Endocrinology Flashcards
1
Q
As acidosis is corrected, what do you have to worry about in DKA rx?
A
Loss of K+ in urine, so should monitor carefully with EKG if necessary
2
Q
When can cerebral edema most commonly happen after treatment initiation?
A
4 to 12 hours
3
Q
What does hypokalemia look like on EKG?
A
U wave and Q-U prolongation
4
Q
What is the Somogyi phenomenon?
A
hypoglycemia leading to rebound hyperglycemia.
So check 2am - if low then it is this
5
Q
What is the rx to somogyi phenomenon?
A
decrease nighttime long-acting insulin
6
Q
What is the Dawn phenomenon in DM?
A
Early AM glucose - check 2am, if still high, than increase nighttime long-acting insulin
7
Q
What do you check on sick days for DM?
A
Check BG frequently, use rapid acting insulins,
8
Q
Describe 2 dose insulin regiment?
A
2 shots/day with short&long acting insulin
2/3 daily in AM, 1/2 in PM
2/3 long-acting, 1/3 short-acting
9
Q
How many hours of TV/computer use/day?
A
2 hrs
10
Q
Obese at age 6, likelihood of obese as adults
A
25%
11
Q
Obese at 12, likelihood of obese as adults
A
75%
12
Q
Most common complication of obesity.
A
Low self-esteem
13
Q
What is the glucose level and serum bicarbonate, effective serum osmolarity in hyperglycemic hyperosmolar syndrome?
A
Glucose >600mg/dL
Serum bicarbonate >15mmol/L, 320 mOsm/L
14
Q
Rx for hyperglycemic hyperosmolar syndrome
A
aggressive hydration with 0.45% saline, insulin later
15
Q
Tall and fat, endocrine or not
A
Obesity
16
Q
Pattern: mildly obese, short, hypoglycemia, micropenis, bone age delay, single maxillary incisor, optic nerve hypoplasia, cleft lip/palate
A
Growth hormone deficiency - hypopituitarism
17
Q
Pattern: floppy baby, mildly retarded, sucking problems, very obese as he grows, hypoplastic penis/scrotum, small testicles and hands/feet, large appetite
A
Prader-willi syndrome
18
Q
Pattern: moderate obesity, round face with short neck, delayed dental eruption, aplasia, short 4th, short 4th metacarpals/metatarsals, extraskeletal calcification, variable hypocalcemia, hyperphosphatemia
A
PseudohypoPTH
Albright hereditary osteodystrophy
19
Q
Pattern: obesity, mental deficiency, retinal dystrophy, polydactyly, syndactyly, brachydactyly, broad, short feet, abnormal kidneys, small penis/tests (hypogonadism)
A
Bardet-Biedl syndrome
A ciliopathic disorder
20
Q
Pattern: obesity later, hypotonia, brushfield spots, clinodactyly with simian crease, endocardial cushion defect, small penis and small testicles
A
Down syndrome
21
Q
Dopamine prolactin relationship
A
DA causes reduction or prolactin
22
Q
DI also causes what?
A
Hypernatremia
23
Q
If hypo or hypernatremia, what labs do you want to do?
A
urine osmolality, serum osmolality, serum and urinary sodium
24
Q
High urine osmolality, low serum osmolality
A
SIADH
25
Low urine osmolality, high serum osmolality
DI
26
Treatment for SIADH
Fluid restrict, 1000cc/m2/day or less
27
What is level of TSH for congenital hypoTH dx?
TSH >25
28
Pattern: prolonged jaundice, severe growth and DD, constipation, coarse features, umbilical hernia and large anterior fontanelle
congenital hypoTH
29
What are endocrine problem should you be aware of in DM patients?
