Author: Dr. Ayush Goel
Q. Whats the diagnosis for this classical appearance ?
 |
| Image courtesy: Dr Ayush Goel |
ANSWER:
Showing posts with label Pediatrics. Show all posts
Showing posts with label Pediatrics. Show all posts
0 Radiology Quiz 5
Author: Dr. Ayush Goel
Q. Whats the diagnosis for this classical appearance ?
2 Pulmonary sequestration
Author: Dr. Ayush Goel
Pulmonary Sequestration = accessory lung (embryonic cut off of a segment from main
lung)
|
Types:
|
Intralobar
|
Extralobar
|
|
|
More common (75-85% cases)
|
Less common (15-25% cases)
|
|
|
Usually Child with recurrent infections
|
Usually Neonate with respiratory distress and cyanosis
|
|
Arterial supply
|
Aorta (Same in both types)
|
|
|
Venous supply
|
usually Pulmonary venous
drainage
|
Usually systemic venous drainage
|
|
Pleura
|
Same pleura as ipsilateral lung
|
Separate pleura from ipsilateral lung
|
|
Site
|
60% cases affect left lower lobe (Most commonly Posterior basal
segment); 40% right lower lobe.
|
Always affect left lower lobe; can be subdiaphragmatic in 10% cases
|
|
|
Sequestrated lung appears hyperechoic than rest of lung on prenatal ultrasonography
|
|
0 Nephrotic Syndrome
Nephrotic Syndrome
- Proteinuria (Albuminuria) > 40 mg/l2/hr (or > 2g /day )
- Hypoproteinemia < 2.5 g/dl
- Hyperlipidemia (Serum cholesterol > 200 mg/dl)
=> 85 % cases are Minimal change disease.
Electron microscopy shows effacement of foot process of podocytes.
=> In Adults Most common is Membranous glomerulonephritis (can have Renal vein thrombosis)
=> Mostly nephrotic syndrome is steroid sensitive, thus DOC - Prednisolone.
Clinical features :
- Oedema
- Hypovolemia
- Loss of Ig in urine - thus infections (Most common Spontaneous bacterial peritonitis - cause Pneumococcus ) (DOC - Cefotaxime)
- Loss of Protein C and S (thus risk of thrombosis)
Most common cause of steroid resistant nephrotic - FSGS
Genetics :
- NPHS 1 - Nephrin ( Mutation causes FINNISH syndrome)
- NPHS 2 - Podocin ( Mutation causes steroid resistant nephrotic syndrome)
DOC for steroid resistant nephrotic :
- Tacrolimus ( Its Calcineurin inhibitor )
- Other Calcineurin inhibitor is Cyclosporin - Side effect Acne , Gingival hyperplasia
Both are nephrotoxic.
- Rituximab (Monoclonal Antibody against CD 20)
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Chronic kidney disease
Chronic kidney disease (previously known as Chronic Renal Failure)
Most Common cause in < 5yr
- Obstructive uropathy (eg: Posterior urethral valve in males)
- Dysplasia/hypoplasia of kidney
Most Common cause in > 5yr
- Acquired ( Glomerulonephritis , HUS )
Complications :
- Azotemia
- Anaemia
- Acidosis
- Bony problems (Rickets , Renal Osteodystrophy)
- CVS problems (in Adults)
- Growth failure
If ESRD (end stage renal disease i.e GFR < 15 ml/min/1.73m2 ) then there will be need of dialysis or transplant (advised in child)
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Acute Renal Failure
Acute renal failure ( better termed as Acute kidney injury )
Markers :
- NGAL ( Neutrophil gelatinase associated lipocalin )
- IL-18
- KIM-1 ( Kidney injury molecule - 1 )
Can be :
- Pre-Renal
- Renal
- Post-Renal
Pre-Renal
(can have hyaline cast)
|
Renal
(granular cast)
|
|
Urine Na
|
<20 meq/l (or < 10 mmol/l)
|
>40 meq/l (or > 20 mmol/l)
|
Urine Osmol.
