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MCQs in Pediatrics Review of Nelson Textbook of Pediatrics – 20th edition. This is question bank based on textbook Nelson Pediatrics. It helps. Nelson Pediatrics Review (MCQs) 19ed. for a late-component or alternative pathway deficiency with CH50 and AP50 Nelson pediatrics review (mcqs) 19ed Watch This Video ○○○ caite.info LATEST nelson PEDIATRICS review MCQs 19 ed pdf pdf free download for freshers experienced students objective books interview questions mcqs lab viva.
Inhaled nitric oxide NO has shown promise in managing respiratory hypoxic failure after burns. This is a controversial point in the discussion of brain death. Various combinations of these clusters also occur. If hypotension is severe and prolonged. Findings on physical examination are normal except for urticaria. Because of high airway resistance.
Renal dysfunction in shock of any type may be due to prerenal or renal etiologies. The patient's preferences Standard order sets Attempts to normalize the blood gases Question. If no intrinsic renal injury occurs.
Hypoxia and hypercarbia will require different strategies. Low rates. Which of the following statements regarding continuous venovenous hemofiltration CVVH is true? Acute tubular necrosis is often reversible. If hypotension is severe and prolonged. The differential diagnosis for the afebrile child with nausea and vomiting should include: Intracranial tumor Explanation: Nausea and vomiting are common complaints in a pediatric practice. Circuit patency is dependent on systemic blood pressure Water and molecules less than Although most are due to non-specific presumed viral infections or other identifiable infections gastroenteritis.
If gastrointestinal motility is normal. Appropriate nutritional support for the child receiving mechanical ventilation for bacterial pneumonia should include: Amino acids. In the severely ill child. This later metabolic problem is often a risk factor for increased morbidity and mortality in an ICU. Removal of the molecules helps treat azotemia but is of value in removing inflammatory cytokines in patients with the systemic inflammatory response syndrome.
See Chapter 57 Question. Which of the following statements is true? Administration of growth hormone diminishes insulin resistance Hyperglycemia is a beneficial state in the catabolic child Branch-chain amino acids are beneficial to the previously normal child Intensive insulin therapy has reduced mortality in adult ICU patients Explanation: Insulin may reverse the catabolic state and prevent hyperglycemia.
An elemental formula via nasojejunal tube Question. This improves nitrogen balance but also reduces the risk of sepsis by lowering the incidence of transmucosal migration bacterial translocation of enteric bacteria.
This is a controversial point in the discussion of brain death. A yr-old boy is struck by a car while walking. On arrival in the ED. The controversy has not been resolved. In the child who has suffered a severe hypoxic ischemic injury. Always be cautious and.
CT scans do not reveal abnormalities until after 1 mo following injury Intracranial pressure monitoring improves outcome Question. Viral gastroenteritis Salmonella infection Type A influenza Question. Cortical brain death proponents want to recognize cortical death alone as the criterion for legal death. Fluid restriction and furosemide infusion Porcine hepatocyte column filtration Continuous hemofiltration plus plasma exchange Explanation: Various modalities have been proposed to treat hepatic encephalopathy.
Today we agree on the legal definition of whole brain death. Hemodialysis Question. What is the most appropriate next step in patient management? In addition to controlling intracranial pressure. Fulminant hepatic failure will lead to encephalopathy. The nature of the nausea.
Liver and kidney damage are irreversible Isolated brainstem function might be preserved Explanation: The brainstem may be preserved in the presence of cortical cell death.
Heart sounds are distinct. A 3-yr-old boy is brought to the ED after falling two stories from an open window. The most appropriate next step in patient management would be: Repeated boluses of Ringer lactate or normal saline solution have been life-saving if administered early and aggressively in pediatric patients with shock.
A 4-yr-old girl sustains a head injury after pulling a television set onto her. He arrives appropriately immobilized and is alert. Further clinical assessment in a stable patient before a head CT is quite appropriate.
The paramedics intubate her at the scene because of inadequate respiratory effort. Immediate head CT Hyperventilation to attain a PCO2 of 25 mm Hg Gentle hyperventilation and an intravenous bolus of mannitol Evaluation of level of alertness and pupil size and reactivity Explanation: At this point. His abdomen is diffusely tender. With time and signs of blood loss. It is clear that he has a femur fracture. Needle thoracentesis of the left hemithorax Explanation: Although an x-ray may be helpful for diagnosis and fluids may improve venous return.
Upright radiograph of the chest Pericardiocentesis Rapid intravenous bolus of Ringer's lactate Arterial blood gas analysis Question. PCO2 45 mm Hg pH 7. PCO2 60 mm Hg Explanation: This reflects the lung protection strategy with permissive hypercarbia. The most appropriate diagnostic study for this patient would be: Abdominal CT is now the standard for blunt pediatric abdominal trauma. Plain abdominal radiographs Question. For the child with ARDS receiving mechanical ventilation the prone position is alternated with the supine position.