10-30% also have Hashimoto, autoimmune polyglandular syndrome II
30
Pattern: growth fall-off, mild relative weight gain, dry skin/myxedema, delayed relaxation phase of DTRs, coarse hair, mild hair loss
HypoTH
31
Range of TSH in hypoTH
>50, 100, 200
32
Pattern: high TSH, microcytic anemia
HypoTH
33
What dose of levothyroxine for newborn with hypoTH?
10-15 mcg/kg/day
34
What dose of levothyroxine for older pts with hypoTH?
100mcg/m2/day
35
If longstanding or TSH>100, what regiment of levothyroxine do you want to give - aka uptitration plan
Start at 1/4 to 1/3 dose and work up slowly over 3-4 weeks
36
What are three major causes of hyperTH?
1. Graves autoimmune
2. Thyroiditis with release of LT4 from gland in acute phase of Hashimoto or DeQuervain (painful, negative Ab's)
3. Inappropriate use of LTH for weight loss
37
Pattern: jittery, weight loss, agitation, fatigue (cannot sleep), heat intolerance-diaphoresis, proptosis/exophthalmos
Graves hyperTH
38
Pattern:
| +TSH receptor Ab, + Anti TP, +Anti TG
Graves hyperTH - don't be fooled by other antibodies
39
What is high risk side effects of methimazole?
agranulocytosis
40
Rx of Graves
1. Radiactive idoine ablation
2. Thyroid surgical resection
3. Can use idoine (to slow down met of TH), prednisone, propanolol
41
Any solitary nodule needs what kind of workup?
Thyroid US
Uptake scan
Fine needle aspiration
42
Percentage of solitary nodule that is malignant
20%
43
Rapid growth, firm nodule, +FH of medullary CA, history of neck radiation
Thyroid cancer
44
Characteristic of thyroid nodule that is less likely to be cancer.
Cystic nodule, TPO/TG Ab's
45
What initiates genital structure development?
Y chromosome with SRY causing gonads to develop towards testicles
46
What does maternal HCG signal in the fetus for genital structure differentiation?
Testes to secrete T, DHT --> external genital vrilization
47
What stabilizes the Wolffian internal structures?
Testosterone
48
Testes also produce AMH to do what?
induce female structures (Mullerian) to regress
49
At what week is genital structure formed?
14 weeks, shape, but not size
50
What hormone/level/problem seen with 46XY DSD
Subnormal T or DHT
| Insensitive to T
51
What hormone/level/problem see with 46 XX DSD
excess maternal androgens, exogenous androgens, fetal adrenal CAH
52
Ovotestes 46 XX, 46 XY or mosiac
both ovary and testicle
53
Pattern: External female genitalia, internal no uterus, testicles present, no pubic/axillary hair, breast tissue at puberty, no menses
Complete androgen insensitivity - X-linked
54
Pattern: ambiguous genitalia, internal male, no uterus, some pubic hair
Partial androgen insenstivity
55
Sign of low DHT?
genitalia not virilized
56
At puberty for pt with 5 alpha reductase deficiency, why can you see virilization?
Testosterone is excessively high and can virilize
57
Pattern:
| Female salt-wasting, ambiguous genitalia,
21-OH deficiency
58
Pattern:
| Male salt-wasting, early adrenarche/puberty
21-OH deficiency
59
Pattern:
| Teenage girl, hirsutism, menstrual irregularities
21-OH deficiency non-classical
60
Pattern:
| Virilized female, hypertension
11-B hydroxylase deficiency
61
what are complications of untreated 11-B hydroxylase deficiency
advanced bone age and early adrenarche
62
Workup of CAH
```
pre +/- post ACTH stim is gold standard
17 OH progesterone (21 OH deficiency)
DOC (11 beta OH deficiency)
17 OH pregnenolone (3-B hydroxysteroid)
Renin/aldosterone
DHEA, andro, and ACTH
```
63
What is the RX for acute salt wasting-adrenal crisis?
