Pediatrics Main Handout - March 2023 - Topnotch Medical Board Prep

TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This wil...

TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. DEFINITION: Neonatal Period – birth up to 1st month old IMPORTANT LEGAL INFORMATION ESSENTIAL NEWBORN CARE The handouts, videos and other review materials, provided by Topnotch Medical Board Preparation Incorporated are duly protected by RA 8293 otherwise known as the Series of time bound chronologically ordered, standard Intellectual Property Code of the Philippines, and shall only be for the sole use of the person: procedures that a baby receives at birth a) whose name appear on the handout or review material, b) person subscribed to Topnotch Medical Board Preparation Incorporated Program or c) is the recipient of this electronic Immediate drying → prevents hypothermia communication. No part of the handout, video or other review material may be reproduced, Uninterrupted skin-to-skin contact → prevents hypothermia, shared, sold and distributed through any printed form, audio or video recording, electronic increases colonization with protective family bacteria and medium or machine-readable form, in whole or in part without the written consent of Topnotch Medical Board Preparation Incorporated. Any violation and or infringement, improves breastfeeding initiation and exclusivity. whether intended or otherwise shall be subject to legal action and prosecution to the full Delayed cord clamping after 1 to 3 minutes → decreases anemia extent guaranteed by law. in 1 out of 3 premature babies and prevents brain hemorrhage in 1 out of 2; prevents anemia in 1 out of 7 term babies. DISCLOSURE Non-separation of mother and baby. Breastfeeding within The handouts/review materials must be treated with utmost confidentiality. It shall be the responsibility of the person, whose name appears therein, that the handouts/review first hour of life prevents 19.1% of all neonatal deaths. materials are not photocopied or in any way reproduced, shared or lent to any person or Please take note of the chronological order and importance of each step. disposed in any manner. Any handout/review material found in the possession of another A common mistake of most students is to answer cord clamping as the 2nd person whose name does not appear therein shall be prima facie evidence of violation of RA 8293. Topnotch review materials are updated every six (6) months based on the current step. Remember this is the 3rd step. Think “delayed” Dr. De Vera trends and feedback. Please buy all recommended review books and other materials listed below. ✔ GUIDE QUESTIONS THIS HANDOUT IS NOT FOR SALE! After catching a newborn full-term male, and placing him in the incubator, you noticed that the air-conditioning system was set to full INSTRUCTIONS and that the radiant warmer was not working. You are worried that the To scan QR codes on iPhone and iPad baby might develop hypothermia which might lead to following except? 1. Launch the Camera app on your IOS device A. Acidosis 2. Point it at the QR code you want to scan B. Hypoglycemia 3. Look for the notification banner at the top C. Hypoxemia of the screen and tap To scan QR codes on Android D. Increased renal excretion of water and solutes 1. Install QR code reader from Play Store E. NOTA 2. Launch QR code app on your device The target temperature for newborns is? 3. Point it at the QR code you want to scan A. 36 - 37°C C. 36.5 - 37.5°C 4. Tap browse website B. 36 - 37.5°C D. 36.7 - 37°C The following are the most common mechanisms of heat loss in the Approach to Topnotch Pediatrics immediate newborn period EXCEPT? Please have the following Topnotch materials at hand: A. Convection o (1)Topnotch Main Handout will serve as your main reference B. Conduction material C. Heat Radiation Please buy the following: D. Evaporation o Nelson Textbook of Pediatrics, 20th / 21st ed E. NOTA o Pedia Platinum THERMOREGULATION This handout is only valid for the March 2023 PLE batch. Newborns are prone to heat loss and hypothermia This will be rendered obsolete for the next batch body surface area of a newborn infant is approximately 3 times since we update our handouts regularly. that of an adult Mechanism Convection Heat energy to cooler surrounding air PEDIATRICS Conduction Heat to colder materials touching the infant By Adrian Salvador M. De Vera, MD, DPPS Radiation Transfer of heat to nearby cooler objects Evaporation Losses from skin and lungs (respiration) Ruby Ann L. Punongbayan, MD, FPPS, MA, FPPSAP Optimal method for maintaining temperature in a stable Contributor: Mary Joeline D. Arada, OTRP, MD neonate? Skin-skin contact TOPIC PAGE Here are some examples of heat loss for you to better remember them. Neonatology 1 Convection: This is the reason why room temperature should be set at 25-28°C. Conduction: Important to pre-heat radiant warmer. Pediatric Nutrition 13 Radiation: Cold metal cabinets inside the operating