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  • Question 1 - A 2 year old girl with a history of oligohydramnios, hypotonia, and scissoring...

    Incorrect

    • A 2 year old girl with a history of oligohydramnios, hypotonia, and scissoring of the legs, presents with delayed walking. She was born by breech delivery. Clinical examination reveals absence of fever and no dysmorphism. Although the limbs look symmetrical and equal, the right leg seems to be shorter. The girl is otherwise healthy. Neurological examination reveals hypotonia with normal reflexes and power. The mother confirms there is no history of myelomeningocele, cerebral palsy, or birth asphyxia. What is the most likely diagnosis?

      Your Answer: Slipped upper femoral epiphysis

      Correct Answer: Congenital dislocation of the hip

      Explanation:

      In this particular case, the symptoms and signs are suggestive for congenital dislocation of the hip. There are several risk factors present including, a breech delivery, female gender (female:male = 6:1) and oligohydramnios. It is also more common in the left hip than the right.

    • This question is part of the following fields:

      • Neonatology
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  • Question 2 - Which of the following maternal factors is associated with oligohydramnios? ...

    Incorrect

    • Which of the following maternal factors is associated with oligohydramnios?

      Your Answer:

      Correct Answer: Maternal thrombotic disorder

      Explanation:

      Oligohydramnios is a deficiency in the amniotic fluid volume, measured via ultrasound. Maternal factors associated with oligohydramnios include conditions where there is placental insufficiency such as chronic hypertension, preeclampsia or a thrombotic disorder, post-term pregnancy, premature rupture of membranes, certain chromosomal abnormalities, and obstructions of the foetal urinary tract. On the other hand conditions that are associated with polyhydramnios (excess amniotic fluid) include maternal diabetes, multiple gestations, Rh incompatibility and pulmonary abnormalities.

    • This question is part of the following fields:

      • Neonatology
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  • Question 3 - Which is the most common cardiac defect in babies with Down’s syndrome? ...

    Incorrect

    • Which is the most common cardiac defect in babies with Down’s syndrome?

      Your Answer:

      Correct Answer: Atrioventricular septal defect (AVSD)

      Explanation:

      Congenital heart defects are common (40-50%)| they are frequently observed in patients with Down syndrome who are hospitalized (62%) and are a common cause of death in this aneuploidy in the first 2 years of life.The most common congenital heart defects are the following:- Endocardial cushion defect (43%), which results in atrioventricular septal defect (AVSD)/AV canal defect- Ventricular septal defect (32%)- Secundum atrial septal defect (10%)- Tetralogy of Fallot (6%)- Isolated patent ductus arteriosus (4%).About 30% of patients have more than one cardiac defect. The most common secondary lesions are patent ductus arteriosus (16%), atrial septal defect, and pulmonic stenosis (9%). About 70% of all endocardial cushion defects are associated with Down syndrome.Valve abnormalities, such as mitral valve prolapse or aortic regurgitation may develop in up to 40-50% of adolescents and adults who were born without structural heart disease.

    • This question is part of the following fields:

      • Neonatology
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  • Question 4 - A term baby with a birth weight of 3.6 kg with hypoxic ischaemic...

    Incorrect

    • A term baby with a birth weight of 3.6 kg with hypoxic ischaemic encephalopathy is intubated and ventilated. Cooling treatment has started. The baby is sedated and paralysed and is being ventilated on the mode continuous mandatory ventilation (CMV). Settings are: targeted tidal volume 14 ml, maximum PIP 25, PEEP 5, rate 60/min, FiO2 0.21. Baby’s oxygen saturations are 100%. Blood gas shows pH 7.47, CO2 2.8 kPa, BE –6.What is the first change that should be made to the ventilation?