Fluids + hydrocortisone 25mg IM/IV then 100mg/m2/d divided q6 hours
64
What is chronic rx for salt wasting and virilizing?
hydrocortisone 15-25 mg/m2/day, fludocort (mineralcortidoid)
Follow up 17OHP, andro, renin levels q3 months
65
What are causes of Addison's disease (primary adrenal insufficiency)?
1. Autoimmune destruction APS1 (AIRE) adrenal and mucocutaneous candidiasis APS2 (Schmidt) adrenal, DM I, Hashimoto
2. Infection (TB)
3. Adrenal hemorrhage post-infection (WFS)
4. Adrenal hypoplasia congenita
5. Adrenoleukodystrophy
66
Pattern: Salt-losing crisis in neonatal period, 21 OH is not deficient, no virilzation in females, normal male genital development
Adrenal hypoplasia congenita
67
What is genetic cause of adrenal hypoplasia congenita
NROB1 (DAX-1)
68
How do you treat adrenal hypoplasia congenita?
Same treatment as in 21 OH, but lower doses of HC are needed than in CAH
69
Pattern:
| Normal neuro development, then loss of milestones, clumsiness, adrenal insufficiency, grumpy and irritable
Adrenoleukodystrophy
| ABCD1 gene x-linked
70
Pattern:
| Hypotension, hyponatremia, hyperkalemia, acidosis, weight loss, vomiting, hyperpigamenation
Acute severe adrenal insufficiency
71
Causes of Cushing symptom
1. Exogenous steroids
2. Cushing's disease (ACTH production)
3. Adrenal tumor
4. Ectopic expression
72
Pattern: growth failure, truncal obesity, acne/hirsutism, hypertension, purple striae, weakened bones
Cushing syndrome
73
How to test for Cushing's syndrome
Overnight dexamethasone suppression, 1mg at 11pm, check cortisol level at 8am, if no suppression, dx made
24-hour urine for free cortisol
74
Pattern:
| Headache, HTN, palpitations, sweating
Pheochromocytoma - caused by excess catecholamines
75
Dx of pheochromocytoma
Urinary catecholamines, urinary metanephrines, MRI of adrenals, MIBG scan
76
Glucose level to dx hypoG
77
Pattern: new born, low glucose, big tongue, umbilical hernia
Beckwith Wiedemann syndrome
78
Pattern: newborn, low glucose, just got UA catheter
malposition of UA catheter too high up supplying excess glucose to pancreas so reactive response
79
4 hormones that raise blood glucose - how many has to be deficient to cause hypoglycemia
epinephrine, glucagon, cortisol, growth hormone
80
Pattern: Large for gestation age baby, with seizures, hypoG, pre and post prandial, needs lots of glucose
Hyperinsulinism
81
Workup in baby with hypoglycemia
paired insulin/glucose, UA for ketones
82
Pattern: hypoG at random times, no ketones
Hyperinsulinsim or FA oxidation
83
Pattern: hypoG and midline defects or nystagmus
Pituitary (GH/Cortisol)
84
Pattern: hypoG after overnight fast,
GH/cort or FA oxidation defect
85
PTH's net effect
increase Ca, decrease P
86
Vit D's net effect
increase absorption of Ca and P
87
Vit D's effect on PTH
Feed back inhibition on PTH
88
PTH increases Ca by 3 mechanisms
1. increase bone resorption
2. increase Ca resorption from kidney
3. increase Vit D (25 to 1,25 dihyd) to increase gut absorption
89
Calcitonin net effect
decreases Ca blocks osteoclastic resorption.
