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GI/GU, Heme, Onco Flashcards

1
Q

inability to retract foreskin

A

phimosis

physiologic (no tx) vs pathologic (tx required)

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2
Q

painful erection
irritation/bleeding
dysuria

A

phimosis

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3
Q

Phimosis treatment

A

stretching exercises wth moisturizer
topical corticosteroid (ie betamethasone)
circumcision

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4
Q

retracted skin in uncircumcised male that cannot be returned to natural position

A

paraphimosis

impaired venous flow - engorgement - arterial compromise

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5
Q 
swelling of penis
penile pain
irritability in non-verbal infant
edema and tenderness of glans
penile shaft flaccid and unaffected
color change if ischemia
A

paraphimosis

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6
Q

Paraphimosis treatment

A

medical emergency

manual reduction in office or ED for dorsal slit procedure

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7
Q

congenital anomaly that results in abnormal ventral displacement of urethra

A

Hypospadias and chordee (abnormal penile curvature)

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8
Q

abnormal foreskin
abnormal penile curvature
2nd opening (1 false)

A

Hypospadias

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9
Q

Hypospadias treatment

A

check normal penile length and curvature
refer to urology
circumcision NOT to be done during newborn period
surgery 6mo

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10
Q

testis not within the scrotum and does not spontaneously descend into scrotum by 4mo
suprascrotal most common

A

Cryptochidism

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11
Q

absent testicle UL or BL with flat underdeveloped scrotum

absent, undescended, retractile or ascending/ectopic

A

Cryptochidism

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12
Q

Cryptorchidism treatment

A

70% spontaneous descent (rare after 6mo)

surgery before 2yo

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13
Q

abrupt onset sever testicular or scrotal pain (may radiate)
n/v
edematous and erythematous scrotum
tender, swollen, slightly elevated testis
absent cremasteric reflex
- phren’s sign (+ = relief of pain when scrotum is elevated epididymitis)

A

Testicular torsion

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14
Q

Testicular torsion diagnostics and treatment

A

doppler US confirms
*medical emergency - immediate consult and surgical detorsion and fixation of both testes
within 4-6 hours = 100% viable
after 24 hours = 0% viable

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15
Q 
younger children:
fever
vomiting
irritability
poor appetite

older children:
dysuria and/or frequency
new onset urinary incontinence
abdominal and back pain

A

Urinary Tract Infection (UTI)

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16
Q

Urinary Tract Infection (UTI) risk factors

A 
female
urinary tract anomalies
sexual activity
catheterization
VUR

girls and uncircumcised boys under 2yo with at least 1 risk factor for UTI are most at risk

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17
Q

Urinary Tract Infection (UTI) diagnostics and treatment

A

UA and culture and sensitivity (C&S)
+ leukocyte esterase (produced when WBC dying)
+ nitrites (G- rods like E.coli)
full septic workup for neonates esp if febrile
*RBUS = 1st line imaging study

abx PO/IV empirically until sensitivity results - 7-14days
amoxicillin, augmente, cephalosporin, Bactrim
f/u UA/C&S not required

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18
Q

When to do RBUS

A

under 2yo with first febrile UTI
any age with recurrent UTI
UTI and +FH renal/urologic disease, poor growth, HTN
don’t respond to abx

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19
Q

retrograde flow of urine from bladder into upper urinary tract

A

Vesicoureteral Reflux (VUR)

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20
Q

hydronephrosis (swelling of kidney) prenatally

febrile UTI in odler child

A

Vesicoureteral Reflux (VUR)

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21
Q

Vesicoureteral Reflux (VUR) diagnostics and treatment

A

*voiding cystourethrogram (VCUG)

low dose prophylactic abx (i.e. Bactrim)
surgical options
aggressive screening with UA if UTI sxs

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22
Q

urinary incontinence during the day after 4yo or at night after 5yo (girls) or 6yo (boys)

A

Enuresis

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23
Q

Enuresis diagnostics and treatment

A

UA with specific gravity (can child concentrate urine? DM)
KUB

behavioral modifications vs pharmacology
timed voiding, increase hydration, no fluids 90min before bed
desmopressin (DDAVP - synthetic ADH) over 6yo

