PEADS- Onco Flashcards

1
Q

Osteosarcoma CF ?

A

Pain, limping, Swelling, decreased ROM, B-signs and pathologic fracture

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2
Q

Who is Osteosarcoma MC in ?

A

Patients <30, with Hx of Retinoblastoma or P53 tumor suppressor cancers.

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3
Q

Pathophysiology of Osteosarcoma ?

A

Malignant, osteoid ( poorly mineralized matrix) and bone forming tumor. Formation of osteoid by osteoblasts

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4
Q

Features of Osteosarcoma on Xray ?

A

Sun-burst appearance
Codman triangle (periosteal elevation due to formation of tumors)

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5
Q

MC location of Osteosarcoma ?

A

Distal femur or proximal tibia (around the knee)

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6
Q

Ewing Sarcoma

A

Bone tumor arising from neuroectodermal cells, mainly affects diaphysis of long bones

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7
Q

CF of Ewing sarcoma ?

A

Pain (mainly at night and progressive)
Swelling after trauma to the bone
erythema
B-symptoms
Hyperthermia

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8
Q

Radiologic findings of Ewing sarcoma

A

onion skin lesions of the periosteum (the fibrous layers that covers the bones).

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9
Q

What is Ewing sarcoma associated with ? mutation

A

11:22, formation of EWS-FI1 fusion protein.

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10
Q

Neuroblastoma features ?

A

palpable mass crosses the midline
rackoon eyes (bleeding into the periorbital tissue)
fast eye movement and jerky movement of the limbs ( Opsoclonus-myoclonus syndrome)
weight loss
prodcues catecholamines ( positive vanillyl mandylic acid in urine).

Tumor of primitive sympathetic ganglia

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11
Q

Confirmation of Neuroblastoma

A

homovanillic and vanllylmandelic acid in urine

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12
Q

What are cranipharyngioma ?

A

Benign slow growing tumor
Suprasellar mass
May present with features of optic chiasm or pituitary compressions

On CT/MRI: calcified or cystic suprasellar mass.

C mnemonic: cranio, clumsiness, calcified, compression

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13
Q

What is the MC tumor that occur in adolesence ?

A

Hodgkin lymphoma

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14
Q

Hall mark of Hodgkin lymphoma ?

A

Reed sternebrg cells (Multineucleated lymphocytes).

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15
Q

CF of hodgkin lymphoma ?

A

B symptoms
Mediastinal mass
Lymphadenopathy ( mainly supraclavicular)

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16
Q

What is highly suspicious of malignancy in general ?

A

Enlarged supraclavicular lymph node

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17
Q

Diff between craniopahryngeoma and pituitary adenoma on imaging ?

A

Craniopharyngioma is calcified, pituitary adenoma is not.

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18
Q

What are the two MC posterior fossa tumors in children ?

A
  1. Medulloblastoma
  2. Astrocytoma
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19
Q

Location of Medulloblastoma ? and CF ?

A

Medulloblastoma: in the cerebellum.
Causes Ataxia, wide based gait and intention tremor.

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20
Q

CF of Neurofibromatosis

A

Cafe au lait spots
Freckeles in axilla and groin
Optic glioma
Scoliosis
Lisch Nodule
Neurofibromas (benign peripheral nerve sheath).

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21
Q

CF of Nutritional Rickets ?

A

Bowing of the legs ( when weight bearing)= genu varum.
Craniotabes (skull bones that depress with pressure)
Rotaric Rosary (costochondral Joints).
Wide wrist due to growth plate enlargement

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22
Q

Xray findings of rickets

A

Metaphyseal widening and cupping

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23
Q

Rx of Rickets

A

Vitamin D and Calcium supplimentation

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24
Q

What is Nieman Pick Disease

A

Autosomal recessive disease, responsible for accumulation of sphingomyelin in different organs( due to def. in sphingomyelinase).

Leads to progressive Loss of motor milestones ( neurodegeneration).
Red-macula
poor feeding \Hypotonia
Hepatosplenomegaly