| it is secreted by C-cells
90
Hypocalcemia s/sx
Tetany, seizures, chvostek (tap on cheek, 7th nerve), trousseau (bp cuff), irritability
91
Hyper/Hypo Ca workup
1) iPTH w/ paired calcium
2) 25, hydroxy & 1,25 dihydroxy
3) Mg (co-factor for PTH secretion) & P
4) Urinary Ca and Creatinine
92
Where is the problem in hypoPTH, pseudoPTH
1) Secretion/production
| 2) bad receptor
93
Labs seen in hypoPTH
low Ca, high PO4, low PTH
94
4 disorders that can causes hypoPTH newborns/infants
1) DiGeorge/velocardiofacial
2) CHARGE assocation
3) Autoimmune polyglandular syndrome I/II
4) Familial hypoPTH (AD)
95
Pattern: cardiac/palatal defects, broad nose, long fingers, T-cell dysfunction (infection), deletion of 22q11 region
DiGeorge/Velocardiofacial
96
Pattern:
| low Ca, high PO4, high PTH (>300)
pseudoPTH
97
What disorder is associated with pseudoPTH
Albright hereditary osteodystrophy
98
Pattern: Short stature, shortened 4th/5th digit, DD, subcutaneous calcification
Alright hereditary osteodystrophy
99
Pattern: low Ca, low P, high Alk Phos
Vit D deficiency
100
Major causes of Vit D deficiency
Decreased intake, absorption (ADEK, CF pt), production (excessive use of sunscreen, dark skinned children at risk)
101
How much VitD needed in first 2 months?
400 IU/day
102
How much to replete VitD deficiency?
1000 IU/day
103
What is calcitriol
activated in Vitamin D - 1,25-dihydroxycholecalciferol or 1,25-dihydroxyvitamin D3
104
Pattern: normal Ca, low P and high urine P, high Alk Phos, bowing of legs, widening cupping and fraying of distal metaphyses
Hypophosphatemic rickets
105
Cause of hypophosphatemic rickets
Genetic - X-linked (not sure what the mutation is)
106
Rx of hypophosphatemic rickets
Calcitriol and P
107
Pattern: polyuria, nephrolithiasis, bone pain, depression, ulcer/nausea
hypercalcemia
108
Pattern/cause:
| Low iPTH, hi Ca, low Phos,
Check PTHrP, most likely high. Secreted by tumor.
109
Pattern/cause:
| Low PTH, hi Ca, hi Phos
Hypervitaminosis D
110
Pattern:
| Normal PTH, hi Ca
Benign familial hypercalcemia fro CASR gene mutation, receptor is insensitive to Ca
111
What genetic disorder should you think of when you see hypercalcemia?
Williams Syndrome
112
Pattern/cause:
| High PTH, elevated Ca, low phos
HyperPTH-parathyroid adenoma
113
Rx for hypercalcemia
1. Hydration
2. furosemide (short term), 3. Glucocorticoids ( short term - incr urinary output), 4. Calcitonin (short term - tachyphylaxis - loses efficacy)
5. Bisphosphonates (holds calcium/phosphorus in bones, more long term)
114
Babies double weight by when and triple by when?
2x by 4 months
| 3x by a year
115
Growth velocity during 1st, 2nd, and 3rd year.
1st year - 24cm/yr
2nd year - 12cm/yr
3rd year - 8cm/yr
(year x GV=24)
116
What is growth velocity in boys and girls at puberty peak?
Puberty peak 10cm/yr (boy)
| 8cm/hr (girl)
117
Pattern:
| Short, delayed bone age, normal labs, following own curve
Constitutional delay of growth AKA late bloomers
118
Pattern:
| Short, normal labs, family is short
Familial short or tall stature
119
Formula for determining potential height of child
If boy - average parents, add 5 inches (13cm from to moms)
| If girl - average parents (subtract 5 inches from dad) +/- 4 inches
120
What should you think of if pt is short
Growth hormone deficiency
121
What genetic mutations associated with growth hormone deficiencies?
HESX, PROPR1, POUF1, GHR (Laron)
122
Pattern:
| Recurrent low blood sugar in infancy, growth slows after 6 months, growth velocity falls from curve
Growth hormone deficiency
123
How do you diagnose GH deficiencies?
