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78 Cards in this Set
- Front
- Back
oligohydramnios effects
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pulmonary hypoplasia, altered facies, positioning defects of feet, breech presnetation Potters sequence
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causes of non immune fetal hdrops
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turners, down, edwards. A thalassemia, parvovirus, CMV, syphilis, toxoplasmosis, tumors, CV malformaitons, tachyarrythmias, diaphragmatic hernia
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cause of immune hydrops
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ab attachement to Rh+. Anemia. Cardiac decompensation. Hydrops
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unfavorable prognosis factors in neuroblastomas. Stage, age, histology, DNA ploidy, N-MYC
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stage 3,4. older than 18 months. Lots of mitosis, anaplasia. Amplified N-MYC
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massive anascara associated with cardiac decompensation and circulatory collapse
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hydrops fetalis
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bulky, pale placenta and hydrops fetalis indicates
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cytomegalovirus
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necrosis, calcification, and fibrosis within chorionic villi often seen in
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cytomegalovirus
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how is CMV trasnmitted to the fetus?
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transplacental.
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clinical manifestations of congenital CMV infection
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hydrops fetalis, swelling of placenta, jaundice, HSM, thrombocytopenic purpura, periventricular necrosis/calcification,pneumonia, deafness, brain damage
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group B strep and other bacterial organisms that can colonize the vaginal canal cause
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an ascending infection and infect placental membranes.
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bacterial contamination of amniotic fluid may lead to
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congenital pneumonia in infant
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PDA, cataracts, deafness
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rubella
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pneumonia alba(fibrosis in lungs), copious nasal discharge, periostitis
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syphillis
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myocarditis
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coxsackievirus B
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multifocal cerebral calcifications, chorioamniotis
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toxoplasmosis-cat, if exposed during pregnancy
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ascending infeciton, small necrotic lesions
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herpes simplex
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urine cytology diagnosis
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CMV
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hydrops, RBC nuclear inclusions
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parvovirus B19
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clinical manifestations of TORCH agents
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microencephaly, microopthalmia, focal cerebral calcifications, cataract, conjunctivitis, pneumonitis, heart disease, hepatomegaly, jaundice, petechiae
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850 gm female twin infant with resp distress. Given surfactant. Poor muscle tone, diminised Moro reflex. Convulsions
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hyaline membrane disease
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what do lungs that have hyaline membrane disease look like?
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firm, deep red,-liver like. Stiff and may sink
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what happens in the brain with infants with hyaline membrane disease/resp distress?
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intraventricular hemorrhage
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where is the intraventricular hemorrhage?
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germinal matrix then extends to ventricles
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cause of resp distress syndrome and thus (HMD) in infants?
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surfactant deficiency-immature lungs, maternal diabetes, twin gestation, hemorrhage, hypothermia
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significance of L/S ratio
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lecithin to sphingomyelin ratio in amniotic fluid. Measure of lung maturity-to assess surfactant
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complications of intraventricular hemorrhage
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seizures, epilepsy, hydrocephalus, white matter necrosis
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primary therapy for resp distress syndrome
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exogenous surfactant, antenatal corticosteroids
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complication of hyaline membrane disease
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bronchopulmonary dysplasia.(fibrosis on one side, expanded acini on the other)
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twin B was 825 gm female also with resp distress, abdomen distended, soft bloody stools. Hypotensive, lethargic, free air in abdomen
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neonatal necrotizing enterocolitis
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pneumatosis intestinalis (gas within wall of bowel) and gas in portal or hepatic veins indicates
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neonatal necrotizing enterocolitis
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what leads to free air in abdomen?
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perforation of necrotic bowel
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complications of NEC?
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malabsorption, liver prob, short bowel syndrome, fistula, sepsis
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risk factors associated with development of GI prob for NEC?
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prematurity, asphyxia, cyanotic congenital heart disease, low birth weight, maternal cocaine abuse
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treatment of NEC?
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stop feeding by mouth, antibiotics to treat infections, treat shock with fluid/electrolytes, surgery
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3 yr old male with firm painless mass in kidney. 140/90
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nephroblastoma-Wilms tumor
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what do wilms tumors look like?
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spherical, pale gray or tan, soft, friable. Sharply demarcated, some bilateral. Nodular,
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3 tissue patterns of Wilms tumors
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blastemal, epithelial, stromal
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what do blastemal tumors look like
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sheets of small round blue cells
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what do stromal tumors look like
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clusters of myxoid, fibrous, smooth muscle and adipose cells along with foci of bone, cartilage, skeletal m.
