Clinico-Pathologic Conference

2015

Post-Graduate Interns
Ospital ng Maynila Medical Center

JA
12-year old
Male
Catholic

Loss of consciousness

Chief Complaint

Swelling of ankles, knees, and elbow joints

including phalangeal joints
intermittent undocumented low grade fever
Paracetamol given
Condition progressed until there was
difficulty in ambulation.

4 months PTA

Condition persisted
Diagnosis not known to the parents
Patient discharged improved
Home meds: Penicillin, Prednisone, AlMgOH

3 months PTA

Joint pains recurred

Ibuprofen: no relief

2 months PTA

PE: swollen knees, ankles, and phalangeal

joints, bilateral
Vital signs were within normal.
Initial impression: Juvenile Rheumatoid
Arthritis
Meds: Aspirin

Upon admission

CBC
Hemoglobin
WBC count
Platelet count

ESR
ASO
C3

107
9.1 (Neutrophils 64%)
924
78 mm/hr
Positive up to 1:4 dilution
1840 mg/L

CXR
Normal
X-ray of bilateral knees and ankles: no bone involvement

Upon admission

Rheumatology service:
Impression: JRA
SGPT/SGOT, urinalysis, Anti Nuclear Antibody: normal
Rheumatoid Factor: not done (financial constraints)
Penicillin discontinued, Aspirin was continued

Still with occasional joint pains and difficulty in ambulation

3 hospital day
rd

Joint pains resolved

Good motor activity
Aspirin decreased

8 hospital day
th

Discharged improved
Follow up at Rheumatology OPD
clinic and Rehabilitation Medicine

11 hospital day
th

Rheumatology OPD
Rheumatoid Factor:
negative

Aspirin continued
Prednisone started
However, patient was lost to follow-up.

Joint pains recurred

Polymigratory
Afebrile

2 weeks PTA

Joint pains recurred

Febrile (38.5C)

Medications were continued

until

1 day PTA

1 episode of post-prandial vomiting

Dysphagia
Loss of consciousness
Re-admitted

Few hours PTA

(+) anorexia
(-) rashes
(-) diarrhea
(-) easy fatigability
(-) headache
(-) pallor
(-) blurring of vision
(-) melena
(-) bleeding tendencies

(-) ear/nasal discharge

(-) constipation
(-) seizure
(-) cough
(-) dysuria
(-) frequency
(-) dyspnea
(-) weight loss

Review of Systems

PAST MEDICAL HISTORY:

FAMILY MEDICAL HISTORY:

BIRTH AND MATERNAL HISTORY:

Born to a 30 y/o G3P2(2012) mother
via NSD at a Lying-In clinic
No feto-maternal complications noted
Mother had irregular pre-natal check-ups at the
local health center, with irregular intake of
multivitamins and ferrous sulfate.
(-) Maternal illness

FEEDING HISTORY:
Px was breastfed up to 1 year of age then
shifted to milk formula (Bear Brand)
Px was feeding on adult diet prior to illness

IMMUNIZATION HISTORY:
(+) BCG
(+) DPT3OPV3
(+) measles
(-) Hepatitis B

DEVELOPMENTAL HISTORY:
At par with age

Asleep, not in cardio-respiratory distress

HR: 90/min RR: 30/min
BP: 140/100 (>p90) Temp: 37.5
Wt: 17.9 kg (p5)
Ht:110 cm (p10)
Weight for age: 85%
Height for age: 94% (mild stunting)
Weight for actual height: 94% (no wasting)

PHYSICAL EXAMINATION

Anicteric sclerae, slightly pale conjunctivae, (-) naso-aural

discharge, (-) tonsillo-pharyngeal congestion
Equal chest expansion, clear breath sounds, (-) retractions,
(-) crackles, (-) wheezes
Adynamic precordium, distinct heart sounds, normal rate,
regular rhythm, (-) murmur
Globular, soft abdomen, normoactive bowel sounds, nontender, no palpable mass
Full and equal pulses, (-) edema, (+) limitation of range of
motion both kneesand ankles, (+) tenderness
metatarsophalangeal joints, knees, ankles, wrists, proximal
carpo-phalangeal joints, (-) erythema

