NNJ.pptx

Pediatric

1. Neonatal jaundice lecture Clinical medicine 24 th 05,2019 julius

2. introduction Over 60% of all newborn babies become jaundiced visibly ? Hemoglobin conconcentration falls rapidly in the 1 st few days from birth due to hemolysis (1g of Hb yields 640micromols of bilirubin b. Red cell life span of the new born is shorter about 70days compared to in adults. c. Hepatic bilirubin metabolism is less efficient in the first few days of life.

3. Why jaundice important It may be a sign of another disorder, eg infection Unconjugated bilirubin can be deposited in the brain , basal ganglia causing kernicterus

4. Physiology of bilirubin 1g of hemoglobin from hem produces 600mcmols(35mg) of unconjugated bilirubin Normal term baby breaks down about 0.5g of hb every 24hours In the plasma unconjugated bilirubin is bound to albumin and 5nonomol/l of free unconjugated bilirubin is normally present Unconjugated bilirubin is taken up in the liver by cytoplasmic protein called ligandin Conjugation takes place by addition of 2 molecules of glucuronic acid in the presence of the enzyme glucuronyltransferase

5. cont Then its transported actively out of the liver cells into bile and then travels into the gut Some converted to urobilinogen by bacteria in colon Within the gut if transit time increases conjugated bilirubin is deconjugated again by the glucuronidase produced by the bacteria in the gut lumen and present in breast milk

6. Note! All these functions of the liver are impaired in the preterm or ill term newborn compared to the normal healthy full term baby In particular defective conjugation cannot cope up with a large postnatal bilirubin load from breakdown of blood cells. In newborns conjugated bilirubin in duodenum meets with an enzyme called beta gluconidase and convertes it into unconjugated bilirubin It goes back to the cycle again for conjugation in the liver cells The cycle is called enterohepatic circulation

7. Definition of jaundice Yellow coloration of the skin and sclera produced by bilirubin tissue deposition Clinical evaluation: babies become clinically jaundiced when the bilirubin level reaches 80-120micromol/l(5mg/dl) Management varies according to the infants gestational age. Age at onset , bilirubin level and rate of rise and the overall clinical condition

8. Classification of jaundice Physiological pathological

9. physiological Usually appears between 48-72hours of age. Maximum intensity on days 4 and 5 in term babies and day 7 in preterm babies and disappear by day 14 It does not extend to palms and soles , does not need treatment Babies remain completely well Sets in on day 3 after birth Disappears within 2weeks Common in newborn especially in pre-term

10. pathological Jaundice starts on the first 24hours of life Jaundice lasts longer than 14days in term babies, 21 days in preterm infants jaundice accompanied with fever or other signs of illness Deep jaundice –palms and soles of baby are deep yellow. Look for cause and treat accordingly.

11. Causes of jaundice Physiological : Due to normal physiological breakdown of large red blood cells mass Pathological: Serious bacterial infection Hemolytic disease of the newborn-blood group (rhesus and ABO incompatibility Congenital syphillis or other intrauterine infections Liver diseases-hepatitis or biliary atresia Hypothyroidism Asphyxia Birth injuries

12. Prolonged jaundice Jaundice more than 1week Congenital biliary atresia Neonatal hepatitis Congenital hypothyroidism Congenital hemolytic anemia-pyruvate kinase ,(red blod cells membrane become weak liable for hemolysis), G6PD enzyme deficiency, spherocytosis

13. Other causes Cephalohematoma BMJ Metaboloc disorders- galactosemia , (Genetic metabolic disorder inability to metabolise body sugar), gylcogen storage disease Drugs: vitamin K, suphur drugs,m ASA (they compete with albumin binding sites(displace bilirubin in binding sites) Genetics.. Gilberts syndrome,(liver does not properly process bilirubin), robin johnson , crigler najjar syndrome

14. Predisposing factors Prematurity Infant of diabetic mother Respiratory distress syndrome

15. Clinical presentation Yellow coloration of skin and mucus membrane, sclera Physical examination: ssigns of sepsis, lethargy, fever, hypothermia, poor feeding, vomitting Hemolytic disease: palor , hepatosplenomegally Extravascular hemolysis; birth trauma associated with cephalohematoma or bruising Polycythemia: ruddy complexion of the skin Cholestatic jaundice: dark urine, light colored stool

16. investigations Bilirubin levels: both total and direct bilirubin Coombs test Septic screen Hepatitis b surface antigen Blood group and rhesus factor of the mother Serological tests Abdominal u/s Liver function tests T3 , T4( triiodothyronine ), thyroxin hormone respectively.

