Il lattante apparentemente sano ma con tachipnea

1. Il lattante apparentemente sano ma con tachipnea Carlo Capristo DIPARTIMENTO DELLA DONNA, DEL BAMBINO E DI CHIRURGIA GENERALE E SPECIALISTICA

2. In the nursery • Tachypnea and cyanosis in the newborn are frequently encountered problems in the nursery. • The incidence of respiratory distress • in newborns ranges from 2.9% to 7.6%. • 4.3% of newborns may require supplemental oxygen therapy. Chest radiographs in a premature infant with respiratory distress syndrome before and after surfactant treatment.  • Hjalmarson O. Epidemiology and classification of acute neonatal respiratory disorders. Acta Paediatr Scand 1981;70:773– 83. • Hjalmarson O. Epidemiology of neonatal disorders of respiration. Int J Technol Assess Health Care 1991;7(Suppl):9–16. • Kumar A, Bhat V. Epidemiology of respiratory distress of newborns. Indian J Pediatr 1996;63:93– 8.

3. The baby is well but…………….. • noticed by: • the parents, • the grandparents, • the family pediatrician • occasionally noted in the • postnatal ward. It is not uncommon to see babies in clinic who have been referred because they are persistentlytachypnoeic. ( notacutelyunwell )

4. Physiology of breathing • breathing begins in utero as early as 11 weeks • at birth huge physiological changes • this is in contrast to human adults with well developed ventilatory control system who demonstrate sustained hyperventilation • in response to hypoxia. • up to 6 months of age hypoxic ventilatory response in term infants shows considerable variability with respect to ventilation and arousal responses with an initial period of augmentation in ventilation followed by a sustained • reduction in ventilation similar to • or below normoxic ventilation. ventilatory control system MacLean JE, Paediatr Respir Rev. 2015 Aug 14.

5. Minute Ventilation in Infancy . Minute ventilation (V) = respiratory rate (RR) X tidal volume (VT) is related to metabolic rate. to respond to increased metabolic demand, minute ventilation can be increased by: • increasing the RR, • increasing the VT, • or both.

6. Minute Ventilation in Infancy . Minute ventilation (V) = respiratory rate (RR) X tidal volume (VT) is related to metabolic rate. to respond to increased metabolic demand, minute ventilation can be increased by: • increasing the RR, • increasing the VT, • or both. In the newborn and young child, increasing the respiratory rate (rather than tidal volume) is the most energy-efficient strategy to cope with higherventilatoryneeds. Ross KR. ClinChestMed. 2014;35:457.

7. Respiratory rate in 1st year of life, awake (solid line) and asleep (dashed line) showing 5th, 50th and 95th centiles.Rusconi F, Pediatrics 1994;94:350–5. The normalrespiratory rate gradually decreases over the 1st year There is no gender difference and the rate is higher in awake babies

8. IS THE BABY TACHYPNOEIC? • Measuring the respiratory rate is not always easy, especially if the baby is moving or crying; both these changethe rate, and the state of the baby (especially awake vs asleep) should be recorded alongside the rate. • Itiseasier to use a stethoscope to listen for breaths rather than simply watching the baby’s chest. Auscultation usually gives a higher rate, presumably due to missed breathsfromobservation alone. • The rate needs to be measured over a full minute • (or the sum of two sets of 30 s), and will need to be repeated a few times if it is variable. 30s + 30s Not x2

9. Respiratory rate and pneumonia in infancy Berman S, Arch Dis Child. 1991;66:81-4 • The respiratory rate (breaths per minute) thresholds are set at: • - 60 for infants < 2 months of age, • - 50 for infants from 2 to 12 months, • - 40 for children aged 1 to 5 years. • WHOrecommends a repeat respiratory rate count for infants under 2 months of age when the initial count is 60 or higher.

