Vignettes: Pediatric Cardilogy # 2

Vascular Rings:
I. True/ Complete:
i] Double aortic arch (aa)
ii] Rt aortic arch (aa) with aberrant Lt subclavian artery and ligamentum arteriosum (LA: formerly called the ductus arteriosus)
NB: Do we have Lt aortic arch with vascular rings? Yes, there are 2 rare such examples (not discussed here).
Lest you forget, 30 % pts of TOF have a rt sided aortic arch. This does not mean that they have vascular rings!

II. Incomplete:

a] Anomalous Innominate artery: Innominate branches further left from aa or branches more posteriorly.
b] Aberrant Rt subclavian artery: Rt subclavian arises from descending aorta.
c] Aberrant Lt pulm artery (rare)
NB: Brachio-cephalic was formerly called innominate artery.
Now, Double arotic arch:

i] Associated cardiac malformations: VSD, TOF, Truncus
ii] Syndromes: DiGeorge, VACTERAL, CHARGE
C/Fs: stridor, dysphagia

1. Cyanotic heart diseases:
"5 Ts Do ESP"
1. TOF
2. Tricuspid Atresia
3. TGA
4. TAPVC
5. Truncus Arteriosus
D : DORV (Double Outlet Rt Ventricle)
E: Ebstein
S: Single Ventricle
P: Pulmonary Atresia
2. Cg HD and radiological appearnace:
1. Boot shaped heart: TOF
2. Egg on string appearance: TGA
3. Snowman appearance (fluffy lung fields due to high PBF): TAPVC
4. Small heart on CXR (and increased PBF): Obstructive TAPVR
5. Masive cardiomegaly on CXR (and decreased PBF): Ebstein

3. Cg HD and operations:
1. Norwood: HLHS
2. Jatene (arterial switch): TGA
4. A way to remember:
RVH and decreased PBF: TOF
RVH and increased PBF: TGA, TAPVC, HLHS
Thus, 3 most common cyanotic CgHD are: TGA, TAPVC and HLHS and all 3 show RVH + Increased PBF
5.
10. Differential Dx of PDA:
Aorto-pulmonary window
i) AP window results from incomplete division of aorta and pulm artery during fetal life.
ii) Clinically, large window: As PVR falls, L --> R shunt would develop and would mimic PDA
Small window: May not be very symptomatic.
11. Think of:
Co Aorta: Turner Syndrome
IAA (Interrupted Aortic Arch): DiGeorge Syndrome
Note: IAA means interruption in aortic arch. Thus its clinical presentation may be similar to Co Aorta.
12. AV canal malformation reminds us of its association with Trisomy 21,ie, in Trisomy 21, AVC is seen.
Now what are the associated cardiac malformations which may be seen in pts of AVC: NeoR book says-
a] TOF in 10 % of pts
b] PDA in 10 % of pts
13. Norwood is done for HLHS.
a] Basis: Norwod is done in 3 stages:
1. Stage 1
2. Stage 2: Glenn
3. Stage 3 : Fontan
Now stage 1 : As we know in HLHS, the LV and Lt sided structures like aortic valve and aorta may be hypolpastic.
Thus there is decreased systemic output. The aim is increase systemic output.
* Atrial septectomy to prevent pulm venous congestion
* Create a single ventricle and send blood from here to both pulmones and systemic: How do we do it?
- If VSD is small, dilate it to make a single venticular chamber. If VSD is already bigger, it practically means that both ventricles make a single chamber.
-Cut MPA from its base and attach aorta here: Thus aorta now arises from RV and as we said, the ventricles are already a single chamber which you can call it to be practically Rt ventricle. Note: Aorta is generally hypoplastic in HLHS. Thus in actual, we cut PA and incorporate it in aorta to make a wider aorta: Neo-aorta.
- Now make a shunt between this neo-aorta and PA so that blood goes to lungs also. This shunt is called central shunt.
Alternatively, you can make a shunt between RV and PA: SANO Shunt this is called.