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24
Q

painful or painless urinating
parent complaining child’s urine is red or brown

check BP, edema, skin for purport, CVA and abd tenderness

A

Hematuria

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25
Hematuria diagnostics
``` microscopic = 3+ RBCs per hpf urine cx if UTI suspected Ca/Cr ratio (over 2.0 abnormal) RBUS f/u yearly ``` ``` gross = COLORED UA and cx BUN/Cr and Ca/Cr with GFR ASO and ANA titers (infection) RBUS ```
26
``` gross hematuria - dark colored urine elevated serum Cr edema HTN urine microscopy with elevated RBCs and *CASTS ```
Glomerular Disease (glomerulonephritis)
27
acute onset cola-colored urine renal insufficiency +ASO titer recent infection with or without culture (1-2wk post infection)
Post-infectious glomerulonephritis (GN)
28
``` abd pain +/- bloody diarrhea maculopapular purpuric rash renal involvement varies (often microhematuria) arthritis/arthralgia ```
Henoch-Schonlein Purpura (HSP) - GN IgA autoimmune response to infectious process that may occur post-URI
29
bloody diarrhea hemolysis renal fault (hematuria) electrolyte problems --> seizures *associated with E.coli O157:H7 infection
Hemolytic-Uremic Syndrome (HUS) *most common glomerular vascular cause of acute renal failure in childhood
30
``` microhematuria proteinuria HTN deafness visual disturbances ```
Alport syndrome hereditary GN - autosomal dominant X-linked
31
Nephrotic Syndrome characteristics
nephrotic range proteinuria (over 1000mg/24hr) hypoalbuminemia edema (esp. of the face) hyperlipidemia
32
``` 2 presentations: "happy spitter" - growing well, healthy clinically "unhappy spitter" FTT fussy/dystonic neck posturing resp. complications feeding refusal occult blood in stool ```
``` Gastroesophageal Reflux (GER) GERD if sxs present ``` common under 6mo
33
Gastroesophageal Reflux (GER) treatment
positional therapy elimination diet or change formula thickened, small, frequent feeds meds: H2 blocker (ranitidine) under 1yo PPI (lansoprazole) over 1yo
34
projectile, non-bilious vomiting after feeding "hungry vomiter" FTT and dehydration distended upper and after feeding prominent peristaltic waves L --> R "olive" sized mass in RUQ
Pyloric stenosis 3-12wks old
35
Pyloric stenosis diagnostics and treatment
CBC, CMP US test of choice UGI if US non-diagnostic --> string sign IV fluids and electrolytes pyloromyotomy
36
bile-stained vomiting first 24-48hrs of life mild abd distention *failure to pass meconium
Congenital Atresia
37
Congenital Atresia types
duodenal (8-10wk gestation) - associated with trisomy 21 embryonic development jejunoileal (over 11-12wk gestation) - associated with CF uterovascular accident colonic (least common) unknown
38
Congenital Atresia diagnostics and treatment
CMP KUB - *double bubble* sign for duodenal atresia dilated loops of bowel and air fluid levels for J/C UGI/contrast enema confirm dx and ID area of obstruction IV fluids, electrolytes, possibly TPN surgery broad spec abx to prevent infection
39
bilious vomiting abd pain, tenderness and distention +/- hematochezia visible peristalsis
Midgut malrotation +/- volvulus under 1mo
40
Midgut malrotation +/- volvulus diagnostics and treatment
KUB UGI - corkscrew appearance barium enema to confirm (only as adjunct) surgical intervention
41
``` intermittent, severe cramps abd pain cries and draws legs to chest vomiting - does not feed *currant-jelly* stools palpable sausage shape in RUQ ```
Intussusception most frequent cause of intestinal obstruction in first 2yr of life
42
Intussusception diagnostics and treatment
CBC, CMP abd US IV fluids surgical consult air enema US-guided = reduces with 74% success
43
painless rectal bleeding obstruction diverticulitis *can mimic appendicitis
Meckel's Diverticulum
44
Meckel's Diverticulum Rule of 2's
2% population 2:1 M:F ratio 2% symptomatic
45
Meckel's Diverticulum diagnostics and treatment
technetium-99 scan (Meckel's scan) surgical resection of diverticula and remnant (vitelline duct)