17. management Hydration Phototherapy Treat the cause feeding

18. phototherapy It reduces the light wave length of unconjugated bilirubin It does by: Geometrical isomerization of bilirubin to produce water soluble isomers which are slowly excreted

19. indications Hemolytic disease of the newborn Small sick babies including those with sepsis Bilirubin levels more than 340micromols/ in mature babies It should be continued till bilirubin is continuously falling consistently and is below the ‘safe’ line for gestation

20. note Note phototherapy conjugated bilirubin baby..cause photodegradation of porphyrins which are raised in the plasma of babies with conjugated hyperbilirubinemia …baby will change to deep brown color

21. Complications of phototherapy Diarhea .. Decreases gut transit time due to irritant effect on the bowel of the photoisomers of bilirubin Increases fluid losss through the skin and gut Exposes the child to the risk of hypothernia /hyperthermia, cause artificial burns Causes eryhromatous rash Corneal detachment if eyes not bandaged due photosensitization

22. Exchange blood transfusion Used when the bilirubin must be lowered urgently because it has reached toxic level, or baby starts to show features of kernicterus

23. Effects of EBT Replacing 85% of infants blood volume Decreasing tissue and serum concentration of bilirubin by 30% It corrects anemia It washes out infants antibodies which are causing hemolysis

24. The procedure Use fresh blood of less than 4days old Umbilical vein catheter 10cm long inserted The whole procedure should take 45-60min Aspirate serially and replace same amount of blood through the umbilical vein. Use small volume syringes for low birth weight guys Closely monitor the child during the whole procedure

25. cont To achieve a 90-95% swap of the baby’s blood, twice his blood volume ( i.e 2x85mls/kg =170mls)should be exchanged

26. Other uses of EBT Severe non hemolytic anemia from any other cause Sepsis To remove drugs or accumulated toxic metabolites in depressed neonates In coagulopathies to remove factors that perpetuate the coagulation disturbances and coagulation factors

27. complications Catheter induced complications- air emboli, aortic or portal vein thrombosis, hemorrhge Hypoglycemia Hypocalcemia ..caused by citrate in the blood stream Hyperkalemia..if blood stayed more than 3says Tissue hypoxia Acidemia Rentrolental fibroplasia- increase oxygen concentration in blood damages eyes of the neonate Ccf due to fluid overload

28. Bilirubin encephalopathy(kernicterus) This is pathological diagnosis There is yellow staining of the basal ganglia Mechanism is uncertain The cells that are prone to this are the metabolically active and receive the largest blood flow In neonates, basically are the cells of the basal ganglia and the midbrain

29. The basal ganglia include Globus pallidus Cutamae Caude nucleus The damage is caused by free bilirubin in the extracellular fluid binding to neural cell membranes with severe and complex biochemical sequele for cell.

30. Clinical presentations History-progressive jaundice Refusal to feed Lethargic Examination: Deep jaundice Hypotonic Floopy hyporeflexia

31. Late featureshypertonia irritable opisthotonus seizure high pitched cry AF bulged

32. complications If left untreated the condition is fatal, or may cause severe brain damage in survivors with the following manifestations Athetoid CP Deafness Upgaze palsy(conjugate, bilateral , limitation of the eye movements in upgaze and /or downgaze Intellectual retardation

33. Risk factors Short gestation Rapidly rising level of serum bilirubin Low levels of plasma albumin for the bilirubin to bind to The presence of hypoxia , acidemia , hypoglycemia, sepsis or some serious illness that might disrupt the BBB

34. prevention Immediate treatment when early signs are present But with late or long standing signs, permanent CNS damage is inevitable.