PHYSICAL EXAMINATION

Neurologic Examination

Conscious, coherent
Pupils 2 mm EBRTL
Full EOMs
(+) corneals
(-) facial asymmetry
intact gross hearing
can shrug shoulders
tongue midline
Meningeals: supple neck
Sensory: withdraws to pain on all extremities
Motor:
5/5
:
5/5
________________
3-4/5 :
3-4/5
Reflexes: ++
Cerebellar: (-) nystagmus; can do finger to nose test

Upon admission

Impression: Malignant Hypertension

secondary to steroid intake
Prednisone at 1.6 mg/kg/day
Aspirin at 54 mg/kg/day
AlMgOH
Furosemide 1 mg/kg/dose given once a day
Nifedipine 5mg/cap SL, prn for BP > 135/85
Referred back to Rheumatology service.

Upon admission
2nd HD

Rheumatology service agreed with diagnosis.

Aspirin
Gradual tapering of Prednisone up to a dose
of 0.25mg/kg/day
Afebrile
No hypertensive episodes or joint pains

Upon admission
2nd HD
3rd-4th HD

Facial edema
Adequate urine output

Upon admission
2nd HD
3rd-4th HD
5th HD

On and off joint pains

Episodes of hypertension
Facial edema persisted
Nifedipine prn continued

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD

Severe joint pains (Nalbuphine given)

Facial edema persisted
Repeat urinalysis, BUN, creatinine : Normal
results
Urine output adequate

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD

No episodes of hypertension
Tapering of Prednisone was continued

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD
9th HD

Facial edema resolved

Adequate urine output
Furosemide discontinued
Episodes of severe joint pains

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD
9th HD
11th HD

More pale
Abdomen slightly enlarged
Palpable masses on both flanks
Repeat CBC: hgb of 56
pRBC transfusion
Post BT hgb: 108

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD
9th HD
11th HD
15th HD

Abdominal UTZ: Enlarged kidneys with

bilateral renal parenchymal disease
Oncology service: Lymphoma R/O
Pheochromocytoma
Suggest: abdominal CT Scan, urinary VMA
determination (not done due to lack of funds),
and possible renal biopsy

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD
9th HD
11th HD
15th HD
19th HD

Nephrology service
Urinalysis and BUN/creatinine: normal

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD
9th HD
11th HD
15th HD
19th HD
21st HD

Nephrology service: intra-abdominal mass

Urine culture and UTZ of the adrenals (not
done due to financial constraints)
Prednisone and Aspirin discontinued
Gradual, progressive abdominal enlargement
Episodes of severe joint pains persisted

Upon admission
2nd HD
3rd-4th HD
5th HD
6th7th HD
8th HD
9th HD
11th HD
15th HD
19th HD
21st HD
28th HD

Abdominal CT scan:
Hepatomegaly with prominent spleen,
enlarged kidneys with obstructive
hydronephrosis, and retroperitoneal
lymphadenopathy. Overall findings were
consistent with a neoplastic process.

30th HD

(+) pallor
Repeat CBC: Hgb of 42
pRBC transfusion given

30th HD
36th HD

Nephrology service: suggest open renal biopsy

Referred to Urology service
Oncology service: requested for tumor markers
and serum LDH: 800 (2x elevated)
Biopsy of the retroperitoneal lymph nodes

30th HD
36th HD
38th HD

Hgb 48
pRBC transfusion
(+) severe joint pains (Tramadol)
Abdominal enlargement persisted

30th HD
36th HD
38th HD
41st HD

Hgb of 64
Platelet count 14
Platelet concentrate transfusion
Difficulty of breathing with distended
abdomen
High back rest and oxygen inhalation at 2 Lpm

30th HD
36th HD
38th HD
41st HD
47th HD

BMA
hypocellular marrow, no megakaryocytes, with
few myeloid series seen, with no signs of
maturational arrest, and most of the cells were
mature lymphocytes
Again started on Prednisone at 1 mg/kg/day

30th HD
36th HD
38th HD
41st HD
47th HD
48th HD

(+) abdominal pain

Ranitidine started

(+) Difficulty of breathing progressing to

gasping respiration
Patient intubated -> cardiac arrest

49 HD
th