10. Respiratory rate and pneumonia in infancy Berman S, Arch Dis Child. 1991;66:81-4 • The respiratory rate (breaths per minute) thresholds are set at: • - 60 for infants < 2 months of age, • - 50 for infants from 2 to 12 months, • - 40 for children aged 1 to 5 years. • WHOrecommends a repeat respiratory rate count for infants under 2 months of age when the initial count is 60 or higher. a repeat count 45 to 60 minutes later will be less than the threshold in more than two thirds of the patients with an upper respiratory infection in contrast to patients with lower respiratory infection who will usually maintain a raised respiratory rate. =

11. Meaning of Respiratory Rate • Although respiratory rate is a useful predictor of lower respiratory infection in young infants, it does not correlate well with hypoxia. • The presence of subcostal retractions • is a more useful predictor of hypoxia • expecially if associated with nasal flaring, • refusal to eat, and abdominal distention. • Berman S, PediatrEmerg Care. 1990;6(3):179-82. • Berman S, Arch Dis Child 1991;66:81–4. RR ≠ SaO2 ≈ SaO2

12. IS THE BABY UNWELL? Certain symptoms elicited from the history at this stage are red flags for concern of a significant underlying condition: ▸ Persistent cough ▸ Apnoeic episodes ▸ Noisy breathing ▸ Poor feeding ▸ Vomiting ▸ Choking when drinking Consider also the possibility of a foreign body when an older sibling may have put something in the baby’s mouth.

13. IS THE EXAMINATION NORMAL? • The child’s weight, height and • head circumference will give an indication • of faltering growth which may be secondary • to tachypnoea or an underlying condition.

14. IS THE EXAMINATION NORMAL? Clinical examination is important, with some red flag warning signs: ▸ Respiratory distress ▸ Cyanosis, paleness ▸ Cardiac murmur ▸ Abnormal femoral pulses ▸ Hepatomegaly ▸ Hypotonia • intercostal and subcostal recession, • tracheal tug, • use of accessory muscles, • grunting, • nasal flaring. Some well babies with tachypnoea have a degree of recession as the only other clinical sign. Balfour-Lynn IM, Arch Dis Child. 2015;100(8):722-7.

15. IS THE EXAMINATION NORMAL? normal • Obviously the presence of crackles, wheeze • and stridor is important for the diagnosis. • Paradoxical breathing, an inward movement • of the chest wall during inspiration, • often with a seesaw thoracoabdominal, • motion must be looked for • (neuromuscular disease, • diaphragmatic abnormality, • upper airway obstruction).

16. IS THE EXAMINATION NORMAL? • The shape of the thorax is checked to ensure normal dimensions: • very small in asphyxiating thoracic dystrophy, • bellshaped in pulmonary hypoplasia.

17. IS THE EXAMINATION NORMAL? • The shape of the thorax is checked to ensure normal dimensions: • very small in asphyxiating thoracic dystrophy, • Barrel-shaped chest is frequently seen in post-term infants who have meconium aspiration syndrome

18. IS THE EXAMINATION NORMAL? • The patency of the nose must be examined to exclude choanalstenosis or unilateral or bilateral atresiaholding the stethoscope over the nostrils • (it may be necessary to pass a nasogastric tube down both sides). • The presence of persistent rhinitis in infancy should make one consider primary ciliarydyskinesia. choanal atresia

19. IS THE EXAMINATION NORMAL? Examinationfocused specifically on the cardiovascular system is also important and often neglected. • Cardiac apex may be visibly displaced (> 5° intercostal space) if there is cardiomegaly. • Precordial palpation may reveal a • right-sided impulse in Scimitar syndrome, • or complex forms of congenital heart • diseaseassociated with atrial isomerism • (visceral heterotaxy or a mirror image • arrangement in primary ciliarydyskinesia). 1 2 3 4 5 Scimitar syndrome, characterized by anomalous venous return from the right lung (to the systemic venous drainage, rather than directly to the left atrium

20. IS THE EXAMINATION NORMAL? • A prominent precordial impulse is an • important sign of cardiovascular disease • with pressure or volume overload of the • heart (valvular diseases, septal defects…). • Femoral pulses which are weak or • difficult to feel (coarctation of the aorta), or • generally weak pulses (myocarditis, dilated • cardiomyopathy, severe aortic stenosisor • other forms of left heart obstruction) can be important signs. • Liver enlargement is usually a manifestation of heart failure, but can be palpable if it is displaced by overinflated lungs.

21. IS THE EXAMINATION NORMAL? • Cyanosiswouldbevisibleif the deoxygenated hemoglobin content is > 3 g% (3 g per 100 mL). • Ifcyanosis is present throughout the body, including the mucous membranes and tongue, it is called central cyanosis. • If cyanosis is limited to the extremities, it is called peripheral cyanosis, also known as • acrocyanosis. • exposure to cold, • polycythemia.

