MIKROPENIS AND HIPOSPADIA IN CHILD
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Transcript of MIKROPENIS AND HIPOSPADIA IN CHILD
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INTRODUCTION
Cardiomyophaties are heart muscle disorders that affect ventricular systolic function,
diastolic function, or both. They are classified by the World Health Organization as (1)
dilated cardiomyopathy (DCM), (2) hypertrophic cardiomyopathy, (3) restrictive
cardiomyopathy, and (4) arrhythmogenic right ventricular dysplasia- cardiomyopathy.1Most
patients have pure forms of these disorders that fulfill strict diagnostic criteria, although
some have overlapping disorders with mixed forms of disease. Despite long-standing interest
in these high-impact disorders, the demographics and underlying causes have been difficult to
ascertain, particularly in children. Dilated cardiomyopathy, a myocardial disorder
characterized by a dilated left ventricular (LV) chamber and systolic dysfunction that
commonly results in congestive heart failure (CHF).
1,2
In some cases, right ventriculardysfunction is also noted and may add to the clinical severity of disease.
Approximately 30% to 35% of patients are reported to have a genetic form of DCM.
Infants and older children, however, appear to have a wider spectrum of causes, 3although
identifying these causes has been difficult. Relatively little information on the incidence of
cardiomyopathies in childhood has been published. Arola et al4 reported an incidence of
DCM of 0.34 cases per 100 000 children per year and a prevalence of 2.6 cases per 100 000
children in Finland, a racially homogeneous population. A large percentage of cases occurred
in infants (1 year of age; 3.8 per 100 000 cases per year). Recently, our group, the Pediatric
Cardiomyopathy Registry (PCMR), reported the incidence of pediatric cardiomyopathy in 2
regions of the United States, New England and the central Southwest.5A total of 467 cases of
childhood cardiomyopathy were reported, yielding an annual incidence of 1.13 per 100 000
infants and children overall, with differences by race, sex, and region. The PCMR report
defines the overall incidence of all forms of childhood cardiomyopathy but has limited details
regarding the causes, risks, and outcomes of specific forms of cardiomyopathy. However,more detailed information focusing on particular forms of cardiomyopathy is required for
clinicians to understand the clinical disorders of individual patients.1,2
The most common presentation of DCM is with signs and symptoms of overt cardiac
failure. In infants, this will usually manifest as feeding difficulties, whereas older children
will usually report a reduction in exercise tolerance, dyspnoea, or oedema.2,3,9
Cardiovascular complications may occur in children with acute leukemia as a result of
anemia, infection, chemotherapy, or leukemic infiltration of the myopericardium. 6Acquired
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cardiac complications are more common than those due to cardiac leukemic infiltration.6
Microscopic leukemic infiltration of the cardiac muscle and pericardium has frequently been
observed during post mortem examinations in patients with acute leukemia. Extranodal
malignant leukemias or lymphomas involving only the heart without dissemination is
extremely rare and very few antemortem cases have been reported. gross disease, particularly
at the time of initial leukemia diagnosis, is rare.7
The following report is a case of a child with dilated cardiomyophaty with acute
lymphoblastic leukemia B Lineage before chemotherapy, who has been hospitalized in Prof.
Dr. R. D. Kandou hospital Manado.
CASE REPORT
A 2 year 9 month old girl, PR, Christian, Minahasa tribe, was admitted to Prof. Dr. R.D.
Kandou General Hospital Manado on April 22, 2013 at 01.30 pm, with a chief complaint is
fever.
History of illness(alloanamnesis, given by the mother)
Patient was brought to Prof Kandou General Hospital with major complain : fever
since 3 days before admission to the hospital. The fever is not to high without shivering,
excessive sweats, and seizure. Patient was given antipyretic. Patient look pale but the parents
didnt recognize it,theres no history of bleeding from the patient. Patient daily activity was
normal. Theres no complain of poor feeding, irritability, and shortness of breath or
difficulty of breathing. Defecation and urination seems normal as usual.
This patient is the second son from 2 brothers, the deliveries were sectio cesaria, no
history of cyanotic attack upon crying and immediately cried.
History of prenatal care and birth
During the pregnancy, his mother had regular antenatal care and had tetanus toxoid
immunization twice. This patient was born with sectio cesaria, immediately cried, aterm,
birth weight was approximately 3400 grams, forgot the birth length.