room are sources of Preventive Pediatrics 18 heat radiation. IMCI 22 Dr. De Vera Growth and Development 23 QUICK SHEET TEMPERATURE CHECKLIST Gastroenterology 26 Recommended room temperature = 25-28°C Nephrology 33 Temperature target for newborns = 36.5-37.5°C Hematology/Oncology 39 Neurology 46 ✔ GUIDE QUESTION Pulmonology 57 All of the ff. is an effect of early skin to skin contact in newborn care except? Rheumatology 70 A. Bonding Cardiology 76 B. Prevents hypothermia C. Prevents anemia Infectious Diseases 86 D. Increases colonization with protective bacterial flora Endocrinology 96 Immunology/ Allergology 102 Classification of Prematurity Based on Birth Weight and Gestational Age: CLASSIFICATION METRIC NEONATOLOGY Birth weight ✔ GUIDE QUESTIONS Low birth weight 3 D. >5 C. Other congenital anomalies are A one-month-old male was brought to your clinic due to scrotal commonly associated with this defect swelling. Mother noticed this to be present since birth. There were no D. Bowel and alimentation is normal other associated symptoms. No fever, no tenderness. Baby was well, with good suck and activity. On physical examination you see this OMPHALOCELE VS GASTROSCHISIS OMPHALOCELE GASTROSCHISIS SAC + - Below the Lateral to the LOCATION umbilicus umbilicus, Right UMBILICAL Center of Left of the defect CORD membrane ASSOCIATED 60% 10% DEFECTS BOWEL Normal Inflamed What is your diagnosis? ALIMENTATION Normal Delayed Surgical, TPN, Surgical, TPN, Answer: Hydrocele MANAGEMENT Hydration Hydration GENITOURINARY ANOMALIES ✔ GUIDE QUESTIONS Hydrocele - accumulation of fluid in the tunica vaginalis (1-2% A preterm baby won’t stop crying. He then developed abdominal distention with abdominal erythema. The baby cries more when of neonates); majority are noncommunicating; resolves by 12 touched. What is your diagnosis? months old NEC Hernias – usually indirect inguinal hernias; presents as a Coagulation necrosis – histologic finding reducible scrotal swelling Thickened bowel walls and air in the bowel wall: Important points to remember: o PNEUMATOSIS INTESTINALIS Hydrocoele – may observe up to 1 year of age What will be your intervention? Undescended testes – may observe up to 3-4 months of age Supportive Inguinal hernia – needs to be repaired surgically Dr. De Vera Two days later, the baby developed pneumoperitoneum. What will be your intervention? Surgery All of the ff. are accepted interventions for Necrotizing enterocolitis except? A. Broad Spectrum Antibiotic Therapy B. Umbilical Catheterization C. IV Volume Replacement D. Surgery TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 7 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. COMPOSITION OF SURFACTANT RECOVERED BY ALVEOLAR Undescended testes WASH o About 4.5% of boys at birth Nelson Textbook of Pediatrics, 20th ed. o Majority descend simultaneously during the 1st 3 months of ✔ GUIDE QUESTION life After catching a full-term baby boy, 2600g, AGA, on the 5th minute of life o By 6 mos old, the incidence decreases to 0.8% you decided to check the oxygen saturation of the patient. A maximum o If the testes has not descended by 4 months, it will remain saturation of 90% was read. The baby has good APGAR score, is undescended. comfortable, and there are no other abnormal physical findings. What is the next step in managing this patient? o Treated surgically not later than 9-15 months old A. Provide O2 support C. Observe and monitor B. Stimulate the patient D. Suction secretions RESPIRATORY CONDITIONS MINUTE(S) OF LIFE TARGET PRE DUCTAL O2 SATS SURFACTANT 1 min 60-65% Surfactant is present in high concentrations in fetal lung 2 min 65-70% homogenates by 20 wk of gestation 3 min 70-75% It appears in amniotic fluid between 28 and 32 wk of gestation Mature levels of pulmonary surfactant are present usually after 4 min 75-80% 35 wk of gestation 5min 80-85% 10min 85-95% SURFACTANT https://qrs.ly/jdeezj6 This image simply shows the huge gamut of possible differential diagnoses for a neonate that presents with respiratory distress. Remember that the problem may not be limited to the lungs (e.g., anemia can present with respiratory distress). Dr. De Vera ✔ GUIDE QUESTION APNEA A preterm 34-week neonate was referred by the nurse due to cessation defined as cessation of breathing for longer than 20 seconds or for of breathing lasting 10 seconds followed by rapid respiration of 50- any duration if accompanied by cyanosis and bradycardia 60bpm. Vital signs are stable, no cyanosis. If another episode occurs what will you advise the nurse? More common in pre-term infants A. Stimulate patient Causes B. Positive Pressure Ventilation o Most common cause is idiopathic apnea of prematurity C. CPR o direct depression of the central nervous system’s control of D. NOTA respiration (hypoglycemia, meningitis, drugs, hemorrhage, seizures) o disturbances in oxygen delivery (shock, sepsis, anemia) o ventilation defects (obstruction of the airway, pneumonia, muscle