DIP Episode 223 - NBME Peds Shelf Add-On: The HY Newborn

Okay, welcome. My name is Devine, I'm a resident. This is episode 223 of the Divine Intervention Podcasts. And in this podcast, I'll be essentially the topic is this is like an add-on podcast for the Pete shelf. You definitely should listen to this before the Pete shelf. It's just a podcast that focuses on some high-youtes stuff related to newborns and most of this I'll introduce to your clinical vignettes and talk about like high-youtes associations and things that you're expected to know for your shelf. And I'll try to be short and sweet with this but again this is an ultra high-yout podcast for your pediatric shelf and also for step two CK because stuff from the Pete shelf is tested on the on the exam. And the thing is this like this podcast actually has a slide deck so just make sure you don't lose it from the website, divininginterventionpodcast.com. So podcasts with an SADN.com. Okay, so let's just jump right into it. So the first question right so what is the high-youtes association right? So the first question is like a newborn with a type 2 RTA and it tells you that you find increased levels of multiple substances in the urine and this child has like a rightful arm that has a much smaller circumference than his right arm. If you see that what are you thinking about on an in-been? I'll really hope you're thinking about a Fankoni syndrome right?

So remember Fankoni syndrome those kids tend to have like a bunch of things going on right so they can have a type 2 RTA right so because the approximate convoluted tubules most of the transporters don't work right so they can get a type 2 RTA kind of business and remember in that type 2 RTA they'll have a normal anion gap that abolycasidosis and the urine peach will be less than 5.5 because the alpha-interculated cells still work just fine. And then the rightful arm being smaller than the right arm it's usually because they have like an absent radius right so just remember thumb-band radius of normalities with Fankoni syndrome. Another disease you may find on an in-been exam they may give you a question about a child that has like PTKI and like on physical exam and also has an absent radius. I want to think about a syndrome known as TAR syndrome that's thrombocytopenia absent radius syndrome that's a high-yield thing to keep in mind for exams. Alternatively they can give you a question about a child that has a human giuma right like a red resolution like on the interglutial folds and then they tell you that this child has PTKI and physical exam then you want to think about something called the Casa Batch Merit syndrome right it's basically just a human giuma that's sequestral split so you can cause thrombocytopenia so you may find PTKI on an exam.

I know some of the stuff I was seeing this podcast you'd be like oh no I don't know about this well don't worry just take them off in-been exams and you see what I mean.

Okay so now let's and remember that Fankoni syndrome is also as you know Caffeoli spots right so remember Caffeoli spots a hyper pigmented macules you can find them in in Fankoni syndrome you can find them in neurofibromatosis type one contrast that with the hypo pigmented macules of the skin that you find in kids with you find in kids where I thought the name of this disorder tuberous sclerosis right those are your Ashley spots okay now the next question says newborn with congenital neck lymphedema and a dimo of the hands and feet this one's pretty easy this is toner syndrome right remember that cystic hygroma that webbing of the neck it's actually an example of congenital lymphedema so instead of writing cystic hygroma which the MbMe knows every human being has memorized they may put congenital lymphedema as an answer that's something when you keep on the back of your mind and remember toner syndrome right don't forget your cardiac associations by cosperiodic valve right and um, cortisional deodor and then they also have like this uh, renal association with horseshoe kidneys waiting for your pose of the kidneys are fused and they are stuck on the infirm as in terricardary right and then what if they give you a question about a newborn that has a super pubic mass on exam and the lower extremities they tell you that oh you try to stimulate these lower extremities and the newborn does in move his feet if you see that I'll really hope you're thinking about some kind of neuro tube defect remember another buzzword for neuro tube defects on exams is the term spinal dysraphysin spinal dysraphysin is the same thing as a neuro tube defect right and the thing is if you have a particularly bad neuro tube defect C for example like a lumbus sacral mylomaningo seal that kid will have like parplegia right so the lower extremities essentially will not work right and he can have like