A yr-old boy is brought to the ED after being kicked in the abdomen by a horse. PCO2 40 mm Hg pH 7. It is most useful for solid organs liver. PaO2 60 mm Hg. When available. PCO2 25 mm Hg pH 7. PaO2 mm Hg. There is no need for hyperoxia. In the support of the child with severe ARDS. PaO2 90 mm Hg. This measure is used to: Redistribute total body edema Equalize pulmonary blood flow to dependent and independent lung segments Prevent atelectasis and increased consolidation in dependent lung segments Explanation: Body position changes help to improve respiratory function in the previously dependent lung segments.
See Chapter 58 in Nelson Textbook of Pediatrics. Transplantation of part of an organ from a donor who will survive the donation process is possible with transplantation of: Heart or lung Heart or liver Cornea or heart Kidney or liver Explanation: Kidney transplantation is quite successful from a related living donor.
Use permissive hypercapnia Accept PaO2 measurements of mm Hg Reduce airway pressures to prevent barotrauma and volutrauma Question. Heart or kidney Question. Normalize blood gases Explanation: To avoid oxygen toxicity. Split or partial liver transplantation from a living related donor is also successful.
In the management of a child with ARDS and respiratory failure. Complications of transplantation particularly likely to be seen with bone marrow transplantationinclude: See Chapter 60 in Nelson Textbook of Pediatrics. Early in the posttransplantation period. Both procedures. Decrease the risk of pressure sores Question. See Chapter 58 in Nelson Textbook of th Pediatrics. Although PaO2 may improve with position changes. This principle of respiratory management has been the most significant improvement in the care of ARDS in the last 10 years.
A pulse is found after 10 min of resuscitative measures. A 2-yr-old boy is pulseless and apneic after extrication from a swimming pool. Maintenance of low urine output and maintenance of lower- than-normal blood pressure Maintenance of low urine output and maintenance of higher- than-normal blood pressure Question.
An intraosseous line is placed. One of the more common pathogens responsible for pulmonary infections in immunocompromised patients following transplantation is: Staphylococcus epidermidis Haemophilus influenzae Clostridium botulinum Streptococcus pneumoniae Pneumocystis carinii Explanation: Pneumocystis carinii pneumonia PCP is seen in any immunosuppressed patient. On arrival. Factors promoting successful acceptance of a transplanted kidney by the recipient include: Maintenance of high urine output and maintenance of lower- than-normal blood pressure Maintenance of high urine output and use of angiotensin- converting enzyme ACE inhibitors Maintenance of high urine output and maintenance of higher-than-normal blood pressure Explanation: It is essential to maintain renal perfusion and renal blood flow.
Forced diuresis is critical to achievement of this goal. The child is transferred to your ED. Prophylaxis against PCP has greatly reduced this potentially lethal complication. Paramedics start CPR. He is not breathing spontaneously. Which of the following interventions is most likely to improve the patient's neurologic outcome?
Administration of high-dose dextrose to improve cerebral metabolism Fluid restriction to minimize potential cerebral edema The addition of positive end-expiratory pressure. He was pulseless and apneic at the scene. Smith install a new swimming pool in their backyard. Fencing is the best preventive measure. See Chapter th 61 in Nelson Textbook of Pediatrics. The best approach is to support normal oxygenation and normal blood pressure to avoid continued cerebral hypoxia and ischemia.
Children under the age of yr cannot "swim" safely. Despite many hopeful new interventions for hypoxic-ischemic neurologic injury. Three months later. Which of the following safety measures would have been most likely to prevent this tragedy?
A lightweight plastic swimming pool cover A pool alarm that sounds when water movement is detected A 5-ft-tall chain link 2. PaCO2 35 mm Hg. On presentation to the PICU. A 4-yr-old boy is admitted to the PICU after resuscitation from a swimming pool submersion. The other interventions are dangerous A. His vital signs are normal and he is comatose. Monitor blood pressure to improve cerebral perfusion pressure and possibly to avoid secondary ischemic injury from raised intracranial pressure.
His initial arterial blood gas is pH 7. See Chapter 61 in Nelson Textbook of Pediatrics. In counseling the family regarding the child's prognosis. Swimming lessons Question. See Cha Question. His condition continues to deteriorate. A growth chart. High-frequency ventilation should also be used before ECMO.
See Chapter 62 in Nelson Textbook of Pediatrics. Hyperbaric oxygenation is of value in severe carbon monoxide poisoning. Therapy is usually started at 5 ppm and titrated to 30 ppm. Inhaled nitric oxide NO has shown promise in managing respiratory hypoxic failure after burns.
Which of the following is the best method for estimating body surface area burn? Body surface area BSA of various anatomic sites changes with growth and development. Hyperbaric oxygen Question. A 5-yr-old girl who accidentally spilled hot water on her face and trunk and is brought to the emergency room.