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History of experienced illness
Patient had history of morbilli, diarrhea, fever-cought dan typhoid fever.
Developmental milestones
Social smile : 2 months
Turning in prone position : 3 months
Sitting : 4 months
Crawling : 6 months
Standing : 9 months
Calling mama/papa : 11 months
Walking : 12 months
History of feeding
Breast feeding : birth6 months
Milk Formula : birth - now
Milk porridge : 48 months
Soft rice porridge : 612 months
Rice : 9 monthsnow
Immunization
He received basic immunization completely as recommended
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Family Tree
Social, Economic and Environmental conditions
She is the second child in the family. His fathers age is 39 years old, a university graduation,
works in Medistar laboratorium, while his mothers age is 34 years old, a university
graduation, a work in Medistar laboratorium too.
They live in a permanent house with 4 bedrooms, occupied by 5 adults and 2 children.
The roof made from metal platform, the wall made from , the floor is ceramic, restroom is
located inside the house, there is electric source, drinking water source is from the dwell, and
garbage is burned regularly.
April 22, 2013 (1stday)
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Body weight 17 kg Z score 2 - 3 (WHO weightheight girl)
Body height 95 cm
Vital signs : Pulse rate : 116 x/min
Blood pressure : 100/70 mmHg
Respiratory rate : 28 x/min
Temperature : 37 C
Head : oval shape, bold hair
Eyes : conjunctiva anemia +/+, sclera icterus -/-
pupil isochoric with diameter 3 mm, responds to lights
Ears : left : wide meatus acusticus externus, normal ear drums
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right : wide meatus acusticus externus, normal ear drums,
no secretes
Nose : no secretes, no nasal flaring.
Mouth : no central cyanosis, tonsils T1/T1 without
inflammatory sign, pharynx without inflammatory sign
Neck : lymph node enlargement (-), no tenderness pain
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4th intercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : 1 cm laterallinea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 3-3 cm
below costal arch, spleen palpable Schuffner III
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
normal muscle tone, physiological reflexes normal, no pathology
reflexes
Genitalia : female, no abnormality
Laboratory
Hemoglobin : 6.5 g/dL (11.0-16.5 g/dL) Hematocrit : 18.1% (35-50%)
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Leukocyte : 81.500/mm3 (3.500-10.000/mm3) Eritrocyte : 2.5 g/dl (3,8-5,5) Thrombocyte : 11.000/mm3 (150.000-390.000/mm3) MCV : 72,4 fl (76-100 fl) MCH : 26 pg/cell (25-35 pg/cell) MCHC : 35.9 g/dl (31-35 g/dL) ANC : 815 Na : 135 mEq/L (134-144 mEq/L) K : 4.1 mEq/L (3.5-5 mEq/L) Cl : 104 mEq/L (98-107 mEq/L) Tubex : +4 Ureum : 30 mg/dl (10-50 mg/dl) Creatinin : 0.7 mg/dl (0.6-1.2 mg/dl)
Uric acid : 8.7 mg/dl (2-7 mg/dl)
Total protein : 6.1 mg/dl (6-8.4 mg/dl) Albumin : 3.6 g/dl (3.5-5.5 g/dl) Globulin : 2.5 mg/dl (2.3-3.5 mg/dl) SGOT : 60 U/l (2-35 U/l) SGPT : 17 U/l (2-45 U/l) CRP : < 6 LDH : 1266 Blood smear : erythrocyte normocytic normochromic, polichromatofilia (-),
normoblast (-), malaria (-). Leukocyte: high leukocytosis, there is domination with
lymphositic lymphoblast. Thrombocyte: severe thrombocytopenia, without aggregation.
Conclusion: acute lymphoblastic leukemia.
