END EXAM Flashcards
Etiology of Metabolic acidosis
- Loss of base (GIT e.g. diarrhea, or renal e.g. renal tubular acidosis)
- Abnormal acid accumulation, e.g.:
a. Lactate in shock, hypoxia or RD
b. Ketones in DKA
c. Uremic toxins in renal failure
d. Some in-born errors of metabolism
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Treatment of Metabolic acidosis
- Treatment of the cause; eg
correction of shock: 20 ml/kg
Oxygen for hypoxia and RD,
Fluids and insulin for DKA - Alkali therapy with sodium bicarbonate
a. Indicated mainly for cases due to bicarbonate loss rather than those with acid
accumulation, especially those with treatable causes
b. Ensure adequate ventilation and perfusion
c. Give 2 mEq/Kg or correct according to base deficit in blood gases, over 30 minConsider the Na content given
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Investigations of Small VSD
-CXR, ECG: Normal
-ECHO: diagnostic
Treatment of small VSD
- Reassurance (Spontaneous closure is common)
- Prophylaxis against infective endocarditis
Examination of small VSD
a. General: Normal
b. Cardiac: Pansystolic murmur
history of large VSD
- Onset: second week of life
- Feeding difficulties, failure to thrive (FTT) and excessive sweating in infants
- Dyspnea, exercise intolerance in older children
- Recurrent chest infections (Cough)
on examination of large VSD
a. General
= FTT (Heart failure)
= Recurrent chest infections
b. Cardiac
= Inspection and Palpation
o Biventricular enlargement mainly the left ventricle (active precordium)
o Systolic thrill over the Lt parasternal area
= Auscultation
o Accentuated S2 (pulmonary hypertension)
o Murmur
- Timing: Pansystolic
- Character: Harsh
- Maximum intensity: Lt parasternal area (3° and 4” spaces) |
- Selective a propagation: ‘ All over the precordium
INV of VSD
- Chest X ray
= Heart: biventricular enlargement
= Chest: Lung plethora - ECG: biventricular hypertrophy (mainly the left ventricle)
- ECHO will assess
- Position and size of the defect and blood flow across
- Pulmonary pressure
- Cardiac dilation and efficacy of contractility
Treatment of VSD
Treatment
A) Medical
= Infective endocarditis (Prophylaxis and treatment)
= Proper nutrition
=» Management of HF: discuss
= Treatment of chest infections
B) Surgical closure (Surgery or catheter)
= Indications:
a. Large defect with failure of medical treatment
b. Infant 6-12 months old with large VSD and pulmonary hypertension
= Surgery should be delayed in stable child with moderate VSD. “Spontaneous closure”
= Surgery is contraindicated in patients with Eisenmenger syndrome
= Heart-lung transplantation is the only surgical option for Eisenmenger syndrome
= Pulmonary artery banding : to limit increased pulmonary blood flow may be
useful in multiple muscular VSD
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what is Tetralogy of fallot
- Large conoventricular VSD
- Pulmonary stenosis (usually infundibular, may be valvular)
- Overriding aorta
- RVH.
clinical picture of tetralogy of fallot
1.Cyanosis:
- Onset: Usually delayed (3-4 months) due to gradual narrowing of the
infundibulum and closure of the PDA
- May appear in the neonatal period (Severe cases)
- May be absent (Pink Fallot): symptoms appear with exercise only
2.Dyspnea
3.Hypercyanotic spell
4.Squatting.
Examination of TOF
B) Examination
a. General
Failure to thrive
Central cyanosis, appear on the under surface of tongue
Clubbing (1-2 years)
b. Cardiac
= Inspection and Palpation
o Left parasternal pulsation (RVH)
o Systolic thrill over the Lt 2” intercostal space.
= Auscultation
o Single S2 (S2 is slightly louder than normal due to anterior displacement of the aorta)
o Harsh ejection systolic murmur at 2™ left intercostal space due to pulmonary stenosis
Inv of TOF
CBC: increased Hb and increased hematocrit (microcytosis if there is iron deficiency)
2. Chest X ray : Coeur en Sabot (= Boot-shape)
3. ECG: Hypertrophy of the right atrium and right ventricle
4. ECHO
5. Cardiac catheterization: to visualize the coronary and pulmonary arteries
Treatment of TOF
PIPI
A) Medical
#Hypercyanotic spells
#Propranolol
= Iron
* Prostaglandin (PG1): in duct dependent pulmonary circulation( done in severe
cases presenting in neonates)
“ Infective endocarditis (Prophylaxis and Rx)
“ Partial exchange transfusion (using FFP or albumin), When? If hematocrit is > 65-
70%
* Prostaglandin (PG1): in duct dependent pulmonary circulation( done in severe
cases presenting in neonates)
B) Surgical
a. Palliative: Blalock-Taussig (anastomosis between Subclavian artery and the
ipsilateral pulmonary artery). Can be considered as “artificial PDA”
b. Total correction (at 6-9 months): Closure of VSD, infundibular resection and
pulmonary valvotomy
treatment of Hypercyanotic spells
- Positioning (knee-chest position/squatting)
- O» therapy
- IV fluid
NaHCOs: to correct acidosis
Sedation (SC Morphine)
IV B-Adrenergic blockers (Propranolol): to relax the infundibulum
Clinical picture of TGA
1.Deep central cyanosis (Early)
- Onset: Within the 1* few hours or days of life and Not relieved by 100% O2
2.Dyspnea
3.Manifestations of HF
4.Recurrent chest infections (Cough…)
Examination of TGA
B) Examination
a. General
= Failure to thrive (Heart failure) ;
= Central cyanosis Central cyanosis
= Clubbing (>1 year) in survivors
b. Cardiac
Inspection and Palpation: Left parasternal pulsation (RVH)
Auscultation
Accentuated S2
Murmurs: No murmur (if intact interventricular septum).
investiagation of TGA
- CBC: increased Hb and increased hematocrit
- CXR
= Heart: Egg-on-side, narrow pedicle (narrow upper mediastinum)
= Chest: Lung plethora - ECG: Hypertrophy of the RV and right atrium
- ECHO is diagnostic and prognostic
Treatment of TGA
- Prostaglandin: maintains the patency of ductus arteriosus
- Catheter: balloon atrial septostomy : Rashkind procedure ( urgent shunt)
- Surgical repair: Within the first 2-3 weeks of life: Arterial switch
most common oragnism causing infective endocarditis
Streptococcus viridans
Staphylococcus aureus
high risk causes of infective endocarditis
= RHD: Lt sided valves > Rt sided valves > Regurgitation > Stenosis
= CHD: Fallot tetralogy, TGA, VSD, PDA, Coarctation (Not ASD secundum)
“ Prosthetic valve, previous infective endocarditis, surgical shunts, residual defect
Intravenous drug use
history of previous endocarditis and cardiac transplantation
investigations of infective endocarditis
A) Laboratory
Blood culture (Repeated 3 times after proper skin decontamination)
= CBC, ESR, CRP
B) Imaging
= CXR, ECG
= ECHO (Vegetations)
= Transesophageal echocardiography for prosthetic valves
Treatment of infective endocarditis
A. Empirical therapy till culture results
1.Anti-staphylococcal penicillin (methicillin) + Aminoglycoside (gentamycin)
2. Suspected methicillin resistant S.aureus: Vancomycin + gentamycin
B. According to culture
Streptococcus viridans: Penicillin G or ampicillin or ceftriaxone for 4 weeks
and gentamycin for 2 weeks
Staph.aureus: vancomycin for 6 weeks AND gentamycin for 5 days.
Enterococci: penicillin or ampicillin for 4-6 weeks AND gentamicin for 2
weeks.
HACEK: ceftriaxone alone or ampicillin + gentamycin for 4 weeks
Vancomycin if penicillin or ceftriaxone are not tolerated
Amphotericin B for fungal infection.
Patients with prosthetic valves should be treated for 6 weeks according to culture
surgical care
in worsening valve obstruction or regurege or large vegetations or periventricular abscess or heart failure
Obstruction
Regurgitation
Large vegetations
Abscess
Heart failure
investigations of acute congestive heart failure
- chest x-ray
- ECG: to diagnose arrhythmia.
- Echocardiography: chamber size and cardiac anomalies.