weakness). TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 8 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. Apnea Management ✔ GUIDE QUESTIONS o immediate management What is the expected chest x-ray finding of the above case? § Stimulation + O2 for 30 seconds, if it does not work → A. Fine reticular granularity of the parenchyma and air § PPV for 30 seconds, if it does not work → bronchograms § Intubate B. Prominent pulmonary vascular markings, fluid in the § CPR anytime if heart rate falls 21% oxygen for at after 18 hours of labor. The neonate was noted to have aspirated least 28 days plus least 28 days plus meconium. Twelve hours after birth, the neonate was noted to Breathing room air at 36 Breathing room air by have grunting, nasal flaring, and intercostal retractions. He was weeks postmenstrual 56 days postnatal age Mild BPD also tachycardic and was hypoxemic at 80% O2 saturation. After age or discharge home, or discharge home, drawing blood from the right radial artery and umbilical artery, a whichever comes first whichever comes first PaO2 gradient was noted. Which of the following is the initial Need† for 30% What is the primary cause of the above case? and/or positive oxygen and/or positive A. Surfactant deficiency pressure (PPV or pressure (PPV or B. Slow absorption of fetal lung fluid Severe BPD NCPAP) at 36 weeks NCPAP) at 56 days C. Persistence of the fetal circulatory pattern of right-to-left postmenstrual age or postnatal age or shunting through the PDA and foramen ovale after birth discharge home, discharge home, D. AOTA whichever comes first whichever comes first TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 9 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. *BPD usually develops in neonates being treated with oxygen and PPV for PERSISTENT PULMONARY HYPERTENSION (PPHN) respiratory failure, most commonly respiratory distress syndrome. Persistence of the clinical features of respiratory disease (tachypnea, Persistence of the fetal circulatory pattern of right-to-left retractions, crackles) is considered common to the broad description of BPD shunting through the PDA and foramen ovale after birth is a and has not been included in the diagnostic criteria describing the severity of result of excessively high PVR BPD. Infants treated with >21% oxygen and/or PPV for non-respiratory PVR normally declines rapidly as a consequence of vasodilation disease (e.g., central apnea or diaphragmatic paralysis) do not have BPD secondary to lung inflation, a rise in postnatal PaO2, a reduction unless parenchymal lung disease also develops and they have clinical in PaCO2, increased pH, and release of vasoactive substances features of respiratory distress. A day of treatment with >21% oxygen means that the infant received >21% oxygen for more than 12 hr on that day. Treatment with >21% oxygen and/or PPV at 36 weeks postmenstrual age or Pathogenesis at 56 days postnatal age or discharge should not reflect an “acute” event, but Maladaptation from acute injury should rather reflect the infant’s usual daily therapy for several days the result of increased pulmonary artery medial muscle preceding and after 36 weeks postmenstrual age, 56 days postnatal age, or thickness and extension of smooth muscle layers into the usually discharge. non-muscular, more peripheral pulmonary arterioles in BPD, bronchopulmonary dysplasia; NCPAP, nasal continuous positive airway response to chronic fetal hypoxia pressure; PPV, positive-pressure ventilation. a consequence of pulmonary hypoplasia (diaphragmatic hernia, GROUND GLASS OPACITY Potter syndrome) obstructive (ex TAPVR, Polycythemia) Other Important Facts About PPHN PaO2 or oxygen saturation gradient between a preductal (right radial artery) and a post-ductal (umbilical artery) site of blood sampling suggests right-to-left shunting through the ductus arteriosus. 2D echo with Doppler is helpful http://learningradiology.com/notes/chestnotes/hyalinemembranepage.htm https://educalingo.com/en/dic-en/ground-glass DISEASE AERATION Take a look at the flow of blood through a PDA. PDA is generally Hyaline Membrane Disease Under acyanotic because the flow is left to right. However, in certain instances Bronchopulmonary Dysplasia Over where PDA is large and/or the pulmonary vascular resistance is high Transient Tachypnea Over (such as in PPHN), shunt becomes right to left and patient therefore Meconium Aspiration Over becomes CYANOTIC! Dr. De Vera Neonatal Pneumonia Over RDS TTN PPHN DEMOGRAPHIC Pre-Term Pre-term or term, CS Term or post term, MAS Ground Glass opacities, Under- Hyper-aerated, Prominent Vascular May be normal OR depending on X-RAY aerated, atelectasis markings co-morbid condition ONSET OF Early onset but relieved with Within first 12 hours of birth. Within minutes of birth SIGNS AND minimal oxygen Cyanosis Grunting SYMPTOMS supplementation Oxygen gradient Progressive worsening of cyanosis NATURAL and dyspnea Recovers rapidly within 3 days Unpredictable