22. IS THE EXAMINATION NORMAL? peripheral cyanosis is seen: • in conditions in which the infant is exposed to a cold environment, • it could also be the presenting sign of serious conditions such as: • sepsis • hypoglycemia • hypoplastic left-heart syndrome • (decreased peripheral perfusion) hence diffuse mottled, bluish-gray appearance of this infant's skin suggestive of systemic poor perfusion Peripheral cyanosis should not be ignored unless other conditions have been ruled out.

23. IS THE EXAMINATION NORMAL? • Abdominal examination is done to exclude an enlarged liver or spleen, or an abdominal mass. • A basic neurological examination is done, particularly for hypotonia which may indicate a neuromuscular disorder. • Always listen over the skull and particularly the occiputfor the murmur of a cerebral arteriovenous fistula, whose only clinical manifestation might be tachypnoea. Iizuka Y, Neuroradiol J. 2011;24(5):772-8

25. Newborn pulse oximetry screening is not just for heart defects. Meberg A. Acta Paediatr. 2015 Sep;104(9):856-7. • A Norwegian population-based (n=50.008) prospective multicenter study of postductal (foot) arterial oxygen study on the first day of life reported a SpO2 < 95% in 0.65% of the infants and 41% of these were, in fact, potentially severe extra-cardiac disorders, such as: • systemic infections – including group B streptococcal septicaemia – • amniotic fluid aspiration, • pulmonary hypertension and • pneumothorax. • Meberg A, J Pediatr 2008;152:761–5. (severe polycythemia) (healthyinfants)

26. Chest Radiography

27. Chest Radiography • The expansion of lungs • (lung volume) on both sides should be checked. • Normal inspiratory films should have 8 intercostal spaces of lung fields on both sides. 1 1 2 2 3 3 4 4 5 5 6 6 7 7 8 8

28. Chest Radiography • The locations of stomach, liver, and heart should be determined to rule out dextrocardiaand situsinversus.  stomach

29. POTENTIAL CAUSES OF TACHYPNOEA WHEN THE DIAGNOSIS IS NOT IMMEDIATELY OBVIOUS • Often the tachypnoea is just one of several signs in a clearly unwell child • a metabolic acidosis • bacterial sepsis; or • significant pain, • for example, from a volvulus. • In some children, • the cause becomes • more clear • from the history • narcotic withdrawal • postbirth asphyxia. • In some, it may be • determined • on first examination • small thorax of Jeune asphyxiating thoracic dystrophy, • bell-shaped chest from pulmonary hypoplasia, • hypotonia associated with neurological or neuromuscular disease, • stridor due to upper airway obstruction • severe neonatal rickets.

30. POTENTIAL CAUSES OF TACHYPNOEA WHEN THE DIAGNOSIS IS NOT IMMEDIATELY OBVIOUS • Respiratory and upper airway • Infection • Cystic fibrosis • Interstitial lung disease • Congenital thoracic malformations • Pulmonary hypoplasia • Congenital diaphragmatic hernia • Diaphragmatic weakness • H-type tracheo-oesophageal fistula • Nasal obstruction

31. Infection. nevertheless, low grade or chronic infection must be considered. • It is likely the child would • have other symptoms and signs • fever • cough • shallow breathing may be helpful • a nasopharyngeal aspirate for respiratory viruses • a cough swab for bacterial culture, • a white cell count, C reactive protein, procalcitonin • Pulmonarytuberculosishasbeendescribed in children under 1 year, with tachypnoea the predominant sign.Arikan-Ayyildiz Z, Turk J Pediatr 2011;53:250–4. Chest radiograph shows cavity within consolidation in the right lobe.

32. Cystic fibrosis babies are diagnosed with cystic fibrosis (CF) at around 3–4 weeks of age newborn screening however screening is not infallible • Most babies are symptom-free from the respiratory perspective, • the majority have suboptimal weight gain, often with abnormal stools. • Infants can present with tachypnoea, recurrentcoughand sometimes • wheeze, but our experience is that itis most unusualfor tachypnoea to be the sole symptom in a baby with CF. • A sweat test must be considered.

