J.N. 2yo/F

J.N.2yo/F

Cherie Tecson. M.D.

Pertinent History Known case of CHD, acyanotic, VSD; Down Syndrome at

birth 7 months PTA (+) generalized pallor

Consult with a local hospital A> t/c blood dyscrasia

Admitted, transfused w/ pRBC 1 week PTA (+) generalized pallor, (+)cough, (+) fever

Consult c/o LHC, A> Pneumonia Treated with Amoxicillin, Salbutamol

2 days PTA (+) pallor with tachypnea and fever Consult with a local physician

CBC: Hgb of 73, Hct of 0.23, WBC 5.3,

seg 36, lym 64, plt 200PER

Pertinent HistoryReview of Systems (+) poor weight gain (-) aural discharge (+) intermittency in feeding (+) dental caries (+) good urine output (-) cyanosis (-) seizures

Family History (+) HPN – maternal grandmother

Pertinent HistoryPast Medical History: (+) CHD, acyanotic, VSD – diagnosed

at 1yo, given Furosemide and Digoxin

Immunization History: (+) BCG (+) DPT1 (+) OPV1

Pertinent History  Developmental History: Good head control Speaks in monosyllables Gesture language Can grasp objects Can roll over Can sit with support 

Approximate Developmental Age: 6-7 months

Pertinent History Nutritional History: Bottlefed since birth. Eats regular table

food at present. Personal and Social History: Patient is 2nd of 3 children Mother is a 39yo teacher Father is 36yo and is unemployed

Pertinent PE Awake, alert, in mild cardiorespiratory distress,

(+) pallor HR 120s RR 40s T 37 BP 90/60 Wt 26kg BSA

0.37m2

Pale palpebral conjunctivae, upslanted palpebral fissures, anicteric sclera, (-) cervical lymphadenopathy, (-) tonsillopharyngeal congestion

Equal chest expansion, no retractions, (+) occasional rhonchi , both lung fields

Dynamic precordium, (-) precordial bulge, distinct heart sounds, (-) thrill, regular rhythm, (+) grade 2/6 holosystolic murmur at the left lower sternal border, (+) LV heave

Pertinent PE Flat abdomen, soft, normoactive bowel sounds,

(-) masses, (-) hepatosplenomegaly No cyanosis, no edema, fair and equal pulses,

CRT <2 secs, (+) pale nailbeds

Neurologic Exam Awake, irritable, active No cranial nerve deficits Motor: moves all extremities spontaneously Sensory: withdraws to pain, all extremities DTRs +2, all extremities (-) Babinski, (-) Clonus

Working Impression Down syndrome CHD, acyanotic, VSD Pneumonia, community acquired Rule out acute leukemia

Course in the Pay WardProblem 1: HematologicS> 6 month history of generalized pallorO> HR 120s RR 40s T 37 BP 90/60

(+) generalized pallor, (-) CLADs, (-) hepatosplenomegaly

A> To consider Acute leukemia vs MDSP> Work-ups requested CBC:

54/0.176/4.31/0.27/0.51/0.05/0.02/0.01/stabs 0.07/blast 0.04/28, retic ct 0.013

Course in the Pay Ward Seen by Dr. Lesaca-Medina (2nd HD)PBS review: ~5% WBCs = blasts most blasts large w/ scanty, non-granular,

blue cytoplasm w/ round or slightly irregular nucleoli and prominent punched-out nucleoli

One blast with cytoplasmic blebs (+) large platelets (+) poor, dysplastic segmenters Nucleated RBCs

Course in the Pay Ward Assessment: t/c Acute leukemia vs MDS Placed on O2 at 5lpm/FM Transfused with 2 aliquots pRBC Repeat HHP:82/0.254/22 Transfused with 1 aliquot pRBC Weaned to 2-3lpm/NC discontinued (4th

HD) Repeat CBC: 86/0.261/2.91/blast 0.04/ 21 Transferred to Charity (7th Pay Ward Day)

Course in the Pay WardProblem 2: CardiacS> Known case of Down syndrome with VSDO> Dynamic precordium, distinct heart sounds, (-)

thrills, regular rhythm, (+) grade 4/6 HSM at LLSBA> CHD, acyanotic, VSDP> Started on Dobutamine (5mcg/kg/min) 2D echo: CHD, intact VSD, large 7-8mm L to R

shunting, LVH 1st HD: (+) systolic thrill on the LLSB, with a grade

4/6 HSM LLSB Dobutamine Lanoxin (0.004mkdose) BID Furosemide (0.5mkdose) restarted

