Neonatal Hypoglycaemia and Precocious Puberty Flashcards

1
Q

What is the definition of hypoglycaemia?

A

Abnormally low plasma glucose level in a neonate, enough to cause seconday naurologicla injury (<2.5mmol/l).

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2
Q

What is the mechanism of hypoglycaemia in a neonate?

A

May be transient (<1/52) or prolonged. Due to any condition that increases metabolic demand. Neonates have impaired ketogenesis and hepatic glycogen stores, therefor eincreased demand will quickly lead to a fall in glucose. Highest risk at 6-12h post delvery. Majority due to hyperinsulinism .

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3
Q

What are the transient causes of neonatal hypoglycaemia?

A

prematurity, IUGR, prenatal asphyxia, Beckwyth Wiedemann syndrome, hypothermia, sepsis, maternal diabetes, erythroblastosis fetalis (increased insulin) B agonist tocolytics (terbutaline)

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4
Q

What are the prolonged causes of neonatal hypoglycaemia?

A

Congenital hyperinsulinism, inborn errors of metabolism, nesidioblastosis/islet cell adenoma.

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5
Q

What is the epidemiology of neonatal hypoglycaemia?

A

10% neonates if first feed after 6h.

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6
Q

What would you find in history and exam of a neonate with hypoglycaemia?

A

Irritability, tremulousness, birsk Moro, poor feeding, aopnea, tachypnea, distress, lethargy, drowsy, low temperature, seizures.

Specific: macrosomia (hyperinsulinism) jaundice (galactosaemia and sepsis) hepatomegaly (GSDs).

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7
Q

What investigations would you do for hypoglycaemia?

A

Routine bloods: low glucose and high ketones.

Specific bloods: Insulin (hperinsulinism) c-peptide, cortisol (hypoadrenalism) GH (hypopitiutarism) lactate/pyruvate (GSDs) ketone bodies, NH4.

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8
Q

WHat is the management of hypoglycaemia?

A

Mild symptoms: if no contraindications, managed with increased enteral feeds. Monitor blood sugar.

Severe symptoms:

  1. IV dextrose bolus 2-5mg/kg 10% DX followed by infusion. 5-8mg/kg/min.
  2. Volume increase if there is need for increased glucose (not strength)
  3. Dextrose >12.5% infused through central venous line not peripheral.
  4. Reqiring >10mg/kg/min IV consider glucagon IM/IV, diazoxide/ocreotide (reduce insulin), hydrocortisone if adrenal insuficiency suspected.

Surgery may be required for islet cell adenoma/mesidioblastosis. Caproscopic or open pancreatectomy.

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9
Q

What are the complications/ prognosis of hypoglycaemia?

A

Untreated, low head size, IQ, brain anomalies.50% survivors have long term cmx.

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10
Q

What is the definition of Precocious puberty (partial)?

A

Early partial sexual developpment, minimal development in absence of other pubertal stigmata.

Premature thelarche: isolared breast. Adrenarche: pubic hair. Menarche: bleed

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11
Q

What is the aetiology of Precocious puberty (partial)?

A

PT: period of relatively high but decreasing activity of HPO axis at 6m-2y.

PA: Early maturation of normal androgen secretion

PM: Spontaneous regression of ovarian cyst, hypothyroid, McAlbSyn (see complete)

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12
Q

What is the epidemiology of Precocious puberty (partial)?

A

PT relatively commoni n Afrocaribbean. PA also.

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13
Q

What is the history/ exam of Precocious puberty (partial)?

A

PT: isolated unilateral or bilateral breast development between 6m-2y. Non progressive and associated with no areolar development.

PA: Self limiting, inccrease in growth rate.

PM: May occur physiologically postnatally. Distinguish from foul smelling blood discharfe, form trauma infection or abuse. And bleeding from the urinary tract. Detailed hx and exmaination required.

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14
Q

Which investigations should be done in Precocious puberty (partial)?

A

General:may not be necessary in PT.

Pathology: LSH/FSH/Oes levels, GNRH/ACTH testing

Radiology: USS ovaries and uterus, bone age radiograph

Culture of urine, vagina discharge.

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15
Q

What is the management of Precocious puberty (partial)?

A

Specialist paediatric endocrinologist. No intervention usually requried

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16
Q

What are the complications of Precocious puberty (partial)?

A

PT: can progress to precocious puberty.

PA: may progress to PCOS.

Isolated PM: depends on cause.

Psychosocial impact.

17
Q

What is the prognosis in Precocious puberty (partial)?

A

Usually good. Normal puberty and stature in most.

18
Q

What is Precocious puberty (complete)?

A

Early onset of pubery (F period <10 change <8, M change <9)

19
Q

What is the central aetiologies of Precocious puberty (complete)?

A

Normal development chronologically early. Hyopthalamic GnRH secretion

o Idiopathic

o CNS dysfunction

§ Hypothalamic harmatome – mass containing GnRH

§ Destruction from tumors craniopharyngioma, ganglioneur

§ SOL such as arachnoid cysts

§ Hydrocephalus

§ Infections

§ Head trauma

20
Q

What is the peripheral aetiologies of Precocious puberty (complete)?

A

Stimulation by a hormone other than hypothalamic GnRH

o Inappropriate sex steroid synthesis

§ CAH

§ Tumors (adrenal, ovarian granulosa, testicular Leydig cell)

§ McCune Albright syndrome – hyperpigmented lesions similar to CAL spots, polostotic fibrous dysplasia and endocrine abnormalities such as MTN and precocious puberty and amenhorrea/galactorrhea.

o Exogenous sex steroids

§ OCP

§ Topical oestrogens

21
Q

What is the epidemiology of Precocious puberty (complete)?

A

F>M. 8-25% girls in West.

22
Q

What is in the Hx/ Exam of Precocious puberty (complete)?

A

General: early development of pubertal stages, must perofrm full CN exam

Specific: signs of disease i.e. ICP, pigmented lesions in McCAlb syndrome.

23
Q

What investigations are used in Precocious Puberty (Complete)?

A

Blood LH, FSH , oestrogen, LHRH

CT/MRI if indicated. USS uterus and ovaries. Wrist XR for bone age.

24
Q

What is the management of Precocious puberty (complete)?

A

specialist paediatric endocrinologist.

25
Q

What are the complications and prognosis of Precocious puberty (complete)?

A

Bone maturation and short stature, psychological issues. Pgx depends on Aet.