Topic list Flashcards
(209 cards)
1
Q
Classification of pneumonia
A
Typical bacteria, atypical bacteria, viral
Based on inflitrate: lobar, bronchopulmonary, interstitial
Based on place of acquisition: community-acquired or hospital acquired.
2
Q
Symptoms of pneumonia
A
– fever, weakness, lethargy.
– cough, tachypnea, wheezing, dyspnea, cyanosis
– vomiting, diarrhea, abd. pain
– convulsions, apathy, restlessness
– tachycardia, cardiac decompensation
3
Q
Specific symptoms of pneumonia based on pathogens
A
Unproductive cough: viruses, M. pneumonia, C. pneumonia
Herpes labialis: S. pneumonia
Erythema multiforme: M. pneumonia
4
Q
Characteristics and differences in typical-, atypical-, and viral pneumonia
A
Atypical bact. > 5y, all seasons, mild fever, gradual, unproductive cough, dyspneavery rare.
Typical bact all ages, winter, sudden, high fever, dyspnea frequent, productive cough, creptiation at ascul., alveolar inf. on x-ray, common w. pleural effusion
Atypical viral: all ages, winter, sudden, high fever, unproductive cough, dyspnea frequent, interstital inf. on x-ray, not typical w. pleural effusion
5
Q
Age related pathogens
A
Newborn: GBS, staph. aureus
1-3 mo: RSV, chlam. trachomatis, S. pneumonia
3mo - 5y: RSV, S. pneumonia
>5y: M. pneumonia, Chl. pneumonia
6
Q
Diagnosis of pneumonia
A
– Lab: incr. WBC, decr. neutrophils, increased ESR and CRP.
– Imaging: CXR (empyema, pulm. abscess and PTX suggests bacterial origin) and US
– Specific testing: hemoculture (in suspected bacterial pneumonia), mycoplasma and chlam. testing (in suspected atypical bact.), Rapid antigen test (in suspected viral, e.g. RSV)
7
Q
Treatment of pneumonia
A
Mild lower RTI in young children and infants do not require ab treatment.
Bacterial pneumonia emperical treatment:
– Mild: < 5y = amoxicillin, >5y = macrlide
– Severe: 0-6mo = cefotaxime + ampicillin, > 6mo = II and III gen. cephalosporins (cefuroxime, cefotaxime + macrolide.
Prevention: vaccination
8
Q
What is cystic fibrosis?
A
Genetic autosomal recessive disorder, CFTR gene mutation. Chloride channel disorder leading to dysfunction of exocrine glands. It’s a multiorgan disease affecting reproductive tract, sinus, lungs, sweat gl., liver, pancreas and GI tract
9
Q
Most common symptoms of cystic fibrosis
A
Respiratory (90% of CF patients)
10
Q
Respiratory symptoms of CF
A
– Irreversible lung injury
– Cough, bronchitis, PTX, fibrosis, cor pulmunola, respiratory failure
11
Q
What happens with chloride in CF
A
Decreased uptake of Cl in sweat glands, increased uptake in mucous membranes.
(increased NaCl in sweat of babies, but thick sticky secretions in mucous membranes)
12
Q
Diagnosis of CF
A
Sweat test (high Nacl), Guthrie screening test, genetic testing
13
Q
Define obstructive bronchitis
A
Swelling or inflammation of the main airways (bronchi) of the lungs
14
Q
Etiology of obstructive bronchitis
A
– Common cold
– Acute asthma exacerbation
– Viral bronchiolitis
– Foreign body aspiration
– Acute bronchitis
– Croup
– Tracheomalacia
– CF
15
Q
Pathophysiology of asthma bronchiale
A
Respiratory hyperactivity –> Incr. cap. permeability and histamine release –> incr. gland secr. and proliferation –> decreased airflow due to airway narrowing and hyperinflated + collapsed alveoli –> insufficient ventilation
16
Q
Symptoms of asthma
A
Cough, wheezing in expiratory, dyspnea, chest tightness
17
Q
Treatment of asthma
A
Acute:
– SABA: salbutamol, terbutaline
– SAMA: ipratropium
Chronic:
– 1st line: Inhaled corticosteroids (budesonide, fluticasone)
– 2nd line: bronchodilation: LABA (formoterol, salmeterol)
18
Q
Define acute subglottic laryngitis
A
Pseudocroup. Inflammation of tissues in subglottic space +/- tracheobronchial tree.
19
Q
Pathogens of pseudocroup (acute subglottic laryngitis)
A
Parainfluenza virus, RSV
20
Q
Common symptoms of pseuodocroup
A
Commenly starts at night and resolves suddenly.
– Inspiratory stridor, hoarsness, barking cough.
21
Q
Treatment of pseudocroup
A
Mild – cold humidified air, fluid intake, rectal steroids
Moderate – hospital admission, epinephrine inhalation, systemic glucocorticoids
Severe – same as moderate + ICU admission
22
Q
What is the croup score?