IGF-1, IGF-BP3, bone age of hand/wrist (not specific), GH stimulation tests?, Pituitary MRI
124
Side effects of GH
pseudotumor cerebri, scoliosis, SCFE, thyroid and cortisol deficiences
125
6 common syndromic short stature
1. Trisomy 21
2. Turner Syndrome
3. Noonan syndrome
4. Prader-Willi syndrome
5. Pseudo-hypoPTH (Albrights hereditary osteodystrophy)
6. Russell-Silver syndrome
126
Pattern:
Short stature, low hairline, wide spaced nipples, poor breast development, elbow deformity, shortened metacarpal, webbed neck
Turner
127
Pattern:
Short stature, low hairline, shield chest, right sided cardiac (pulmonary stenosis), no renal defects, usually fertile, boy or girl
Noonan syndrome
128
Difference between Turner and Noonan
```
Cardiac problem:
Turner - Coart/bicusp AV; Noonan - pulmonary stenosis
Renal
Turner - yes; Noonan - no
Fertility
Turner - infertile; Noonan - fertile
```
129
3 common symptoms b/w Turner vs Noonan
Short stature, low hairline, shield chest
130
Pattern:
| Short stature, DD, obesity/hyperphagia, hypogonadotropic, hypogonadism (small phallus/testicles, pubertal delay)
Prader-Willi syndrome
131
Pattern:
| Small at birth, limb assymetry, triangular head, cryptoorchidism, no catch-up growth
Russell-Silver
132
7 common causes of tall stature
1. Klinefelter syndrome (47XXY)
2. marfan syndrome
3. Homocystinuria
4. HyperTH (end height not different, just accelerated growth)
5. GH excess (gigantism)
6. Precocious puberty (only temporary)
7. Soto syndrome (cerebral gigantism)
133
Pattern: short stature, triangular facies, low hairline, high arched palate, hypoplastic nipples and increased carrying angle
Turner - missing X
134
What heart and renal conditions is associated with Turner syndrome?
Coarctation of aorta
| Horseshoe kidney
135
What test do you do when you are suspicious for Turner?
Karyotyping, LH/FSH (they should be high because of ovarian failure)
136
What thyroid problem is associated with Turner syndrome?
Antibody positive chronic lymphocytic thyroiditis and hypoTH
137
What is the initial screen for hypoTH?
TSH, then free thyroxine and antithyroid antibodies (thyroperoxidase, antimicrosomal or anithyroglobulin)
138
Presence of Y chromosome in Turners results in what?
Androgenization, gonadal malignancy often requiring ppx gonadectomy
139
What is the height and weight growth like for Cushing syndrome?
Weight is normal to high, attenuation of height growth
140
What are the two thyroxine binding protein? Which is seen in excess in 1 to 8000 individuals? What blood measurement does this problem make inaccurate?
TBG - can be seen in excess
TBPA
TT4 is useless
141
T3 is made in what two ways?
15% by thyroid gland, rest from deiodination from T4
142
What conditions affect deiodination of T3?
Fed state is proportional to
143
Can hypoTH affect bone?
Yes, it can delay bone age
144
What is the most common cause of acquired primary hypoTH?
Chronic lymphocytic thyroiditis (Hashimoto's)
145
What antibodies must be measured to evaluate for lymphocytic thyroiditis?
Again thyroperoxidase enzyme and thyroglobulin
146
If you are still suspicious a child has hypoTH but negative for antithyroid antibodies, what additional testing should you do?
TH US and then technetium or radioactive iodine imaging
147
Describe in DETAIL 6 features of hyperTH
1. Weight loss and increased appetite
2. Decreased strength, muscle weakness, loss of thenar and hypothenar eminence muscle mass, decreased sports performance.
3. Moist skin, thinning hair, skin darkening
4. Poor sleep, nervousness, anxiety, irritability, pruritus, nocturia, hyperactivity, tremors
5. Decreased school performance
6. Decreased menses in girls
148
In addition to high free T4, what other blood value is high in hyperTH?
