Pediatrics Must-Knows Flashcards
Adenohypophysis arises from
Neurohypophysis arises from
Rathke’s pouch; neural ectoderm or nervous tissue
Most common type of tumor areas in the Rathke’s pouch in children/adult
Craniopharyngoma; prolactinoma
Other name of ADH
Vasopressin or Arginine VP (AVP)
Definitive diagnosis of hypopituitarism
Absent or low levels of GH
Vasopressin deficiency?
Central DI
Vasopressin insensitive kidneys?
Nephrogenic DI
Drug that causes Nephrogenic DI
Lithium
Diabetes Insipidus is manifested by polyuria (__cc/kg/hr) and polydipsia, _______mOsm, spgr _______
5cc/kg/hr; 50-200 mOsm; spgr 1.001-1.005
Electrolyte imbalance for DI
Hypernatremia and Hyperchloremia
Etiology of DI for 50% of cases
Other causes:
Brain tumor Idiopathic Head trauma CNs infection Histiocytosis
Precocious puberty is defined as premature appearance of secondary sexual characteristics
Breast bud before __y/o in females
Testicular enlargement ___y/o in males
8; 9
Most common cause of gonadotropin dependent precocious puberty?
Hypothalamic hamartomas
Triad of McCune Albright syndrome
Precocious puberty, polyostotic fibrous dysplasia, unilateral café-au-lait spots
Treatment for precocious puberty
Leuprolide acetate
Delayed puberty is the absence of any sign of puberty.
Boys?
Girls?
14 y/o
13 y/o
Structures that arise from mullerian duct
FT, prostatic utricle, uterus, cervix, vagina
Homologues of prostatic utricle
Uterus, cervix, vagina
Structures that arise from Wolffian duct
Rete testis, rete ovarii, epididymis, gartner’s duct, bas deferens, seminal vesicle
Congenital absence of uterus and vagina
Rokitansky-Meyer-Kuster-Hauser syndrome
Deficiency in CAH
21-hydroxylase
Deficiency in CAH tested in the Philippines
17-hydroxylase
Most common manifestation of CAH
Ambiguous genitalia
Treatment of CAH
Cortisol
CAH pathogenesis
NB at risk for neonatal adrenal crisis due to sodium loss due to absence of aldosterone
Most common cause of genital ambiguity
CAH
Most common cause of congenital hypothyroidism
Thyroid dysgenesis (85%)
Most common location of ectopic thyroid tissue
Lingual pr base of the tongue
Pathogenesis of Congenital Hypothyroidism
Inborn of errors of thyroxine synthesis and maternal transfer of TRBAb
Manifestations of Congenital Hypothyroidism
Prolonged jaundice, large tongue (due to ectopic thyroid tissue, MR, umbilical hernia, sluggisu
Syndrome that is characterized with intractable neonatal hypoglycemia, macrosomia, midline abdominal wall defects (omphalocele, umbilical hernia, diastasis recti), facial nevus flammeus, a characteristic earlobe creases (5 common features)
Beckwith-Wiedemann Syndrome
3 embryonal tumors in BWS
Wilms tumor, hepatoblastoma, rhabdomyosarcoma
Most common cause of thyroid disease in children
Thyroiditis
Most common cause of acquired hypothyroidism
Thyroiditis
Other names of thyroiditis
Lymphocytic thyroiditis, hashimoto’s thyroiditis, autoimmune thyroiditis
Thyroiditis with diffuse fibrosis that makes thyroid hard and woody apperance
Reidel’s thyroiditis
Other names of subacute thyroiditis
Viral thyroiditis, De Quervain thyroiditis
Catecholamine-secreting tumor of the chromaffin cells
Pheochromocytoma
Metabolite of pheochromocytoma
Urine VMA
DOC of pheochromocytoma
Phenoxybenzamine
Adrenal medulla tumors in children will be most likely be
Neuroblastoma
Locations of neuroblastoma
Abdomen, either from adrenals or retroperitoneal ganglia
Histopathological feature of neuroblastoma
Homer-Wright pseudorosettes
