Pediatrics 3

What are the causes of jaundice in a neonate? ( 6 marks)

1 MARK for classification and 1 mark for example in each category

Early onset (first 24 hours, haemolytic jaundice) 1mark

• Rhesus haemolytic disease

• ABO incompatibility                    1mark

• G6PD deficiency

• Hereditary spherocytosis

Normal onset  1mark

• Physiological (all newborns get a degree of jaundice peaking at 4–5 days)

• Bruising

• Polycythaemia                                                          1mark

• Causes of early jaundice

Late onset (>14 days, prolonged jaundice) 1mark

• Persistence of a pathological earlier jaundice

• Breast milk jaundice

• Neonatal hepatitis                            1mark      

• Biliary atresia

• Hypothyroidism

• Galactosaemia

Jaundice can also be a non-specific marker of neonatal infection at any stage.

Why is neonatal jaundice potentially dangerous? (3 marks)

Neonatal jaundice can be dangerous, as unconjugated bilirubin can cross the blood–brain barrier. Very high levels can lead to kernicterus which can cause deafness and choreoathetoid cerebral palsy.

What is the cause in this infant? Give three reasons why do you think this is the cause in this infant? ( 4 marks)

The cause is :ABO incompatibility. Reasons are:

His mother is O+ and will therefore have anti-A and anti-B antibodies in her blood. These antibodies can cross the placenta and lead to haemolysis in infants with either blood group A or B.

Haemolysis is further confirmed by the positive DAT, decreased haemoglobin and raised reticulocyte count. The presence of a few spherocytes is common in ABO incompatibility.

What is the treatment of neonatal jaundice? (2 marks)

There are graphs that provide guidelines stating at what bilirubin level the treatment with phototherapy and an exchange transfusion are indicated.

What investigations would you do for jaundice, in general?

1. Total, direct and indirect billirubin levels

2. LFTs

3. FBC + peripheral smear(hemolysis)

4. Reticulocyte count (increased in hemolysis)

5. Coombs test (autoimmune hemolysis)

6. Hepatitis screen

7. PT/INR (liver synthetic function)

Explain the pathophysiology of therapeutic hypothermia? 3marks

• The main aim is to decrease the temperature, resulting in a decrease of the metabolic rate and demand of the brain.

• Thus, this results in a decrease of chemicals released during hypoxic injury, therefore minimize damage caused by these chemicals

What is the target infant temperature which you should target during therapeutic hypothermia?

33.5

Define pancytopenia. 2marks

Pancytopenia is a hematological condition in which all three major blood cell lines are decreased:

• Red blood cells (RBCs) → leading to anemia

• White blood cells (WBCs) → leading to leukopenia/neutropenia

• Platelets → leading to thrombocytopenia

Name 5 causes of pancytopenia. 5marks

1. Bone Marrow Failure

• Aplastic anemia

• Myelodysplastic syndromes (MDS)

• Leukemia (especially acute leukemias)

• Lymphoma involving the marrow

• Multiple myeloma

2. Marrow Infiltration (Myelophthisis)

• Metastatic cancer

• Fibrosis (myelofibrosis)

• Granulomatous diseases (e.g., TB, sarcoidosis)

3. Infections

• HIV, hepatitis B/C, EBV, CMV

• Sepsis

• Malaria, kala-azar (visceral leishmaniasis)

4. Nutritional Deficiencies

• Vitamin B12 deficiency

• Folate deficiency

• Copper deficiency

5. Autoimmune Diseases

• Systemic lupus erythematosus (SLE)

• Hemophagocytic lymphohistiocytosis (HLH)

6. Drugs and Toxins

• Chemotherapy, radiotherapy

• Chloramphenicol, linezolid, sulfonamides

• Alcohol, benzene

7. Hypersplenism

• Due to splenomegaly – sequestration and destruction of blood cells

In a patient with pancytopenia, you notice that her reticulocyte production index is 0.1%. Name which investigation you will consider doing.

This indicates severely reduced bone marrow activity, meaning the marrow is not compensating for the low blood counts. This points to a central (bone marrow) cause of pancytopenia. Investigation to consider would be Bone marrow aspiration and biopsy.

