As it is evident from Table 1, the presence of one or more of the above conditions can be suspected when progressive psychomotor retardation, visceromegaly
, facial dysmorphism, skeletal abnormalities, or ocular findings are present.6
No lymphadenopathy or visceromegaly
. On investigations, he was confirmed to have APL (see Table 1).
Height, weight, blood pressure and urine analysis; evidence of corneal opacification should be sought; oculomotor gaze palsies and pingueculae; presence of jaundice, pallor, ecchymoses, purpura and petechiae; spinal deformity, defects of joint movement particularly hip assessment; presence of visceromegaly
(hepatosplenomegaly) and signs of chronic liver disease; neurological evaluation to include symptoms of parkinsonism for evaluation of type of Gaucher disease; and quality of life questionnaire.
The abdomen was soft, painful on palpation, with no epigastric visceromegaly
. The lower limb extremities showed mild grade II edema, bilateral inguinal lymphadenopathy of 5 cm, painful on palpation.
Right hypochondrium was especially examined for assessment of Murphy's sign, palpable mass and visceromegaly
. Systemic review was also done to see any co-morbidity.
Foam-cell infiltration and visceromegaly
are common features in all forms, but neurologic involvement occurs only in types A and C and not in type B.
ART-naive HIV-positive children with large visceromegaly
or l ymph nodes
She had neither lymphadenopathy nor visceromegaly
. Diagnostic testing found leukocyte count 12,600 cells/[mm.sup.3], CD4+ cells within normal limits, and no immunosupression.
Abdomen was soft and depressible with no tenderness or palpable masses or visceromegaly
. The rest of the physical examination was unremarkable except for a slow ambulation with the help of a cane.
(40,41) The visceromegaly
and hematologic abnormalities usually respond quite well to this treatment within one year.
ISSD is clinically distinct and very serious (1, 2); visceromegaly
, coarse facial features, failure to thrive, and early death are its main features.