A
Score based on inspiration, stridor, cough, retraction + nasal flaring and cyanosis.
mild = 1-2 p
moderate = 3-5 p
severe = 6-10 p
23
Q
Alarming signs of CNS diseases
A
– Acute encephalopathy
– Signs and symptoms on increased intracranial pressure
– Meningeal signs and symptoms
24
Q
Signs and symptoms of increased intracranial pressure
A
Cushing’s triad:
1. bradycardia
2. irregular respiration
3. increased blood pressure
25
Signs of increased intracranial pressure in infants
Bulging of the fontanelle, setting-sun eyes, irritability
26
Meningeal signs and symptoms
Nuchal rigidity, Brudzinski sign and Kernig sign
27
Define acute encephalopathy
Acute onset of mental changes +/- hypnoid disturbance of conciousness (bw. wake state/confusion/deep coma)
28
Etiology of encephalopathy
Trauma, neurology infection, vascular/hemotological disorders, ischemic lesions, tumor, intoxications, fluid electrolyte imbalance, acid-base disturbance, endocrine disorders, renal- and hepatic insufficiency, Reye disease, cong. metabolic disorder, chronic neurological diseases with sudden onset.
29
What is lumbar puncture used for?
Used to obtain sample of CSF for microbiological, biochemical or metabolic analysis.
30
Contraindications of lumbar puncture
Thrombocytopenia or coagulation defect
Raised ICP
Significant cardiorespiratory compromise
31
Where is the lumbar puncture done?
L3-L4 intervertebral space
32
Name some inflammatory diseases of the nervous system
Meningitis, encephalitis
33
Etiology of meningitis
Septic (bacterial)
Aseptic (non-bacterial)
Granulomatous
34
Laboratory characteristics of septic, aseptic and granulomatous meningitis
Septic: CSF > 1000, very high proteins, low glc
Aseptic: CSF < 1000, high proteins, norm glc
Granulomatous: CSF < 1000, very high proteins, low glc
35
Pathogens causing bacterial meningitis
< 3 mo - 10 y: GBS, S. pneumonia, N. meningitis,
In > 10y: mainly N. meningitis only
36
Define meningitis
Inflammation of the meningitis. Inflammation is in the subarachnoid space, causing swollen tissue and CSF changes.
37
Symptoms of meningitis based on age
Infants: fever/hypothermia, vomit, irritable, bulging fontanelle
Older children: fever, loss of appetite, joint and muscles pain, altered mental state, increased ICP, positive meningeal signs
All ages: petechiae, seizures, photophobia, fever
38
Assessment/treatment of meningitis
1. ABCDE assessment
2. OMV (IV or OM access)
3. Fluid resuscitation
4. Vasopressors
5. Take lab tests: WBC, CRP, PCT, liver, kidney, glc, coagulation, b.gas, microbiology
6. Antibiotics: <3mo: cefotaxime + ampicillin, >3mo: cefriaxone
7. Steroids: dexamethasone or hydrocortisone
39
Prophylaxis against meningitis
Meningococcus A/C/W/Y + B. vaccination
HiB vaccination
Pneumococcus vaccination
40
Define encephalitis
Inflammation of brain tissue. Usually involves meninges as well. Caused by direct injury or viral infection.
41
Prophylaxis given to people close contact with meningitis patients
Ciprofloxacin
42
Types of encephalitis
Viral
Immune-mediated
Autoimmune
43
Etiology of viral encephalitis
Enterovirus, arbovirus, herpes, adenovirus, TBE
44
Mechanism of immune mediated encephalitis
ADEM: acute dissaminated encephalomyelitis (demyelination disease in kids)
45
Mechanism of autoimmune encephalitis
NMDAe: antibodies against NMDA receptors cause the inflammation
46
Signs and symptoms of encephalitis
Fever, headache, light sensitivity, vomiting, change in consciousness, hallucination, delirium, seizures
47
Diagnosis of encephalitis
CSF: detect virus, autoantibodies
MRI
EEG
48
Treatment of encephalitis
– HSV: acyclovir
– ADEM, NMDAe: high dose methylprednisolone, IVIG, plasmapheresis
– Supportive treatment: pain management, electrolytes
49
Complications of encephalitis
Cognitive impairment, epilepsy
50