33. Interstitial lung disease • crackles, • recession, • hypoxaemia, • eventualy • failure to thrive. a heterogeneous group of rare diseases most often accompanied by: Kuo CS, Curr Opin Pediatr 2014;26:320 in some infants , tachypnoea is the only obvious symptom, and the severity of symptoms and signs varies. Neuroendocrinecellhyperplasia, previouslyknown as persistent tachypnoea of infancy • often associated with: • recession, • crackles • and sometime hypoxia. Deterding RR, Pediatr Pulmonol 2005;40:157–65

34. European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Bush A, Thorax. 2015;70(11):1078-84. THE STARTING POINT: WHEN TO SUSPECT CHILD—TYPICAL PRESENTATIONS Shortlyafter birth The earliest presentation of chILD is shortly after birth, with unexplained respiratory distress in a term baby. This frequently and rapidly proceeds to intubation and ventilation, with relentlessly progressive respiratory failure leading to death or lung transplantation, if the latter is available. First 2 years of life Later presentations of chILD are very non-specific and symptoms in descending order of frequency are:fast breathing, failure to thrive, typically dry cough, and wheeze in 25% ofcases in the absence of respiratory tract infection. X X X X X

35. Staging the severity of childhood interstitial lung disease (chILD) (awake) Pediatric interstitial lung disease revisited. Fan LL, Pediatr Pulmonol 2004;38:369–78.

36. European protocols for the diagnosis and initial treatment of interstitial lung disease in children. Bush A, Thorax. 2015;70(11):1078-84. SURGICAL LUNG BIOPSY procedure mustonly be undertaken by an experienced surgeon, who is confident of obtaining adequate biopsies (at least 10×10×10 mm); a very superficial biopsy, which does not contain distal airways, may result in diagnosticerror.

37. Interstitial lung disease • Surfactant protein B deficiency is lethal in neonates so is unlikely to present with just tachypnoea. • Inherited surfactant protein C deficiency, • Tachypnoea and other signs of respiratory distress are likely to be present.Thouvenin G, Arch Dis Child 2010;95:449–54. • A chest CT scan can be highly suggestive, although diagnostic certainty may require a lung biopsy, and gene mutation studies. chestradiograph and computed tomography scan of a patient at 18 months showing diffuse ground glass pattern and interstitial thickening.

38. OUTCOME in the absence of an underlying condition • tachypnoea usually resolves, • but may take as long as 6–12 months. this means that the cause in most babies is never determined. • the improvement may be due to: • maturation of automatic control of • breathing (based in the brainstem), • 2) response to chemoreceptors, or • 3) a change in the mechanical properties • (compliance and volumes) of the lungs • and chest wall.

39. OUTCOME in the absence of an underlying condition • tachypnoea usually resolves, • but may take as long as 6–12 months. this means that the cause in most babies is never determined. • the improvement may be due to: • maturation of automatic control of • breathing (based in the brainstem), • 2) response to chemoreceptors, or • 3) a change in the mechanical properties • (compliance and volumes) of the lungs • and chest wall. In those with a specific diagnosis, the outcome will depend on the actual diagnosis.

40. Summary & Conclusions • Respiratory rate hastobecountedwith the stethoscope (for 30 sec. twicethanadd) while the childisasleep. • Tachypnoea (> 60 breaths/min in the first monthsof life) maybeanself-solvingproblembut can alsobe a signof a severe conditionsexpeciallyifassociatedwithhypoxia, failuretothrive, and cardiacsigns and symptoms. • Persistent cough, Apnoeic episodes, Noisy breathing, Poor feeding, Vomiting, Choking when drinking are signs for concern.

41. Summary & Conclusions • If the clinicalexaminationisnotcompletely negative imediateinvestigations are: SaO2 and ChestX-ray, followedbyECG & Echo, and bloodgases. • When the diagnosisisnot immediate obviousreferalisnecessary for: CT scan, bronchoscopy & biopsy, and genetics. • Whenyour are facedwith a tachypnoeicinfantitwillbewisetomaintain a certaindegreeofuncertaintyand to look forall the possibilities. • Ifyouonly trust yourmemory and experience I amafraidyoumay do some mistakes.

42. Algorithmfor management of a baby with tachypnoea. CXR, chest radiograph; SpO2, oxygensaturation. SaO2 < 95% it is possible to wait for 4 weeks Balfour-Lynn IM, Arch Dis Child. 2015;100:722-7.

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