Diseases Of The Newborn Part2

PEDIATRICS 2 BLOOD AND HEMOSTATIC MECHANISM Dr. Alabastro 2nd shifting / Sept. 15, 2008 Trans group: Chubimbonbon Blood and Hemostatic Mechanism: •

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Leucocytosis and elative neutrophilia in 1st 24 hours Relative lymphocytosis after 1st week RBC with relatively low levels of reduced Glutathione Deficient capacity of liver to conjugate Bilirubin Normal hemostatic mechanism depends upon establishment of normal intestinal flora and elaboration of Vitamin K

Anemia Definition: • Full term : Hct < 45%; Hgb < 14.0 g/dL • Pre-term: Hct < 40%; Hgb < 13.5 g/dL • Acute hemorrhage at time of bit may not be reflected in initial Hct • Capillary Hct is higher than central hematocrit due to sludging • If pH is low, central H”ct may be inaccurate (high) because of vasoconstriction Causes: • Blood Loss: pre-natal, intrapartum, post-natal • Hemolysis: isoimmune hemolysis, congenital defects of the erythrocytes, abnormalities of erythrocyte membrane, disorders of hemoglobin synthesis, acquired defects of erythrocytes • Underproduction of erythrocytes: Damond-Blackfan Syndrome, parvovirus, vitamin deficiencies History Data: • Hemorrhage: feto-fetal transfusion, internal/external hemorrhage, OB bleeding, post-natal bleeding • Obstruction to placental blood flow: tight nuchal cord

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Low Hgb

Hgb = 17–19 mg/dL

Accelerated RBC hemolysis: isoimmunohemolysis, infection, hemoglobinopathies, erythrocyte enzyme defects, red cell membrane disorders, DIC Manifestations: Pallor Tachycardia with or without hemic murmur Respiratory distress initially tachypnea Weak pulse Shock Combination of sign points to decompensation: EMERGENCY

Retic count

LOW Congenital Hypoplastin Anemia

NORMAL OR HIGH Coombs Test

(+) Immunoe Hemolytic Anemia ABO, Rh Minor Blood Group

(-) MCV

LOW Chronic IU Blood Loss a-thalassemia

NORMAL OR HIGH Peripheral Blood Smear

NORMAL Infection Blood Loss Misc

ABNORMAL ….

Laboratory: • Samples of work-up obtained before treating CBC with peripheral smear, reticulocyte count, Coomb’s test, blood type and cross-matching, coagulation studies if indicated • Serial Hbg/Hct necessary to detect anemia early before signs of decompensation occur Management: • Immediately transfusion in baby with pallor and/or respiratory distress in 1-3 hours • Immediate plasma expanders or resuscitation in baby with hypotension or shcok • Partial exchange with packed RBC in anemic hydrops baby • Whole blood or packed RBC diluted with plasma may be pushed IV in continuing hemorrhage Features of Coagulation System • Prothrombin time of full-term and pre-term infants is only slightly outside the adult range of 10-12 sec (14+/1.3) • PTT longer in newborn than adults (51/57+/-10/10.5) • Factors XI and XII low in preterm infants • PTT is of little value in the sick newborn prolonged even without evidence of hemorrhage BLEEDING DISORDERS

MARY YVETTE ALLAIN TINA RALPH SHERYL BART HEINRICH PIPOY KC JAM CECILLE DENESSE VINCE HOOPS CES XTIAN LAINEY RIZ KIX EZRA GOLDIE BUFF MONA AM MAAN ADI KC PENG KARLA ALPHE AARON KYTH ANNE EISA KRING CANDY ISAY MARCO JOSHUA FARS RAIN JASSIE MIKA SHAR ERIKA MACKY VIKI JOAN PREI KATE BAM AMS HANNAH MEMAY PAU RACHE ESTHER JOEL GLENN TONI

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Local cord, scalp, GIT, abdomen, lung, intraventricular hemorrhage Generalized acquired hemorrhage: Hemorrhagic disease of the newborn, DIC, liver