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25
DD of Cherry Red macula ?
Nieman Pick disease Tay sachs disease
26
Pathophysiology of Taysachs disease
Beta-hexosaminidase deficiency. | Has hyperreflexia, unlike Nieman pick disease
27
What is Rett syndrome ?
Neurodevelopmental disorder leads to loss of speaking skills, abnormal gait, repetetive hand movement after having normal development. MC in girls
28
Classical Signs of Biliary cyst ?
1. pain 2. jaundice 3. RUQ mass
29
Differentials of Jaundice in neonates
1. during first 24 hours: pathologic 2. 2-4 days of birth: Physiologic (Unconjugated Hyperbilirubenemia) 3. 2 weeks of birth: breast milk jaundice ( Unconjugated Hyper.. due to increased deconjugation in the intestine). 4. Biliary Atresia: chronic, progressive with poor weight gain.
30
Diff between Breast Feeding and Breast Milk jaundice
Breast feeding jaundice: decreased breastfeeding --> decreased bowels movements --> inc. enterohepatic circulation and reabsorption of bilirubin --> High unconjugated hyperbilirubenemia. ( within 1 week of birth) Breast Milk Jaundice: high glucoronidase in breast milk --> increases deconjugation of bilirubin in the intestines --> hyperbilirubenemia. (within 2 weeks of birth)
31
High risk features of head trauma on young children > 2 years
LOC Vomiting AMS Svere mechanism of injury ( Fall >1.5m) Basilar skull fracture features (like CSF or rhinorrhea
32
High Risk features of Head Trauma in < 2years
Altered mental status (fussy behavior) * Loss of consciousness * Severe mechanism of injury (fall >0.9 m [3 ft], high impact, MVC) * Nonfrontal scalp hematoma * Palpable skull fracture
33
Routine Newborn care
1. skin to skin ( to regulate body temp, preventing hypothermia) Then after one hour 2. Vitamin k 3. Hep B vaccine 4. erythromycin eye ointment
34
Why do we give Vitamin K injection at birth ?
To prevent Vitamin k deficient bleeding
35
Cause behind Vit.K deficient bleed ?
1. poor placental transfer 2. dbreastfeeding (poor in vit. k) 3. lack of Intestinal flora to produce Vit. K
36
Friedreich ataxia
progressive Ataxia (uncoordinated muscle movement) Gait ataxia ( uncoordinated movement of the lower limbs, wide based gait) due to destruction of spinocerebellar tract Loss of sensory and proprioception: due to destruction of dorsal coloumn and ganglia. Skeletal abnormalities: kyphosis In ward or outward chest High arched foot (pes cavus) Hypertrophic Cardiomyopathy
37
CF of Intracranial HTN ?
throbbing headache Vision loss Pulsatile tinnitus papilledema
38
CF of GBS
1. preceeded by Campylobacter or resp. infection 2. Parasthesia asc with ascending muscle weakness, symmetric 3. absence of deep tendon reflexes 4. Might lead to resp. compromise
39
Pathophysiology behind GBS ?
ABs. directed against myelin on nerve fibers
40
Diganosis of GBS ?
albuminocytologic dissociation ( high protein, normal Leukocyte count)
41
Patient with GBS should be assesed for ?
Spirometry for Lung failure, and need for intubation.
42
Diff between botulinism and GBS ?
GBS is ascending Botulinism is descending weakness
43
What is the MC cause of Pediatric Stroke ?
Sickle cell disease
44
What is the main manifestation of sickle cell trait ?
Renal Papillary necrosis ( leading to necrosis and sloughing of the papilla appearing as a hematuria)
45
When does sickeling of cells of those with SCT occur ?
when under stress ( low oxygen concentration and dehydration) Mainly in Renal papilla
46
What is the most common cause of Osteomyelitis in children with SCD ?
Staph aureus Salmonella enteriditis
47
Nephrolithiasis Vs. Renal Papillary necrosis on US ?
Nephrolithiasis: Hydronephrosis RPN: Increased echogenicity of the medulla.
48
Diff between Alport syndrome and IgA nephropathy
poth have hematuria and normal complement levels. Alport is asc with sesnsorineural hearing loss and type IV collagen mutation. X-linked. Longitudenal splitting of GBM. IgA nephropathy: IgA deposition in mesangial cells.
49
Hemato
50
2 complications of SCD ?
1. Splenic sequesteration crisis 2. Aplastic crisis
51
What is splenic sequesteration crisis ?
pooling of both RBCs and platlets inside of the spleen, leading to symptoms of shock (hypovolemia, tachycardia) Anemia, thrombocytopenia inc. reticulocyte count.
52
