PEDIATRICS. MEDICINE. LOADIN OB-GYNE.

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UNIVERSITY OF PERPETUAL Dr. Jose G. Tamayo HELP

Medical Center
Clinicopathologic Conference
June 27, 2011

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PHYSICAL EXAMINATION

2. HPI: 4. HPI PX CHART 12 weeks PTA 8 weeks PTA Syncope 3. HPI: Volvulus of BMI 17.9 Bowel and 10 weeks PTA sigmoid colon (+) Jaundice bladder Disoriented Central Venous Pale Palpebral incontinence Several tonicCatheter onjunctiva ECG: old clonic seizure Distended Abdomen inferior MI CT : contusion (+) Splenomegaly (-) arrythmia with CK: normal subarachnoid CT scan and hemorrhage MRI: normal (-) arrythmia

1. 71/Female
Fever Dyspnea 3/7/13 hypotension
-

CK: normal 5. HPI CT scan and 4 days PTA MRI: normal Confused Hyperthermia Leukocytosis Hypotension Tachypnea Tachycarda Gentamicin, metronidazole,

AUSCULTAT ION:

PATHOLOG Y

Grade 3 systolic ejection murmur

DAY 1

Coarse crackles on both lungs

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JAUNDICE
INTRAHEPATIC INTRAHEPATI C
LOCATION OF BILIRUBIN METABOLISM

PREHEPATIC PREHEPATIC

HEMOLYTIC POSTHEPATIC POSTHEPATI INFECTION INTRINSICANEMIAS CONJUGATIO EXTRINSIC DEFECT IN C N EXCRETION DERANGEME GENETIC ENZYME DEFECTS IN Viral Hepatitis Gilbert Dubin-Johnson DISORDERS OFCholelithiasis NT THE Compression HEMOGLOBIN of DEFECTS RBC MEMBRANE Syndrome Tree duethe Syndrome DISORDERS Biliary to STRUCTURE Periampullary Schistosomiasis Crigler-Najjar Disorders Hereditary G6PD Sickle Cell Choledocholithi Very rare (esp. in Asia) Syndrome Jaundice in teenage year Spherocytosis asis Deficiency Anemia

Cholangiocarcinoma Common in tropical and Most common inherited the patient Common amongand Gilbert Syndrome & South UnremarkableAge of Cardiovascular Almost exclusive to Pancreatic CA were Elevated MCHC White abnormality of theEarly manifestations of RBC East Asians are associated subtropical countries cholelithiasis Cell and at birth JaundicedDifferential Thalasse males (X-linked unlikely to our patients pattern manifestations membrane jaundice Jaundice Male predilection group Jaundice Count of inheritance) age Early splenomegaly Hepatosplenomegaly Manifested by Prominent pallor, chronic Anemia (-) Hepatosplenomegaly mias Unremarkable imaging (-) Fever Elevated MCHC Thalassemias Abdominal pain splenomegaly, anemia, & marked Elevation of liver anemia, (-) Course of findings jaundice, and thesplenomegaly enzymes gall Disease stones

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Ballas SK. Complications of Sickle Cell anemia in adults: Hematology Oncology Ninth Edition Schwartz's Principles ofeffective2006 p389-97, PediatricGuidelines for Surgery,Management.

Thalassemias THALASSEMI THALASSEMI PREVALENT IN HUMID COUNTRIES MAJO A A Heterozygous: No (MEDITERRANEAN, ARABS, & ASIAN) First 2 years of life MOST COMMON GENETIC DISORDERS clinical manifestation R Severe Anemia

RBC Transfusion for survival

except for Mild Anemia Homozygous: Incompatible with life

INTER MEDIA

MINO R
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Clinically silent

American Family Physician 2ndINTERNAL MEDICINE Harrison's PRINCIPLES OF ed. Mosby Elsevier, E. Goljan, Pathology, Journal.2009Aug15; http://www.medicinen

Destruction Epistaxis of Removal of Biliary Obstruction Normal Urine THALASSEMIAS Developing Malabsorp -THALASSEMIAHemolysis Diminished Increased Alpha Normoblast in Defect ProductionD ofERBCE of Chains IndirectNormal tion of OLV R S s Globin Hgb Tetramers within theStool Color fats & Bilirubin Ineffective Chain ! Blood Transfusion tion Hypochromia Normoblast Decreased soluble Hypoxia Erythropoie Produc Microcytosis s vitamins episodes of sis Increased (ANEMIA) Blood Increased Moderate StoneTransfusion Arise from 2 to 6 Epistaxis (Vit. K) Autosomal Erythropoi Anemia years ofMassive age Conjugation Recessive Formatio Erythroid Resolved by etin Cholecyst Efficient Laparoscopic Hyperplasia Increased Direct Splenic Oxygen n olithiasis Capable(Marrow & Secretion Jaundice, Cholecystectomy without Bilirubin INTERMEDIA Pallor in 2 siblings. Epigastric (Obstruct Hepatosplenomegaly, sequestra Nutrients Pain regular blood & Weight Gain Extramedullary
Enlargement of transfusion Sites) spleen & liver Removal of cystic Nutrients duct stone transient Abdominal Splenomegaly

PATHOPHYSIOLOGY

Bleeding:

Decreased release Jaundice Tea-colored Urine of Gall GI Manifestations, distention; Retarded Jaundice Subsided Mirizzis Skeletal Defects Traubes Bone Deformitiesdefective bladder, Cachexia syndrone RBC space Cystic Increased Icterus PMI 4th ICS obliteratio duct, absorption of LMCL Spontaneous passage of bile dietary Iron n frequency of occurrence CBD duct stones: and relation to clinical tion
Biliary Stones

ion) Acholic Stools

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Jejunal segment thickening

Vichinsky EP: Changing patterns of thalassemia worldwide. Ann N Y AcadSci 2005, 1054:18-24 Cunningham MJ, Macklin EA, Neufeld EJ,OF PEDIATRICS, Clinical NELSON TEXTBOOK Cohen AR:MH Thompson presentation SE Tranter, Thalassemia 18/E Research Network. Complications of beta-thalassemia major in North America. Department of by Saunders, Bristol Trust, Copyright Cotran Pathologic Basis2004, 104:34-39 Robbins and 2007Surgery, North anof Disease. Blood imprint of

Hemolytic Anemia, FINAL IAGNO DprobablySIS Beta-Thalassemia Intermedia; Probable transient Mirizzis Syndrome; Cholecystolithiasis, S/P Laparoscopic Cholecystectomy

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Thank You for Listening!!!

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