course COURSE Symptoms peak within 3 days then improves Prevent with antenatal steroids Supportive TREATMENT Surfactant replacement Supplemental O2 Treat underlying cause PEEP ✔ GUIDE QUESTIONS A newborn infant born at 38 weeks AOG developed jaundice at the 12th hour of life. Within the next 8 hours, the jaundice spread up to the mid abdomen. Careful examination revealed some hepatosplenomegaly, chorioretinitis, and mild hydrocephalus. Based on the given data, what is the estimated level of bilirubin in this neonate? LUNG PATHOLOGY RESPIRATORY DISTRESS A. 5mg/dL IN NEONATES IN NEWBORNS B. 10mg/dL C. 15mg/dL https://qrs.ly/mgeezj8 https://qrs.ly/qweezjd D. 20mg/dL SUPPLEMENT: Respiratory Distress in the Newborn When you have newborns with chorioretinitis and other features like hydrocephalus and organomegaly, TORCHes should always be Five common signs considered. Tachypnea (RR >60) Dr. De Vera Retractions Nasal flaring Grunting Central cyanosis TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 10 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. ✔ GUIDE QUESTIONS PHYSIOLOGIC PATHOLOGIC In relation to the above case, if a blood test was performed and is Jaundice visible only Appears on the consistent with the bilirubin levels estimated against the physical on the 2nd-3rd day first 24-36 hours of life exam, what is this child’s risk classification based on the Bhutani chart? Serum bilirubin is A. High Risk Peaks at 5-6 mg/dL B. High Intermediate Risk rising at a rate faster than on the 2nd-4th day C. Low Intermediate Risk 5 mg/dL/24 hours D. Low Risk Decrease to below 2 mg/dL Serum bilirubin >12 mg/dL between 5-7 days of life or 10-14 mg/dL in preterm TB increases not TB increases > 5mg/dL/day > 0.5 mg/dL/hour Decline to adult levels by Jaundice persist after 10-14 days of life 10-14 days Direct reacting bilirubin is >2 mg/dL at any time Physiologic jaundice is a DIAGNOSIS OF EXCLUSION. JUST ONE FEATURE of pathologic makes it pathologic. Students often fail to remember this. Dr. De Vera JAUNDICE WITHIN 24 H KEY CLUE MOST LIKELY ETIOLOGY First born child ABO-incompatibility Second born child Rh-incompatibility History of prolonged second stage of labor Sepsis Neonatorum No prenatal check up History of maternal infection during TORCH infection pregnancy JAUNDICE AFTER 24 H ONSET OF MOST LIKELY Nelson Textbook of Pediatrics, 20th ed. KEY CLUE In relation to the above case, what is the best step in the management JAUNDICE ETIOLOGY of the child’s hyperbilirubinemia? 2-3 days Baby otherwise normal Physiologic A. Evaluate for phototherapy Mother supplements 3-4 days Breastfeeding B. Administer phenobarbital feeding with sugar water C. Follow up within 2 days >1 week Baby is purely breastfed Breast Milk D. Exchange transfusion In relation to the above case, which diagnostic modality can help COMPARISON OF JAUNDICE RELATED TO BREASTFEEDING confirm the diagnosis? BREAST FEEDING BREAST MILK A. CT Scan JAUNDICE JAUNDICE B. Blood Culture Onset 3-4 DOL End of the 1st week C. Indirect Coombs Test D. Newborn Screening Inadequate nursing, Substance in In relation to the case above, which among the ff. will most likely be Factors decreased caloric breastmilk: seen in this patient? intake Glucuronidase A. Coombs Test Positive Duration Few days 3 weeks – 3 mos B. Triangular Cord Sign Continue C. (+) blood culture growth of gram-positive cocci Treatment Stop for 2 days breastfeeding 10h/day D. Intracranial Calcifications The most serious complication of hyperbilirubinemia in the newborn is: An easy way to remember which comes first. It’s alphabetical, F comes A. Severe anemia before M. BFeeding jaundice occurs earlier than BMilk jaundice. Dr. De Vera B. Heart failure C. Respiratory distress ✔ GUIDE QUESTION D. Encephalopathy A 3-day old infant is jaundiced from the head down to the upper trunk. Jaundice appearing between the second and third day after birth in full- His serum bilirubin level is probably between: terms infants is likely due to: A. 6-8 mg/dL A. Normal changes B. 9-12 mg/dL B. Acute hemolysis C. 12-14 mg/dL C. Sepsis neonatorum D. 15-18 mg/dL D. Erythroblastosis fetalis The most common cause of jaundice in neonates is: A. Physiologic B. Acute hemolysis C. Sepsis neonatorum D. Erythroblastosis fetalis NEONATAL JAUNDICE JAUNDICE RISK FACTORS IN NEONATAL HYPERBILIRUBINEMIA Jaundice visible on the 1st day of life A sibling with neonatal jaundice or anemia Unrecognized hemolysis (ABO, Rh, other blood group, incompatibility); UDP-glucuronyl transferase deficiency (Crigler-Najjar, Gilbert disease) Non-optimal feeding (formula or breast-feeding) Deficiency of glucose-6-phosphate dehydrogenase KERNICTERUS Infection (viral, bacterial). Infant of diabetic mother. Immaturity (prematurity) Results from deposition of unconjugated bilirubin in the basal Cephalohematoma or bruising. Central hematocrit >65% ganglia and brainstem (polycythemia) Kernicterus is rare in healthy infants if the serum level is less East