a urinary retention because they essentially have some kind of my lopathy right and remember like again a neuro tube defect you can prevent that by you know giving mom adequate amounts of folic acid right and don't forget that a lumbus sacral mylomaningo seal has a well-established mbmi association with a key area two malformation and anal key area two malformation contrast that with an anal key area one malformation that has an association with with syringo myria so that's something very high you'd want to keep at the back of your mind on exams and then overlapping the next position says overlapping overlapping digits a rocker bottom fits the child dies 10 days after birth this was pretty easy this is tried so many 18 right this is an Edward syndrome right so remember they tend to have overlapping digits rocker bottom feet these child's these children tend to not leave very long right and remember if you don't watch screen the alpha-fiddle protein the astral and and beta-hecgob will be decreased in those gates that's something high you know and then the inhibition is kind of like popping the air but usually normal on mbmi's contrast that with Down syndrome where the beta-hecg and the inhibin will be increased or the other things will be decreased right so that those are high your quartz cranial results you want to know for your exam and I know some of you can be saying okay divine how do I differentiate try so many 18 which is Edward syndrome from try so many 13 which is pato syndrome do we do that on an mbmi is essentially they may have a rocker bottom feet but they will tell you that the kid has like cleft cleft palate if you see that think more about pato syndrome which is try so many 13 okay so the next question just talks about both what pathophysiology so this is one way your friends at the mbmi kind of integrate embryology with the peach shelf and step two CK

so these are things you sort of kind of want to know right so for example if they give you a question about a newborn with a squirrel swelling and they tell you that oh that this finding a transceal we need to the pen light this one's pretty easy you want to be thinking about like a hydrocel right a hydrocel right so a hydrocel is where you have like you know like failed closure right like there's still like potential of the person of the tunicavaginatus right and one I guess I didn't throw in here but if you see a newborn you know with like big scrotum and although usually they make this move like a bigger bigger person like like a person in 20s 30s having marital problems because they're not able to have kids and then they tell you that you have like this bug or worm sensation in the scrotum right you want to think about a varicose seal right that usually arises from problems with the with the development of the pump in a form plexus right so that's something you want to keep in mind and then if they give you a question about like a midline neck mass that moves with swallowing I'll say that again midline neck mass that moves with swallowing if you see that you absolutely want to think about a thyraglossodoxist right so remember a thyraglossodoxist in a rise where you have failed obliteration of the thyraglossodox it's a midline mass and it moves with swallowing that's very high already moves with swallowing right remember the thyraglossodox develops from the base of the tongue the foreman's eca right okay now the next one neck mass lateral to the midline does not move with swallowing again this one should be pretty easy this is just a very simple arm bring your cleft bring your cleft cyst right so again it's lateral to the midline and it does not move with swallowing right if you see this again think about a bring your cleft cyst it's a problem with ectodermal deve

lopment versus a thyraglossodoxist that's a problem with endodermal development right it's like an endodermal dirif structure right so so lateral to the midline does not move with swallowing think about a bring your cleft cyst it basically arises because you have problems with the like field like obliteration of the second third and fourth bring your clefts remember those bring your clefts are derived from ectodermal structures and now that I'm just going over this some ideas just drummed in my mind if they give you a question about a newborn has a calcified supercella mass right you want to think about a cranial firing geomer right so sometimes you may see referred to as a d'Amantino-madas cranial firing geomer remember that arises from oral ectoderms sometimes they may call it like raki spouch on an in-beam example and then what if they give you a question about a newborn that has trouble turning his head to the other side and they tell you that this newborn's head is tilted if you see this you want to think about like congenital tauticolis right usually it happens from like problems with the sternoclid or mastoid muscle that's the buzzword you want to remember like they may have like shortening or like fibrosis of the sternoclid or mastoid muscle because maybe the child can tilt it is head funny and weird in the uterus right so that's just something you want to keep in mind and then what if they give you a question about a newborn with a scape-point abdomen and respiratory distress and then they tell you that they have a nitsugastric tube it's completely inserted you know so it doesn't like stop as you'll see in a syvagela trigial or t-fisular but you know it goes all the way in but then they check on image and they find that it's just in the thoracic cavity if you see that you want to think about a diaphragmatic hernia right so the scape-point abdomen is because t

