endocrine- ILA + lecs Flashcards
Diagnosis: Child presents with weight loss; polyuria and polydipsia (?); and 1 day hx of less PU, stomach ache and unresponsiveness. GCS is 8 at ED. She appears 5% dehydrated
T1DM DKA
How would you manage T1DM in a child?
Correct dehydration evenly over 48 hours. This will correct the dehydration and dilute the hyperglycaemia and the ketones. Correcting it faster increases the risk of cerebral oedema.
Give a fixed rate insulin infusion. This allows cells to start using glucose again. This in turn switches off the production of ketones.
Other important principles:
Avoid fluid boluses to minimise the risk of cerebral oedema, unless required for resuscitation.
Treat underlying triggers, for example with antibiotics for septic patients.
Prevent hypoglycaemia with IV dextrose once blood glucose falls below 14mmol/l.
Add potassium to IV fluids and monitor serum potassium closely.
Monitor for signs of cerebral oedema.
Monitor glucose, ketones and pH to assess their progress and determine when to switch to subcutaneous insulin.
What causes reduced GCS in DKA and how do you treat it?
Raised ICP- To reduce ICP can give hypotonic saline or mannitol
How do manage shock from DKA?
If in shock give 0.9% saline. Dehydration then corrected over 48 hrs with saline, Kcl and insulin.
Difference between adults and children DKA management?
In contrast to adults it is permissible to start insulin before having a potassium level back on your patient. Plus give IV fluids before insulin
What are the targets for ketones, bicarb, glucose and potassium in DKA? What do you do if these are not achieved?
The recommended targets are a reduction of the blood ketone concentration by 0.5 mmol/L/hour, an increase of the venous bicarbonate by 3.0 mmol/L/hour, a reduction of capillary blood glucose by 3.0 mmol/L/hour and to maintain K+ between 4.0 and 5.5 mmol/L. If these rates are not achieved then the insulin infusion rate should be increased.
What fluids do you give a child in DKA initially and then after 48 hrs?
- Saline .9%, 10ml/ kg (to avoid cerbral oedema) bolus
2. 0.9% saline plus glucose for maintain plus KCl
What is the follow up mx for DkA?
Check ups for htn, renal disease, eyes, feet, circulation
Check for autoimmune problems such as thyroid and coeliac
S/C insulin
Lifestyle advice
Diagnose neonate presenting with high TSH and low weight
Congenital hypothyroidism
What is the most likely cause of congenital hypothyroidism:-
a) worldwide?
b) In the UK?
c) In a consanguineous pedigree -incest?
a) Iodine deficiency
b) Maldescent of the thyroid (thyroid fails to descend from the sublingual position to larynx in fetal development)
c) Dyshormonogenesis- an inborn error of thyroid hormone synthesis
How could you differentiate clinically between maldescent of the thyroid and other causes of congenital hypothyroisism?
In maldescent the thyroid remains as a lingual mass
What condition has the introduction of screening helped prevent?
Congenital hypothyroidism in olden days was kretinism are usually detected with routine neonate biochemical screening (guthrie test) - looks for raised TSH
Symptoms of congential hypothyroisim?
faltering growth, feeding problems, jaundice, constipation, pale skin, large tongue, hoarse cry, goitre, umbiblical hernia, delayed development, hypotonia, macro Glossia
How would CH be treated?
Levothyroxine
What are the differential diagnoses for a collapsed and shocked neonate?
Pneumothorax
Patent ductus arteriosum – give prostaglandins
Metabolic eg hypoglycaemia, electrolyte imbalance eg raised ammonia means urea cycle problem
Non accidental injury- shaken baby
Infection- sepsis
Obstruction
What is your immediate management of neonate in shock?
Clear airway, oxygen, ventilate Septic screen Obs, blood gas and glucose Weight Keep warm Do platelt and netrophil count, U+Es (lactate, urea, creatinne, elctrolytes), cultures, CRP
What does pH of 7.36, low sodium, high potassium,low co2 and low o2, low base excess and low HCO3 mean on the blood gas?
metabolic acidosis
potassium is high as is exchanged for H+ ions to incrase pH
Causes in neonates of metabolic acidosis?
Cardiac lesion, congenital adrenal hyperplasia, hypovalaemic shock and sepsis will cause metabolic acidosis
What is congenital adrenal hyperplasia?
Congenital adrenal hyperplasia most commonly deficient in enzyme that means no glucocorticoids and mineralcoritcoids (aldosterone - low sodium and high potassium)
Diagnose with low sodium, high K, metabolic acidosis and hypoglycaemia
Difference between gluco and mineralcorticoids?
Glucocorticoids are cortisol for when unwell
Mineracorticosoids eg aldosterone keep sodium, chloride and potassium (salts) IN CONTROL
How would you manage CAH in the first 24 hours a?
IV saline, glucose and hydrocortisone initially. Later give flusdocortisone once had hydrocortisone. Give salbutamol in children to reduce potassium.
How would you manage CAH long term
Long term have glucocorticoids (hydrocortisone) to suppress ACTH levels and allow normal growth. Give long term mineralcorticoids ( fludrocortisone) if there is salt loss. Monitor growth, skeletal maturation and plasma androgens.
Fix genitalia later
How do baby girls present with CAH?
With virilisaion of the external genitalia in female infants with clitoral hypertrophy
In male penis enlarged and scrotum pigmented - usually missed and so present with a salt crisis: vomiting and weight loss or with precocious puberty
What is antenatal treatment for CAH ?
If strong Fhx can offer mother dexamethasone tablets
What is the SE on female baby if womans takes dexa in pregnancy?
Dexa can cause organomegaly in female genitalia
What are the determinants of growth?
Parent pheno and genotype Nutrition Pregnancy Organ integrity Psycho social GH and hormones
What is chondrogenesis?
process by which cartilage is developed, achondroplasia and hypochondroplasia are disorders of this process which may lead to dawarfism
How do adults versus newborns grow?
- Newborns: larger head, smaller mandible, short neck, chest rounded, abdomen prominent, limbs short
- Adults: relative growth of limbs compared to trunk
What are the normal ranges on a growth chart?
normal ranges are between 3rd and 97th centile.
What are the different types of head growth problems?
Microcephaly – familial or can be cause by hypoxia or infection
Macrocephaly – raised ICP
Craniosynsotosis- skull bones start to fuse prematurely- head shape distorted
Plagiocephaly – flattening head
When are the fastest stages of growth and when does growth end?
Fastest growth rate in utero and infancy
• Growth ends with fusion of epiphyses (Oestrogen effect)
what are the first stages of puberty in girls vs boys?
Breast buds = first in girls
Increase in testicular volume = first in boys
When does puberty start (early + late) in boys and girls?
Female puberty: early: 8 late: over 13
Male puberty early start: before 9 late start: after 14
What are the Tanner stages in boys?
I Prepubertal: No pubic hair • Testicular length <2.5 cm • Testicular volume <3.0 mL
II • Sparse growth of slightly curly pubic hair, mainly base of penis • Testes > 3 mL (testes enlargement first thing) (>2.5 cm in longest diameter) • Scrotum thinning and reddening
III • Thicker, curlier hair spread to mons pubis • Growth of penis in width and length; further growth of testes
IV • Adult-type hair, not yet spread to medial surface of thighs • Penis further enlarged; testes larger, darker scrotal skin colour
V • Adult-type hair spread to medial surface of thighs • Genitalia adult size and shape
