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PICU Morbidity

PICU Morbidity. Khlaire D. Pioquinto, M.D. Objectives. To present a case of an infant with jaundice. To discuss the probable treatable causes of acute liver failure in infancy. General Patient Information. CC 2 months old Male Date of Admission: January 2, 2014. Vomiting.

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PICU Morbidity

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  1. PICU Morbidity Khlaire D. Pioquinto, M.D.

  2. Objectives • To present a case of an infant with jaundice. • To discuss the probable treatable causes of acute liver failure in infancy.

  3. General Patient Information • CC • 2 months old • Male • Date of Admission: January 2, 2014

  4. Vomiting Chief Complaint

  5. History of Present Illness • Product of IVF, no consanguinity • Born full term via NSD to a 36 y.o. G1P1 (1001) at 37 1/7 weeks AOG • BW 2655g, BL 47, HC 31cm, MT 38 weeks, AGA, AS 9,9 • Stayed in the NICU for hypoglycemia (Hgt 12 mg/dL), hyperbilirubinemia sec. to ABO incompatibility, clinical sepsis (blood culture negative), completed 7-day course of IV Ampicillin and Cefotaxime • Sent home on the 8th day of life stable with decreased jaundice

  6. History of Present Illness

  7. History of Present Illness ER Consult

  8. Maternal History • In-vitro fertilization – 2x, worked up for TORCH infection – told to have normal results • Regular prenatal check-up • No infection • OGCT – normal • No BP elevations • HBsAg nonreactive • No intake of teratogenic drugs

  9. Past Medical History • Normal newborn screen

  10. Family History • Maternal side: (+) hypertension, diabetes, psoriasis

  11. At the ER • T 36.8C HR 140 RR 40 BP 80/50 • Wt 3.8kg (z-score = 0.5) • Generalized jaundice • Ictericsclerae, pink conjunctivae • HC 39cm; flat, patent fontanelles • Nonsunken eyeballs, moist lips • Clear breath sounds • Regular cardiac rhythm • Soft abdomen, distended, AC 38cm • Scrotal mass, (+) transillumination, right • Full pulses, no cyanosis

  12. Hyperbilirubinemia, etiology to be determinedVomiting, unspecifiedHydrocoele, right Assessment

  13. Plans Diagnostics Therapeutics IVF: D5IMB at maintenance rate • CBCPC • Urinalysis • Bilirubin levels • ALT, AST • Hgt • Whole abdominal ultrasound

  14. Hgt = 84 mg/dL

  15. Ultrasound of the abdomen • Mildly prominent-sized kidneys, may be due to mild inflammation changes or may still be normal. • Unremarkable liver, pancreas and spleen. • Partially contracted gallbladder. • Negative biliaryectasia. • Negative for mass ascites.

  16. 3rd Hospital Day

  17. PLAN PICU Transfer

  18. Lactulose q6 Corrected via with proper IVF with K incorporation Vitamin K 1mkdose SIVP, OD N-Acetylcysteine

  19. 25% albumin transfusion

  20. Plans Therapeutics Diagnostics Daily AC monitoring Referral to a Geneticist for metabolic genetic workup of hepatic failure Urinary organic acid profile Referral Hepatobiliary Surgeon • Hypokalemia and Hyponatremia correction • Acetylcysteine 500mg/IV every 4 hours • Spironolactone 25mg/tab, 6mg pptab/NGT BID (1.5mkdose) • Vit A, D, E, K, UDCA, Zinc

  21. Family conference • Discussed the diagnosis of CMV hepatitis and hepatic failure • Acquisition of CMV infection • Patient probably acquired infection via transmission transplacentally from the mother (1-3 mos of pregnancy) • Plan • Confirm CMV infection (test mother) • Metabolic work-up: test liver parameters to monitor for prognostication purposes • Liver biopsy – cannot be done yet

  22. Family conference • Option: • Ultimately, liver transplant • Supportive treatment: • Nutrition, Vitamins, UDCA, Spironolactone, Albumin transfusion, Lactulose • Goals: • Decrease jaundice • Normalize PT, PTT • Prognosis • Prevalence

  23. 3rd Hospital day Ampicillin IV 105mkday Amikacin IV 57mkday Vitamin ADEK UDCA Spironolactone N-Acetylcysteine Lactulose Ranitidine 4mg/IV q 8

  24. 4th Hospital Day

  25. 4th Hospital Day Transferred to regular room on the 6th Hospital Day

  26. Treatable cause of acute liver failure in infancy • Cytomegalovirus infection • Petechiae or purpura (79%), hepatosplenomegaly (74%), jaundice (63%), prematurity and blueberry muffin spots consistent with extramedullaryhematopoiesis. • Laboratory abnormalities: elevated hepatic transaminase and bilirubin levels (as much as half is conjugated), anemia, and thrombocytopenia. • Hyperbilirubinemia persists beyond the period of physiologic jaundice.