T3 and TSH IgG in Graves
149
How can you test whether hyperTH is due to subacute thyroiditis or Graves disease?
Radioactive iodine
Subacute thyroiditis - low uptake
Graves disease - high uptake
150
What is the major life-threatening complication of rx for DKA?
Cerebral edema
151
Outline the major steps to Rx of DKA?
1. Hydrate but sparingly
2. Correcting K and insulin
3. Correcting acidosis
152
How should fluid replacement be done in DKA?
Calculated steady replacement of 0.45% or 0.9% with glucose and K as needed
153
How should K replacement be done in DKA?
As soon as child starts urinating, then start K repletion
154
What caution should be taken when correcting peripheral acidosis?
Bicarbonate dissociates in bicarbonate ion and CO2 so can increase intracellular and CNS acidosis to increase risk of cerebral edema
155
What happens with sodium in DKA?
High losses in urine
156
What thyroid problem is seen in 10% of type 1 diabetics?
Chronic lymphocytic thyroiditis - Hashimoto thyroiditis (autoantibodies - like the ones that cause the diabetes)
157
What can pt with Hashimoto thyroiditis eventually develop?
Interestingly HypoTH or Graves disease - ? burns out or acquire additional antibodies
158
What gene is associated with polylglandular autoimmune syndrome type 1?
AIRE
159
What autoimmune disorder is a part of polyglandular autoimmune syndromes?
Type 1 diabetes, candidiasis, hypoPTH, Addison, pernicious anemia, vitiligo
160
What autoimmune problems are associated with polyglandular autoimmune syndrome type 2?
Autoimmune thyroid disease, diabetes, adrenal insufficiency
161
What is the most likely outcome in baby with congenital hypoTH who does not receive treatment within the first few days of life?
Cognitive deficits
162
What are the signs of growth hormone excess?
Widened spaces between teeth, large hands and feet, increased soft-tissue thickening
163
What disorder is upper to lower segment ratio useful in clinical diagnosis?
Marfan syndrome
164
What test should be ordered to work up male precocious puberty?
Serum T, 17-hydroxyprosterone, dehydroepiandosterone, LH/FSH and HCG (which mimics LH to activate Leydig cells to produce T)
165
What tumors makes HCG?
Germ cell in CNS, mediastinum, liver (hepatoblastomas)
166
Adrenoleukodystrophy is caused by what gene mutation and what builds up as a result?
ABCD1, defective oxidation of LCFA in peroxisomes. VLCFA accumulate in myelin, adrenal glands and elsewhere
167
What is the typical pattern on growth chart for familial short stature?
In first 2 years, reach certain growth percentile and follow the curve
168
What age is bone age radiograph useful for predicting adult height?
6 or 7 years
169
What signs can be associated with early menarche?
Thelarche a couple of years before
170
What are signs of gonadal puberty in girl?
Menarche and thelarche (breast bud)
171
What are signs of adrenal puberty (adrenarche or pubarche)?
Adult body odor, pubic hair, acne
172
What is Addison's disease?
Primary adrenal insufficiency
173
What happens to sodium and potassium in Addison disease?
Low serum sodium (cannot hold it without mineralcorticoid), high K (cannot excrete K)
174
What happens to cortisol and ACTH levels in Addison?
Low cortisol, high ACTH
175
Addison is a deficiency in what?
Aldosterone
176
What are complications of aldosterone deficiency?
Dehydration, break down of muscle tissue, hyponatremia, hyperkalemia, elevated BUN and acidosis
177
What are manifestation of ACTH deficiency?
Cortisol deficiency --> weight loss, nausea, inability to maintain blood pressure, low IV volume leads to release of vasopressin
178
Why isn't there hyperkalemia in ACTH deficiency?
Aldosterone is still released
179
Hyperpigmentation accompanies which adrenal axis problem?
