Clinical Pediatric Casess

A 22-month-old girl with no significant co-morbidities presented to Paediatric
 ER with a history of 36 hours of diarrhoea and vomiting associated with a 2 minute toniceclonic seizure with associated eye-rolling and post-ictal phase at home. On arrival to 
 ER she proceeded to have two further 2 minute toniceclonic seizures within 2 hours of the first. She had profuse diarrhoea and vomiting. She had been seen twice previously in hospital for Bronchiolitis and Viraleinduced wheeze. She had no known allergies, no recent travel abroad and was up to date with her immunisations. Her paternal aunt had febrile seizures as a child. There were no developmental concerns.
On examination she was afebrile throughout her time in A
þ E, well perfused with a CRT of less than 2 seconds, heart rate of 160 and respiratory rate of 40. Her general examination was normal however she had a GCS of 14 and was floppy in a post-ictal state.
She was admitted for a fluid challenge requiring continuous NG dioralyte and proceeded to have short generalised toniceclonic seizures about every 90 minutes on the ward. Bloods including glucose were normal. After the eighth seizure she had a CT head and LP which were normal. 48 hours into the illness she proceeded to have a generalised toniceclonic seizure lasting 7 minutes followed by increased tone on the right side in both upper and lower limbs. She was treated with buccal midazolam followed by IV Lorazepam 10 minutes later. After another 30 minutes with ongoing hypertonia on right side she was started on Phenytoin. She was started on IV Ceftriaxone and Aciclovir. After 2 hours the seizure ceased and she had no further seizures. She had been having seizures for a total of 24 hours.
Q1. What is the most likely cause for her seizures?

a) Febrile convulsions
b) Epilepsy
c) Meningitis
d) Rotavirus

e) Herpes Encephalitis

Q2. What type of seizures can occur in this condition?
a) Symmetrical seizures
b) Generalised seizures
c) Partial seizures
d) Partial seizures secondarily generalised
e) Absence seizures

Q3. Over what period of time do the seizures characteristically occur in this condition?

a) 12 hours
b) 24e48 hours
c) 3e5 days

d) 1 week


Answer

A1. Rotavirus
A2. aed
A3. 24e48 hours


A stool sample came back as positive for Rotavirus. Rota-virus is well documented to cause short seizures both generalised and focal, however case reports of status epi-lepticus are rare in afebrile children with rotavirus and is an important part of counselling parents whose children have gastroenteritis symptoms and present with seizures. Previous Asian studies have shown an incidence of afebrile seizures in children being 2.06% with the highest incidence being between 1 and 2 years (4.67%). These seizures most commonly happened on the third day of diarrhoea, typically
in a cluster lasting 24 hours with no status epilepticus reported. Seizures are usually symmetrical and generalised. However partial seizures and partial seizures secondarily generalised have also been described. Over the last 3 years non-Asian studies have been published showing similar results with a mean age of 17 months and maximum seizure duration lasting 10 minutes over 24-48 hours.

Children with rotavirus gastroenteritis are a frequent presentation to most paediatric units. Rarely they are noted to have short self-resolving seizures and parents can be counselled appropriately regarding this complication. It is very unexpected for a patient with no other co-morbidities who is afebrile to develop status epilepticus as a complication of rotavirus gastroenteritis and therefore patients are not usually counselled about this. Recognising this clinical picture and what it is helps to calm both the physician and the patient’s family, given the anxiety-provoking nature of clustered convulsions.

Clinical Cases

CASE 1: 

A 10-month-old girl presented with a history of being grizzly, quiet, off food and tugging at her ears for 18 hours with associated low-grade pyrexia. She had 2 loose bowel motions and 6 wet nappies that day with good fluid intake. There were no other concerns and the only past medical history was gastroschisis. On examination she was well perfused with good hydration status. She was tachycardic in association with a pyrexia but nil else to find. After a night of observation her heart rate had normalised, as had her temperature. She had vomited three times and appeared grizzly, pale and lethargic with dry mucous membranes. By the afternoon she was tolerating fluids well, apyrexial and the diarrhoea and vomiting had resolved. She was discharged home.
The next afternoon she reattended not tolerating fluids but still passing urine with associated sleepiness. On examination she had a congested throat but otherwise appeared well. She was kept in overnight as she was still not drinking good volumes and given Difflam spray to encourage fluid intake. The next morning she had another low-grade temperature but other observations were within normal limits. She was tolerating fluids. On examination she was irritable and pulling at her ears. She was noted to have some mild neck stiffness.
Q1. At this point what would your management plan be?

a) Continue encouraging oral fluids and observe through the day.
b) Pass NGT for fluid resuscitation.
c) Discharge home.
d) Blood tests for FBC, CRP and U
þ Es
e) Start antibiotics for Pharyngitis.
e) Blood tests for FBC, CRP, U
þ Es, blood culture and LP then start on Ceftriaxone and Dexamethasone.

