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Sensory, motor, and autonomic neuropathy in patients with multiple symmetric lipomatosis.

Clinical evaluation of 33 male patients affected by multiple symmetric lipomatosis has revealed a previously unreported high prevalence of somatic and autonomic neuropathies. In 84% of the patients, clinical examination revealed signs or symptoms of neural disturbances, ranging from a vibratory sensory loss to severely incapacitating trophic ulcers or Charcot's arthropathy. Electrodiagnostic investigations demonstrated a significant reduction of motor and sensory conduction velocity in the peroneal and sural nerves. Morphometric studies of nerve and muscle biopsies from five patients with multiple symmetric lipomatosis revealed a significant reduction in myelinated fiber density (4435 +/- 593 fibers/mm2 in MSL vs 7660 +/- 800 in controls; p less than 0.05), a selective reduction in the large fibers of 7 to 10 micron in diameter, and signs of chronic denervation-reinnervation processes. Bedside tests for autonomic neuropathy were abnormal in 15 of 20 patients studied. Metabolic studies in these patients confirmed a significant increase in plasma high-density lipoprotein fractions consistent with the diagnosis of hyperalphalipoproteinemia, and a significant reduction in plasma low-density lipoprotein fractions (hypobetalipoproteinemia) associated with a marked enhancement of lipoprotein lipase activity in adipose tissue. Thus, a metabolic factor has to be considered in the pathogenesis of MSL neuropathy.

Adult↗

Congenital infiltrating lipomatosis of the face: clinicopathologic evaluation and treatment.

Congenital lipomatosis of the face is characterized by collections of nonencapsulated, mature lipocytes which infiltrate local tissues and tend to recur after surgery. These lesions represent a distinct clinicopathologic entity that has not been previously reported in this location in children. Three children with congenital lipomatosis of the face were treated at the Institute of Reconstructive Plastic Surgery over a 2- to 14-year follow-up period. In each instance, pathologic evaluation by light and electron microscopy revealed similar lesions sharing the following morphologic criteria: (1) nonencapsulated tumors containing mature fat cells, (2) infiltration of adjacent muscle and soft tissue, (3) absence of malignant characteristics, (4) absence of lipoblasts, (5) presence of fibrous elements in conjunction with increased numbers of nerve bundles and vessels, and (6) hypertrophy of subjacent bone. All three lesions recurred after numerous excisions, some of which were extensive. All were benign by histologic examination and remained so for as long as 14 years. Surgical treatment improved the aesthetic appearance of each child despite evidence of tumor persistence. Although these tumors are benign, we recommend an early aggressive surgical approach to control the infiltrative nature of their growth and to improve facial appearance.

Child↗

Lipomatosis of the pancreas: an unusual cause of massive steatorrhea.

Fat replacement of the exocrine pancreas is a rare cause of exocrine pancreatic failure. We report two adult patients (a 25-year-old woman and a 63-year-old man) with weight loss and massive steatorrhea in whom abdominal computed tomograms were diagnostic of pancreatic lipomatosis. In both patients, oral pancreatic enzyme replacement in association with cimetidine led to a marked reduction of steatorrhea and weight gain. Pancreatic lipomatosis should be suspected in cases of severe exocrine pancreatic insufficiency in the absence of abdominal pain and diabetes. Computed tomogram scanning should lead to an increasing detection rate of this unusual condition.

Adult↗

Nontropical chyluria associated with pelvic lipomatosis.

A patient never exposed to filarial parasites presented with intermittent chyluria and was found to have coincidental pelvic lipomatosis. The pathophysiology of chyluria and pelvic lipomatosis are reviewed and their possible relationship in the present case is discussed.

Aged↗

Pelvic lipomatosis with ureteral encasement and recurrent thrombophlebitis.

Multiple complications occurred in a patient with severe pelvic lipomatosis and recurrent superficial thrombophlebitis. Distal ureteral and rectal encasement by lipomatous infiltration caused obstructive uropathy and rectal deformity without substantial compromise of renal or gastrointestinal function. Clinical evidence suggests an association between pelvic lipomatosis and recurrent superficial thrombophlebitis.

Humans↗

Mediastinal lipomatosis.

