Clinical Pathology Conference: Case of BCR (21/F with JIA, Cushing Syndrome, and Sepsis)

Comprehensive practice flashcards covering the clinical case presentation, diagnostics, hospital course, therapeutic interventions, acute deterioration, and resuscitation of patient BCR.

What are the baseline demographic details and chief complaint of patient BCR upon presentation?

The patient is a $21$-year-old female, single, unemployed, Roman Catholic, from Metro Manila, whose chief complaint was difficulty of breathing.

How did the patient's history of present illness describe the symptoms three days prior to admission?

Progressive difficulty of breathing (shortness of breath even at rest with no aggravating or alleviating factors), intermittent fever with a $T_{\text{max}}$ of $39.0\,^\circ\text{C}$ (relieved by Paracetamol $500\,\text{mg}$), and $5$ episodes of postprandial, non-bloody, non-mucoid vomiting (about half a cup each).

What significant past medical history and steroid medication history were reported for patient BCR?

She was a known case of Juvenile Idiopathic Arthritis (JIA) diagnosed in $2001$ treated with Ibuprofen $400\,\text{mg}$ TID until $2015$ (then lost to follow-up), and in July $2015$ was maintained by a private doctor on Dexamethasone $4\,\text{mg/tab}$, $1$ tab BID, which she took irregularly.

What were the initial vital signs recorded upon physical examination?

BP: $90/60\,\text{mmHg}$, HR: $150\text{s}\,\text{bpm}$, RR: $40\text{s}\,\text{cpm}$, $O_2$ saturation: $98\%$ on $2\text{--}3\,\text{LPM}$ via nasal cannula, and Temperature: $40.0\,^\circ\text{C}$.

Which physical examination findings were indicative of exogenous steroid use (Cushing syndrome)?

Moon facies on HEENT examination and a flabby globular abdomen with purplish striae.

What chest and lung examination findings were noted on admission?

Coarse crackles localized to the right lung, mid to base, with equal chest expansion and no retractions.

What abnormality was noted on the initial chest X-ray on admission?

Haziness in the right paracardiac region with a magnified heart.

What were the notable complete blood count (CBC) findings on the first hospital day?

WBC was elevated at $19.0 \times 10^9\,\text{L}$, Neutrophils were increased at $0.87$ ($87\%$), and Lymphocytes were decreased at $0.10$ ($10\%$).

What abnormalities were found on initial blood chemistry regarding renal function and electrolytes?

Creatinine was increased at $387\,\mu\text{mol/L}$, Potassium ($K^+$) was decreased at $2.86\,\text{mmol/L}$, and Chloride ($\text{Cl}^-$) was decreased at $94.5\,\text{mmol/L}$, while BUN and Sodium were normal.

What were the results of the initial coagulation studies?

Prolonged PT at $20.4\,\text{seconds}$ (control $11.9\,\text{seconds}$, INR $1.84$) and prolonged APTT at $36.9\,\text{seconds}$ (control $28.5\,\text{seconds}$).

What was the interpretation of the initial Arterial Blood Gas (ABG)?

Partially compensated respiratory alkalosis (pH $7.47$, $p\text{CO}_2$ $25.8\,\text{mmHg}$, $p\text{O}_2$ $44.7\,\text{mmHg}$, $\text{HCO}_3$ $18.2\,\text{mEq/L}$, $O_2$ Sat $84.4\%$).

What did the admission urinalysis reveal on chemical and microscopic examinations?

Chemical testing showed trace protein and $+3$ blood; microscopic examination revealed $45\text{--}50\,\text{RBC/HPF}$, few bacteria, and few amorphous urates.

What was the initial admitting impression for the patient?

Sepsis secondary to CAP-MR (Community-Acquired Pneumonia - Moderate Risk), Juvenile Idiopathic Arthritis, not in flare, and Cushing Syndrome secondary to exogenous steroids.

What initial therapeutic regimen was ordered on the second hospital day?

PNSS at $125\,\text{cc/hr}$, soft diet with SAP, Ceftriaxone $1\,\text{gm}$ IV OD, Azithromycin $500\,\text{mg/tab}$ OD, Paracetamol $300\,\text{mg}$ IV q4 PRN for fever, and Ibuprofen $400\,\text{mg}$ q6 PRN for joint pains.

What updated assessment was given by the rheumatology service on the third hospital day?

Juvenile Idiopathic Arthritis in flare, Sjogren syndrome, Hypokalemia, Acute Kidney Injury secondary to Sepsis secondary to CAP-MR on top of probable chronic kidney disease secondary to JIA (membranous nephropathy), and rule out pericardial effusion.

What additional medications were added on the third hospital day following rheumatology evaluation?

Piperacillin + Tazobactam $2.25\,\text{gm}$ IV q6, Hydrocortisone $25\,\text{mg}$ IV q12, Vitamin K $1\,\text{amp}$ IV q6 for $8$ doses, and Tramadol $50\,\text{mg}$ IV q8 for pain.

How did the patient's renal function and fluid status change on the fourth hospital day?

Positive fluid balance was managed with Furosemide $40\,\text{mg}$ IV q2 for $2$ doses; serum creatinine improved from $387\,\mu\text{mol/L}$ to $197.4\,\mu\text{mol/L}$, and potassium corrected to $3.63\,\text{mmol/L}$.

What medication adjustment was made by endocrinology on the fifth hospital day?

The dose of Hydrocortisone was increased to $100\,\text{mg}$ IV q6 due to an elevated ESR while potassium dropped to $2.7\,\text{mmol/L}$ and WBC dropped from $12.7$ to $4.9$.

Why was an ANA (antinuclear antibody) test omitted between hospital days 6 and 8 despite high C3 levels?

ANA was not done due to financial constraints.

What acute deterioration occurred later on the ninth hospital day while the patient was lying down?

Stiffening of extremities with peripheral and circumoral cyanosis, upward rolling of eyeballs lasting $3\text{--}5\,\text{minutes}$, and a sudden drop in sensorium (no eye opening to pain, no verbal output, does not follow commands).

What was the ER clinical assessment and immediate intervention following the sudden event on day 9?

Assessment was pulmonary embolism rule out CVA; the patient was intubated and hooked to a mechanical ventilator.

What complication was noted regarding the endotracheal tube on the repeat chest X-ray on the ninth hospital day?

The endotracheal tube tip was located in the right main bronchus, and repositioning was suggested.

What abnormalities were found on cranial CT scan performed on the ninth hospital day?

Mild Cerebral Atrophy, mucus retention cyst versus polyp in the right maxillary sinus, and bilateral proptosis.

What findings were observed upon endotracheal suctioning on the tenth hospital day when the patient desaturated to $80\%$?

Bloody mucoid aspirate per endotracheal tube.

What terminal cardiac rhythm was captured on ECG on the tenth hospital day, and what medication was ordered?

Supraventricular tachycardia (SVT) with right bundle branch block (RBBB); Adenosine IV was ordered.

What final resuscitation measures were performed before the patient was pronounced dead?

CPR was performed for $12\,\text{minutes}$, and $4$ doses of epinephrine were administered after the heart rate and blood pressure dropped to zero.