he abdominal contents are in the thoracic cavity right and remember the buzzword pathophysiology you want to remember there is because there is like field development or no more development of the plural peritoneal membrane I mean though buzzword kind of makes sense plural peritoneal membrane it's a membrane that divides the plural cavity up high from the peritoneal cavity download right so field development of the plural peritoneal membrane that's very high you to know for example and then the next slide right so they give you a question about a newborn male it has no massive felt in the scrotum what is the child at increased risk of in the future well this one's pretty easy right this is script or kid isn't right if those things don't descend by 12 months right there's an increased risk of like a testicular cancer even if it descends before 12 months they still an elevated risk compared to the normal child for a testicular cancer right and then the next question says what's the kind of lung disease restrictive or obstructive in a newborn with a powerful depression in the anterior chest wall on exam right if you see this unless it's like in the middle like middle on tear chest wall you want to think about like pectosex cavatum remember it's like if you excavates the earth right you'll form a depression in the earth so pectosex cavatum has a very strong association with restrictive lung disease because the person's lungs may not expand right so this would be an example of restrictive lung disease right so they'll have decreased lung volumes if you want to if you see would be normal or increased right but the DLC will be normal because there's nothing intrinsically wrong with the lungs there's just something anatomically wrong with the surroundings of the lungs right so the DLC will be normal on that visa circumstances so this will be restrictive disease now what if th

ey give you a question about a newborn male with an asymmetric chest wall and they tell you that oh his right chest wall appears depressed compared to the left side and they tell you that oh imaging is notable for the absence of multiple ribs if you see this you want to think about something called pollen syndrome essentially they'll have like like non-development or poorly poor development of the pectoralis muscles right and there are some other associations you may want to know your test with this right they tend to have like webbed fingers they may have like poorly developed ribs or they may actually be missing some ribs right and they may have like no axillary hair and stuff like that if you see that you absolutely want to think about pollen syndrome on an MD mix again it's just one of those things I know you may be saying oh divine this is super loyal I promise you it is not I promise you it is not okay now what if they give you a question about a newborn with pale blue extremities and they say what's the next best step in management just warm this child reassure the parents this is acrocyanosis right basically if you see peripheral cyanosis that's fine it's just because newborns have like a lot of problem with like regulating like the abasculatone to conserve and release heat right so they have like viso modo instability right so this is acrocyanosis that's absolutely fine but remember this gets a one on the upgird scale now the next one just says diagnosis association is just a picture I got the image from our Wikipedia you essentially see like another nipple on this picture this is something known as a super numerary nipple this one is just something you want to be able to recognize on a test because there's this famous thing called the male client that runs from the like the breast area right to the groin right so you can actually develop nipples along that r

egion it's been nine for the most part doesn't cause many things it's just probably more of a cosmetic problem to people that have it okay now the next one right so an African-American newborn with a dark hyper pigmented markup over the interglutial folds this is something that's known as a Mongolian spot and they try to trick you into choosing child abuse on your test don't do that it's been nine doesn't do anything just again dark hyper pigmented markup and you'll be in an African-American kid on an exam now red-reize lesion over the neck or face in a newborn what's your next best step in management again this is a do not touch lesion just reassure the parents this is a strawberry or capillary human germa although if it involves the eye of the early you may need to intervene surgically but for the most part it's been nine it will go it will resolve right so they may give you an in-beam question and say what is the most likely outcome it will resolve on its own before the child is you know usually by the age of five or six years it's not a huge deal right and then what if they give you a question about a newborn with a white reflex on a fondoscopic exam if you see that right that's right no blastoma right that's right no blastoma remember these kids tend to be at an increase risk of osteosarcoma in the future right remember it arises from an R.B.

tumor suppressor gene mutation right so you have like even if you have genetic mutations you keep going from the G1 to the S-phase a wily nilly right and remember a white reflex though with like a patoz planomegaly on exams you know you may want to think about cataracts from like one of those galactose problems like essential galactocemia for example remember those kids right they tend to one of the most common causes of death is like E.