Although his initial clinical appearance sounds ominous. Of the following. Serial examination is the best method to follow the course and predict the outcome. The head has a greater body surface area in the youngest children.
Penicillin is not an immediate therapy. Boluses may be added to preemptive therapy Question. Which of the following regimens will provide the best pain management? Grafting follows. Aggressive use of topical antibacterial agents with frequent dressing changes Use of intravenous appropriate antibiotics Excision of the burn wounds and grafting Explanation: To prevent infection and to facilitate healing.
Use of topical analgesics Question. Preemptive narcotics before the procedure and an anxiolytic are the best ways to avoid future behavioral problems and to provide appropriate pain relief. See Chapter 62 in Nelson Textbook of th Pediatrics.
Initial treatment is to remove the clothing and place warm saline dressings over the wound. A yr-old boy spilled gasoline on his legs. All of the following statements about brain death are true except: Brain death alone can be used as a justification for withdrawing all life support. See Chapter 64 in Nelson Textbook of Pediatrics. Declaration of brain death in children younger than 2 mo of age requires two assessments.
This remains objectionable because of the rare patients who wake up from PVS. With further experience. Radionuclide angiogram Apnea test Cerebral angiogram Question. Ancillary tests to confirm brain death include all of the following except: Electroencephalogram Head ultrasound study Explanation: Head ultrasonography demonstrates structural problems. Doppler sonography may be of value. It may take some time. None of the above. Acceptance of brain death is sufficient to declare full legal death and should facilitate more organ donation opportunities Question.
Some suggest that neocortical brain death should be used. Correct performance of an apnea test requires: The apnea test helps define absent brain stem reflexes related to respirations. With whole brain death.
Risks associated with the use of succinylcholine include all of the following except: Akinesia is: Analgesia Sedation Absence of movement Explanation: It simply means no movement. See Chapter 65 in Question. Amnesia Nystagmus Question. Use before. See Chapter 65 in Nelson Textbook of Pediatrics. Avoid hypoglycemia Prevent acidosis Question.
Deep sedation is associated with all of the following except: Loss of airway protective reflexes Loss of airway patency Loss of cardiovascular stability Apnea Seizures Explanation: Most agents producing deep sedation will actually suppress physical and electrical seizure activity.
Fentanyl anesthesia in neonates does all of the following except: Fentanyl is an effective and safe analgesic anesthetic in newborns and has improved the outcome of complex surgeries in the neonatal period.
Deep sedation is a risk factor for a cardiopulmonary arrest and should only be performed in a setting equipped for response to apnea and bradycardia. Sedation and analgesia must also be used when a patient receives neuromuscular blocking agents. She is on oral morphine and is experiencing increased frequency of uncontrollable pain. Seizures Explanation: With much better and safer neuromuscular blocking muscle relaxants.
Hyperkalemia Malignant hyperpyrexia Myoglobinuria Elevated intracranial pressure Question. A yr-old girl with metastatic primitive neuroectodermal tumor PNET of the bone is a hospice patient. Malignant hyperthermia is associated with all of the following except: Narcotic agents are not associated with the development of this potentially serious and lethal familial condition. The spectrum may range from masseter spasms or a mild increase in intraoperative temperature and CO2 elimination to severe muscle rigidity.
The most appropriate response to her stated fear would be: Remind her that her daughter is terminally ill. There is a great difference between addiction and tolerance to the effects of an opiate.
Many people lay and medical believe in myths and don't understand opiate drugs. A positive family history for the disorder Question. Her mother is hesitant to change to intravenous morphine because of fear that her daughter will become addicted to the medication.
He describes the pain as sharp. When the patient described in Question 82 learns that the pain medication might be changed. Because she is afraid of needles Because she doesn't want to bother the nurse All of the above Explanation: The statement by a child who should be in pain that he or she no longer hurts often indicates a fear of something else. Other approaches to pain management that might be considered for the patient described in Question 82 include: Adjuvant analgesics Complementary interventions.
See Chapter 66 in Nelson Textbook of Pediatrics. He is able to walk on the leg and is attending school. A bright yr-old boy has had persistent right leg pain for 6 mo after a minor soccer injury has healed. This patient has classic symptoms of what used to be called reflex sympathetic dystrophy but is now called complex regional pain syndrome. Ask her to trust your judgment that this is the best route to take Question.
Results of all imaging studies are normal. Always be cautious about what children with chronic pain say. What is the likely reason she might have misled the nurse? Because she knows her mother is against the idea. The type of pain the patient is experiencing is most likely to be: Neuropathic Explanation: Neuropathic pain may be difficult to diagnose. Adding other non-opiate analgesic medications and using other non-drug related therapies are especially useful in children with poorly controlled chronic pain.