|
>500
|
<300
|
Blood urea / creatinine
|
>20:1
|
< 20 : 1
|
Fractional excretion of Na %
|
<1
|
>1
|
MC cause of ARF in
- Newborn : Birth Asphyxia
- Children ( 1 - 3yr ) : 1. HUS ; 2. ATN
MC Complications ( and their management ) :
- Fluid overload ( so restric fluids to 400ml/m2 - dextrose)
- Hyperkalemia ( so - Nebulize beta 2 agonist , IV -Na Bicarb , IV Insulin with dextrose , Resins , Haemodylasis , Ca Gluconate for cardiac stabilization )
- Hyponatremia -dilutional ( manage same as 1 )
- Dilutional anaemia ( so transfuse RBC )
- Increase phosphate , decrease calcium ( so Restrict phosphate, use phosphate binders )
- Metabolic acidosis ( so use Na Bicarb )
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Alport Syndrome
Alport Syndrome
Mutation involving Alpha 5 domain of type 4 Collagen.
( Confuser : Alpha 3 domain of type 4 collagen is the antigen involved in Good pasture syndrome )
Most commonly - X-Linked dominant
Triad :
- Sensorineural deafness
- Anterior Lenticonus (pathognomonic)
- Haemorrhagic nephritis
Electron microscopy is must for diagnosis.
GBM changes - Striations / irregularities , splitting of lamina densa giving Basket weave appearance.
Treatment : Symptomatic
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Schwartz method / equation for estimating GFR in children
Schwartz equation :
GFR (mL/min/1.73 m2) = ( k × Height in cm) / Creatinine in mg/dL
Creatinine value depends on
- Method of calculation : Jaffe's reaction ; Enzyme assay (best)
- Muscle mass
k is a constant that depends on age.
Thus GFR will depend on :
- Age
- Height
- Muscle mass
- Method of calculating creatinine.
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Celiac disease
Celiac disease ( Coeliac disease )
- Gluten sensitivity
Thus if wheat is introduced in diet , there can be -
1. Chronic Diarrhoea
2. Recurrent diarrhoea
3. Abdominal distention
4. Vomiting
5. Anorexia
6. Failure to thrive - Can be asymptomatic
- Age group : 6 months - 24 months
MCQ point - HLA DQ2 , DQ8
MARSH Criteria
- Disease of small intestine in which
- Villous atrophy
- Crypt hyperplasia
- Transmural inflammation - If restrict gluten - mucosa returns to normal
- If gluten challenge - mucosa goes back to original state
Best Test :
- Anti Endomysial Antibody (Specificity 97 - 100%)
- Anti TTG (Tissue transglutaminase antibody )
Treatment : Life long gluten restriction
i.e NO TO - Wheat , Barley and Rye.
i.e NO TO - Wheat , Barley and Rye.
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 ORS - oral rehydration salts solution
mmol/L of
|
WHO ORS
|
ReSoMal
|
Low Osmolar ORS
|
Na |
90
|
45
|
75
|
K
|
20
|
40
|
20
|
Glucose
|
111
|
125
|
75
|
Cl-
|
80
|
70
|
65
|
Citrate
(increases shelf life)
|
10
|
7 + Mg , Zn , Cu
|
10
|
- WHO ORS was based on cholera stools.
- In Rotavirus - Na loss is 50 - 70 mmol/L
- Malnourished children are Na overloaded and K depleted - hence ReSoMal is used.