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Working diagnosis
Suspect acute lymphoblastic leukemia Thyphoid Fever Overweight
Treatment
IVFD NaCl 0.45 in D5% + 20 mEq natrium bicarbonate (Hyperhidration for 48 hours) 84-85 ml/hour = 28-29 gtt/min. Balance-diuresis every 4 hours, if balance + 100, give
furosemide injection 8 mg
Chloramphenicol 3x500mg per oral Allopurinol 2x60mg peroral Paracetamol syrup 3x1 cth (250 mg) CrossmatchPacked red cell Obsevasion Nutrition RDA child 1-3 years
Calory 102 kcal/kgbw, protein 1.23/kgbw, water 115-125/kgbw
Calory 1734 kcal/day, protein 20.91 gr protein / day, water 1.995ml/day
Planning
Transfusion with packed red cell/PRC (PRC: Hb x BW x 4270 ml), at 20.00-23.00 :transfusion PRC 180 ml.
Consult to pediatric cardiologi division Consult to ENT and Dentist department X photo thorax Bone marrow puncture Mantoux test
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Blood Culture
Follow up
April 23-24, 2013 (2nd
-3th
day)
Complaint : Vommiting 2 times, no blood, no fever, no abdominal pain
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 112 x/min
Blood pressure : 90/60 mmHg
Respiratory rate : 28 x/min
Temperature : 36.6 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : linea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 3-3 cm
below costal arch, spleen palpable Schuffner III
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
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Laboratorium
BMP result from Prodia : Suspek ALL, Hyperceluler with Limfoblas L1 domination sugestif ALL FAB Class L1 (limfoblas 76,4%)
Mantoux test (-) PCV 24% Waiting Blood culture result Elektrocardiography result:
Sinus rhythm,
QRS rate : 83x/minute ( normal 80-120x/minute)
P wave : 1mm, 0,08 sec
PR interval : 0,12 sec
Qt : 0,32 sec
QoTC : 0,02 sec
QRS interval : 0,1 sec
VAT at Vi 0,02 mm VAT at v6 : 2,5 mm
No abnormalities in QRS, ST segment, P wave, T wave
Conclusion : Normal ECG
Working diagnosis
suspect acute lymphoblastic leukemia Thyphoid Fever Overweight
Treatment
IVFD NaCl 0.45 in D5% + 20 mEq natrium bicarbonate (Hyperhidration until 24-04-2013, 12.00 WITA) 84-85 ml/hour = 28-29 gtt/min. Balance-diuresis every 4 hours, if
balance + 100, give furosemide injection 8 mg
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Ranitidin Injection 2x20mg IV Ondansentron Injection 2x4mg IV Chloramphenicol 3x500mg per oral Allopurinol 2x60 mg peroral Paracetamol syrup 3x1 cth (250 mg)
Planning
Thrombocyte transfusion 1 unit (24-04-2013 16.00) Waiting pediatric cardiologi division consult result Consult result from ENT and dentist was normal
April 25-27, 2013 (4th
-6th
day)
Complaint : No fever, no vommiting
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 116 x/min
Blood pressure : 90/60 mmHg
Respiratory rate : 30 x/min
Temperature : 36 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : linea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
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- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 3-3 cm
below costal arch, spleen palpable Schuffner III
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
Laboratorium 27-04-2013
Hemoglobin : 11,3 g/dL Hematocrit : 32% Leukocyte : 52.850/mm3 Eritrocyte : 3,81 g/dl Thrombocyte : 16.000/mm3 MCV : 85,6 MCH : 30,4 MCHC : 35,6 Ureum : 48 mg/dl Creatinin : 1.1 mg/dlGFR = 48% Uric acid : 8.3 mg/dl Total protein : 6.1 mg/dl Albumin : 3.6 mg/dl Globulin : 2.5 mg/dl SGOT : 63 U/l SGPT : 32 U/l Urinalysis : in normal limit
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Echocardiografi result (27-04-2013)2D : Sinus Solitus
AV-VA concordance
Left atrium and ventrikel dilatation
Not found VSD, ASD and PDA
Aortic arch is at the left side of the heart, no coartion of the aortic arch
M-mode : Heart contractility decreased (EF 29%, FS 13%), LA/AO 1.79
Conclusion : dilated cardiomyophaty
Suggestion : digoxin 0.01 mg/kg divided into 2 doses
Echocardiografi 3 weeks again
BMP Dharmais : Morfology ALLL1, B Lineage X photo thorax : CTR 55%
Working diagnosis
Acute lymphoblastic leukemia B Lineage Dilated Cardiomyopathy Acute Kidney Injury, injury stadium Thyphoid Fever Overweight
Treatment
Chloramphenicol 3x500mg per oral Allopurinol 2x60mg Digoxin 3x0.09mg Paracetamol syrup 3x1 cth (250 mg) Low sodium diet 1gr/day
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Planning
Thrombocyte transfusion 1 unit (26-04-2013 06.00) Packed red cell transfusion 180 cc pre furosemide 9mg IV (25-04-2013 17.30) Begin chemotherapy at 30-04-2013 with Indonesian ALL high risk protocol 2006.