- Laboratory: arterial blood gas, CBC, ESR, CRP, ASOT and cardiac markers (Creatine kinase-MB and troponin)
treatment of acute congestive heart failure
- Supportive measures
Rest in semi sitting position
Fluid restriction to 60-70%
Salt restriction
Oxygen therapy: to reduce distress and correct hypoxia
Digoxin therapy to improve myocardial contractility
Diuretic therapy: Furosemide (Lasix) to reduce preload
After load reducing agents as captopril
Grade 2: vasodilators nitroglycerine infusion, IV Dobiutamine, cpap
grade 3: IV dopamine, capropril vasodilator
investigations of Portal Hypertension
- Upper GIT endoscopy: detect esophageal varices
- Abdominal ultrasonography and Doppler:
= Direction of flow within the portal system
= Patency of the portal vein and
= Presence of portosystemic collaterals. - CT angiography and MR venography (demonstrate vessel patency)
- Liver function test
- Investigation for the cause:
Hepatitis markers, autoimmune screening, sweet chloride test, liver biopsy
treatment of variceal hemorrhage
Managment of portal hypertension
1. Emergency therapy for bleeding varices:-
Hospitalization: Anti-shock measures: blood transfusion, intravenous fluids.
Correction of coagulopathy: vitamin k, fresh plasma, platelets transfusion.
Nasogastric tube placement.
H2 receptor blocker (ranitidine) IV to decrease risk of bleeding from gastric
erosion
Third generation cephalosporins.
Vasopressin infusion if bleeding persists.
2. Emergency endoscopy (if hemodynamically stable): and either injection sclerotherapy
or band ligation.
3. Emergency shunt:
Trans jugular intrahepatic Porto-systemic shunt (TIPSS)
Surgical Porto-systemic shunts
investigations of an infant with cholestasis
- complete liver function tests: attention to GGT
- investigation of treatable causes
galactose-1-phosphate-uridyl transferase for galactosemia
TORCH IgM and DNA by PCR
CBC, CRP, blood and urine cultures for neonatal sepsis and UTI
succinyl acetone for tyrosinemia - bile salts
- alpha 1 antitrypsin
- andominal US to choledocal cyst and biliary atresia
- liver biopsy to diagnose:
biliary atresia
Alagille syndrome: paucity of interlobular bile ducts
treatment of cholestais
A L.E is chol
kasai portoenterostomy
replacement therapy
symptomatic treatment
liver translantation for end stage liver disease
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investigations of down syndrome
Laboratory
1. Karyotyping (for patient and his mother) to determine the genetic type of Down syndrome and risk of recurrence.
2. Complete blood count, if leukemia is suspected.
3. Thyroid profile and regular blood glucose checking.
imaging
1. Plain X ray
* Chest for pneumonia.
- Abdomen to exclude GIT anomalies (in neonates’ e.g. duodenal atresia).
2. Echocardiography: to exclude cardiac anomalies.
3. Abdominal ultrasonography: to exclude renal and gastrointestinal anomalies
Regular hearing and vision testing
treatment of down syndrom
rehabilitation and management of complications
1. Diagnosis and management of complications and associated anomalies:
e.g. heart failure, chest infections and hearing aids if needed
1. General measures: special schools for rehabilitation and education.
2. Specific measures: speech therapy and physiotherapy.
investigation for turner syndrome
Laboratory
1. Karyotyping: 45 XO
2. Hormonal study (gonadal failure); increased FSH and LH
3. Thyroid profile (hypothyroidism).
Imaging
1. X ray to determine bone age
2. Echocardiography: may be aortic coarctation
3. Abdominopelvic ultrasound: may be renal anomalies (horse shoe, ectopic
kidney) , uterine anomalies, ovaries (streaks of connective tissues)
Investigations of nephrotic syndrome
A) Laboratory
a. Urine
e Urinalysis: Proteinuria (3+ or 4+)
e Urine protein/Creatinine ratio > 2
e 24 hour urine proteins: > 40 mg/m’/ hr (needs timed urine collection which is
difficult)
e Proteinuria is selective (mainly albumin ,no high molecular weight proteins)
b. Blood
e Serum albumin < 2.5 g/dL
e Serum cholesterol > 200 mg/dL
e Kidney functions: Normal
e Complement (C3 & C,): Normal (No consumption)
== Invasive: Renal biopsy (not routine); indicated in:
=Age at onset <1yror>10yrs
= Gross hematuria
= Persistent hypertension
=Renal impairment
= Hypocomplementemia (J C3 and/or C,)
= Family history of NS
= steroid resistant NS: failure to achieve remission after 4-6 weeks of steroid therapy
treatment of nephrotic syndrome
- Place of Treatment
a. Home management: Most cases with mild to moderate edema
b. Hospital management: 1™ attack or relapses with marked edema or complications - Supportive Management e
a. Diet:
= Salt restriction
* Protein intake: Normal intake or mildly increased
“ Lipid restriction ‘ Hypovolemia
= Fluid restriction in cases of severe hyponatremia ———
b. Edema
= Mild: Salt restriction
= Moderate: Diuretics; Furosemide (1-2 mg/Kg/day)
= Severe: Salt-free albumin + Furosemide (1-2 mg/Kg/day)
c. Infection: Antibiotics (3 generation cephalosporin) - Specific Management
Induction: Prednisone (2 mg/Kg/day)
Divided into 3 doses after meals for compiete 4 weeks
> If the child is steroid-responsive (Maintenance):
o Shift to alternate-day therapy (2 mg/Kg/day Single morning dose)
o With gradual tapering over 3 months
> If the child is steroid resistant :non minimal lesion
o Do renal biopsy
o Shift prednisone to alternate-day therapy with gradual tapering
o Add angiotensin-converting enzyme inhibitors: adjuvant to reduce
proteinuria
o Add other immunosuppressive drugs: cyclosporine or tacrolimus
b. Treatment of Relapses
As the initial therapy, but with shorter induction and longer maintenance
- Specific Management
c. Treatment of frequently relapsing and steroid dependent NS
- Medications used: Cyclophosphamide, Mycophenolate mofetil
investigations of APSGN
- Laboratory
a. Urine (Urinalysis):
Color: Brown, tea or cola-like or smoky
Proteinuria (mild to moderate)
b. Blood
= KFT (1111Urea &1111 creatinine): may be impaired
= Evidence of recent Streptococcal infection (111 ASOT, throat or skin contact)
= v v C3 (returns to normal within 8 weeks)
= Normal C4 - Imaging: Renal U/S
- Invasive: Renal biopsy is rarely indicated
a. Severe renal impairment (Rapidly progressive GN = RPGN)
b. Persistent hematuria or proteinuria > 6-12 months
c. Normal C3
d. Persistent hypocomplementemia > 3 months
Treatment of APSGN
Treatment
1. Place of management
b. Hospitalization: Complicated cases (renal failure, HF, encephalopathy)
2. General measures
. Diet:
= Salt restriction
= Fluid balance
b. Rest: During the oliguric phase
3. Supportive treatment
a. Edema
® Salt restriction, fluid balance, diuretics (Furosemide 1-2 mg/Kg/day)
= Ca channel blockers (Amlodipine 0.6 mg/Kg/day)
4. Treatment of complications
a. Renal failure: Fluid balance, diuretics, dialysis (in severe cases)
b. Heart failure:
> Preload reduction: Diuretics
> Afterload reduction: ACE inhibitors
> Inotropes: Dopamine (digitalis should be avoided)
c. Hypertensive encephalopathy: Antihypertensive (IV Hydralazine or diazoxide)
investigations of pyelonephritis
= Midstream urine collection
= Bladder catheterization
= Suprapubic aspiration (Method of choice in sick newborn and infants)
urine analysis
Pyuria (Pus cells > 5/HPF) is suggestive of UTI
WBC casts is suggestive of pyelonephritis
Urine culture
= Colony count > 100,000 single pathogen is diagnostic
= Any bacterial growth in a suprapubic aspirate or catheter sample is diagnostic
= The presence of more than one organism suggests contamination
Blood:
= CBC: leukocytosis & neutrophilia
= ESR, CRP & blood culture
Imaging
a. Ultrasonography
= Indications:
o First episode of febrile UTI (pyelonephritis)
o Frequently occurring lower UTIs (cystitis)
= Value:
o Diagnosis of obstructive uropathy (hydroureter and hydronephrosis)
o Detection of renal scarring
b. Renal scan (DMSA)
= Detection of renal scarring
= Estimation of renal function (total & split function)
c. Voiding cystourethrogram (VCUG)
“ Indications:
o Febrile UTI with abnormal renal US
o Febrile UTI with abnormal renal scan (DMSA)
= Value:
o Diagnosis of VUR
o Diagnosis of PUV & neurogenic UB
Treatment UTI
Treatment should be started without delay then modified according to the culture result
Cystitis: oral Trimethoprim-sulfamethoxazole for 5 days
pyelonephriris: Ceftriaxone Or Cefotaxime Or Oral 3 gen. cephalosporin for 7-14 days
نشرب مية كتير و نعمل حمام كتير لحد الاخر
ناكل زبادي و نتجنب الامساك
Third generation cephalosporin 1/3 dose
investigations of Iron defeciency anemia
Blood picture:
- Low Hb.
