Revision

1. Revision Krzysztof Narębski Toruń

2. Problems to discuss • Taking a history • Physical examination • General condition assessment • Pediatric resuscitation • Neoplasms (malignant diseases)

3. Taking a history • Present problem (opened question) • What seems to be a problem ? >> • Goal oriented history and physical examination ! • Review by system • Past medical history • Family and social history

4. Physical examination • Establish your own scheme but • Always tailor examination to anamnesis • Always pay attention what diseases are commonly presented according to age • Examine a child in the presence of parents

5. Physical examination cont. • Every element of physical examination must be perform to obtain an information ! • Convince your supervisor what are you performing any gesture for ! • The same with the laboratory and other tests !

6. General condition • General state (good, intermediate, poor, severe to agonal) • Routine procedure or • Resuscitation

7. „SICK CHILD”, „SEPTIC” A - Arousal, Alertness, Activity, Apathy B-Breathing difficulties C- poor Colour (pale) and Circulation (Cold peripheries) D-Decreased fluids intake (fewer than half anormal intake) and Decreased urine output (fewer than 4 wet nappies a day)

8. Level of awareness, Coma A - Alert V- responds to Voice P - responds to Pain U - Unresponsive

9. Pediatric resuscitation • Respiratory problems as primary problem ! hence • Firstly check for breathing & airways obstruction and give 5 initial breaths • Secondly check for pulse 15 compression : 2 breaths Rate 100 – 120 compressions / min

10. Basic Life Support – Airways & Breathing Trained resuscitator

11. Basic Life Support - Circulation Trained resuscitator

12. Drug of life • Adrenaline = epinephrine • 10 – 30 mcg / kg • intravenously (iv) or intraosseous (io)

13. Site ofIO infusion

14. Site of IO infusion

15. Malignant diseases in childhood General informations

16. Terminology • In anglo-saxon terminology cancer is used as a general term for malignant disease. • In anglo-saxon terminology tumour is used as a term for neoplasm (malignant or benign). but in reality (distinguishing in polish language) • Cancer = neoplasm from epithelial tissue • Tumour = nodule, bump, sth. protuberant

17. Relative frequency of different types of cancer in children in the UK.

18. Neoplasms (tumours) - elements • Leukaemia affects children at all ages (although there is an early childhood peak). • Neuroblastoma and Wilms tumour (nephroblastoma) are almost always seen in the first 6 years of life. • Hodgkin lymphoma and bone tumours have their peak incidence in adolescence and early adult life.

19. Five-year survival rates showing the considerable improvement over the last 50 years. (ALL, acute lymphoblastic leukaemia; NHL, non-Hodgkin lymphoma.)

20. Neoplasm incidence • Fortunately not common • Around 1 child in 500 develops cancer by 15 years of age. • Each year, in Western countries, there are 120-140 new cases per million children aged under 15 years, equivalent to about 1500 new cases each year in the UK.

21. Neoplasms – general info 1 Clinical presentation: • Local tumour (mass effect - obstruction) vs disseminated infiltration (end stage disease) • Hence very often delayed diagnosis

22. Neoplasms – general info 2 Investigations: Think about neoplasm in differential diagnosis !!!

23. Neoplasms – general info 3 Think about neoplasm according to pediatric populations !!! • Small children: teratoma, retinoblastoma, neuro i nephroblastoma • Preschool & school children: leukaemia, brain tumors, Non-Hodgkin lymphoma • Adolescent: Hodgkin disease, sarcoma (soft tissues and bone tumors)

24. Neoplasms – general info 4 Investigations: Initial symptoms can be very non-specific and this can often lead to significant delays in diagnosis. Once a diagnosis of malignancy is suspected, the child should be referred to a specialist centre for further investigation.

25. Some detail information about specific (most common neoplasms) Leukaemia Brain tumours Lymphomas Neuroblastoma

26. Leukaemia • Peak incidence at 2-5 years. • Clinical symptoms and signs result from disseminated disease (infiltration of the bone marrow or other organs with leukaemic blast cells). • In most children, leukaemia presents insidiously over several weeks but in some children the illness presents and progresses very rapidly.

27. !!! !!!