Chemotherapy started without doxorubicin.
Echocardiografi 3 weeks again.
April 28-30, 2013 (7th
-9th
day)
Complaint : No fever, vommiting 2 times
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 100 x/min
Blood pressure : 100/60 mmHg
Respiratory rate : 28 x/min
Temperature : 36,8 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : linea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 4-4 cm
below costal arch, spleen palpable Schuffner III
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Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
Laboratorium
Hemoglobin : 8.0 g/dL Hematocrit : 22.5% Leukocyte : 80.100/mm3 Eritrocyte : 2.63 g/dl Thrombocyte : 8.000/mm3 Feces : in normal limit
Working diagnosis
Acute lymphoblastic leukemia B Lineage High Risk Induction Phase Week 0 day 1 (30-04-2013)
Dilated Cardiomyopathy Acute Kidney Injury, Injury stadium Thyphoid Fever Overweight
Treatment
IVFD NaCl 0.45 in D5% + 20 mEq natrium bicarbonate (Hyperhidration start 28-04-2013, 07.00 am) 84-85 ml/hour = 28-29 gtt/min. Balance-diuresis every 4 hours, if
balance + 100, give furosemide injection 8 mg
Methrotrexate 10 mg intatekal (30-04-2013) Dexamethasone tab (0.5mg) begin at 30-04-2013 day 1 1--0, day 2 1-1-1, day 3 2-1-1,
day 4 3-2-1, day 5 3-2-2
Chloramphenicol 3x500mg per oral Allopurinol 2x60mg
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Digoxin 3x0.09mg Paracetamol syrup 3x1 cth (250 mg) if needed
Planning
Thrombocyte transfusion 2 unit (29-04-2013 and 30-04-2013) Packed red cell transfusion 200 cc pre furosemide 9mg IV (30-04-2013)
May 1 - 2, 2013 (10th
-11th
day)
Complaint : No fever, no vommiting
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 100 x/min
Blood pressure : 130/90 mmHg
P90 106/65 mmgHg, P95 110/69 mmHg, P99 : 117/76 mmHg,
Krisis 165/113 mmHg
Respiratory rate : 28 x/min
Temperature : 36,7 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : linea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
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bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 4-4 cm
below costal arch, spleen palpable Schuffner IV
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
Laboratorium 02-05-2013
Hemoglobin : 10.7 g/dL Hematocrit : 32% Leukocyte : 1.300/mm3 Thrombocyte : 7.000/mm3 Diff Count : 0.2/0.3/0/15.1/74.4/10 ANC : 196.3 Ureum : 40 mg/dl Creatinin : 0,7 mg/dlGFR 76 % Uric acid : 4.3 mg/dl Total protein : 5.6 mg/dl Albumin : 3.1 mg/dl Na : 132 mEq/l K : 4.6 mEq/l Cl : 103 mEq/l Ca : 8.1 mg/dl Blood culture : no growth
Working diagnosis
Acute lymphoblastic leukemia B Lineage induction phase week 0 Dilated Cardiomyopathy
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Thyphoid Fever Hypertension grade II Neutropenia Overweight
Treatment
Furosemide injection 2x20mg IV Dexamethasone tab as scheachuled Chloramphenicol 3x500mg per oral (until 02-05-2013, 10 days) Digoxin 3x0.09mg Captopril 3x6.25mg Paracetamol syrup 3x1 cth (250 mg) if needed Isolation room
Planning
Chemotherapy as scheachuled Echocardiografi 18-05-2013
May 3 - 8, 2013 (12th
-17th
day)
Complaint : No fever, no vomiting, intake (+)
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 100 x/min
Blood pressure : 100/60 mmHg
Respiratory rate : 28 x/min
Temperature : 36,7 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
