hemophilia a slide lapkas anak
Post on 12-Jan-2016
225 Views
Preview:
DESCRIPTION
TRANSCRIPT
HEMOPHILIA AHEMOPHILIA ACOMPILED BY :
Silvia Yanita Karina 11010260Timotius Kevin Natanael 11010296
SUPERVISIORED BY :dr. Tiangsa Sembiring, M.Ked(Ped), Sp.A(K)
Normal Normal HemostasisHemostasis
XX IIII
IIIIXXIXIX
TFTF--Bearing CellBearing Cell
Activated PlateletActivated Platelet
PlateletPlateletTFTF
VIIIaVIIIa VaVa
VIIIaVIIIa VaVa
VaVa
VIIaVIIa
TFTF VIIaVIIa XaXa IIaIIa
IXIXVV VaVa
IIII
VIIIVIII/vWF/vWF
VIIIaVIIIa
IXaIXa XX
IXaIXa
IXaIXaVIIaVIIaXaXa
IIaIIa
IIaIIa
XaXa
What is Hemophilia?What is Hemophilia?
• Hemophilia is an inherited bleeding disorder in which there is a deficiency or lack of factor VIII (hemophilia A) or factor IX (hemophilia B)
Why it happens
Hemophilia AHemophilia A
• It is caused by the lack of activity of the plasma protein factor VIII which affects the clotting property of blood.
Symptoms of Hemophilia ASymptoms of Hemophilia A
•Bruising
•Spontaneous bleeding •Blood in the urine or stool
•Prolonged bleeding
•Bleeding into joints with pain and swelling
•Gastrointestinal tract and urinary tract hemorrhage
Hemophilia A and B are classified as mild, moderate, or Hemophilia A and B are classified as mild, moderate, or severe, depending on the amount of clotting factor VIII or severe, depending on the amount of clotting factor VIII or IX in the bloodIX in the blood
Screening TestScreening Test
Complete Blood Count (CBC) Activated Partial Thromboplastin Time
(APTT) Test Prothrombin Time (PT) Test Fibrinogen Test Clotting Factor Tests : factor VIII
Case ReportCase Report
MVN, a 6 year 3 months old boy, with 19 kg of body weight and 115 cm of bodylength, came to pediatric departement of non infection unit in Haji Adam Malik General Hospital Medan on 4 June 2015with chief complaint of left knee swelling.
History of disease :
Left knee swelling had been suffered by the patient for the recent three days. Swelling in the left ankle is not found. History of gum bleeding was not found. History of fever was not found. History of nausea was not found. History of trauma was not found.
Urination : positive, normal. Defecation : positive, normal.
AntropometryAntropometry : :
BW/BL: 19/20,5 x 100% = 92,6 % BW/age: 19/21 x 100% = 90,4 % BL/age : 115/116,5 x 100% = 98,7 %
Feeding HistoryFeeding History
From birth to 6 months : Breast milk only From 6 months to 9 months : Breast milk with
rice porridge From 9 months to 1,5 years : Breast milk with
soft rice From 1,5 years until now : Family food
History of Growth and DevelopmentHistory of Growth and Development
BW of born : 3000 gram BL of born : 50 cm Sitting : 7 months Crawling : 9 months Standing : 12 months Walking : 14 months
Physical ExaminationPhysical Examination
Generalized status : Body weight: 19 kg, Body length: 115 cm, Body weight in 50th percentile according to age: 21 kg Body length in 50th percentile according to age: 116,5
cm Body weight in 50th percentile according to body
length: 20,5 kg BW/BL: 19/20,5 x 100% = 92,6 % BW/age: 19/21 x 100% = 90,4 % BL/age : 115/116,5 x 100% = 98,7 %
Present Present statusstatus
Consciousness : GCS 15 (E4 V5 M6)Blood Pressure: 100/70 mmHgHeart Rate : 100 x/iRespiratory Rate: 22 x/iBody Temperature: 37,2⁰C.
Anemic (-). Icteric (-). Cyanosis (-). Edema (-). Dyspnea (-).