The end-result of stage 1:
There is only one ventricle (RV so as to speak), blood flows out from this single ventricle through Neo-aorta to systemic circulation and lungs( through aorta- PA shunt).
* From above physiology, we can understand one more concept:
Norwood stage 1-Post-operatively- how much O2 sats you will aim for:
Give just enough oxygen so that the baby should be maintaining SPO2 of 70 to 75 (i, PaO2 of 40). Not more than this.
Why: The single ventricle has to supply pulm and systemic blood and it should be in a ratio of 1: 1, ie, Qp and Qs should be 1: 1. If you give higher O2, it will result in pulm vaso-dilation ---> more pulm blood flow, lesser systemic output.
Stage II for HLHS: Bidirectional Glenn is done
As you know, after stage I, ventricles are essentially speaking single ventricle (RV).
In this intermediary procedure, aim is to decrease volume overload on RV until stage III can be done.
So what we do:
a] Shunt ( i assume it is aorto-pulm shunt) is removed.
b] Do Glenn, ie, Connect SVC directly to PA.
So what will happen: A bidirectional shunt allows flow to both RPA and LPA. This increases PBF without increasing volume load on RV.
Stage III: Modified Fontan
What is Fontan: Rerouting of SVC and IVC to PA bypassing the ventricles create separate systemic and pulmonary circulations.
NB: In order to do Fontan, pt must have normal PVR. It is unsuccessful in infants with raised PV resistance.
Note: Glenn and Fontan are the basic procedures for For TA (tricuspid atresia). TA is either corrected by Glenn at 4- 12 mo of age OR by Fontan at 1.5 - 3years of age.
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14. In AV canal malformations: ECHO may show "Goose Neck" sign. This is a typical board q.
15. TOF with VSD: PA are always of decreased size.
TOF with intact ventr septum: PA are of good size.
16. Factors which increase PVR (by vaso-constricting the pulm blood vessels): Dr IL-bawi's pet q
a] Hypoxia
b] Hper-carbia. NB: Hypercarbia causes pulm vaso-constriction but cerebral vaso-dilation.
c] acidosis
d] hypo-thermia
e] Pain
f] catecholamines: eg: epi,dopa,dobutamine
g] endothelin 1: produced by endothelial cells
h] Serotonin
17. Pulmonary vaso-dilators:
opposite of above factors( see q 16), and:
a] Viagra
b) PG
18. DKS( Damus Kaye Stansel):
Dr Ilb says that DKS is done to bypass inoperable AS. He further says that the DKS and Norwood are similar and infact, DKS is the basis of Norwood. In Norwood, infact we go much deeper into aorta, in DKS we donot.DKS is not done for PS.
What the book says about DKS:
a] indications:
Single ventricle with severe subaortic stenosis
DORV with a subpulmonic PS
TGA with severe RV outflow (aortic) obstruction
b] procedure: PA is transceted before the bifurcation,and main Pulm trunk is sewn into aorta. This enables the LV outflow to enter the original PA trunk and then aorta. A pulm homograft is sewn between RV and the pulm artey bifurcation.
19. Co Aorta may be present as a single lesion or in combination with other lesions such as VSD.
A bicuspid aortic valve is present in most pts with Co Aorta.

Peds Endo # 1

Precocious puberty:
is defined as the appearance of breast development prior to age 6 to 7 years in Caucasian girls and prior to 5 to 6 years in African-American girls, as is sexual development before 9 years in boys.Delayed Puberty:If there is no evidence of Sexual Maturity Rating stage 1 genital development by 13 years in girls and 14 years in boys.

1. PCO: Polycystic ovarian syndrome
q 73/08 describes a typical example: A 16-year-old p/w irregular menstrual periods. She had menarche at 11 years of age and experiences menstrual periods every 2 weeks to 3 months. She has noticed increased acne, facial hair growth, and a 20-lb weight gain over the past year .

= This is PCO.
a] Abnormalities observed in patients who have PCOS occur in four key areas: 1) increase in LH secretion, 2) increase in adrenal androgen production, 3) increase in body mass, and 4) onset of adult patterns of insulin resistance.
b] Other criteria often used to support a diagnosis include insulin resistance, perimenarcheal onset, elevated LH-to-FSH ratio, and US abnormalities.
c] Patients are at increased risk for DM, obesity, insulin resistance, infertility, and impaired quality of life.