46
``` anorexia migrating abd pain (periumbilical to RLQ) vomiting after onset pain fever guarding rebound tenderness +Rovsing, obturator and ileopsoas signs ```
Appendicitis common in 2nd decade of life rare under 5yo
47
Appendicitis diagnostics and treatment
H&P US elevated WBC fluids, electrolytes and abx pre-op appendectomy
48
failure to pass meconium in first 48-72hr of life bilious vomiting abd distention reluctance to feed/irritable older children: FTT and chronic constipation "squirt" or "blast" sign with releasing finger from anal canal
Hirschsprung's Disease first 6wk of life more than 20% develop enterocolitis
49
occurs secondary to absence of ganglion cells in the mucosal and muscular layers of the colon peristaltic waves cannot extend beyond this zone of de-nervation
Hirschsprung's Disease most common cause of lower bowel obstruction in neonates
50
Hirschsprung's Disease diagnostics and treatment
rectal biopsy is GOLD STANDARD contrast enema resection of aganglionic segment colostomy colorectal anastomosis after bowel has rested
51
``` diarrhea/abd pain +/- hematochezia weight loss and growth failure arthritis uveitis nutrient deficiency and anemia ```
Inflammatory Bowel Disease Crohn's and Ulcerative colitis 20-30% present under20yo more prevalent in whites
52
Inflammatory Bowel Disease diagnostics and treatment
colonoscopy Crohn's = skip lesions, cobblestone appearance Ulcerative colitis = continuous involvement, erythematous and friable mucosa with small erosions disease maintenance (anti-inflammatories) biologics and immunomodulating agents steroids used for flare-ups ONLY
53
``` fever severe abd pain bloody stool/vomiting dehydration leukocytosis growth/development issues ```
Diarrhea >3 watery stools per day infectious vs diet related
54
Diarrhea treatment
hydration abx sometimes anti-motility agents rarely
55
``` +/- fecal leakage (encopresis) abd discomfort decreased bowel sounds and distention stool mass may be palpated in LLQ anal/rectal exam --> fissures, anatomic placement, check for fecal impaction ```
Constipation increased prevalence 5-6yo
56
Constipation red flags
``` weight loss/poor growth anorexia, fever, hematochezia, vomiting hx delayed passing of meconium acute onset failure to respond to conservative measures ```
57
Constipation diagnostics and treatment
``` CBC, CMP, TTG, IgA, TSH abd xray r/o impaction MRI spine (neuro) rectal biopsy (Hirschsprung's) barium enema (anatomical malformations) ``` ``` decrease dairy intake polyethylene glycol to relieve impaction laxative therapy counseling GI referral if worse or alarming sxs ```
58
Neonatal and Breast Milk Jaundice
hyperbilirubinemia (indirect bili most commonly) benign - resolves spontaneously breast milk = appears in 1st wk of life
59
Pathological Jaundice
hyperbilirubinemia that requires intervention ``` diagnostics: serum bili maternal blood group and Ab titers baby blood group Coombs test (hemolytic anemia) CBC, retic count, peripheral smear septic/infectious workup ```
60
jaundice dark urine light stools increase in direct bilirubin treat with liver transplant
Post-Hepatic Jaundice (Biliary Atresia) rare disease that destroys the bile ducts
61
lethargy tachycardia pallor irritability and poor oral intake (infants)
Anemia (acute)
62
abnormal skin pigmentation short stature skeletal malformations progressive pancytopenia
Fanconi anemia autosomal recessive up to 90% develop bone marrow failure in first 10yr of life
63
Fanconi anemia diagnostics and treatment
pancytopenia bone marrow hypoplasia or aplasia (hypocellular) *often misdiagnosed as ITP supportive hematopeoitic stem cell transplant (HSCT) is DEFINITIVE tx
64
weakness/fatigue pillow frequent, severe infections purpura, petechiae, bleeding
Acquired Aplastic Anemia
65
Acquired Aplastic Anemia diagnostics and treatment
normocytic anemia pancytopenia low relic count *overwhelming infection and severe hemorrhage are leading COD supportive (transfusions) stop offending agent abx if infection HSCT if absolutely needed