colisepsis right they'll have a patoz planomegaly remember the galactocemia you typically have it from a deficiency of UDP no from galactose one phosphate uridil transfer is right so again just something you want to keep in mind on a test and then what if they give you a question about a newborn with central sanosis at birth and they tell you that when the kid cries the sanosis disappears if you see this you want to think about a quinolatrizia right essentially your next step in management on a test is to try to pass a nizogastric tube through the nose and then you'll notice that man I'm not able to advance this angitube if you see that that's quinolatrizia don't forget the charge association right so they may ask you a question and say which of the four-line is a likely other finding on physical exam if you see that again you know you want to think about the other things right like coloboma that's like a keyhole defect in the iris the H is like like you know like heart they may have like heart problems they is for like atrija quenni right those kids right when they cry they think up because they open their mouth and they breathe through their mouth right but you know they're kind of sad not it when they are not crying because they're using their nose and then the R is like renal problems and the G is for like G you anomalies and then the E is for ear problems right so you know kind of keep those things in the back of your mind for example and then what did they give you a question about a newborn with a large tongue and hypoglycemic seizures and a red apple quadrant muscle exam if you see this you want to think about something called a Beckwith Widamann syndrome right so it's an overgrowth disorder right so they tend to have macroglossia on physical exam right and then because they have like overgrowth of many things right they may have like hyperplasia of the pancre

atic I let beta cells and if those beta cells on the go hyperplasia you produce a crap ton of insulin and then they can get hypoglycemia and have seizures as newborns remember hypoglycemic seizures in a newborn you can also find that in an infant of a diabetic mom because that infant is seeing all this glucose from mom right sees all this glucose from mom in utero so you know the intestinal in pancreatic I let beta cells you know the go hyperplasia as well so they will keep producing a crap ton of insulin is not like they just shrink the moment the newborn is born right that doesn't happen that way right so they may have like hypoglycemic seizures although remember hypocalcemia is another thing that can cause seizures in an infant of a diabetic mom on an exam and also hypocalcemic seizures may be something you'll find with the George syndrome right remember catch 22 like 22 Q 11 are problems on in baby exams and then remember the right upper quadrant mass right is a hepato blastoma again it tends to be found in Beckwith Whitman syndrome I remember Beckwith Whitman syndrome also has an association with Williams tumors on tests right and remember they may also have hemahypertrophy right so like one side of the body is bigger than the other and then the add on question there is like oh new one with a large tongue and on Belycor hernia just think about like hypothyroidism in this kid remember the most common cause of hypothyroidism in a newborn is a is a thyroid is genesis right and then what if they give you a question so the next one right newborn that has not popped in the first 40 days of life this one is pretty easy that's myconium elius it can be caused by one of two things right so it can be from a herch born disease right like a kid with Down syndrome or it can be from cystic fibrosis right and then calcified abdominal mass that crosses the midline in a newborn th

is is neuroblastoma right it's neuroblastoma remember they can if you see it's a mid it crosses the midline and it's calcified and if you're soaring the midia stinem right it's shopping the posterior stinem because it's a neurobis tumor I remember the association with something called obso clonus myoclonus syndrome right so those kids will have like weird movements of the eyes and weird movements of the extremities on an exam and then what if they give you a question about a non calcified abdominal mass that does not cross the midline if you see this right that's a one that's worms to run it's pretty easy and then they tell you next question like urethral midias on the ventral surface of the penis that's actually a hypospedia right so if you hold out you know if a person has an erect penis like the under surface if you see like the urethral midias instead of coming out at the tip comes out at the bottom that's a hypospedia if it comes out at the top which is the drossal surface that's an epispedia the epispedia actually tends to be worse it tends to be associated with a congenital problem known as a bladder extra fee okay so that's something to keep in mind and then also a biopsy of a benign rash on the trunk and extremities in a newborn and it tells you that oh this rash spares the palms and soles if you see this it's usually like like it's like a macular rash this is ETN right so erythema toxic commonly on the tour it's benign but if you do a biopsy you'll find your synophilis the reason of those weird bizarre things that your friends at the mbimmy kind of expect you to know for example and then what if they give you a question about a newborn with seizures and this newborn has intracranial calcifications on imaging and a port wine stain on the forehead if you see this is a uh uh uh uh starchwebber syndrome something they'm calling it like nivas phlegmias like fl a