Q2. What is the most common cause of bacterial meningitis in children more than 3 months?

a) H influenzae Type B
b) Meningococcus C
c) Pneumococcus
d) Meningococcus B

e) Group B Streptococcus


Q3. Which of the following signs are contraindications to performing a lumbar puncture?

a) Tachycardia
b) Spreading purpura
c) Platelets 55
109/litre
d) GCS 14

e) Focal neurological signs

Answer



A1 e) In view of these subtle signs of meningism she had bloods taken, an LP done and was started on IV Ceftriaxone and Dexamethasone. The CSF was turbid with a glucose of less than 0.3, protein of 2.67, 90% polymorphs and 10% lymphocytes with gram negative diplococci grown later confirmed to be meningococcus. Her bloods showed a CRP of 169 and the blood culture grew meningococcus as well. She was treated with 4 days of Dexamethasone and 7 days of Ceftriaxone as per the NICE guidance for Meningococcal Meningitis.

A2 d) Following the introduction of vaccinations Meningococcus C, Hib and Pneumococcus have all decreased their presentation with Meningococcus B being the most common cause of bacterial meningitis in children more than 3 months.

A3 b,c,e) Contraindications to LP in a child with suspected meningitis include signs suggesting raised intracra-nial pressure: reduced or fluctuating level of consciousness
i.e. Glasgow Coma Scale score less than 9 or a drop of 3 or more; relative bradycardia and hypertension; focal neurological signs; abnormal posture or posturing; unequal, dilated or poorly responsive pupils; papilledema; abnormal ‘doll’s eye’ movements or shock, extensive or spreading purpura, after convulsions until stabilised, coagulation abnormalities: coagulation results outside the normal range; platelet count below 100
109/litre; receiving anticoagulant therapy or local superficial infection at the lumbar puncture site or respiratory insufficiency.

This case shows how difficult it can be to identify meningitis. Once neck stiffness was identified she was investigated. However literature shows how non-specific the presentation of meningitis can be. A systematic review of prospective data in 2010 showed that complaints of bulging fontanelle, neck stiffness, seizures (outside febrile-convulsion age range) or reduced feeds raised concern about the presence of meningitis while on examination, jaundice, being toxic or moribund, meningeal signs, neck stiffness, bulging fontanel, Kernig sign, tone up, fever of more than 40 C and Brudzinski sign independently raised the likelihood of meningitis. The absence of meningeal signs and an abnormal cry independently lowered the likelihood of meningitis. The absence of fever did not rule out meningitis. This review concluded that no isolated clinical feature is diagnostic, and the most accurate diagnostic combination is unclear.
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Case 2


A normally delivered term baby weighed 3.67 kg (50th centile). At 
home, he breastfed well and had immunisations at 2 and 3 months with no complications. He stopped breastfeeding at 16 weeks and started a weaning diet soon after. At 5 months he was admitted with a 2-week history of cough, unresponsive to amoxicillin and cephalexin. He had pallor on coughing but no apnoeas. He had mild intercostal recession during feeding only, which was not reduced. Despite this, baseline oxygenation saturations were 84% in air, improving in 1 l/min nasal oxygen. His admission weight was 6.4 kg (2nd to 9th centile). No organism was identified and he was discharged 6 days later to complete 10 days of clarithromycin. Three weeks later he developed Rotavirus-positive diarrhoea, which settled with frozen breast milk. His weight was 6.48 kg (2nd centile). Three weeks later he was readmitted with ongoing diarrhoea and a chesty cough, vomiting phlegm. His weight was 6.18 kg (<0.4th centile). He had a wasted appearance, crackles in the right mid-zone, and respiratory rate of 40 per minute with mild recession. He had no eczema. Chest x-ray showed generalised haziness and stool remained Rotavirus-positive. Oxygen saturations were normal in air. Cefotaxime and clarithromycin were started. Over the course of the next 3 days, he clinically deteriorated and required invasive ventilation via endotracheal tube; oxygenation was harder to achieve than carbon dioxide clearance and he developed evidence of severe barotrauma, with pneumomediastinum and surgical emphysema .

(a)  Which of the following investigations considered before his acute deterioration is most likely to be abnormal?

Sweat test
 Coeliac screening
 Immunoglobulins and lymphocyte subsets
Bone scan Urine organic acids

(b)  Which investigation is needed urgently following intubation?

 Respiratory sample for Pneumocystis jirovecii
Respiratory sample for Aspergillus
 Respiratory viral screen 
Respiratory mucus elastase 
Surfactant proteins


(c)  What specific treatment
would you start blindly while waiting for results?

High dose steroids
Antifungals
 High dose co-trimoxazole 
High dose co-trimoxazole and steroids 
Surfactant 


The answer

(a)  Immunoglobulins and lymphocyte subsets

 (b)  Respiratory sample for Pneumocystis jirovecii
 (c)  High dose co-trimoxazole

MCQs In Allergy

A 13-year-old girl has a history of acute difficulty breathing when playing basketball. Her symptoms include inspiratory wheezing/stridor, increased respiratory rate, throat tightness, and chest discomfort. Premedication with adequate doses of albuterol has no effect.
What is the most likely diagnosis?