Asian, Mediterranean, Native American heritage than 25 mg/dL Clinical manifestation o Phase 1 – poor sucking, stupor, hypotonia, seizure o Phase 2 – hypertonia, opisthotonos, fever o Phase 3 – hypertonia TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 11 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. HYPERBILIRUBINEMIA COOMBS TEST NEGATIVE Coombs test negative with ↑ Hb and ↑B1 DIRECT HYPERBILIRUBINEMIA polycythemia (ALWAYS PATHOLOGIC) infant of diabetic mother 1. Intrahepatic Cholestasis SGA o sepsis / TORCHeS delayed cord clamping o prolonged TPN twin transfusion / maternal-fetal transfusion o hypothyroidism o galactosemia Coombs test negative with normal / ↓ Hb and normal o cystic fibrosis reticulocyte count o alpha-1-antitrypsin deficiency enclosed hemorrhage 2. Extrahepatic Cholestasis increased enterohepatic circulation o choledochal cyst o biliary atresia decreased calories (e.g. breastfeeding jaundice) § paucity of bile ducts disorders of conjugation (e.g. breastmilk jaundice) Coombs test negative with normal / ↓Hb & ↑reticulocyte count COOMBS TEST with characteristic RBC morphology Direct Coombs test – used to detect antibodies that are bound o Spherocytosis to the surface of RBCs o Elliptocytosis Indirect Coombs test – detects antibodies against RBCs that are with non-characteristic RBC morphology present unbound to the patient’s serum o G6PD deficiency Clinical uses: o Pyruvate kinase deficiency 1. Blood transfusion preparation 2. Antenatal antibody screening ✔ GUIDE QUESTIONS An infant is born premature at 36 weeks to a mother with prolonged preterm rupture of membranes. He developed jaundice on the 3rd day INDIRECT HYPERBILIRUBINEMIA of life and has poor suck, is lethargic, and has retractions. You suspect COOMBS TEST POSITIVE = ISOIMMUNIZATION him as having Gram-negative sepsis. Which antibiotic therapy will Rh / ABO incompatibility (before treatment) most likely benefit this child? A. Ampicillin + amikacin ABO INCOMPATIBILITY B. Trimethoprim + sulfamethoxazole Most common cause of hemolytic disease of the newborn C. Ciprofloxacin Occurs in 20-25% of pregnancies but hemolysis develops in only D. Imipenem + Cilastatin 10% of such offspring The most important risk factor that predisposes a neonate to sepsis is: A. History of maternal infection Mother is type O and baby is either A or B B. Prematurity Most cases are mild; jaundice C. Route of delivery Mild hepatosplenomegaly D. Unestablished normal flora Phototherapy; if severe, IVIG or exchange transfusion ABO incompatibility lab test results: EVALUATION OF HYPERBILIRUBINEMIA 1. Weakly to moderately (+) direct Coombs test 2. Spherocytes in blood smear 3. Hemoglobin is usually normal but maybe as low as 10-12 g/dL 4. Increased reticulocyte count in 10-15% 5. Increased B1 (may reach 20 mg/dL in 10-20%) RH INCOMPATIBILITY Rh antigenic determinants are genetically transmitted from each parent & direct the production of blood group factors (C, c, D, d, E, e) Each factor can elicit a specific antibody response where 90% are due to D antigen. Conditions when Rh incompatibility occurs: 1.When Rh+ blood is infused into a Rh- woman by error, or; 2.When Rh+ fetal blood with D Ag inherited from a Rh+ father enter the maternal circulation during pregnancy, with spontaneous or induced abortion, at delivery Ab formation against D Ag may be induced in the unsensitized Rh- recipient mother → rise in IgM initially then rise in IgG crossing the placenta Rarely occurs during the 1st pregnancy because transfusion of Rh+ fetal blood into a Rh- mother occurs near the time of delivery, too late for the mother to become sensitized & transmit antibody to her infant before delivery. Injection of anti-D gamma globulin (RhoGAM) into the mother CAUSES OF EVALUATION OF immediately after the delivery of each Rh+ infant reduces Rh HYPERBILIRUBINEMIA HYPERBILIRUBINEMIA hemolytic disease https://qrs.ly/5leezm2 https://qrs.ly/u3eezlx Rh incompatibility lab test results: Please study this flowchart as it is a guide to evaluate jaundice / Before treatment: hyperbilirubinemia. Some key points. 1. Direct Coombs test is + 1. Direct hyperbilirubinemia = pathologic = cholestasis 2. Anemia 2. In neonates, it is mostly indirect hyperbilirubinemia 3. Increased reticulocyte count 3. First thing to check is Coombs if positive think RH vs ABO 4. B1 rises rapidly in the 1st 6 hours of life 4. Coombs (-) with increase Hgb = polycythemia 5. Coombs neg with normal Hgb and normal retic = physiologic 5. B2 may also be elevated Dr. De Vera TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 12 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. PEDIATRIC NUTRITION STAGES OF LACTATION ✔ GUIDE QUESTIONS FIRST 0-7 DAYS: COLOSTRUM The most practical and pertinent guide in evaluating nutritional status 37-84 mL/day; D1-2 of life in children is: Watery (>80% water) A. Biochemical studies of food and determination of vitamin levels protein-rich B. Regular or periodic follow up of