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Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : linea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 4-4 cm
below costal arch, spleen palpable Schuffner IV
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
Laboratorium 06-05-2013
Hemoglobin : 8.4 g/dL Hematocrit : 26.9% Leukocyte : 1.190/mm3 Thrombocyte : 48.000/mm3 Diff Count : 0/1.7/0/10.1/83.2/5 ANC : 120 Ureum : 63 mg/dl Creatinin : 0,6 mg/dl LDH : 1064 Na : 138 mEq/l
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K : 4.4 mEq/l Cl : 99 mEq/l Ca : 10.4 mg/dl
Working diagnosis
Acute lymphoblastic leukemia B Lineage induction phase week 1 Dilated Cardiomyopathy History of Hypertension grade II Neutropenia Overweight
Treatment
Vincristine injection IV 1 mg (07-05-2013) Furosemide injection 2x20mg IV for 5 days until 06-05-2013 Cotrimoxasole syrup 1x1 cth for 2 days (4-5 may 2013) Dexamethasone tab 3-3-2 Digoxin 3x0.09mg Captopril 3x6.25mg for 3 days untul 04-05-2013 Paracetamol syrup 3x1 cth (250 mg) if needed Isolation room
Planning
Chemotherapy as scheachuled Echocardiografi 18-05-2013
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May 8 - 14, 2013 (18th
-24th
day)
Complaint : fever (+), no vomiting, intake (+)
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 112 x/min
Blood pressure : 100/70 mmHg
Respiratory rate : 28 x/min
Temperature : 37,7 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
- Percussion : left margin : linea midclavicularis sinistra.Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 4-4 cm
below costal arch, spleen palpable Schuffner IV
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
Laboratorium
10-05-2013 13-05-2013
Hemoglobin : 9.4 g/dL 7.9 g/dl Hematocrit : 26.9% 21.5%
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Leukocyte : 1.300/mm3 1.900/mm3 Thrombocyte : 43.000/mm3 48.000/mm3 Diff Count : 0/0/0/8/90.4/1.6 0/0/4/14/80/2 ANC : 104 342 Ureum : 32 mg/dl Creatinin : 0.4 mg/dl LDH : 422 Na : 142 mEq/l K : 4.02 mEq/l Cl : 106.2 mEq/l Ca : 10.6 mg/dl
Working diagnosis
Acute lymphoblastic leukemia B Lineage induction phase Dilated Cardiomyopathy Neutropenia Overweight
Treatment
Methrotrexate 10 mg intatekal (14-05-2013) Vincristine 1 mg IV (14-05-2013) Ceftriaxone injection 2x650mg IV for 6 days (May 8-14 2013) Gentamicin Injection 1x80mg IV for 6 days (May 8-14 2013) Dexamethasone tab 3-3-2 Digoxin 2x0.09mg Paracetamol syrup 3x1 cth (250 mg) if needed
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Isolation room
Planning
Packed red cell transfusion 200 cc pre furosemide 8mg IV (13-05-2013) Chemotherapy as scheachuled Echocardiografi 18-05-2013
May 15 - 18, 2013 (18th
-21th
day)
Complaint : fever (+), no vomiting, intake (+)
General conditions : Look moderately ill, GCS E4V5M6 /compos mentis
Vital signs : Pulse rate : 112 x/min
Blood pressure : 100/70 mmHg
Respiratory rate : 28 x/min
Temperature : 37,7 C
Head : conjungtiva is not anemic, sclera is not icteric, no head nodding
Chest : symmetrical respiratory movements, no retraction
Heart :
- Inspection : no visualization of ictus cordis- Palpation : ictus cordis palpable at the 4thintercostals space at 1cm
lateral from linea midclavicularis sinistra
-
Percussion : left margin : linea midclavicularis sinistra.
Right margin : linea parasternalis dextra.
Upper margin : 2nd3thintercostal space.