double me us on a test um yeah it's just something we want to keep at the back of your mind i'd tend to have like problems in a trigeminal nerve distribution usually like uh trigeminal nerve v1 right so the ophthalmic nerve and then uh both were pathophysiology so the seventh question here so newborn with bilios vomiting are you cannot interpret the image the mbimmy this is going to be my rotation with mid-god volvulus um essentially um you'll essentially see uh the boswapathophysiology here is uh you have like weird rotation of the mid-god of the god around the supermiss anterior cadre and then what if they give you a question about a newborn with bilios vomiting and you see a double bubble sign on imaging uh this is the one elatrizia right remember it's an associate of Down syndrome and then a newborn uh and the problem is of the boswapathophysiology is a failure of re-canalization okay it's a failure of re-canalization that's something to commit to memory on exams and then what if they give you another question about a newborn with bilios vomiting triple bubble sign on imaging this is general atrizia and the boswapathophysiology here is actually a vascular insult in utero okay it is not it does not arise from a failure of re-canalization i promise you this is floridae high you to know for exams and then they tell you oh newborn has not popped in the first 48 hours of life is not there to have endocardial cushion defects on echocardiography this is clearly herchprone's disease essentially the thing that's happening to this kid is um they have like failed migration of neurocrest cells okay so the my enteric and the mysena splexus plexus i do not develop right so like the albax plexus mysena splexus so the my enteric and the subucosal plexus the myeteric plexus is albax plexus the subucosal plexus is mysena splexus okay so they did not develop so this kid has a proble

m state and then you're in coming out of a newborn zombie licas right this is from a p10 urecus um this one's pretty easy essentially right the urecus connects the bladder to the umbilikus in utero is supposed to obliterate if it does not obliterate and it stays p-tent then you'll see you're in coming out from the from the umbilikus of a newborn and then the last slide second to the last slide whoop sorry so triggers right so newborn wears fiery difficulty um on exam he's not yet to have a small chin and his tongue is displaced downwards this is the pierroban sequence uh pier like i think it's p i e double r e and then roban is like our obi sequence is just something one to recognize this kids may have respiratory difficulty because essentially the tongue is obstructing the ear and then a newborn with lower extremities so i can say i know cis and pink opera extremities there's a reo femoral postally on physical exam this is a partition of the order right so they may have like well developed opera extremities polydeveloped lower extremities or they may have like you know like pretty good pulses in the opera extremities and then the femoral pulses are barely this is a partition of the order remember it has an association with a um turner syndrome right and you remember classically you'll find a three sign you'd find a three sign the three sign okay on imaging you'd find the three sign on imaging and then um the radio femoral pulse delay again because usually when you pop it the femoral pulse you should i mean the radio pulse you should be able to pop it the femoral pulse at roughly the same time if there is a delay in those things think about the partition of the order and you fix it with surgery drugs are not going to do squat for that jump and then newborn with bounding pulses and a white pulse pressure on exam you want to think more about like a patent doctor's arter

iosus um remember that has an association with like a congenital rebel syndrome um so they may have like like bounding femoral pulses if you see that you know kind of think about a pda on a test and then a newborn with one on bilicol artery and one on bilicol vein uh because normally right you should have two on bilicol arteries and one on bilicol vein if you see this you want to think about like problems with like their kidneys right tends to be like renal abnormalities or nbiming exams and then the final slide this versus versus that right so coupled succedenium versus cephalochymatoma a coupled succedenium the big thing you just want to keep at the back of your mind is uh um so this one you know it's pretty it's pretty easy essentially the thing that happens with coupled succedenium is that um you have like just like edema from you know kind of like the child had been all banged up as the child was being delivered um for the most part it's been nine it's gonna resolve within a few days it's not anything you need to be like super super worried about in the kid and um remember it it it's the the lesion tends to um cross sutra lines right so it tends to cross sutra lines that's just one of those weird things I want to keep at the back of your mind it crosses like basically it's just a demon swelling of the child's head and it crosses sutra lines versus a cephalochymatoma that's a subperial still hemorrhage you need to memorize that both word okay and it tends to be confined to the sutra lines so it does not cross sutra lines right and then the last thing I think I want to explain here and again my apologies that I'm rushing so much I have like a meeting that I'm already literally already late for but I just want to get this podcast out of the way um the thing I guess I want to uh uh uh uh gastroskises versus um phallosil so it's high you to know that gastroskises uh t