Management of diarrhoea in child (along with ORS) -
1. Probiotics are used in rotavirus infections :
- give Saccharomyces boulardii and Lactobacillus rhamnosus
2. Zinc : 2 RDA (i.e 2 x 10 mg/day) during diarrhoea + 2 wks after diarrhoea subsides
3. Anti Secretory : Racecadotril- enkephalinase inhibitor
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Acute flaccid paralysis
Acute flaccid paralysis (AFP)
Age group : 0 - < 15 years
Provided pseudoparalysis is ruled out :
- Scurvy
- Osteomyelitis
- Syphilis
- Hypokalemia
Causes of AFP :
1. Paralytic poliomyelitis
- Asymmetrical
- at onset of paralysis - high grade fever
2. Traumatic neuritis : History of IM gluteal
3. Transverse myelitis : (Usually viral) Varicella
4. Guillain-Barré Syndrome - GBS (AIDP)
- follows C.Jejuni diarrhoea - 10 days later - GBS
other - Mycoplasma - Ascending , Areflexic , Symmetrical paralysis
- Proximal muscles affected 1st
- CSF : Albumino-cytological dissociation (at the end of 2nd week) - MCQ
- NCV : decreased , symmetrical demyelinating neuropathy
- Treatment : Iv-Ig , Plasmapheresis
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Encephalitis (Most common causes and salient points)
Most common causes :
- Sporadic encephalitis - Herpes
- Epidemic encephalitis - Arbovirus
- Viral encephalitis - Enterovirus (also MC meningoencephalitis)
- Brainstem encephalitis - Rabies
Viral encephalitis : causes
- Enterovirus (80%)
- Arbovirus
- Herpes
- Mumps
HSV encephalitis :
- MRI - Temporal lobe hyperintensities on T2 and Flair
- CSF : haemorrhagic ; PCR for HSV
- Treatment - Acyclovir
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Meningitis in child
Meningitis
- 1st 2 months - E.Coli (MC)
- 2months - 12 years - Pneumococcal and Neisseria.
95% Meningitis in child - affects 1month - 5years age group.
|
Condition
|
Organism
|
|
Defect of complement system
C5-8 and properdin system
|
Meningococcus
|
|
Congenital / acquired defect
across mucocutaneous barrier
|
Pneuococcal
|
|
T.Lymphocte defect
|
Listeria
|
|
Lumbosacral Myelomeningocele
and Dermal sinus
|
Staphylococcus ; Enteric
bacteria
|
|
Penetrating CNS trauma
; CSF shunt infection
|
Coagulase –ve Staph.
|
|
Recurrent meningitis in
CSF leak patients
|
Pneumococcal
|
Investigation :
85% cases have bacteremia
therefore -
1. Blood culture
2. CxR
3. Lumbar puncture - Increased ICT is absolute contraindication
2. CxR
3. Lumbar puncture - Increased ICT is absolute contraindication
- Turbid grossly
- Neutrophil leucocytosis
- Protein (normal 50mg%) - raised
- Sugar (normal 2/3 blood sugar) - decreased (permeability to glucose in meninges decreased ; increased anaerobic glycolysis in adjoining brain tissue)
Treatment :
- 3rd generation cephalosporins - ceftriaxone.
- Pneumococci resistant to cephalosporins - Vancomycin
Complications :
- SIADH
- Sub-dural effusions
- Seizures
- Increased ICT
- Cerebral edema
MC Neurological sequelae : Deafness (Sensorineural) , because of labrynthitis.
- Prevented by Dexamethasone (0.15mg/kg) 15-30 mins before antibiotics.
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Status Epilepticus in Pediatrics
Pediatric Status Epilepticus
MC cause
- Prolonged febrile seizures
- Sudden withdrawal of antiepileptic drugs
- 1st Midazolam (0.1 - 0.3 mg/kg) or Diazepalm (0.3 - 0.5 mg/kg) - Quick action
- 2nd Phenytoin (20 mg/kg )
- If No effect - give Lorazepalm (0.1 mg/kg)
- If No effect - repeat phenytoin (10 mg/kg)
- If No effect - Phenobarbitone (20 mg/kg)
- If No effect - repeat phenobarbitone (10 mg/kg)
- Still no effect - injection Valproate 20/30 mg/kg
- Midazolam infusion 2ug/kg/min , increase every 15 mins to 20ug/kg/min
ICU (Ventilator)
General anaesthesia (Thiopentone)
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Febrile Seizures
Febrile Seizures
- Age group : 9 months - 5 years
- MC during childhood, comes in 14-18 months (Mostly)
Typical :
- GTCS
- < 10 mins duration
- Single febrile episode
- Risk of epilepsy later - 1%
Atypical :
- Partial seizures
- > 15 mins
- Multiple febrile episodes
- Risk of epilepsy later - 7%
Recurrence : 30 - 50 %
Thus Intermittent prophylaxis is used : Fever - Paracetamol , Diazepalm / Clobazam (oral)
Note :
- Rule out meningitis as a differential diagnosis.