2. Q 133/06
You see a 6-year-old girl whose mother tells you the child has had adult body odor since the age of 5 years. On physical examination, you note that she has pubic hair with a sexual maturity rating of 3. What kind of personality will you see in this isolated early adrenarche:

Ans: an obese African-American and has a growth rate of 2 inches in the past year

Theory:
i) Breast bud development (Thelarche) is the first sign of puberty in 90% of Caucasian girls, with pubarche (appearance of pubic hair) occurring within 6 months of thelarche. Pubarche may be the first sign of puberty in 10% of these girls and in a larger proportion of African-American and Hispanic girls. Progression through puberty takes 3 to 5 years. The pubertal growth spurt occurs in the earliest phases of female puberty (stage 2 to 3 thelarche); menarche usually occurs later in puberty at about stage 4 thelarche.

ii) Now, African-American girls, particularly those overweight, often have early isolated adrenal puberty (adrenarche) at a relatively early age. The appearance of pubic hair at age 6 years, if associated with a normal growth rate, is considered within the range of normal in this population.
A rapid growth rate or other signs of androgen effect (clitoromegaly) deserve more thorough evaluation for the source and nature of the androgen.

NB: Adrenarche is less common as the first sign of puberty in Caucasian girls, although it may occur.

Progression of full puberty (thelarche) in any girl who also has adrenarche at 6 years of age requires a more substantial evaluation, including bone age radiography.

3. Q 10/2008: During the health supervision visit of a 5-year-old girl, you notice pubic hair (SMR 3). Her height is at the 75th percentile and weight is at the 95th percentile. She has no acne or clitoromegaly. Her mother tells you the girl developed an adult body odor around 8 months ago, and the mother noticed the pubic hair about 6 months ago. She adds that the pubic hair is a little more noticeable now than when she first saw it.
Of the following, the MOST important initial screening study is:
Ans: Bone age radiographs.

This is Adrenarche. Early adrenal puberty (adrenarche) is the usual reason for slow development of pubic and axillary hair without evidence of rapid masculinization (increased growth rate, clitoral enlargement, acne) in children older than 4 years of age. The growth rate is stable in such children, and the only signs of masculinization are usually adult body odor followed by increased pubic and axillary hair, as described for the girl in the vignette. The first step in evaluation of such children is to determine their bone age. The bone age rarely is more than 1 year advanced beyond chronologic age, and if bone radiographs document that fact, usually only clinical follow-up is necessary.
ii) If the bone age radiograph is advanced more than 1 year beyond chronologic age, there is a possibility that the patient may have late-onset congenital adrenal hyperplasia (CAH). This condition is caused most commonly by mild 21-hydroxylase deficiency and is associated with an elevated 17-hydroxyprogesterone value either at baseline or following adrenocorticotropic hormone (ACTH) stimulation.

4. A 8 yrs gilr has developed breast buds (Thelarche). How soon she will achieve menarche? q 149/06

Ans: within 2- 3 yrs of initiation of thelarche.

i) Although the age of normal initial development of breast tissue (thelarche) is a bit earlier than previously assumed, the pace of female puberty has not changed in the past 30 years.

Menarche usually occurs within 2 to 3 years after the onset of thelarche.
Girls typically reach menarche when they have reached sexual maturity rating stage 3 to 4 thelarche (Item C149A), but menarche is a marker of uterine estrogen effect and does not correlate precisely with pubertal staging.

In Caucasian girls, the earliest normal onset of pubertal change now is considered 7 years; for African-American girls, the age is 6 years. Many girls who develop breast tissue early have a slower progression to menarche. The average age at menarche has changed little in the past 30 years (about 12 years for African-American girls and 12.5 years for non-Hispanic Caucasian girls). The earliest normal age for menarche is 9 years. The growth spurt in girls usually occurs at the very beginning of puberty and is influenced by stimulatory low levels of estrogen. By sexual maturity rating stage 3 puberty, the growth rate begins to slow.

5. Question 10/07
A 15-year-old boy comes to your office: expresses concern that he is only 5 ft, 2 in tall and is not competitive in track. P/E: he appears healthy, has a height of 62 in, and weighs 96 lb. His testes are 8 mL in volume bilaterally, there is slight pubertal phallic enlargement, and he has Sexual Maturity Rating 3 pubic hair. He has a small amount of subareolar breast tissue. His last health supervision visit was 2 years ago. He did not have pubic hair at the last visit, and his testes were described as "prepubertal" in size.

