Dr. R. Pradheep. DNB Resident Pediatrics. Southern. Railway. Hospital.

Transcription

1 Hyperbilirubinemia in an Infant Pradheep Railway Dr. R. DNB Resident Pediatrics. Southern Hospital.

2 A 2 ½ month old male baby born out of 3 rd degree consanguinity presented to us with c/o yellow discolouration of eyes and passing clay coloured stools since 2 weeks Full term NVD BW 2.15 kg did not cry immediately at birth Kept in NICU for 6 days. No H/O any antenatal infections. Breast feeding started on the next day. In the 2 nd month mother noticed yellow discolouration of the eyes but child was not investigated.

3 In the 8 th week of life, child was admitted for fast breathing and fever in Chattisgarh. The investigations revealed,. Sr Bilirubin of 7.5,Direct bilirubin of 4.5 USG abdomen showed Hepatomegaly, The child was referred to our hospital for further evaluation.

4 On Examiation Child alert well, thriving HR: 120/ min RR:40/ min Eyes :Icterus present Weight :3.8 kg No signs of any deficiencies Umblical Hernia present P/A - Hepatomegaly of 4 cm from the RCM, liver span 8 cm, firm,non tender with rounded edges. Posterior fontanalle: wide open 1.5 cm x1.5cm Clay coloured stools observed.

5 Lab Results LFT (12/5/12) Total bilirubin: 7.5mg/dl Direct bilirubin: 4.5mg/dl SGOT :86U/L SGPT :160U/L GGT: 139U/L PT :50 sec Alkaline phosphatase:1137 IU/L Calcium 8.9mg/dl Phosphorus 3.5mg/dl Other results like urine routine and culture, TORCH screen,urine for reducing substances, Hepatitis panel were normal.

6 T3:135.2 miu/ml (81-281) T4: 0.6 miu/ml ( ) TSH:26.98 miu/ml USG of Thyroid : Normal study Tc99 scan: Normal study USG Abdomen Liver: with uniform ECHO texture. Intra hepatic biliary radicles were normal. CBD normal. Portal and hepatic veins normal. Gall bladder :normal

7 The child was started on Thyroxine at 15 micrograms/ kg and vitamin supplements. Ursodeoxy cholic acid was given(15 mg/kg). The child drastically showed improvement by the fourth day of administration of Thyroxine. Lab values after 4 days were, Total bilirubin:6.4mg/dl Direct bilirubin: 4.0mg/dl SGOT :64U/L SGPT : 120U/L Alkaline phosphatase: 1023U/L GGT:109 U/L

8 Present values(12/9/12) Total biliubin :1.0mg/dl Direct bili:0.5mg/dl SGOT :31U/L SGPT : 46U/L Alkaline phosphatase: 326U/L TFT: TSH: 4.4mIU/ml T4: 1.2ng/dl Developmental mile stones are normal Posterior fontanel closed

9 Discussion Any infant who is jaundiced beyond two weeks of life should be evaluated for neonatal cholestasis. Neonatal cholestasis is defined as accumulation of bile substances in blood due to impaired excretion. These infants should always have fractionated serum bilirubin levels checked to differentiate the conjugated hyperbilirubinemia of cholestasis from unconjugated hyperbilirubinemia that is usually benign and spontaneously resolves. Conjugated hyperbilirubinemia, pale stools and dark urine are the cardinal features of neonatal cholestasis. The differential diagnosis of cholestasis is extensive and a systematic approach is helpful to quickly establish the diagnosis.

10 Suspected neonatal cholestasis Examin e Stool colour Sick infant Cultures,TORCH,Urin e reducing substances Well looking infant Pale stool Pigme nted stool

11 Pale stool Pigmented stool USG HIDA Liver Biopsy USG Liver Biopsy

12 AIIMS SCORING INDEX Bed side method to differentiate BA from NH: 1)Age <6 weeks : 2 >6 weeks: 1 2) Jaundice Fluctuating, mild to moderate:2 Severe(> 8mg%) :1 3)Stool Normal or light yellow: 2 Clay coloured : 1 4)Urine Normal or light yellow: 2 Dark yellow : 1 5) Liver Soft and smooth: 4 Firm :1 Result: >10: NH <10:BA

13 AIIMS Index 10 or more Less than 10 Phenob 5 mg /kg for 7 days and Immediate technitium scan surgery Technitium scan

14 Technitium scan Excretion into gut in 24 hours Neonatal cholangiogram Hepatitis No excretion into gut Operative and Kasai s procedure

15 Congenital hypothyroidism is one of the most urgent diseases of the neonate. When diagnosed and treated at an early stage, its most important complication, mental retardation, is preventable. The signs of congenital hypothyroidism are nonspecific in neonates. Only 5% of the cases have characteristic clinical findings. One of the most important and earliest signs is prolonged jaundice during the neonatal period. Management of cholestasis is mostly supportive, consisting of medical management of complications of chronic cholestasis like pruritus and nutritional support for malabsorption and vitamin deficiency.

16 The mechanism behind The mechanism of cholestasis in hypothyroidism has not been fully explained but some reports have shown that thyroxine and triiodothyronine have a relaxing effect on sphincter of Oddi. Spincter off Oddi regulates bile flow. The absence of the relaxing effect of thyroxine results in delayed emptying of the biliary tract. In addition, thyroid hormones also affect cholesterol metabolism. Biliary secretion of cholesterol is reduced in hypothyroidism There have been some reports of common bile duct stones associated with hypothyroidism

17 Take home messages An elevated conjugated bilirubin is always an abnormal finding and requires additional evaluation. Never ignore the mothers complaint that the childs urine is high coloured and stools are pale coloured. Never diagnose physiological jaundice in a 2 week old child. Ensure proper follow up in cases of neonatal jaundice. Never forget to rule out hypothyroidism as a cause for Neonatal cholestasis. It important to make an early diagnosis.