Ehler's Danlos Syndrome picture

Ehlers-Danos syndrome is a group of inherited disorders characterized by excessive looseness (laxity) of the joints, hyperelastic skin that is fragile and bruises easily, and/or easily damaged blood vessels. The syndrome sometimes involves rupture of internal organs.

Facts on Primary mediastinal large B-cell lymphoma(C.T.)

Primary mediastinal large B-cell lymphoma accounts approximately 5% of large B-cell lymphoma, which are usually disseminated or present in the abdomen. There appears to be a younger age of diagnosis (30s) and female predilection (F:M 3:2), when compared to other large-cell lymphoma, which usually present in the 50s and have a male predilection (M:F 2:1).

Unfortunately from an imaging point of view, these demographics are similar to those of nodular sclerosing Hodgkin lymphoma, which is a common cause of a primarily mediastinal lymphoma.

There Clinical presentation is usually due to the mass effect of the typically large anterior mediastinal mass. SVC compression resulting in SVC syndrome is relatively common, present in up to 35% of cases.

Radiographic features
The vast majority of patients have an anterior mediastinal mass. Middle and posterior mediastinal involvement, either in isolation or contiguously with the anterior mediastinal component is uncommon.

Plain film
Chest radiography demonstrates a soft tissue anterior mediastinal mass.

CT
CT of the chest usually shows:

* soft tissue attenuating mass
* usually large at diagnosis ~10cm diameter
* mass effect common

• bowing and compression of trachea
• SVC obstruction : up to 35% cases

* cystic areas are common : up to 44%
* calcification pre-treatment is uncommon, but recognised
* chest wall invasion may occur
* pleural effusion(s) / pericardial effusion

• seen in up to a third of cases
• pleural effusion may be associated with poor outcome

“CT of the chest of 22 year old demonstrates a large mediastinal mass with significant external compression of the SVC. A moderately sized pericardi...”

Case of renal function in Diabetic

A 70 year old diabetic has chronically deteriorating renal function. An intravenous urogram reveals ring shadows and filling defects within dilated calyces. The most likely diagnosis is

• a) pelvi-ureteric obstruction
• b) horse-shoe kidney
• c) medullary sponge kidney
• d) renal tuberculosis
• e) renal papillary necrosis

The correct answer is (E)

Explanation
Clinically, renal papillary necrosis is a spectrum of disease, most common in patients with diabetes, analgesic nephropathy or sickle cell disease. Patients may have an acute fulminating illness with rapid progression or may have a chronic disease that is incidentally discovered on excretory urography. Some patients may chronically pass necrotic tissue in their urine , and some may never pass papillae. Although the diagnosis may be made from the passage of necrotic papillae in the urine, most often it is made from the excretory urogram. The radiographs show various degrees of renal involvement with either medullary or papillary changes causing irregular sinuses or medullary cavities or classic ring shadows. Retained necrotic papillae may calcify, especially in association with infection. Furthermore, this necrotic tissue may form the nidus for chronic infection. Opportunistic fungal infections have been reported. Renal sonography may be useful to diagnose papillary necrosis.