Primary adrenal insufficiency (high ACTH)
180
Pattern: firm, enlarged thyroid
Hashimoto, antibody against thyroperoxidase and thyroglobulin
181
What is random glucose level that indicates DM?
>= 200mg/dL
182
HgbA1c level for DM dx
>=6.5%
183
Fasting plasma glucose level for DM dx
>= 126mg/dL
184
what are marker antibodies for DM Type 1
Anti-islet, anti-insulin, Anti-GAD65, Anti-ZnT8A
185
How much B-cell mass must be destroyed before symptoms of diabetes occur?
80-90%
186
What is the honeymoon period in DM type 1?
With insulin, remaining live cells light up and start producing insulin, because they too need insulin to function - but then they are attacked
187
Insulin's 4 roles
1. Increases glucose uptake, along with amino acids and lipids
2. Decreased glucagon production and glycogenolysis
3. Enhances glycogen, protein and fat synthesis
4. Inhibits fat breakdown (lipolysis) and ketogenesis
188
What four hormones increase glucose?
1. Growth hormone
2. Glucagon
3. Epinephrine
4. Cortisol
189
What causes the lactic acidosis in DKA?
Hypertonic dehydration
190
What pH and HCO3 classifies as mild, moderate, severe DKA?
Mild
191
How to get true sodium level in setting of hyperglycemia?
Corrected Na+ = measured Na + 0.016 (measured glucose - 100)
192
For what three reasons do you give insulin in treatment of DKA?
1. Turns off ketogenesis
2. Minimizes osmotic diuresis
3. Enhances tissue substrate utilization
193
Does bicarbonate and phosphate affect recovery rate in DKA?
No
194
What is harm of excessive chloride in DKA Rx?
Prolong acidosis
195
When Na+ is low in obtunded DKA pt, what do you need to think of?
Cerebral edema secondary to SIADH
196
Pattern: HA, decrease in LOC, decreased HR, increased BP 4 to 12 hours after rx initation
Cerebral edema
197
Pattern:
| Small at birth, limb assymetry, triangular head, cryptorchidism, no catch up growh
Russell-silver - Will use GH for them although not deficient
198
Adrenarche
Aka pubarche - development of pubic hair and acne secondary to adrenal androgens
199
Thelarche
Development of breast tissue
200
Gonadarche
Enlargement of testicles or ovaries
201
LH acts on what
gonad to produce T and ovarian androgens
202
FSH acts on what
Gonads to produce estrogens and inhibin
203
Tanner 2 female
Areola - labial hair
204
Tanner 3 female
Mound and smooth contour - hair creeps to mound
205
Tanner 4 female
Nipple starts protruding - fuller hair
206
Tanner 5 female
Full contour - triangular
207
Progression of female development
```
Thelarche - 8 to 13
Adrenarch/pubarch 9-14
Growth spurt
Menarche - 2 yrs after start of puberty average 12.4 yr
T-A-G-me
```
208
Tanner 2 male
testicle enlarge, dropping of testicle
209
Testicle enlargement occurs to what volume
to 4cc
210
Progression of male development
```
Testicular enlargement 9-14
Pubic hair follows 6mo-1yr later
Penile enlargement
Growth spurt Tanner III-IV
Axillary hair - then facial hair
T-H-P-G-A
```
211
Age of puberty that qualifies as precocious
8 for girls
| 9 for boys
212
Precocious puberty workup
LH, FSH, estradiol/testosterone, bone age (often advanced 2-3 years)
213
How do you interpret leuprolide (GNRh) stimulation test
Rise in LH --> PP
No rise - benighn premature thelarche
Suppression - peripheral puberty
214
Pattern:
| Early development, low LH&FSH, high T or E2
Peripheral precocious puberty
215
What is cause of peripheral precocious puberty in boys only?
