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115 Cards in this Set
- Front
- Back
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Pyloric stenosis most common in and associated with
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1st born males
Tracheoesophageal fistula and maternal history of pyloric stenosis |
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Nonbilious emesis around 3 weeks which progresses to prjectile emesis after most/all feedings
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Pyloric stenosis
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Abdominal ultrasound findings in pyloric stenosis
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Hypertophic pylorus
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Barium swallow results in pyloric stenosis
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String sign or pyloric beak
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Meckel's Diverticulum
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Failure of the omphalomesenteric (vitelline) duct to obliterate
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Most common congenital abnormality of small intestine, affects 2%
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Meckel's Diverticulum
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Patient under 2 presents with sudden, painless rectal bleeding
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Meckel's Diverticulum
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Diagnosis of Meckel's diverticulum
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Meckel scintigraphy scan (technetium-99m pertechnetate)
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Hirschsprung's disease
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Congenital lack of ganglion cells in the distal colon leading to uncoordinated peristalsis and decreased motility.
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Hirschsprung's disease associated with
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Males
Down's Waardenburg's syndrome Multiple endocrine neoplasia (MEN) type 2 |
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Neonate presents with failure to mass meconuim within 48 hours of birth, bilious vomiting and failure to thrive
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Hirschsprung's disease
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Diagnosis of Hirschsprung's disease
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Barium enema: narrowed distal colon with proximal dilation
Full-thickness rectal biopsy: absence of myenteric (Auerbach's) plexus and submucosal (Meissner's) plexus, hypertrophied nerve trunks |
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Newborn presents with bilious emesis, crampy abdominal pain, distention, and blood or mucous in stool
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Malrotation with volvulus
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Diagnosis of volvulus
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Upper GI: abnormal location of ligament of Treitz.
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Treatment of volvulus
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NG tube, IV fluids, surgical repair
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Most common GI emergency in neonates
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Necrotizing Enterocolitis (NEC)
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Premature infant with feeding intolerance, delayed gastric emptying, abdominal distention and bloody stools.
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Necrotizing enterocolitis (NEC)
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Diagnosis of necrotizing enterocolitis
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Plain abdominal film: pneumatosis intestinalis
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Indications for surgery in necrotizing enterocolitis
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1) Perforation
2) Radiographic worsening on serial plain films |
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Complications of necrotizing enterocolitis
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Intestinal strictures
Short-bowel syndrome |
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Treatment of necrotizing enterocolitis
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NPO
Gastric decompression with orogastric tube Correct electrolyte abnormalities TPN IV antibiotics |
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B-cell deficiency in boys only
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Bruton's agammaglobulinemia
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Infections seen in Bruton's agammaglobulinemia
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Pseudomonas
Strep pneumoniae Haemophilus Seen after 6 months when passive immunity wanes |
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Absent B cells
High T cells Absent tonsils or other lympoid tissue |
Bruton's agammaglobulinemia
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Treatment for Bruton's agammaglobulinemia
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Prophylactic antibiotics
IVIG |
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Combined B and T cell defect in 20s and 30s
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Common variable immunodeficiency (CVID)
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Risks associated with CVID
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1) Pyogenic upper and lower respiratory infections
2) Lymphoma 3) Autoimmune disease |
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Treatment for CVID
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IVIG
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Most common immunodeficiency
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IgA deficiency
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Presents with anaphylactic transfusion reaction
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IgA deficiency
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For this immunodeficiency, DO NOT give immunoglobulins
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IgA deficiency. Can lead to production of anti-IgA antibodies
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Presents with tetany, secondary to hypocalcemia, in first days of life
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DiGeorge Syndome (thymic aplasia)
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Infections associated with DiGeorge Syndrome
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Fungi
Pneumocystis jiroveci pneumonia |
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DiGeorge Syndrome
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Thymic aplasia
T cell disorder |
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Treatment for DiGeorge Syndrome
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Bone marrow transplant
IVIG PCP prophylaxis Thymus transplantation (alternative) |
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Presents with oculocutaneous telangiectasias and progressive cerebellar ataxia
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Ataxia-telangiectasia
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Cancers associated with ataxia-telangiectasia
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1) Non-Hodgkin's lymphoma
2) Leukemia 3) Gastric carcinoma |
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SCID
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Severe lack of B and T cells leading to frequent infections
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Infectious associated with SCID
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1) Bacterial
2) Chronic candidiasis 3) Opportunistic organisms |
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Treatment for SCID
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Bone marrow transplant
Stem cell transplant IVIG PCP prophylaxis |
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Patient presents with eczema, bleeding, and otitis media
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Wiskott-Aldrich syndrome
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Immunoglobulin profile in Wiskott-Aldrich syndrome
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High IgE
High IgA Low IgM |
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Risks associated with Wiskott-Aldrich syndrome
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1) Atopic disorders
2) Lymphoma/Leukemia 3) S. pneumoniae 4) S. aureus 5) H. flu type B |
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Treatment and prognosis in Wiskott-Aldrich syndrome
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IVIG
Antibiotics Bone marrow transplant Rarely survive into adulthood |
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Chronic Granulomatous Disease
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Deficient superoxide production by PMNs and macrophages
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Patient presents with anemia, lymphadenopathy and hypergammaglobulinemia
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Chronic granulomatous disease
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Risks associated with chronic granulomatous diseases
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1) Chronic infections of skin, pulmonary, GI, and urinary tract.
2) Osteomyelitis and hepatitis 3) Catalase + organisms 4) Aspergillus |
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Diagnosis of chronic granulomatous disease
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Nitroblue tetrazolium test
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Treatment for chronic granulomatous disease
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Daily TMP-SMX
Judicious use of antibiotics during infection IFN-gamma Bone marrow transplant Gene therapy |
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Leukocyte adhesion deficiency
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Defect in chemotaxis of leukocytes
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Newborn presents with omphalitis with delayed separation of umbilical cord
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Leukocyte adhesion deficiency
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Diagnosis of leukocyte adhesion deficiency
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No pus with minimal inflammation in wounds
High WBCs in blood |
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Treatment for leukocyte adhesion deficiency
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Bone marrow transplant
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Chediak-Higashi syndrome
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Defect in neutrophil chemotaxis/microtubule polymerization
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Oculocutaneous albinism
Neuropathy Neutropenia |
Chediak-Higashi syndrome
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Increased risk associated with Chediak-Higashi syndrome
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1) Strep pyogenes
2) S. aureus 3) Pseudomonas |
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Diagnostic findings in Chediak-Higashi syndrome
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Giant granules in neutrophils
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Treatment for Chediak-Higashi syndrome
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Bone marrow transplant
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Presents with recurrent episodes of angioedema lasting 2-72 hours provoked by stress or trauma. Can lead to life-threatening airway edema
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C1 esterase deficiency (hereditary angioedema)
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Diagnosis of C1 esterase deficiency (hereditary angioedema)
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Total hemolytic complement (CH50) to assess the quantity and function of complement
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A patient with C1 esterase deficiency (hereditary angioedema) should receive this prior to surgery
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1) Purified C1 esterase
2) FFP |
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Terminal complement deficiency (C5-C9)
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Inability to form membrane attack complexes (MAC)
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Infections associated with terminal complement deficiency
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1) Meningococcal
2) Gonococcal 3) Rarely lupus or glomerulonephritis |
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Multisystemic acute vasculitis affecting children <5, mostly Asians
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Kawasaki Disease
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Children untreated for Kawasaki Disease are at risk for
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Aneurysmal expansion
MI |
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Treatment for Kawasaki disease
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High dose ASA
IVIG |
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A patient <16 presents with morning stiffness and gradual loss of motion for at least 6 weeks
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Juvenile Idiopathic Arthritis
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Juvenile Arthritis where
1) 4 or less joints involved 2) ANA + 3) RF - 4) Uveitis 5) No systemic symptoms |
Pauciarticular (oligoarthritis)
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Juvenile Idiopathic Arthritis where
1) Five or more joints involved 2) Symmetric 3) Rarely RF + 4) ANA + 5) Rarely systemic symptoms |
Polyarthritis
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Juvenile Idiopathic Arthritis where
1) Recurrent high fever 2) Hepatosplenomegaly 3) Salmon-colored macular rash 4) RF - 5) ANA - |
Still's Disease (systemic onset)
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Treatment for juvenile idiopathic arthritis
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NSAIDs
Strengthening exercises Corticosteroids Immunosuppressive medications TNF (etanercept) |
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Most common viral causes of otitis media
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1) Influenza A
2) RSV 3) Parainfluenza |
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Most common bacterial causes of otitis media
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1) S. pneumoniae
2) Nontypable H. flu 3) Moraxella catarrhalis |
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Treatment of acute otitis media
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High dose amoxicillin (80-90mg) x 10 days
Augmentin (amoxicillin + clavulanic acid) for resistant cases) |
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Most common cause of bronchiolitis
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1) RSV
Others: Parainfluenza Influenza Metapneumovirus |
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Infant < 2 presents with low-grade fever, rhinorrhea, cough and apnea. PE reveals tachypnea, wheezing, intercostal retractions, crackles, prolonged expiration, expiratory wheezing and hyperresonance to precussion
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Bronchiolitis
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Treatment for bronchiolitis
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Supportive
Hospitalize when in respiratory distress (02 sat < 92%) |
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Indication for RSV prophylaxis with RespiGam or Synagis
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High risk patients < 2
Hx of prematurity Chronic lung disease Congenital heart disease |
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Most common causes of croup
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1) Parainfluenza type I
2) Parainfluenza 2 and 3 3) RSV 4) Influenza 5) Adenovirus |
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Steeple sign on AP neck
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Croup
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Treatment for moderate croup
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Supplemental O2
Oral and IM corticosteroids Nebulized racemic epinephrine |
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Patient presents with acute-onset high fever, dysphagia, drooling, muffled voice, inspiratory retractions, cyanosis and soft stridor. Sitting in tripod or sniffing dog position
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Epiglottitis
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Most common causes of epiglottitis
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H. flu prior to immunization
Now it is strep and H. flu |
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Treatment of epiglottitis
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Secure airway, do not examine throat without anesthesiologist or otolaryngologist present
Thumbprint sign on x-ray |
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Most common cause of bronchiolitis
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1) RSV
Others: Parainfluenza Influenza Metapneumovirus |
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Infant < 2 presents with low-grade fever, rhinorrhea, cough and apnea. PE reveals tachypnea, wheezing, intercostal retractions, crackles, prolonged expiration, expiratory wheezing and hyperresonance to precussion
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Bronchiolitis
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Treatment for bronchiolitis
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Supportive
Hospitalize when in respiratory distress (02 sat < 92%) |
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Indication for RSV prophylaxis with RespiGam or Synagis
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High risk patients < 2
Hx of prematurity Chronic lung disease Congenital heart disease |
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Most common causes of croup
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1) Parainfluenza type I
2) Parainfluenza 2 and 3 3) RSV 4) Influenza 5) Adenovirus |
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Steeple sign on AP neck
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Croup
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Treatment for moderate croup
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Supplemental O2
Oral and IM corticosteroids Nebulized racemic epinephrine |
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Patient presents with acute-onset high fever, dysphagia, drooling, muffled voice, inspiratory retractions, cyanosis and soft stridor. Sitting in tripod or sniffing dog position
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Epiglottitis
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Most common causes of epiglottitis
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H. flu prior to immunization
Now it is strep and H. flu |
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Treatment of epiglottitis
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Secure airway, do not examine throat without anesthesiologist or otolaryngologist present
Thumbprint sign on x-ray Intubation, tracheostomy IV antibiotics ceftriaxone or cefuroxime |
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Most common cause of viral meningitis
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Enterovirus
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Most common causes of bacterial meningitis in children < 3
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S. pneumoniae
N. meningitidis E. Coli |
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Patient presents with headache, high fever, and nuchal rigidity
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Bacterial meningitis
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Kernig's Sign
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Pain on knee extension when hip is flexed
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Brudzinski's sign
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Pain with passive neck flexion
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Treatment of meningitis in neonates
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Ampicillin and cefotaxime or gentamicin
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Treatment of meningitis in older children
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Ceftriaxone and vancomycin
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APGAR
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0-2 points 5 minutes after birth
Appearance (blue, pale pink, pink) Pulse (0, <100, > 100) Grimace with stimulation (nothing, grimace, grimace and cough) Activity (limp, some, active) Respiratory effort (0, irregular, regular) |
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APGAR Scores
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8-10 - Good
4-7 - Possible need for resuscitation 0-3 - Immediate resuscitation |
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Neonatal jaundice
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Elevated serum bilirubin >5mg.dL) due to increased hemolysis or decreased excretion
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Type of neonatal jaundice that is always pathologic
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Conjugated (direct) hyperbilirubinemia
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Complication of unconjugated hyperbilirubinemia that results from irreversible bilirubin deposition in basal ganglia, pons, and cerebellum. Levels >25 - 30mg/dL
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Kernicterus
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Risk factors for Kernicterus
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1) Prematurity
2) Asphyxia 3) Sepsis |
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Patient presents with polyhydramnios in utero, increased oral secretions, inability to feed, gagging, aspiration, pneumonia, respiratory distress
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Tracheoesophageal fistula
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Diagnosis of tracheoesophageal fistula
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CXR showing NG tube coiled in the esophagus, presence of air in GI tract, confirm with bronchoscopy
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Tracheoesophageal fistula associated with
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VACTERL abnormalities
Vertebral Anal Cardiac Tracheal Esophageal Renal Limb |
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Presents with respiratory distress, sunken abdomen, bowel sounds over left hemithorax
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Congenital diaphragmatic hernia
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Treatment of congenital diaphragmatic hernia
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High-frequency ventilation or extracorporeal membrane oxygenation to manage pulmonary hypertension, surgical repair
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Presents with polyhydramnios in utero, often premature, associated with GI stenoses or atresia
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Gastroschisis
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Herniation of intestine only through the abdominal wall next to the umbilicus (usually on right) with no sac
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Gastroschisis
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Treatment of gastroschisis
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Surgical emergency! Single-stage closure is possible in only 10% of cases
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