35. Neonatal sepsis Definition Etiology/significance Risk factors Route of infection Diagnosis Management prevention

36. definition Invasion by bacteria in the first 28days of life Occurs in 0.1% of live born infants Significance: A major cause of neonatal mortality 2/3 rd of neonatal mortality deaths occur In the first 2weeks, and are largely preventable.

37. Routes of infection Transplancental Ascending vaginal infection Exogenous-post partum infection, nosocomical infection, mechanical equipments used to handle the baby if contaminated

38. Risk factors Sex: male: female 2:1 Premature rupture of membranes Congenital malformation Low birth weight Interpartum hemorrhage Immature immune system

39. Maternal related risk factors Prolonged labor Difficult delivery Maternal fevers Infected birth canal Genital/vaginal warts

40. Environmental related Hands of the attendant Apparatus Feeds and medication Air-borne from birth attendant

41. classification Early onset..within 1week of delivery Late onset…………after one week Etiology”: Early onset: causes E.coli , GBS others are fungi, chlamydia, H. influenza and clostridium species Early onset has high mortality rate

42. Clinical features of early onset NNS Refusal to feed Lethargy Hypothermia/hyperthermia Jaundice Tarchypnea Recession, diarhea , vomiting,irritability , pseudoparalysis (voluntary restriction or inhibition of movements because of pain, incoordination. Poor weight gain Petechial/ septic spots

43. Late onset nns Occurs after one week of life It has low mortality rate Acquired from the mother Organisms include: staphylococcus aureus , epidermidis , E.coli , pseudomonas , candida, enterobacter

44. Clinical features r/s: cyanosis, grunting and dyspnoea GIT: intestinal obstruction in generalised sepsis or necrotising enterocolitis CNS: high pitched cry, retracted neck, bulging anterior fontanel, seizures Hematological: bleeding from puncture sites Sclerema : hardening of the skin…not specific feature of any disease

45. investigations Full hemogram , IT ratio, c reactive protein Blood cultures Csf analysis Chest radiology Surface swab for culture Urine culture, biochem , microscopy Abdominal x ray, ultrasound

46. management Specific supportive

47. specific Benzylpenicillin 50,000 iu /kg 12hourly Gentamycin 3-5mg/kg bwt depending on birth weight/ age Second line: cephalosporin 3 rd gen, gentamycin changed to amikacin If no clinical or laboratory evidence of infection after 3 rd day..kindly stop treatment If pseudomonas organism is isolated; give gentamycin plus ceftazidime If no clinical suspicious and culture is negative stop after 7days and if its positive treat for 7days

48. Supportive treatment Feeding Fluid management Oxygen Anticonvulsants If seizures Sunction Treat anaemia

49. prevention Hand washing before handling babies Incubation care Isolate sick babies Clean environment and equipment Clean babies Keep sick staff away Avoid overcrowding Treat mothers infection Ensure immunization

50. Congenital syphilis Caused by treponema pallidum , a bacteria Fetus acquires it in utero through transplancetal Hematogeneous from mother circulation Direct contact of the chancre during delivery

51. Clinical features Snuffles (syphilitic rhinitis) prolonged, severe, poor feeding (early symptoms) Rash maculopapular desquamation rash over palms and soles, around mouth and anus Mucocutaneous lesions Hepatosplenomegally Frontal bossing Severe consolidated pneumonia at birth

52. cont Most infants are asymptomatic at the time of diagnosis If untreated, most infants develop symptoms within the 1 st 5weeks of life. Severely ill infants may be born hydrops with profound anemia

53. Hematological changes Hemolytic anemia Thrombocytopenia

54. Complications/late features Osteochondritis Interstitial keratitis Frontal bossing Hutchisons teeth Saddle nose Eighth cranial nerve palsychatton joints The combination of interstitial keratitis, eighth cranial nerve deafness and hurtchson teeth is commoly refered as hurtchsons triad

55. investigations Csf exam: reveal pleocytosis , elevated proteins ( neurosyphilis ) Skin scrappings for micrscopy : treponema organisms Non treponemal syphillitic test Serological test VDRL