      Your Answer:

      Correct Answer: Decrease rate

      Explanation:

      Ventilatory rates of 40-60 breaths/min should be provided initially, with proportionally fewer assisted breaths provided if the infant’s spontaneous respiratory efforts increase. Although this practice has not been extensively studied, initial inflation of the new-born’s lungs with either slow-rise or square-wave inflation to a pressure of 30-40 cm H2 O for approximately 5 seconds has been reported to result in more rapid formation of Functional Residual Capacity (FRC).At the moment of delivery and first breath, the neonatal lung is converting from a fetal non-aerated status to a neonatal status. The neonatal lung requires gas exchange, and this necessitates the development of FRC with the resorption of lung fluid and the resolution of most of the atelectasis. Therefore, initial slow ventilation with more prolonged inspiratory times may be useful to assist in this task, balanced against the need to avoid inappropriate inspiratory pressures.Flow-controlled, pressure-limited mechanical devices are acceptable for delivering PPV. These mechanical devices control flow and limit pressure and be more consistent than bags. Self-inflating and flow-inflating bags remain a standard of care. Laryngeal mask airways are effective for assisted ventilation when bag-mask ventilation and intubation are unsuccessful.

    • This question is part of the following fields:

      • Neonatology
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  • Question 5 - A baby born at 25 weeks gestation around 24 hours ago is reported...

    Incorrect

    • A baby born at 25 weeks gestation around 24 hours ago is reported to be in a critical state. Birth weight is 550g and the baby shows a poor respiratory effort. The membranes ruptured just prior to the delivery with no signs of maternal fever or sepsis. The infant received no antenatal steroids. After delivery, the neonate was subsequently intubated, resuscitated and given a dose of Curo surf. he was extubated onto CPAP within 6 hours. UVC and UAC access was established and benzylpenicillin and gentamicin were administered. Small amounts of maternal breast milk were given for mouthcare. The baby's cardiovascular system was stable and early ABG samples were within acceptable ranges. At 24 hours of age the baby deteriorates with numerous desaturations and bradycardias requiring reintubation. First gas following reintubation shows pH 7.19, CO2 8.6 kPa, BE -10. Oxygen requirement is 60%. FBC is unremarkable. The baby is cool peripherally and central CRT is 3-4 seconds. There is a loud machinery murmur. Abdominal radiograph shows a football sign. What is the most likely cause of the baby's deterioration?

      Your Answer:

      Correct Answer: Spontaneous intestinal perforation

      Explanation:

      Spontaneous intestinal perforation (SIP) of the new-born, also referred to as isolated perforation or focal intestinal perforation (FIP) of the new-born, is a single intestinal perforation that is typically found at the terminal ileum. SIP occurs primarily in preterm infants with very low birth weight (VLBW, birth weight <1500 g) and extremely low birth weight (ELBW, birth weight <1000 g), umbilical catheter placement, maternal drug use and twin-to-twin transfusion syndrome. SIP is a separate clinical entity from necrotizing enterocolitis, the most severe gastrointestinal complication of preterm infants.

    • This question is part of the following fields:

      • Neonatology
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  • Question 6 - A floppy new-born baby has epicanthic folds, a thin upper lip and smooth...

    Incorrect

    • A floppy new-born baby has epicanthic folds, a thin upper lip and smooth philtrum. He was monitored closely throughout pregnancy for intra-uterine growth retardation (IUGR) and a ventricular septal defect. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Fetal alcohol syndrome

      Explanation:

      Fetal alcohol syndrome (FAS) is a common yet under-recognized condition resulting from maternal consumption of alcohol during pregnancy. The diagnosis of fetal alcohol syndrome (FAS) is based on findings in the following 3 areas: (1) characteristic facial anomalies (see image below), (2) growth retardation (intrauterine growth restriction and failure to have catch-up growth), and (3) CNS involvement (cognitive impairment, learning disabilities, or behavioural abnormalities).Key characteristic craniofacial abnormalities include the following:- Smooth philtrum- Thin, smooth vermilion border of the upper lipShort palpebral fissures (< 10th percentile for age)Other craniofacial abnormalities are as follows:- Midface hypoplasia- Microphthalmia- Strabismus- PtosisCNS and neurobehavioral abnormalities include the following:- Microcephaly- Intellectual impairment (mild-to-moderate mental retardation)- Cognitive impairment- Developmental delay- Irritability in infancy- Hyperactivity in childhood or attention deficit hyperactivity disorder (ADHD)- Seizures- Delayed or deficient myelination- Agenesis or hypoplasia of the corpus callosumSkeletal abnormalities include the following:- Radioulnar synostosis- Flexion contractures- Camptodactyly- Aberrant palmar creases, especially hockey-stick palmar crease- Clinodactyly- Klippel-Feil anomaly- Hemivertebrae- Scoliosis- Dislocated jointsOther major congenital anomalies include the following:- Cleft palate- Heart defects- Renal anomalies- DiGeorge sequenceFunctional problems include the following:- Refractive problems (e.g., myopia, astigmatism)- Hearing lossGrowth deficiency includes the following:- Infant small for gestational age (< 10th percentile for weight or length)- Postnatal growth deficiency

    • This question is part of the following fields:

      • Neonatology
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  • Question 7 - A 7 day old male infant, born at term, presents with umbilical bruising...

    Incorrect

    • A 7 day old male infant, born at term, presents with umbilical bruising and bleeding. History reveals he wasn’t given vitamin K after birth. Blood exam is normal but with a high prothrombin time and activated partial thromboplastin time. What is the most appropriate treatment?

      Your Answer:

      Correct Answer: Intravenous vitamin K plus fresh frozen plasma

      Explanation:

      The clinical picture suggests a vitamin K deficiency haemorrhagic disease of the new-born and requires IV vitamin K and fresh frozen plasma. This usually presents with gastrointestinal bleeding, bleeding from the umbilical stump and bruising after 2–7 days.

    • This question is part of the following fields:

      • Neonatology
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  • Question 8 - A new-born baby is born with a midline lumbosacral cystic lesion. Occipitofrontal circumference...

    Incorrect

    • A new-born baby is born with a midline lumbosacral cystic lesion. Occipitofrontal circumference was above the 90th percentile.The next best step is?

      Your Answer:

      Correct Answer: Cranial ultrasound

      Explanation:

      The baby may have hydrocephalus and no surgery can be done until it is excluded.Cranial sonography is an important part of neonatal care in general, and high-risk and unstable premature infants, in particular. It allows rapid evaluation of infants in the intensive care units without the need for sedation and with virtually no risk. Expectedly, sonography represents an ideal imaging modality in neonates due to its portability, lower cost, speed, and lack of ionizing radiations. Although there are numerous indications for cranial sonography, it appears to be most useful for detection and follow-up of intracranial haemorrhage, hydrocephalus, and periventricular leukomalacia (PVL).

    • This question is part of the following fields:

      • Neonatology
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  • Question 9 - A new-born child is not moving his left arm properly. He had shoulder...

    Incorrect

    • A new-born child is not moving his left arm properly. He had shoulder dystocia during childbirth. His arm is hanging down with the shoulder internally rotated, elbow extended, forearm pronated and wrist flexed. What is the most likely diagnosis?

      Your Answer:

      Correct Answer: Erb palsy

      Explanation:

      Erb’s palsy is a paralysis of the arm caused by injury to the brachial plexus, specifically the severing of the upper trunk C5–C6 nerves.The infant with an upper plexus palsy (C5-C7) keeps the arm adducted and internally rotated, with the elbow extended, the forearm pronated, the wrist flexed, and the hand in a fist. In the first hours of life, the hand also may appear flaccid, but strength returns over days to months.The right side is injured in 51% of cases. Left side occurs in 45% of patients and bilateral injuries, in 4%.

    • This question is part of the following fields:

      • Neonatology
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  • Question 10 - Which of the following is a risk factor for neonatal death and stillbirth?...

    Incorrect

    • Which of the following is a risk factor for neonatal death and stillbirth?

