Hemophilia A Slide Lapkas Anak

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








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








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.

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Hemophilia A Slide Lapkas Anak