- Auscultation : regular heart beat,no audible murmur , gallop (-)Lungs : symmetrical movement, symmetrical vocal fremitus,
bronchovesicular breath sound, without rales nor wheezing
Abdomen : flat, soft, with normal bowel sound, liver palpable at 4-4 cm
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below costal arch, spleen palpable Schuffner IV
Extremities : warm, no cyanotic, capillary refill time less than 2 seconds,
Laboratorium
18-05-2013
Echocardiografi
2D : Sinus Solitus
AV-VA concordance
Heart chamber was normal
Not found VSD, ASD and PDA
Aortic arch is at the left side of the heart, no coartion of the aortic arch
M-mode : left ventrikel systolic function normal (EF 87%, FS 54%), LA/AO 0.85
Conclusion : normal intracardiac
Suggestion : theres no contraindication to give sitostatika drugs
Working diagnosis
Acute lymphoblastic leukemia B Lineage induction phase Neutropenia Overweight
Treatment
Digoxin 3x0.09mg until 18-05-2013 (20 days) Paracetamol syrup 3x1 cth (250 mg) if needed Isolation room
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Planning
Chemotherapy as scheachuled
DISCUSSION
Dilated cardiomyopathy (DCM) refers to congestive cardiac failure secondary to dilatation
and systolic dysfunction (with or without diastolic dysfunction) of the ventricles
(predominantly the left ventricle). DCM is the most common type of heart muscle disease in
children. All 4 cardiac chambers are dilated and are sometimes hypertrophied. Dilation is
more pronounced than hypertrophy, and the left ventricle is affected more often than the right
ventricle. The cardiac valves are intrinsically normal.
8
Injury to the myocardial cell is the initiating factor that leads to cell death. If
considerable cell loss occurs, the myocardium fails to generate enough contractile force to
produce adequate cardiac output. This results in the activation of the following compensatory
mechanisms: The renin-angiotensin-aldosterone system, sympathetic stimulation, antidiuretic
hormone production, release of atrial natriuretic peptide. These compensatory mechanisms
help to maintain cardiac output in the initial phase; however, as myocardial damage
progresses, persistent and excessive activation can be detrimental to cardiac function, leadingto overt congestive heart failure. Over-stretching of the ventricles causes myocardial
thinning, cavity dilation, secondary valvular regurgitation, and compromised myocardial
perfusion. The resulting subendocardial ischemia perpetuates myocyte damage.8
Myocardial remodeling is an important contributor to worsening heart failure. Lost
myocyte cells are replaced with fibrous tissue, thereby decreasing the compliance of one or
more ventricles and adversely affecting performance. Aldosterone, angiotensin II,
catecholamines, endothelins, and mechanical factors, such as excessive myocardial stretch
and ischemia, have been identified as mediators of remodeling.8
The most common presentation of DCM is with signs and symptoms of overt cardiac
failure. In infants, this will usually manifest as feeding difficulties, whereas older children
will usually report a reduction in exercise tolerance, dyspnoea, or oedema.2,3,9
In this case, from the history of illness we didnt found any symptoms like reduction
in exercise tolerance, dyspnoea, oedema and feeding difficulties. From physical examination
we found the vital sign in normal limit, the heart palpation , ictus cordis palpable at the
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4thintercostals space at 1 cm lateral from linea midclavicularis sinistra, percussion of the heart
left margin was 1 cm lateral linea midclavicularis sinistra. Freom the physical examination
we can said that the heart is larger.
Although the etiology of the majority of DCM cases remains unknown even after
extensive investigation, it is important to thoroughly exclude potentially reversible causes of
the DCM. Studies estimate that a definitive cause can be found in around 30-40% of DCM
cases. Table 1 outlines most of the possible underlying etiologies, with the remainder being
classified as idiopathic DCM. This is a diagnosis of exclusion, and at first presentation an
extensive of panel of investigations should be performed, with the aim of identifying the
conditions outlined below.8
Table 1 : Secondary Causes of dilated cardiomyophaty.8
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From table 1 we can see that leukemia and anemia are etiology and risk factor of
DCM. This patient had anemia, although her family didnt know since when her child look
pale. Because theres no history of bleeding in this patient, anemia in this patient can be the
complication of leukemia.