Normal or Low Hct

BILIRUBIN PATHWAY • Unconjugated bilirubin – nonpolar, insoluble, indirect bilirubin (B1) causes kernicterus CAUSES OF INDIRECT HYPERBILIRUBINEMIA • Overproduction of bilirubin: hemolysis, extravated blood, polycythemia, increased enterohepatic circulation • Undersecretion of bilirubin: decreased hepatic uptake of bilirubin, decreased conjugation, decreased transport of conjugated bile out of hepatocytes • Combined: perinatal infection, multisystem disorder NON-PHYSIOLOGIC JAUNDICE Definition: • Cord bilirubin > 3 mg/dL

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Clinical jaundice in the 1st 24 hours Rate of rise of bilirubin > 5 mg/d/day Total serum bilirubin >12.9/mg/dL in term, >15 mg/dL in preterm Direct bilirubin >1.5 mg/dL Jaundice persisting > 1 week in term, > 2 weeks in preterm

Non Sp Morpho Abnormalities

Spherocytosis Elliptocytosis Stomatocytosis pyknocytosis

ABO incompatibility G6PD deficiency Pyruvate kinase def Other enzyme def a-thalassemia

Inc indirect

Coomb’s (+) isoimmunizatio n Coomb’s (-)

Hct Normal or Low High transfusion Delayed clumping SGA

Extravascular blood Inc. Enterohepatic Circulation -Breastfeeding -Pyloric Stenosis Metab.-Endocrine -Galactosemia -Hypothyroidism Other -IDM

FACTORS ASSOCIATED WITH AN INCREASE IN BILIRUBIN LEVELS: • Race: Oriental, American, Indian, Greek

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Blood types, Rb, Coombs, Hct, Red cell morphology, reticulocyte count

Normal

Sp Morpho Abnormalities

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Measure total and direct bilirubin

Intrauterine infection Sepsis Biliary atresia Bile plugs Galactosemia Choledochal cyst Tyrosinosis Cystic fibrosis A-1 antitrypsin def

Abnormal

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Jaundice

Inc Direct

Red cell morphology & reticulocyte count

Maternal: diabetes, HPN, OCP, 1st trimester bleeding, decreased plasma zinc Drugs: Diazepam, Oxytocin, epidural anesthesia Labor and delivery: PROM, breech delivery Infant: LBW, prematurity, breastfeeding, caloric deprivation, infection, increase in weight loss, delayed meconium passage and cord clamping, decrease fluid intake, low Zinc and Mg, male sex

Management • Surgical determination of serum bilirubin, Hgb, Hct reticulocyte count if hemolytic • Do specific testing if non-hemolytic • For indirect phototherapy, decrease enterohepatic circulation, stop breastfeeding for 1-3 days, supportive, exchange transfusion, Phenobarbital • For direct: depends upon case • Manage underlying cause or refer to surgery SEPSIS AND MENINGITIS Etiology: bacterial, viral, fungal, parasitic Predisposing factors: • Prolonged ROM • PROM • Foul smelling amniotic fluid and baby • Maternal fever • Prematuriy • Unexplained fetal distress • Previous septic infant • Unsterile delivery • Unsterile practices • Contaminated equipments • Infected personnel/caretakers Manifestations • Lethargy • Poor feeding

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Laboratory Sepsis work-up • CBC (WBC and differential) • Urinalysis • CSF analysis (WBC and differential, protein) • Chest and abdominal X-ray • Cultures (blood, CSF, urine)

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- pH 7.5 or less at 5 min - pH 7.2 or less at 10 min - base deficit of 10 at 10 min

Apnea Tachypnea Cyanosis Diarrhea Metabolic acidosis Temperature instability Abdominal distention Petechiae/purpura Hepatospenomegaly Seizures Tense fontanelle

RESUSCITATION 1. Drying, warming, positioning, suction 2. Tactile stimulation 3. Oxygen 4. Bag and mask ventilation 5. Chest compression 6. Medications ITEMS READILY ACCESSIBLE FOR RESUSCITATION 1. Ambu bag 2. Respiratory mask 3. Endotracheal tube 4. Laryngoscope 5. Suction apparatus glucose,