weight and height High in Ig/protective factors: lactoferrin, lysozyme C. X-ray studies of bone D. Dietary history Na, vit A/K & growth factors Colostrum is produced over the first 2 days of lactation. The secretion Low levels: fat & carbohydrates has higher protein and lower fat and lactose than mature human milk. TRANSITIONAL MILK Likewise, it is especially rich in: A. IgM Between colostrum & mature milk B. IgG Rising levels of macronutrients C. IgD MATURE MILK D. IgA The whey-to-casein ratio in mature human milk is about: D 10-14 of life A. 1:1 Colostrum content + high fat & lactose B. 1:4 C. 3:2 INVOLUTIONARY MILK D. 4:1 produced when breastfeeding frequency decreases Whey is better than Casein. Think bodybuilders. reverts to being more like colostrum Dr. De Vera Until when can you feed breastmilk solely to a child? BREAST MILK COMPOSITION A. 3 months casein to whey ratio - low B. 6 months o 10:90 in early milk C. 12 months o 40:60 in mature milk D. 2 years o 50:50 in late lactation Exclusive up to 6 mos. Then complimentary feeding. Breastfeeding is fat globules bound by membranes rich in: best for baby up to 2 years old and BEYOND… Dr. De Vera o phospholipids – cell growth & brain development o cholesterol – facilitates myelination of the CNS FEEDING: 1ST 6 MONTHS OF LIFE Breast milk has MORE compared to Formula milk Initiated as soon as after birth o Exceptions o Within 1-4 hrs § Iron § Vit D Schedule: “Self-regulation” by infant § Vit K o 1st wk 60-90mL/feeding: 6-9 feedings/24hrs o Middle of the night feedings: from birth to 6-8wks, beyond ✔ GUIDE QUESTION o By 9-12 mos: 3 meals/day + snacks Overall, the 3-month-old male infant is apparently well but he o There is no need to feed infants every time they cry. regurgitates 10-20 mL of milk usually after feeding, what is the best management for this condition? A. Advise Thick Feeding BREAST MILK B. Endoscopy BREAST TIMING OF C. Observe and Reassurance CONSISTENCY CONTENT D. Upper GI Endoscopy MILK RELEASE At the start of High lactose, BREAST MILK STORAGE GUIDELINES PPS Foremilk Watery TEMPERATURE DURATION feeding high protein As feeding Room temperature 25°C 1 hour progresses Hind the fat Refrigerator 4°C 8 days until towards Creamy milk content of Freezer (1-door) 2 weeks the end of foremilk) Freezer (2-door) 3 months feeding Deep freezer (-20°C) 6 months TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 13 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. GERD Important points to remember: TB – after two weeks of treatment, mother no longer considered Epidemiology: infants’ condition peak at 4 mos & resolve infectious, may do direct breastfeeding. Prior to this, expressed mother’s mostly at 12 mos of age & nearly all at 24 mos old milk can be given Genetic predisposition: AD (Chromosome 13q, 14 & 19) HIV – not absolutely contraindicated. In times where no safer alternative Clinical manifestations: is available, HIV mother can breastfeed provided that: o infants: postprandial regurgitation, signs of esophagitis 1. Shortest duration possible (irritability, arching, choking, gagging, feeding aversion), 2. Exclusive breastfeeding (mixed feeding increases risk of AGE which increases transmission rate because of damaged intestinal villi) failure to thrive Dr. De Vera o Older children: regurgitation, abdominal & chest pain; SYMPTOMS ACCORDING TO AGE respiratory manifestations related to asthma or sinusitis MANIFESTATIONS INFANTS CHILDREN ADOLESCENTS AND ADULTS Diagnosis: Barium study of esophagus & upper GIT (poor Impaired quality of life +++ +++ +++ sensitivity and specificity) Regurgitation ++++ + + Definitive test: esophageal pH probe Excessive crying / irritability +++ + - o Esophageal pH monitoring of distal esophagus: document Vomiting ++ ++ + acidic reflux episodes (normal value of distal esophageal acid Food refusal / feeding ++ + + exposure 2 grams) may produce abdominal pain and Hyperostosis osmotic diarrhea Vit A Intoxication Absence of metaphyseal changes ✔ GUIDE QUESTIONS MINERALS IN BREAST MILK A deficiency of this trace element is associated with skin ulcers, reduced IRON immune response and hypogonadal dwarfism: The iron content of breast milk is unaffected by maternal iron A. Zinc status, maternal iron deficiency, or supplementation B. Chromium Infants’ iron requirements are largely met from body stores built C. Cobalt up in utero D. Copper Irritability, pruritus, painful extremities, with brawny swelling, coarse Combined with breastfeeding, these stores are usually sufficient hair, dry skin, seborrhea and increased intracranial pressure is seen in: to meet infants iron needs for 6 – 12 months. A. Hypervitaminosis A Lesser in breast milk but more bioavailable B. Hypervitaminosis D C. Hypovitaminosis A ZINC D. Hypovitaminosis D Infant’s requirement in the first 6 months are largely met by fetal In a Southwestern town in Mindanao, children are fed mostly with corn as staple. The most common vitamin deficiency encountered in