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[Angiographic differential diagnosis of hydronephrosis (author's transl)].

The diagnosis of hydronephrosis is made by excretory urography with late films and retrograde pyelography. Renal arteriography in hydronephrosis permits exact evaluation of vascular supply and parenchymal thickness. In three cases selective arteriography was performed because of a non-functioning kidney or suspected space-occupying lesion. The combination of arteriogram and urogram--if necessary together with percutaneous puncture--allows to differentiate between hydronephrosis, avascular tumors, renal cysts, polycystic renal disease, renal abscess, subcapsular renal hematoma, fibrolipomatosis, xanthogranulomatous pyelonephritis, and tuberculosis.

Abscess↗

[Contusion of the ulnar nerve with fibro-lipomatous hypertrophy. A case report].

The autor describes a case of compression syndrom of the ulnar nerve at the elbow after a blow-injury. At the operation the nerve presented a considerable fibro-lipomatous intraneural hypertrophy with extention over almost the whole length of the extremity. Furthermore, a similar case of the deep branch of the radial nerve is mentioned, presenting the same pathological condition. Till now, only cases affecting the median nerve, have been described in the world literature.

Adult↗

Launois-Bensaude syndrome (Madelung's disease).

A case of Launois-Bensaude Syndrome (Madelung's disease) in a chronic alcoholic is presented and the typical features are discussed. This entity should be considered in patients who present with doughy, symmetrically distributed, adipose masses.

Alcoholism↗

Naevus superficialis lipomatosus. A clinicopathological report of twenty cases.

Naevus superficialis lipomatosus (NSL) is a rare malformation consisting of multiple or solitary papules usually occurring on the lower trunk or gluteal regions. In this study the clinical features of twenty new cases are described with a detailed account of the histopathology. Notable were the marked variability in the amount of ectopic fat found in the lesions, and the presence of associated abnormalities of the connective tissue, the vessels, and the skin appendages. In early lesions, mononuclear cells differentiating into lipoblasts could be identified around proliferating capillaries. Two clinical types were distinguished. First, the classical type (7 cases) consisting of multiple lesions occurring in a zonal distribution, and secondly the solitary papule or nodule (13 cases). The latter type can be difficult to distinguish from a skin tag with fatty herniation.

Adipose Tissue↗

Combined orthognathic and facial aesthetic surgery with case reports.

There are multiple hard- and soft-tissue considerations in facial surgery. The expertise of the oral and maxillofacial surgeon in treating patients with multiple facial injuries has shown time and time again that hard- and soft-tissue reconstruction can be performed either as simultaneous or as delayed procedures. The benefits of a total facial analysis approach to facial surgery are based upon aesthetic, functional, rejuvenative, and self-esteem issues. Proper diagnosis coupled with a comprehensive treatment plan utilizing a team approach provides for both simultaneous and delayed procedures to be completed and can provide for safer and more predictable outcomes. The expanding range of possibilities exists to provide patients with treatment options for improvement in the aesthetic, functional, and rejuvenative aspects of their facial features. A rather fast metamorphosis has taken place from the late 1960s to the present period. This has carried orthognathic surgery from its infantile state of a technical procedure to a sophisticated surgical procedure(s) creating an outcome that is unsurpassed by any other specialty.

Adult↗

Launois-Bensaude syndrome in a female with type 2 diabetes.

BACKGROUND: The Launois-Bensaude syndrome (LBS) is a rare disease characterized by an accumulation of multiple lipomata with a preponderance at the thorax, abdomen, upper arms and the thighs. Frequently, the condition is associated with past or present alcohol abuse with no clear temporal coincidence between the onset and termination of lipomata growth and onset or termination of alcohol consumption. Due to the massive accumulation of adipose tissue, the patients frequently have features of the metabolic syndrome as hypertension, impaired fasting glucose or diabetes mellitus, hyperuricemia or hyperlipidemia. CASE REPORT: A 79-yr-old female observed an increase of fat mass especially at the upper arms, the thighs and the thorax in combination with a weight gain of 19 kg within 2 years without any changes in the nutrition habits. The unique features confirmed the diagnosis of LBS. Interestingly, she had diabetes mellitus, hypertension and hyperlipidemia before the manifestation of LBS and without any history of heavy alcohol consumption. Furthermore, the condition predominantly affects males. In her very case, treatment with sultanol, as successfully performed in some cases, could not be recommended due to her cardiac insufficiency. A surgical approach is of limited value due to the frequent relapses of the lipomata. CONCLUSIONS: Establishing the diagnosis of LBS is an important step for patients confronted with an inexplicable physical disfigurement that is not related to excessive nutrition. The disease is often not diagnosed because of its rareness, but its features are unique and easily to be distinguished from 'simple' truncal obesity.