Localized Localized statusstatusHead : Light reflex (+/+), isochoric.Pale inferior palpebral conjunctiva (-/-). Icteric sclera (-/-). Ear, nose, and mouth were within normal limit.
Neck : Lymph node enlargement was not found.
Thorax: Symmetrical fusiform. Chest retraction was not found. HR: 100 x/i, regular, murmur was not found. RR: 22 x/i, regular, ronchi was not found. Breath sound: vesicular. Additional sound was not found.
Abdomen: Soepel. Liver unpalpable, spleen unpalpable. Peristaltic (+) normal.
Extremities: Pulse 100x/i, regular, adequate pressure and volume, warm, CRT <3”. Hemarthrosis was found at left knee.
Differential Diagnosis: Hemophilia A Hemophilia B von Willebrand disease
Working Diagnosis: Hemophilia A
Management:Management:
Injection Koate 500 IU/12 hours for 3 days
Diet with normal food 1450 kkal with 38 g protein
JuneJune, 5th 2015, 5th 2015
S Left knee pain, chest hematoma
O Cons: CM, Temp: 36,9⁰C. BW: 19 kg
Head : Light reflex (+/+), isochoric. Pale inferior palpebral conjunctiva (-/-). Ear,
nose, and mouth was in normal limit.
Thorax: Symmetrical fusiform. Chest retraction was not found. HR: 94 x/i, regular,
murmur was not found. RR: 22 x/i, reguler, ronchi was not found.Breath sound:
vesicular. Additional sound was not found.
Abdomen: Soepel, liver unpalpable, spleen unpalpable. Peristaltic (+) normal.
Extremities: Pulse 94 x/i, regular, adequate pressure and volume, warm, CRT < 3”.
Left knee pain was found.
A Hemophilia A + arthropathy genu (S)
P Management:
- Injection Koate 500 IU/12 jam/IV (D2)
JuneJune, , 66thth 20152015
S Left knee pain
O Cons: CM, Temp: 37⁰C. BW: 19 kg
Head : Light reflex (+/+), isochoric. Pale inferior palpebral conjunctiva (-/-). Ear,
nose, and mouth was in normal limit.
Thorax: Symmetrical fusiform. Chest retraction was not found. HR: 98 x/i, regular,
murmur was not found. RR: 22 x/i, reguler, ronchi was not found.Breath sound:
vesicular. Additional sound was not found.
Abdomen: Soepel, liver unpalpable, spleen unpalpable. Peristaltic (+) normal.
Extremities: Pulse 98 x/i, regular, adequate pressure and volume, warm, CRT < 3”.
Hematoma was found in the left knee.
A Hemophilia A
P Management:
- Injection Koate 500 IU/12 jam/IV (D3)
- Diet with normal food 1450 kkal with 38 g protein
JuneJune, , 77thth 20152015
S Left knee pain was not found.
O Cons: CM, Temp: 37,2⁰C. BW: 19 kg
Head : Light reflex (+/+), isochoric. Pale inferior palpebral conjunctiva (-/-). Ear,
nose, and mouth was in normal limit.
Thorax: Symmetrical fusiform. Chest retraction was not found. HR: 96 x/i, regular,
murmur was not found. RR: 22 x/i, reguler, ronchi was not found.Breath sound:
vesicular. Additional sound was not found.
Abdomen: Soepel, liver unpalpable, spleen unpalpable. Peristaltic (+) normal.
Extremities: Pulse 98 x/i, regular, adequate pressure and volume, warm, CRT < 3”.
Hematoma was found in the left knee.
A Hemophilia A
P outpatient
Laboratory Findings :Laboratory Findings :
Parameters Patient Control
aPTT 44,8 sec 32,0 sec
April, 22nd 2015
Parameters Value Normal Value
Factor VIII 2,7 % 55-150 %Factor IX 70,9 % 70-140 %
March, 17th 2015
SUMMARYSUMMARY
MVN, a 6 year 3 months old boy, with 19 kg of body weight and 115 cm of bodylength with chief complaint of left knee swelling was diagnosed with Hemophilia A
The diagnosis was established based on history talking, clinical manifestation and laboratory findings.
top related