- Microcytic hypochromic anemia
- low MCV and MCHC
- Reticulocytic count is normal. It shows mild increase with therapy.
Blood chemistry:
- Low serum iron < 50mcg % (normal: 90 -150 pg/dl)
- Low serum ferritin |< 10 ng (normal: 30 -150 ng/ml)
- Increased iron binding capacity (normal: 250 -350 ug/dl).
Detect the cause
- clinical history to discover dietary problems.
- systemic clinical examination to rule out other causes of anemia
- Stool analysis: to detect Ankylostoma - blood in stool — bilhariziasis
- Endoscopy might be indicated: to exclude peptic ulcer or chronic H-Pylori infection
treatment of iron deficiency anemia
Treatment of the cause: (Schistosomiasis: Praziquantel) (Ankylostoma: Albendazole)
lron therapy:
Oral iron therapy:
- Ferrous sulfate or gluconate 3-6 mg/kg elemental iron in 3 divided doses/day in between meals
- Iron supplementation should be continued until the Hb is normal and then for a minimum of a further 3 months to replenish the iron stores.
Parenteral iron dextran preparations: In poor compliance or malabsorption.
iron dextran
Diet: Rich in iron (Meat, liver, green vegetables) and vitamin C
Successful ion therapy
day1 Apetitie improved
day2 Bone marrow erthyroid hyperplasia
day3 reticulocytosis
by 1st month elevated hemoglobin 1 gm
4-6 weeks increased stores
investigations of thalasemia
CBC: low Hb, microcytosis, anisocytosis, target cells, poikilocytosis.
Hemoglobin electrophoresis or High Performance Liquid Chromatography (HPLC):
o Inthe affected child: Hb F is markedly elevated (10-90%) with reduced Hb A.
o Parents: increased of Hb A2 > 3.5% (normal: 3%)
treatment of thalassemia
Supportive treatment
Restrict Iron in diet. Folic acid 1 mg/day. Hepatitis B vaccine. Calcium and vitamin D
Lifelong Repeated packed RBCs transfusions:
10 -15 ml /kg every month to keep Hb level >10 gm/dl (hypertransfusion).
= Iron chelating agents: (should be started after 10 times blood transfusion)
Deferroxamine (Desferal): 20 — 40 mg/kg by S.C. Pump over 10 hours, Sdays/week.
Deferiprone: Oral chelating agent (25mg /kg/dose, three doses per day)
Deferasirox: oral chelating agent (20-40 mg/ kg/ day)
= Splenectomy:
Indications: Huge splenomegaly or hypersplenism after age of 4
Splenectomy care:
o Before splenectomy: Vaccination (pneumococci- meningococci- H. influenza)
o After splenectomy: lifelong daily oral penicillin prophylaxis is advised or Long acting penicillin prophylaxis
=” Bone marrow transplantation: (best below 3 years)
Induction of fetal hemoglobin synthesis
hydroxyurea can stimulate Hb F production
” Treatment of complications:
- Gall bladder stone: cholecystectomy
- Diabetes: insulin therapy
- Short stature: growth hormone
investigations of sickle cell anemia
To prove anemia: CBC shows Low hemoglobin
To prove hemolysis:
e Blood film: reticulocytosis (raised reticulocyte) & abnormal appearance of
the red cells = sickled shaped)
e Blood chemistry: elevated serum indirect bilirubin, serum iron, serum ferritin, decreased iron binding capacity, decreased the haptoglobin.
e Increased urinary urobilinogen
Hb electrophoresis: Hb S is present (> 50%) no HbA
Parents: HbS 20-40% HbA60-—80%
Treatment of sicke shaped
A A water oxygen blood
Treatment of acute crises — Painful crises should be treated with:
1. Oral or intravenous analgesia according to need (may require opiates);
Good hydration (oral or intyavenous as required);
Infection should be treated wii!) antibiotics;
Oxygen therapy when oxygen saturation is reduced;
Exchange transfusion is indicated for acute chest syndrome, stroke and priapism.
Treatment of chronic problems —
1. Hydroxycarbamide, a drug which increases their HbF production
2. Bone marrow transplant,
Investigations of Acute hemolytic anemia
G6PD
Investigations:
= During the attack:
- CBC: Anemia ( normocytic normochromic)
- Blood film shows Heinz bodies
- Reticulocytosis in blood film (hemolysis)
- Chemistry: indirect hyperbilirubinemia -hemoglobinemia — hemoglobinuria
treatment of G6PD
= Urgent packed red cell transfusion (10 ml/kg) is lifesaving in very severe hemolysis
= Prevention of subsequent attacks: A list containing oxidants materials (drugs, chemicals and food) should be given to parents.
investigations of aplastic anemia
Blood picture: Pancytopenia
Bone marrow examination: hypocellular bone marrow
investigations of faconi anemia
= CBC will show pancytopenia
= Bone marrow aspirate and biopsy will show hypocellular bone marrow
= Chromosomal breakage study : increased
chromosomal breaks of peripheral blood lymphocytes to identify affected family members or for prenatal diagnosis.
= Skeletal survey might show absent thumb or radius
= abdominal U/S might show renal anomalies
treatment of fanconi anemia
- Supportive therapy :( Transfusion for controlling anemia — antibiotics for infection — coagulation factors for bleeding)
- Bone marrow transplantation from normal, HLA- matched donor
inv Acquired aplastic anemia
- CBCwill show pancytopenia
- Bone marrow aspirate and biopsy will show hypocellular bone marrow
- Chromosomal breakage study (spontaneous and induced) will be normal
treatment of Acquired aplastic anemia
Treatment:
Supportive therapy (controlling anemia — infection — bleeding)
- Immunosuppressive therapy (Anti-thymocyte globulin (ATG) and/ or cyclosporine)
e Moderate and Severe cases:
- Bone marrow transplantation (BMT) is the treatment of choice from HLA matched sib.
investigation of acute leukemia
- CBC: anemia -thrombocytopenia - WBC (increased, decreased or normal)
- Peripheral blood film may show blast cells
- Bone marrow examination: is essential to confirm the diagnosis (blast cells)
- Lumbar punctureto identify disease of CSF
- Chest X-ray to identify mediastinal mass characteristic of t-cell disease
treatment of Acute lymphoblast leukemia
i. Supportive treatment:
= Blood & platelet transfusion
= Treating the infection
= Hydration
Chemotherapy:
Combination of chemotherapy
#Induction of remission: vincristine - asparagenase - —prednisone — cytarabine
= Intrathecal: methotrexate, hydrocortisone- cytarabine
#Systemic continuation therapy: 2- 3 years- {6 mercaptopurine — methotrexate}
. Bone marrow transplantation in relapsing cases
INV of ITP
CBC:
- Thrombocytopenia, usually < 20,000 / mm? (Normal: 150,000 - 400.000mm?)
- May be low Hb due to blood loss.
- Normal WBCs count with relative lymphocytosis.
¢ Bone marrow examination:
- Megakaryocytes are normal or increased in number with defective budding
¢ Anti-platelet antibodies: are found
treatment of ITP
in moderate cases
Prednisone: Dose 2 mg/kg/d to inhibit antibody synthesis and phagocytic activity.
For 2 weeks
Intravenous immunoglobulin (IVIG): dose 1 gm/kg/day, Duration for 2 days.
in severe cases: I.V. methyl prednisolone 20 mg /kg / day for 5 days
IVIG
platelet transfusion after starting steroid
In chronic cases: (>12 months):
- Careful evaluation for associated disorders
(E.g. SLE: frequent screening of autoantibodies).