In the diagnosis of diabetes ketoacidosis, what signs and symptoms and hx do you look for to make the diagnosis?

1. Newly dx diabetes

2. Known with diabetes with a previous admission of diabetes ketoacidosis

3. Hyperglyceamia(reagest strip/laboratory)

4. Ketones(reagent strip)

What are precipitating causes of DKA?

• Infections

• Psychosocial

• Omission of insulin

• Deficient patient/parent/school education

• Health service problems

What are the potential pitfalls in the dx of DKA?

1. Children may appear well despite gross metabolic imbalance

2. Abdominal symptoms may mimic an acute abdomen

3. DKA can occur without pronounced hyperglycaemia

4. Hyperglycaemia may cause false hyponatremia

5. Creatinine assay may be falsely elevated by ketones(acetoacetate)

6. Outdadated reagent strips may not show ketones

What are the factors which indicate a need for increased vigilance?

1. Multiple admissions

2. Toddler/adolescent

3. Significant weight loss

4. Educationally disadvantaged

DKA assessment of severity. (Hemodyanamic status, hydration, level of conciousness, acidosis)

• Mild= normal perfusion, dehydration <5%, fully concious, alert, pH >/= 73.

• Moderate= Norm BP, decreased perfusion(CRT>/= 3sec), dehydration </=10%, drowsy, pH <7.3

• Severe= Low BP(poor peripheral pulses), decreased perfusion, dehydration >10%, depressed LOC, pH<7.1

Outline the principles of management of DKA (6marks)

Acute management:

1. Resuscitation(ABC)

2. Rehydration with ivi fluids with no suaga (0.9%NaCl)

3. Insulin iv infusion

4. Correct electrolyte imbalances or prevent hypokalemia/hyponatremia

5. Correct metabolic acidosis with fluids

6. Antibiotics

7. Monitor input and output

Long term management:

1. Daily subcutaneous insulin requirements

2. Dietician refferal

3. Health education and counselling adherence

4. Regular follow up/dietary diary

Side effects of steroid use

STEROIDS

S- Stomach ulcers (gastritis, peptic ulcers)

T- Thin skin & easy bruising, striae

E- Edema & Electrolyte imbalance (Na↑,K↓)

R- Round face (moon face), Redistribution of fat (Buffalo hump)

O- Osteoporosis, Opportunistic infections

I- Increased blood sugar (hyperglycemia/diabetes)

D- Depression, Delirium, other psychiatric changes

S- Suppressed adrenal glands (adrenal insufficiency)

You are seeing a 2-day old neonate with a weight 3.2 kg and normal apgars, born NVD at term by 28 years old, Gravida 2, Para 2. Mom did not attend Antenatal care, with a positive RPR but rest of booking bloods are normal. You noted the baby to be jaundiced and has a hepatosplenomegaly.

Mention 5 other clinical manifestations of congenital syphilis (early or late)? (5)

🍼 Early Congenital Syphilis (birth to 2 years)

• Skin rashes

• Mucous membrane lesions

• Anaemia / Thrombocytopenia

• Lymphadenopathy

• Painful osteochondritis of long bone

• Oedema / Hydrops

• Signs of meningitis

• Snuffles

• Pseudoparalysis

• Failure to thrive

• Gummata

• Eye and ear abnormalities

 Give 3 differential diagnosis of congenital syphilis in a newborn? (3)

• Sepsis

• Congestive heart failure

• Congenital Rubella, Toxoplasmosis, CMV

• Disseminated herpes simplex

• Haemolytic disease of the newborn

Serologic test for syphilis? (2) (one answer to count for 2 marks)

• VDRL (Venereal Disease Research Laboratory test) or RPR (Rapid Plasma Reagin test) with titres  

• Antibodies (IgG and IgM) to T. Pallidum antigen

 

Late signs of congenital syphillis

🧒 Late Congenital Syphilis (>2 years)

• Frontal bossing

• Hutchinson’s triad:

  • Hutchinson teeth (notched incisors)

  • Interstitial keratitis (visual impairment)

  • Sensorineural deafness

• Saddle nose

• Saber shins (anterior bowing of tibia)

• Clutton’s joints (painless swelling of knees)

• Mulberry molars

💉 Treatment for congenital syphillis

Penicillin G IV for 10 days

🛑 Do not miss doses; otherwise, restart full course.