Difference in lesions of central- and peripheral facial nerve palsy
Central facial palsy: supranuclear and nuclear lesion
Peripheral facial palsy: infranuclear lesion
51
Etiology of central nerve palsy
MS, poliomyelitis, cerebral tumors, lacunar infarction, stroke, TIA
52
Etiology of peripheral nerve palsy
Infections: herpes zoster oticus, HSV, EBV, Lyme
Trauma
Bells palsy (idiopathic)
Inflammation
Tumor
53
Clinical signs of central nerve palsy
– Paralysis of inferior 1/4 of the face contralat. to the lesion
– Loss of nasolabial folds and drooping of lower lip
– Preservation of forehead and brow movements
54
Clinical signs of peripheral nerve palsy
– Paralysis of lateral half of the face ipsilateral to the lesion
– Loss of forehead and brow movements
– Inability to close eyes and drooping of eyelids
– Loss of nasolabial folds and drooping of lower lip
55
Diagnosis of facial nerve palsy
– History
– Lab tests
– Imaging
– Inspection
56
Diagnosis of facial nerve palsy
– History
– Lab tests
– Imaging
– Inspection
57
Testing motor function of facial nerve
Frontal branch: Wrinkling of forehead
Ophthalmic branch: Rapid blinking, lip closure
Oral branch: Baring of teeth, whistling, inflating cheeks
58
Testing of parasympathetic function of facial nerve
Schirmer's test:
Gaustometry: evaluation of taste anterior 2/3
59
Testing of sensory function of facial nerve
Skin around the ear de
60
Phenotypes of seizures in childhood
Generalized, focal, myovlonic, non-compulsive
61
Etiology of seizures
- Febrile convulsions (2/3 causes under 3y)
- Epilepsy
- CNS pathology
- Metabolic problems, electrolyte disorders, toxins
- Idiopathic (10-20% in all ages)
62
Define seizures
A seizure is a clinical event in which there is a sudden
disturbance of neurological function caused by an
abnormal or excessive neuronal discharge. Seizures
may be epileptic or non-epileptic.
63
Causes of seizures
Epilepsy
* Idiopathic (70–80%) – cause unknown but
presumed genetic
* Secondary
– Cerebral dysgenesis/malformation
– Cerebral vascular occlusion
– Cerebral damage, e.g. congenital infection,
hypoxic-ischaemic encephalopathy,
intraventricular haemorrhage/ischaemia
* Cerebral tumour
* Neurodegenerative disorders
* Neurocutaneous syndromes
Non-epileptic
* Febrile seizures
* Metabolic
– Hypoglycaemia
– Hypocalcaemia/hypomagnesaemia
– Hypo/hypernatraemia
* Head trauma
* Meningitis/encephalitis
* Poisons/toxins.
64
Define febrile seizures
seizure accompanied by a fever in
the absence of intracranial infection due to bacterial
meningitis or viral encephalitis
65
Management of seizure first 5 min
Provide safe environment and assess pt. with ABCDE. Keep airway open, start O2 and exclude hypoglycemia. Most of the seizure resolve within 5 min
66
Management of seizures 5-10 min
Now it's status epilepticus (>5min). Give benzodiazepines. Give 2 doses with 2-3 min in bw.
67
Management of seizures 10-20 min
If benzo was ineffective, run ABG (to exclude electrolyte imbalance), regularly assess ABCDE.
drugs: levetirecetam, valproic acid
68
Managament of seizure after 15 min
Admit to ICU/emergency dep., intubate and sedate.
Drugs: potent antiepileptics (propofol, ketamine) and muscle paralytics. Consider rare etiologies (NORSE).
69
Complications of seizures
Hypoventilation –> hypoxia, hypercapnia
Rhabdomyolysis –> kidney failure
Incr. lactic acid –> met. acidosis
If >24h –> brain edema, CNS injury, high mortality
70
Pathomechanism of febrile seizures
Increased body temperature leads to cytokine release and neuronal hyperexcitability
71
Complication of febrile seizure
High risk of developing epilepsy with recurrent episodes of febrile seizures
72
Define DM1
Chronic hyperglycemia due to destruction of B-cells in the pancreas, so they are unable to produce insulin. This is an autoimmune process.