28. Case History A 4-year-old girl was generally unwell, lethargic, looking pale and occasionally febrile over the last 9 weeks. No benefit of two courses of antibiotics for recurrent sore throat. She returned to general practitioner when developed a rash. Examination showed pallor, petechiae, modest generalised lymphadenopathy and mild hepatosplenomegaly. A full blood count showed: • Hb 8.3 g/dl • WBC 15.6 × 109/L • Platelets 44 × 109/L. • Blast cells were seen on the peripheral blood film. Bone marrow examination confirmed acute lymphoblastic leukaemia.

29. Brain tumours Symptoms related to raised intracranial pressure: - vomiting (in the morning) - headaches, change in behavior - increased head circumference (tense fontanels, separation of suture) Focal neurological signs depending on the site of the tumour: - regression of skills (bicycle, painting etc.) - visual disturbance, seizures - various neurological signs (cranial nerves)

30. Brain tumours Investigations: • Brain tumours are best characterised on MRI scan. • Lumbar puncture must not be performed without neurosurgical advice if there is any suspicion of raised intracranial pressure.

31. Brain tumours

32. Lymphomas • Lymphomas are malignancies of the cells of the immune system and can be divided into Hodgkin and non-Hodgkin lymphoma (NHL). Uncontrolled proliferation of B or T cells outside bone marrow (different signs and symptoms). • NHL is more common in childhood. • Hodgkin l. more common in adolescence.

33. Lymphomas Clinical features: • Classically presents with painless lymphadenopathy, most frequently in the neck (can start in mediastinum or abdomen). • Lymph nodes are much larger and firmer than the benign lymphadenopathy commonly seen in young children.

34. Lymphomas Investigations: • Whole lymph node examination (preferred to biopsy), radiological assessment of all nodal sites and bone marrow biopsy is used to stage disease and determine treatment.

35. Case History A 14-year-old girl complained of a cough for 2 weeks which was non-productive and worse at night. She had seen her GP and her chest was clear. She returned 2 weeks later, as she had noticed a swelling in her neck. On examination non-tender large anterior cervical lymph node. On chest X-ray a large mediastinal mass. Differential diagnosis: • T-cell non-Hodgkin lymphoma • Hodgkin lymphoma Diagnosis: A biopsy of the mass confirms a diagnosis of Hodgkin lymphoma.

36. Chest X-ray showing a large mediastinal mass. Normal chest

37. Lymphomas Hodgkin disease NHL

38. Neuroblastoma • Neuroblastoma and related tumours arise from neural crest tissue in the adrenal medulla and sympathetic nervous system. • Spontaneous regression sometimes occurs in very young infants (rarely). • Spectrum of disease from the benign (ganglioneuroma) to the highly malignant (neuroblastoma - most common before the age of 5 years).

39. Neuroblastoma • At presentation, most children have an abdominal mass, but the primary tumour can lie anywhere along the sympathetic chain from the neck to the pelvis (different signs and symptoms). • Over the age of 2 years, clinical symptoms are mostly from metastatic disease, particularly bone pain, bone marrow suppression.

40. Case History Jack, a 3-year-old boy, was taken to his GP because he was not eating as well as usual and had a distended abdomen. Recently, he appeared reluctant to walk and sometimes cried when he was picked up. His grandmother noticed lost weight. On examination, the GP confirmed that he seemed generally miserable and pale and palpated a large abdominal mass. The boy was hypertensive.

41. Case History cont. An initial ultrasound examination confirmed the abdominal mass and an MRI scan characterised a very large upper abdominal mass in complex relationship with the left kidney and the major vessels but extending towards the midline, suggestive of neuroblastoma.

42. MRI showing a large left-sided primary neuroblastoma arising from the adrenal region and distorting blood vessels.

43. Neuroblastoma

44. Other neoplasms • Wilms tumour (nephroblastoma) originates from embryonal renal tissue. Over 80% of patients present before 5 years mostly with a large abdominal mass, often found incidentally in an otherwise well child. • Retinoblastoma is a malignant tumour of retinal cells. Most cases present within the first 3 years of life. The most common presentation is a white pupillary reflex.

45. White pupillary reflex in retinoblastoma

46. Proposal of initial medical record 1. Info from history : 1 year old, cough, fatigue 2. Info from exam.: dyspnoea, rales, wheeze 3. Differential diagnosis: Infection (pneumonia, bronchitis) Foreign body aspiration Asthma Tumour, cystic fibrosis 4. Initial tests: blood cell count with film, CRP, ionogram, glucose, viral tests, chest x-ray 5. Initial diagnosis and treatment

47. Thank you for your attention !