Retinoblastoma features
Cat’s eye
Leukocoria
Flexner-Wintersteiner bodies/rossettes
Components of Triple screen test
AFP, beta HCG, estriol
Other names of Triple screen test
Kettering or Bart’s test
Basic defect of PKU
Deficiency of phenylalanine hydroxylase or its cofactor tetrahydrobiopterin (BH4)
Cofactor that required to convert phenylalanine to tyrosine
Tetrahydrobiopterin
Physical examination findings of PKU
Seborrheic or eczematoid rash, mousy or musty odor
Histopathologic findings of G6PD deficiency
Heinz bodies, reticulocytosis
Enzyme that is responsible for decarboxylation of leucine, isoleucine and valine
Alpha ketoacid dehydrogenase
Inborn error of metabolism that has deficiency in the branched chain alpha ketoacid dehydrogenase
MSUD
Purely ketogenic amino acid
Leucine and isoleucine
Odor of MSUD
Caramel odor
Physical examination findings of MSUD
Hypertonicity, muscular rigidity and opisthotonus
Syndrome deficient of 7-dehydrocholesterol reductase deficiency characterized by pre- and postnatal growth retardation, MR, facial dysmorphic features, hypospadia, syndactyly between toes 2
Smith-Lemli-Opitz syndrome
Deficiency of hexosaminidase A deficiency, GM2 gangliosidosis characterized with cherry red spot with optic atrophy
Tay-Sachs Disease
Deficiency of both hexosaminidase A and B characterized by moderate hepatosplenomegaly and coarse granulations in BM histiocytes
Sandhoff disease
Deficiency in glucocerebrosidase characterized by hypotonia, crumpled tissue appearance
Infantile Gautcher disease
Disease with characteristic histiocytes (Gaucher cells) in marrow smears and liver and spleen biopsies
Infantile Gaucher Disease
Deficiency in Sphingomyelinase characterized by marked enlargement of liver with histopathologic finding of vacuolated histiocytes in the BM
Infantile Niemann-Pick Disease
Deficiency of ceramidase characterized by hoarse cry due to fixation of laryngeal cartilage with histopathologic finding of periarticular and subcutaneous swellings and progressive arthropathy
Faber disease or Lipogranulomatosis
Peroxisomal disorder in abnormal very long chain fatty acid metabolism manifests at birth, IUGR, developmental delay, lim contractuee, hepatomegalu, floppy baby, abnormal facies
Cerebrohepatorenal (Zellweger) disease
Enzyme deficient in Zellweger disease
Pipecolote oxidase
Deficiencies of phytanoyl-CoA hydroxyladse (chromosome 10) or peroxin-7 (chromosome 6) characterized by neurologic damage, cerebellar degeneration, and peripheral neuropathy
Think of Referee from fighting
Refsum disease
Autosomal recessive disease characterized by intermittent red, scaly rash over the face, neck, hands and legs resembling that of pellagra (niacin deficiency) and cerebellar ataxia
Hartnup disease
X-linked recessive disorder deficient in Cu-dependent enzymes, characterized by pili torti
Menkes disease (Kinky- or Steeply hair disease; trichopoliodystrophy)
Hepatolenticular degeneration due to decreased levels of ceruloplasmin
Wilson’s disease
3 organs that accumulate copper in Wilson’s disease
Cornea (Kayser-Fleishner rings)
Liver (cirrhosis)
Brain (in basal ganglia)
ABCD of Wilson’s disease
Asterixis
Basal ganglia ceruloplasmin
Ceruloplasmin decreased, cirrhosis, corneal deposits), Cu accumulation, carcinoma, chorea
Dementia
Most common cause of Charcot-Marie tooth in 70-80% of cases
Duplication of a large region on the short arm of chromosome 16 that includes PMP22 (Peripheral Myelin)