= constitutional delayed puberty
It is not exercise-induced growth delay or undernutrition.

Puberty is considered delayed in boys: if there is no testicular enlargement by age 14 years. Unless other features are present on physical examination or history, it is very difficult to to separate boys who have delayed puberty clinically from those who have true hypogonadotropic hypogonadism until failure of pubertal progression persists for several years. After puberty commences, adult testicular size usually is achieved by 3.2±1.8 years. The boy described in the vignette is of normal height for age and is progressing into puberty, based on review of his previous visit and his present appearance. Most likely, he has only mild constitutional delayed puberty. Most boys who have constitutional delayed puberty are underweight and do not have other signs of chronic illness. Exercise and weight loss rarely induce the type of marked growth delay in boys that is seen in girls who are competitive gymnasts or active in ballet. Although Klinefelter syndrome may lead to failure to progress through puberty, the increasing testicular size of the boy in the vignette makes this diagnosis unlikely. Prolactinomas can inhibit pubertal growth, but are not common. Underweight caused by undernutrition must be severe before it inhibits growth and puberty. Only close follow-up is required for a boy who seems to be progressing into puberty. However, if there is evidence of failure to progress into puberty, a full evaluation to determine if the boy has an underlying chronic illness such as celiac disease or inflammatory bowel disease, hypergonadotropic hypogonadism as seen in Klinefelter syndrome, or other endocrine disorder such as a prolactinoma, is indicated.

6. Q 26/ 07A 16-year-old boy is brought to your office by his parents because he wants medicine to help him grow and develop muscles. He has been working out for 1 year without much effect. On physical examination, his height is 63 in (adjusted mid-parental height, 73 in), he weighs 106 lb, and he has testes that are 8 mL in volume. He appears healthy but very young. His father says that he was very small as a boy but was given three injections of growth hormone when he was 16 and grew to his present height of 71 in during late high school and college.
Laboratory test results for the boy are normal, including a complete blood count, erythrocyte sedimentation rate, electrolytes, blood urea nitrogen, creatinine, prolactin, insulin-like growth factor-1, tissue transglutaminase antibody, quantitative immunoglobulin A, free thyroxine, and thyroid-stimulating hormone.
A bone age radiograph is reported to resemble that of a 13-year-old.Of the following, your BEST approach is to:
= Counsel that this is delayed puberty and that he will grow over the next few years.

NB: A normal insulin-like growth factor-1 concentration does not entirely rule out GH deficiency, but the boy's regular growth rate, reasonable height, strong family history for pubertal delay, and signs of puberty make growth hormone deficiency very unlikely.

7. Q 42.07 presents a 16 yrs old f with arrest of puberty and bitemporal visual field defects and asks you what to measure[choose one]: IGF or prolactin or LH or TSH

Answer is: Prolactin

i) The 16-year-old girl described in the vignette has had arrest of pubertal development with failure of menarche for at least 2 years. The bitemporal visual field deficits imply the presence of a large suprasellar mass.

The two most common causes for such a mass lesion are :
pituitary macroadenomas and craniopharyngiomas.

The most common macroadenoma is a prolactinoma.
Elevations of prolactin values could be due to increased prolactin secretion from a prolactinoma or to compression of the pituitary stalk by another pituitary tumor, a craniopharyngioma, or other space-occupying lesion.

Because stalk compression interferes with the dopaminergic inhibitory influences on pituitary release of prolactin, prolactin concentrations of up to 200 mcg/L may be due solely to stalk compression. Prolactin concentrations in that range interfere with normal menstrual cycling and suppress normal puberty.
A large tumor also might interfere with pubertal and other pituitary hormones because of its mass. Accordingly, measuring prolactin will be most helpful in determining whether the girl has a prolactinoma.

ii) Rx of Prolactinomas: usually respond to treatment with medication such as cabergoline; surgery rarely is required.

8. Q 58/07A normal-appearing 9-month-old boy is discovered on routine herniorrhaphy to have bilateral Fallopian tubes and a rudimentary uterus. Biopsy of the gonads performed during this procedure revealed normal testicular tissue. On examination today, his phallus is normal in size and appearance, and his descended testes are both 2 mL in volume.The MOST likely sex chromosome complement for this child is: XY

Note that he will be XY because he has everything needed to be a boy!! That is easy! But there is one additional probelm: why he has fallopian tubes and rudimentary uterus!