66
``` sometimes asymptomatic pallor fatigue/irritability played motor development hx of Pica (non-nutritive cravings) ```
Iron Deficiency Anemia 1-2yo and females of child-bearing age AA and Hispanics more than whites
67
Iron Deficiency Anemia diagnostics and treatment
microcytic, hypochromic anemia low Hgb and ferritin ``` close monitor iron supplement (6mg/kg/d) ```
68
pallor glossitis specific: paresthesia, weakens, unsteady gait decreased vibratory and proprioception
Megaloblastic Anemia vitamin B12 deficiency (neuro deficits) folic acid deficiency
69
Megaloblastic Anemia diagnostics and treatment
macrocytic (elevated MCV and MCH) decreased folic acid and/or vitamin B12 *hypersegmented nuclei in large neutrophils Vitamin B12 and/or folic acid supplementation --> vit B12 shows methylmalonic acid
70
Congenital Hemolytic Anemias
``` Hereditary Spherocytosis (HS) Thalassemia Sickle Cell disease G6PD Deficiency lead poisoning ```
71
Hereditary Spherocytosis (HS)
hemolytic anemia red cell membrane defect = trapped in spleen jaundice, gallstones, splenomegaly spherocytes on peripheral smear *increased osmotic fragility supportive, +/- RBC transfusion splenectomy
72
Thalassemia (alpha or beta)
hemolytic, microcytic, hypochromic anemia *Hbg electrophoresis for dx supportive, +/- RBC transfusion iron monitoring splenectomy (wait intel at least 6yo) HSCT for severe beta thalassemia
73
Sickle Cell disease
``` hemolytic anemia homozygous HbSS vaso-occlusion = PAIN (abd esp.) gallstones, jaundice, splenomegaly and functional asplenia growth failure and delayed puberty ``` Hgb electrophoresis *Jolly bodies in asplenic pts. avoid precipitating factors *hydroxyurea helps painful episodes stem cell transplant
74
G6PD deficiency
hemolytic anemia red cell ENZYME defect with X-linked recessive inheritance episodic hemolysis at times of exposure to oxidative stress or certain drugs/food substances *Heinz bodies on peripheral smear (denatured Hgb) supportive and avoid triggers
75
Lead Poisoning
mild hemolytic normocytic anemia *basophilic stippling on peripheral smear chelation therapy
76
splenomegaly and plethora (deep red color) h/a and lethargy (increased blood viscosity = thrombosis) elevated Hgb tx: phlebotomy
Congenital Erythrocytosis (inherited polycythemia)
77
occurs in response to hypoxemia tx: correction of underlying disorder (i.e. chronic pulmonary disease or cyanotic congenital heart disease) phlebotomy when indicated
Secondary Polycythemia (acquired - more common)
78
Bleeding disorder screening
CBC peripheral smear PT/INR (extrinsic pathway - 7 and TF) - prothrombin time monitor Warfarin tx aPTT (intrinsic pathway - 8, 9, 11, 12) - activated partial thromboplastin time +/- bleeding time (prolonged in platelet disorders) differentiate von Willebrand disease vs hemophilia
79
multiple petechiae ecchymosis epistaxis 2-5yo most common and often following a viral infection
Idiopathic Thrombocytopenia Purpura (ITP) most common bleeding disorder of childhood immune-mediated against own platelets
80
Idiopathic Thrombocytopenia Purpura (ITP) diagnostics and treatment
thrombocytopenia normal WBC and Hgb (unless hemorrhage) normal PT and aPTT *diagnosis of exclusion ``` 90% have spontaneous remission *prednisone avoid NSAIDs/ASA (compromise platelet function) bleeding precautions IVIG for more severe bleeding splenectomy for emergent scenarios ```
81
prolonged bleeding from mucosal surfaces epistaxis, menorrhagia, GI easy bruising
von Willebrand Disease most common INHERITED bleeding disorder autosomal dominant
82
von Willebrand Disease diagnostics
``` normal PT prolonged or normal aPTT (vWF is a co-protein for factor 8) normal or decreased factor 8 normal or deceased vWF *prolonged bleeding time ```
83
von Willebrand Disease treatment
desmopressin (synthetic ADH) causes release of vWF and factor 8 from endothelium vWF replacement therapy
84
