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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

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J.N. 2yo/F

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  1. J.N.2yo/F Cherie Tecson. M.D.

  2. 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 200 PER

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

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

  5. 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

  6. 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

  7. 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, upslantedpalpebral 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. 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

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

  10. Course in the Pay Ward 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. 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

  12. Course in the Pay Ward • Assessment: t/c Acute leukemiavs 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)

  13. Course in the Pay Ward 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 • 1st HD: (+) systolic thrill on the LLSB, with a grade 4/6 HSM LLSB • DobutamineLanoxin (0.004mkdose) BID • Furosemide (0.5mkdose) restarted

  14. Course in the Pay Ward 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. Working Impression • Down syndrome • CHD, acyanotic, VSD • Pneumonia, community acquired • Acute leukemia versus Myelodysplastic syndrome

  16. 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

  17. Course in the Ward • CXR done: NSCF • Blood CS final: (+) Enterobacteraerogenes, 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

  18. 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

  19. 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

  20. 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)

  21. 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

  22. 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

  23. 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

  24. 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

  25. 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

  26. Course in the Ward 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. 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

  28. 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

  29. Course in the Ward On 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

  30. Course in the Ward On 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%

  31. Course in the Ward On 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 36.2-38.20C 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. Course in the Ward • 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. 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

  34. 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 • Enterobacteraerogenes sepsis, resolved • Post-chemotherapy myelosuppression PCOD: Intracranial bleed secondary to DIC secondary to sepsis

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