18.6.2013(pediatrics)41 Flashcards
Incision site in pediatric tracheostomy
Midway Btw lower border of thyroid cartilage and supra sternal notch(skin incision)
Vertical incision over trachea
Over 3rd and 4th tracheal rings
Indications for tracheostomy
Intubation not possible
Assisted ventilation needed for more than 4-6 weeks
Complications of tracheostomy
Hemorrhage
Subcutaneous Emphysema
Pneumothorax
Pneumomediastinum
Most common early complication of pediatric tracheostomy
Subcutaneous emphysema
Extent of trachea
C6 to T4
Diff btw pediatric and adult tracheostomy
Tube is not cuffed in pediatric tracheostomy
$ Common sites of mongolian spot
Presacral area Buttocks(common site) Thigh Legs Back Shoulder
Cause of mongolian spot
Arrest of melanocytes in mid dermis during migration
Age of onset of scurvy in infants and children
6-24months
Diff btw scorbutic and rachitic rosary
Sharp and angular in scurvy
Dome shaped and semicircular in rickets
Cause of scorbutic rosary
Separation of epiphysis of ribs
Backward displacement of ribs
Features of vit C deficiency
Subperiosteal hemorrhages Pseudo paralysis Scorbutic rosary Gum changes(more over incisor) Sicca syndrome Poor wound healing Swollen joints Peri follicular hemorrhages Hyperkeratosis of hair follicles
Manifestation of severe vit C deficiency
Skeletal muscle degeneration
Cardiac hypertrophy
Bone marrow depletion
Adrenal atrophy
Bone changes of rickets are common in
Knee
Wimburger sign is seen in
Congenital syphilis
Vit C deficiency
Skeletal features of vit C deficiency
Osteoporosis Signet ring epiphysis Pencil thin cortex Sub periosteal hemorrhage Corner sign Trummerfield zone Pelken sign White line of frenkel(at metaphysis) Wimberger sign
Wimberger sign
Epiphyseal centres of ossification have ground glass opacity,surrounded by white ring
Corner sign
Well calcified cartilage projecting beyond the limits of shaft
Pelken sign
Epiphyseal spur
Trummerfield zone
Fragmentation above calcified cartilage in metaphysis
Better indicator of vit C stores
Leukocyte vitamin C
Craniotabes is seen in
Rickets
Osteogenesis imperfecta
Hydrocephalus
Syphilis
Erythema toxicum disappears in
1-3 days
Erythema toxicum spares
Palms and soles
Port wine stain is a _________ newborn finding
Normal
But doesn’t disappear spontaneously
Causes of splinter hemorrhage
1.minor trauma SABE vasculitis Severe RA peptic ulcer disease Hypertension Chronic glomerulonephritis Cirrhosis Scurvy Malignant neoplasm Trichinosis Psoriasis
COCPR
Compression only or hands only CPR
Should be performed by lay providers
Sequence of events in CPR
CAB except in newborns
Nonperfusing arrythmias
Also known as Malignant arrythmias VF pulseless VT PEA pulseless bradycardia Asystole
Two rescuer CPR in infants and children
15:2
defibrillator used in
Even in infants
Depth of chest compressions
Atleast 2 inches(adult)
Atleast 1/3rd depth of chest(children)
Syndromes associated with Wilms tumor
WAGR Denys drash Beckwidth wideman Hemihypertrophy Sporadic aniridia Genitourinary abnormalities NF1 Sotos syndrome Pearlman syndrome Von wilibrand disease
Mutations in WAGR
Deletion Chr 11p13
WT1 and aniridia gene PAX6 are located
Denys drash
Male pseudohermaphroditism
Early renal failure(mesangial sclerosis)
Missense mutation in WT1 gene
Beckwidth wideman
Hemihypertrophy
Visceromegaly
Macroglossia
Adrenal cytomegaly
Mutation in Beckwidth wideman
WT2 in chr11
Diff btw neuroblastoma and Wilms tumor
Stippled calcification and hemorrhage
Pushes the kidney inferolaterally,without distortion of collecting system
Crosses the midline
Lung metastasis is rare
Paraneoplastic syndrome (opsomyoclonus)
Can extend into spinal canal and cause compression
Neuroblastoma pushing the kidney inferolaterally without distortion of collecting duct
Drooping lily sign
Tumor markers in neuroblastoma
HVA
VMA
Most common site of metastasis of neuroblastoma
1.Long bones and skull Bone marrow Liver Skin Lymphnode
Mean age of diagnosis of Wilms tumor
3 yrs
80% cases are less than 5 yrs
Rule of 10 in Wilms tumor
10% B/L Unfavourable histology Vascular invasion Pulmonary mets at presentation Calcification
Histological variants in neuroblastoma
Neuroblastoma
Ganglioneuroma
Ganglioneuroblastoma
Diploidy and prognosis of neuroblastoma
Hyperdiploidy,near triploidy indicates better prognosis if age of onset is less than 1 yr
Near diploid indicates worse prognosis
Stage 4S neuroblastoma
Less than 1 yr Subcutaneous nodules Small primary tumor No bone involvement Massive liver
Neurological manifestations of neuroblastoma
Horner syndrome
Cord compression
Opsomyoclonus (dancing eyes)
Ataxia(dancing feet)
Hormones secreted by neuroblastoma
catacholeamines
VIP
Chemotherapy for neuroblastoma
Cisplatin or carboplatin
Cyclophosphamide
Etoposide
Doxorubicin
Familial predisposition to neuroblastoma
ALK gene (anaplastic lymphoma kinase) mutation
IHC staining of neuroblastoma
Neuron specific enolase
Histological picture of neuroblastoma
Homer wright pseudo rosettes
Dense core granules(contain catacholeamines)
Mature neuroblastoma
Contains Schwann cells
Chr abnormalities in neuroblastoma indicating poor prognosis
1p and 11q loss
17q gain
Poor prognostic factor in neuroblastoma,cellular expression
NMYC amplification
TRK B
telomerase expression
TRKA indicates good prognosis
Age,prognosis in neuroblastoma
Less than 18 months indicates good prognosis
Stages of neuroblastoma with good prognosis
Stage 1,2,4S
Mitosis-karyorhexis index in neuroblastoma
<200/5000 indicates good prognosis
Stools of formula fed infants
Bacteroids
Clostridium
Breast fed infants have bifidobacterium
pH of breast fed infant stool
Acidic because of acetate produced by bifidobacterium
SGA
Weight less than 10 percentile for their gestational age
Or below 2 standard deviations
Causes of bossing of skull
Rickets Thalassemia Congenital syphilis Achondroplasia Hurler Lowe Cleidocranial dysostosis Ectodermal dysplasia Ehlers danlos
Causes of craniotabes
Physiological Rickets Congenital hydrocephalus Osteogenesis imperfecta Lacunar skull Treacher collins
Breast feeding in HIV positive mother
Exclusively top feeding
Breast feeding for 6month and rapid weaning
Intusucception is associated with which infection
Adenovirus type C
NMYC amplification presents as
Extra chromosomal double minutes
Or
Homogenously staining region of other chromosomes
Location of N-myc
Chr 2p
Drug used for differentiation of neuroblastoma
Retinoids
Tumors in Denys drash syndrome
Wilms
Gonadoblastoma
Overgrowth in Beckwidth wideman is due to
IGF2
Mutations causing Wilms tumor
WT1 and WT2
Beta catenin
Increased risk of developing wilms tumor in opposite kidney is indicated by
Nephrogenic rest
Wilms tumor unresponsive to chemotherapy
P53 mutation
Intestine commonly involved in NEC
Distal illeum and proximal colon
Pathognomonic x ray findings in NEC
Pneumatosis intestinalis
Portal venous gas(severe disease)