these stores accumulated in the last trimester of pregnancy → children is: thereafter should be met by appropriate complementary foods A. Niacin B. Folate IODINE C. Thiamine Iodine accumulates in the mammary gland and levels in breast D. Riboflavin milk reflect maternal diet. In children with malnutrition, the most seriously compromised immunologic function is: In areas where iodine deficiency is common, maternal A. Antibody production supplementation is necessary. B. Phagocytosis Please remember this. Most micronutrients in breastmilk are not affected C. Cell-mediated immunity by maternal diet/status. Vitamin D and Iodine are affected by maternal D. Complement activation status. One of the macronutrients severely affected in malnutrition are For Iron and Zinc, please remember that full-term neonates are born with proteins (think Kwashiorkor). Therefore, problem in proteins → sufficient stores that are enough for the first 6 months of life. problem in antibodies. Dr. De Vera Dr. De Vera Micronutrient Clinical Clues Remarks A 5-year-old male child is brought to the clinic for being weak and wants 90% bioavailable in her child to be dewormed. It has been going on for the past 6 months Retinal piments, bone, according to the mother. She says that the child usually is prone to breast milk Vitamin A tooth and epithelium having diarrhea and usually he has episodes every month. On Premature babies development examination, the child looks apathetic, he is underweight, there is some more vulnerable edema of the bilateral lower extremities, the abdomen is protuberant, Bone formation Content in the hair is sparse thin with reddish streaks, what is the most likely Vitamin D Rickets and breastmilk depends diagnosis in this patient? Osteomalacia on maternal status A. Marasmus RBC hemolysis in B. Non-edematous protein energy malnutrition Vitamin E Anti-oxidant premature infants C. Kwashiorkor Prevented by D. Pellagra Vitamin K VKDB prophylaxis Affects bones and joints UNDERNUTRITION Vitamin C Perifollicular Scurvy Greatest risk occurs in the 1st 1000 days (0-24 months) hemorrhages Term malnutrition covers undernutrition to over-weight Gum swelling International standards of determining anthropometry using Stores are adequate for 6 months WHO charts Iron & Zinc Content in breastmilk NOT affected by o Height for age (length for age if L → pulmonary respiratory tract infections since she was an infant. She was apparently diagnosed to have a “heart disease” but was lost to follow-up. On PE, vascular obstructive disease (Eisenmenger's syndrome) → PA she weighs 9 kgs, (+) gr 4/6 systolic regurgitant murmur heard loudest is prominent with RVH and pulmonary hypertension → at the left lower sternal border. Which chambers of the heart are likely bidirectional shunt causes cyanosis enlarged on chest radiograph? ✔ GUIDE QUESTIONS A. Right side B. Left side A 3-year-old girl was brought to your clinic for a well child visit. Upon C. Both left and right auscultation, you noted a grade 3/6 systolic murmur described as D. None of the above “blowing” on the upper left 2nd ICS and a widely split S2. There is no history of cyanosis. The other PE findings are unremarkable. 2D echo This is a case of ventricular septal defect based on the characteristic revealed RVH and RAH with a shunt defect measuring 3mm at the site murmur which is systolic regurgitant in character and timing and of the fossa ovalis. What is true about this disease EXCEPT? heard best at the LLSB. The left side of the heart initially enlarges in A. Surgery should be attempted immediately VSD because of the left to right direction of the shunt and more blood B. Spontaneous closure is 87% for lesions < 8mm enters the pulmonary circulation leading to enlargement of the C. Large defects may lead to heart failure pulmonary artery and the left chambers of the heart. Dr. Punongbayan D. LA is not enlarged TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 79 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. This is a case of atrial septal defect. Main features are the ff: acyanotic, CYANOTIC CONGENITAL HEART DISEASES systolic blowing murmur heard best on the left 2nd ICS with a widely split S2 and right-sided enlargement. Surgery is not needed at this I. DECREASED PULMONARY BLOOD FLOW point if the defect is 3mm or less. The most common type of ASD is Obstruction (TV, RV or pulmonary valve level) & a pathway by ostium secundum (50-70%) which is present at the site of fossa ovalis. which systemic venous blood can shunt from R->L & enter the The widely split S2 results partially from RBBB which delays both the electrical depolarization of the RV and the ventricular contraction systemic circulation resulting in delayed closure of the pulmonary valve. Tricuspid atresia Dr. Punongbayan Tetralogy of Fallot What produces the widely split S2 in ASD? Single ventricle with PS results partially from RBBB which delays both the electrical Degree of cyanosis depends on the degree