LOOK BEYOND THE OBVIOUS DR. PADMASANI L. N DR. ARUNA RAJENDRAN PRESENTOR : DR. VIGNATHA SAJJA

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1 LOOK BEYOND THE OBVIOUS DR. PADMASANI L. N DR. ARUNA RAJENDRAN PRESENTOR : DR. VIGNATHA SAJJA

2 HISTORY Baby X, 5 year old, Girl from Madhya Pradesh CHIEF COMPLAINTS : Fever and body pain x 5 days Abdomen pain x 1day One episode of vomiting

3 HISTORY HISTORY OF PRESENTING ILLNESS : Fever for 5 days High grade, intermittent, no chills / rigors. Headache & pain in the limbs since 5 days Abdominal pain since 1 day Severe 1 episode of vomiting Containing food, not blood/bile stained Tiredness and Breathlessness x 1 day

4 HISTORY No h/o bleeding manifestations ( Haematemesis, Melaena ) No h/o blood transfusions No h/o convulsions/hemiplegia/psychic changes No h/o visual disturbances No h/o diarrhoea or constipation No h/o pica No h/o walking barefoot / passing worms in stools

5 HISTORY H/o PAST ILLNESS : Jaundice one year back took treatment with the local doctor (Details not known) Pain in the limbs off and on since one year of age (Taking pain killers) No h/o chronic infections TB, HIV No previous h/o treatment with drugs leading to anemia (Chloramphenicol, quinine, sulfonamides)

6 HISTORY Antenatal History,Natal History, Post Natal History : Non contributory B.Wt 2.5 kg, No h/o Neonatal jaundice DEVELOPMENTAL HISTORY: Normal IMMUNIZATION / NUTRITIONAL HISTORY : Not contributary

7 HISTORY FAMILY AND SIBLING HISTORY : SOCIO ECONOMIC HISTORY : Class IV (According to Modified Kuppuswamy scale) 5 YEARS 1 YEAR No h/o bleeding tendencies, anemia, jaundice, gall stones, blood transfusions, splenectomy in the family. 1 year old brother - healthy (according to the parents) H/O anemia during pregnancy mother (no BT)

8 EXAMINATION Vital signs Temperature F, HR 126/min RR 28/min, BP 100/60 mmhg Spo 2 96% in Room air General Physical examination Pallor Bony tenderness + No Icterus / Cyanosis /Clubbing / Koilonychia /Lymphadenopathy / Oedema / Dysmorphic facies /rashes / signs of avitaminosis

9 EXAMINATION ANTHROPOMETRY : PARAMETER OBSERVED EXPECTED INFERENCE (WHO) Weight 12.6 kg 18.2 kg < 3 rd centile Z score < -2 Height 95 cm 109 cm < 3 rd centile Z score < -3 Weight for Height Mid arm circumference 12.6 kg 14 kg 15 th centile Z score cm cm Normal

10 SYSTEMIC EXAMINATION Abdomen - Liver - 4 cm below RCM, firm, non tender, rounded margins. Spleen 7 cm below LCM, firm, tender, rounded margins. Cardio Vascular System Normal. Other systems Normal.

11 LIVER 4 cm below RCM SPLEEN 7cm below LCM

12 CLINICAL DIAGNOSIS Fever with Anaemia and HSM 1.Malaria (Fever, Hepatosplenomegaly, pallor, body pains ) 2.Acute leukaemia (Fever, Hepatosplenomegaly, pallor, bone pains) 3.Haemolytic Anaemia (Hepatosplenomegaly, severe pallor, previous history of jaundice)

13 INVESTIGATIONS Hb- 4.4gm% Total count- 3400cells/dl. RBC lakhs MCV MCH-28.1 MCHC Platelets-54,000 PT-14.2/12.2 PTT- 40.8/28.3 INR Reticulocyte count-3.2 (Corrected Reticulocyte count 1.2) Blood group- O +ve. DCT- negative

14 INVESTIGATIONS Total bilirubin-3.95 Direct SGOT- 65 SGPT- 38 Alkaline phosphatase- 109 LDH- 853 Total protein- 7.7 Albumin- 4.0 Globulin- 3.7 BUN- 21 Creatinine- 0.3 Na K Cl HCO

15 INVESTIGATIONS Peripheral smear examination: RBC- Microcytic hypochromic with many macrocytes with marked anisopoikilocytes, target cells, fragmented RBC. WBC- Leukopenia Platelets- Thrombocytopenia, aprox 50,000 cells/mm 3 Hemoparasites - many trophozoites of PLASMODIUM VIVAX seen IMPRESSION: Pancytopenia with plasmodium vivax infection.