Mediastinal lipomatosis is a benign condition characterized by a large amount of mature adipose tissue within the mediastinum. It widens the mediastinum and may simulate mass lesions, thus leading to diagnostic errors. We describe a new case of a huge mediastinal lipomatosis and review 36 cases previously reported in the literature. Computed tomography has an important role in the diagnosis of this disease. To avoid invasive and unnecessary procedures, this diagnosis should be considered in any patient with Cushing's syndrome.

Adipose Tissue↗

Epidural lipomatosis in steroid-treated patients.

Epidural lipomatosis is a condition in which excess adipose tissue is deposited circumferentially about the spinal cord in the epidural space. It is most frequently seen in patients on chronic steroid treatment for a variety of medical problems and can present as nonspecific back pain, radiculopathy, or frank spinal cord compression. Diagnosis and treatment have generally relied on multi-level decompressive laminectomy after myelography and computed tomography. The immunocompromised state and the reported postoperative mortality (22%) of these patients, however, suggests that nonoperative therapy may be preferable whenever possible. Five cases of epidural lipomatosis are reported, and previous literature is reviewed for presentation, evaluation, and treatment of this condition.

Adult↗

Replacement kidney lipomatosis after renal transplantation.

Renal sinus lipomatosis consists of abnormal fatty proliferation of the renal sinus, hilus, and perirenal spaces. Its pathogenesis is unknown, although it is generally associated with aging, renal atrophy, and long-standing chronic inflammation or urinary tract infection. Although this condition is rare, it may be important to recognize it in renal transplant recipients. We describe three cases of replacement kidney lipomatosis that occurred after cadaveric renal transplantation and discuss the possible differential diagnosis of this condition.

Adult↗

Encephalocraniocutaneous lipomatosis: a new neurocutaneous syndrome.

Encephalocraniocutaneous lipomatosis is a congenital neurocutaneous disorder with the distinguishing histopathological features of dysgenesis and neoplasia of the adipose tissue. The dominant clinical features of the syndrome include convulsions beginning in infancy, mental retardation, and unilateral cutaneous and ophthalmological lesions with ipsilateral cerebral malformations. A patient with this rare disorder of ectomesodermal dysgenesis has been studied in order to classify clinically and histologically the associated skin lesions. To our knowledge, this is the fourth case of encephalocraniocutaneous lipomatosis reported in the English literature.

Brain↗

Adrenergic regulation of fat-cell lipolysis in multiple symmetric lipomatosis.

The responsiveness towards catecholamines of normal and lipomatous fat cells of two male subjects suffering from multiple symmetric lipomatosis has been studied by means of a newly developed bioluminescent method for microdetermination of glycerol. In contrast to previous findings, lipomatous fat cells displayed normal alpha 2-and beta-adrenergic responsiveness in both subjects studied. The apparent discrepancies between the results of the present study and those reported by others therefore support the contention that multiple symmetric lipomatosis is a symptom with various underlying causes rather than a nosological entity.

Adipose Tissue↗

Bannayan syndrome--generalized lipomatosis associated with megalencephaly and macrodactyly.

This paper reports an autopsy case of a 5-year and 11-month-old girl with generalized subcutaneous lipomatosis, megalencephaly, and macrodactyly. Marked emaciation and poor prognosis were the characteristic findings of this case. We thought that this case was identical with a rare syndrome which was initially reported by BANNAYAN in 1971. Autopsy disclosed diffuse lipomatosis in the thoracic and abdominal cavity, and infiltration of fat tissue to the pancreas. Hyperplasia of the small intestinal mucosa and Peyer's patch, and a pedunculated polyp of the sigmoid colon were the unique findings which could not be seen in the previously reported cases.

Abnormalities, Multiple↗

Lipomatosis of the bladder presenting as bladder cancer.

A case of bladder lipomatosis in an 81-year-old man is presented. The preoperative diagnosis was bladder tumor. A transurethral resection of the bladder was performed and a pathological examination revealed lipomatosis of the bladder. This entity is extremely rare and, to our knowledge, this is the second case reported in the English published works.

Aged↗

Encephalocraniocutaneous lipomatosis: case report and review of the literature.