Other work up as appropriate: ABG, coagulation studies, stool culture and sensitivity ang gram stain, CRP Cranial UTZ / CT Scan

Management Do sepsis word-up on all babies considered as septic Antibiotic coverage, penicillin derivative and amino glycoside Supportive management as needed: transfusion, thermoregulation, glucose, seizures, vital signs Anticipate complications • DIC • NEC • Feeding problems • Complications of meningitis (SIADH, ventricular obstruction, seizures, subdural effusion, relapse)

BASIC REQUIREMENTS IN CARE OF NEWBORN 1. Immediate resuscitation 2. Establishment of respiration 3. Adequate nutrition 4. Normal body temperature 5. Avoidance of contact with infection ASPHYXIA Imbalance in oxygenation Causes: Fetal 1. Placental separation 2. Inadequate perfusion of maternal side of placenta 3. Interruption of umbilical blood flow Neonal 1. Airway obstruction 2. Excessive fluid in lungs 3. Weak respiratory effort 4. Sequel to fetal hypoxia CRITERIA FOR ASPHYXIA 1. Historical factors (relative) -Fetal distress fetal scalp acidosis 2. Physical exam (relative) 1 min APGAR score of 2 or less 5 min APGAR score of 5 or less 3. Blood gas pH (absolute)

OXYGEN - Blow by oxygen for cyanosis with spontaneous respiration and a heart rate >100 bpm - initial oxygen concentration close at 100% - Nasal cannula or facial mask - The closer the distance to the nostril, the higher concentration of O2 delivered BIG VALVE MASK - use of apnea and HR < 100 bpm - appropriate for baby’s size and gestation not to exceed 750ml - arte at 40-60 mins - CI: thick meconium stain and diaphragmatic hernia ENDOTRACHEAL INTUBATION Indications: 1. Need for prolonged positive pressure ventilation 2. Ineffective bag and mask ventilation 3. Suspected diaphragmatic hernia UMBILICAL CANNULATION Medications used in resuscitation 1. Epinephrine 0.1-0.3 ml/kg of 1:10,000 rapid IV bolus 2. NaHCO3 2 mEq/kg slow IV 3. Glucose 10% 2ml/kg IV then infusion of 8-10mg/kg/min 4. Naloxone HCl 0.01mg/kg rapid IV bolus 5. Ca gluconate 100mg/kg slow IV 6. Atropine 0.01 mg/kg slow IV or IM NECROTIZING ENTEROCOLITIS (NEC) • Most common life threatening emergency of the GIT in the newborn period • Necrosis – final common pathway of response to in the newborn gut

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Onset usually occurs in the 1st 2 weeks or as late as 3 month of age in VLBW infants Most frequent site: distal ileum and proximal colon Triad: intestinal ischemia, oral feedings,(metabolic substrate) and pathological organisms Greatest risk factor: prematurity Complications: perforation, adhesion, diarrhea and malabsorption

Pathogenic/Predisposing Factors • Bowel immaturity and bowel injury • Perinatal asphyxia • Infection: E. coli, Klebsiella, Pseudomonas, Salmonella, Clostridium, Staphylococcus epidermidis, and rotavirus • Hypertonic substances in gut • Indwelling umbilical catheter • Exchange transfusion

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Placement of NGT Polycythemia Oral feeding

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Clinical Manifestation

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Significant residual

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Gastric aspirate or vomiting EC

 3 early signs of

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Abdominal distention  Signs of sepsis: temp instability, lethargy Massive GI hemorrhage Shock and DIC

