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Pediatric path by BKM Flashcards

1
Q

Ratio for placentomegaly?

A

Fetal:placental weight

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2
Q

When do fetal nucleated RBCs appear?

When do they disappear?

A

Around 8 weeks

12 weeks

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3
Q

When are fetal nucleated red blood cells normal in later development?

A

Andes mountains and other areas of high elevation

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4
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/010-hp-3-14C0EBCF74D4C908E81.jpg

A

Amnion nodosum

Associated with oligohydramnios

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5
Q

Leading cause of premature birth?

A

Acute chorioamnionitis

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6
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/peds-14C0EC0395709225BDD.jpg

A

Placental listeriosis

Can cause intervillous abscesses and septic infarcts

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7
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/april_2003_-_fig_3-14C0EC16EED3B7F1A21.jpg

A

Candida: microabscesses on surface of umbilical cord

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8
Q

What are TORCH infections?

A

Congenitally acquired infections that cause significant morbidity

  • Toxoplasma
  • Other (syphilis/HIV)
  • Rubella
  • CMV
  • HSV
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9
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/plac085-14C0EC51F544CE9EC5F.jpg

A

CMV villitis (TORCH)

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10
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/20110306-malaria_20cdc_20infected_20placental_malaria-14C0EC6F67F3FBB6803.jpg

A

Placental malaria

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11
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/plac016-14C0EC82A7375710E61.jpg

A

Meconium staining in macrophages

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12
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/plac037-14C0EC991CD4C8AD7F2.jpg

A

Maternal atherosis (fibrinoid necrosis) and smooth muscle hypertrophy of maternal spiral arterioles

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13
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/placental_intervillous_thrombus_01_1-14C0ECB89394DAF9AF1.jpg

A

Intervillous thrombus (fetomaternal hemorrhage)

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14
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/kingbent_401705636492593_intervillous_fibrin_thrombus_placenta_sl_09_37136_20x-14C0ECC31D32B3E8A03.jpg

A

Intervillous thrombus (fetomaternal hemorrhage)

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15
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/peds-14C0ECE90FC3775449E.jpg

A

Massive perivillous fibrin deposition

Associated with HELLP syndrome and recurrent reproductive failure

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16
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/plac014-14C0ECFC1D349DD44DA.jpg

A

Retroperitoneal abruption

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17
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/placental_infarct_-_intermed_mag-14C0ED1A5A232EDD0F3.jpg

A

Placental infarction with ghost villi

20-30% is survivable
Over 15% probably maternal hypertension
Over 50%, consider lupus anticoagulant

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18
Q

Four associations with chorangiosis (>10 capillaries in 10 tertiary villi under 10X)

A

Maternal diabetes
Placentomegaly
Delayed villous maturation
Chronic villitis

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19
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/chorangioma_-_intermed_mag-14C0ED609EC3164E0F8.jpg

A

Chorangioma

Most incidental

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20
Q

What is chorangiomatosis?

A

Diffuse multifocal change associated with extreme prematurity, congential malformations, IUGR, delayed villous maturation, avascular villi

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21
Q

3 pathologic changes in placenta that are associated with fetal neurologic impairment?

A

Severe chorioamnionitis

Extensive avascular villi

Diffuse chorioamnionic hemosiderosis

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22
Q

___% of babies with a single umbilical artery have associated fetal anomalies?

A

20

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23
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/testes_gct_chorioca1-14C0EDEFE1260276E30.jpg

A

Choriocarcinoma

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24
Q

https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/uterus_placentalsitetrophoblastictumor3-14C0EE04EB7742D9BB4.jpg

A

Placental site trophoblastic tumor:

Abnormal proliferation of intermediate trophoblast, most behave benignly. Uterine perforation is the major problem.