73
Symptoms of hyperglycemia in infants and children
Infants: vomiting, dehydration, coma
Children: Polyuria, enuresis, polydypsia, weight loss, blurred vision
74
Diagnosis of DM1 in children
– Investigate b.glc and ketones and/or urinary glc and ketones
– Fasting glc > 7mmol/L + random b.glc > 11 mmol/L twice
– Clinical signs
75
Frequent differential diagnosis/misdiagnosis of DM1 in children
Misdiagnosis done bc. of the symptoms:
– Kussmaul breathing –> lung/heart disease
– Polyuria –> UTI
– Unconsciousness –> meningitis, encephalitis
76
Causes of DKA
Infections (50%)
Not taking insulin
Puberty
77
Clinical signs of DKA
Metabaolic acidosis
Hyperglycemia
Ketones in blood/urine
78
Symptoms of DKA
– Fq. urination
– Incr. thirs
– Dry mouth
– Blurry vision
– Sweet breath (bc. of ketones)
– Nausea, vomiting
– Abd. pain
79
Treatment of DKA
1. ABCDE assessment
2. Fluids (balanced crystalloids)
80
Treatment of DKA
1. ABCDE assessment
2. Fluids (balanced crystalloids)
3. Insulin
4. Insulin + glc
5. Electrolyte resuscitation (K+)
81
Fluid therapy in children
0. fluid bolus = 10ml/kg
1st. 10kg = 150ml/kg
2nd. 10kg = 50ml/kg
3rd. 10kg = 20ml/kg
82
Define congenital adrenal hyperplasia
group of autosomal recessive defects in the enzymes that are responsible for cortisol/aldosterone/androgen synthesi
83
Characteristic of all CAH (cong. adrenal hyperplasia) subtypes
- Low levels of cortisol
– High levels of ACTH
– Adrenal hyperplasia
84
Subtypes of CAH (cong. adrenal hyperplasia)
21b-hydroxylase defect (95%)
11b-hydroxylase defect (5%)
17a-hydroxylase defect (rare)
85
Pathophysiology of CAH
Low levels of cortisol leads to lack of negative feedback to the pituitary. Increased ACTH –> adrenal hyperplasia + increased synthesis of adrenal precursor steroids
86
Clinical features of CAH
– Low levels of cortisol leads to hypoglycemia
– Adrenal crisis
21b-hydroxylase defect: hypotension
11b-hydroxylase defect: hypertension
17a-hydroxylase defect: hypertension
21b-hydroxylase defect and 11b-hydroxylase defect
– female clitoromegaly or male genitalia, virilization, infertility, precocious puberty
– male normal external genitalia, precocious puberty
17a-hydroxylase defect
– female normal ext. genitialia at birth, delayed puberty, sexual infantilism
– male: female ext. genitalia, delayed puberty, sexual infantilism
87
Treatment of CAH
Replace the deficient hormones and reduce excess androgen production. Glucocorticoid replacement therapy is indicated in all subtypes (hydrocortisone in pediatrics).
21b – fludrocortisone
11b – spironolactone
17a – spironolactone, estrogen replacement ther.
88
Etiology of primary congenital hypothyroidism
Ectopic thyroid gland (50%)
Thyroid aplasia/hypoplasia (30%)
Thyroxin synthesis defects
89
Most common etiology of hypothyroidism in children
The most common cause is an autoimmune reaction that destroys the thyroid gland. (autoimmune thyroiditis, Hashimoto)
90
Etiology of secondary congenital hypothyroidism
Pituitary aplasia/hypoplasia, perinatal stress
91
What is congenital hypothyroidism?
Congenital hypothyroidism (CHT) is a condition resulting from an absent or under-developed thyroid gland (dysgenesis) or one that has developed but cannot make thyroid hormone because of a 'production line' problem (dyshormonogenesis). Babies with CHT cannot produce enough thyroid hormone for the body's needs.11. sep. 2019
92
Symptoms of congenital hypothyroidism
Usually asymptomatic and picked up on screening. Otherwise:
Failure to thrive
Feeding problems
Prolonged jaundice
Constipation
Pale, cold, mottled dry
skin
Coarse facies
Large tongue
Hoarse cry
Goitre (occasionally)
Umbilical hernia
Delayed development
93
Complication of untreated hypothyroidism
Cretinism: impaired dev. of brain and skeleton.
Low stature (in autoimmune thyroiditis, which can go on for a very long time undiagnosed bc. of no screening for it)
94
Screening of congenital hypothyroidism
Measure TSH from dried b. sample in postnatal 48-72h. Increased levels are indicative of cong. hypothyroidism
95
Symptoms and clinical signs of Hashimoto's thyroiditis
Dry skin, thin hair
Bradycardia
Goitre
Short stature/growth restriction
Delayed puberty
Obesity
Vold intolerance
Cold peripheries
96
Treatment of Hashimoto's thyroiditis
Thyroxine
97
Most common cause of hyperthyroidism in pediatrics
Grave's disease
98
Define Grave's disease
Immune (autoimmune) disorder leading to increased production of thyroid hormones
99
Symptoms of Graves' disease
Heat intolerance, excessive sweating (incr. met. rate)
Weight loss (incr met. rate)
Glucose intolerance
Exapthalmos
Tachycardia
Increased BP
Anxiety, insomnia
100
Treatment of Graves' disease
Antithyroid medications: methamizole
Symptomatic treatment: beta blockers (propanolol)
Radioactive iodine ablation: potential first line treatment in patients >10y
Surgery (near-total thyroidectomy): in children >5y not improved with antithyroid drugs
101
Define neonatal hyperthyroidism
Transplacental passage of maternal TSH receptor antibodies from mother with Graves' disease to its babies. Occurs in approx. 5% of babies born with mother having Graves' disease.
102
When does symp. of hyperthyroidism in neonates appear?
