2.  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 200

3. Review of Systems  (+) poor weight gain  (-) aural discharge  (+) intermittency in feeding  (+) dental caries  (+) good urine output  (-) cyanosis  (-) seizures Family History  (+) HPN – maternal grandmother

4. Past Medical History:  (+) CHD, acyanotic, VSD – diagnosed at 1yo, given Furosemide and Digoxin Immunization History:  (+) BCG  (+) DPT1  (+) OPV1

5. 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

6. Nutritional History:  Bottlefed since birth. Eats regular table food at present. Personal and Social History:  Patient is 2 nd of 3 children  Mother is a 39yo teacher  Father is 36yo and is unemployed

7.  Awake, alert, in mild cardiorespiratory distress, (+) pallor  HR 120s RR 40s T 37 BP 90/60 Wt 26kg BSA 0.37m 2  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

8.  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

9.  Down syndrome  CHD, acyanotic, VSD  Pneumonia, community acquired  Rule out acute leukemia

10. Problem 1: Hematologic S> 6 month history of generalized pallor O> HR 120s RR 40s T 37 BP 90/60 (+) generalized pallor, (-) CLADs, (-) hepatosplenomegaly A> To consider Acute leukemia vs MDS P> 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

11.  Seen by Dr. Lesaca-Medina (2 nd 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

12.  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 (4 th HD)  Repeat CBC: 86/0.261/2.91/blast 0.04/ 21  Transferred to Charity (7 th Pay Ward Day)

13. Problem 2: Cardiac S> Known case of Down syndrome with VSD O> Dynamic precordium, distinct heart sounds, (-) thrills, regular rhythm, (+) grade 4/6 HSM at LLSB A> CHD, acyanotic, VSD P> Started on Dobutamine (5mcg/kg/min)  2D echo: CHD, intact VSD, large 7-8mm L to R shunting, LVH  1 st HD: (+) systolic thrill on the LLSB, with a grade 4/6 HSM LLSB  Dobutamine  Lanoxin (0.004mkdose) BID  Furosemide (0.5mkdose) restarted

14. Problem 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

15.  Down syndrome  CHD, acyanotic, VSD  Pneumonia, community acquired  Acute leukemia versus Myelodysplastic syndrome

16.  On ward admission, transfused with pRBC (10cc/kg) x 1 aliquot and 1 unit platelet concentrate  BMA done on 2 nd HD  Dry tap  On repeat HHP: 127/0.365/20  BT of Plt con  On repeat HHP: 68/0.30968  BMA repeated on 10 th 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 14 th HD: (+) Febrile episodes

17.  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 28 th HD

18.  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 14 th day of antibiotics: (+) Pseudomonas aeruginosa  Ceftazidime  Meropenem (60mkd)  Amikacin continued (15mkd)  Repeat CBC: 87/0.251/0.31/lympho 0.05/40  On 39 th ward day: (+) 3 episodes of watery stools  On PE: soft abdomen, normoactive bowel sounds  Losses replaced with PLR volume/volume

19.  On the 40 th 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 41 st 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

20.  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)

21.  On the 42 nd 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

22.  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

23.  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

24.  On the 43 rd 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

25.  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

26. At 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

27.  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

28.  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

29. On the 44 th 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

30. On the 45 th 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%

31. On the 46 th 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 36.2-38.2 0 C At 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

32.  Hyperventilation done  Fundoscopy done: (+) diffuse papilledema, OU  Given Mannitol 2.5cc/kg/dose  Stat cranial CT scan facilitated At 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

33.  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

34.  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