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25
Embryo is what period?
4-8 weeks
26
What is the difference between a malformation and a deformation?
Malformation results from an INTRINSICALLY abnormal developmental process Deformation is an abnormal shape that results from mechanical forces (like oligohydramnios)
27
What is the definition of a disruption?
Morphologic defect resulting from extrinsic interference (teratogenic malformation, amniotic band) Not hereditable but can be genetically predisposed
28
Features of Beckwidth-Weidemann?
Placentomegaly Macroglossia Omphalocele Wilm's tumors/hepatoblastoma
29
Genetic cause of Beckwidth Weidemann?
11p15 Most cases sporadic Can be rearrangement of gene or uniparental disomy from dad or methylation issue
30
Inheritance pattern of Alagille's syndrome?
AD
31
4 features of Alagille's Syndrome?
Paucity of bile ducts causing chronic cholestasis Pulmonary stenosis Wide nasal bridge and deep set eyes Developmental delay
32
Rocker bottom feet associated with which trisomy?
18 (Edwards)
33
Genetics of Cri-du-chat?
5p-
34
Three most common abnormalities of embryo?
Neural tube Cleft lip Limb defects
35
What is a choristoma?
Hamartomatous tissue that occurs as heterotopic tissue in a site not native to its site
36
What percentage of babies with congenital heart defects have additional developmental anomalies?
25-45%
37
Prune belly syndrome associated with what?
Obstruction/malformation of the urinary tract and bladder
38
Three leading caues of mortality data <1 year?
Perinatal: IUGR, RDS, birth asphyxia Congenital anomalies SIDS
39
Three leading causes of death in children 1-14 yo?
1.   Accidents 2.   Cancer 3.   Congenital anomalies
40
Preterm?
>38 weeks
41
Large for gestational age?
>90%ile
42
Small for gestational age?
<10%ile
43
What % of newborns have a major malformation?
3%
44
What transplacental virus is associated with spontaneous abortion or hydrops fetalis?
Parvo B19
45
Two agents associated with early sepsis?
E. coli | Group B strep
46
Two agents associated with later sepsis due to long latent period?
Listeria | Candida
47
What finding is strongly associated with SIDS?
Intrathoracic petechiae (80%)
48
What condition produces "orange sand in the diaper"?
Leich-Nyhan X linked
49
Defect in Lesch Nyhan?
Hypoxanthine guanine phosphoribosyl transferase (HPRT) screws up purine metabolism Excess uric acid, de novo purine synthesis Gout, MR, self mutilation
50
ADA deficiency causes infection with what major infections?
Pseudomonas Candida Pneumocystis carinii Viral
51
Histologic finding in ADA deficiency?
Virtual absence of lymphoid tissue in nodes, spleen, tonsils, and appendix
52
3 sphingolipidoses?
Tay sachs Niemann Pick Gaucher's Disease
53
Tay sachs deficiency? Accumulated substrate?
Hexosaminidase A GM2 ganglioside
54
3 pathologic findings found in Tay Sachs?
Enlarged cerebral gyri, narrowing of sucli Cerebellar and brain stem atrophy Ballooning giant neurons
55
Enzyme deficiency in Niemann Pick disease? Accumulated substance?
Sphingomyelinase Sphingomyelin
56
Three types of Niemann Pick disease?
Type A: Infantile (severe) Type B: somatic form Type C: adolescent form
57
Presentation of Type A Niemann Pick?
Hepatosplenomegaly by 6 months Cherry red spot on macula Loss of mental/motor functions Death by third year
58
Pathology findings in Niemann Pick disease?
Enlarged foamy macrophages in bone marrow, spleen, lymph nodes, adrenal, and alveoli of lungs
59
Deficiency in Gaucher's disease? Accumulate substance?
beta glucosidase Glucocerebroside
60
Three types of Gaucher's disease?
I: reticulendothelial storage (80%) II: Infantile cerebral form III: Adolescent form
61
Presentation of Gaucher's disease?
Hepatosplenomegaly (usually in second decade), bone pain, easy bruising, fever, pneumonia
62
Pathologic findings in Gaucher's disease?
Enlarged lipid-laden histiocytes (Gaucher's cells) throughout the reticuloendothelial system
63
What is one of the main mucopolysaccharidoses?
Hurler's disease
64
Enzyme deficiency in Hurler's disease? Accumulated substrate?
alpha-L-iduronidase Heparan and dermatan sulfate
65
Presentation of Hurler's disease?
``` Corneal opacity Dwarfism Hepatosplenomegaly Broad nose MR Death before 10 years ```
66
Deficiency of glucose 6 phosphatase causes what?
Von gierke's disease (type I glycogen storage disease)
67
Clinical presentation of Von Gierke's disease?
``` Massive hepatomegaly and cirrhosis Xanthomas Growth retardation Bleeding Hyperuricemia ```
68
Pathology of Von Gierke's disease?
Pale liver cells, steatosis, excessive glycogen storage in liver and kidney
69
Where is the CF gene?
7q31-32
70
Risk factor for tracheoesophageal fistula?
Prematurity
71
How often are associated anomalies found in cases of tracheoesophageal fistula?