ends to be to the right of midline okay to the right of midline it tends to not be covered by any like membranes right and there's like a high risk of like bowel ischemia because you have like essentially like uh uh peritoneal cavity and uh bowel just fleed out uh with amniotic fluid and uh yeah there's a lot of problems that can arise with that and it tends to be not associated with many other bad things right contrast that with an phallosil that is in the midline so it's like coming out right through the umbilikus and it tends to be covered over by a membrane so you may be like oh this is better it's not because it tends to be as usual like many other things like congenital heart problems and all this badness right so that's just something you want to keep at the back of your mind and as I do at the end of every um session I do offer one or one to the end for many exams right so step one two ck two cs step three pre-clinical medical exams 30-ish of exams if you're medicine resident titular for like the ibi m boards and uh uh and the intruding exam same thing for pizza resident if you're interested in that and then I also offer these booster courses for all the USML exams it's 20 hours for each of the USML exams again I've done it with tons of people they found it to be like extremely extremely extremely useful and then I also do like consulting if you're like a medicine in a plan to residency so like an ira sap or a coleson in a plan to med school so like an amcassap I do offer like one on one like coaching or advising you know like personal statement editing rec letters I did your application I do more interviews again I've worked with people from multiple specialties and with this current cycle all the people I worked with matched right so um if that's something you're interested in just reach out to me either through the website or you send me an email at divine

intervention podcasts with an srbend at gmail.com and please subscribe to the youtube channel um the podcast uh I have the podcast on apple podcasts or Spotify and google play so please subscribe and then uh please uh don't forget to say uh also subscribe to the Word Press website and then if you have like a college student buddy that needs to learn for like any of the pre-med subjects jen cameo came physics bio came histology physiology offered to learn for all those things so thank you for listening please listen to this podcast before you take your pitch off and before you take step two ck the stuff is floridly high-yout so have a wonderful rest of your day please do not panic whatever is going on in the world will pass right this to sharp pass that should be your model so have a wonderful rest of your day make the most of the opportunity that has been presented to you I'll see you next time god bless you thank you

Type 2 RTA combined with physical findings like a "thumb-band radius" (smaller right arm).

Thyroglossal cysts are midline and move with swallowing; Branchial cleft cysts are lateral to the midline and do not move with swallowing.

Neuroblastoma.

Patent Ductus Arteriosus (PDA).

Duodenal Atresia.

Gastroschisis lacks a covering membrane; Omphalocele is covered by a sac/membrane.

Caffeoli spots are hyperpigmented macules found in Fanconi Syndrome.

Thrombocytopenia (low platelets) and an absent radius.

Neonatal Cyanolatrizia.

Paraplegia, urinary retention (due to myelopathy), and a high association with an anorectal malformation (specifically, anal Key Area II).

Thyroglossal duct cyst; arises from the base of the tongue/thyroglossal tract.

Gastroschisis is an open defect (no membrane); Omphalocele involves herniation through a sac-like structure.

Macroglossia, overgrowth disorder, potential for hypoglycemia/seizures due to pancreatic islet beta cell hyperplasia, and sometimes hepatoblastoma.

Caffeoli spots are hyperpigmented macules found in Fanconi Syndrome.

Thrombocytopenia (low platelets) and an absent radius.

Neonatal Cyanolatrizia.

Paraplegia, urinary retention (due to myelopathy), and a high association with an anorectal malformation (specifically, anal Key Area II).

Thyroglossal duct cyst; arises from the base of the tongue/thyroglossal tract.

Gastroschisis is an open defect (no membrane); Omphalocele involves herniation through a sac-like structure.

Macroglossia, overgrowth disorder, potential for hypoglycemia/seizures due to pancreatic islet beta cell hyperplasia, and sometimes hepatoblastoma.