Because he has the persistent müllerian duct syndrome.

A peptide hormone (müllerian inhibiting substance [MIS] or anti-müllerian hormone [AMH]) produced by the testis induces regression of the müllerian ducts during fetal development.

These ducts become the fallopian tubes and uterus as well as the upper part of the vagina if there is no regression.

This is an autosomal recessive disorder and is associated with an XY chromosomal pattern.

9. Question 74/07a 1-day-old infant has mild clitoromegaly (Item Q74A) and palpable masses in the labial folds bilaterally. Chromosome studies reveal : the infant has an XY karyotype.Of the following, additional history is MOST likely to reveal that the infant's:

Maternal aunts are infertile.

Now,
i) The baby described in the vignette, who has mild masculinization and palpable gonadal masses with an XY karyotype, is an undervirilized XY infant = male pseudohermaphroditism. Obviously!
No problem so far.

ii) Because gonads are present below the inguinal ligament, there is testicular tissue.
Even with careful evaluation, an underlying cause cannot presently be determined in up to 40% of undervirilized XY infants.

However, one of the most common findings is androgen insensitivity (AIS), which may be complete (CAIS) or partial (PAIS). The finding usually represents a defect in the androgen receptor and is inherited in an X-linked pattern. Therefore, some maternal aunts are likely to have the same disorder, have an XY chromosomal pattern, and be infertile.

10. Question 90/08

A newborn: her genitalia appear abnormal. There is an enlarged clitoris (1.5 cm in length) (Item Q90A) and a small amount of posterior labial fusion. Amniocentesis was performed during pregnancy, and the karyotype was 46,XX.Of the following, the maternal history is MOST likely to reveal exposure to:

Danazol/ ethinyl estradiol/ progesterone in oil/ organo P pesticides

Answer: Danazol

Note that it is a girl with male features. Thus a male harmone is the culprit and among choices, danazol fits the scheme.

i) Danazol, used to treat endometriosis, is a modified progestogen that has an androgenic effect. It has produced virilization in female fetuses.

Exposure after the first trimester can lead to clitoromegaly without midline fusion. Earlier exposure for more than 8 weeks after fertilization may cause labial fusion of varying degrees associated with clitoromegaly, as described for the newborn in the vignette.ii) Ethinyl estradiol is an estrogen commonly found in oral contraceptive agents. It has not been associated with masculinization of female fetuses.

iii) Progesterone in oil is the natural progesterone and has not been associated with masculinization; only substituted synthetic progestins have this effect.

11. Read q 186/07

12. q 202/07 shows a 14 yrs/m who is not getting taller and PREP will lead u to suspect GH def. But be carreful, here the boy is losing wt around the time he is attenuating.

T = Measure tissue transglutaminase antibody

However, severe attenuation of weight gain preceding attenuation of linear growth almost always is a sign of a severe underlying disorder.
NB: Celiac disease is a common cause of short stature associated with underweight. If Ig A levels are normal, measurement of TTG antibody is the best screening study for celiac disease.

NB: Measures of insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein 3 are good screening studies for growth hormone deficiency, although hepatic IGF-1 production also is stimulated by insulin and may be low in underweight children.

12. q 218/07 is interesting and confusing.

CRITERIA OF BRAIN –COOLING

Brain cooling to arrest brain damage is rapidly gaining acceptance in neonatal medicine. Newborn babies who have suffered a hypoxic insult peri-natally could be given the benefit of brain cooling. Time is of essence as it has to be started within 6 hours of birth.
Following are the criteria to brain cool.

