Neonatal Case Conference Hypoglycemia (Brandau) - MT Flashcards Preview

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Flashcards in Neonatal Case Conference Hypoglycemia (Brandau) - MT Deck (21):
1

What is an infant at risk for if it has a 2 vessel umbilical cord (single umbilicl artery)

 

other congenital defects

2

If an infant hs a single ubilical artery, ultrasound should be used to screen what area of the body?

Abdomen and Kidneys

3

Prematurity, intrauterine growth retardation, perinatal asphyxia, and Sepsis can cause persistent hypoglycemia in infants. What are some other causes in infants? (x6) 

1. infant of diabetic mother

2. hypothermia

3. disorders of gluconeogenesis/glycogenolysis

4. hyperinsulinism

5. primary and secndary adrenal insufficiency

6. congenital hypopituitarism

4

What are genetic syndromes associated with a micropenis? (x3)

Smith-Lemli-Opitz syndrome

Kallmann syndrome

Prader - willi syndrome

5

An infant presents with microcephaly, low set ears, and micrognathia. In addition to being concerned about the Cardiac defects that appear 50% of the time as endocardial defects, what other abnormality occur in this syndrome 70% of the time?

- What is this syndrome?

- Genital abnormalities (cryptoorchism, micropenis, hypoplastic scrotum) occur 70% of the time

- Smith lemli opitz syndrome

6

What syndrome does this baby have?

What defects do these pictures show?

Q image thumb

- Smith Lemli opitz syndrome

- micropenis, low set ears, microcephaly, Mandibular hypoplasia (micrognathia)

7

A patient has presented to you with loss of the sense of smell. He has an unusually small penis with undescended testes.

Another patient presents with a history of no menstruations at puberty and no breast development and has also lost her sense of smell.

What syndrome do both of these patients probably have?

What causes this condition?

what is another name for it?

- Kallmann syndrome

- decreased production of hormones that direct sexual development (testosterone, LH, FSH)

- hypogonadotropic hypogonadism

8

In addition to the hearing loss, what can happen to the kidneys, mouth, and limb coordination of a patient with Kallmann syndrome?

 

- unilateral renal agenesis

- Cleft palate

- Bimanual synkinesis (movement of one hand mirrors the other)

9

1. What syndrome does this child have?

2. At birth what characterized this child and indicated he had the syndrome he currently suffers from? (x5)

Q image thumb

1. Prader-Willi syndrome

2. He likely had hypotonia, failure to thrive, feeding difficulties, hypogonadism, developmental delays

10

Prader willi syndrome is the most common form of what disorder?

syndromic obesity

11

Around the age of 2 patients with prader willi syndrome will with behavioral problems, and characteristic facial features.

What are the two recognizable signs that appear at this time?

A deficiency in what hormone is also noted?

- Hyperphagia and Obesity

- Growth hormone resulting in short stature and small hands/feet

12

What are three common endocrine causes of Micropenis in newborn? 

1. Hypogonadotrophic hypogonadism secondary to pituitary and hypothalamic lesions

2. Primary testicular failure

3. Apparent micropenis secondary to virilization of female exterinal genetalia secondary to 21alph hydroxylase and 11beta hydroxylase deficiency

13

Congenital Hypopituitarism is caused by genetic defects controlling transcription factors for what?

differentiation of anterior pituitary

14

GH deficiency in congenital hypopituitarism has little effect on fetal growth that is dependent on what 3 molecules?

- Insulin, IGF-1, IGF-2

15

GH deficiency in newborns can include what metabolic disturbance, and what breakdown products to accumulate? (2 answers)

- Hypoglycemia

- Jaundice of direct or indirect bilirubin

16

LH andFSH deficiency in newborn males can present with what 3 things?

1. Micropenis

2. testicular hypoplasia

3. undescended testes

17

ACTH deficiency is almost always associated with cortisol deficiency resulting in what metabolic deficit?

Hypoglycemia

18

How does ACTH deficiency affect aldosterone levels?

It doesn't, hence there is no salt wasting or hyperkalemia

19

Manifestation of TSH deficiency are similar to those of congenital hypothyroidism. What are they? (x7)

- Large fontanelle

- lethargy

- constipation

- Hoarse cry

- Hypotonia

- Hypothermia

- Jaundice

20

Causes of congenital hypopituitarism (x9)

- Head injury

- congenital

- inflammation

- infection

- lesions

- surgery

- TB

- Radiographic therapy

- tumor

21