Some would also premedicate with a benzodiazepine. Unless the parents will create a stressful situation. Separation from her parents and a benzodiazepine Question. Crutches to eliminate weight bearing Home schooling to reduce stress All of the above Question.
Which of the following combination of interventions is most likely to be helpful to her? Education abut the procedure and hypnotherapy Oral sucrose and a pacifier Distraction and a local anesthetic Explanation: Local anesthesia plus distraction is a helpful combination for bone marrow aspiration. Low-dose tricyclic antidepressants. Other types of treatment that may be considered for the child described in Question 85 include: Physical therapy Explanation: Physical therapy is very useful in rehabilitation.
Indications for admission to the hospital after a burn injury may. Visceral Peripheral Question. A 3-yr-old girl is scheduled to have a bone marrow aspiration for the first time. Which type of medication should first be considered to treat the pain in the child described in Question 85? Neuropathic pain is quite resistant to opiates. It also teaches the patient to use the extremity without an increase in disability. Inhalation injury Question.
A 3-mo-old. All of the following statements are true except: Risk of postoperative apnea is increased by anemia Accepted standard of care includes overnight inpatient apnea monitoring for this infant after general anesthesia because of a significant risk of postoperative apnea Postponing the surgery incurs a small but real risk of incarceration. Anemia is an independent risk factor for apnea. The HMO clerk approves the surgery on an outpatient basis.
The risk of postoperative apnea decreases with age. Retrospective analysis of anesthetic risk supports delaying elective surgery in the first mo of life. If apnea does not occur in the first 12 hr. These risks must be balanced against findings of other studies that suggest that delay in repairing inguinal hernia may result in incarceration that cannot be reduced and in additional complications associated with more emergent surgery. The other choices indicate that the patient is at high risk and requires hospitalization.
Suspected child abuse Electric burns through an extremity Perineal burns Poor follow-up No tetanus immunization Explanation: Lack of immunization against tetanus may be managed with tetanus toxoid and if a wound is large or dirty with tetanus immune globulin. Former premature infants up to perhaps wk postconceptual age should be monitored for at least hr after anesthesia.
Postoperative severe apnea can occur in former premature infants up to roughly wk postconception.
The infant is feeding well and gaining weight and has no requirement for supplemental oxygen. The infant had received mechanical ventilation for the first 6 days of life and had apnea of prematurity that resolved 5 wk previously.
Although apnea is reported to be much less common after spinal anesthesia for inguinal surgery in formerly premature infants. Auscultation of the chest would most probably show rales.
Aspiration may occur. Although a rapid-sequence induction may reduce the likelihood of aspiration. Malignant hyperthermia is an inherited muscle disorder that produces acute hypermetabolism. All of the following are true except: Prior to your examining the patient. Clinically significant aspiration pneumonitis is comparatively uncommon in children after anesthesia and surgery but should be suspected if tachypnea. Anesthesia was maintained with halothane in a mixture of nitrous oxide and oxygen.
The most appropriate approach to management is to: Administer ceftriaxone IM after a blood culture and have the parent and child return to the office in the morning If the child is older than 6 mo. Untreated pain increases respiratory rates. You are called because the child has a fever of Atelectasis is the most common cause of fever immediately after surgery. The anesthesia record notes stable vital signs during surgery.
In response to social overtures being held. The nurse notes from the record that anesthetic induction was performed with thiopental sodium and succinylcholine. A previously healthy 7-mo-old white baby boy presents one summer day with a temperature of This 3-mo-old had pneumococcal meningitis.
The most likely diagnosis. In addition to observing color. Clinical observation of young patients is critical in helping you to evaluate and distinguish the degree of risk of infection and physiologic impairment. See Chapter 49 in Nelson Textbook of Pediatrics. The most appropriate initial therapy is: Despite an uncertain etiology.
Movement of a painful extremity. The circulation needs to be re- established to perfuse vital organs. Administer acetaminophen and reassess after the infant is no longer febrile Administer ceftriaxone after obtaining a blood.
After receiving normal saline pushes. He has a 1-day history of diarrhea five stools in 24 hr and is now unresponsive to verbal commands or painful stimuli.
Paradoxical irritability may be present with all of the following except: Paradoxic irritability is present when a child becomes anxious and cries during attempts to cuddle and hold the patient. After the lumbar puncture. He has been otherwise well prior to this hospital admission. Past medical history reveals surgery for closure of the spina bifida at age 3 days. Herpes simplex encephalitis Meningococcemia Salicylate poisoning Hemorrhagic shock encephalopathy syndrome Explanation: Hemorrhagic shock encephalopathy syndrome may look like heat stroke.
It has a high mortality rate and morbidity. Bananas may cross-. The presentation includes urticaria. The most likely diagnosis is: Ampicillin hypersensitivity Urosepsis Reactive airway disease Status epilepticus Latex anaphylaxis Explanation: Latex allergy is common in children with multiple surgical procedures and those who have required catheterization for urinary retention. See Chapter 57 in Nelson Textbook of Pediatrics. Malignant hyperthermia Question.