Clinical evidence of moderate to severe HIE is defined by criteria A, B and C below:

A. Infant equal or > 36 weeks GA and at least one of the following:
* Apgar score equal or less than 5 at 10 min after birth
* Continued use for resuscitation, including endotracheal or mask ventilation at 10 min after birth.
* Acidosis defined as either umbilical cord pH or any arterial pH within 60 min after birth < 7.00
* Base deficit equal or greater than 16 mMol/L in umbilical cord blood sample or any blood sample within 60 minutes after birth.
B. Infants with moderate to sever encephalopathy consisting of altered state of consciousness ( as shown by lethargy, stupor, or coma) and at least one of the following:
* hypotonia
* Abnormal reflexes , including oculo-motor or papillary abnormalities
*Absent or weak suck.
* Clinical seizures

If the infant is paralyzed, assume an abnormal evaluation for criteria B and proceed to criteria C

C. Infant has an amplitude-integrated EEG/ cerebral function monitor ( aEEG/ CFM) recording of atleast 20 minutes duration that shows either moderately/severely abnormal aEEG background activity (score of 2 or 3) or seizures.

Source: Olympic Cool-Cap system

Pediatric Annotations # 4

1. DDH:
i] Developmental dysplasia of hips is no longer called Congenital Displocation of Hip (CDH) because it has been seen that dislocation of hips can occur after birth also (in infancy).

ii] Click Versus clunk: Click is what you feel, while clunk is one that we hear also. These are not exact definitions as there is no common agreement on these two terms.

iii] Provocative testing includes the Barlow and Ortolani maneuvers, which involve adduction of the flexed hip with gentle posterior force, and abduction of the flexed hip with gentle anterior force, respectively. The Barlow test attempts to identify a dislocatable hip, while the Ortolani exam attempts to relocate a dislocated hip. Due to variations in technique, the Barlow and Ortolani tests have been shown to have a high degree of operator dependence.

iv) Incidence less in cultures (example, India) where there is a practice of mothers carrying the baby on their sides.

2. Note that under 18 years of age, all vaccines are gievn as 0.5 ml with these exceptions:
*Flu shot: under 36 months: 0.25 mL, after 36 to 59 months: 0.5 ml
*Rotateq: 2 mL

3. P/F ratio:
PaO2/ Fio2. Normal >200; In ARDS it is <200.

4. Definition of ARDS:

i] Acute onset or following a catastrophic event
ii] Diffuse B/L disease on CXR
iii] P/F ratio of <200
iv] Non-cardiac origin of pulmonary disease

5. Pathology of ARDS:
a] Increased permeability of alveolar-capillary memebrane: This leads to decreased compliance, decreased FRC, and increased dead space
b] Pulmonary HTN
c] Pulmonary shunting

6. Treatment of ARDS:

a] Vent strategy:
* High PEEP/MAP * Prone position
* iNO (because Pulm HTN occurs in ARDS and hence iNO will help)
* And of course ECMO
NB: In ARDS, another vent strategy is to employ Inverse ratio ventilation. This means: breath starts before expiratory flow from prior breath reaches baseline --> Auto-PEEP with recruitment of alveoli. The Cx of this strategy: #Increased MAP results in decreased cardiac output; # pneumothorax, # Co2 retention. b] Steroids

7. West Nile Fever: culex mosquito spreads it.

8. CPAP Vs BiPAP (Bilevel positive Airways Pressure).

In BiPAP, we set two different pressures for inspiration and expiration.

9. ADHD:

a] While asthma affects 8 % of pediatric population, ADHD has a prevalence of 3- 8 %. Thus ADHD is also a fairly common disease.

b] The meds (stimulants) help 2 components: Inattention and hyperactivity. Over last many years, pharmaco-therapy has gained a wider beneficial acceptance.

c] Questionnaires for testing for ADHD:

1. Connors Scale: cumbersome

2. ADHD Rating Scale (De Paul): easier and popular (our B & D Clinic uses it all the time)

3. Vanderbilt scale also good.

d] ADHD can be diagnosed not generally before 3 years of age. If a for eg a 2 yrs kid hyperactive, and you feel that she/he fits in the mould of ADHD, still you are not going to treat this kid by medication anyway as the dx is generally not made in kids less than 3 years of age.

10. Tests for assessing Development:

A] Based upon information from parents:

1. PEDS ( Parents' Evaluation of Developmental Status)

2. Ages and Stages Questionnaire 3. Infant Development Inventory

B] Based upon eliciting skills from children:

1. DDST II: Denver

2. CAT/ CLAMS Development Assessment: infact our B&D likes it extensively. 0-36 months

3. BINS (Bayley Infant Neurodevelopmental Screen) 3- 24 Mo

4. BDIST ( Battelle Developmental Inventory Screening Test) 12- 96 Mo

Pediatric Annotations # 3

1. In Bacterial meningitis (BM), role of dexamethasone (DXM):

a] Adjunctive therapy with DXM should be considered in selected cases of BM in infants and children. NOT recommended for neonates.

b] DXM has reduced hearing impairment in kids with H influenzae type b meningitis when given with or before starting antimicrobial therapy.