May appear at birth or be delayed for 10 days (bc. of maternal antithyroid medication passing transplacental to the baby).
103
Symptoms of neonatal hyperthyroidism
Irritable, restlessness, tachycardia, poor weight gain, diffuse goiter, microcephaly (bc. of premature fusion of cranial sutures)
104
Complications of untreated neonatal hyperthyroidism
Cardiac failure and intellectual disability
105
Treatment of neonatal hyperthyroidism
usually resolves within 1-3 months. If not, treat symptomatic infants with methamizol and propanolol.
106
Hypocalcemia etiology
Vitamin def., diabetes, acute renal failure, prematurity, DiGeorge syndrome
107
Hypocalcemia clinical features
Usually asymp., but spasms, seizures and tetanus can occur
108
Hypocalcemia treatment
In asymp infants: initiate feeding is enough
If not: Ca substitution
109
Hypercalcemia etiology
Idiopathic infantile hypercalcemia, hyperparathyroidism, Williams syndrome, hypercalcemia of malignancy, Vit D intoxication, renal failure, Addisons disease, iatrogenic (e.g. thiazide diuretics).
110
Hypercalcemia clinical features
Anoreia, nausea/vomit, constipation, abdominal pain, polyuria, polydypsia, drowsiness, depression
111
Hypercalcemia treatment
Treat underlying condition
112
Hyperparathyroidism
PTH –> bone resorption –> release Ca and P in blood AND increase P excretion in kidneys. Therefore hyperPTH gives high PTH in blood, high Ca in blood and low plasma P. It's uncommon in children.
Etiology: Rickets, MEN1 and 2, parathyroid adenoma
113
HypoPTH
Etiology: failure in prathyroid development (agenesis, dysgenesis), magnesium deficiency (failure in PTH secretion)
Clinical: low plasma Ca and PTH, high plasma P
114
Define Rickets
Disorder of impaired mineralization of cartilaginous growth plates due to Vit D deficiency. Only occurs in children bc. the growth plates haven't yet fused. (the adult version would be osteomalacia)
115
Etiology of Rickets
Vitamin D deficiency. Breast milk has low amounts of it and therefore newborns need supplementation.
116
Pathophys of Rickets
Vitamin D deficiency –> hypocalcemia –> defective growth plate mineralisation. Hypocalcemia –> incr. PTH –> decr. P –> also impaired mineralisation
117
Clinical features of Rickets
Bone deformities
– bending of long bones
– distension of bone-cartilage junctions: rachitic sign, marfan sign, craniotabes, genu varum
– increased fracture risk
– late closing of fontanelles
– impaired growth
118
Diagnosis of Rickets
Lab: decr. Ca and P, incr. PTH and ALP
Imaging: bone deformities
Bone biopsy
119
Diagnosis of growth disorders
– Sex, background, genetics
– Bone age
– Calculation of mid-parental height
– Calculation of target heigh channel
120
What is target height channel?
Determination wether child is growing according to familys genetic background.
Boys: (fathers height + mothers height)/2 + 13cm
Girls: (fathers height + mothers height)/2 - 13cm
121
Etiologies of short stature
Non-pathological family short stature
Endocrine etiologies
– Hypothyroidism
– GH deficiencies
– Glucocorticoid excess
– DM1
Genetic etiologies
– Turner syndrome
– Downs syndrome
– Williams synrome
– CF
Psychosocial etiologies
– Maternal substance use
– Psychoscoial short stature
– Psychiatric conditions (anorexia)
122
Etiologies of tall stature
Non-pathological family tall stature
Endocrine etiologies
– Hyperthyroidism
– Obesity
– GH excess
Genetic etiologies
– Fragile X syndrome
– Marfan syndrome
– Klinefelter
– Triple X syndrome
123
Pubertal stages
Tanner stages:
– breast development
– genital development
– pubic hear development
124
What is considered precocious puberty?
Appearance of secondary sexual characteristics before the age of:
Girls – 8 years
Boys – 9 years
125
Classification of precocious puberty
Central (true) precocious puberty: gonadotropin dep.
Peripheral precocious puberty: gonadotropin indep.
Benign pubertal variants
126
Define central precocious puberty
Elevated GnRh (gonadotropin) levels. Most commonly idiopathic, but can be due to CNS problems, obesity etc.
It leads to an early activaiton of hypothalamo-hypophysial axis and abnormally early onset of puberty. Follows normal pattern of puberty, it's just early.
127
Diagnosis of central precocious puberty
Serum LH and FSH increased
GnRh stimulation test, Gn should increase
Brain MRI/CT: rule out CNS lesion
128
Treatment of central precocious puberty
GnRH agonist
129
Define peripheral precocious puberty
Indep of GnRH. Due to peripheral synthesis or exogenous exposure to sex hormones. Either due to incr. androgen production or estrogen production or increased b-hCG production.