: - Prednisone & IVIG.
- OR Splenectomy (75% curative).
- OR Immunosuppressive therapy (e.g. Cyclosporine).
investigations of Hemophilia A
- Phase | coagulation defect (prolonged PTT)
- Specific factor Vill assay (reduced below normal) Normal > 60% & Carrier 30 -60% (female).
- mild hemophilia 5-30%: (bleeding with trauma or surgery)
- moderate hemophilia 1-5%: (bleeding with minor trauma)
- severe hemophilia < 1%: (spontaneous joint bleeding)
treatment of Hemophilia A
- Cold compresses minimize bleeding in mild cases
- Replacement (essential in moderate or severe cases)
= I.V. infusion of cryoprecipitate (Plasma concentrate of factor VIII) (Dose:
25 — 50 Unit/ kg every 12 hours). This dose applies to hemarthrosis.
= I.V infusion of Purified plasma derived factor VIII concentrate
= I.V infusion of Recombinant factor 8.
#Prophylactic F VIII in severe hemophilia (2 times per week)
- Desmopressin:In mild hemophilia A it increases endogenous release of FVIII
(ineffective in hemophilia B). - Physiotherapy specially after immobilization to prevent muscle wasting and joint contracture
investigations of Congenital Hypertrophic Pyloric Stenosis
Laboratory
1. Hypochloremic metabolic alkalosis
2. Hypokalemia
imaging
1. Abdominal US: confirm the diagnosis.
2. Barium meal: shows Narrow pyloric canal “String sign”]
treatment of congenital hypertrophic pyloric stenosis
1- Correction of fluid & electrolyte disturbance
2- Pyloromyotomy (Ramstedt)
investigations of celiac disease
- Serology: Tissue transglutaminase & anti-endomysial antibodies
- Endoscopy: Jejunal biopsy shows flat mucosa with villous atrophy
TTT of celiac disease
- Gluten-free diet for life is essential for reversal of growth failure, improvement of symptoms and reduction of the risk of intestinal malignancy
- Oat is allowed
investigations of infant of diabetic mother
- Laboratory: Hematocrit, blood glucose, calcium and magnesium.
- Imaging: Chest X ray, Echocardiography, abdominal ultrasound.
Managment of IDM
- Delivery should be in a hospital where the newborn can be carefully monitored
- After birth: monitoring for metabolic disturbances especially hypoglycemia
a. Blood glucose should be done within the first hour of delivery, then at least
hourly for the first 6-8 hours postnatal
b. Asymptomatic infants if clinically well should start early feeding.
b. Breastfeeding should be started as soon as the infant is stable.
d. If the infant is unable to tolerate oral feeding , glucose should be given by
peripheral IV infusion at rate of 6-8 mg/kg/minute - Careful monitoring of complications > NICU admission for, unstable baby
Echo, abdominal US, CT, MRI
investigations of Hypoxic ischemic encephalopathy 1182
nvestigations
1. Prenatal :Fetal biophysical profile - Doppler U/S( assess the cord blood flow)
2. Perinatal: blood gas analysis (acidosis) and saturation(hypoxia)
3. Postnatal:
Laboratory
a. Monitoring of ABG — saturation — blood glucose — serum electrolytes — temperature — serum calcium].
b. Renal function —bleeding profile.
c. Liver function tests
Imaging
a. Cranial ultrasound
b. Electroencephalogram (EEG, cerebral function monitor ) to detect :abnormal background activity , early encephalopathy or seizures
c. MRI: at 7-10+ 14 days: bilateral abnormalities in basal ganglia and thalamus
d. Echo (cardiac dysfunction and persistent pulmonary hypertension may occur)
treatment of Hypoxic ischemic encephalopathy
Therapeutic hypothermia (cooling to a rectal temperature of 33-34 C°) for 72 hours should started within 6 hours of birth
Treatment of seizures by anticonvulsants : discuss
Treatment of hypotension by volume and inotropic support
Treatment of hypoglycemia(glucose 10%) and hypocalcemia (Ca gluconate IV)
treatment of Isoimmune Hemolytic disease due to Rh incompatibility
- Intrauterine intra-umbilical blood transfusion
- Induction of labor: between 33-34 weeks, with marked hemolysis.
- Phototherapy, exchange transfusion if needed following delivery.
- Elimination of foreign red cell antigens by giving Human anti-D globulin to the
mother
a. Timing: It is administered at 28-32 weeks gestation and
b. Again within 72 hours of delivery or abortion.
c. Dose: IM injection of 1 ml to neutralize fetal protein
investigations of unconjugated hyperbilirubinemia
CBC w 2 hemolytic w septicemia w thyroid
1. Blood picture
HB % and reticulocytosis>hemolysis.
WEC count and I/T ratio> septicemia.
2. To exclude hemolytic disease
Blood grouping (ABO and Rh) for baby and mother.
Coombs’test (to detect maternal antibodies that coat the baby’s RBCs).
3. To exclude hemolytic anemia
Enzyme assay: G6PD deficiency.
RBCs morphology and osmotic fragility test: spherocytosis.
4. If septicemia is suspected: C reactive protein- Erythrocyte sedimentation ratecultures.
5. Thyroid profile if not done in neonatal screening program.
treatment of unconjugated hyperbilirubinemia
. Monitoring
1. Screen serum bilirubin level (TSB) or transcutaneous bilirubin (TCB) of any neonates before discharge
2. Plot total serum bilirubin on an hour-specific nomogram that identifies risk zones
Phototherapy indicated in Bilirubin level higher than 15 mg/dl in full term newborn
Exchange transfusion
Indications of phototherapy
- Bilirubin level higher than 15 mg/dl in full term newborn or according to hour specific nomogram
- Before, after or in between exchange transfusions (complementary treatment)
- Prophylactic in very low birth weight infants, hemolytic disease of the newborn
indications of exchange transfusion
- Hemolytic disease of the newborn >early exchange transfusion
a. Cord bilirubin 5 mg/dl or more, and or
b. Cord Hb 10 gram % or less.
c. Bilirubin level is rising over 1 mg/dl /hour in spite of phototherapy.
d. The baby has a risk of kernicterus - Bilirubin level more than 25 mg/dl in spite of phototherapy.
investigations of respiratory distress
- Chest X ray
* Diffuse reticulogranular pattern
* Air bronchogram (air in the major bronchi appears in contrast with the white background of collapsed alveoli).
* Complete opacification of both lung fields (white lungs) in severe conditions. - Blood gases hypoxia, hypercapnia and acidosis
- Electrolytes , calcium and glucose
- Other investigations : blood picture , C reactive protein and ESR
treatment of RDS
Treatment
1. Basic life support measures
a. Thermoregulation
b. Correction of acidosis, fluid and electrolyte balance.
2. Prophylactic antibiotics until cultures appear
3. Monitoring
a. Heart rate, respiratory rate, arterial blood pressure and temperature.
b. Oxygen saturation by transcutaneous O2 pulse oximeter.
c. Arterial blood gases (Pa02, PaCO2, pH and base deficit).
d. Hemoglobin, electrolytes, calcium, glucose and albumin.
4. Correction of hypoxemia
a. In the delivery room : prophylactic Nasal continuous positive air way pressure (CPAP)
b. NICU management : Nasal CPAP or Intermittent positive pressure ventilation (IPPV) or high frequency ventilation in cases of risk of air leak
. Surfactant replacement therapy
a. Natural or synthetic surfactant can be given via an endotracheal tube
investigations of transient tachypnea of newborn
X-ray
a. Prominent pulmonary perihilar markings (dilated lung lymphatics).
b. Fluid in the interlobar fissures and minimal pleural effusion
Treatment of the transient tachypnea in newborn
- Basic life support measures
a. Thermoregulation
b. Correction of acidosis, fluid and electrolyte balance. - Prophylactic antibiotics until cultures appear
- Monitoring
a. Heart rate, respiratory rate, arterial blood pressure and temperature.
b. Oxygen saturation by transcutaneous O2 pulse oximeter.
c. Arterial blood gases (Pa02, PaCO2, pH and base deficit).
d. Hemoglobin, electrolytes, calcium, glucose and albumin. - Supplemental oxygen to maintain adequate arterial oxygen saturation.