However the same benefit has not been seen with Pneumococcal meningitis (NB: though Pneumococcal menigitis is more known to cuase hearing loss: this is a separate issue, though).

c] When used, DXM should be given immediately before or at the time antibiotics are started. Once ABx have been begun, DXM is unlikely to improve the outcome for patients with BM.

That is why you might have seen the practice of giving IV dexamethasone 20 min before starting IV Antibiotics.

2. Non-Lactose Fermenters: Remember the mnemonic " SPACE"

Serratia, Pseudomonas, Acinetobacter, Citrobacter, Enterobacter.

3. Advise to a mother for her newborn:

a] Start breast-feeding at one breast and change to another after 10-15 min of feeding. When you start breast feeding next time, start from another breast.

b] Exclusive breast-fed babies require multi-vitamin supplements.

c] Exclusively breast-fed babies should be started on Vitamin D 400 Units per day within 2 months of birth.

d] Pattern of supplemental foods:

4- 6 Months:

Is the time to initiate supplemental foods. Start single grain infant cereal with a spoon (2- 3 x/day)

6-9 Months:

Milk: 3- 4 feeds/day (24- 32 Oz /day)

Continue infant cereal (rice, oatmeal, barley; no wheat until 9 Month of age). Also give strained or mashed vegetable or fruits (It is generally advised that weaning should be started with cereals, and then vegetables and then fruit. Because fruit acceptability is good, thus if you start fruit firts, infant will be reluctant to take other stuff).

Tell mom to give fruit juice only when the baby is able to take in a sippy cup (4- 6 Oz max/day).

NB: Each new food be tried alone for 3- 5 days before introducing another new food.

9- 11 Mo:

Milk: 3- 4 feeds per day (16- 32 oz). Also, at 11 -12 months, introduce whole milk in a cup.

If mom wants to give egg to the baby: use egg yolk first, then introduce whole egg (yolk is less allergic).

This is also the age to try strained meats,yogurt, cheese, tofu, Finger foods

12 Mo:Whole milk in a cup

All finger foods are okay, except foods with the potential of choking hazards (whole grape, nuts, popcorn).

To quantify, milk intake for various age:

0-2 Mo: 16- 32 Oz

2- 4 Mo: 20-3 6Oz

4- 6 Mo: 24- 40 Oz

6- 9 Mo: 24- 32 Oz

9- 11 Mo: 16- 32 Oz

12 Mo: 16- 20 Oz

Hygiene practices in the newbron : Instruct the mom while she is taking care of a newly discharged neonate:

a] Use only lukeworm water on your baby's face. A mild soap such as Dove unscented, Neutrogena, Basis or Leve 2000 may be used on the rest of the body.Do not use ivory soap. Sponge bathe until the umbilical cord comes off.

b] Care of umb cord: clean the umb cord at every diaper change with alcohol pads/swabs/ or soaked with a q-tip. Make sure to get down to the base of the cord. The cord usually falls off in 10-21 days.

When the cord drops off, the baby may begin to take the tub baths.

c] Lotions are unnecessary if you are using a moisturized soap as previously mentioned. It may be used if the skin is excessively dry.

4. Isolation precautions:

a] Parvo virus: Fifth disease: When rash appears, patient is no longer contagious.

NB: In sicklers infected with Parvo virus: Longer isolation (droplet precaution) is needed ,because patient sheds virus for longer time.

b] Infectious mononucleosis:

No isolation required. But physical rest is imposed to avoid risk of splenic rupture. The exact guidelines are:

Restriction in physical activity is needed for 4 weeks whether spleen is palpable or not. After 4 weeks, the Pediatrician examines the patient again, if spleen is still palpable: more physical rest is imposed.

5. Risk of infection following a deer tick bite of developing Lyme disaease: 2- 4 % in Long Island,NY. It is 95 % in shelter island.

