Monday, April 16, 2018

Croup (laryngotracheobronchitis) : summarized


Croup (laryngotracheobronchitis)

Dr Tayyab Javed

Definition: infection of glottic and subglottic region.most common form of acute upper respiratory obstruction.
Etiology:Mostly viral , caused by parainfluenza virus.
Symptoms:initial upper respiratory tract infection symptoms like rhinorrhea,pharyngitis,cough,fever for 1-3days then barking cough,hoarseness,stridor
provoking factors:agitation,crying
Signs:
hoarse voice,coryza,inflammed pharynx,stridor and signs of respiratory distress in the form of increasing respiratory rate,nasal flaring,suprasternal,infrasternal recession,intercostal retraction.
Investigations:
croup is clinical diagnosis but AP-radiograph neck shows typical subglottic narrowing or steeple sign.
Differential diagnosis:
1.diptheria
2.measles
3.foreign body
4.retropharyngeal or peritonsillar abscess
5.angioedema

Treatment:
1.airway management most important 
   a.suctioning
   b.calm and pacify the child
2.breathing & Circulation
   a.chest rise
   b.respiartory rate monitoring
   c.pulse oximetry 
  d. P.R , pulse volume, CRT , and Blood Pressure
3.hypoxia treatment by oxygen inhalation
4. Nebulization with Epinephrine HCl  (0.5mL/Kg of 1:1000 solution diluted in 3ml Normal Saline )(with maximum dose as :
4Year or less > 2.5ml/dose
More than 4 yr 5ml/dose

If the Patients stridor worsens and pt. deteriorates such that there is CNS depress, bradycardia, hypotension , then endotracheal intubation is the last resort.

Give Oral steroids or I/M Steroid to all children with croup:
reduce inflammation,
Dexamethasone , dose :   0.6mg/kg/dose  Only once.
May be given orally or intramuscularly.

oral prednisolone(deltacortil) is less effective.

contraindictaions to steroids:
children with varicella or tuberculosis infection

discharging criteria:
1.no stridor at rest
2.normal pulse oximetry
3.normal level of consciousness
4.have received oral steroids

Acute epiglottitis : summarized


Acute epiglottitis

Dr Tayyab Javed

Definition:inflammation of supraglottis.potential lethal condition
Etiology is both bacterial and viral.
Symptoms:
progressive and fulminant course of high fever,sore throat,dyspnea,respiratory obstruction and with in hours child looks toxic,difficulty in swallowing,laboured breathing,drooling of saliva
Signs:
hyperextended neck to maintain airway.
drooling.
sitting upright,leaning forward with chin up mouth open
air hunger
restlessness
cyanosis
coma
stridor
Investigations:
   a.laryngoscopy shows large cherry red swollen epiglottis.It should be performed by ENT specialists and in intensive care unit.
   b. lateral radiograph of neck shows thumb sign
   c.cultures of blood,epiglottic surface
   d. CBC, CRP/ESR
Treatment:
medical emergency
1.airway 
    a.suctioning if visible secretions
    b.child should be calmed and pacified
    c.anxiety provoking interventions should be avoided such as intravenous line placement,inspecting oral cavity,placing the child supine,phlebotomy
2.breathing
    a.chest rise
    b.respiratory rate
3. Assess circulation
4.endotracheal/nasotracheal intubation and tracheotomy
       a.this procedure should be performed in intensive care unit.
       b.prior to tracheotomy intubation and general anasthesia should be performed
        to avoid complications.
      c.endotracheal tube should be 0.5-1mm smaller than estimated age.
      d.it should be in placed until oedema and spasm have subsided. Early 
        endotracheal intubation reduces mortality in acute epiglottitis,.
      e.Post intubation protocol should be followed i-e epinephrine and dexamethsone 0.5mg/kg/dose 6-12hour prior to extubation then every 6hour for 6 doses.
5.antibiotics
      cefotaxime 100-200mg/kg/day IV TDS
                              OR
      ceftriaxone  50-100mg/kg/day IV BD
                              OR
      meropenum 60-90mg/kg/day IV TDS.
antibiotics should be continued for 10days.
6.rifampin prophylaxis
      indications..
all household members including
        a.immunocompromised i-e malnourished child,having chronic ailment like tuberculosis,malignancy ,taking steroids.
       b.child younger than 4yrs of age and incompletely immunized
       c.younger than 12mon of age and has not completed primary vaccination.
      dose..
        20mg/kg PO OD 4days max dose 600mg
Note..
 racemic epinephrine and corticosteroids are ineffective.