- IV fluids, then tube feeding until respiratory rate has decreased enough to allow oral feeding
Managment of bilirubin induced neurological dysfunction ( kernicterus)
- Suspected BIND : immediate exchange transfusion , preceded by phototherapy till
blood - Established cases: Immediate exchange transfusion to minimize further brain
damage - Prior to discharge :Auditory evoked potential> auditory brainstem response (ABR)
audiometry
investigations for congenital infections
> Virus detection by cultures or PCR
Urine, blood, throat, nose (rubella)
Urine, saliva, blood (CMV)
Skin scrapping, serum, CSF, urine, throat, oro and nasopharynx, conjunctiva (HSV)
Antibodies IgM for toxoplasmosis and rubella
managment of conginetal infections
- Intensive supportive care when needed: Fluid balance, Oxygen, thermoregulation,
nutrition - Specific antiviral and antimicrobial agents, e.g. ganciclovir for CMV, acyclovir for
HSV. - Hepatitis B vaccine for all newborns
investigations of neonatal sepsis
- Complete blood count with differential
= WBCs <5,000 cells/mm? and ANC (Absolute Neutrophilic Count )<1,000 are most
predictive of infection
= WBCs >20,000 and Immature/Total neutrophils (I/T ratio) >0.3 are suggestive of infection
= Thrombocytopenia, PT, PTT, INR in severely ill neonates
- CRP: serial measures
- Cultures
Blood culture and other cultures (urine culture obtained by catheterization or
suprapubic aspiration, endotracheal tube and central line cultures)
» Lumber puncture for suspected meningitis: CSF analysis (cells, protein, glucose) & CSF culture
- Others
= Hyperglycemia, metabolic acidosis may be present
= Chest X ray and arterial blood gas for suspected pneumonia
treatment of Neonatal sepsis
- Antibiotics
Type: Empirically (first line): Ampicillin + Aminoglycoside (as gentamycin), then
according to results of the culture.
Duration: 14 days in proven sepsis, 3 weeks in meningitis and 6 weeks in septic
arthritis - Supportive
= Respiratory support including mechanical ventilation for pneumonia
= Volume and pressor support for hypotension and poor perfusion
= Anticonvulsants for seizures
investigations of rickets
A) Laboratory
=Serum calcium: Usually normal but may be decreased in cases with: bone Ca depletion, parathyroid exhaustion or with the use of high dose of vitamin D
=Serum phosphorus: ↓↓ (N = 4.5-6.5 mg/dl)
“Serum alkaline phosphatase: 11 (Earliest manifestation)
decreased vit D and increased Parathormone
B) Imaging (Radiological improvement start to occur after 2 wks of vitamin D therapy)
metaphysis broadning, cupping and farying
during healing: dense concave white line of calcification
Diaphysis ↓↓ bone denisty and fractures
epiphysis: ↑↑ joint space
then improved bone denisty
managment of rickets
Prevention
1. Nutritional education: Value of breastfeeding, proper weaning…
= Diet rich in vitamin D: Oily fish (salmon, sardines), egg yolk, liver, butter, fortified milk
= Avoid rachitogenic diet
2. Vitamin D supplementation [40 IU = 1 yg]
= Full-term: 400 IU/day (since birth)
= Preterm: 800 IU/day (as early as the 1* month)
3. Sun exposure (UVR)
Treatment
A) Vitamin D therapy
a. Oral therapy
“Dose
- Vitamin D3: 3000-4000-5000 IU/day for 2-4 weeks
b. Parenteral therapy
“Dose: Vitamin D3: 600.000 IU (Shock therapy)
= Duration: Single IM dose
= Advantages
- More rapid
- No need for parents compliance
= Disadvantages (Side effects)
- Tetany
- Hypervitaminosis D
B) Treatment of complications
a. Tetany: IV Ca gluconate 10% “1 ml/Kg” (Slowly while monitoring heart rate,
why?)
Monitoring of HR is essential
Stop if bradycardia occurs
b. Deformities and Fractures: Orthopedic care (After complete bone healing)
c. Infections: proper antibiotics
d. lron deficiency anemia: Iron (6 mg/Kg/day)
investigations of marasmus
Investigations for comlications
CBC: Anemia, leukocytosis (Infection)
Other markers of infections: CRP, ESR, stool analysis…
Serum proteins: Not markedly J (DD: kwashiorkor)
Electrolytes, blood glucose
Investigations of non-nutritional marasmus
Echocardiography
liver function test
renal function test
X-ray
investigations of kwashiorker
Laboratory
= CBC: Anemia, leukocytosis (Infection)
= Serum albumin: ↓↓ (N = 3.5-5.5 g/dl)
= Serum globulins: ↓↓ a and B-globulins (↑↑ y-globulins due to infections)
= Electrolytes:
o Hyponatremia. It is dilutional (N: 135- 145 mEq/L)
Total sodium increased (aldosterone), but serum sodium decreased (water retention)
o Hypokalemia an hypoglycemia
o Hypomagnesaemia
managment of marasmus
Prevention of protein energy malnutrition
1. General measures: Environmental sanitation, Socioeconomic development Adequate food supply, prevention of infection
2. Proper antenatal care
3. Promotion of breastfeeding: It is the simplest, cheapest and most effective method
4. Nutritional education: Value of breastfeeding, proper weaning…
5. Regular assessment of child health and growth pattern: Growth curves
6. Regular assessment of nutritional state: Early manifestations of nutritional disorders
7. Proper management of childhood infections: Especially GE…
treatment:
1. hospitalization in
=kwashiorker or
=Marasmic kwashiorkor
=3” degree marasmus
=Complications
- managment plan
Infection (GE, pneumonia…): Proper antibiotics
Shock: Shock therapy (Immediate IV fluid: Lactated ringer’s 20 ml/Kg)
- Dehydration: IV fluid therapy (Deficit therapy)
- Electrolyte disturbances: should be corrected
- Anemia: Packed RBCs
- Hypoglycemia (IV glucose),
- hypothermia: adequate clothing or radiant warmer
Nutritional Managment
↑↑ calories 150 Kcal/Kg/day (start with 75 and increase amount and concentration according to the child tolerance
rate of increase (5-10 kcal/kg/day) oraly or by nasogastric tube
According to the age:
= Milk-fed: Milk (Lactose-free may be used initially) then standard formula
weaned infants: balanced diet (50 C, 35f, 15p)
Vitamins
Vitamins
= Vitamin A
- <6 months: 50.000 IU/day
- 6-12 months: 100.000 IU/day
- > 12 months: 200.000 IU/day
_ = Vitamin B, C, D, E, Folic acid
Minerals
® Iron (4-6 mg/kg/day) as zinc
mangment of kwashiorker
Prevention of protein energy malnutrition
1. General measures: Environmental sanitation, Socioeconomic development Adequate food supply, prevention of infection
2. Proper antenatal care
3. Promotion of breastfeeding: It is the simplest, cheapest and most effective method
4. Nutritional education: Value of breastfeeding, proper weaning…
5. Regular assessment of child health and growth pattern: Growth curves
6. Regular assessment of nutritional state: Early manifestations of nutritional disorders
7. Proper management of childhood infections: Especially GE…
treatment:
1. hospitalization in
=kwashiorker or
=Marasmic kwashiorkor
=3” degree marasmus
=Complications
- managment plan
Infection (GE, pneumonia…): Proper antibiotics
Shock: Shock therapy (Immediate IV fluid: Lactated ringer’s 20 ml/Kg)
- Dehydration: IV fluid therapy (Deficit therapy)
- Electrolyte disturbances: should be corrected
- Anemia: Packed RBCs
- Hypoglycemia (IV glucose),
- hypothermia: adequate clothing or radiant warmer
Nutritional Managment
↑↑ proteins 4-6 gm/Kg/day (start with 1 gm and increase amount and concentration according to the child tolerance
rate of increase oraly or by nasogastric tube
According to the age:
= Milk-fed: Milk (Lactose-free may be used initially) then standard formula
weaned infants: high protein diet (egg, meat, chicken, beans)
Vegetables and fruits
Vitamins
= Vitamin A
- <6 months: 50.000 IU/day
- 6-12 months: 100.000 IU/day
- > 12 months: 200.000 IU/day
_ = Vitamin B, C, D, E, Folic acid
Minerals
® Iron (4-6 mg/kg/day) as zinc
investigations of Acute post infectious polyneuropathy: Guillain Barré syndrome
- Diagnosis is mainly clinical: Acute ascending symmitrical paralysis with hypotonia
1. CSF analysis (2 weeks after the onset of paralysis)
Increased protein but with normal cell count and glucose (Cytoalbuminous dissociation)
2. EMG electromyography
3. Decreased nerve conduction velocity ( diagnostic)
treatment of Guillian barre syndrome
- ICU and mechanical ventilation for cases with respiratory muscles paralysis or
bulbar paralysis (lifesaving). - IV gamma globulin in all patients for 5 successive days. The best choice
- Plasmapharesis.
