pulm ht in sickle cell disease final
TRANSCRIPT
PREVALANCE OF PULMONARY HYPERTENSION IN CHILDREN WITH SICKLE CELL DISEASE
Patel Parvez, Sharma Sujata, Shah Nikita, Chatterjee Goutomi, Sharma Ratna, Manglani Mamta.
Division of Paediatric Haematology-Oncology.
Lokmanya Tilak Municipal Medical College & General Hospital, Sion, Mumbai
Introduction
• Pulmonary hypertension (PHT) is a widely recognized complication of hereditary hemolytic anemia including sickle cell anemia, thalassemia, hereditary spherocytosis and paroxysmal nocturnal hemoglobinuria.
• Recent studies have found the prevalence of pulmonary hypertension in children to be between 16% and 26.2 %.
• Sickle cell disease is one of the disease at risk to develop PHT.
Aims and Objectives
• Aims :
To determine the prevalence of elevated pulmonary artery pressures in children with sickle cell disease.
• Objectives :• To identify the risk factors that are associated with the
development of elevated pulmonary artery pressures.
• To correlate with various demographic characteristics.
• To study variation in clinical presentation and biochemical markers
• To study the transfusion requirements and treatment details in patients of pulmonary hypertension.
Materials
• Design: Observational study
• Place of the study: Paediatrics Haematology-Oncology Division, LTMGH and LTMMC Sion.
• Duration of the Study: March 2013- February 2014 (12 Months)
• Sample size: 50
• Inclusion Criteria: All patients between the ages of 5 to 18 years diagnosed to have Sickle Cell Syndromes (Hb-SS, Hb-SC, Hb-SD, Hb-S/β+,Hb-S/β0 ) who were registered at our Centre for regular treatment.
• Exclusion Criteria: Patients with pulmonary stenosis / or any other structural obstruction to pulmonary blood flow were excluded.
• History included the • Patient’s age at diagnosis• Age at first transfusion, • Frequency of transfusion,• Frequency and types of crisis• Treatment details. • A complete physical examination was performed and findings were
noted. • Special emphasis was laid on the presence of anemia, icterus,
hepatosplenomegaly and stigmata of crisis.
Methodology
Investigations
• Complete hemogram with reticulocyte count,• Liver function tests,• Renal function tests, • Chest radiograph, • Electrocardiogram• Pulse-oximeter
• 2D Echogram. :- Pulmonary Hypertension
Normal : TRV < 2.5 m/second (PAP <30 mmhg)
Mild : TRV of 2.5 to 2.9 m/second (PAP 30-39 mmhg)
Moderate : TRV ≥ 3 m/second (PAP >40 mmhg)
to Severe ( Reference from ASC )
RESULTS Age and Gender Distribution of Patients
5 to 10 years 11 to 15 years 15 to 18 yrs
Males 11 15 4
Females 9 10 1
2.5
7.5
12.5
17.5
22.5
27.5
1115
4
9
10
1
No.
of p
atien
ts
Community Distribution of Patients
Boudha, 27
Adivasi,8
Bairagi, 1
Mahar, 1
Oriya, 1
Banjara, 1
Patel, 1
Suryavanshi, 1
Konkani, 2
Muslim, 2
Dhobi, 2Mang, 1 Prajapati, 1 Katkari, 1
Boudha Adivasi Bairagi Oriya Banjara Patel Suryavanshi Konkani Muslim Dhobi Mang Prajapati
Katkari
First Presenting Symptom (n=50)
Painful crisis with pallor
Fever with pallor Lump in abdomen with pallor
Severe Pallor with failure
05
10152025303540
35 (70%)
13 (26%)
1 (2%) 1 (2%)
Number of patients
Frequency of Clinical Presentation
Bony Crisis
No Crisis
VO+Hemolytic
Acute Chest
Syndrome
Sickle Hepato
pathy
Hemolytic
Splenic Sequestr
ation0
5
10
15
20
25
30
35
4036
4 42 2 1 1
Grades of PHT
62%, (31)
24%, (12)
14%, (7)
No PHT
Mild PHT
Moderate PHT
Correlation of PHT with Types of Sickle Cell Disease
Types of Sickle Cell Disease
PHTTotal
(N=50)Mild Moderate Normal
Sickle cell homozygous
8 (16%) 2 (4%) 16 (32%) 26
HbS/ β+ thalassemia
3 (6%) 5 (10%) 14 (28%) 22
Hb S/ D disease 1 (2%) 0 (0%) 1 (2%) 2
Total12 7 31 50
Correlation of PHT with Clinical Presentation
0
5
10
15
20
25
30
12
2 2 2 1 0 0
24
2 20 0
2 1
PHT NO PHT
Association of various Factors with PHT
Parameters PHT N Mean SD SE of Mean
T value P value Significance
No of VO crisis No 31 1.3548 0.984 0.17495 2.130 0.046 Yes
Yes 19 1.7368 1.77375 0.40693
No. of PRC transfusion No 31 3.4516 7.04196 1.26477 2.231 0.043 Yes
Yes 19 6.1579 8.31507 1.90761
Correlation of Hb with PHT
Mea
n Bas
eline H
b
Mea
n Hb on
trea
tmen
t0
2
4
6
8
10
12
7.25
9.58
6.31
9.26
Normal PHT
Parameters PHT N=50 Mean P value
Significance
Hemoglobin
at baseline
No 31 7.2581 0.046 Yes
Yes 19 6.3158
Hemoglobin
on
treatment
No 31 9.5806 0.496 No
Yes 19 9.2632
Cont.…Parameters PH
TN=50 Mean SD SE of
MeanT value P value Significance
Indirect bilirubin No 31 0.8710 1.1472 0.20605 2.303 0.045 Yes
Yes 19 1.7368 1.2841 0.29461
Serum LDH
No 31 885.789 265.21 67.32870 2.127 0.045 Yes
Yes 19 996.774 374.87 60.84433
Retic count baseline No 31 3.4516 1.1786 0.21169 2.156 0.036 Yes
Yes 19 4.4211 2.0087 0.46084
Cont.…Parameters PH
TN=50 Mean SD SE of
MeanT value P value Significance
Age of starting
Hydroxyurea (years)
No 31 6.9677 3.84260 0.69015 1.943 0.049 Yes
Yes 19 6.0000 2.90593 0.66667
Mean doseof
Hydroxyurea(mg/Kg/day)
No 31 19.7419 4.93266 0.88593 2.108 0.045 Yes
Yes 19 20.5789 5.55093 1.27347
conclusion
• Out of 50 patients, 19 (38%) PHT • 10 (52.63%)- Sickle cell homozygous, • 8 (42.11%) -HbS/β+ Thalassemia,• 1 (5.26%) - Sickle HbS/D disease.
• Severity Of PHT• 12 (24%) Mild PHT, • 7 (14%) Moderate PHT.
Cont.…
• Patients who had raised biochemical markers of hemolysis had significant association with raised pulmonary hypertension.
• Patients with PHT had more no. of crisis as compared to those with normal pulmonary pressures. (p=0.046)
• Hydroxyurea had been started at an earlier age in patients with PHT.
Thank You