A) Exercise-induced asthma.
B) Gastroesophageal reflux disease.
C) Musculoskeletal chest pain.
 D) Hyperventilation.
E) Vocal cord dysfunction.

MCQs In Calcium Hemeostasis

Q 1

Which of the following statements about calcium homeostasisis incorrect?

a. Approximately 45% of serum calcium is bound to protein.

b. Parathyroid hormone stimulates bone resorption via its actions on osteoblasts.

c. Activated vitamin D inhibits the enzyme 1a-hydroxylase.

d. The daily calcium requirement for a teenage girl is 800 mg per day.

e. Wholemeal bread increases the dietary calcium absorption.

The Answer :

E

Wholemeal bread increases dietary calcium absorption. Calcium is available in milk and dairy products, as well as in fortified white and brown flour. It is also well absorbed from vegetables such as broccoli. 

Absorption is, however, reduced in the presence of phytates, which are present in wholegrain cereals, unleavened bread and pulses. Oxalates in spinach and rhubarb also impair absorption. 

Activated vitamin D inhibits the enzyme 1a-hydroxylase via negative feedback mechanisms.

 The recommended calcium intake for a teenage girl is 800 mg per day; reference nutrient intake levels vary according to age.

Mature osteoclasts do not appear to have parathyroid hormone receptors.

 PTH has several actions on bone, some direct and some indirect. In bone, PTH receptors are

located on osteoblasts but not on osteoclasts. Initially and transiently, PTH causes an increase in

bone formation by a direct action on osteoblasts. (This brief action is the basis for the usefulness of intermittent synthetic PTH administration in the treatment of osteoporosis.) In a second, longlasting

action on osteoclasts, PTH causes an increase in bone resorption. This second action on osteoclasts

is indirect and mediated by cytokines released from osteoblasts; these cytokines then increase the

number and activity of the bone-resorbing osteoclasts.

Thus, the bone-forming cells, osteoblasts, are required for the bone-resorbing action of PTH

on osteoclasts.

The initial event in bone degradation is the attachment of osteoclasts to the bone surface following their recruitment. PTH binding to osteoblasts triggers the synthesis of ODF, also known as RANKL or osteoprotegerin ligand.

 PTH stimulates the expression of this cell membrane protein in osteoblastic cells. Th is ligand binds to the ODF receptor (receptor activator of nuclear factor-κB [ RANK] ) expressed on the hemopoietic osteoclastic precursors and stimulates their diff erentiation into functional osteoclasts. Th ese 2 cell surface proteins, RANK expressed on osteoclast precursor cells and its partner RANKL expressed on osteoblasts, are the key regulators of osteoclast formation and function.

Activation by RANKL increases the expression of specifi c genes leading to osteoclast maturation. When an osteoclast precursor encounters an osteoblast, the resulting interaction between RANK and RANKL stimulates the osteoclast precursor to mature into a fully diff erentiated, bone-resorbing osteoclast.

--------------------------

A 14-year-old girl presented to A&E with acute abdominal pain and vomiting. Questioning uncovered a history of thirst, polyuria, lethargy and malaise, increasing over the previous few weeks. This had led to her missing several weeks of schooling. One year previously, she had been given a diagnosis of Osgood–Schlatter’s disease after presenting with bilateral knee pain. She had improved with physiotherapy
but was still troubled by frequent bone pains. She had reached all developmental milestones as expected and had been otherwise well. Her paternal uncle was diabetic, but there was no other family history of note.
On examination, this was a slim girl who appeared mildly dehydrated. Her abdomen was soft and non-tender with normal bowel sounds and no masses. She was afebrile, and there were no other relevant findings.
No surgical cause could be found for her symptoms. She was normoglycaemic on both bedside and laboratory blood glucose analysis (5.4 mmol/l, reference fasting range 3.4–5.5 mmol/l), and a urine dipstick test showed protein+, blood+ and ketones negative.

Blood testing revealed these values (reference range in brackets):
Na 148 mmol/l (135–145 mmol/l);
K 3.7 mmol/l (3.5–5.3 mmol/l);
urea 8.6 mmol/l (2.0–6.0 mmol/l);
creatinine 85 mmol/l (30–90 mmol/l);
total Ca2+ 2.92 mmol/l (2.15–2.55 mmol/l);
PO4 1.06 mmol/l (1.0–1.8 mmol/l);
alkaline phosphatase 170 IU/l (100–400 IU/l);
albumin 25 g/l (24–48 g/l).


CASE :

Question 2

What is this girl’s calcium level when corrected for serum albumin (corrected calcium reference range
2.2–2.7 mmol/l)?
a. 3.22 mmol/l
b. 3.54 mmol/l

c. 2.85 mmol/l
d. 3.85 mmol/l

e. 4.22 mmol/l.

Question 3

What is your first-line treatment for this girl’s symptomatic hypercalcaemia?

a. Sodium bicarbonate infusion
b. 0.9% saline infusion
c. Oral prednisolone
d. Bisphosphonate infusion
e. Furosemide.