Encephalocraniocutaneous lipomatosis is a congenital disorder characterized by unilateral cerebral malformations and ipsilateral scalp, face, and eye lesions. Distinguishing histopathologic features are dysgenesis and neoplasia of the adipose tissue. A Caucasian boy had soft tumors and elastic papules on his head since birth, associated with atrophic areas, and a bilobed lesion on the upper right eyelid. On the bulbar conjunctiva of the right eye, an oval 6-mm lesion was present. Ultrasonogram, computerized tomographic scan, and magnetic resonance imaging revealed a dilation of the right lateral ventriculus, a mass on the pontocerebellar angle, agenesia of the corpus callosum, an arachnoidal cyst on the right hemisphere, microcalcifications, and pachygyria. The histology of a soft cutaneous tumor was consistent with a fibrolipoma, and dispersed extracellular lipid globules in the upper dermis were found on electron microscopy. The diagnosis suggested by these findings was encephalocraniocutaneous lipomatosis. Even in view of the rarity of the syndrome (11 cases described in the literature), this patient seems unusual because of the bilateral distribution of the cutaneous lesions and because of the agenesia of the corpus callosum. The peculiar ultrastructural findings require further confirmation.

Brain Neoplasms↗

Encephalocraniocutaneous lipomatosis: clinical spectrum of systemic involvement.

Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome with involvement of ectomesodermal tissues. Unilateral lipomatous hamartomas of the scalp and eyes are the hallmarks of this disorder. Associated findings are extremely variable, ranging from minor abnormalities to severe, debilitating disease. We report two unrelated Brazilian patients with encephalocraniocutaneous lipomatosis, one presenting with the full-blown spectrum of cutaneous, ocular, and central nervous system manifestations, and the other with minor extracutaneous manifestations after a 5-year follow-up period.

Abnormalities, Multiple↗

Spinal epidural lipomatosis. Case report and review of the literature.

Spinal epidural lipomatosis (SEL) is a condition in which excess adipose tissue is deposited circumferentially about the spinal cord in the epidural space. It is most frequently seen in patients on chronic glucocorticosteroid therapy for a variety of medical problems. This is a case report of lumbar epidural lipomatosis in a bodybuilder with radiculopathy supposedly induced by anabolic steroid intake.

Adipose Tissue↗

Diffuse lipomatosis in the leg after poliomyelitis.

Two elderly women with gradual enlargement of the leg following paralytic poliomyelitis are reported. From the clinical appearance, plain radiograms and angiographic studies the enlargement was thought to be caused by adipose tissue. On exploration the muscles within the calfs were found to be entirely replaced by adipose tissue. Histological examination revealed diffuse lipomatosis involving skeletal muscle, fasciae and in one case even periosteum and bone. Peripheral nerves exhibited fibrosis and myelin degeneration and vessel walls showed unusual angioma-like changes. Since it is known from the literature that peripheral nerve lesions of different kinds can be followed by adipose overgrowth we believe that there is a pathogenetic relationship between poliomyelitis and the diffuse lipomatosis in the present cases.

Adipose Tissue↗

Epidural lipomatosis in a six-year-old dachshund.

A six-year-old female dachshund was examined because of intermittent lameness in its left pelvic limb and periodic back pain. Myelography, epidurography and computed tomography (CT) revealed a dorsal displacement of the dural sac in the lumbosacral region caused by a soft tissue mass which had the specific density of fat. The mass was removed via a dorsal laminectomy in the lumbosacral area and a histological examination confirmed that it was adipose tissue. The clinical signs resolved after the surgery and a follow-up CT five months later showed no evidence of compression of the dural sac. The diagnosis of epidural lipomatosis in this dog was based on the clinical findings, the results of diagnostic imaging, and the surgical and histological findings, all of which revealed many similarities with epidural lipomatosis in man.

Animals↗

Replacement lipomatosis of the kidney: diagnosis by computed tomography and sonography.

The sonographic and computed tomographic features in a case of replacement lipomatosis of the kidney are presented, along with pathologic correlation. Computed tomography demonstrated a staghorn calculus and marked atrophy of the renal parenchyma, along with diffuse increase in renal sinus and perirenal fat. Sonography showed thinning of the renal parenchyma and high-amplitude echoes throughout the kidney, corresponding to the increase in renal sinus fat. The prospective diagnosis of replacement lipomatosis, and differentiation from other causes of nonfunction of the kidney due to staghorn calculus, can be readily made.

Adult↗