Patients with SCC who have splenic infarct should be given ?
Vaccination ( conjuagted polysaccharide of S. pneumonea) and twice daily penicillin for prophylaxis of enxapsulated bacteria
53
Management of SCD and vasooclusive crisis ?
Folate Penicillin Tx Hydroxyurea
54
What is the SE of hydroxyurea ?
Myelosupression
55
RF for Anemia of Prematurity ?
Occurs after birth due to decreased EPO. In premature neonates low EPO is exacerbated by short rbc life span and as a result anemia is exacerbated
56
Management of Anemia of Prematurity ?
1. iron 2. decrease blood letting 3. Transfusion.
57
What are Howel Jolly bodies and how do they appear on a smear
Howel jolly bodies are remnants of the nucleas in red blood cells, usually removed by spleen. Appear as a blue granule in RBC Their presence indicate hyposplenism or removed spleen
58
Hb in Beta Thallasemia Minor
Low Hb A (alpha and Beta subunit) High Hb A2 ( alpha and delta)
59
Hb in Beta thallasemia Major
Low Hb A High Hb A2 High Hb F (alpha and Gamma)
60
CF of Beta Thallasemia major
Microcytic anemia
61
Management of Beta thallasemia Major ?
Transfusion dependant Needs chelating agents to present Iron overload
62
What is fanconi anemia
bone marroe failure, leading to pancytopenia. Inherited DNA repair defect Features: Short stature abnormal thumbs Hypopigmented lesions
63
CF of Malarial Infection ?
Cyclic fever ( ever 2-3 days).
64
CF of whooping cough ?
3 stages: 1. Catarrhal phase: rhinorrhea, lethargt 2. Whooping cough: posttusive emesis, cyanosis and apnea 3. recovery phase
65
Which organism causes Whooping cough ?
Bortedella pertusis ( grame negative coc-bacilli)
66
Rx of Whooping cough
Macrolide like azithromycin
67
Common causes of Meningitis in Children < 1 month
Group B strep (Strep agalactea) E.coli Listeria monocytogenes HSV
68
MC of Pharyngitis ?
Group A streptococcus ( Strep pyogenes)
69
Complication of Strep. Phayngitis
Scarlet fever
70
CF of Scarlet fever ?
Sand paper rash, mainly in the groin and axillar. Fever, pharyngitis, tonsillar erythema Perioral pallor, compared to red cheeks Tender anterior cervical nodes Peeling of the rash ( desquamation).
71
Rx of Scarlet fever ?
Like strep pharyngitis with penicillin
72
Erysipelas
Caused by Group A strep. well demarcated, warm and tender area.
73
CF of staphylococcus Scalded skin syndrome ?
Blisters and bulla mainly in the flexure regions. peri-oral rash and crusting Positive nekolski sign ( on pressure on bulla) Caused by Staph aureus infection
74
Rx of ssss ?
Nafcillin or Vancomycin
75
What is Bullous impetigo ?
Localized form of SSSS
76
What is tinea versicolor
Fungal infection by Melassezia yeat. Appears after skin exposure, where rest of the skin tans and the rash doesnt. With KOH: yeast and hyphea appears as spagetti and meatballs.
77
What is pytriasis rosea ?
Rash on the body christmas tree like, mild scaling Presents after a viral infection. Self limiting
78
Common causes of meningitis in Children > 1 month
1. N. meningitidis 2. strep. pneumonea
79
Cause of HUS ?
Shiga toxin producing E.coli ( OH157: H7)
80
CF of HUS
Microangiopathic hemolytic anemia ( due to vascular damage) Thrombocytopenia AKI Usually preceded by bloody diarrhea
81
CF of Trachoma ?
Caused by Chlamydia Trachomatis Common in crowded areas with lack of sanitation 1. conjuctival follicles 2. Trichiasis ( ingrowing eyelashes), this will lead to scarring of the cornea and blindness. Rx with Azithromycin
82
CC of conjuctivits after birth ?
gonococcal and chlamydial
83
CSF finding in bacterial meningitis ?
1. WCC >1000 2. LOW Glucose 3. High Protiens
84
CSF findings in Viral meningitis
1. WCC <1000 2. Glucose: N 3. Proteins <150
85
MC causes of otitis externa
1. Pseudomonas 2. staph
86
Rx of Otitis externa
Fluoroquinolones
87
Organism causing bloody diarrhea and their complications
E.coli o157:H7 --> HUS Shigella --> seziures Salmonella --> Bacteremia Campylobacter --> GBS
88
Organism causing watery diarrhea ?
E.coli (enterotoxigenic) Giardia intestinalis Clostridium perfringes
89
CF of Giardiasis ?
Foul, oily stool abdo cramps Weight loss transmission: fecal oral route, contaminated water and crowded areas. Patho: disruption of epithelia
90
What type of rash do you see with Varicella ?
Vesicular rash; prurtic; not painful
91
Painful vesicular rash