You are seeing a 3 years old Nangula referred in from Oshakati state hospital with a history of loss of appetite, weight loss and generalized lymphadenopathy.On Examination you find that Nangula is very pale, having sores in the buccal mucosa that are bleeding ,she is also having a petechial rash on the trunk and limbs. When you palpate her abdomen there is a hepatosplenomegaly 5 CM below the costal margin.The blood results from Oshakati hospital are as follow:

CRP 150, ESR 50, WCC 160 , HB 2, Platelets 9 neutrophils 0.5, albumin 20, Na 141, K 5, urea 10, creatinin 110

What is your most likely diagnosis? (2 marks)

Acute leukaemia or acute lymphocytic leukaemia ( 2 marks)

NB!! Give one mark if answer is malignancy/cancer of the bone marrow or blood)
- No marks if only answer malignancy or cancer without specification

How will you confirm the diagnosis in a)? (1 mark)

And what are the expected findings? (1 mark) total 2 marks

• Bone marrow aspiration
  - Expected findings: present of blasts that replaces the normal bone marrow elements

What are the warning signs of the condition mentioned above? (6 marks)

• Unusual swellings or masses

• Pallor (anaemia) with/without fatigue or lethargy

• Unexplained weight loss

• Abnormal bleeding- unexplained bleeding and/ or easy bruising (epistaxis, petechiae, ecchymosis)

• Unexplained and/ or prolonged pain or limping

• Prolonged and/ or unexplained fever

• Acute onset neurological symptoms not due to infection (early morning headaches, often with nausea and vomiting, convulsions, abnormal speech, balance, walk, and/ or behaviour or regression of milestones, schoolwork deterioration).

• Sudden eye or vision changes (white spot and/ or eye squint)

A Baby is noticed to have abdominal distension, vomiting and passing bloody stools.  An abdominal X-ray was done.

What is the most-like diagnosis? (1)

Necrotizing Enterocolitis (NEC)

List 2 Radiological features demonstrated to confirm this diagnosis? (2)

• Distended bowel loops

• Pneumatoceles

• Bowel wall thickness

• Loss of normal polygonal gas shape

Consequently, the baby needed invasive respiratory support and the haemoglobin dropped to 7.0 g/dl. Red blood cells transfusion was ordered.  What are the 4 major possible transmissible infections that should be discussed with the parents prior to a blood products’ transfusion? (2)  

• HIV

• Hepatitis C

• Hepatitis B

• Syphilis

A 2-year-old child presents to POPD with a history of watery diarrhoea for 4 days, associated with 4 episodes of vomiting and poor feeding. The child is lethargic with laboured breathing and subcoastal recessions. Vitals: Spo2 89% on room air; HR 154; RR 45 BP: 45/80 (MAP 42); Temp 38.4.

Define shock(2)

An acute syndrome that reflects the inability of the pulmonary and circulatory system to provide adequate perfusion, oxygen and nutrients to meet the physiological and metabolic demands of organs, tissues and cells. (Circulatory inadequacy)

Name 4 types of shock with a possible cause for each. (4)

• Hypovolemic shock e.g., loss of intravascular fluid – dehydration, haemorrhage or fluid shifts

• Distributive shock e.g., septicaemia and anaphylaxis

• Cardiogenic shock e.g., cardiac dysfunction

• Dissociative shock e.g., profound anaemia and carbon monoxide poisoning

• Obstructive shock e.g.  pneumothorax and cardiac tamponade

• Septic shock – encompasses multiple forms of shock (hypovolemic; distributive; cardiogenic) - many mechanisms are operative in septic shock

• Neurogenic shock e.g. spinal cord trauma

Name 6 clinical signs you would find in this child. (3)

• Pulse: tachycardia and weak pulses

• BP: hypotension

• Breathing: tachypnoea, grunting, acidotic breathing

• Limbs: cool, clammy, pale or blue

• CNS: agitation, depressed level of consciousness, coma

• Laboratory: metabolic acidosis

How would you manage this child in POPD? (Write a step-by-step algorithm) (5)

Early recognition of sepsis and initial management in the first critical hour.