Manifestations of Charcot-Marie tooth disease
Peroneal muscular atrophy Foot drop High arched foot Stork legs Hammer toes
Vasculitis of small vessels with IgA and C3 deposition in small vessels of the skin and glomeruli typically URTI
HSP or Anaphylactoid purpura
Hallmark finding of HSP
Palpable purpura
Biopsy result of HSP
Leukoclastic angiitis
Most common vasculitis in children
HSP
Cardiac complication of Kawasaki disease
Coronary artery aneurysm
Other name of Kawasaki disease
Febrile mucocutaneous lymphadenopathy
Pulseless disease, vasculitis of large vessels, chronic, inflammatory infiltrate, preference for aorta and its branches, pulseless and claudication
Takayasu arteritis
Necrotizing vasculitis of small and medium vessels, bx: fibrinoid necrosis with thrombosis and infarction, ssx of HPN, hematuria, purpura, CVA, TIA, myocarditis, bone pain and blindness
Panarteritis or polyarteritis nodosa
Classic presentation of TEF
Choking, coughing, cyanosis occurs with an attempt at feeding
VACTERL in TEF
Vertebral, anal anomalies, cardiac, tracheo-esophageal fistula, renal anomalies, limb anomalies
2 other treatment aside from surgery for Achalasia
Nifedipine
Botulinum toxin
Surgical procedure for the treatment of achalasia
Heller myotomy
Radiologic finding of Hypertrophic pyloric stenosis
Shoulder sign, double tract sign, string sign
Surgical procedure for Hypertrophic pyloric stenosis
Ramstedt pyloromyotomy
Triad of volvulus
Severe epigastic pain
Intractable retching with emesis
Inability to pass a tube into the stomach
Duodenal atresia is associated with:
Down syndrome
Most common cause of intestinal obstruction between 3 months and 6 years of age
Intussuseption
2 types of intussuseption
Ileocolic or ileocecal
Ileoileocolic
Most common type of intussuseption
Ileocolic or ileocecal
Investigation of the RLQ for retraction which can be an indication of intussuseption
Dance’s sign
Radiologic finding of intussuseption
Coiled spring sign
Condition with prolonged elevated levels of conjugated bilirubin after 14th DOL
Neonatal cholestasis
Surgical procedure for biliary atresia
Kasai or hepatoportoenterostomy performed before 8 weeks
Emergency medical condition primarily seen in premature infants where portions of the bowel undergo necrosis
Necrotizing enterocolitis (NEC)
Radiologic finding of NEC
Pneumatosis intestinalis
Most common sites of NEC
Distal ileum and proximal colon
Physiologic jaundice presents ________
Resolves in ____week (term), ____weeks (preterm)
After 48th hour of life
1 week
2 weeks
Pathologic jaundice presents ________
Persists beyond in ____week (term), ____weeks (preterm)
1st 24 hours of life
1 week
2 weeks
Breastfeeding jaundice occurs at ____DOL
Accentuated ___________________
Factors: ______________
Duration: ______
3-4 DOL
Unconjugated hyperbilirubinemia
Decreased milk intake with dehydration
Few days
Breast milk jaundice occurs at \_\_\_\_DOL Accentuated \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ As high as \_\_\_\_mg/dL during the 2nd-3rd week Factors: \_\_\_\_\_\_\_\_\_\_\_\_\_\_ Duration: \_\_\_\_\_\_
After 7th DOL
Increased B1 in 2% of breastfed term infants
10-30
Presence of glucuronidase in some breast milk
3 weeks to 3 months
Level of bilirubin for icteric sclerae
2-3 mg/dL
Level of bilirubin requiring phototheraphy
15 mg/dL
Level of bilirubin in kernicterus or bilirubin encephalopathy
20mg/dL
Early signs of kernicterus (3-4days)
Lethargy
Poor feeding
High pitched cry
Hypotonia
Late signs of kernicterus (>1week)