bleed in response to injury/trauma or surgery severe, spontaneous bleeding onset of bleeding may be delayed by several days commonly occurs INTO joints and muscles --> spontaneous hemarthrosis is very severe and can lead to joint destruction and deformity
Hemophilia A and B type A = factor 8 deficiency (rare) type B = factor 9 deficiency (Christmas disease) X-linked inheritance
85
Hemophilia diagnostics
normal platelet count, PT and bleeding time prolonged aPTT (intrinsic pathway) may be normal in mild disease normal vWF
86
Hemophilia treatment
``` Desmopressin (type A) factor replacement (more effective) ```
87
shock (end organ dysfunction) hematuria, petechiae, purpura persistent oozing from needle puncture evidence of thrombotic lesions
Disseminated Intravascular Coagulation (DIC) hemorrhage and microvascular thrombosis *clotting AND bleeding problem
88
Disseminated Intravascular Coagulation (DIC) diagnostics
decreased platelets prolonged aPTT and PT decreased fibrinogen level increased D-dimer and fibrin degradation products *everything is abnormal
89
Disseminated Intravascular Coagulation (DIC) treatment
ID and treat triggering event (sepsis, trauma or malignancy) replacement therapy anticoagulation therapy when indicated
90
Liver disease and vitamin K deficiency
Liver is major site of synthesis for: prothrombin, fibrinogen, factors 5, 7, 9, 10, 12, 13 normal to low platelet count prolonged PT and aPTT Vitamin-K dependent factors include: 2, 7, 9, 10 normal platelet count prolonged PT and aPTT treat underlying condition and give vitamin K at birth
91
Inherited Thrombotic Disorders
Protein C, Protein S and Antithrombin deficiencies Factor V Leiden Mutation hypercoaguable increased risk for thrombosis may see DVT or PE + family hx thrombosis
92
Protein C deficiency
activated protein C inactivates activated factors 5 and 8 stops the clotting process when you don't need it pts may develop Warfarin-induced skin necrosis
93
Protein S deficiency
protein S is a cofactor that activated protein C to help stop the clotting process when you don't need it
94
Factor V Leiden Mutation
more commonly seen -- point mutation factor 5 resistant to inactivation by activated protein C 35-fold increased risk of indecent VTE if taking oral contraceptives
95
Antithrombin deficiency
major physiologic inhibitor of thrombin clinically presents as VTE *efficiency of heparin may be significantly diminished
96
Inherited Thrombotic Disorders treatment
``` anticoagulation prophylaxis (UFH, LMWH, Warfarin) 1st episode VTE = anticoag for min 3mo ```
97
Henoch-Schonlein Purpura (HSP) diagnostics and treatment most common small vessel vasculitis boy 2-7yo
normal platelet (despite purpura) elevated ASO titer elevated serum IgA supportive
98
uncontrolled proliferation of malignant lymphoid precursor clones most common pediatric CA (3-7yo) stain with TdT pancytopenia with circulating blasts bone pain fatigue
Acute Lymphoblastic Leukemia (ALL)
99
uncontrolled proliferation of malignant myeloid precursor clones stain with myeloperoxidase *Auer rods on peripheral smear pancytopenia with circulating blasts bone pain fatigue
Acute Myeloid Leukemia (AML)
100
Acute Leukemia (ALL/AML)
pancytopenia - pillow, tachycardia, SOB, ecchymosis, fever infiltration of organs skin gum hypertrophy, CNS and thymic mass (ALL) liver, spleen and nodes
101
Acute Leukemia (ALL/AML) diagnostics
``` *bone marrow biopsy confirms CBCD (blasts) and CMP flow cytometry (looks for abnormal WBC) coag tests (DIC) lumbar puncture if neuro sxs (ALL) uric acid ```
102
Acute Leukemia (ALL/AML) treatment
``` hydration transfusions PRN abx allopurinol or rasburicase to decrease uric acid +/- allogenic transplant with chemo ```
103
``` may be asxs fever, dyspnea anorexia and early satiety (splenomegaly) weight loss night sweats ``` strikingly elevated WBC found on routine CBC
Chronic Myeloid Leukemia (CML) translocation of philadelphia chromosome
104
Chronic Myeloid Leukemia (CML) treatment
*Gleevec (TKI) shuts down transcription/translation hydrea (brings down WBC if too high) allogenic transplant monitor