Eczema herpeticum ( areas of eczema with painful vesicles overlying caused by herpes virus).
92
Morbiliform, cephalocaudal rash is ?
Measels | cough, coryza, conjunctivitis, koplik spots
93
When is the Varicella Zoster vaccine given ?
Age 1 and 4
94
HSV encephalitis feature on imaging
Intracranial Hemorrhage
95
Toxoplasma and CMV neonatal infection on imagining
Intrcranial calcification CMV: spreak Toxoplasma: periventricular
96
Neonatal Tetanus
C. tatni producing a toxin that leads to absence of inhibitory Neurotransmitters as a result --> contraction of all muscles (neck, resp, arms and legs) hypertonicity of muscles. And difficulty feeding because of trismus. Sterile csf
97
Management of Neonatal tetanus ?
Abx ( penicillin), tanus Immunoglobulin.
98
Difference between Bacterial and Viral Rhinosinusitis ?
Bacterial: 1. Fever >3 days 2. Symptoms > 10 days 3. Resolution of fever and developing of fever again.
99
CF of herpangina
Caused by Coxaievirus Pharyngitis fever Gray vesicles in the oropharynx, tonsillar oillars, uvula
100
CF of herpatic gingivostomatitis
Caused by HSV type 1 Fever Pharyngitis Erythematous gingiva Cluster of vesicles on anterior oral mucosa/lips.
101
What is Chronic Granulomatous disease
Disease related to superoxide production deficinecy. Macrophages will surround the organism, form an abscess but they are unable to neutralize it. X-lined. Uusllay catalase positive orgn. infection like Staph, serratia and fungal infection.
102
DD of recurrent infections
1. Recurrent sinupulmonary with GI infections and absent tonsils ( Absence of B-cells and immunoglobulin): X-linked hypogammaglobulenemia 2. Recurrent infections with catalase positive org and abscess formation ( No oxidative burst, chronic granulomatosis) 3. Recurrent infections with encapsulated bacteria: complement deficiency) 4. Recurrent bacterial, viral and fungal infections ( revelas severe combined immunodeficiency, indicating adenosine deaminase deficiency).
103
Differentiating between X-linked agammaglobulinemia and Common variable immunodeficiency
both have problems with B cells both have recurrent sinopulmonary infections and GI infect. X-linked: small tonsils, low b cell count CVID: improper activation, low immunoglobulins, normal B cell count
104
Hyper IgM syndrome
Deficiency in Cd40 ligand present on T cell, leads to absence of class switching of B cells from igM to other immunoglobulins. high IgM, low IgG and low IgA
105
SCID
Defect in T cell ( CD3) formation, thus absence of B cells ( CD 19) leads to fungal, bacterial and viral infection RX stem cell transplant
106
RF of RDS in neonates ?
Prematurity Maternal DM
107
Pathophysiology behind RDS in neonates of diabetic mothers ?
1. Maternal hyoerglycemia --> fetal hyperglycemia --> fetal hyperinsulinemia --> counteracts the effect of cortisol --> inhb. sphingomyelin --> thus absence of surfactant.
108
Intervention in cases of primary nocturnal enueresis ?
1. Behavioral changes ( peeing before sleeping, avoid drinking before bed time, reward system, eneuresis alarm) 2. and desmopressin
109
Complications of Supracondylar fractures ?
Compartment syndrome ( increasing pressure and swelling of a certain compartment), pain not responsive to analgesia and 4 ps Pallor, parasthesia, pulslesness, poikilothermia.
110
Confirming diagnosis of Gastroschesis?
1. eleavted afp in pregnant mother 2. Free floating intestine on pre-natal US
111
CF of meckels diverticulum ?
Painless Bloody stool No abdominal pain, cramps or fever No diarrhea or vomit
112
Diagnostics of Meckels diverticulum ?
Meckels scan or 99 technitum to identify gastric mucosa secreting hydrochloric acid.
113
Diff b/w Post Strep glomerulo and IgA nephropathy ?
post strep: 3 weeks after infection, low complements levels.
114
Laryngomalacia
Collapse of larynx ( aretynoid cartilage) causing inspiratory stridor.
115
Inborn Errors of Metabolism
116
What is Galactosemia ?
A disease that leads to galactose accumulation, due to GALT enzyme deficiency. Acc of Galactose and its byproducts in the body and lesne lead to hepatomegaly, catarct. | GALT: Galactose 1 phosphate uridylyltrasnferase
117
CF of Galactosemia ?
Vomiting Hepatomegaly, jaundice, elevated liver enzyme Poor oral intake leads to hypotonia and lethargy, low glucose levels. Catarct