Meningeal signs are rare in < 18months , therefore in these children do Lumbar puncture also. - Prolonged febrile seizures can cause Mesial Temporal Sclerosis.
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Infantile Spasms
Infantile Spasms (Salaam Seizures = West syndrome)
- Onset at 4 - 8 months life.
- Flexor Spasm (Symmetrical contracture of head trunk and extremities)
- Rarely extensor or mixed
- During sleep or arousal.
EEG - Hypsarrhythmia ( Chaotic pattern of high voltage bilateral synchronous waves)
Types : Idiopathic ; Secondary
Idiopathic = Cryptogenic ( Good prognosis )
Secondary -
- Birth asphyxia
- Structural malformations of brain
- Tuberous sclerosis
- Downs syndrome
Drug of choice : ACTH ( to suppress CRH - corticotropin releasing hormone)
DOC in cases of Tuberous sclerosis - Vigabatrin
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Juvenile myoclonic epilepsy
Juvenile myoclonic epilepsy
Also known as Janz syndrome.
Typically manifests first between 12-18 years age.
Seizures :
- Myoclonic (frequently in the morning)
- GTCS (majority patients have this along with myoclonic)
- Absence (1/3 rd can have these)
MCQ point : There is never a complete remission but response to valproate is very good.
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
2 Micro and Macrocephaly
Micro and Macrocephaly
Microcephaly : > 3 SD below mean
Macrocephaly : > 2 SD above mean
Newborn Head circumference - 35 cm , By 2year sge - it reaches 48cm (i.e 90% of adult head circumference is achieved in 2yr)
Other causes of Microcephaly :
- Familial (AR)
- Down's syndrome
- Edward syndrome
- PEM
- HIE
- Hypoglycemia
- Fetal Alcohol syndrome
- Fetal hydantoin syndrome
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
1 Cystic Fibrosis
Cystic Fibrosis
- Mutation in CFTR gene located on Chromosome 7
- Delta F 508 is the most common mutation (66-70%) seen in CFTR gene. (Though in India, this mutation is seen in 25-30% cases)
- It is considered most common lethal disorder in caucasians.
Normally : CFTR gene ---> Cl channel --- Cl ions out of cell.
Mutation : Cl remains inside. Thus
- Mucous comes
- Recurrent infections {Most common : Early - Staph ; Late - Pseudomonas (mucoid) Pneumonia , Burkholderia cepacea }
- Pancreatic insufficiency - (in Adutls - due to gall stone , alcohol ) , (In Child - due to Cystic Fibrosis)
- Meconium Ileus
- Azoospermia
Diagnosis :
- Sweat Cl > 60meq/L on 2 occasions (Normally < 40 )
- Nasal electrode potential difference (diagnostic) (Not in India)
- CFTR mutation
- GER - Barium swallow
- Tc based Nuclear Scan
- Lung function test : Increased RV/TLC
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
0 Bronchiolitis
Bronchiolitis
- Inflammatory obstruction of small airways
- Usually < 2years ( mostly at 6 months )
- Viral infection : RSV ( 50% cases)
Risk factors :
- Top feed ( Breast milk is protective as it has IgA against RSV )
- Smoking mothers
Clinical features :
- Viral prodorme
- Wheezing
Note :
- Wheezing in < 2year old - think Bronchiolitis
- Wheezing in Adults - think Asthma.
Chest X-Ray : Hyperinflation.
Drug of choice : Humidified Oxygen. ( Antibiotics are not used )
Q. When is Ribavirin used and when is Palivizumab used in cases of Bronchiolitis?
Answer : When associated with
- Cyanotic congenital heart disease - Use Ribavirin or Palivizumab
- Chronic Lung disease patients (Preterm - Bronchopulmonary hypoplasia) - Use Palivizumab
MedicoNotebook - Founder : DrShiviMudgal , Co-Founder : DrAyushGoel
Subscribe to:
Posts (Atom)