Irritability Seizure Apnea Hypertonia Fever
Mechanisms of phototherapy
Geometric photoisomerization of conjugated bilirubin
Structural isomerization: lumirubin
Photooxidation
Chronic signs of kernicterus (3 years of age)
Athetpid cerebral palsy High frequency hearing loss Paralysis of upward gaze Dental dysplasia Mild MR
Pharmacologic therapy for kernicterus
Phenobarbital: induction of glucoronyl transferase activity, enhances hepatic uptake
Metalloporphyrins: inhibits heme oxygenase
If phototherapy and pharmacologic treatment does not work
Exchange transfusion
Most common congenital anomaly of the respiratory tract
Choanal atersia
Most common type of chonal atresia
Bony (90%)
Membranous (10%)
Most common manifestation of choanal atresia
Others
- Nasal obstruction (if unilateral, asymptomatic)
- Cyanosis improved with crying if bilareral nasal obstruction
- Inability to insert NGT
CHARGE syndrome is _________
Coloboma
Heart disease
Atresia choanae
Retarded growth and development or CNS anomalies
Genital anomalies or hypogonadism or both
Ear anomalies or deadness or both
Most common congenital laryngeal anomaly
Laryngomalacia
Most frequent cause of strudor in infants and children
Larnyngomalacia
2nd most common cause of stridor
Congenital subglottic stenosis
Congenital anomaly of the larynx characterized by biohadic stridor, recurrent or persistent croup
Congenital subglottic stridor
Most frequent cause of airway obstruction requiring trachepstomy in infants
Larngotracheal stenosis
Acquired laryngotracheal stenosis is related to ___________
ET intubation
The most common respiratory problem of a premature infant
Hyaline membrane disease
DOC that decreasea the incidence and severity of HMD
Corticosteroid
When the transient phenomenon of TTN lasts?
6-24 hours
Radiologic finding of TTN
Hyperaeration, prominent vascular markings (sunburst pattern), prominent minor fissure
AVP acts at what part of the kidney
Distal tubules
Most common etiology of rhinosinusitis
Rhinovirus
Most common complication of rhinosinusitis
Otitis media
Enzyme for conversion of testosterone to DHT
Alpha reductase
Peak incidence of acute pharyngitis
4-7 years old
Most common cause of acute pharyngitis
Viral
Bacterial causes of acute pharyngitis
Group A beta hemolytic strep (strep pyogenes)
Mycoplasma
Arcanobacterium haemolyticum
Reapiratory condition that presenta as sore theoat and fever worse at nigr, seal-like cough, copl night air usually helps
Croup or laryngotracheobronchitis
Condition that involves prtial teatis determination in the presence of a Y chromosome
Incomplete gonadal dysgenesis
Etiologic agent of croup
Parainfluenza
Radiologic sign of croup
Steeple sign or subglottic narrowing
Condition seen as dysmorphic syndrome that has defect in testosterone biosynthesis
Male pseudohermaphroditism
Radiologic finding of epiglotitis
Thumb print sign
What is Wolfram syndrome?
DI, DM, optic atropht, deafness
Most common cause of bacterial tracheitis
S. Aureus
Etiologic agent for pneumatocele pneumonia
S. Aureus
Common disease of the LRT of infants; etiologic agent RSV
Bronchiolitis
Most common hematologic disease of infancy in childhood
IDA
When should exclusively breastfed infants must be supplemented with Iron?
4 months
What diagnostic test should be measured for idiopathic or unexplained DI in addition to serial MRI scan?
Beta HCG
Test for Vitamin B12 absorption
Schilling test
What vitamin has excessive methymalonic acid in the urine?
