He was first evaluated at our center in 2012 at 4 months of age with a perinatal history of cholestasis and hypoglycemia; severe jaundice (Kramer scale score of 5); severe hepatosplenomegaly (liver and spleen 5 cm below the costal margin
); failure to thrive, weighing 5150 g (z score of -2.51) and measuring 60.5 cm (z score of -1.43), with a BMI of 14.1 (z score of -2.35); blood dyscrasia; impaired liver function, with severe hepatic and canalicular enzyme level elevations; and very high total cholesterol, low HDL-C, and high LDL-C levels.
The T6-8 supply the area below the xiphoid and parallel to the costal margin
; T9-12 supply the periumbilical area and the lateral abdominal wall between the costal margin
and iliac crest; L1 supplies the anterior abdomen near the inguinal area and thigh .
VHW brown with ripple pattern covering almost all surface; basal area as in FW; marginal area with the proximal half darker, central area mixed with ochre, and distal half bluish; 2 or 3 black small spots in basal region; convex dark and fine basal line between 3A and inner margin; sub-basal darker curved line from costal margin
to the anal margin, as the same line of the FW; post-discal band similar to VFW with proximal line convex from Cu[A.sub.1] to anal margin, where it is enlarged; 2 ocelli in Rs-[M.sub.1] and [M.sub.1]-[M.sub.2] with a small white pupil in each one; 3 creamy spots in [M.sub.2]-[M.sub.3], [M.sub.3]-Cu[A.sub.1], and Cu[A.sub.1]-Cu[A.sub.2] and 2 smaller similar in Cu[A.sub.2].
1d): Triangular, longer than hind wing, costa widely arched, apex round, termen slightly concave, discoidal cell elongated, covered half length of wing; veins, Sc (Subcosta) arises from the base of wing, ending at the middle of the costal margin
, R (Radial) arises next to and parallel to Sc, at distally forks into R1 and Rs, later being divided into R2, R3, R4 and R5, R2 and R3 parallel and ending before apex of the cell, R4 ending at the apex of the cell, R5 ending on the terminal margin, M1 begins from upper apex of discal cell, M2 begins from middle of the discal cell, M3 begins from lower apex of the discal cell, Cu1 and Cu2begin separately from discal cell for an unequal distance, A2 arises from the axillary region, separately from the distal cell up to the tornus of the wing.
With the line of electrodes 4 cm dorsal to the midaxillary line and a line along the rostral side of the costal margin
, abdominal pressures >90 cm [H.sub.2]O continued to be induced; however, endotracheal tube pressures were <90 cm [H.sub.2]O, showing some reduced transmission of abdominal pressures to the trachea.
The liver was palpable 3cm below the costal margin
but there was no splenomegaly.
The criteria for the diagnosis of HMS are: splenomegaly (>10 cm below the left costal margin
), increased IgM that exceeds twice the reference value, high titers of antibodies specific against Plasmodium sp.
There was hepatomegaly 4 cm below the right costal margin
. No dysmorphic features were observed.
Liver edge was 5 cm below right costal margin
, with a span of 11 cm, firm in consistency, non-tender, and no audible bruit.
The operation table was flexed just above the level of the iliac crest to maximally widen the space between the iliac crest and the costal margin
for the best possible port access.
On his physical examination, he had mild hepatomegaly (2 cm below the costal margin
) and massive splenomegaly (18 cm below the costal margin