of obstruction to depolarization of the RV and the ventricular contraction pulmonary blood flow resulting in delayed closure of the pulmonary valve TETRALOGY OF FALLOT Occurs in 10% of all CHDs Most common cyanotic heart defect beyond infancy 4 abnormalities: large VSD, RVOT obstruction, RVH, overriding of the aorta RVOT obstruction is most frequently in the form of infundibular stenosis (45%) Manifestations of TOF: Ejection click; single S2; gr 3-5/6 systolic ejection murmur at the mid and ULSB with radiation to the upper back (from PS) CXR: small heart size, decreased pulmonary vascular markings; concave main PA with an upturned apex (couer en sabot or CONGENITAL ACYANOTIC HEART DISEASES boot-shaped heart) DISEASE HEART SOUNDS OTHER PE FINDINGS Systolic ejection Right sided ASD murmur at 2nd LICS enlargement widely split S2 Left sided enlargement; Systolic regurgitant biventricular VSD murmur at LLSB hypertrophy if with loud and single S2 Eisenmenger syndrome Continuous Bounding pulses; “machinery-like” wide pulse pressure; PDA murmur at the 2nd left left-sided enlargement, infraclavicular area enlarged aorta Boot-shaped heart of TOF ✔ GUIDE QUESTIONS ✔ GUIDE QUESTIONS Which of the following is associated with the presence of an endocardial An 18-month-old male patient was brought to the clinic because of cushion defect? intermittent cyanotic episodes more prominent in the lips, mouths, A. Noonan syndrome fingernails, and toenails which lasts for a few minutes and goes away. B. Marfan syndrome Sometimes the mother notices that the child assumes a squatting C. Hunter-Hurler syndrome position. On examination there is a systolic murmur heard loudest over D. Down syndrome the left sternal border. What is the most likely diagnosis? Atrioventricular septal defect or ECD is associated with Down A. VSD C. TGA syndrome. B. TOF D. TAPVR Marfan syndrome is an inherited disorder of the connective tissue Given the age of the patient and features of cyanosis, systolic murmur causing abnormalities in the eyes, bone, heart, and blood vessels on the left sternal border, and the relief noted upon squatting, these (mitral valve prolapse and progressive enlargement of the aorta). are consistent with Tetralogy of Fallot, the most common cyanotic Hunter syndrome or mucopolysaccharidosis (MPS II) – a rare genetic congenital heart disease in infants and young children. disorder wherein glycosaminoglycans build up in body tissues; due to Dr. Punongbayan a deficiency of iduronate-2-sulfatase causing heparan sulfate and What is the main pathophysiologic mechanism behind the dermatan sulfate to accumulate in all body tissues → thickening of hypercyanotic spells or Tet spells in TOF? cardiac valves resulting in improper valve closure A. due to increased systemic vascular resistance Noonan syndrome – genetic disorder with facial anomalies, short B. due to overload and pulmonary congestion stature, webbed neck, chest deformities, undescended testes; C. due to decreased pulmonary blood flow pulmonary stenosis is the common cardiac defect. D. due to increased left to right shunting Dr. Punongbayan Short of doing a 2D echo, what is one method of distinguishing cyanotic MECHANISM OF HYPOXIC SPELL congenital heart disease from pulmonary disease? A. Chest x-ray C. Hyperoxia test B. ECG D. ABG HYPEROXIA TEST 100% FiO2 (O2 hood/rebreather mask) for 10-15 mins Principle: in the absence of fixed cardiac shunt, 100% O2 will increase alveolar pO2 → increase in pulmonary venous and systemic arterial pO2 CHILD PRESENTING WITH CYANOSIS BASIC CASE DIAGNOSIS Cyanosis manifesting within Transposition of great few hours at birth or within Arteries few days of life Cyanosis manifesting after the first year of life, usually in an Tetralogy of Fallot infant or a toddler TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN Page 80 of 107 For inquiries visit www.topnotchboardprep.com.ph or email us at [email protected] This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly.  TOPNOTCH MEDICAL BOARD PREP PEDIATRICS MAIN HANDOUT BY DRS. DE VERA AND PUNONGBAYAN For inquiries visit www.topnotchboardprep.com.ph or https://www.facebook.com/topnotchmedicalboardprep/ This handout is only valid for March 2023 PLE batch. This will be rendered obsolete for the next batch since we update our handouts regularly. PATHOPHYSIOLOGY AND MANAGEMENT II. INCREASED PULMONARY BLOOD FLOW Not associated with obstruction to pulmonary blood flow Cyanosis due to either abnormal ventricular-arterial connections or total mixing of systemic venous & pulmonary venous blood within the heart Transposition of the great vessels Total anomalous pulmonary venous return Truncus arteriosus TRANSPOSITION OF THE GREAT VESSELS What is the pathophysiology of this condition? The aorta arises from the RV carrying desaturated blood to the body; the PA arises posteriorly from the LV carrying oxygenated blood to the lungs Result: complete separation of pulmonary & systemic MANAGEMENT OF HYPO