The first symptom in most patients with NPD-A is hepatosplenomegaly
which is typically observed by the age of 3 months.
The patient is a male, half-brother of the patient in case 1, currently 11 years old, onset of symptoms at age 3, presenting with hepatosplenomegaly
. In his first evaluation at our center at 4 years of age, clinical examination showed enlarged abdominal circumference, round liver 5 cm below the right costal margin, spleen 3 cm below the left costal margin, and growth deficit, weighing 15 kg (z score of -1) and measuring 96 cm (z score of -2.4) with a body mass index (BMI) of 16.5 (z score of 0.92).
It showed massive hepatosplenomegaly
with multiple splenic infarctions (Fig.
with hepatic steatosis can be demonstrated due to lipid accumulation.
The diagnosis of HLH must be based on clinical and laboratory criteria, according to the 2004 guidelines of the Histiocyte Society, which include continuous fever, lymphadenopathy and hepatosplenomegaly
, cytopenias that affect at least two or three cell lineages, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis, low NK-cell activity, hyperferritinemia, and high soluble interleukin-2-receptor (soluble CD25) levels (5).
Clinical exam also revealed hyperreflexia, hepatosplenomegaly
, and diffuse supracentimetric lymph adenopathy.
It tends to occur in younger patients with hepatosplenomegaly
, systemic symptoms, and cytopenia.
Secondary syphilis manifests as generalized lymphadenopathy, hepatosplenomegaly
, arthralgia, optic neuritis, meningitis, glomerulonephritis, and so on.
Motor system upper limb, cerebellum which is performed by the trainer and then rehearsal is carried out in groups, systemic examination related to CVS, Auscultation of pericardium, superficial and deeply palpation, visceral palpation, Motor System (Lower limb), cerebellum and gait, Auscultation, examination of musculoskeletal system, visceral palpation plus causes of hepatosplenomegaly
, sensory system, demonstration of dermatomes, investigations related to respiratory system, discussion on investigations related to CVS, Percussion, auscultation of abdomen and genitourinary system, cerebral examination, Positioning, GPE, Inspection, Palpation, Percussion and Auscultation of GIT, CVS and respiratory systems.
Abdominal examination revealed mild hepatosplenomegaly
. Rest systemic examination including cardiovascular, respiratory and nervous systems, was normal.
He was having hepatosplenomegaly
, deformed thorax, and widened wrist joint [Figures 1 and 2].