STATUS EPILEPTICUS in children : summarized

STATUS EPILEPTICUS
Dr Tehreem Fatima
DEFINITION: A seizure lasting more than 30 minutes or recurrent seizures for more than 30 minutes during which the patient does not regain consciousness. However, in clinical practice, seizure lasting >5 min is taken as Status Epilepticus; therefore,  after initial resuscitation, the anti-convulsive treatment is started as soon as possible to prevent neurological damage.
DIANOSIS AND MANAGEMENT: Rapid directed Hx & PE; detailed examination after stabilization; lab studies should proceed concurrently with stabilization (choice based on age &likely etiology).
BASIC AIM: to terminate the seizure, resuscitate patient, treat complications and prevent recurrence; all should go hand in hand.
Primary assessment and resuscitation
Airway: Ensure a patent airway and put the child in the recovery position
If the airway is not patent, use an airway maneuver or airway adjunct
Airway compromise is an indication for intubation

Breathing: Assess breathing – signs of respiratory distress, respiratory rate, SpO2, breathing pattern (central, depressed)
Give high flow O2 via face mask to all children

Circulation: Assess circulation – pulse rate, BP, CRT, CVS exam

Hypertension indicates a possible cause for the convulsion, or more likely is a result of it. Malignant hypertension requires urgent management.

Establish Intravenous/Intraosseous access and request STAFF NURSE on duty to draw following samples from IV line:

Blood tests:
BSL, CBC, U&E (including Na,K,Ca,Mg),Blood Gas and Blood C&S(suspected meningitis)

Signs of shock? Manage shock (IV crystalloid @20ml/kg upto 60ml/kg + cardiac support)

Suspected meningoencephalitis? Give empirical antibiotic coverage (IV ceftriaxone+Vancomycin+Acyclovir)

Signs of raised intracranial pressure? Consider mannitol 0.25 g/kg
Give Paracetamol suppository in case of fever(febrile seizures)
Disability: Conscious level(AVPU), pupil size & reaction, Assess posture (decorticate/decerebrate), SOMI, Anterior fontanelle

Exposure: Assess for Temperature, Petechial/purpuric rash, Signs of trauma
--> As you proceed, keep the attendants ( preferably parents) counseled about what you are suspecting, and what treatment is being given to the patient !!!

Secondary assessment
Emergency treatment of the convulsion
 After ABC resuscitation and exclusion or treatment of hypoglycemia, the priority is to stop the convulsion according to the APLS algorithm.
History taking:
Duration of fitting, Recent trauma, Treatment given, Poison ingestion, History of epilepsy, Last meal, Current febrile illness, Known illnesses
Ongoing management should be continued in PICU:
-->Pass NG tube for gastric decompression, restrict fluids to 60% of maintenance, monitor UOP.
-->Attach cardiac monitor to monitor cardiac rhythm
-->Defer LP in case of cardiovascular instability/reduced conscious level. Consider CT head
-->Identify and treat metabolic causes/poisoning, Check Fundus (papilledema, retinal hemorrhages)
--> Meanwhile, keep the attendants counseled.

First-Line Drugs:
 IV Lorazepam is DOC. If unavailable, use IV Diazepam/Midazolam.
In case, no IV access established:
1-Give Buccal/Nasal Midazolam(first choice)  2-Rectal Diazepam 3. IM midazolam/diazepam
Doses:
IV Diazepam:  0.3-0.4mg/kg
Buccal/Nasal Midazolam: 0.2-0.5 mg/kg (Max 5 mg)
Rectal Diazepam: 0.5mg/kg (Max 10 mg)
IV Midazolam: 0.15 mg/kg
Buccal midazolam; turn child to one side, put syringe (without needle) b/w lower gums & cheek. If resisting/secretions, give half in both nostrils (use thick preparation)
Repeat again after 5 min, if seizure uncontrolled

After 5 min if still uncontrolled:
Second-Line Drug:
IV Phenytoin: Loading Dose:- 15-20mg/kg(in 20 cc N/S @ 1m/kg/min)--- give under continuous ECG & BP monitoring—add in maintenance dose
If still uncontrolled after 10 min of loading:
Third-Line Drugs:
IV Leveteracitam: (If Liver disease/Metabolic disease/coagulopathy/ on chemotherapy) -20-30 mg/kg @ 5 mg/kg/min infusion—add in maintenance dose

If seizure still uncontrolled:
Give maintenance dose of inj Phenytoin.
ADMIT and SHIFT the patient to PICU and start Midazolam infusion if fits are still not controlled:
Inj Midazolam-0.2 mg /kg bolus then infusion @ 1 μg/kg/min, increasing 1
μg/kg/min, every 5- 10 min, till seizures stop, up to a maximum of 30 μg/kg/min, start tapering 24 h after seizure stops @ 1 μg/kg/min, every 3 h. However strict monitoring for respiratory depression should be there