May not follow normal pattern of puberty.
130
Diagnosis of peripheral precocious puberty
Serum LH and FSH decreased
GnRH stim. doesnt lead to incr. Gn
Incr. serum testo/estrogen
131
Define disorders of delayed pubertal growth and sexual maturation
Absent or incomplete developemnt of secondary sexual characteristics by age of 14 in boys and 13 in girls
132
Etiologies of delayed pubertal growth
Hypergonadotropic hypogonadism
– primary gonadal insufficiency (kinefelter, turner, androgen insensiticity)
– secondary gonadal insufficiency (chemother., infection, trauma, autoimmune)
Hypogonadotropic hypogonadism
– CNS lesion
– Kallmann syndrome
– Prader-Willi syndrome
133
Define Henoch-Schonlein purpura/IgA vasculitis
Autoimmune complex-mediated small vessel vasculitis. Most commonly occurs in children > 5y. Often preceeded y an URTI.
134
What is the mechanism of Henoch-Schonlein purpura?
Exposure to allergen/antigen (e.g. infection/drugs) –> stimulation of IgA production –> deposition of IgA immune complexes in vascular walls –> activation of complement system –> vascular inflammation and damage
135
Symptoms of Henoch-Schonlein purpura
Typical triad:
– palpable purpura
– arthritis/arthralgia
– abdominal pain
– renal disease (hematuria)
136
Treatment of Henoch-Schonlein purpura
Usually self-limiting, which only requires supportive care (NSAIDS for pain, rest, hydration)
Severe disease: systemic glucocorticoids, acute dialysis, antihypertensives, renal transplantation, IV fluids,
137
Define Kawasaki syndrome
Acute necrotizing vasculitis of unknown etiology. Primarily affects children <5y, more common in asian population.
138
Clinical diagnosis of Kawasaki syndrome
Requires at least 5d of fever + one of:
– 4 or more other specific symptoms
– under 4 specific system + coronary artery involvement
Specific symptoms:
– erythema + edema of hans and feet
– desquamation of fingertips and toes
– polymorphous rash originating from trunk
– conjuctivitis without exudate
– oropharyngeal mucositis: strawberry tongue
– vercival lymphadenopathy
139
Treatment of Kawasaki syndrome
IVIG: reduce risk of coronary artery aneurysms
Oral aspirin high dose: anti-inflam. effect
Oral aspirin low dose later: for anticoag. effect
IV glucocorticoids: lower the risk of coronary involvement
!!! Kawasaki syndrome is a rare exception to the CI of giving aspirin to children
140
Complications of Kawasaki syndrome
Coronary artery aneurysm, AMI, myocarditis, ventricular dysfunction, arrythmias
141
What is MISC?
Complication of COVID-19 manifesting with severe illness, hyperinflammation, multi organ failure etc.
Symp: fever, GI symp., etc
Criteria:
– Age <21y
– fever
– + inflam. markers
– involvement of 2+ organs
– severe illness requiring hospitalization
Tr: not sure yet what is most effective
- steroids
- antithrombotic treatment
– immunomodulators
142
Treatment of UTI
3rd gen cephalosporins
Aminoglycosides
!!50% are resistant against ampicillin and amoxicillin
Neonates: ampicillin + gentamycin
143
List kidney malformations
Renal agenesis
Renal hypoplasia
Horseshoe kidney
Kidney dysplasia
Multicystic dysplastic kidney
Ectopic kidney
Hydronephrosis
Duplex kidney
144
Ureter development disorders
Pyelouretral junction stenosis
Ureter-vesicular stenosis
Uterocele
Vesico-ureteral reflux
145
Bladder malformations/disorders
Bladder extrophy
Bladder diverticulum
Urachus persistens
146
Urethra malformations
Posterior urethral valve
Hypspadius
Epispadius
147
Define glomerulonephritis
Damage to the gomeruli. It's often caused by your immune system attacking healthy body tissue. Glomerulonephritis does not usually cause any noticeable symptoms. It's more likely to be diagnosed when blood or urine tests are carried out for another reason.