50-70% (GU, GI, CV)
72
What is the most common type of tracheoesophageal fistula?
Esophageal atresia with TEF to distal esophageal segment
73
Most frequently occurring cystic lung disease in children?
Congenital pulmonary airway malformation
74
What type of congenital pulmonary airway malformation (CPAM) is associated with severe anomalies like renal agenesis?
Type 2 (50%)
75
Hirschprung's disease associated with what gene?
RET
76
Meckel diverticulum is remnant of what?
Vitelline duct
77
Imperforate anus is associated with other anomalies how often?
50% (including VATER association)
78
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/7-14C0F1C50D53500CCEB.jpg
Autosomal recessive polycystic kidney disease.  Cysts are perpendicular to the capsule
79
What defect is associated with urinary tract obstruction and may have cartilage present?
Cystic renal dysplasia Renal parenchyma shows iimmature mesenchyme surrounding tubules
80
Most common pediatric neoplasia?
Leukemia (then CNS)
81
Four most common benign tumors in children?
Hemangioma Lymphangioma Fibromas Teratomas
82
5 small round blue cell tumors?
``` Neuroblastoma Lymphoma Rhabdomyosarcoma Ewing's sarcoma/PNET Blastemal Wilms ```
83
IHC for neuroblastoma? Genetic markers?
NSE N-myc amplification 1p-
84
3 stains for rhabdomyosarcoma?
Desmin Myogenin MyoD
85
Two Ewings stains?
MIC2 CD99
86
Two stains for blastemal wilm's?
Cytokeratin | EMA
87
Genetic markers for alveolar rhabdomyosarcoma?
t(2;13) | t(1;13)
88
Genetic marker for embryonal rhabdomyosarcoma?
11p15.5 loss
89
Four main types of NHL in children?
Lymphoblastic Burkitt Nonburkitt (pleomorphic) Large cell
90
Neuroblastomas typically arise from what?
Parasympathetic ganglion in renal hilum
91
What is a neuroblastoma with ganglion cells? With further differentiation?
Ganglioneuroblastoma Ganglioneuroma
92
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/peds-14C0F2EE2F96B0A669A.jpg
Ganglioneuroblastoma
93
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/peds-14C0F3061480374D145.jpg
Ganglioneuroma
94
Three types of favorable histology in neuroblastoma?
1. Differentiation toward ganglia 2. Stroma rich 3. Low mitotic karyorrhexis index - <100 in age 1.5-5 - <200 in age <1.5
95
Bad px for what neuroblastoma location?
Adrenal (50%)
96
How does age reflect prognosis in neuroblastoma?
2 Bad
97
Ploidy and prognosis in neuroblastoma?
Aneuploid (hyperdiploid or near triploid) good Diploid or tetraploid bad
98
Genetics and prognosis in neuroblastoma?
N-myc amplification over 10 copies: bad
99
>50% of childhood soft tissue sarcomas are?
Rhabdomyosarcomas
100
Three lcoations for embryonal rhabdomyosarcoma?
Head and neck Around eyes GU tract
101
Px of embryonal vs alveolar RMS?
Embryonal better than alveolar
102
What genes are involved in alveolar RMS?
PAX3-FOX01a PAX7-FOX01a
103
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/embryonal_rhabdomyosarcoma_histo_3-14C0F36B76A385CD896.jpg
Embryonal RMS
104
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/alveolar_rhabdomyosarcoma_histo_1-14C0F3742B6168FB8E6.jpg
Alveolar rhabdomyosarcoma
105
What other small round tumor can stain for CD99 when looking for Ewing/PNET?
ALL
106
Three elements of a Wilm's tumor?
1.   Blastema: small blue cells 2.   Epithelial: tubular and glomeruloid 3.   Meschyme: spinded tissue and mesenchymal elements like mature striated muscle
107
Cortical rind on imaging?
Nephroblastomatosis NOT Wilm's but looks like it histologically.  Benign.
108
Four basic patterns of Wilms Tumor?
Mixed (Majority) Bastemal predominant Epithelial predominant Stromal predominant
109
Bad prognosis in Wilms Tumor?
Anaplasia Nuclear diameters three times the size of a neighboring cell of the same type.  Also atypical mitotic figures.
110
Genetic difference between WAGR and Denys-Drash?
The 11p13 mutation affects DNA binding to WT1 in DD.  In WAGR, WT1 is deleted.
111
Two presenting features of Denys Drash?
Gonadal dysgenesis Nephropathy
112
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/clear_cell_sarcoma_-_very_high_mag-14C0F3F895528FD332D.jpg
Clear cell sarcoma of kidney (Bone metastasizing tumor)
113
What is it? What has the same genetic mutation?
Rhabdoid tumor Atypical teratoid rhabdoid tumor of brain (15%)
114
What mutation?
t(12;15) ETVt-NTRK3 Mesoblastic nephroma (well circumscribed proliferation of spindled cells)
115
Two lesions containing the t(12;15) ETVt-NTRK3 mutation?
Mesoblastic nephroma Infantile fibrosarcoma
116
Rb gene is where?
13q14
117
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/png/1crgr130mvz5y47fs07b-w_m-14C0F45BF6064A99D03.png
Retinoblastoma (Flexner-Wintersteiner rosettes)
118
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/desmoplasticsmallroundbluecelltumor14-14C0F46DEB9421E0F0C.jpg