- Physiotherapy should start from the second week of illness.
investigation of Duchenne muscular dystrophy
- Serum CPK (creatine kinase) .Marked elevation even at birth (can be used as
screening test). - Electromyography
- DNA studies: deletion on Dystrophin gene
- Muscle biopsy: diagnostic (absent Dystrophin).
treatment of duchenne muscular dystrophy
- Nutritional support and physiotherapy.
2 . Night splints to prevent contractures - Scoliosis is managed with a truncal brace - Treatment of chest infections and respiratory aids as CPAP.
- Corticosteroids: to preserve mobility and prevent scoliosis (its action may be
inhibition of muscle proteolysis, anti-inflammatory effect.). - Gene therapy is now available.
investigations of spastic CP
- CT brain - MRI brain
May determine the location and extent of structural lesion.
May show associated malformations or brain atrophy. - EGG
- TORCH screening to define the cause
- Auditory assessment and VEP:(visual evoked potential)
treatment of Spastic CP
Rehabilitation
2. Physiotherapy.
2. Positioning and splints to prevent contracture.
3. Associated complications and neurological problems as
a. Epilepsy: antiepileptic drugs
b. Orthopedic management: Tendon releases in contracture
c. Treatment of chest infection
d. Special feeding program
INV of acute bacterial meningitis
2.24
CSF examination
1. Differentiating bacterial meningitis from tuberculous and viral meningitis from table
2. Culture and sensitivity tests are essential: if bacterial infection is suspected
3. Detection of antigens (PCR) and antibodies (ELIZA) of viral infection
4. Ziehl-Nielsen staining of the CSF if TB meningitis is suspected
other investigations
1. CBC: marked leukocytosis with bandemia
2. Blood culture
3. Kidney functions test and electrolytes
4. Coagulation screen if DIC is suspected
5. If TB is suspected: chest X ray, tuberculin rest.
6. CT with contrast to detect meningeal enhancement
7. MRI brain for better visualization of cerebral infarcts.
Treatment of Acute Bacterial meningitis
Treatment:
A. supportive: ABC
CCI
=Care of coma
=Anticonvulsants: diazepam and phenobarbitone.
= IV fluid if meningitis is complicated by shock (otherwise fluid is restricted to minimize cerebral edema
Assisted ventilation if respiratory failure occurs.
B. Specific treatment
a. Antibiotics: IV for at least 10- 14 days (in neonates 3 weeks).
Neonates and infants below 2 months:-
Third generation cephalosporins e.g. Cefotaxime 200 mg kg/day plus ampicillin 100mg/kg/day.
Infants and children above 2_months:-
Third generation cephalosporin e.g. Cefotaxime 200mg/kg/day or ceftriaxone plus vancomycin
Dexamethasone in H influenza infection to decrease incidence of gliosis and hearing loss
C. Follow up to detect late complications e.g. Epilepsy and mental retardation
B26
treatment of acute tonsilitis
Oral penicillin V (50.000 unit/kg/day) or Amoxicillin 50 mg/kg/day
In penicillin allergic patients oral erythromycin (50 mg/kg/day) for 7-10 days
investigations of acute tonsilitis
Throat swab culture and rapid antigen-detection tests (RADs)
investigation of otitis media
ear examination by otoscope reveals congested bulging eardrum
treatment of otitis media
= An oral broad-spectrum antibiotic (Amoxicillin-clavulanate) 80-90 mg/kg/day in 2divided doses) to cover both gram-positive and gram-negative organisms is prescribed for 7-10 days.
= If penicillin allergy, use third generation cephalosporin.
inv of pneumonia
- Chest x ray:
a. Confirm the diagnosis
b. Complications: effusion — empyema — pneumatoceles — lung abscess. - Blood gas: in severe cases lowered 02 tension and raised C02
- Complete blood count, ESR, CRP: DD/ bacterial and viral causes
- Culture and sensitivity test: morning nasopharyngeal aspirate or sputum culture.
Investigations of Acute bronchiloitis
- Chest X-ray: hyperinflation of the lungs with focal atelectasis.
- Blood gas analysis; hypoxia- CO2 retention.
- RSV antigen detection from nasopharyngeal secretions.
TTT of Acute bronchiloitis
- Infants with minimal or mild respiratory distress (at home)
a) Close observation: Increasing distress is an indication for hospitalization.
c) Careful feeding to avoid aspiration. - Infants with moderate to severe respiratory distress (at hospital)
a. Oxygen therapy to correct hypoxemia.
b. I.V maintenance fluid therapy to prevent dehydration.
c. Nebulized salbutamol
d. No Corticosteroids or antiviral agents. (are not beneficial)
e. Mechanical ventilation used in those with severe respiratory failure
TTT of pneumonia
A. Hospital management (7-10 days):
1. Indications
= Severe pneumonia (severe RD) or complicated pneumonia
= Small infants (Less than 6 months)
2. Supportive measures:
- Humidified oxygen - IV fluid (NPO) — suction — mechanical ventilation
3. Specific treatment:
Broad spectrum combined parenteral antibiotics to cover G+/-
(Ampicillin 50- 100 mg/kg/day + gentamycin 4-6 mg/kg/day).
4. Treatment of complication:
Drainage of empyema.
Mechanical ventilation (in respiratory failure)
B. Home management for most cases:
1. In Older children with mild pneumonia without distress.
2. Oral or better intramuscular antibiotics.
3. Amoxicillin 50 mg/kg/day or better broader-spectrum antibiotics such as amoxicillin-clavulanic acid for 7-10 days.
investigation of cystic fibrosis
Sweat test, to confirm that the concentration of chloride in sweat is elevated.
Sweat chloride test
Sweating is stimulated by applying a low-voltage current to pilocarpine applied to the skin.
= The sweat is collected into a special capillary tube or absorbed onto a weighed piece of filter paper.
.
< 30 mmol/L = negative result
30-59 mmol/L = borderline
> 60 mmol/L = consistent with CF.
Confirmation of diagnosis can be made by testing for gene abnormalities in the CFTR protein where AF508 is the commonest gene mutation.
Managment of Cystic fibrosis
The aim of therapy is to prevent progression of the lung disease and to maintain adequate nutrition and growth.
A. Respiratory management
B- Nutritional , GIT and hepatic management
C-Psychological management
A. Respiratory management
- Anti-inflammatory drugs
Anti-inflammatory drugs for CF corticosteroids and nonsteroidal anti-inflammatory drugs)
2. Antibiotics
Prophylactic oral antibiotics (flucloxacillin)
Additional oral antibiotics
Vigorous parenteral antibiotics for 14 days
Inhaled antipseudomonal antibiotics daily for Chronic Pseudomonas infection
3. Mucolytics
Nebulized DNase, hypertonic saline, or N-acetylcysteine may be helpful to decrease the viscosity of sputum and to increase its clearance.
- Physiotherapy:
Children should have physiotherapy at least twice a day, aiming to clear the airways of secretions. - Bronchodilators
As reversible airway obstruction occurs in many children with CF. - Lung transplant
Bilateral sequential lung transplantation for end stage CF lung disease
B. Nutritional, GIT and hepatic management
= Nutritional status should be assessed regularly.
= A high-calorie diet is essential.
= Most patients require fat-soluble vitamin supplements.
= Pancreatic insufficiency is treated with oral enteric-coated pancreatic enzymes replacement therapy
= Oral bile acid therapy aimed at improving biliary secretion for cystic fibrosis associated liver disease.
C. psychological and emotional support for CF patients
and their families.
treatment of measles
Management
Prophylaxis
1. Active immunization : see immunization
2. Passive immunization with gamma globulin (0.25 ml/kg I.M) 2-5 days after exposure
Treatment
1. Symptomatic: antipyretics - decongestants.
In immunocompromised patients, the antiviral drug ribavirin maybe used.