Constitutively active LH receptor in familial male gonadotropin-independent precocious puberty
216
Pattern:
| Cafe au lait, fibrous dysplasia, prec pub
McCune Albright Syndrome - G protein abnormalitiy
217
Rx of central precocious puberty
Lueprolide, histrellin, GnRH agonists constant will agent, decreased LH/FSH
218
Rx of peripheral puberty
Remove offending agent, block peripheral effects of sex hormones (androgen receptor blockers; estrogen receptor blockers)
219
Delay puberty workup
LH,, FSH, estradiol, testosterone, TSH, chemistry panel, ESR, bone age
220
Age when puberty is considered delayed
13 in girls, 14 in boys
221
Pattern:
| Normal height pre-adolescence, no growth spurt, may have adrenarche, anosmia
Kallman syndrome
| hypogonadotropic hypogonadism
222
Two disorders associated with panhypopit
septo-optic dysplasia, empty sella syndrome
223
2 genetic syndromes associated with delayed puberty
Prader Willi syndrome
| Leptin deficiency
224
Hypergonadotropic hypogonadism 5 causes
1. Mumps orchitis
2. Turner syndrome
3. Klinefelter
4. Functional ovarian failure
5. Trauma, chemo, gonadal removal
225
Pattern:
| Tall stature, eunuchoid proportions, gynecomastia, stalled puberty, testicles noted to be small during puberty
Klinefelter - start developing and then stall
226
What is the most common cause of childhood hypoglycemia?
ketotic hypoglycemia
227
What is the cause of ketotic hypoglycemia?
Imbalance between glucose utilization and production through hepatic, renal glycogenolysis and gluconeogenesis
228
Pattern: fasting hypoglycemia in child less than 8yo
Ketotic hypoglycemia
229
To determine if hyperinsulinism is the cause of low blood glucose, what should you measure and when and what results to anticipate?
At time of hypoglycemia, measure insulin and c-peptide. C-peptide is secreted with insulin so if low when insulin is high, more likely to be excess amt of exogenous insulin.
230
What is best first test for suspected ketotic hypoglyemia?
urine dipstick for ketones
231
Pattern: seizures, hypotonia growth delay, signs of rickets (prominent forehead, rachitic rosary, enlarged wrists and ankles).
Rickets: vit D deficiency
232
Pattern: slowed mentation, stupor, constipation, polyuria, renal calculi, extreme thirst
Hypercalcemia
233
Pattern: neuromuscular irritability like hypocal, nausea, loss of appetite
hypomagnesemia
234
Pattern: muscle weakness and changes in mental status, no neuromuscular irritability
Hypophosphatemia
235
What are some causes of hypercalcemia?
hyperparathyroidism, hypervitaminosis A (stimulates osteoclast activity), hypervitaminosis D, immoblization (osteoclast activation and relative bone resorption)
236
Rx for familial hypophosphatemic rickets?
Oral neutral phosphate salts with calcium (b/c phosphate takes Ca+ into bone)
237
Pattern: 2 year old with unilateral breast tissue
Premature thelarche caused by release of FSH and LH stimulating ovarian estrogen production and will regress by 2 years of age
238
Pattern: 3yo with lump that moves with swallowing and centrally located
Thyroglossal cyst --> surgical consult
239
What is the kinetics of ultrashort-acting insulins
Action begins about 10 to 15 minute after administration, peaks at 1 hr and lasts no longer than 3 to 4 hours
240
What are rules for sick days for type 1 diabetic kid?
1. Check blood glucose every 2 hours
2. check urine or blood ketones every 2 to 4 hours
3. Provide small amounts of normal short-acting insulin every 2 to 3 hours as long as his blood glucose is >100mg/gL and he can consume sugar-containing liquids to stay hydrated
241
Causes of polyuria
DM, renal disease, DI, hyperTH, hyperCal, hypomag
242
Pattern: 11yo boy, nocturnal enuresis, no glycosuria, serum glucose normal, no UTI
hypercalcemia