  27. Treatable cause of acute liver failure in infancy • Cytomegalovirus infection CMV PCR: 298 copies/mL

  28. Treatable cause of acute liver failure in infancy • Hepatitis B • Mother and patient: HBsAg negative • Galactosemia • newborn screen negative, no diarrhea on exclusive breastfeeding • Hereditary fructose intolerance • no intake of fructose yet • Congenital syphilis • Both mother and patient are VDRL negative

  29. Treatable cause of acute liver failure in infancy • Neuroblastoma • Ultrasound liver normal in size with homogenous parenchymalechopattern, smooth contour, no discrete focal mass lesion, no intrahepatic bile duct dilatation, portal vein normal in caliber, gall bladder partially contracted 2.6 x 0.62 cm, visualized common bile duct 0.26 cm in diameter

  30. Treatable cause of acute liver failure in infancy • Hereditary tyrosinemia • urine organic acid, succinylacetate screen negative • Mitochondrial defects • serum lactate normal at 11.8 mg/dl (NV 4.5-19.82) • HIV • Mother negative in her IVF work up

  31. Treatable cause of acute liver failure in infancy • Neonatal hemochromatosis • serum ferritin slightly increased at 413 ng/ml (NV 8.7-71 • Transferrin saturation 68% (NV 15-55%) • Reticulocyte count normal at 0.006( NV 0.005-0.15) • Liver biopsy not done as INR has been >3.5 despite Vitamin K daily.   • No active bleeding.  

  32. 11th Hospital Day Discharged

  33. Acute Liver Failure probably secondary to Severe Neonatal HepatitisHepatic Encephalopathy Final Diagnosis

  34. Home Medications • Multivitamins • Vit ADEK • UDCA • Zinc • N-Acetylcysteine 500mg/IV every 4 hours • Propranolol 10mg/tab, 4mg/pptab 3x a day • Spironolactone 4mg/pptab 2x a day • Oral KCl 5meq BID

  35. Update • 4months old • Currently in Singapore where the patient is continuously being worked up pending possible liver transplant

  36. Galactosemia • Most common metabolic cause of liver disease in the neonate. • Inherited as an autosomal recessive trait and develops because of the deficiency of galactose-1-phosphate uridyltransferase(GALT). • Clinical manifestations: jaundice, hepatosplenomegaly, feeding difficulties and vomiting, hypoglycemia, lethargy, irritability, seizures, cataracts. • Delayed diagnosis results in cirrhosis and mental retardation • Management consists of substituting a lactose-free formula for breast-feeding or for a standard formula, and, later, a galactose-free diet. Cloherty J. Eichenwald E., Hansen A., Manual of Neonatal Care, 7th edition

  37. Hereditary Fructose Intolerance • deficiency of the enzyme aldolase B • asymptomatic until they ingest fructose, sucrose, or sorbitol • Clinical manifestations: hypoglycemia, jaundice, hepatomegaly, vomiting, lethargy, irritability, seizures, and abnormal LFTs • Management is done by elimination of sucrose, fructose, and sorbitol from the diet Cloherty J. Eichenwald E., Hansen A., Manual of Neonatal Care, 7th edition

  38. Hereditary Tyrosinemia • A low protein diet may be required in the management of tyrosinemia. Recent experience with NTBC has shown to be very effective. The most effective treatment in patients with tyrosinemia type I seems to be full or partial liver transplant.

  39. Hereditary hemochromatosis • a common autosomal recessive disorder that results in excessive iron deposition in the liver as well as in extrahepatic sites • Also known as neonatal iron storage disease • frequently are premature or are small for gestational age • features of liver failure with hypoalbuminemia, hypoglycemia, coagulopathy, low fibrinogen, and, frequently, thrombocytopenia and anemia.

  40. Liver dysfunction. Galactosemia is the most common metabolic cause of liver disease in the neonate. Hepatomegaly with hypoglycemia and seizures suggest glycogenosis type I or III, gluconeogenesis defects, or hyperinsulinism. Hereditary fructose intolerance (when there is ingestion of fructose or sucrose, in the neonate usually a soy formula), tyrosinemia type I, neonatal hemochromatosis, and mitochondrial diseases can also present predominantly with liver dysfunction in the neonate. Cholestatic jaundice with failure to thrive is observed primarily in alpha-1-antitrypsin deficiency, Byler disease, and Niemann-Pick disease type C.

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