Call medical officer/ senior for help

Airway and breathing

a. Give supplemental oxygen to optimize blood oxygen content and oxygen delivery to tissues

b. Maintain or restore airway, oxygenation, and ventilation.

c. Intubate if required

Circulation

d. Obtain intravenous (IV)/Intraosseous (IO) access

e. Collect blood culture and other necessary bloods-fbc, uke, lft, crp, coagulation studies, lactate (before antibiotics are given if possible- do not delay antibiotics or fluid therapy for blood)

f. Start broad-spectrum, empiric antimicrobial therapy

Fluid therapy:

i. administer a 0.9% NaCL or Ringers lactate fluid bolus of 10 to 20 mL/kg

g. Reassess- HR and RR should decrease and BP improve, LOC should improve if not

h. Repeat 0.9% NaCL or Ringers lactate fluid bolus of 10 to 20 mL/

i. Start vasoactive agents if shock persists after 40 to 60 mL/kg (or sooner if signs of fluid overload develop or myocardial dysfunction is present).

 i. Consider hydrocortisone if refractory shock

ii. Consider blood products if indicated

Disability

j. Treat hypoglycaemia, hypo/hyperthermia, correct hypocalcaemia

• Call PICU/High care for admission

• Blood glucose- correct hyperglycaemia mostly and hypoglycaemia

• Blood gas- correct metabolic acidosis and other derangement

 

Name at least 3 investigations you would do and why? (3) 

• FBC- WCC and differential can indicate sepsis, low platelets can be due to DIC

• CRP or Procalcitonin- monitor as inflammatory marker

• Electrolytes and ALT check for end organ damage such AKI, for liver dysfunction

• Coagulation profile- to check for DIC

• And of septic work up- blood cultures, urine and stool mcs

State 6 clinical signs to look out for when giving fluid resuscitation. (3)

Signs of fluids overload or fluid response

· Palpate central pulses again, check for pulse rate reducing and volume improving

· Check if there is an improvement in level of consciousness

· Blood pressure should improve

· Reduce in respiratory rate if patient was acidotic

· Check that child is passing urine or the volume has improved

· Basal crepitation

· Enlarging hepatomegaly

· Periorbital oedema 

A 9 y.o female child presents to KSH POPD with a 1-week history of joint pain; initially on the right knee, then moved to the right elbow and now on the left knee and fever. Two weeks prior to the onset of joint pain, she was seen at Maxulili clinic for a sore throat and fever and was treated with Amoxicillin for 5/7, paracetamol and multivitamin.

O/E: the patient appears ill, not in respiratory distress. Vitals: Temp 38.1 °C, HR 120 bpm, BP 100/60, sat 95% on RA.

ENT: mildly inflamed tonsils, no exudate

CVS: regular pulses, S1+, S2+, a grade 3 pan-systolic murmur was heard. Rest of the systems were normal

What is the most likely diagnosis? (2)

Acute Rheumatic fever

What is the most common organism responsible for this condition? (2)

Group A beta-haemolytic streptococci (Streptococci pyogenes)

Give 3 major and 3 minor criteria and the diagnostic criteria for this condition (8)

Major criteria

· Carditis

· Polyarthritis

· Subcutaneous nodules

· Erythema marginatum

· Sydenham chorea

Minor

· Arthralgia

· Fever

· ESR >30 and or CRP >3

· Prolonged PR interval

Diagnosis is made by the presence of 2 major criteria, or 1 major criteria and 2 minor criteria together with evidence of a preceding streptococcal infection.