In chronic severe anemia, low Hgb reduces systemic vascular resistance (SVR)10as
the result of decreases in blood viscosity and enhanced nitric oxide-mediated
vasodilation11,12. Low SVR reduces blood pressure (BP) and causes baroreceptor-mediated
neurohormonal activation10. The increased sympathetic and renin-angiotensin activity
decreases RBF and glomerular filtration rate, resulting in salt and water retention by the
kidneys and expansion of the extracellular and plasma volumes. The combined effect of
volume expansion and vasodilation increases the cardiac output
10
, which may help to increaseoxygen transport. We can see the mechanism in figure 1. The increased myocardial workload
due to hemodynamic and neurohormonal alterations observed in chronic anemia10 could
cause adverse LV remodeling. LV hypertrophy and dilation are observed in animal models of
severe anemia13, and they may contribute to poor outcomes. Hemoglobin may be inversely
related to ejection fraction (EF)14,15, and whereas anemia is related to brain natriuretic peptide
(BNP), a marker of LV dysfunction, anemia remains an independent predictor of adverse
outcomes in the presence of BNP and EF, suggesting that these variables may have their
effect through different mechanisms.14 How long anemia condition can become
cardiomyophaty still unknown. Until now theres no data support it.
Figure I : Possible Sequence of Events Involved in the Pathogenesis of Heart Failure
in Chronic Severe Anemia.16
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Cardiovascular complications may occur in children with acute leukemia as a result of
anemia, infection, chemotherapy, or leukemic infiltration of the myopericardium.6Acquired
cardiac complications are more common than those due to cardiac leukemic infiltration.6
Microscopic leukemic infiltration of the cardiac muscle and pericardium has frequently been
observed during post mortem examinations in patients with acute leukemia. Extranodal
malignant leukemias or lymphomas involving only the heart without dissemination is
extremely rare and very few antemortem cases have been reported. gross disease, particularly
at the time of initial leukemia diagnosis, is rare.7
We diagnosed this patient with dilated cardiomyophaty from echocardiography. From
the echocardiography result from 27-04-2013, the conclusion are Cardiomyopathy dilated
with decreased heart contractility (EF 29%, FS 13%, LA/AO 1,79) and given digoxin 0.01mg/kgBB divided into 2 doses, and planning to perform echocardiografi 3 weeks again.
Acute lymphoblastic leukemia is an acute, rapidly progressing form of leukemia that
is characterized by the presence in the blood and bone marrow of large numbers of unusually
immature white blood cells destined to become lymphocytes. Acute lymphoblastic leukemia
is also called acute lymphocytic leukemia and is abbreviated ALL. 17-19 ALL is the most
common cancer occurring in children, representing almost 25% of cancer among children.
Approximately 2,000 children less than 15 years of age diagnosed with ALL in the USA each
year. It has a striking peak incidence between 2-5 years of age and occurs more frequently in
boys than in girls, at all ages.18,20,21
In virtually all cases, the etiology of ALL is unknown, although several genetic and
environmental factors are associated with childhood leukemia. Although the cause of ALL in
humans is unknown, leukemic transformation is unlikely to be the result of a single event but
rather the culmination of multiple processes involving complex interactions between host
susceptibility, chromosomal damage secondary to physical or chemical exposure.17-19
The clinical features of ALL are variable and are associated with the classification of
the disease. PrecursorB-cell ALL is primarily a disease of children, with 75% of cases
presenting in children less than 6 years of age and accounting for 80% to 85% of ALL in the
majority of patients with ALL. The clinical presentation is variable, and the symptoms may
appear insidiously or acutely, depending on the extent of disease. Patients commonly present
with a short history of fatigue, or spontaneous bleeding. 17,18Malaise, lethargy, weight loss,
fevers and night sweats are often present but typically are not severe. Compared to acute
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myeloid leukemia (AML), patients with ALL experience more bone and joint pain. Rarely,
they may present with asymmetric arthritis or low back pain. Children experience these
symptoms more frequently than adults. Young children may have difficulty walking due to
bone pain. CNS involvements is also more common in ALL compared to AML. Nausea,
vomiting, headache or papilloedema may results from meningeal infiltration. Testicular
involvements, presenting as painless, unilateral mass, is noted at diagnosis in approximately
2% of boys. The physical examination is often notable for pallor, generalized
lymphadenopathy, sign associated with thrombocytopenia, such as gingival bleeding,
epistaxis, petechiae/ecchymoses, or fundal hemorrhages and hepatosplenomegaly.
Lymphadenopathy, hepatosplenomegaly, and other extramedullary involvements are frequent
in precursor B-cell ALL.21-23
In this case, from the history of illness we found the symptoms pallor and also history
of fever with nausea, vomiting. From physical examination we also found conjunctiva
anemia, lymphadenopathy and hepatosplenomegaly.