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Laboratory Examination • CBC, Hgb, Hct, WBC differential, platelet evidence of sepsis o Immature to total neutrophil ratio > 0.2-0.5 • ABG: hypoxiametabolic acidosis • Abdominal x-ray: o Mucosal damage o Pneumatosis intestinalis (intramural air) – bubbly appearance (pathognomonic of NEC) o Portal vein gas – sign of severe disease(pathognomonic of NEC) o Pneumoperitoneum – indicates perforation • Blood culture Management • OGT and intermittent suction • Septic workup • Start antibiotics – 2ndary infection • Monitor w/ lab exams • Blood component replacement • Adequate metabolic balance • Monitor abnormal girth • NPO for 7-10 days, reintroduce with formula/breast milk • IV fluids • Refer to surgery: consider barium enema if with signs of obstruction • Respiratory status support: ventilation Thermoregulation • Mechanism of heat production primarily by metabolism of brown fat • Mechanism of heat loss: convection, conduction, evaporation • NTE: axillary temp at 36.4-37.2 deg • Keep warm immediately in delivery room and nursery Avoidance of Contact with Infection • Hand washing • Attire • Asepsis for procedures • Recognize infection early • Blood precaution JAUNDICE AND HYPERBILIRUBINEMIA Biliverdin  Bilirubin (enzyme: biliverdin reductase) Etiology of Indirect Hyperbilirubinemia Indirect –B1; Direct – B2 1. Overproduction of bilirubin (increased load of bilirubin to be metabolized by the liver) • Hemolytic anemia, polycthemia, shortened red celllife as a result of immaturity or

3. 4.

transfused cells (extravasated blood), increased enterohepatic circulation, infection Damaged or reduced activity of transferase enzymes • Genetic deficiency, hypoxia, infection, hypothermia and thyroid deficiency Transferase enzyme block • Drugs and other substances requiring glucoronic acid conjugation for excretion Absence or reduced amounts of the enzymes or reduced bilirubin uptake by the liver cells • Genetic defect, prematurity

Factors increasing toxic bilirubin

effects of elevated of unconjugated

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Reduced retention of bilirubin in the circulation o Hypoproteinemia o Displacement of bilirubin from its binding sites on albumin by competitive binding of drugs such as sulfisoxazole and moxalactam o Chuen-Lin herbal tea o Acidosis o Increased free fatty acid concentration secondary to hypoglycemia o Starvation o Hypothermia • Increased permeability of blood brain barrier or nerve cell membranes to bilirubin • Increased susceptibility of brain cells to its toxicity o Asphyxia, prematurity, hyperosmolarity, infection Non-Pharmacologic Jaundice Definition • Cord bilirubin > 3mg/dl

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Vlinical jaundice in 1st 24-26 hours Rate of rise of serum bilirubin >5mg/dl/24 hr Jaundice persisting after 10-14 days

Management Indirect – phototherapy, dec. enterohepatic circulation, stop feeding for 1-3 days, supportive, exchange transfusion, Phenobarbital Direct – depends on cause

From Nelson: (Magbasa pa rin po kayo ng Nelson. Baka hindi ito enough. Hindi kasi nagbibigay si Dra. ng powerpoint.) Hypocalcemia • Hypocalcemia is common in sick and premature newborns. • Most infants are born with calcium levels that are higher in cord blood than in maternal blood because of active placental transfer of calcium to the fetus. • Fetal calcium accretion in the third trimester approaches 150 mg/kg/24 hr, and fetal bone mineral content doubles between 30 and 40 weeks of gestation. • All infants show a slight decline of serum calcium levels after birth; the decline reaches trough levels at 24 to 48 hours, the point at which hypocalcemia usually occurs. • Total serum calcium levels of less than 7 mg/dL and ionized calcium levels of less than 3 to 3.5 mg/dL are considered hypocalcemic.

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Early neonatal hypocalcemia occurs in the first 3 days of life and is often asymptomatic. Transient hypoparathyroidism and a reduced parathyroid response to the usual postnatal decline of serum calcium levels may be responsible for hypocalcemia in premature infants and infants of diabetic mothers. Congenital absences of the parathyroid gland and DiGeorge syndrome also have been associated with hypocalcemia. Treatment with calcium alone does not relieve symptoms or increase serum calcium levels; for this to occur, the hypomagnesemia also must be treated. Sodium bicarbonate therapy, phosphate release from cell necrosis, transient hypoparathyroidism, and hypercalcitoninemia may be responsible for early neonatal hypocalcemia associated with asphyxia. Early-onset hypocalcemia associated with asphyxia often occurs with seizures as a result of hypoxicischemic encephalopathy or hypocalcemia. Late neonatal hypocalcemia, or neonatal tetany, often is the result of ingestion of high phosphatecontaining milk or of the inability to excrete the usual phosphorus in commercial infant formula. Neonatal hypocalcemia may be prevented by administration of IV or oral calcium supplementation at a rate of 25 to 75 mg/kg/24 hr. Early asymptomatic hypo-calcemia of preterm infants and infants of diabetic mothers often resolves spontaneously. Symptomatic hypocalcemia should be treated with 2 to 4 mL/kg of 10% calcium gluconate given intravenously and slowly over 10 to 15 minutes, followed by a continuous infusion of 75 mg/kg/24 hr of elemental calcium. If hypo-magnesemia is associated with hypocalcemia, 50% magnesium sulfate, 0.1 mL/kg, should be given by IM injection and repeated every 8 to 12 hours.