148
Clinical signs of glomerulonephritis
Hematuria
Oliguria
Edema
Hypertension
Variable proteinuria
149
Etiology of glomerulonephritis
Post infectious (most common)
- Bacterial (strep., staph.,)
- Viral (herpesvirus: EBV, varicella, CMV)
- Fungi (candida, aspergillus)
- Parasites (toxoplasma, malaria)
Others (less common)
- MPGN
- IgA nephropathy
- SLE
150
Treatment of glomerulonephritis
Treat all life-threathening conditions:
– hyperkalemia, HT, acidosis, seizures, hypocalcemia
Supportive treatment and monitoring:
– fluid balance
– HT: CCB, a-block., DO NOT USE ACEi
– Ab if bacterial cause (penicillin against strep)
151
Diagnosis of glomerulonephritis
Urinalysis (hematuria, proteinuria, RBC casts
Blood (CBC, autoAb, albumin)
Renal US
152
Most common cause of glomerulonephritis
Post-streptococcal glomerulonephritis
– Strep. pyogenes (GAS)
153
Define nephrotic syndrome
Proteinuria > 3g/day
Hypoalbuminemia <25g/L
Edema
Hyperlipidemia
154
Etiology of nephrotic syndrome
Primary: congenital, infantile
Secondary:
– minimal change disease (85%)
– focal segmental glomerulosclerosis
– membrane proliferative glomerulonephritis
– membranous glomerulonephritis
155
Clinical features of nephrotic syndrome
Edema: initially preorbital, then becoming generalized with pitting edema)
Foamy urine
Hypercoaguable state
HTN
156
Diagnosis of nephrotic syndrome
Urinalysis: protein+++
Microscopy: hematuria/RBC casts
Culture
Protein:creatinin ratio (>300mg/mmol)
Lipids (hyperlipidemia)
157
Treatment of nephrotic syndrome
Fluid resuscitation + prevention of hypovolemia
Oral steroids
Prophylaxis against bacterial infection (esp. pneumococci)
Immunosupressants (cyclophosmamide)
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Complications of nephrotic syndrome
Infection (bc of decr. IgG)
Thrombosis (hypercoag. state)
Hypovolemia
Acute renal failure
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Cyanotic congenital heart defects types
R-L shunt: tetralogy of fallot
Separate circulations: transposition of great arteries
Complete mixing: tricuspid atresia, truncus arteriosus, hypoplastic left heart syndrome, TAPVR
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Tetralogy of fallot
1. Pulmonary stenosis
2. Ventricular septal defect
3. Misplaced aorta
4. Right ventricular hypertrophy
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Clinical features of tetralogy of fallot
Boot-shaped heart on XR
Cyanosis
Shortness of breath
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Diagnosis of tetralogy of fallot
Ultrasound
Echocardiogram
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What is transposition of great vessels?
Pulmonary artery is rising from LV and aorta is arising from RV. It's associated w. maternal DM
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Clinical features and treatment of transposition of great vessels
Signs: cyanosis
Tr: Shunting (ASD), PGE (prostaglandin) adm. to maintain PDA until surgery.
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Tricuspid atresia
Absent tricuspid valve resulting in no blood flow between RA and RV. This leads to RV hypoplasia and RA dilation due to volume overload.
Clinical: cyanosis, diminished peripheral pulses
Tr.: surgery (making shunting)
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Define truncus arteriosus
Underdeveloped aorticopulmonary septum, trunkis arteriosus not diveded into aorta and pulm. trunk. This results in a mixture of ox. and deox. blood.
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Which disease is truncus arteriosus associated with?
DiGeorge syndrome
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Symptoms and treatment of truncus arteriosus
Cyanosis, resp. distress, systolic murmur, bounding peripheral pulses.
Tr.: surgery.
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Non-cyanotic congenital heart defects
L-R shunts: ASD, VSD, AVSD, PDA
Obstructive lesions: AS, PS, aortic coarctation
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Atrial septal defect (ASD)
– Defect in atrial septum.
– Associated with Down's and fetal alcohol syndrome
– L-R shunt
– Split S2
– Paradoxical emboli
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Ventricular septal defect (VSD)
– Defect in ventricular septum
– Most common congenital heart defect
– Ass. with Down's syndrome, TORCH, maternal DM
– L-R shunt: dilation of LV bc of incr. preload)
– Tr.: Loop diuretics, ACEi, surgery
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Patent ductus arteriosus (PD)
– Failure of the closure (usually close within 12-24h)
– Associated with Rubella
– L-R shunt
In utero, DA shunts blood from right to left in order to bypass the lungs. If DA stays open, the blood starts to shunt the other way bc. of increased systemic circulation.
– Ascultation: machine like murmurs, continous over systole + diastole
– Complication: eisenmenger syndrome
– Tr.: indomethacine (decrease prostaglandin and closure of DA)
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Coarctation of aorta
Narrowing of the aorta. Divided into infantile and adult form.
Infantile: ass. with PDA, lower extr. cyanosis
Adult: not ass. with PDA, HT in upper extr. and hypotension in lower extr.
Important to due 4 limb oxymetry to look for this. If decreased in legs –> possible coarctation that needs to be double checked
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Classification of malabsorption
Impaired intraluminal digestion
Intestinal malabsorption
Malabsorption due to fermentation (maldigestion of cbh)
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Define malabsorption
Malabsorption is difficulty in the digestion or absorption of nutrients from food. Malabsorption can affect growth and development, or it can lead to specific illnesses
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Leading cause of malabsorption
Cystic fibrosis
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Etiology of malabsorption
Coeliac disease, cystic fibrosis, cow’s milk protein intolerance, cholestatic liver disease,
short gut syndrome, post-necrotising enterocolitis (NEC)
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Malabsorption manifest as:
– abnormal stools
– failure to thrive or poor growth in most but not all
cases
– specific nutrient deficiencies, either singly or in
combination.