Antibiotics for complications as otitis media and pneumonia.
Large doses of gamma globulin in encephalitis to reduce complications.
Oral vitamin A (400,000 IU) modulates the immune response (reduces morbidity).
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Investigations of scarlet fever
- Throat culture: group a beta hemolytic streptococcus.
- Serologic tests: A significant rise of Antistreptolysin O titer (ASO titer).
- CBC: Leukocytosis(PNL 10,000 — 20,000 /mm*) and anemia
- Elevated ESR and CRP
treatment of scarlet fever
- Antibiotic therapy:
= Oral penicillin V: 400. 000 IU/dose 4 times/day /10 days
= Procaine penicillin: injection for 10 days
= Erythromycin (50 mg/kg/day)in penicillin sensitive patient for 10 days - Antipyretics for the high fever.
treatment of chicken pox varicella
Prophylaxis:
1. Alive attenuated varicella vaccine
2. Post exposure; VZIG: [varicella zoster immunoglobulin] early in the incubation
Treatment:
1. Antipruritic agents (local and systemic)
2. Antipyretics.
Aspirin should not be used as it increases the risk of Reye
syndrome (acute encephalitis with fatty infiltration of the liver)
3. Antibiotics are indicated only if secondary bacterial infections occur.
- Antiviral drugs: Acyclovir I.V in ( immunocompromised — encephalitis— pneumonia)
treatment of Juvenile idiopathic arthritis
- Drugs:
Non-steroidal anti-inflammatory drug (NSAIDs) e.g. Ibuprofen — diclofenac (oligoarticular)
b. Disease-modifying anti-rheumatic drugs (DMARDs): Methotrexate (1* line drug in polyarticular type) - Leflunomide.
C. Biological agents: Anti-tumor necrosis factor-a (Etanercept) 2” line in polyarticular type, Anti-interleukin-1 (Anakinra) and anti-interleukin-6 (Tocilizumab) in systemic type following steroid therapy.
D. Steroids: intra-articular steroids (oligoarticular type), systemic steroids (systemic type), topical steroids (uveitis). - Physiotherapy: to strengthen muscles and increase the range ofjoint motion.
- Periodic slit-lamp ophthalmologic examinations: to monitor for asymptomatic uveitis
- Family and psychological support.
Investigations of SLE
Laboratory:
1. CBC, CRP, ESR,
2. kidney function tests
3. Liver function tests
4. Serum albumin
5. Urine analysis.
6. Anti-nuclear antibodies (ANA), anti dsDNA and anti-Smith antibodies.
7. Hypocomplementemia (Reduced C3, C4, CH50).
8. Anticardiolipin IgG or IgM.
9. Lupus anticoagulants.
10. Coombs test
Radiological:
1. Echocardiogram, chest X ray, pelvi abdominal ultrasound.
2. Brain MRI in cases with neuropsychiatric manifestations.
Renal biopsy: For staging of lupus nephritis
treatment of SLE
- Sunscreen and avoidance of prolonged direct sun exposure may help control
disease by sunblock creams, proper nutrition, vitamin D - Hydroxychloroquine: help to control skin and joint manifestation and improve
outcome in all SLE patients. - Corticosteroids (oral and IV): the main stay of treatment in SLE.
Oral 1-2mg/kg/day or pulse methylprednisone - Steroid-sparing immunosuppressive agents: Methotrexate and azathioprine (joint
and blood affection), Mycofenolate mofetil and cyclophosphamine (renal and
neurological affection). - Treatment of complications
investigations of HSP
Laboratory
- Complete blood picture: normal platelet count
- ESR, CRP : elevated (inflammation)
- Urine analysis (screen for hematuria)
- Stool analysis (screen for occult blood in stool)
- Blood Urea, creatinine and C3: screen for nephritis
- lg A level (maybe elevated)
Imaging
1. Abdominal X ray and ultrasound if there is any doubt regarding gut perforation or intussusception
- Magnetic resonance imaging and magnetic resonance angiography in cases with CNS manifestations for diagnosis of cerebral vasculitis
treatment of HSP
- Supportive treatment with maintenance of good hydration and electrolyte balance.
- Control of pain with simple analgesics
- Control of hypertension if necessary
- Prednisone has been advocated with severe gastrointestinal disease or hemorrhage.
- Management of HSP nephritis with intravenous methyl prednisolone and cyclophosphamide
investigations of Kawasaki
Laboratory Findings
1. Leukocytosis with neutrophilia, elevated ESR and CRP, hypoalbuminemia,
thrombocytosis after week 1
2. Sterile pyuria
3. Elevated serum transaminases and plasma lipids.
Echocardiography should be performed at diagnosis and again after 2-3 weeks of illness. If the results are normal, a repeat study should be performed 6-8 weeks after onset of illness.
treatment of DKA
Hospitalization, better in ICU
1. Initial measures
A: Open the airway. B: Oxygen therapy. C: IV line.
- Fluid therapy
a) Resuscitation (Shock therapy)
If the patient is shocked give bolus of 10 -20 ml /kg isotonic saline 0.9% rapidly over 30 min and reassess could be repeated 3 times
b) Deficit therapy : according to the degree of dehydration
Deficit (litres) = % dehydration x body weight (kg)
mild=50ml/kg
moderate=100ml/kg
severe=150ml/kg
c) Maintenance: According to body weight or surface area
First 10 kg of body weight: 100 ml/kg/24 hours.
For each kg from 11-20 kg: 50 ml/kg/24 hours
For each kg above 20 kg: 20 ml/kg/24 hours
1. The total fluid (deficit plus maintenance) are given over (48 hours). Correction
must be gradual, rapid rehydration > cerebral edema
@ Use normal saline (0.45% saline is used in hyperosmolar cases).
When blood glucose has fallen tos 300 mg/dl add glucose to the IV fluids to
avoid hypoglycemia
@ If the patient shows mild to moderate dehydration start the deficit + maintenance therapy without bolus fluids
- Insulin therapy
Started after resuscitation, after one hour from fluid therapy
Continuous low dose IV infusion method. NO BOLUSES
1500.1unit/kg/hour, adjust the dose (rate of infusion) according to the blood
glucose level. Infusion must be continued until correction of ketoacidosis
Monitor the blood glucose regularly aiming for gradual reduction as rapid
reduction cerebral edema.
When glucose reaches 300mg/dl, glucose is added to fluids to maintain
glucose >200 mg/dL
When metabolic acidosis is corrected and the patient can tolerate feeding,
sub cutaneous insulin is given then intravenous insulin is stopped - Acidosis
The use of bicarbonate for correction of acidosis in DKA is generally not
recommended except in case of severe metabolic acidosis not
responding to therapy (pH 7 or less)
This is because:
a) Most cases will be self-corrected by fluids and insulin
b) Bicarbonate can exacerbate hyperosmolarity and electrolyte disturbance
c) Alkali therapy may result in alkalosis that has its own hazards.
@ Resistant acidosis: suspect sepsis or renal failure - Potassium therapy
- Potassium is added 30-40 mEq/L after the resuscitation, after the first hour and
after the patient passes urine provided K is not elevated.
Initial plasma potassium may be low or high but it will fall following treatment
with insulin and rehydration
If hypokalemia is present , it should be urgently treated - Clinical and laboratory monitoring
- Hourly capillary blood glucose (strips)
Plasma glucose, Na, K, blood gases and urinary ketones (plasma ketone by strips
if available) and repeat every 2-4 hours
Renal functions, CBC and sepsis screen if suspected - Management of complications
* Shock: hypovolemic (polyuria, vomiting & dehydration) or septic
Brain edema from rapid correction of hyperosmolarity and hypovolemia and
from cerebral ischemic hypoxia (shock)
Pulmonary edema from hyperosmolarity and HF(2ry to acidosis)
Cardiac arrhythmias (secondary to acidosis and electrolyte disturbance especially K)
2.150
treatment of Kawasaki
- Aspirin
= It is used as anti-inflammatory and antithrombotic
Anti-inflammatory
80-100 mg/kg/6hs or 30-50 mg/kg/6hours until patient is afebrile
Antithrombotic: 3-S5mg/kg/day until normalization of ESR and platelets
= Aspirin should be continued for 2 years if coronary abnormalities have
resolved
#Aspirin is continued for life if coronary abnormalities persisted
* Children with large coronary artery aneurysm should receive anticoagulants as
warfarin - IV immunoglobulin
228400 mg/kg/day on four consecutive days or single dose 2g/kg infused over 8-12 “
hours; it reduces the risk of coronary artery disease when administered within 10
days of the onset of fever. - Corticosteroids
investigations of DKA
hyperglycemia
glucosoria
ketonemia
ketonuria
ABG
Metabolic acidosis
CBC
CRP
ESR
2.150
Investigations of Hypothyrodism
Laboratory: Thyroid profile: T3, T4 and T.S.H.