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Hyperphosphatemia • (>8 mg/dL) usually occurs in infants with hypocalcemia after the first week of life. Vitamin D deficiency states and malabsorption also have been associated with late-onset hypocalcemia. The clinical manifestations of hypocalcemia and hypomagnesemia include apnea, muscle twitching, seizures, laryngospasm, Chvostek sign (facial muscle spasm when the side of the face over the seventh nerve is tapped), and Trousseau sign (carpopedal spasm induced by partial inflation of a blood pressure cuff). The latter two signs are rare in the immediate newborn period. Occasionally, heart failure has been associated with hypocalcemia. Neonatal Drug Addiction and Withdrawal • Infants may become passively and physiologically addicted to medications or to drugs of abuse (heroin, methadone, barbiturates, tranquilizers, amphetamines) taken chronically by the mother during pregnancy; these infants subsequently may have signs and symptoms of drug withdrawal. • Many of these pregnancies are at high risk for other complications related to IV drug abuse, such as hepatitis, AIDS, and syphilis. • In addition, the LBW rate and the long-term risk for sudden infant death syndrome are higher in the infants of these high-risk women. Opiates • Neonatal withdrawal signs and symptoms usually begin at 1 to 5 days of life with maternal heroin use and at 1 to 4 weeks with maternal methadone addiction.

• The treatment of late hypocalcemia includes immediate management, as in early hypocalcemia, plus the initiation of feedings with formula containing low phosphate levels. Subcutaneous infiltration of IV calcium salts can cause tissue necrosis; oral supplements are hypertonic and may irritate the intestinal mucosa.

Hypomagnesemia • (<1.5 mg/dL) may be seen simultaneously with hypocalcemia, especially in infants of diabetic mothers. • During exchange transfusion with citrated blood, magnesium decreases 0.5 mg/dl; approximately 10 days is required for it to return to normal. • Treatment consists of IM injection of Magnesium sulfate. Hypermagnesemia • May occur in newborn infants of mother treated with MgSO4 during labor. • At high serum level, CNS is depressed and infants have depressed and infants have profound respiratory depression requiring mechanical ventilation.

May be associated with failure to pass meconium (Meconium plug syndrome). Occurs when serum magnesium value is >2.8 mg/dL but symptoms rarely occur at levels below 5 mg/dl. IV calcium and diuresis can reduce magnesium levels.

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Clinical manifestations of withdrawal include sneezing, yawning, ravenous appetite, emesis, diarrhea, fever, diaphoresis, tachypnea, high-pitched cry, tremors, jitteriness, poor sleep, poor feeding, and seizures. The illness tends to be more severe during methadone withdrawal. The initial treatment includes swaddling in blankets in a quiet, dark room. When hyperactivity is constant, and irritability interferes with sleeping and feeding, or when diarrhea or seizures are present, pharmacologic treatment is indicated. Seizures usually are treated with phenobarbital. The other symptoms may be managed with replacement doses of a narcotic (usually tincture of opium) to calm the infant; weaning from narcotics may be prolonged over 1 to 2 months.

Cocaine • Cocaine use during pregnancy is associated with preterm labor, abruptio placentae, neonatal irritability, and decreased attentiveness. Infants may be SGA and have small head circumferences. Usually no treatment is needed.

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Hi 3B. Aral lang ng mabuti. Konti na lang and Clerkship here we come.