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foci of anaplastic cells may be present and are associated with?
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unresponsiveness of tumor to therapy
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anomalies and syndromes associated with Wilms tumor?
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WAGR, Denys-Drash, Beckwith-Wiedman, nephrogenic rests, trisomy 18
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WAGR syndrome presents with
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WT, genital anomalies, mental retardation, aniridia
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Denys Drash presents with
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WT, male pseudohermaphroditism, chronic renal failure
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Beckwith Wiedman presents with
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gigantism, everything megaly
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what stage is Residual non-hematogenous tumor confined to abdomen. Lymph nodes positive in hilum, periaortic chain. Residual tumor at margins.
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stage 3.
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treatment of wilms tumor
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tumor/kidney removal, chemotherapy
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what is an important prognostic feature for WT
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anaplasia
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other renal tumors seen in children
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mesoblastic nephroma, clear cell carcinoma, rhabdoid tumor
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Renal tissue lagging in maturation, which is found in 30-40% of Wilms tumor. They are associated with bilateral Wilms tumors.
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nephrogenic rests
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3 month old infant with watery diarrhear, left abdominal mass on kidney, extending across pidline.
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wilms, NEUROBLASTOMA-age.
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where are neuroblastoma tumors commonly seen?
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adrenal, retroperitoneum, mediastinum
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what do neuroblastoma tumors look like
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red, hemorrhagis with areas of gray-white tissue and small cysts, calcification often present
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histologic features of neuroblastoma tumors
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small round,blue cell. ROSETTES. Necrosis, calcification, ganglion cells
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most frequent single solid tumor in kids
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neuroblastoma
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what are some ways neuroblastoma may present
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watery diarrhea, large abdominal mass, fever, weight loss, blueberry muffin baby, bone pain, raccoon eyes
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what lab tests may be helpful in diagnosing neuroblastoma?
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VMA, HPA
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if the neuroblastoma tumor is located on the adrenal gland, unfavorable or favorable?
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Unfavorable
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if the neuroblastoma tumor is located on the liver, unfavorable or favorable?
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favorable. Stage IVS
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neural tube defects have what defiency
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folate deficiency
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anterior end of tube isnt properly closed
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anencephaly
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malformed CNS tissue extends thru a defect in cranium
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encephalocele
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most common form of NTD
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spina bifida-failure to close of caudal region of neural tube
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what protein is elevated in NTD
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alpha fetaprotein (AFP) except for spina bifida occulta
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1 yr old white female-resp difficulty. Low weight gain. Malodorous stools. Pseudomonas infections, pale. Distended abdomen, intercostal retractions
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CF
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what does the lung show in someone with CF
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distended bronchioles-mucus plugs. Bronchiectasis with lung abscesses
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a disorder of exocrine glands affecting both mucus-secreting and eccrine sweat glands, leads to viscid mucinous secretions and obstructive disease in the lungs, pancreas, and liver
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CF
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how is CF diagnosed
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DNA polymorphism, Alkaline phosphatase, SWEAT CHLORIDE test
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other causes of bronchiectasis
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Local bronchial obstruction, immotile cilia syndromes, post-infectious bronchiectasis and cartilage defects, Kartageners
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what agents would effect patients with CF most commonly?
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Pseudomonas aeruginosa (mucoid form), Staph. Aureus, Aspergillus
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defective gene in CF
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CFTR
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complications of CF
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pneumonia, chronic pancreatitis, infertility, pumonary infection
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what are components of Kartagener syndrome
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immotile cilia-dynein arms(bronchiectasis), infertility, cytus inversus, sinusitis
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pathogenesis of CF
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CFTR mutated. Impairs Cl- transport to lumen. Na, H20 absorbed, making secretion more viscid. Obstruciton-necrosis, fibrosis, infection.
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APGAR stands for
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appearance, pulse, grimace(reflex), activity, resp effort
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what does diagrphragmatic hernia lead to
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pulmonary hypoplasia
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how does diagphragmatic hernia lead to pulmonary hypoplasia?
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squishes the lungs. Cant develop. Liver also goes up into chest
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one of the most frequent occuring anomalies of lung and thorax
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diagphramatic hernias
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clinical differences between left and right sided diaphragmatic hernias
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left side more common-spleen, bowel, left lobeof liver. Right side-liver protects it
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