Course in the Pay WardProblem 2: Infectious S> (+) 1 week history of cough with

progressive tachypnea and fever O> Equal chest expansion, no retractions,

(+) occasional rhonchi , both lung fields A> pneumonia, community acquired P> Started on Chloramphenicol (100mkd),

discontinued after 1 day Initial blood CS: NGA5D

Working Impression Down syndrome CHD, acyanotic, VSD Pneumonia, community acquired Acute leukemia versus Myelodysplastic

syndrome

Course in the Ward On ward admission, transfused with pRBC

(10cc/kg) x 1 aliquot and 1 unit platelet concentrate

BMA done on 2nd HD Dry tap On repeat HHP: 127/0.365/20 BT of Plt con On repeat HHP: 68/0.30968 BMA repeated on 10th Ward Day: AML On repeat HHP: 91/0.267/8 BT of plt con On repeat HHP: 85/0.25/41 BT of pRBC On the 14th HD: (+) Febrile episodes

Course in the Ward CXR done: NSCF Blood CS final: (+) Enterobacter aerogenes, S:

Ceftazidime, Amikacin Repeat CBC:

63/0.187/3.170.511/0.423/0.050.003/34 BT of pRBC and plt con facilitated Repeat HHP: 84/0.251/40 Repeat Blood CS: NGA2D Chemotherapy started (2007 Chemo Protocol for

patients with Down Syndrome and AML from Journal of Clinical Oncology 12/1/07, Vol25,No34) + CNS leukemia prophylaxis on 28th HD

Course in the Ward Chemo meds: IT Methotrexate (D0), Doxorubicin

(D1-2), Cytarabine (D1-7), Etoposide (D3-5) CBC 6 days postchemo:

91/0.263/1.65/0.770/0.23/15) Blood CS on 14th day of antibiotics: (+)

Pseudomonas aeruginosa Ceftazidime Meropenem (60mkd) Amikacin continued (15mkd) Repeat CBC: 87/0.251/0.31/lympho 0.05/40 On 39th ward day: (+) 3 episodes of watery stools On PE: soft abdomen, normoactive bowel sounds Losses replaced with PLR volume/volume

Course in the Ward On the 40th Ward Day: (+) 2 episodes of

postprandial vomiting Plain abdominal x-ray: Good bowel gas pattern Fecalysis: yellowish, brown, soft, (-) RBC, (+) 3-

6 WBC On the 41st Ward day: (+) episodes of vomiting,

bilous, 3 episodes with 4 episodes of loose stools

On PE: soft, hypoactive bowel sounds Assessment: To consider septic ileus

Course in the Ward Placed on NPO, Hgt Q12 with BE NGT inserted: drained 400cc coffee ground

material, replaced with PLR volume/volume Started on Famotidine (0.8) Dobutamine increased to 8mcg/kg/min O2 support increased to 10pm/FM ABG: compensated metabolic acidosis with

respiratory alkalosis (7.379/27.7/148.8/16.4/7.1/98.8)

Course in the Ward On the 42nd HD, referred for bloody output per

NGT, ~30cc Replaced with PNSS volume/volume Assessment: t/c Disseminated intravascular

coagulation probably secondary to sepsis, Rule out fungal sepsis

Meropenem increased to 120mkd Oral meds placed on hold Placed on standby intubation CBC: 64/0.182/0.04/0/0.03/plt ct 3 BT of pRBC and plt con facilitated

Course in the Ward PT/PTT: 12.7/17/0.49/1.61; 34.9/66.9 BT of FFP facilitated Calcium noted: 1.81 Calcium gluconate (100mkdose) Q8 started Fluconazole (10mkd) started Vitamin K (1) OD started Latest blood chem: hypokalemia 1.4 Fast correction (0.5mkdose) given Referred to PICU for co-management

Course in the Ward At 4:20 pm, consented to intubation Intubated by Anesthesia service w/ ET 4.5 L 10.5

@ MV settings 100% 18/5 RR 20 Itime 0.5 VBG post-intubation: 7.312/43.6/46.9/22/-

3.4/77.5 RR increased to 30, PIP increased to 20 Seen by PICU Amikacin shifted to Vancomycin (60mkd) Fluconazole ordered to shift to Amphotericin B NAC 1g IV Q4 ordered KCl (1mkdose) fast correction given MV settings revised to 100% 20/8 RR 20

