Pediatrics(2)

•Maintain pre transfusion Hb level at 9 -10 g/dl.

•Keep mean post-transfusion Hb at 13.5-15.5g/dl.

•Keep mean Hb 12 - 12.5 g/dl.

•The above targets allow for normal physical activity and growth, abolishes chronic hypoxaemia, reduce compensatory marrow hyperplasia

which causes irreversible facial bone changes and paraspinal masses.

•Transfusion interval?

•Usually 4 weekly interval (usual rate of Hb decline is at 1g/dl/week).

•Interval varies from individual patients (range: 2 - 6 weekly).

•Transfusion volume?

•Volume: 15 - 20mls/kg (maximum) packed red cells (PRBC).

•Round-up to the nearest pint of crossmatched blood provided. i.e. if calculated volume is just > 1 pint of blood, give 1 pint, or if calculated volume is just < 2 pints, give 2 pints.

This strategy minimizes the number of exposure to immunologically different units of blood product and avoid wastage of donated blood.

Note:

•In the presence of cardiac failure or Hb < 5g/dl, use lower volume PRBC (< 5ml/kg) at slow infusion rate over > 4 hours with IV Frusemide 1 mg/kg

(20 mg maximum dose).

•It is recommended for patients to use leucodepleted (pre-storage, post storage or bedside leucocyte filters) PRBC < 2 weeks old.

•Leucodepletion would minimize nonhaemolytic febrile reactions and

alloimmunization by removing white cells contaminating PRBC.

Thalassaemia intermedia

•A clinical diagnosis where patients present later with less severe anaemia at > 2 years of age usually with Hb 8g/dl or more.

•Severity varies from being symptomatic at presentation to being asymptomatic until later adult life.

•Assessment and decision to start regular transfusion is best left to the specialist.

-- Anemia of chronic disease (e.g: chronic pyelonephritis, chronic renal failure, bacterial endocarditis, osteomyelitis ) due to:

-• Impaired erythropoietin production.

-• Anemia can also be associated with hypothyroidism.

-• Sideroblastic anemia (Extremely rare, heterogeneous group of diseaeses, either acquired (drugs, toxins, malignancy), or congenital . diagnosis by bone marrow

-biopsy (presence of sideroblasts) anemia

-- Anemia due to increased red blood cell destruction (haemolysis)

-• Congenital

-→ Red cell membrane defects - including hereditary spherocytosis.

-→ Red cell enzyme abnormalities - including glucose-6-phosphate dehydrogenase (G-6-Pd) deficiency, pyruvate kinase deficiency.

-→ Hemoglobinopathies - including sickle cell disease,

thalassaemias. - • Acquired

-→ Autoimmune haemolysis

-→ Isoimmune haemolysis (haemolytic disease of the newborn, blood transfusion reactions).

-→ Infections (including malaria, septicaemia)

-→ drugand toxin-induced

-→ disseminated intravascular coagulation

-→ Hypersplenism.

-- Anemia due to blood loss

-• Including gastrointestinal blood loss, traumatic, heavy menstruation in girls.

-Complications

-- Pulmonary edema

-- Congestive heart failure

-- Acute respiratory distress syndrome (ARdS)

-Investigations according to clinical situation

-- FBC and reticulocyte count and peripheral blood smear

-examination

-- Blood film for malaria parasites

-- Stool examination for eggs of hookworm / Stool for occult blood,

-ova and parasites

-- Sickling test

-- Hemoglobin electrophoresis

-- Analysis for nutritional deficiencies

-- Bone marrow aspiration to assess the decreased production of red cells

-- Coombs direct and indirect (in cases of hemolytic anemia)

-- Iron studies (Fe, Ferritin, TIBC, transferring % saturation)

-- Other investigations will be dependent on the clinical evaluation of the patient

-Management

-- Obtain a detailed history from the patient or care givers

-- Examine the anaemic patient carefully and perform the

-appropriate investigations with a goal of:

-• Confirming that the patient is anaemic