How will you work up this child? (4)

· FBC: Leukocytosis

· Inflammatory markers: CRP and ESR

·ASOT (Anti-Streptolysin O Titre)

· Throat swab

· Echo

· ECG

· Blood culture (to rule out infective endocarditis)

· CXR

How will you treat this patient? (4)

· Admit the child

· Antibiotics: Benzathine benzylpenicillin IM as a single dose or Pen VK for 10/7

· Bed rest, adequate fluid intake, avoidance of respiratory irritants

· Anti-inflammatory treatment: Aspirin, prednisone

· Prevention of future attacks: Benzathine benzylpenicillin IM monthly or Pen VK BD

Kapanga, a 9-month-old boy, presents with a 3-day history of diarrhoea and a 1-day history of poor appetite and weakness. On examination, his weight-for-height is less than the -3 Z-score; his mucus membranes are dry and his skin returns slowly to baseline following a skin pinch. He appears lethargic, pale and has oedema.

List your likely diagnoses (problems). (3)

SAM with oedema

AGE with severe dehydration

Anaemia secondary to? Iron deficiency

List important investigations and the rationale for each. (0.5x6=3)

FBC: Rule out infection, Rule out anaemia

U&E: Rule out electrolyte imbalance: Hypo/hypernatremia. Hypokalemia.

           Rule out AKI

CMP: electrolyte derangements in malnutrition

CRP: Rule out infections

Blood culture: Rule out bacteremia

 

Write clear instructions for the nurses regarding the management of the patient in the ward (5)

1. Resomal 5ml/kg every 30min for 2 hours, the F75 alternating with resomal 5-10mls every hour for the next 4-10 hours

2. Monitor the child’s progress every 30 min for the first two hour, then monitor hourly

3. Attainment of target hydration weight: stop giving resomal if child has reached the target hydration weight

4. Monitor for clinical signs of fluid overload :

   • Child weight exceeds the target weight

   • Increased respiratory and pulse rate

   • Engorged jugular vein

   • Increased edema

5. Monitor clinical signs of improvement

   • Respiratory rate

   • Pulse rate

   • Urine frequency

   • Strict input/output monitoring

   • Stool or vomit frequency

6. Monitor Temperature

   • Warm the child is axillary Temp is <35 degree Celsius

7. Monitor HGT 2 hourly

   • Maintain HGT >3 mmol/L

At the next review you notice that his hands and feet are cold, capillary refill time is 5 seconds and he has a heart rate of 140 with a thready pulse.

What is the diagnosis? (1)

Hypovolemic shock

Discuss the steps you would take to manage this diagnosis. (5)

1. IV fluids at 15mls/kg over 1 hour. Use the following solutions

   • ½ DD

   • Ringers Lactate solution with 5% dextrose

   • Or 0.45% saline with 5% glucose

2. Observe the child and check respiratory rate (should not increaese

3. If there are signs of improvement (Pulse rate and respiratory rate slower, improvement in capillary refill time, peripheries warming up):

   • Repeat IV infusion at 15mls/kg over 1 hour, then

   • Switch to oral or NG rehydration with ReSoMal at 5-10mls/kg in alternate hours with F75 for up to 10 hours

   • Initiate re-feeding with F75

   • Continue to chee respiratory rate and pulse rate every 5-10 minutes

   • Continue to monitor weight changes

4. If the child fails to improve after 1 hour on IV fluids, the treat for septic shock

   • Give maintenance IV fluids 94mls/kg per hour)while waiting for blood

   • When blood is available, transfuse RCC/ fresh whole blood at 10mls/kg slowly over 3 hours

   • Provide IV antibiotics

5. If the child deteriorates during IV rehydration ( shows signs of fluid overload or congestive heart failure

6. If the respiratory rate increases by 5 breathes per min, or pulse rate increases by 20 beats per minute, liver enlarges, fine crackles occur throught the lung field, jugular vein pressure increases or gallop rhythm develops. STOP the infusion because IV fluids can worsen the child’s condition by inducing pulmonary oedema

What are the risks of management? (2)

Heart failure

Pulmonary oedema

As part of your side room investigations you perform a blood glucose measurement, which is 2.9mmol/l.

Discuss management options in this particular situation? (2)

Asymtomatic:  give 50mls bolus  of 10% glucose or 10% sucrose orally and start feeding F75 immediately and follow the feeding schedule

Symptoomatic: 10% dextrose  5mls/kg IV, followed by 50mls of 10% dextrose  by NGT

A urine dipstix performed reveals 3+ leucocytes and 2+ nitrites.