Aged↗

[Nasu Hakola disease: a report of the first two cases in Bolivia].

INTRODUCTION: Nasu Hakola disease (NHD) is a progressive dementia that presents accompanied by bone cysts and, at random, epilepsy. It is an autosomal recessive hereditary disease and its genetic defect is located at the 19q13.1 chromosome. The genetic mutation was identified at DAP 12. It appears that DAP 12 is expressed in the microglial activation and the differentiation of macrophages in the central nervous system and, at the same time, in the osteoclasts in charge of bone remodelling. This double character consisting of dementia and bone cysts, which contain triglycerides and thin PAS positive membranes in a bone with cortical erosion and medullary hypoplasia, enables us to differentiate this disease from other frontotemporal neurodegenerative disorders, such as Pick s disease. At the same time this also allows it to be distinguished from multiple sclerosis, metachromatic leukodystrophy, Marchafava Bignami disease, and prion diseases (such as new variant Creutzfeldt Jakob). CASE REPORTS: In this paper we describe two cases of NHD, also known as polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy, in which progressive dementia, bone cysts and epilepsy were identified. Serious brain atrophy was found and confirmed by imaging studies and brain biopsies, which were also used to rule out other degenerative diseases of the frontal lobe, as well as Creutzfeldt Jakob disease. CONCLUSIONS: Both cases meet all the necessary criteria to satisfy a diagnosis of NHD. This is a hereditary, little known disease whose genetic alterations (i.e. mutations) are still in need of further study. It mainly affects males, who suffer the onset of dementia in their thirties. The neurological disorders constitute a frontal syndrome, due to predominant prefrontal involvement, and they occur in the dorsolateral area, with disorders affecting the executive and planning functions; in the orbitofrontal area, which is reflected in social maladjustment and clear obsessive compulsive traits; and also in the medial or cingulate area, which manifests itself as apathy and lack of motivation. When dealing with this disease, in addition to symptomatic therapy, genetic counselling is also important.

Adaptor Proteins, Signal Transducing↗

[Fatty replacement of the pancreas in a patient with asymptomatic pulmonary cystic fibrosis].

The authors report the case of a 26-year-old woman with hepatomegaly, who had recurrent and progressive nausea and abdominal pain. Computed tomography and magnetic resonance imaging demonstrated fatty replacement of the entire pancreas resulting from cystic fibrosis. The pulmonary disease was totally asymptomatic. Fatty replacement of the pancreas is the most frequent pattern in older patients with cystic fibrosis. This pattern correlates with pancreatic exocrine dysfunction.

Adipose Tissue↗

CT diagnosis of macrodystrophia lipomatosa. A case report.

Radiographs and CT scans of a 45-year-old male with progressive enlargement of his right upper limb and shoulder are presented. Extensive soft-tissue hypertrophy with linear radiolucent bands (fat) limited to the lateral aspect of the limb were seen. Exostoses-like bony overgrowth were also seen along interphalangeal joints. At CT, hypertrophic adipose tissue intermingling with muscle fibers was demonstrated, a diagnostic finding distinguishing the lesion from plexiform neurofibrolipomatosis, Klippel-Trenaunay syndrome and other angiomatous lesions.

Arm↗

[Computed tomography of retroperitoneal neoplasms].

CT findings of retroperitoneal neoplasma in 50 cases (51 tumors) were reviewed. There were 28 (55%) malignant tumors and 23 (45%) benign ones. MFH and liposarcoma were the most common malignant tumors and neural origin tumors were the most common benign ones. Differentiation is difficult on the basis of CT features alone. Benign tumors were usually smooth and well defined, and malignant ones ill-defined, irregular in shape, heterogenous in density with massive necrosis. The characteristic CT appearance of liposarcoma is the CT attenuation value by fat density. Neural origin tumors are usually located near the spine. They may have thick wall cystic appearance or are dumbbell shaped, showing expansion or extrinsic pressure to the adjacent bone structures. MFH, hemangiopericytoma and other malignant tumors may have marked enhancement after contrast administration. Non-resectability is shown as: 1. big vessels encased by tumor over 90 degrees, 2. adjacent organs or structures invaded by tumor, 3. multiple masses, and 4. huge tumor invading into the pelvis. Local recurrence is common after surgery. Follow-up CT scans every 6 months in a 2 year period is suggested for early detection of recurrence.

Adult↗