A burn wound characterized by the absence of painful sensation. He is on ampicillin prophylaxis for recurrent urinary tract infections since birth and has to be catheterized for urination.
A yr-old boy with spina bifida experiences respiratory distress during induction of anesthesia for an orthopedic procedure. This defines a full-thickness burn also known as a third-degree burn. Autosomal dominance inheritance is characterized by all of the following except:.
It affects males more commonly than females Explanation: All others are very typical of autosomal dominant inheritance. The sex ratio should be equal. Often the parents are not affected because the disease is a spontaneous mutation in the child. Neurofibromatosis is an example of an autosomal dominant disease. See Chapter 69 in Nelson Textbook of Pediatrics, 17th edition.
Autosomal recessive disorders are characterized by all of the following except:. Greater incidence than that of autosomal dominant disorders Explanation: Autosomal dominant disorders are more common because only one gene is needed for the disease to be manifested. In autosomal recessive disorders, two genes are needed.
The risk of an autosomal recessive disease in the offspring of a consanguineous mating between first cousins is:. This risk is twice the risk of an autosomal recessive trait being manifest in non-consanguineous mating.
X-linked dominant Explanation: This is the classic pattern of an X-linked dominant condition. These are rare conditions, which include hypophosphatemic rickets and incontinentia pigmenti IP. IP is lethal to the male fetus and thus no males are born alive with this disorder. See Chapter 69 in Nelson Textbook of Pediatrics, 17th ed. A disease passed on only through the mother and affecting both sons and daughters, with variable manifestations in the affected siblings, is most likely:.
See Chapter 63, page Often, coma is seen immediately after the injury and is sustained. In some cases, such as with an epidural hematoma, a child may be alert at presentation but the condition may deteriorate after a period of hours.
A similar picture can be seen in children with diffuse swelling, in whom a talk-and-die scenario has been described. Clinicians also should not be lulled into underappreciating the potential for deterioration of a child with moderate TBI GCS score with a significant contusion, because progressive swelling can potentially lead to devastating complications. In the comatose child with severe TBI, the second key clinical manifestation is the development of intracranial hypertension.
The development of brain swelling is progressive. Need for ICP-directed therapy may persist for longer than a week. A few children have coma without increased ICP, resulting from axonal injury or brainstem injury. Children with moderate to severe traumatic brain injury require intracranial pressure monitoring and treatment in the critical care unit to prevent progressive swelling. If neuromuscular blockade is needed, it may be desirable to monitor the EEG continuously because status epilepticus can occur; this complication will not be recognized in a paralyzed patient and is associated with raised ICP and unfavorable outcome.
Other first-tier therapies include the osmolar agents mannitol and hypertonic saline given in response to ICP spikes. If ICP remains refractory to treatment, careful reassessment of the patient is needed to rule out unrecognized hypercarbia, hypoxemia, fever, hypotension, hypoglycemia, pain, and seizures.
Repeat imaging should be considered to rule out a surgical lesion.
Guidelines-based second-tier therapies for refractory raised ICP are available, but evidence favoring a given second-tier therapy is limited. In some centers, decompressive craniectomy is used. Others use a pentobarbital infusion. Other second-tier therapies such as lumbar CSF drainage are options. See Figure ; also Chapter 63, page Which of the following statements regarding cooling treatment for perinatal hypoxic ischemic encephalopathy is not true?
Exclusion criteria have included coagulopathy, bleeding, and hemodynamic instability. According to the American Heart Association AHA guidelines predominantly for adults after a cardiac arrest when the initial event was associated with ventricular fibrillation , cooling should be initiated as soon as possible after return of spontaneous circulation but may be beneficial even if delayed hr ; it should be induced by means of surface cooling with cooling blankets; application of ice packs to the groin, axillae, and neck; use of wet towels; and fanning.
In perinatal asphyxia, cooling should be maintained for 72 hr. Shivering should be prevented with sedation and neuromuscular blockade. Temperature should be continuously monitored.
Hypothermia in children has been associated with an increased risk for neutropenia and sepsis. Therapy is adjusted for both symptoms and EEG evidence of seizures. As therapy escalates, continuous EEG monitoring should be considered to help titrate therapy. For refractory status epilepticus, newer therapies include mapping of the seizure focus followed by neurosurgical resection, IV lidocaine, or levetiracetam. Ischemic strokes in children are generally not the result of atherosclerotic plaque migration, as they are in adults.
Instead, damage to the intima of cerebral arteries can form a thrombotic nidus. In sickle cell disease, chronic turbulent blood flow likely leads to vascular damage. In intracerebral hemorrhage, blood vessel wall integrity is compromised, leading to extravasation of blood into the parenchyma or dural spaces.