If PICU admission delayed or ventilation facility not available then use:
1. Inj Levetiracetam or inj Valproate if not used earlier
2. Paraldehyde (If available)-0.4 mL/kg per rectal; 50:50 solution in olive oil or N/S

 If seizures still persist on midazolam infusion, consider RSI of anesthesia (seek help from Anesthetist) :
Inj Thiopental: Loading Dose: 4mg/kg bolus followed by 3-5 mg /kg /hr infusion rate (up to 8 mg/kg/hour) to achieve Burst suppression. Start tapering after 24 h seizure free period
Inj Propofol: using 0.5 %, 1% or 2% injection :(age: 1mo- 17 yr) initial: 2.5-4 mg/kg at a rate of 20-40 mg every 10 seconds  until response ; followed by infusion @ 9-15 mg/kg/hr.
___________________________________________________________________

Celiac disease : summarized

Celiac disease

Dr Nimrah Shehzadi

          Immune mediated systemic disorder elicited by gluten and related prolamines in genetically susceptible individuals.
Genetics & pathogenesis :
Concordance in monozygotic twins :100%
Strongest association with HLA haplotypes DQ2 and DQ8.
Clinical presentation:
6 months to 2 yrs of life :
·         Vomiting
·         Chronic diarrhea
·         Abdominal distension
·         Failure to thrive
·         Anorexia
·         Muscle wasting
·         Irritability
·         Occasionaly constipation,rectal prolapse or intussusception
>2 yrs and older children : (can present in adult age also)
·         extraintestinal manifetations :
·         Iron deficiency anemia non responsive to iron therapy
·         Skeletal :Rickets,osteoporosis,arthritis,arthralgia,enamel hypoplasia of teeth
·         Muscular atrophy
·         Neurological : periphery neuropathy,epilepsy,irritability,cerebral occipital calcifications, ataxia
·         Endocrinal :short stature,delayed puberty ,Secondary hyperparathyroidism
·         Dermatological : dermatitis herpetiformis,erythema nodosum,alopecia areata.
·         Respiratory:idiopathic pulmonary hemosiderosis
·         Cancers :Non-hodgkin lymphoma and enteropathy associated T cell lymphoma.
Associated disorders:
·         Autoimmune thyroiditis
·         Type 1 diabetes
·         Addisons's disease
·         Sjogren syndrome
·         Autoimmune cholangitis
·         Autoimmune hepatitis
·         Primary biliary cirrhosis
·         Downs,Williams and turner syndrome
·         Selective IgA deficiency
Diagnosis :
·         Serological testing : Initially get Anti-Tissue transglutaminase 2 IgA antibody levels (anti TTG 2-IgA) levels. If levels are high such that >10 times of normal, then send endomysial antibody levels and HLA .Both of which if positive confirm the diagnosis of Coeliac disease. However, if anti TTG2-IgA levels are low, then get total IgA levels ( which can be low in coeliac patients) and hence TTG2-IgA levels can come out low despite the patient having coeliac disease. In case of  normal IgA, with high TTG2-IgA levels but <10 times the normal , jejunal biopsy is done at multiple sites for making the diagnosis.
Biopsy and antibody levels should always be sent once patient is on gluten diet.
Withdrawal of Gluten diet with improvement of symptoms , or in case symptoms are improved and reintroducing gluten that then causes the symptoms also confirms the diagnosis.
·         Genetic testing:for HLA DQ2& DQ8 detection
·         CBC :anemia
·         Iron studies
·         Duodenal biopsy:villous atrophy and flat mucosa
·         Endoscopy :to visualize bowel for intestinal atrophy
·         S.ca and vit D levels
·         MRI brain: occipital calcifications
Treatment:
·         Lifelong strict adherence to gluten free diet(<50mg /day)
·         Gluten free vitamins and supplements (calcium,folate,iron,vitamin b12,vit D,vitamin K,zinc)
·         Steroids : to control intestinal inflammation in case of coeliac crisis (severe fluid electrolyte imbalances, infection, hypo/hyperglycemia,hypoalbuminemia)
Follow up :
·         Assessment of symptoms
·         Physical examination for growth parameters
·         Adherence to gluten diet
·         TG2 antibody levels: reduced levels indicate gluten free diet adherence
Assessment and investigation for other autoimmune disorders.