1. Low T4 (normal range 5-12 microgram/dl).
2. High TSH (normal: 0.5 — 4 micro unit/L). TSH is markedly elevated (above 50 micro unit/L). It is the most sensitive test for primary hypothyroidism.
Imaging
1. Plain X ray: delayed bone age. it is characteristic for congenital hypothyroidism
2. Thyroid scanning (radioactive iodine : lodine 123) it is essential for diagnosis of the
cause (it can differentiate between aplasia, ectopic dysplasia, and malfunction of
the thyroid gland)
3. Thyroid Ultrasonography
Neonatal thyroid screening
It is implemented in Egypt for all newborns to prevent mental retardation.
1. Timing: between 3” and 7” day of life.
2. Technique: a blood drop is obtained by heel prick on filter paper and analyzed for
TSH.
3. Interpretation: if TSH level > 20 mU/L, an immediate blood sample is withdrawn and
analyzed. If data are confirmed treatment is immediately initiated.
2.141
treatment of congenital hypothyrodism
Lifelong therapy with L thyroxin to maintain normal growth, T3 and T4
1. Onset: should be started before 2 weeks of age to reduce the risk of impaired neurodevelopment.
2. Dose
= Neonates: 10-15 microgram /kg/day.
= Children: 100 microgram /m2 /day.
- Monitoring
= Clinical assessment
Physical: Height every 3 months and Bone age every year.
Mental development and I.Q.
Sexual: Pubertal development should be within normal.
« Laboratory assessment: good response is associated with:-
T4 on high normal
TSH on low normal.
treatment of noctornal enuresis
- Simple Measures: (Children > 4 years)
. Explain to the parent and child that the problem is common and beyond
conscious control so punishment should be avoided.
b. VJ Fluid intake in the evening
c. Urination before sleep
d. Waking the child up few hours after sleep to void
e. Rewarding for dry nights - Drug therapy (Children > 6 years)
a. Anticholinergic (4< Bladder capacity): Oxybutynin : may be needed in nonmonosymptomatic nocturnal enuresis
b. Desmopressin (synthetic vasopressin analogue): Single night dose
c. Enuresis alarms (conditioning therapy): Auditory or vibratory alarm is attached
to a wetness sensor in the underwear (wake the child up for urination)
2.172
inv of rhematic fever
- Acute phase reactants
* CBC: Leukocytosis
* Elevated ESR and CRP - Evidence of recent Streptococcal infection:
* Recent scarlet fever.
* Positive throat culture.
* Rapid antigen test.
* Antistreptococcal antibodies.
* High titer of:
Antistreptolysin O titer (ASOT).
Antistreptokinase.
Antihyaluronidase.
Anti-DNase. - Cardiac assessment
* CXR: Cardiomegaly
* ECG. Tachycardia and prolonged PR interval
* Echocardiography: assesses chamber enlargement, valve affection, and cardiac contractility and detects pericardial effusion.
INV of Gastroenteritis
For Gastroenteritis:
A. To identify the causative organism:
1. Stool analysis: Parasites (E. histolytica & G. lamblia), WBCs (Bacterial etiology)
2. Stool culture & sensitivity: Bacteria & viruses
3. CBC, CRP, ESR, Blood culture: help in diagnosis of bacterial infections:
B. To detect complications
1. KFTs (Urea & Creatinine): Renal failure.
2. Electrolytes: Na, Ca, K.
3. Blood gases: Metabolic acidosis.
4. Prothrombin time, platelet count, fibrin degradation products: DIC.
TTT of Gastroenteritis
Home management:
1- Oral rehydration solution (glucose facilitated sodium absorption)
Dose: Mild (50-80 ml/kg), moderate (80 ml/kg)
Route: 1 spoon every 2 mins or nasogastric tube
2- Feeding
3- Symptomatic Treatment: Antipyretics and anti-emetics.
4- Specific Treatment: Metronidazole ( parasites), Tetracycline (cholera), Ampicillin
(shigella or salmonella). However antibiotics may interfere with normal flora so
increasing pathogens.
5- If persistent diarrhea: give lactose free formulas, soy protein based formula, and
vitamin A
B) Hospital management:
IV fluid therapy for isonatemic dehydration
Shock therapy: 10 ml normal saline over 20-30 mins. May be repeated by max. 30 ml.
Deficit therapy: mild (50ml/kg) moderate (80ml/kg) severe (100ml/kg) of glucose:
saline ratio 1:1 over 6-8h. Add K when patient passes urine and serum K is not elevated.
Maintenance therapy: 1st 10 kg (100ml/kg) 2nd 10 kg (50ml/kg) the (20ml/kg) of
glucose 5% to saline ratio 4;1 over 16-18 h
* If dehydration is still present: give maintenance +deficit.
* If dehydration has been corrected switch to oral intake.
IV fluid therapy for hypernatremic dehydration
Shock therapy: same.
Deficit therapy: glucose: saline ratio 2:1 over 6-8h. Add K
Maintenance therapy: same.
* maintenance and deficit should be added and given over the period of correction
(1.25 times maintenance rate)
* correction should be slowly to avoid brain edema. Na must not decrease more than
12 mEq/L per 24h.
* Resistant cases may need dialysis
IV fluid therapy for Hyponatremic dehydration
Shock therapy: same
Deficit therapy: glaucose to saline ratio is 1:1
investigation and treatment of Intussusception
INV:
1. Abdominal US.
2. Plain X ray abdomen: distended loops, absence of gas in distant colon.
3. Barium enema: Claw sign of coiled spring sign.
TTT:
Depends of the time of presentation:
1. Air enema: pneumatic reduction.
2. Resection-anastomosis: in neglected cases
inv of acute hepatitis
to diagnose acute hepatic injury
CBA AA EE
Bilirubin level (above 10 mg/d
Low serum albumin
High blood ammonia level
Metabolic Acidosis.
Hypoglycemia, Hypokalemia and Hyponatremia.
To diagnose the causative virus (hepatitis markers)
1. Hepatitis A:
* IgM antibodies to hepatitis A (anti HAV IgM): recent infection (acute disease).
* IgG anti HAV antibodies persist after recovery (immunity).
2. Hepatitis B:
* HBsAg (surface antigen) followed by anti HBc (core antibodies) IgM: indicate
recent infection.
* Anti-HBs (surface antibodies) appear after recovery and indicate immunity.
* Persistence of HBsAg (surface antigen) and anti HBc IgG indicate chronicity.
3. Hepatitis C
* Anti-HCV antibodies: these antibodies denote exposure to infection but do not
mean recovery or development of immunity.
* HCV-RNA antigen detected by PCR denotes viremia.
* Viral load can be assessed by quantitative PCR for treatment purposes.
TTT of acute hepatitis
Treatment of any correctable condition:
1. Post viral: oral Antiviral drugs e.g., for hepatitis B. oral direct acting antivirals (DAA)
for hepatitis C e.g., nucleotide/nucleoside analogue
2. Autoimmune hepatitis prednisolone and azathioprine.
3. Wilson disease: Penicillamine is a copper chelating agent
Liver supportive measures:
1. Nutritional support
* Vitamin, minerals and carbohydrate rich diet.
* Fat in the form of medium chain triglycerides.
- Ascites
* Sodium and fluid restriction and diuretics.
* Albumin infusions or Paracentesis for refractory ascites. - Encephalopathy
* Treat precipitating factors e.g., GIT bleeding, sepsis
* Oral neomycin
* Protein restriction
* Oral lactulose to ↓ ammonia reabsorption (it ↓ colonic pH and ↑ colonic transit)
Liver transplantation in end stage liver disease:
