.

JAUNDICE

IS NOT HEPATITIS ALL THE TIME

CASE PRESENTATION
My patient Mr. xyz 12 years old male child from Lahore presented in shalamar pediatric emergency on 1st may 2012 With complaints of : Jaundice 1 month Fever 15 days

HOPI:
According to mother child was alright 1 month back when she noticed yellowish discoloration of skin n sclera that gradually increased along with the generalized pallor as well urine was also dark colored without any evidence of blood in it

Child was taken to GP who treated him as acute viral hepatitis n Hepamerz was given for 10 to 15 days According to mother the yellowness decreased but persisted n pallor increase

15 days back he also developed fever

That was initially low grade n intermittent for a weak then became high grade n continuous documented up to 104F Associated with rigors n chills every time Only temporarily relieved with medication

2 days back he developed c/o abdominal pain

on n off that was generalized mild to moderate in intensity not radiating to back or groin preceded by nausea n followed by vomiting from last 1 day n then get relieved

Vomiting
4 to 5 episodes for 1 day not tolerating any food or liquid non projectile vomitus containing food particles n mucous no blood or greenish liquid n small to large in quantity No C/O loose motions or constipation No c/o bleeding from any body orifice or bruises over the body No urinary problems, headache, photophobia, night sweats,

BIRTH BIRTH HISTORY HISTORY

Child was delivered at home by dai with no routine antenatal checkups to any gynecologist n no noteworthy complication reported On 2nd day of life child was admitted in shalamar NNU due C/O fever n jaundice n remain admitted for 22 days According to mother phototherapy was given for several days along with iv

PASTPAST HISTORY HISTORY:PAST HISTORY

H/O recurrent attacks of jaundice n pallor whenever child gets sick n particularly when becomes febrile since infancy H/O blood transfusions twice at MYO hospital due marked pallor Multiple hospital admissions n investigations done in past but no record available No H/O repeated infections, petichae, bruises, rite hypochondrial pain, leg ulcers, altered sensorium, abnormal jerky movements of the body

17years

14 years

12years

6years

4years

1year

FAMILY HISTORY : Parents non-consangious marriage 5 siblings 2 brothers n 3 sisters His elder sister who is 14 years of age has similar complaints No such history in other family members n parents n no H/O of H/O cholesystectomy or splenectomy in family

Developmental history

Normal Achieved milestones at appropriate age School going child with average position in his class Student of fifth class

Vaccination history:-

few vaccinations done whose record not available

:-

Feeding history

Takes home made all meals eagerly as well the junk food for last 1 month his appetite has reduced

Socioeconomic history

Belongs to lower class Father is a laborer depends on daily earnings Have 1 Marla own house fully covered with a small single room without adequate ventilation n sun exposure n 8 family members sharing it

EXAMINATION:EXAMINATION EXAMINATION
12 years old Extremely pale n sick looking child lying on bed well oriented in time place n person with fever 104 f Pulse n heart rate 90/min B.p 110/70 R/R 25/min Wt 33kg 50th centile Ht 135 cm. 25th centile dehydrated Jaundice +v No slapped cheek rash, clubbing petichae bruises edema or dysmorphic features , lymphadenopathy, hyper pigmentation, leg ulcers, thalassemic facieses, skeletal deformities.

GIT GIT: scaphoid abdomen with umbilicus central n everted no visible veins scar marks or hyper pigmentation Soft n slightly tender on palpation with no guarding or rigidity spleen palpable almost 3 cm BCM with smooth rounded contours Liver not palpable No evidence of fluid in peritoneal cavity Bladder sounds audible

EXAMINATION cont
REST of the systemic examination normal No Chest deformity symmetrical expansion with thoraco-abdominal breathing pattern resonant percussion note vocal fremitus n resonance normal On auscultation NVB with bilateral equal air entry n no added sounds Apex beat in left 4th intercostal space just lateral to mid clavicular line no thrill or rite parasternal heave Heart sounds s1 n s2 audible with flow

Normal tone power n reflexes with intact sensations n cranial nerves so as the cerebellar functions n gait including the higher mental functions Regarding urogenital kidneys n bladder not palpable n male genitalia with bilateral descendant testes

DIFFERENTIAL DIAGONOSIS
Chronic Hemolytic anemia (G6PD, AUIH, HS) Compensated liver disease ( Hepatitis, Wilson's disease) Chronic Malaria

MANAGEMENT: ,

INVESTIGATIONS
CBC
HB = 6.3 RBC = 3.0*10^6 WBC=5500 MCH=21.0 platelet=173 MCHC=36.0 HCT=19.9 MCV=66.3 POLY 40% LYMP 52%

RETIC COUNT 10%

PERIPHRAL SMEAR

SPHEROCYTOSIS + anisocytosis, poikilocytosis polychromasia platelets n + WBC normal in no n Microcytosis ++ morphology Hypochromia ++
Bilirubin total 3.7 SGPT 38 Indirect 1.8 SGOT 77 Direct 1.9 Gamma G.T 1 Alkaline phosphatase 281 Albumin 4.1

LIVER FUNCTION TESTS

MP SLIDE
VIRAL MARKERS

NORMAL

NEGATIVE

THYPHIDOT NEGATIVE IGM

DIRECT COOMBS TEST ABDOMINAL USG OSMOTIC FRAGILITY TEST NEGATIVE

MILD HEPATOSPLEENOMEGALY REST WAS NORMAL

Mean corpuscular fragility 0.50% saline normal 0.4 to 0.45 % saline

Child was also examined for KF-RINGS but no evidence found

FINAL DIAGONISIS
Triad of jaundice anemia n splenomegaly Positive family history Increased Retic count Spherocytes on smear Positive osmotic fragility test

HEREDITARY SPHEROCYTOSIS

NEXT STEP
FAMILY FAMILY SCREENING SCREENING

Surprisingly both mother n father are normal Only his one eldest sister is suffering from HS WHOSE RETIC 8% N HB 8gm/dl

14 years

12years

6years

4years

1year

Follow up plan

Our patient is suffering from some sort of non dominant HS PT was discharged home at HB 11.1 n fever free Next plan is to repeat Hb after 2 weeks n follow for decompansation then we will refer him for splenectomy