6. Two ways to diagnose Lyme disease:

a] ELISA test: 10 % false positives

b] Western blot: confirmatory

7. Ch. Lyme disease does not need antibiotic treatment.

8. Deer tick: is the vector for Lyme disease

All ticks have 8 legs, so do spiders. Thus if a mom brings a bug to the ER or your office, that does not have 8 legs, it is not a tick.

Deer Tick has a SCUTUM (dark colored head like). Thus if you see a scutum, it is deer tick.

Some types of ticks:

Deer tick: Ixodes sp

Dog tick: Dermacenter sp

Lone star tick

9. A mom calls your office or ER and she is concerned about a tick-bite and hence Lyme disease, ask her to bring the tick if possible): At your office or ER you figure out that it is a deer tick. Thus treatment of the patient is based upon:

## If tick was attached for >72 hrs (index of engorgement): Give once single dose of doxycycline prophylaxis.

10. Post -Bone Marrow transplant:

The main concerns are:

0- 1 Month after the BM transpalnt: : Neutropenia,line infections, nosocomial infection, resp infection are the main concerns.

1- 6 Mo: CMV, EBV, PCP

> 6 Mo: Fungal, CMV

In transpalnt patients:

a] Always try to identify the infections in recepient (i.e,patient: which you will of course as described above), but also in donors (like, whether donor was + for CMV or not!)

b] Always place PPD before transplant.

11. Ranitidine can cause neutropenia.

12. Proning in patients of ARDS:

In PICU, you see that patients of ARDS are made to lie down in prone position (from usual supine). This helps re-distribut the debris of lungs, and helps improve V/Q mismatch.

13. Landau- Kleffner syndrome (LKS) and KLS (Kleine Levin Syndrome).

14. Dysautonomia is a disorder of peripheral nervous system. There are 2 classes: familial dysautonomia (Riley day syndrome) or a non-familial dysautomomia.

15. Cocaine: As we know it is a substance known for its abuse potential.

Whereas, Cockayne Syndrome: AR, DNA repair defect

Edward Alfred Cockayne (1880–1956), after whom this disease is named, was a London physician who concentrated particularly on hereditary diseases of children.

Cockayne syndrome is a rare inherited disorder in which people are sensitive to sunlight, have short stature, and have the appearance of premature aging. In the classical form of Cockayne syndrome (Type I), the symptoms are progressive and typically become apparent after the age of 1 year. An early onset or congenital form of Cockayne syndrome (Type II) is apparent at birth.

NB: Interestingly, unlike other DNA repair diseases, Cockayne syndrome is not linked to cancer.

16. Do you know that melatonin has a role to play in sleep! I know an infant who slept very less when he was about 6 months old. After a lot of frustation and non-intervention recommended by his peditrician, parents insisted for Melatonin levels measurement. They were found to be low, the patient was started on sublingual mealtonin supplement as BID doses and he showed inmprovement.

17. Amantadine: for influ A; Oseltamivir: for flu B

Note: amantadine not effective for flu B.

18. Tick-borne diseases in USA:

The ticks that transmit these diseases belong to the family Ixodidae, also known as hard ticks.

Remember 3 types of above ticks: 1.Blacklegged tick/ Ixodes scapularis 2. Dog tick/ Dermacenter variabilis 3. Lone star tick/ Amblyomma americanum


20. Hurler Syndrome:

a] is type I MPS. AR
b] X ray findings: Oar-shaped ribs: narrow at the origin, become wider as they go towards insertion. 'Beaking' of vertebrae. Narrowing of lower ileal segment.

c] Urine screening reveals metachromasia because of excretion of Glycosaminoglycans. Dx is established by measuring activity of the lysosomal alpha-iduronidase in peripheral WBcs.

d] Rx: Children with severe form of MPS benefit from 'STEM cell Transplants'. This results in attenuation of neurological progression, improved mobility and quality of life, and longer lifespan. Children with attenuated form of the disease can now be trated with specific enzyme replacement therapy: Laronidase (brand name Aldurazyme) . It improves mobility, exercise tolerance, and cardiac function.
Theapies for other MPS are or soon will be available, including type 6 (Marateaux- Lamy disease: galsulfase, brand name Naglazyme), and type 2 (Hunter Syndrome: idursulfase, brand name Elaprase).