16 INVESTIGATIONS Urine routine & culture: Normal Blood culture: No growth

17 TREATMENT GIVEN Anti malarials Analgesics Urgent PRBC transfusion Supportive treatment. Child became afebrile on day 5 of admission and Intensity of bone pain decreased

18 FEVER CHART Chloroquine added

19 DIAGNOSIS PLASMODIUM VIVAX MALARIA Unanswered questions 1. Severe bone pains since one year of age 2. Moderate to massive splenomegaly 3. Prior h/o jaundice

20 SIBLING ON EXAMINATION Examination Pallor + Liver Just Palpable. Spleen 4 cm, firm, non tender, round margins.

21 INVESTIGATIONS SIBILING s investigations: Hb- 5.9 Total counts- 23,000 Platelet count 3.00 MCV MCH MCHC Reticulocyte count- 2.9 DCT- Negative

22 PERIPHERAL SMEAR OF SIBLING SHOWING SICKLE CELLS

23 INVESTIGATIONS Peripheral smear examination: RBC- Anisopoikilocytosis, NCNC, sickle cells, target cells, nucleated RBCs seen in plenty. Few microspherocytes seen. WBC- corrected count 23,000. Leukocytosis with left shift. Platelets- Adequate. Hemoparasites- Absent. IMPRESSION: Sickle cell anemia

24 SICKLING TEST

25 SIBLING

26 PARENTS Father and mother underwent Hb Electrophoresis

27 FATHER

28 MOTHER

29 DIAGNOSIS IN INDEX CASE PLASMODIUM VIVAX MALARIA UNDERLYING SICKLE CELL ANEMIA INFECTION precipitated VASO-OCCLUSIVE CRISIS & SPLENIC SEQUESTRATION

30 TREATMENT Planned treatment: Tab Pencillin prophylaxis Cap Hydroxyurea Vaccines against Encapsulated organisms (Hemophilus.influenza, Streptococcus.Pneumonia, Meningococcus)

SICKLE CELL ANAEMIA Prevalence in India 1 0 18 % in north eastern India 0

32 SICKLE CELL ANAEMIA Prevalence in India % in north eastern India % in western India % in central India 1 40 % in southern India.

36 TREATMENT : Hydroxyurea Pain management Red blood cell transfusion Immunization Penicillin prophylaxis Hematopoiteic stem cell transplantation

37 ANTICIPATORY GUIDANCE Spleen palpation. Trans cranial Doppler ultrasound. Screening for pulmonary / renal involvement Retinopathy. Echocardiography.

38 SICKLE CELL ANEMIA AND MALARIA 2 HbAS has protection from PLASMODIUM FALCIPARUM AND NOT PLASMODIUM VIVAX HbAS had no effect on the prevalence of symptomless parasitemia. 50% protective against mild clinical malaria. 90% protective against severe or complicated malaria.

40 Splenic sequestration crises Anemia, thrombocytopenia, hypovolemic shock, and sudden death Classical age HbSS 6 months to 3 years HbSß, HbSC even in older children (> 10 years) Sickle cell anemia (HbSS) autosplenectomy by 5 years Sickle cell disease (HbSß, HbSC) splenomegaly can persist in older children Treatment transfusion; later date - splenectomy Naymagon L 1, Pendurti G 1, Billett HH 1. Acute Splenic Sequestration Crisis in Adult Sickle Cell Disease: A Report of 16 Cases. Hemoglobin

41 PRECIPITATING FACTORS - CRISIS A Acidosis, anesthesia, anxiety, (high) altitude B Bouts of infection, bad habits, e.g. smoking, alcohol C Cold exposure D Dehydration E Exercise (vigorous) F Folate deficiency (e.g. megaloblastic crisis) G General surgery H Hypoxia I Infection O Other trauma, menstruation.

42 CONCLUSION Even when the diagnosis is obvious, Seek an explanation for each symptom.

43 REVIEW OF LITERATURE 1. Narendra Kumar Gupta and Meenakshi Gupta; Sickle Cell Anemia with Malaria: A Rare Case Report; Indian J Hematol Blood Transfus Mar; 30(1): H. Franklin Bunn. The triumph of good over evil: protection by the sickle gene against malaria. Blood 2013; 121: N A Alli, M Patel, H D Alli et al. Recommendations for the management of sickle cell disease in South Africa. SAMJ 2014;104(11) :

44 Thank You

Sickle cell disease. Fareed Omar 10 March 2018

Sickle cell disease. Fareed Omar 10 March 2018 Sickle cell disease Fareed Omar 10 March 2018 Physiology Haemoglobin structure HbA2: 2α and 2δ chains (2-3%) HbF: 2α and 2γ chains (