlew delivered a standout performance at the recent Aquatics GB Championships, breaking his own national record in the 100-metre butterfly with a time of 53.24 seconds at the London Aquatics Centre.
READ MORE HERE: https://t.co/gHPwmlOdiD
During my visit to Stony Hill yesterday, I had a chance to reconnect with one of my teachers from St. Catherine High School, Ms. Hyacinth “Esther” McLean.
Engaging with her brought back so many memories.
Teachers like Ms. McLean are the quiet architects of our future. They guide, inspire, and invest in young minds, often without recognition.
I am deeply grateful for her dedication and the lessons that have stayed with me all these years.
This is Mariano Barbacid, a Spanish cancer researcher who recently made the news after finding a cure for an aggressive form of pancreatic cancer in mice. His lab developed a new triple therapy regimen targeting the KRAS cancer pathway, implicated in 90% of pancreatic cancers. The combination of daraxonsib, a RAS(ON) inhibitor, afatinib (EGFR/HER2 inhibitor) and SD36 (a selective STAT3 PROTAC) led to complete elimination of tumour cells over a 200 day study period, bringing much needed hope to this often devastating condition.
The man who allegedly abducted the six-year-old girl from school in Clarendon on Tuesday, apparently dragged her through bushes to evade those searching for her.
READ MORE HERE: https://t.co/ZbvsWxxp1N
A crash broke a pole last weekend. Normally, repairing this means a scheduled outage
Knowing you’ve waited long enough, our crews decided to work LIVE to keep your power ON during repairs.
We can't do this every time. Tek Time Drive! Accidents damage the grid & can slow us down