56. treatment Parenteral penicillin prefered DOC Penicillin G for 10-14/7 Non treponemal antibody titres should be monitored during treatment period and thereafter

57. Neonatal meningitis Inflammation of the pia and arachnoid mater which are membranes covering the brain and spinal cord Etiology: E.coli , and group b streptococci. Others are H. influenza and streptococci

58. Clinical presentation Sudden onset as in nns Late onset: convulsions, coma, bulged anterior fontanel, neck stiffness

59. Predisposing factors Prematurity Maternal genital infection Birth trauma Prolonged labor Umbilical sepsis meningomyelocele

60. investigations Septic screen Hemogram Csf exam UECs

61. treatment Specific: iv benzylpenicillin plus aminoglycosides Late: cephalosporins plus amikacin

62. complications Cerebral abscess Hydrocephalus Epilepsy Mental retardation

63. Hemolytic disease of the newborn Used to be common Causes hydrops fetalis , anemia and severe neonatal jaundice The red blood cells of the newborn are attacked by antibodies from the mom. The attack begins while the baby is still in the womb and caused by incompatibility between the mothers and babys blood.

64. What happens Maternal antibodies cross the placenta and attack fetal red blood cells During pregnancy , some of the mothers antibodies are transported across the placenta to fetal circulation. This is important for the infant to acquire immune, but also cause HDU as they target fetal red blood cells.

65. cont A major cause of HDU is incompatibility of the RH blood group between the mom and fetus Commonly triggered by: D antigen others C,c E, e can also cause hemolysis Pregnancies at risk are the D negative carrying a fetus who is D positive acquired from mom. The mom immune response to the fetal D antigen is to form antibodies against it (anti-D)

66. sensitization During first pregnancy it takes place if the mom blood come incontact with D positive from baby An immune response is mounted, causing sensitization Once a mom is sensitized to the D antigen the serum will contain anti-D A small amount of blood is needed for sensitization to occur This typically occurs during the first pregnancy

67. cont Sensitization occurs during labor, and increased during prolonged, or complicated labor..increased risk of sensitization It can also occur earlier in pregnancy e,g . during prenatal bleed or miscarriage, medical procedure

68. During subsequent pregnancies HDU occurs during subsequent pregnancies Initially, the maternal anti-D that is formed at the time of sensitization is of igM , which does not cross the placenta In subsequent pregnancies, a repeat encounter with the RhD antigen stimulates the rapid production of type igG anti-D which can be transported across the placenta and enter fetal circulation

69. cont Once in the fetal circulation anti-D attaches to the Rh D antigens found on the fetal red blood cells, marking them to be removed. The rate of hemolysis determines whether the nature of HDU is mild, moderate or severe

70. mild Small increase in the rate of hemolysis is tolerated by the fetus Mild anemia, jaundice which resolve without treatment In moderate: severe jaundice with rapid increase of bilirubin within 24hours of delivery. This may cause kernicterus

71. In severe destruction Severe anemia Liver, spleen, and other organs increase their production of RBCs to compensate for the loss This causes the liver and spleen to enlarge ( hepatospleenomegally ) and liver dysfunction Immature red blood cells spill into the circulation thus called hydrops fetalis

72. A complication of severe HDU Hydrops fetalis is the complication Fetal tissue become swollen (edematous). Its fatal in utero or soon after birth.

73. ABO incompatibilty It arises when mom has igG antibodies from previous exposure to A or B antigens The antibodies crosses the placenta by active transport and affect the fetus or newborn Sensitization of the mom to fetal antigens may have occurred by previous transfusions or by conditions of pregnancy that result in transfer of fetal erythrocytes into maternal circulation such as:

74. cont 1 st trimester abortion Ectopic pregnancy Amniocentesis Manual extraction of placenta Normal pregnancy

75. Clinical presentation Mild anemia compared to RH isoimmunization .. Few cells have anti-A or anti-B antibody binding sites compared to RH antigenic sites

76. prevention Mom carrying rhesus positive child is given anti-D at gestation period above28weeks and within seventy hours after birth. This antibody binds to fetal RH-positive erythrocytes entering the maternal circulation during the fetal-to-maternal transfusion at birth.

77. Perinatal mortality