118
Management of Galactosemia ?
Galactose free formula (soy-based).
119
Pathophysiology behind homocystenuria
Acc of Homocystiene due to def, of enzymes like Methionin synthase and cystethione synthese.
120
CF of Homocystenuria ?
Fair skin Marfinoid habitus ( tall arms and legs) skin and joint hyperlaxity Intellectual disability downward subluxation of lense Homocystiene in urine ( urine turns red with sodium nitroprusside test)
121
dd OF homocystenuria with Marfan syndrome
Marfa
122
Wilson disease ?
Acc of copper in the liver, brain and cornea Leading to Psychiatric and personality changes Kayser- Fleischer rings in the eyes Parkinsonism, ataxia, dysarthria Low ceruloplasmin and High urinary excretion of copper.
123
What is ceruloplasmin ?
It is a carrier of copper
124
Endo and OBGYN
125
How to treat heavy bleeding due to anovulation ?
High dose Oral contraceptive (estrogen and progestin) to stabilize the endometrium.
126
Fibroadenoma
breast nodule presents in young women < 30 years. Solitary Increase in size and tender around menses It is a rubbery mobile, well circumscribed
127
What is hydantoin Syndrome ?
Congenital malformations seen in neonates who were exposed to anti-epileptics in utero. like Phenytoin, valproate and carbamazepine --> leading to low folate.
128
CF of Hydantoin Syndrome
Wide anterior fontanelle Neural tube defects Cardiac defects cleft lip and palate Finger hypoplasia Microcepahly
129
How to prevent Hydantoin Sybdrome
lowest dose of anti-epileptic before preg and high dose folate
130
Fetal alcohol syndrome
Microcephaly Midface hypoplasia small palpebral fissure Smooth philtrum ( vertical lines above the lip) Thin vermillion Behavioral and motor retardation autism like social problems.
131
Congenital Syphillis
Rhinitis Hepatomegaly Maculopapular rash
132
CF of tuner syndrome
45,XO Webbed neck Horse shoe kidney Congenital lymph edema ( non-pitting, because it is rich in protein edema of the hands and the feet) coartaction of aorta bicuspid aortic valve dysgenesis of ovary
133
Kallman Syndrome
46, XX Failure of Gnrh and olfactory neuron to migrate Absnece of GnRH. Low LH and FSH, low GH No axillary hair, no breast growth, short stature. Anosomina (unable to identify smells)
134
RF of Cerebral palsy ?
Low birth weight Preterm birth
135
136
Cyanosis and Hypoxemia refractory to oxygen raises suspicion of ?
Truncus arteriosus
137
CF of Diageorgy syndrome ?
- hypocalcemia ( PTH hypoplasia) - conotruncal abnormalities ( truncus arteriosus, cleft palate, low set ears). - Thymic hypoplasia/ aplasia ( T cell lymphopenia and decreased humoral activity due to inability of Tcells to activate b Cells).
138
Gastro
139
RF of constipation in Peds
New changes: Toilet training School Initiation of solid food and cows milk
140
Complications of CF ?
Vitamin K deficiency and bleeding
141
CF of kartageners syndrome ?
Sinupulmonary infections Stus invertus Bronchiectasis
142
DERMA
143
how does a congenital melanocytic nevus look like ?
It looks like a brownish patch of melanocyte covered with hair
144
How does congenital dermal melanocytosis look like
blue-gray patches
145
Milestones at 2 months ?
GM: Lifts head FM: 50% of time fists open L: Coos S: social smile
146
Milestones at 4 months ?
GM: Turn over/ rolling.. sits with trunk supported FM: fists open all the time L: turns to voice Social: enjoys looking around | 4, roll, open fist, turn
147
MS at 6 months
GM: sits up supported on hands FM: transfer objects from one hand L: babbles S: stranger anxiety
148
MS at 9 month
GM: pull to stand FM : 3 pincer grasp L: baba, dada S: wave bye
149
MS at 12
GM: walks, kicks ball FM: 2 pincer grasp L: another word to mama, dada S: seperation anxiety
150
MS at 18 months
GM: Runs FM: tower of 4 blocks L: 25 vocab S: parallel play
151
MS at 2 years:
GM: up and down stair, one leg at a time FM: toer of 6 blocks L: 50 vacob, 2 word sentence S: follows 2 step command, start toilet train.
152
MS at 3 years
GM: tricyle, up and down stairs FM: copies circle L: 3 words sent, S: imaginative play
153
MS AT 4 years
GM: balances, hop on one leg FM: Copies a cross L: complete speech, identifies colors S: cooperative play
154
MS AT 5 years
GM: skips FM: copies a square L: counts to 10 S: completes toilet training
155