The diagnosis ALL can be made by history of illness, physical examination and
laboratory finding. History of illness and physical examination is already discussed in the
above. ALL can usually be diagnosed from the presence of blast cell in the peripheral blood,
a bone marrow aspiration, cytochemical staining characteristics, immunophenotype, and
cytogenetic features. The diagnosis ALL is strongly suggested by peripheral blood findings
indicative of bone marrow failure. Anemia (hemoglobin < 10g/dl) and thrombocytopenia
( 10.000/mm3occurs in half of
patients, but only 20% of patients with the initial leukocyte count is greater than 50.000/mm3.
ALL is diagnosed by a bone marrow evaluation that demonstrates more than 25% of the bone
marrow cells as a homogeneous population of lymphoblast. The morphological recognition of
lymphoblast in the blood and bone marrow and their phenotypic characterization are of majorimportance to the correct diagnosis and classification of ALL. Immunophenotyping of
leukaemic lymphoblasts by flow cytometry is essential to establish the correct diagnosis and
define cell lineage. Although acute lymphoblastic leukemia can be readily sub classified
according to the many steps of normal B-cell and T-cell differentiation, the only findings
with therapeutic importance are T-cell, mature B-cell, and B-cell precursor phenotypes. We
must also determine the prognostic factors for every patient with ALL to choice of the
treatment protocol because the single most important prognostic factor in ALL is the
treatment. The important predictive factors are age, gender, the initial leukocyte count, CNS
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leukemia, peripheral blast count 1 week after the first chemotherapy, abnormality
chromosome, phenotype and mediastinal mass. A chest radiograph is necessary to determine
whether there is a mediastinal mass or not. Patients considered to be at higher risk are
children who are older than 10 years or less than 1 year of age or who are have an initial
leukocyte count of more than 50.000/mm3, have mediastinal mass, CNS leukemia and
peripheral blast count 1000.22,23
We do the laboratory examination for this patients, the haemoglobin level just 6.5
g/dl, white blood cell count 81.500/mm3, thrombocytopenia 11.000/mm3, differential count
0/1.7/0/10.1/82/5 with lymphoblast 76.4% in bone marrow aspiration result, absolute
neuthropil count/ANC just 120, and result of blood smear is acute lymphoblastic leukemia.
The result of phenotype is B-cell precursor phenotypes. we was already done the chest x-rayexamination for this patients to know about mediastinal mass, but in this patients the chest x-
ray was no abnormality. Based on the history of illness, physical examination and laboratory
finding, we able to diagnose this patient as ALL B-cell precursors. We use Indonesian
protocol ALL High risk 2006 for this patients, it means that the prognosis is poor.
Doxorubicin in the first 3 weeks because this patient had dilated cardiomyophaty.
Cardiomyopathy resolved after digoxin therapy. We done the echocardiografi again 3 weeks
after treatment and the intracardiac was normal. After that we gave doxorubicin again as
treatment. Every Friday until Sunday we give cotrimoxazole for prophylactic to pneumonia.
We give this patient digoxin 0.01 mg/kg divided into 2 doses for 20 days. This drug,
also referred to as digitalis, increases the strength of your heart muscle contractions. It also
tends to slow the heartbeat. Digoxin reduces heart failure symptoms and improves your
ability to live with dilated cardiomyopathy. Digoxin improves myocardial contractility,
reduces heart rate, and lowers sympathetic stimulation in chronic heart failure. It inhibits the
Na+-K+ ATPase pump. Sodium preferentially exchanges with calcium, increasing theintracellular calcium and resulting in an increase in contractility.17After 20 days of digoxin
treatment and packed red cell transfusion, the echocardiografi result was normal, Complete
recovery of LV function is possible in children with IDC. Recovery may occur within the
first year after initial examination in some patients, but longer periods are needed in the
majority of patients in whom LV function ultimately returned to normal.25
Prognosis of this patient is dubia at malam, because from beginning the patient
diagnosed with high risk ALL.
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Protokol ALL High Risk
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Nutrition Status
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Thorax Photo
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Echocardiography 27-04-2013
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Echocardiography 18-05-2013
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Electrocardiography