Which antibiotics would you start? (1)

• Ampicillin 50mg/kg IV every 6 hours for 2/7, then Amoxicillin 25-40mg/kg BD 5/7

• Gentamicin 7.5mg/kg IV OD 7/7

You receive Kapanga’s blood results back. The most striking abnormality that you note on the full blood count is a haemoglobin level of 7.3g/dl.

What parameters would guide you regarding the need for transfusion? (3)

Symptomatic anemia

· Hypotension,

· Tachypnoea,

· Tachycardia,

· Systolic ejection murmur/gallop rhthm,

· Signs of congestive heart failure (CHF): such as tachycardia, gallop rhythm, tachypnea, cardiomegaly, wheezing, cough, distended neck vein, and hepatomegaly.

· Altered mental status,

· Evidence of tissue hypoxia (eg, lactic acidosis, ischemic changes on electrocardiogram)

Adriano is a 3year old boy from Angola. He is RVD unexposed, HIV rapid test negative. He is moderately stunted, but otherwise thriving well. His mother was speaking through a translator explaining how he first presented at the age of 9months with swollen, extremely painful fingers and abdominal pain. She explains further that she noticed that he was jaundiced since two weeks after birth and this is still persistent. He often has excruciating pain in the limbs and this requires him to be admitted in the hospital. She has been seeking help in Angola, but to no avail. She now heard that she might be helped at Katutura Intermediate Hospital and thus present to POPD, you are the Intern on call.

What is the most likely diagnosis? (1)

Sickle cell disease

How would you confirm the diagnosis? (3)

Hemoglobin electrophoresis:

• Hemoglobin SS (HbSS, 80-90%)

• Hemoglobin F (HbF, 2-20%)

• Hemoglobin  A2 (HbA2, 2-4%)

What was the acute condition Adriano first presented with at 9months? Explain the pathophysiology why this acute condition occurs (2)

• Dactylitis or bone infarction: Infarction of bone marrow and cortical bone in the metacarpals, metatarsals, and proximal phalanges.

You examined Adriano and discovered a big left-sided abdominal mass, the Mother explain that this has always been there, however today it is much bigger. As you palpate him he started crying due to this left-sided abdominal pain.

What is this phenomenon called and how would you manage it? (1)

Acute splenic sequestration crises: a simple blood transfusion therapy should be considered.

The mother further reports, on one occasion while he was asleep he was awakened by pain in his penis lasting four hours.

What is this condition called?  and what home-remedies would you give the mother to manage this condition? (2)

Priapism

At the onset of priapism, patients should be advised to drink extra fluids, use oral analgesics, and attempt to urinates.

After 2 months Adriano presents to POPD again, this time his mother tells you he had chest pain, cough and fever. He was seen 2 days ago at his local Clinic and treated with antibiotics and analgesia, but there was no improvement. Adriano’s condition progressively worsened and now he is back due to difficulty breathing. His oxygen saturation is 85% in room air and has crepitations on the right lower lobe.

What is this life-threatening condition called and how will you manage it? (2)

Acute chest syndrome

analgesia, oxygen, incentive spirometry, bronchodilators, antibiotics, and transfusion

Besides the above acute conditions, which other acute conditions could Adriano present with in the future, if his condition is not managed adequately? (2)

• Infections

• Severe Anemia due to aplastic crisis, hyperhemolysis

• Stroke

• Kidney infarction or medication toxicity

• Myocardial infarction

• Venous thromboembolism

What are the complications of Adriano’s medical condition as diagnosed in question a) above? (2)

●Pain

●Anemia

●Neurologic deficits or seizure disorder

●Pulmonary conditions

●Impaired kidney function and hypertension

●Osteoporosis and complications of bone infarction

●Cardiomyopathy with diastolic dysfunction

●Hepatotoxicity (transfusional iron overload or medications) and pigmented gallstones (chronic hemolysis)

●Delayed puberty and reduced growth

●Chronic leg ulcers

●Proliferative retinopathy

 

A 5-year-old boy, Jayden, arrives in the emergency department convulsing. You note that it is a generalized tonic-clonic seizure