Desmoplastic small round cell tumor T(11;22), EWS-WT1, different breakpoints than Ewings
119
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/gliageek_atypical_20teratoid_20tumor_20x400-14C0F4F5708142CDE78.jpg
Atypical teratoid/rhabdoid tumor
120
What do you want to look for in Atypical teratoid/rhabdoid tumor?
Loss of INI1
121
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/7-17bl-14C0F50FCB65D560772.jpg
Medulloblastoma
122
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/1134815-1157758-231-14C0F51D29B4475792D.jpg
Adamantinomatous craniopharyngioma
123
Top three primary hepatic neoplasms in children?
Hepatoblastoma HCC Infantile hemangioendothelioma
124
Hepatoblastomas usually occur where?
Unifocally from right lobe of liver
125
What two syndromes are associated with hepatoblastomas?
Beckwith-Wiedemann FAP
126
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/liver_hepatoblastoma5_fetalpattern-14C0F56CF1B6BDB4203.jpg
Hepatoblastoma, epithelial type (six different types)
127
Most common trisomy in abortuses?
Trisomy 16
128
Polydactyly common in which trisomy?
Tri 13
129
What is the most common terminal deletion syndrome?
Del 5p (cri du chat)
130
How to remember DiGeorge syndrome/velocardiofacial syndrome?
CATCH 22 | Cardiac, Abnormal face, T cell deficit, cleft palate, hypocalcemia 22q11 microdeletion
131
CP concern with DiGeorge syndrome?
Irradiated blood products.  T cell defect increases chance of transfusion related GVHD
132
Only mucopolysaccharidosis that isn't autosomal recessive?
Hunter's (X linked recessive)
133
Hurler syndrome deficiency
α-L-iduronidase deficiency
134
Three organisms that cause recurrent infections in cystic fibrosis?
S. aureus P. aeruginosa Burkholderia cepacia
135
Characteristic finding in males with cystic fibrosis?
absent vas
136
What is maternal PKU?
Mothers with PKU who are not diet restricted during pregnancy have heterozygote infants with mental retardation, microcephaly, and congenital heart disease
137
What is the Guthrie test?
Test for PKU, preferably 48-72 hours after birth, that depends on a bacillus bacteria that becomes more active in the presence of excess phenylalanine
138
What are the 6 cyanotic congenital heart diseases? (that cause right to left shunt)?
``` Tetrology Transposition Total anomalous pulmonary venous return Tricuspid atresia/stenosis Truncus arteriosus Pulmonary stenosis ```
139
6 Acyanotic congenital heart diseases that cause a left to right shunt?
``` Atrial stenosis ASD VSD PDA Hypoplastic left heart Coarctation ```
140
Most common cyanotic heart disease?
``` tetrology of fallot   Pulmonary stenosis   Overriding aorta   VSD       (which cause) right ventricular hypertrophy ```
141
Metabolic defect in pyloric stenosis? Presentation?
Hypochloremic alkalosis First born boy with projectile vomiting
142
Most common cause of hydrops fetalis? Two others?
Thalassemia Twin-twin transfusion Chronic fetomaternal hemorrhage
143
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/peri136-14C7B23EA976157B0A8.jpg
Adrenal cytomegaly in Beckwith-Wiedemann syndrome Not a neoplasm
144
Where does the intraventricular hemorrhage of prematurity come from?
Germinal matrix; high blood flow, fragile vessels Increased venous pressure due to underlying immature lung disease
145
Most common part of the gut affected by necrotizing enterocolitis?
ileocecal region
146
https://s3.amazonaws.com/classconnection/76/flashcards/7519076/jpg/monochorionic_diamniotic_twins__intervening_membrane-14C7B2C9AB84CD0F73F.jpg
Diamniotic monochorionic
147
When does twin-twin transfusion overwhelmingly occur?
In DiMo pregnancies.  Virtually unheard of in DiDi and MoMo
148
Asymmetric IUGR mostly caused by?
Maternal factors (vascular disease, drugs, cigarettes) Head is spared
149
Symmetric IUGR mostly caused by?
Fetal causes: chromosomes, infections, early uterine injury
150
Most common pediatric tumor?
Hemangioma
151
What is Kasabach-Merritt syndrome?
Infant with large hemangioma sufficient to cause thrombocytopenia, anemia, splenomegaly, purpura, and even death
152
Staining profile for rhabdomyosarcoma? (6)
``` Sarcomeric actin Desmin Myosin Myoglobin Nuclear myogenin MYOD1 ```
153
5 positive stains in Wilm's Tumor?
``` WT1 CD56 Vimentin Desmin MyoD1 ```
154
Most common place for WT to metastasize?
Lungs
155
What do neuroblastomas secrete?
Catecholamines
156
Most common congenital malignancy?
Cutaneous metastatic nodules of congenital neuroblastoma "blueberry muffin" baby 3-7% involute with necrosis, maturation to ganglioneuroma
157
What is the most common mesenchymal tumor of the liver in childhood?
Infantile hemangioendothelioma

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