      Your Answer:

      Correct Answer: Parental unemployment

      Explanation:

      Risk factors for neonatal death and stillbirth (weak predictive value):- African American race- Advanced maternal age- History of fetal demise- Maternal infertility- History of small for gestational age infant- Small for gestational age infant- Obesity- Paternal age- Poverty and poor quality of careSo in this case the risk factor is the parental unemployment

    • This question is part of the following fields:

      • Neonatology
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  • Question 11 - A neonate of Asian parents presents with jaundice appearing less than 24 hours...

    Incorrect

    • A neonate of Asian parents presents with jaundice appearing less than 24 hours after birth. You are concerned about glucose-6-phosphate dehydrogenase deficiency. What is the mode of inheritance of this condition?

      Your Answer:

      Correct Answer: X-linked

      Explanation:

      Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common enzyme deficiency in humans.It has a high prevalence in persons of African, Asian, and Mediterranean descent. It is inherited as an X-linked recessive disorder.G6PD deficiency is polymorphic, with more than 300 variantsG6PD deficiency can present as neonatal hyperbilirubinemia. Besides, persons with this disorder can experience episodes of brisk haemolysis after ingesting fava beans or being exposed to certain infections or drugs. Less commonly, they may have chronic haemolysis. However, many individuals with G6PD deficiency are asymptomatic.Most individuals with G6PD deficiency do not need treatment. However, they should be taught to avoid drugs and chemicals that can cause oxidant stress. Infants with prolonged neonatal jaundice as a result of G6PD deficiency should receive phototherapy with a bili light.

    • This question is part of the following fields:

      • Neonatology
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  • Question 12 - The new-born hearing test is best carried out within the first four or...

    Incorrect

    • The new-born hearing test is best carried out within the first four or five weeks of life, until what age can it be performed?

      Your Answer:

      Correct Answer: 12 weeks

      Explanation:

      Ideally, the test is done in the first 4 to 5 weeks, but it can be done at up to 3 months of age.The identification of all new-borns with hearing loss before age 6 months has now become an attainable realistic goal. In the past, parents and paediatricians often did not suspect a hearing loss until the age of 2-3 years, after important speech and language milestones have not been met. By the time these milestones are missed, the hearing-impaired child has already experienced irreversible delays in speech and language development.

    • This question is part of the following fields:

      • Neonatology
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  • Question 13 - A child presents with lymphoedema. Clinical examination reveals she has widely spaced nipples...

    Incorrect

    • A child presents with lymphoedema. Clinical examination reveals she has widely spaced nipples and a systolic murmur. Her femoral pulses are absent. Her mother admits she did not have any scans during gestation. What is the most probable diagnosis?

      Your Answer:

      Correct Answer: Turner syndrome

      Explanation:

      Turner syndrome is a genetic disease that affects females. It presents with wide-spread nipples, low hairline, lymphoedema, short 4th metacarpals, high-arched palate, cardiac problems, and horseshoe kidneys.

    • This question is part of the following fields:

      • Neonatology
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  • Question 14 - Which of the following is responsible for the closure of the ductus arteriosus...

    Incorrect

    • Which of the following is responsible for the closure of the ductus arteriosus at birth?

      Your Answer:

      Correct Answer: Reduced level of prostaglandins

      Explanation:

      The ductus arteriosus is normally patent during fetal life| it is an important structure in fetal development as it contributes to the flow of blood to the rest of the fetal organs and structure. From the 6th week of fetal life onwards, the ductus is responsible for most of the right ventricular outflow, and it contributes to 60% of the total cardiac output throughout fetal life. Only about 5-10% of its outflow passes through the lungs.This patency is promoted by continual production of prostaglandin E2 (PGE2) by the ductus.In the foetus, the oxygen tension is relatively low, because the pulmonary system is non-functional. Coupled with high levels of circulating prostaglandins, this acts to keep the ductus open. The high levels of prostaglandins result from the little amount of pulmonary circulation and the high levels of production in the placenta.At birth, the placenta is removed, eliminating a major source of prostaglandin production, and the lungs expand, activating the organ in which most prostaglandins are metabolized. In addition, with the onset of normal respiration, oxygen tension in the blood markedly increases. Pulmonary vascular resistance decreases with this activity.Normally, functional closure of the ductus arteriosus occurs by about 15 hours of life in healthy infants born at term. This occurs by abrupt contraction of the muscular wall of the ductus arteriosus, which is associated with increases in the partial pressure of oxygen (PO2) coincident with the first breath. A preferential shift of blood flow occurs| the blood moves away from the ductus and directly from the right ventricle into the lungs. Until functional closure is complete and PVR is lower than SVR, some residual left-to-right flow occurs from the aorta through the ductus and into the pulmonary arteriesA balance of factors that cause relaxation and contraction determine the vascular tone of the ductus. Major factors causing relaxation are the high prostaglandin levels, hypoxemia, and nitric oxide production in the ductus. Factors resulting in contraction include decreased prostaglandin levels, increased PO2, increased endothelin-1, norepinephrine, acetylcholine, bradykinin, and decreased PGE receptors. Increased prostaglandin sensitivity, in conjunction with pulmonary immaturity leading to hypoxia, contributes to the increased frequency of patent ductus arteriosus (PDA) in premature neonates.Although functional closure usually occurs in the first few hours of life, true anatomic closure, in which the ductus loses the ability to reopen, may take several weeks. The second stage of closure related to the fibrous proliferation of the intima is complete in 2-3 weeks.

    • This question is part of the following fields:

      • Neonatology
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  • Question 15 - According to NICE guidelines, which of the following factors pose an increased risk...

    Incorrect

    • According to NICE guidelines, which of the following factors pose an increased risk of severe hyperbilirubinemia?

      Your Answer:

      Correct Answer: History of a previous sibling requiring phototherapy for jaundice

      Explanation:

      Identify babies as being more likely to develop significant hyperbilirubinemia if they have any of the following factors:- gestational age under 38 weeks- a previous sibling with neonatal jaundice requiring phototherapy- mother’s intention to breastfeed exclusively- visible jaundice in the first 24 hours of life.

    • This question is part of the following fields:

      • Neonatology
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  • Question 16 - A term baby with a birth weight of 4.5 kg with meconium aspiration...

    Incorrect

    • A term baby with a birth weight of 4.5 kg with meconium aspiration syndrome is intubated and ventilated. Conventional ventilation was unsuccessful and so a trial of high-frequency oscillatory ventilation has commenced. Settings are mean airway pressure 14 cmH2O, delta P 25, rate 10 Hz, FiO2 1.0. Baby’s oxygen saturations are 84%. Blood gas shows pH 7.32, CO2 6.5 kPa, BE –4. Chest X-ray shows poorly inflated lungs.What is the first change that should be made to the ventilation?

      Your Answer:

      Correct Answer: Increase mean airway pressure

      Explanation:

      The baby needs more oxygen saturation which could be attempted by increasing the mean airway pressureAfter initial resuscitation and stabilization, the following should be the ventilator settings used:Rate: 30-40/minutePeak inspiratory pressure (PIP) – determined by adequate chest wall movement.An infant weighing less than 1500 grams: 16-28 cm H2O.An infant weighing greater than 1500 grams: 20-30 cm H2O. Positive end expiratory pressure (PEEP): 4 cm of H2O OR 5-6 cm if FiO2 > 0.90.FiO2: 0.4 to 1.0, depending on the clinical situation.Inspiratory time: 0.3-0.5 sec.After 15 to 30 minutes, check arterial blood gases and pH.If the PaO2 or the O2 saturation is below accepted standards, the FiO2 can be raised to a maximum of 1.0. If the PaO2 or O2 saturation is still inadequate, the mean airway pressure can be raised by increasing either the PIP, PEEP, inspiratory time or the rate, leaving inspiratory time constant.If the PaCO2 is elevated, the rate or peak inspiratory pressure can be raised.

    • This question is part of the following fields:

      • Neonatology
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  • Question 17 - Congenital hypothyroidism is associated with which of the following clinical features? ...

