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