Vitamin B12/cobalamin
Most common familial and congenital abnormality of the red cell membrane
Hereditary spherocytosis
Hematologic disease that has defect on the cytoskeleton protein, specifically ankyrin and spectrin
Hereditary spherocytosis
Test for hereditary spherocytosis
Osmotic fragility test
Blood coagulation derangement in Factor VIII
Increased PTT; normal BT, PT
Painful bump below the knee is the manifestation of what condition
Osgood Schlatter
Blood coagulation derangement in Factor IX
Increased PTT; normal BT, PT
Cause of idiopathic central DI in 50% of cases
Hypophysitis
Most common hereditary bleeding disordee
VWF disease
Blood coagulation derangement in VWF disease
Prolonged BT and PTT
Hematologic condition with deficiency in Vitamin K, 1972, more common in breastfed infants due to lack of free K, absence of bacteria responsible for Vitamin K synthesis in the intestines
Hemorrhagic disease in the newborn
Drugs associated with the inhibition of vasopressin release
Halothane Opiate antagonist Phenytoin Ethanol Alpha-adrenergic agent
Mainstay treatment for DI
Fluid therapy
Total fluid intake is limited to how much
1L/m2/24 hours
Most common childhood CA
Leukemia
Most common leukemia as seen also in Down Syndrome, ataxia-telangiectasia, fanconinsyndrome, NF type
ALL (77%)
Peak incidence of ALL
4 years old
Environmental factors associated with ALL
Ionizing radiation
Drugs
Alkylating agents
Benzene
Medical treatment for ALL
Vincristine, dexamethasone or prednisone, L-asparaginase
Drugs associated with acquired nephrogenic diabetes insipidus
Lithium Demeclocycline Foscarnet Clozapine Amphotericin Methicillin Rifampin
Remission of ALL is defined as
<5% blast in marrow and return of neutrophil and platelet count to near normal
Leukemia predominant neonatal/congenital leukemia
AML
Diagnostic finding for AML
Myeloperoxidase and Auer rods
Higher incidence of conditions in AML
Down syndrome, fanconi anemia, diamond-blackfan anemia
Leukemia uncommon in children with only 3% under the age of 20; more common in 55-65years
CML
Pathogenesis of CML
t (9,22) philadelphia chromosome
Pathogenesis of Burkitt lymphoma
t (8,14)
Most common congenital anomaly of the respiratory tract
Choanal atersia
Most common type of chonal atresia
Bony (90%)
Membranous (10%)
Most common manifestation of choanal atresia
Others
- Nasal obstruction (if unilateral, asymptomatic)
- Cyanosis improved with crying if bilareral nasal obstruction
- Inability to insert NGT
CHARGE syndrome is _________
Coloboma
Heart disease
Atresia choanae
Retarded growth and development or CNS anomalies
Genital anomalies or hypogonadism or both
Ear anomalies or deadness or both
Most common congenital laryngeal anomaly
Laryngomalacia
Most frequent cause of strudor in infants and children
Larnyngomalacia
2nd most common cause of stridor
Congenital subglottic stenosis
Congenital anomaly of the larynx characterized by biohadic stridor, recurrent or persistent croup
Congenital subglottic stridor
Most frequent cause of airway obstruction requiring trachepstomy in infants
Larngotracheal stenosis
Acquired laryngotracheal stenosis is related to ___________
ET intubation
The most common respiratory problem of a premature infant
Hyaline membrane disease
DOC that decreasea the incidence and severity of HMD
Corticosteroid
When the transient phenomenon of TTN lasts?
6-24 hours
Radiologic finding of TTN
Hyperaeration, prominent vascular markings (sunburst pattern), prominent minor fissure
AVP acts at what part of the kidney
Distal tubules
Most common etiology of rhinosinusitis
Rhinovirus
Most common complication of rhinosinusitis
Otitis media
Enzyme for conversion of testosterone to DHT
Alpha reductase
Peak incidence of acute pharyngitis
4-7 years old
Most common cause of acute pharyngitis
Viral
Bacterial causes of acute pharyngitis
Group A beta hemolytic strep (strep pyogenes)
Mycoplasma
Arcanobacterium haemolyticum
Reapiratory condition that presenta as sore theoat and fever worse at nigr, seal-like cough, copl night air usually helps
Croup or laryngotracheobronchitis
Condition that involves prtial teatis determination in the presence of a Y chromosome
Incomplete gonadal dysgenesis
Etiologic agent of croup
Parainfluenza
Radiologic sign of croup
Steeple sign or subglottic narrowing
Condition seen as dysmorphic syndrome that has defect in testosterone biosynthesis
Male pseudohermaphroditism
Radiologic finding of epiglotitis
Thumb print sign
What is Wolfram syndrome?
DI, DM, optic atropht, deafness
Most common cause of bacterial tracheitis
S. Aureus
Etiologic agent for pneumatocele pneumonia
S. Aureus
Common disease of the LRT of infants; etiologic agent RSV
Bronchiolitis
Most common hematologic disease of infancy in childhood
IDA
When should exclusively breastfed infants must be supplemented with Iron?
4 months
What diagnostic test should be measured for idiopathic or unexplained DI in addition to serial MRI scan?
Beta HCG
Test for Vitamin B12 absorption
Schilling test
What vitamin has excessive methymalonic acid in the urine?
Vitamin B12/cobalamin
Most common familial and congenital abnormality of the red cell membrane
Hereditary spherocytosis
Hematologic disease that has defect on the cytoskeleton protein, specifically ankyrin and spectrin
Hereditary spherocytosis
Test for hereditary spherocytosis
Osmotic fragility test
Blood coagulation derangement in Factor VIII
Increased PTT; normal BT, PT
Painful bump below the knee is the manifestation of what condition
Osgood Schlatter
Blood coagulation derangement in Factor IX
Increased PTT; normal BT, PT
Cause of idiopathic central DI in 50% of cases
Hypophysitis
Most common hereditary bleeding disordee
VWF disease
Blood coagulation derangement in VWF disease
Prolonged BT and PTT
Hematologic condition with deficiency in Vitamin K, 1972, more common in breastfed infants due to lack of free K, absence of bacteria responsible for Vitamin K synthesis in the intestines
Hemorrhagic disease in the newborn
Drugs associated with the inhibition of vasopressin release
Halothane Opiate antagonist Phenytoin Ethanol Alpha-adrenergic agent
Mainstay treatment for DI
Fluid therapy
Total fluid intake is limited to how much
1L/m2/24 hours
Most common childhood CA
Leukemia
Most common leukemia as seen also in Down Syndrome, ataxia-telangiectasia, fanconinsyndrome, NF type
ALL (77%)
Peak incidence of ALL
4 years old
Environmental factors associated with ALL
Ionizing radiation
Drugs
Alkylating agents
Benzene
Medical treatment for ALL
Vincristine, dexamethasone or prednisone, L-asparaginase
Drugs associated with acquired nephrogenic diabetes insipidus
Lithium Demeclocycline Foscarnet Clozapine Amphotericin Methicillin Rifampin
Remission of ALL is defined as
<5% blast in marrow and return of neutrophil and platelet count to near normal
Leukemia predominant neonatal/congenital leukemia
AML
Diagnostic finding for AML
Myeloperoxidase and Auer rods
Higher incidence of conditions in AML
Down syndrome, fanconi anemia, diamond-blackfan anemia
Leukemia uncommon in children with only 3% under the age of 20; more common in 55-65years
CML
Pathogenesis of CML
t (9,22) philadelphia chromosome
Pathogenesis of Burkitt lymphoma
t (8,14)
Most common congenital anomaly of the respiratory tract
Choanal atersia
Most common type of chonal atresia
Bony (90%)
Membranous (10%)
Most common manifestation of choanal atresia
Others
- Nasal obstruction (if unilateral, asymptomatic)
- Cyanosis improved with crying if bilareral nasal obstruction
- Inability to insert NGT
CHARGE syndrome is _________
Coloboma
Heart disease
Atresia choanae
Retarded growth and development or CNS anomalies
Genital anomalies or hypogonadism or both
Ear anomalies or deadness or both
Most common congenital laryngeal anomaly
Laryngomalacia
Most frequent cause of strudor in infants and children
Larnyngomalacia
2nd most common cause of stridor
Congenital subglottic stenosis
Congenital anomaly of the larynx characterized by biohadic stridor, recurrent or persistent croup
Congenital subglottic stridor
Most frequent cause of airway obstruction requiring trachepstomy in infants
Larngotracheal stenosis
Acquired laryngotracheal stenosis is related to ___________
ET intubation
The most common respiratory problem of a premature infant
Hyaline membrane disease
DOC that decreasea the incidence and severity of HMD
Corticosteroid
When the transient phenomenon of TTN lasts?
6-24 hours
Radiologic finding of TTN
Hyperaeration, prominent vascular markings (sunburst pattern), prominent minor fissure
AVP acts at what part of the kidney
Distal tubules
Most common etiology of rhinosinusitis
Rhinovirus
Most common complication of rhinosinusitis
Otitis media
Enzyme for conversion of testosterone to DHT
Alpha reductase
Peak incidence of acute pharyngitis
4-7 years old
Most common cause of acute pharyngitis
Viral
Bacterial causes of acute pharyngitis
Group A beta hemolytic strep (strep pyogenes)
Mycoplasma
Arcanobacterium haemolyticum
Reapiratory condition that presenta as sore theoat and fever worse at nigr, seal-like cough, copl night air usually helps
Croup or laryngotracheobronchitis
Condition that involves prtial teatis determination in the presence of a Y chromosome
Incomplete gonadal dysgenesis
Etiologic agent of croup
Parainfluenza
Radiologic sign of croup
Steeple sign or subglottic narrowing
Condition seen as dysmorphic syndrome that has defect in testosterone biosynthesis
Male pseudohermaphroditism
Radiologic finding of epiglotitis
Thumb print sign
What is Wolfram syndrome?
DI, DM, optic atropht, deafness
Most common cause of bacterial tracheitis
S. Aureus
Etiologic agent for pneumatocele pneumonia
S. Aureus
Common disease of the LRT of infants; etiologic agent RSV
Bronchiolitis
Most common hematologic disease of infancy in childhood
IDA
When should exclusively breastfed infants must be supplemented with Iron?
4 months
What diagnostic test should be measured for idiopathic or unexplained DI in addition to serial MRI scan?
Beta HCG
Test for Vitamin B12 absorption
Schilling test
What vitamin has excessive methymalonic acid in the urine?
Vitamin B12/cobalamin
Most common familial and congenital abnormality of the red cell membrane
Hereditary spherocytosis
Hematologic disease that has defect on the cytoskeleton protein, specifically ankyrin and spectrin
Hereditary spherocytosis
Test for hereditary spherocytosis
Osmotic fragility test
Blood coagulation derangement in Factor VIII
Increased PTT; normal BT, PT
Painful bump below the knee is the manifestation of what condition
Osgood Schlatter
Blood coagulation derangement in Factor IX
Increased PTT; normal BT, PT
Cause of idiopathic central DI in 50% of cases
Hypophysitis
Most common hereditary bleeding disordee
VWF disease
Blood coagulation derangement in VWF disease
Prolonged BT and PTT
Hematologic condition with deficiency in Vitamin K, 1972, more common in breastfed infants due to lack of free K, absence of bacteria responsible for Vitamin K synthesis in the intestines
Hemorrhagic disease in the newborn
Drugs associated with the inhibition of vasopressin release
Halothane Opiate antagonist Phenytoin Ethanol Alpha-adrenergic agent
Mainstay treatment for DI
Fluid therapy
Total fluid intake is limited to how much
1L/m2/24 hours
Most common childhood CA
Leukemia
Most common leukemia as seen also in Down Syndrome, ataxia-telangiectasia, fanconinsyndrome, NF type
ALL (77%)
Peak incidence of ALL
4 years old
Environmental factors associated with ALL
Ionizing radiation
Drugs
Alkylating agents
Benzene
Medical treatment for ALL
Vincristine, dexamethasone or prednisone, L-asparaginase
Drugs associated with acquired nephrogenic diabetes insipidus
Lithium Demeclocycline Foscarnet Clozapine Amphotericin Methicillin Rifampin
Remission of ALL is defined as
<5% blast in marrow and return of neutrophil and platelet count to near normal
Leukemia predominant neonatal/congenital leukemia
AML
Diagnostic finding for AML
Myeloperoxidase and Auer rods
Higher incidence of conditions in AML
Down syndrome, fanconi anemia, diamond-blackfan anemia
Leukemia uncommon in children with only 3% under the age of 20; more common in 55-65years
CML
Pathogenesis of CML
t (9,22) philadelphia chromosome
Pathogenesis of Burkitt lymphoma
t (8,14)