The usual pathology in children with heart disease is embolism from diseased valves or intracardiac devices and right-to-left shunts that leads to cerebrovascular occlusion.
The most effective strategy to minimize any ventilator complication is regular assessment of extubation readiness and liberation from mechanical ventilation as soon as clinically possible. See Chapter 65, page The initial ventilator settings are determined by: Patients with severe forms of reactive airways disease e. Diseases associated with decreased time constants decreased static compliance, e. Diseases associated with prolonged time constants increased airway resistance, e.
Per the IOM, high-quality health care by definition must be: The Six Dimensions of Quality are effectiveness, efficiency, equity, timeliness, patient safety, and patient-centered care. The IOM emphasizes the concept that all Six Dimensions of Quality need to be met for the provision of high-quality health care. Health care that maximizes outcomes but is not efficient i.
Health care that is highly efficient but limits access also is not high quality. These concepts can be viewed as the overall value proposition—that is, the value created for a patient. Which of the following is a step in the rapid cycle of improvement PDSA? The PDSA cycle is typically aimed at testing small changes and then studying the results to plan and implement the next cycle of change i.
The PDSA cycle specifically requires that improvements be data driven. This is important because many clinicians attempt to make changes for improvement in their practice but do not emphasize the importance of data collection. Organizations need to foster a culture of learning in which each individual will feel accountable for ensuring a safe and quality program, communication is open, and teamwork is valued.
Reporting of errors should be valued, reports of adverse events should be handled confidentially, and those who report errors should be protected from discovery. Developing a culture of learning involves the compassionate and appropriate disclosure of system failures and medical errors to patients and families. Of the following, the most therapeutic approach is: To improve outcome, sequential excision and grafting of 3rd-degree and deep 2nd-degree burns is required in children with large burns.
Prompt excision with immediate wound closure is achieved with autografts, which are often meshed to increase the efficiency of coverings. See Chapter 68, page Which of the following regimens will provide the best pain management? Opiate analgesia, prescribed in an adequate dose and timed to cover dressing changes, is essential to comfort management. Anxiolytic medication added to the analgesic is usually helpful and has more than a synergistic effect.
Small 1st- and 2nd-degree burns of the hands, feet, face, perineum, and joint surfaces also require admission if close follow-up care is difficult to provide. Children who have been in enclosed-space fires and those who have face and neck burns should be hospitalized for at least 24 hr for observation for signs of central nervous system CNS effects of anoxia from carbon monoxide poisoning and pulmonary effects from smoke inhalation.
See Table , Chapter 68, page A burn wound characterized by the absence of painful sensation, bleeding, or capillary refilling is best classified as: The absence of painful sensation and capillary filling demonstrates the loss of nerve and capillary elements. The wound cannot epithelialize and can heal only by wound contraction or skin grafting.
Which of the following statements regarding predictive genetic testing is true? A major caution with predictive testing is that the presence of a gene mutation does not necessarily mean that the disease will develop. Many of the disorders with age-dependent penetrance display incomplete penetrance. A person who inherits a mutation might never develop signs of the disorder. In the USA, the Genetic Information Nondiscrimination Act of protects individuals from genetic discrimination at the hands of health insurers and employers but does not extend protection against discrimination from providers of life, disability, or long-term care insurance.
See Chapter 72, page Genetic counseling is indicated in which of the following clinical scenarios? Which statement regarding treatment of genetic disorders is NOT true?
The underlying defect itself is not altered by treatment. Physiologic therapies are used in the treatment of inborn errors of metabolism. Physiologic treatments can be highly effective, but they usually need to be maintained for a lifetime because they do not affect the underlying genetic disorder. Many of these treatments are most effective when begun early in life before irreversible damage has occurred. This is the rationale for comprehensive newborn screening for inborn errors of metabolism.
Which statement regarding genetic disorders of metabolism is NOT true? This differentiates these infants from those who appear sick at birth due to birth trauma, intrauterine insults, chromosomal abnormalities, or other genetic diseases.
Severe forms of genetic disorders usually become clinically apparent in the newborn period or shortly thereafter. The majority of conditions are inherited as autosomal recessive traits. Most of the genetic metabolic conditions can be controlled successfully by some form of therapy, and a few can be potentially cured by the use of bone marrow or liver transplants.
This underlines the importance of early diagnosis, which can be achieved through screening of all newborn infants. A large number of genetic conditions can be identified by this method. See Chapter 78, page In the newborn period, the clinical findings are usually nonspecific and similar to those seen in infants with sepsis. A genetic disorder of metabolism should be considered in the differential diagnosis of a severely ill newborn infant, and special studies should be undertaken if the index of suspicion is high.