105
``` painless, persistent LAD hepatosplenomegaly night sweats pruritic mediastinal bulky mass ```
Lymphoma (Non-Hodgkin's or Hodgkin's)
106
Non-Hodgkin's Lymphoma (NHL)
slow professing to very aggressive in adults high grade in children (requires treatment) 5th most common peds CA under 15yo
107
Hodgkin's Lymphoma
bimodal (teens-30s, 50s) EBV exposure *Reed Sternberg cells on cytology painful LAD after EtOH supraclavicular nodes
108
Lymphoma (Non-Hodgkin's or Hodgkin's) diagnostics
``` *excisional node biopsy differentiates CBC, CMP, LDH (lactic dehydrogenase = cell turnover) uric acid CT neck, chest, abd, pelvis bone marrow biopsy for staging ``` 2D ECHO if on Adriamycin (can cause DCM or leukemia) pulmonary testing if on Bleomycin
109
Non-Hodgkin's Lymphoma (NHL) treatment
chemo autologous stem cell transplant supportive (allopurinol/rasburicase) steroids are toxic for lymphocytes good prognosis
110
Hodgkin's Lymphoma treatment
chemo +/- radiation (even in children) stem cell transplant for relapse increased risk for leukemia and other lymphomas steroids are toxic for lymphocytes
111
Lymphoma staging
I - 1 area of nodes II - 2 areas of nodes on 1 side of diaphragm III - nodal sites on BOTH sides of diaphragm IV - involvement of extranodal sites (i.e. bone marrow) B sxs = night sweats, weight loss and bulky nodes A = none of the above E = extra nodal site contiguous with nearby node
112
pain and swelling commonly femur or tibia fever, malaise weight loss corresponds with growth spurts
Osteosarcoma most common primary bone malignancy in children and adolescents
113
Osteosarcoma diagnostics and treatment
increased alk phos and LDH MRI CT for mets *biopsy to confirm surgery + chemo + radiation
114
pain and swelling +/- fever 12% have urogenital anomalies high chance of relapse
Ewing's Sarcoma peak 10-15yo males
115
Ewing's Sarcoma diagnostics and treatment
CBC, CMP, LDH CT/MRI PET if you think metastatic (commonly to lungs) *biopsy to confirm ``` surgery chemo (cytoxan and doxyrubicin) +/- radiation ```
116
``` n/v seizures, syncope, hemiparesis papilledema dysphagia personality changes CN palsies FTT ```
Brain Tumor (metastatic vs primary) glial more common than nonglial 2nd COD for neuro
117
Brain Tumor diagnostics and treatment
MRI confirms f/u MRI indicated (progression vs. pseudo) surgery +/- radiation (temodar) and chemo
118
``` abd mass/pain fever, weight loss Horner's syndrome bone pain anemia ``` can synthesize and secrete catecholamines
Neuroblastoma most common solid neoplasm in children 40% adrenal gland
119
Neuroblastoma diagnostics and treatment
bone marrow biopsy abd imaging urine catecholamines watch and wait if low risk surgery (+ chemo if mets to spine) under 1yo better prognosis
120
abd swelling fever hematuria HTN BL involvement in younger children
Nephroblastoma (Wilms tumor) most common renal malignancy under 15yo vs renal cell CA 15-19yo
121
Nephroblastoma (Wilms tumor) diagnostics and treatment
CT or MRI chemo + surgery or surgery + chemo
122
leukocoria (white reflection from retina) sporadic or genetic cause deadly
Retinoblastoma most common intraocular CA of childhood
123
Retinoblastoma diagnostics and treatment
opthal exam intraocular US or CT MRI enucleation (removal of eye) external radiation chemo
124
``` affects soft tissue can resemble fat, muscle or fibrous tissue often in head/neck in younger children also orbit, GU, extremities very rare ```
Rhabdomyosarcoma arises from primitive mesenchymal cells
125
Rhabdomyosarcoma diagnostics and treatment
MRI and CT or PET surgery, chemo, radiation
126
Hepatic Tumors
commonly a primary tumor found by a family member could be mets from Wilms tumor or rhabdomyosarcoma hepatocellular CA 0-4yo and 10-14yo hepatoblastoma under 5yo - R side of liver both require additional imaging to ensure it is not a benign tumor (i.e. adenoma or hemangioma) tx: surgical resection

Pediatrics (3 decks)

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