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Define celiac disease
Immune mediated systemic disorder elicited by gluten. The gliadin fraction of gluten provokes a damaging immunological response in the proximal small intestinal mucosa. The villi becomes shortened and flattened.
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Symptoms of celiac disease
– Profound malabsorptive syndrome at 8–24 months of age after the introduction of wheat-containing weaning foods.
– Failure to thrive, abdominal distension and
buttock wasting abnormal stools and general irritability
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Diagnosis of celiac disease
– Clinical suspiction or high risk group
– Measure transglutaminase antibodies and total IgA
– Biopsy by endoscopy (not done unless highly necessary, then taken from distal duodenum)
– Endomysial antibdoies
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Respiratory distress syndrome caused by
surfactant deficiency
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Symptoms of respiratory distress syndrome
Cyanosis, tachypnea, grunting, dyspnea
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What can happen if giving prolonged ventilation and ox. therapy in newborn
Bronchopulmonary dysplasia
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Prevention of respiratory distress syndrome
Corticosteroids given to mother 1-7 days before delivery
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Acute neonatal respiratory diseases
Transient tachypnea of newborn
Congenital pneumonia
Meconium aspiration syndrome
Milk aspiration
Persistant pulmonary HT of newborn
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Conse of congenital pneumonia
Aspiration of infected amniotic fluid
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TORCH
Toxoplasmosis, rubella, CMV, herpes, others (HIV, syphilis, hepatitis B, herpes zoster).
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Normal bilirubin vs. hyperbilirubinemia
Normal: 0,1-1,2mg/dl
Hyper: >5mg/dl
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Etiology of physiological neonatal jaundice
Hemolysis of fetal hgb and immature hepatic metabolism of bilirubin
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How can you differ between unconjugated and conjugated hyperbilirubinemia?
– Bilirubin in urine: suggest conjugated
– Serum bilirubin <15% of total bilirubin: unconj.
– Serum bilirubin >20% of total bilirubin: conj.
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Define retinopathy of prematurity
Retina with abnormal vessel proliferation
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Define necrotizing enterocolitis
hemorrhagic inflammation of the intestinal wall
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define SIRDS
Abrupt and unexplained death of an infant less than 1 year
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Pediatric etiologis of fever
Infections
Malignancy
Autoimmune
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Etiology of respiratory acidosis
Respiratory type II (hypercapnic)
197
Symptoms of respiratory acidosis
Decreased inotropy, hypoxia, hyperkalemia, emesis, coma, insulin resistance, hypervenitaltion (compensation), increased symp. activity (incr. HR, BP, hyperthermia)
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Etiology of respiratory alkalosis
Primary of secondary hyperventilation.
– Hypoxic resp. failuer
– Salicylate intoxication
– Early sepsis
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Etiology of metabolic acidosis
Incr. anion gap: increased acid (lactic, keto) + renal failure
non-anion gap: loss of HCO3 (renal tubular acidosis, GI acidosis, iatrogenic acidosis, diarrhea)
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Define cholestasis
Impaired bile flow: accumulation in liver and serum of subsances that normally are secreted in the bile – bilirubin, bile acids, cholesterol
201
Etiology of cholestasis
Intrahepatic and/or extrahepatic
– biliary atresia
– alagille syndrome
– alpha-1-antitrypsin deficiency
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Define biliary atresia
Closed/discontinous biliary tracts. Affects both intra- and extrahepatic bile ducts
203
Complications of biliary atresi
Early liver cirrhosis (at approx. 9 weeks of age), high mortality.
204
Forms of biliary atresia
Congenital (10%)
Postnatal/parinatal (90%)
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Clinical presentation of biliary atresia
Prolonged jaundice, hepatomegaly, bleeding, portal HT, cirrhosis
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Treatment of biliary atresia
Creating connection between liver and small intestines to allow bile drainage.
Liver transplant in case of liver cirrhosis
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Define Allagille syndrome
Genetic condition char. by intrahepatic biliary duct aplasia or hypoplasia.
208
Clinical manifestation of Alagille syndrome
Hepatic: jaundice, chirrosis
Facial dysmorphisms
Congenital heart defects
Butterfly vertebrae
Renal dysplasia
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Diagnosis of Alegille syndrome
5 major criteria, 3 required:
– interlobular bile duct absence/cholestasis
– cardiac malformation
– spine deformity
– characteristic face
– ocular abonrmality
Diagnosis done by genetic testing of JAG1 gene