Thalassemia : summarized

Thalassemia

Dr Zubair Bhutta

Group of inherited hemoglobinopathies that affect the synthesis of adult hemoglobin tetramer ( Hb A).
Classification:   based upon affecting the synthesis of α or β hemoglobin chains
1) α-thalassemia : major, intermedia or minor    2)β-thalassemia: major, intermedia or minor   
Β-thalassemia major
·         Is a Clinical diagnosis
·         Results in variety of sign and symptoms i-e severe anemia, growth retardation, hepatomegaly , bone marrow expansion and bone deformity.
·         Transfusion therapy is necessary to sustain life.
·         Symptoms starting during early childhood , usually at age of 6 months
·         Newborn’s cord blood should be screened for haemoglobinopathies with hemoglobin electrophoresis.
Antenatal consideration:
·         Preconception genetic counseling is advised for couples at risk or other relatives of a thelassemia child.
·         Once pregnant , chorionic villous sampling (CVS) at 10-11 weeks gestation or Amniocentesis at 15 weeks gestation to detect point mutations or deletions with PCR technology.
Genetics:
·         autosomal recessive pattern of inheritance
Diagnosis:
·         ON HISTORY: pallor, Poor growth , excessive fatigue, cholelitheasis, pathologic fracture, shortness of breath.
·         ON EXAMINATION: Pallor, jaundice, maxillary hyperplasia/frontal bossing due to massive bone marrow expansion and dental malocclusion, splenomegaly, delayed SMR, delayed growth .
·         D/D: iron deficiency anemia, lead toxicity, sideroblastic anemia, other hematological anemia or  hemoglobinopathies
Investigations: CBC Hb is 10-12 g/dl with thelassemia trait and 3-8 g/dl with Thelassemia major before transfusion Hematocrit 28-40% in thalassemia trait and may fall to << 10% in β-thalassemia major Peripheral Blood Picture Microcytosis(MCV <70fl) ,Hypochromia(MCH<<20pg), High %age of target cells, Reticulocytes count is elevated RDW(Red Cell Distribution Width) always normal range 11.5-14.5 in thalassemia trait Mentzer index Mean corpuscular volume / RBC count( interpretation:<<13 thalassemia or >13 suggestive for iron deficiency anemia) DNA analysis  screening tool for children and adults
Hemoglobin electrophoresis:

Hb A(α2β2)
Hb A2(α2δ2)
Hb F(α2ϒ2)
Normal
>90%
2.5%
<2%
Thalassemia minor
Dec.
10%
7%
Thalassemia intermedia
20-40%
Increased
60-80%
Thalassemia major
Absent or Dec.
>3%
>90%

Management and Treatment:
β-Thalassemia Trait: No therapy required
β-Thalassemia minor:1) NO therapy needed unless Hb falls to level that causes symptoms 2)Iron supplementation should not be given unless iron deficiency confirmed with low ferritin
β-Thalassemia major:  
·         Regular Transfusion scheduled 1)to increase post transfusion Hb to 13-14g/dl 2) mean Hb to pre transfusion 9.5-10.5 g/dl
·         Tx required every25-30 days.
·         Monitor serum ferratin levels in regular transfusion child every 6 months.
·         Formula for Required Transfusion: Req. Tx=(13-current Hb)X 3 X Wt.
Iron Chelation Therapy:
·         Initiation of therapy: 1)age >2 yrs and above   2)after 1st yr of transfusion therapy 3)Serum Ferratin level ≥1,000ng/ml
·         Goal of Chelation therapy: Serum ferritin level ≤1000ng/ml
·         3 Available iron chelator agents:  1)Deferoxamine   2)Deferasirox   3)Deferiprone
·         Desferrioxamine: (Inj.Desferal 500mg) Subcutaneous with infusion pump, Dose starting with 30 mg/kg/day over 8-12hours, 5 days a week( gap for 2 days) and may increase to 50 mg/kg /day, starting not less than 5 years side effects: ototoxicity, retinal changes,and bone dysplasia with truncal shortening
·         Deferasirox: (Tab.Asunra 400mg) Oral tablet,Dose starting 20mg/kg/day,regular without gap,may increase max. 40 mg/kg/day, side effects: gastrointestinal damage, potential kidney damage, thrombocytopenia.
·         Deferiprone: oral tablet, starting 50 mg/kg/day in 3 divided doses, regular and may inc. max to 75mg/kg/day, side effects: transient agranulocytosis(required weekly CBC to screen this side effect)
REGULAR FOLLOW UP : should be on S/Ferritin, Echocardiography, TFT’s,RFT’s,LFT’s,Hepatitis B,C screening. Growth monitoring.

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