Signs and symptoms such as lethargy, poor feeding, convulsions, and vomiting may develop as early as a few hours after birth. See Chapter 78, page and Fig. Initial laboratory studies to investigate for metabolic disease in an ill infant should include: An elevated blood ammonia level is usually caused by defects of urea cycle enzymes. Infants with elevated blood ammonia levels from urea cycle defects commonly have normal serum pH and bicarbonate values; without measurement of the blood ammonia level their defect may remain undiagnosed and they may succumb to their disease.
Elevation of serum ammonia levels also is observed in some infants with certain organic acidemias. These infants are severely acidotic because of accumulation of organic acids in body fluids. When blood ammonia, pH, and bicarbonate values are normal, other aminoacidopathies e.
A 2 day old boy manifests poor feeding, vomiting, and lethargy leading to coma. Laboratory data reveal respiratory alkalosis and hyperammonemia. The urine orotic acid level is also elevated.
The most likely diagnosis is: In neonatal hyperammonemia, most of the symptoms are related to brain dysfunction due to the elevated ammonia. The affected infant is normal at birth but becomes symptomatic within a few days of protein feeding. Refusal to eat, vomiting, tachypnea, and lethargy can quickly progress to a deep coma.
Convulsions are common. See Figure for an algorithm to diagnose the cause of hyperammonemia. In the case of OTC deficiency, laboratory studies will not demonstrate acidosis. A marked increase in urinary orotic acid distinguishes OTC deficiency from other disorders.
See Figure in Chapter 79, page For most lysosomal storage disorders, carrier identification and prenatal diagnosis are available; a specific diagnosis is essential to permit genetic counseling.
Which of the following disorders is X-linked? See Chapter 80, page It most frequently presents in the first 6 mo of life with developmental delay followed by progressive psychomotor retardation and the onset of tonic-clonic seizures. A typical facies is characterized by low-set ears, frontal bossing, a depressed nasal bridge, and an abnormally long philtrum. Hepatosplenomegaly and skeletal abnormalities similar to those of the mucopolysaccharidoses, including anterior beaking of the vertebrae, enlargement of the sella turcica, and thickening of the calvarium, are present.
A 4 mo old girl presents with developmental delay, an exaggerated startle response to loud noise, and macrocephaly. On physical examination, the child has decreased eye contact and a cherry-red spot in each retina. The clinical manifestations of Sandhoff disease are similar to those for Tay-Sachs disease. The diagnosis of infantile Tay- Sachs disease and Sandhoff disease is usually suspected in an infant with neurologic features and a cherry-red spot.
Affected infants usually develop normally until 4 to 5 mo of age when decreased eye contact and an exaggerated startle response to noise hyperacusis are noted. Macrocephaly, not associated with hydrocephalus, may develop. In the 2nd yr of life, seizures develop that may be refractory to anticonvulsant therapy. Neurodegeneration is relentless, with death occurring by the age of 4 or 5 yr. A 15 yr old presents with chronic fatigue and severe bone pain of 1 year's duration. He has hepatosplenomegaly and a normal retinal examination.
Laboratory studies reveal normocytic anemia and thrombocytopenia. Radiographs of the distal femur reveal Erlenmeyer flask deformities. Clinical manifestations of type 1 Gaucher disease have a variable age at onset, from early childhood to late adulthood, with most symptomatic patients presenting by adolescence. At presentation, patients may have bruising from thrombocytopenia, chronic fatigue secondary to anemia, hepatomegaly with or without elevated liver function test results, splenomegaly, and bone pain.
Most patients develop radiologic evidence of skeletal involvement, including an Erlenmeyer flask deformity of the distal femur. A 14 mo old girl presents with irritability, poor walking, genu recurvatum, and hypotonia.
Physical examination reveals absent deep tendon reflexes. Laboratory data reveal an increased cerebrospinal fluid protein level and decreased nerve conduction velocities. The clinical progression of the disease relates to the pathologic involvement of both central and peripheral nervous system, giving a mixture of upper and lower motor neuron and cognitive and psychiatric signs. Deep tendon reflexes are diminished or absent.
Gradual muscle wasting, weakness, and hypotonia become evident and lead to a debilitated state. As the disease progresses, nystagmus, myoclonic seizures, optic atrophy, and quadriparesis appear, with death in the 1st decade of life. On evaluation, decreased nerve conduction velocities, increased cerebrospinal fluid protein, metachromatic deposits in sampled segments of sural nerve, and metachromatic granules in urinary sediment are all suggestive of the disorder.
A 15 yr old Ashkenazi Jewish girl is seen because of chronic fatigue. On examination, she seems pale and thin and has a somewhat large abdomen. Her spleen is felt in the iliac fossa. She is mentally alert and has a history of normal development and normal school performance. Her blood cell count shows hemoglobin, 9.
Gaucher disease should be considered in the differential diagnosis of patients with unexplained organomegaly, who bruise easily, have bone pain, or have a combination of these conditions. Evaluation may show bruising from thrombocytopenia, chronic fatigue secondary to anemia, hepatomegaly with or without elevated liver function test results, splenomegaly, and bone pain.
