18.6.2013(pediatrics)41 Flashcards by rakesh durai

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

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Indications for tracheostomy

Intubation not possible

Assisted ventilation needed for more than 4-6 weeks

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Complications of tracheostomy

Hemorrhage
Subcutaneous Emphysema
Pneumothorax
Pneumomediastinum

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Most common early complication of pediatric tracheostomy

Subcutaneous emphysema

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Extent of trachea

C6 to T4

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Diff btw pediatric and adult tracheostomy

Tube is not cuffed in pediatric tracheostomy

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$ Common sites of mongolian spot

Presacral area
Buttocks(common site)
Thigh
Legs
Back
Shoulder

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Cause of mongolian spot

Arrest of melanocytes in mid dermis during migration

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Age of onset of scurvy in infants and children

6-24months

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Diff btw scorbutic and rachitic rosary

Sharp and angular in scurvy

Dome shaped and semicircular in rickets

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Cause of scorbutic rosary

Separation of epiphysis of ribs

Backward displacement of ribs

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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

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Manifestation of severe vit C deficiency

Skeletal muscle degeneration
Cardiac hypertrophy
Bone marrow depletion
Adrenal atrophy

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Bone changes of rickets are common in

Knee

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Wimburger sign is seen in

Congenital syphilis

Vit C deficiency

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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

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Wimberger sign

Epiphyseal centres of ossification have ground glass opacity,surrounded by white ring

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Corner sign

Well calcified cartilage projecting beyond the limits of shaft

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Pelken sign

Epiphyseal spur

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Trummerfield zone

Fragmentation above calcified cartilage in metaphysis

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Better indicator of vit C stores

Leukocyte vitamin C

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Craniotabes is seen in

Rickets
Osteogenesis imperfecta
Hydrocephalus
Syphilis

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Erythema toxicum disappears in

1-3 days

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Erythema toxicum spares

Palms and soles

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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)