Itime 0.8 Maintained on Midazolam (0.2mkdose) Q2

Course in the Ward On the 43rd HD: Still persistently febrile, no hypotensive

episodes, O2 sats 95%, liber edge palpable 2cm BRCM

ABG @ 100% 20/8 RR 20 Itime 0.8: 7.474/31/61.9/22.7/1/93.3

I: 1385 O: 973 +412 fluid balance UO 5.5 cc/kg/hr

BT of pRBC and plt con continued Serum potassium: 1.8 fast correction at

1mkdose given

Course in the Ward Conferred with service consultant Furosemide maintenance placed on hold IVF revised to D5LR + 26meqs KCl/L

(0.25meqs/kg/hr) Conferred with PICU IVF revised to D5LR + 40mews KCl/L (del

0.3meqs/kg/hr) Post-BT Furosemide decreased to

0.3mkdose

Course in the WardAt 7:15pm (+) acute onset pallor with anisocoria, dyspnea,

mottling and cyanosis On ambubagging, O2 sats: 61% Given 20cc/kg PLR On auscultation: HR 0 Code called PALS initiated 10cc/kg PLR given Dopamine (20), Dobutamine (20) started Revived after 30 minutes

Course in the Ward Assessment for code: t/c IC bleed Post-code: BP 110/40, HR 150s, RR 42; cold

extremities, fair pulses, (+) subcostal and intercostal retractions, clear breath sounds

Given 2meqs/kg NaHCO3 Given another 20cc/kg PNSS IV bolus Repeat ABG: 7.095/40.5/31.1/12.4/-17.4/39.3 PIP increased to 22, RR increased to 30 Repeat ABG @ 100% 22/8 RR 30 Itime 0.8:

7.472/18.5/93.5/13.5/-6.3/97.5 RR weaned up to 25

Course in the Ward At this time, noted with increasing

abdominal girth Repeat ABG: 7.386/23.6/40.2/14.1/-

9.3/73.4 Noted Serum K at 1.5 fast correction

with KCl at 1mkdose

Course in the WardOn the 44th HD Referred for desaturations, 40-65%, with eye blinking Assessment: t/c Acute symptomatic seizure probably

secondary to IC bleed Loaded with Phenobarbital (20mkdose) Ordered for EEG and stat Cranial CT scan Seen by PICU: shifted to D5NR +40meqs KCl MV settings revised to 100% 20/8 RR 20 I time 0.8 ABG done @ 100% 20/8 RR 24 Itime 0.8:

7.281/20.6/68.5/9.7/-14.5/91% Given 2meqs/kg NaHCO3

Course in the WardOn the 45th HD Repeat blood CS: NGA2D CBC: 165/0.438/0.19/0/0.10/28 I: 1060 O: 330 +730 fluid bal UO 1.8cc/kg/hr HR 120-130s, RR 28-50, Temp 36.5-37, O2 sats

80-98% ABG @ 100% 20/8 RR 24 Itime 0.8:

7.429/28.5/87.3/18.8/-3.3/96.8 maintained At 3:50pm, noted with (+) crackles, BLF, bipedal

edema, puffy eyelids IVF rate decreased to FM + 20%

Course in the WardOn the 46th HD I: 844 O: 20 +824 fluid bal UO 0.1cc/kg/hr BP 90-110/60-70, HR 120-160, RR 30-42, T 36.2-

38.20CAt 11am Referred for (-) UO Given 20cc/kg PNSS IV bolus ABG: 7.29/31.7/66/15.2/-10/90.3 Given 2meqs/kg NaHCO3 Noted with anisocoria, right pupil 5mm, left pupil

3mm, NRTL, HR 110/60, RR 24, HR 160’s Assessment: t/c increased ICP probably

secondary to IC bleed

Course in the Ward Hyperventilation done Fundoscopy done: (+) diffuse papilledema, OU Given Mannitol 2.5cc/kg/dose Stat cranial CT scan facilitatedAt 3pm Referred for poor pulses, HR 160s, BP 0 Given 20cc/kg PNSS IV bolus On repeat BP: 100/0 20cc/kg PNSS IV bolus given Noted with multiple petechiae over the face and

chest with bleeding per NGT, per orem and per nostrils, anterior fontanels tense

Course in the Ward After 3 minutes, referred for lack of

response to tactile stimulation On auscultation, HR = 0, BP = 0 Code called PALS initiated Patient not revived after 30 minutes of

resuscitation Pronounced expired at 1:06pm Post-mortem care rendered

Final Diagnosis Pseudomonas aeruginosa sepsis Disseminated intravascular coagulation Acute myelogenous leukemia CHD, acyanotic, VSD 7-8 mm L to R shunting Down syndrome s/p Cycle 1 chemotherapy Enterobacter aerogenes sepsis, resolved Post-chemotherapy myelosuppression

PCOD: Intracranial bleed secondary to DIC secondary to sepsis