    Incorrect

    • Congenital hypothyroidism is associated with which of the following clinical features?

      Your Answer:

      Correct Answer: Cardiomegaly

      Explanation:

      Congenital hypothyroidism results from insufficient levels of thyroid hormone in the body since birth, which is either due to dysgenesis of the thyroid gland or dyshormonogenesis. The important features of this disease include coarse facial features (macroglossia, large fontanelles, depressed nasal bridge, hypertelorism, etc.), failure to thrive, cardiomegaly, hypotonia, umbilical hernia, and low core body temperature among many others. Frontal bossing is the usual feature of rickets and acromegaly. Mitral regurgitation is not usually associated with congenital hypothyroidism.

    • This question is part of the following fields:

      • Neonatology
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  • Question 18 - Which of the following congenital diseases is NOT associated with raised alpha-fetoprotein levels?...

    Incorrect

    • Which of the following congenital diseases is NOT associated with raised alpha-fetoprotein levels?

      Your Answer:

      Correct Answer: Down's syndrome

      Explanation:

      Alpha-fetoprotein has significance primarily as a tumour marker, but maternal levels are frequently measured to detect some of the congenital abnormalities, in which the levels of alpha-fetoprotein are either increased or decreased. The conditions associated with raised alpha-fetoprotein levels are: spina bifida, anencephaly, myelomeningocele, oesophageal atresia, congenital nephrotic syndrome, and turner’s syndrome. Down’s syndrome or trisomy 21 is associated with low levels of maternal alpha-fetoprotein.

    • This question is part of the following fields:

      • Neonatology
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  • Question 19 - A 23-week-old neonate was delivered in triage following a quick delivery, there was...

    Incorrect

    • A 23-week-old neonate was delivered in triage following a quick delivery, there was no time for the administration of antenatal steroids. The baby was successfully intubated, and the first dose of surfactant had been given. Despite good chest wall movement and 2 min of chest compressions the heart rate remains very slow. The baby is approximately 18min old when the consultant arrives. Which of the following is the most appropriate next step in the management of this case?

      Your Answer:

      Correct Answer: Consider reorientating care

      Explanation:

      The withdrawal and withholding of care has been reported in neonatology for over 30 years. ‘Withdrawal’ of life-saving treatment implies the elective discontinuation of ongoing life-supportive measures. ‘Withholding’ of treatment occurs when interventions necessary for immediate survival, such as surgical intervention or resuscitation (bag and mask/endotracheal ventilation, cardiac massage or adrenaline) are deliberately not administered.Withdrawal of treatment most commonly takes the form of withdrawing of ventilatory support. In the extremely premature neonate who has had poor response to bag and mask ventilation, this may mean stopping ventilation breaths, explaining to the parents the reason for discontinuation and offering comfort care according to the parents’ wishes. Parents should be prepared for the sequelae that may follow withdrawal from mechanical ventilation. The infant may become agitated secondary to hypoxia, display terminal gasping and, depending on the size and gestation of their infant, tachypnoea, intercostal recession or stridor may be evident. Parents may want to be involved in the extubation process. Following ventilator withdrawal, the length of time prior to death cannot be predicted and can be a significant cause of parental anguish if this is not explained. A side room and privacy should be made available for the family. NICE guidance suggests that women ‘should not be encouraged to hold their dead baby if they do not wish to’, although this is very much dependent on individuals.

    • This question is part of the following fields:

      • Neonatology
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  • Question 20 - Which of the following is incorrect? ...

    Incorrect

    • Which of the following is incorrect?

      Your Answer:

      Correct Answer: The embryonic phase is the first 20 weeks in utero

      Explanation:

      The embryonic period comprises of the first 8 weeks of pregnancy. It is divided into a preembryonic phase (from the 1st to the 3rd week), in which the three germinal layers arise, and into the embryonic phase proper (from the 4th to 8th week), in which the embryonic organ anlagen arise.

    • This question is part of the following fields:

      • Neonatology
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