List FOUR things you would do immediately (4)

1. Establish and maintain adequate airway, breathing, and circulation

2. Identify and treat hypoglycemia

3. Stop the seizure and thereby prevent brain injury: Lorazepam/ diazepam IV/PR/Midazolam

4. Identify and treat life-threatening causes of convulsions such as trauma, sepsis, meningitis, encephalitis, or structural brain lesion

His seizure stops and you get further history from his mother. He had been fitting for about 15 minutes before they got to the hospital. She reports that the seizure started in the left upper and lower limbs and then involved the whole body. This is his first seizure. Mum had recorded a temperature of 38 degrees Celsius prior to the seizure.

List THREE differential diagnoses for the seizure (except for a febrile convulsion) (3)

Any three

●Genetic

●Structural: Head trauma (concussion), ischemia, or bleeding (intraparenchymal or subarachnoid hemorrhage), perinatal asphyxia or in utero stroke, brain tumor, a neurodegenerative or neurometabolic disease, aftermath of acute hypoxic and toxic/metabolic events

●Metabolic: Glucose transporter deficiency, creatine deficiency syndromes, mitochondrial cytopathies hypocalcemia, hyponatremia

●Immune Rasmussen encephalitis and anti-N-methyl-D-aspartate (NMDA) receptor encephalitis

 ●Infectious: HIV encephalopathy, neurocysticercosis, malaria, tuberculosis, meningitis, encephalitis, sequelae of prior meningitis or encephalitis.

●Psychogenic nonepileptic seizures (PNES)

What are the criteria for making a diagnosis of a febrile convulsion? (5)

●A convulsion associated with an elevated temperature greater than 38°C

●A child older than six months and younger than five years of age

●Absence of central nervous system (CNS) infection or inflammation

●Absence of acute systemic metabolic abnormality that may produce convulsions

●No history of previous afebrile seizures

What is the definition of Status epilepticus? (2)

SE is either a single unremitting seizure lasting longer than five minutes or a frequent clinical seizures without an interictal return to the baseline clinical state.

aList FOUR investigations that are indicated in this child. (4)

• FBC

• U&E

• CMP

• CT Brain

• EEG

The child starts convulsing again after 5 minutes.

What other drugs would you administer as second- and third-line agents? (2)

Second line: Levetiracetam, phenobarbitone,  phenytoin/fosphenytoin, and valproate

Third line: continuous infusion of midazolam (preferred) or pentobarbital

2y/o female known to oncology with an abdominal mass. She started chemotherapy about 3 months ago. The patient is known with normal renal functions for these past 3 months. She now presented after a 3 week break with the mass increasing in size and straining when urinating. When you do her routine bloods, you notice these U&E result:

K+ 6.9/Na+ 139/Urea 13.4/Cr230

Interpret these U&E Results.

Acute Kidney Injury complicated by hyperkalaemia

Define acute kidney injury in children.   (2)

A sudden and often reversible reduction in the kidney function, as measured by increased creatinine or decreased urine volume.

Group and list general causes of acute kidney injury (ONE cause under each heading is enough).

Pre-renal	Renal	Post Renal
· Dehydration · Haemorrhage · Septicaemia · Nephrotic syndrome · Hepatorenal Syndrome/ · Cardiac Failure	· Haemolytic uraemic syndrome · Glomerulonephritis · Interstitial Nephritis	· Posterior urethral valves · Pelviureteric junction dysfunction · Vesicoureteric junction dyfunction · Kidney Stones

List FOUR complications of acute kidney injury which are indications for dialysis (4)

• Metabolic Acidosis

• Hyperkalaemia

• Uraemia

• Pulmonary Oedema

You notice the patient is on Half strength Darrow's with dextrose (1/2 DD) which contains potassium. How would you manage the high potassium? (6)

• Stop ½ DD and change to a potassium free solution

• Repeat U&E to confirm hyperkalaemia

• Calcium gluconate

• Salbutamol nebs

• Normal saline Fluid bolus

• Stat dose Lasix

• Sodium bicarbonate

• Dextrose and actrapid / just dextrose bolus

• K-Exelate