Disease Severity Scoring System (DSSS) of Cappellini et al for Non-Transfusion-Dependent Thalassemia (NTDT): Comorbid Conditions

Description

Cappellini et al for developed a Disease Severity Scoring System (DSSS) for patients with non-transfusion-dependent thalassemia (NTDT). One component deals with comorbid conditions.The authors are from the University of Milan, University College London, American University of Beirut, University of Athens, Mahidol University (Bangkok) and the University of Cagliari.

Patient selection: non-transfusion-dependent thalassemia (NTDT)

Parameters:

(1) splenomegaly

(2) renal (estimated GFR)

(3) nephrolithiasis

(4) pulmonary hypertension

(5) leg ulcers

(6) endocrinopathies

(7) skeleton

(8) extramedullary hematopoiesis

Endocrinopathies:

(1) diabetes mellitus (insulin dependent vs not)

(2) hypoparathyroidism

(3) hypogonadism and >= 16 years old

(4) hypothyroidism

(5) adrenal insufficiency

(6) growth hormone deficiency

Skeleton:

(1) final height below the 3rd percentile on standardized chart

(2) osteopenia or osteoporosis

(3) skeletal deformity

(4) bone pain

(5) pathologic fractures

(6) dental malocclusion

(7) otitis media

(8) chronic sinusitis

Parameters	Findings	Points
splenomegaly	no spleen	0
	not enlarged	0
	extent 12 to 20 cm by ultrasound	1
	extent > 20 cm by ultrasound	2
estimated GFR	>= 60 mL/min/1.73 sq m	0
	15 to 59.5	1
	< 15 mL/min/1.73 sq m	2
nephrolithiasis	absent	0
	present	1
pulmonary hypertension	absent	0
	TRV 2.6 to 3.2 m/s	1
	TRV2.6 to 3.2 m/s AND other echocardio features	2
	TRV > 3.2 m/s	2
	pulmonary hypertension confirmed on cardiac catheterization	2
	currently active and scored 2 points	add 1
leg ulcers	absent	0
	present	1
	currently active	2
endocrinopathies	none	0
	1 or 2 by history	1
	insulin dependent diabetes mellitus	2
	3 or more by history	2
	currently active	add 1
skeleton	absent	0
	1 or 2 by history	1
	3 or more by history	2
	currently active	add 1
extramedulalry hematopoiesis	absent	0
	present by X-ray	1
	compressed structures by history	2
	currently active	add 1

where:

• TRV = tricuspid regurgitant jet velocity

subscore =

= SUM(points for all 8 comorbid parameters)

Interpretation:

• minimum subscore: 0

• maximum subscore: 19

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Disease Severity Scoring System (DSSS) of Cappellini et al for Non-Transfusion-Dependent Thalassemia (NTDT): Comorbid Conditions

Description

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