A 7 mo old boy has been healthy and developing normally since birth. His mother now reports that he has decreased eye contact with her, even during feedings. The infant also startles very easily when there is a loud noise in the house. Of the following, the most appropriate diagnostic test to confirm the etiology of these findings is the measurement of: A 3 yr old boy was normal at birth but developed progressive coarsening of the face and developmental delay.
In addition to coarse facies and mental retardation, the physical examination reveals a cardiac systolic regurgitant murmur, hepatomegaly, joint stiffness, and short stature. CT scan reveals hydrocephalus. There is no corneal clouding.
All affected family members have been boys. It has been observed in a few females and this is explained by skewed inactivation of the X chromosome carrying the normal gene. Patients with severe MPS II have features similar to those of Hurler disease except for the lack of corneal clouding and the somewhat slower progression of somatic and central nervous system deterioration. Coarse facial features, short stature, dysostosis multiplex, joint stiffness, and mental retardation manifest between 2 and 4 yr of age.
See Chapter 82, page Acute cardiomyopathy has been found in some infants younger than 1 yr of age.
Inheritance is autosomal recessive. According to the International Classification of Diseases, major causes of U. The lowest neonatal mortality rate occurs in infants of mothers who receive adequate prenatal care and who are yr of age. Pregnancies in both teenagers and women older than 40 yr, particularly primiparous women, are at increased risk for intrauterine growth restriction, fetal distress, and intrauterine death.
A short interpregnancy interval is associated with increased risk. Oligohydramnios becomes most evident after 20 wk of gestation, when fetal urination is the major source of amniotic fluid. Rupture of the membranes is the most common cause of oligohydramnios and must be ruled out if oligohydramnios is suspected, especially if a normal-sized bladder is seen on fetal ultrasound evaluation.
Oligohydramnios causes fetal compression abnormalities such as fetal distress, clubfoot, spadelike hands, and a flattened nasal bridge. The commonest cause of Enuresis in children is a Urinary tract infection b Spina bifida c Psychologic stress d Diabetes mellitus. The upper segment: Lower segment ratio at 2 years of age in a normal child is a 1.
Which one is not unfavorable for fetal development a Herpes b Rubella c Alcohol d Tetracycline. A three year old child can do which of the following except a Ride a tricycle b Build a tower of ten cubes c Knows his age and sex d Use scissors to cut out pictures e Count three objects correctly.
Average birth weight of Indian child is a 2. Taste perception of baby develops at a Birth b 4 months c 6 months d 8 months. Increase is length in the first year of life is a 12 cms b 15 cms c 25 cms d 35 cms. A child with mental age of seven and a chronological age of seven years has an I.
Milestones at 10 months of age are a Pincer grasp b Arranging 3 cubes c Walks few steps without support d Says mama dada.
Child changes Rattle from one hand to another at the age of a 3 months b 6 months c One year d 2 years. IQ Intelligence quotient of a child means a The creative efficiency of the child b The capability of the child to perform intellectual tasks in relation to other children of same age c The efficiency of memory of child d Qualification of the learning ability of child.
In asymmetrical IUGR which organ is not affected? A two year old child was brought to the OPD by his parents with complaints of not eating anything On amination her weight is Which of the following acts can a one year old child perform? Prenatal diagnosis is possible for all of following except a Sickle cell trait b Beta Thalassemia c Ectodermal dysplasia d Duchennes muscular dystrophy. A 7 year old boy with reduced height and weight for age for past one year is likely to have a Malnutrition b Lymphoma c Chronic infection d Measles.
Intra uterine growth retardation can be caused by all except a Nicotine b Alcohol c Propranolol d Phenothiazine. Which of the following childhood disorder improves with increase in age a Conduct disorder b Emotional problems c Temper tantrum d Sleep disorder. A normal infant sits briefly leaning forward on her hands, reaches for and grasps a cube and transfers it from hand to hand. She babbles but cannot wave bye-bye nor can she grasp objectives with the finger and thumb.
Her age is a 4 months b 7 months c 10 months d 14 months. Atavism is when a child resembles his a Parents b Grand parents c Cousin d Twin. Two carpal bones are radiologically seen in the wrist X-ray of most of the children by the end of a 1 year b 2 years c 3 years d 4 years.
An infants sits with minimal support, attempts to attain a toy beyond reach, rolls over from supine to prone position and does not have pincer grasp is at a development of a 3 months b 4 months c 6 months d 8 months. Vitamin K deficiency occurs in the following except a Biliary obstruction b Artificial milk feeds c Oral antibiotic Therapy d Malabsorption. Breast feeding is best for the baby a But has no effect on the mother b But is likely to affect the mothers appearance significantly c But may cause breast cancer in the mother d And is also good for the mother.