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PubMed1995

Effect of anterior cruciate ligament reconstruction with patellar tendon or prosthetic ligament on the morphology of the other ligaments of the knee joint. An experimental study in dogs.

The effect of anterior cruciate ligament replacement on the other ligaments of the knee, including the medial or lateral collateral and posterior cruciate ligaments, was studied histologically in the canine knee. The anterior cruciate ligament was replaced either with the medial third of the patellar tendon or with a prosthetic ligament. Histologic analysis was performed 3 months after prosthetic ligament implantation and 1 year after patellar ligament reconstruction. In all ligaments, numerous pathologic alterations were found. Degenerative changes such as glycosaminoglycan accumulation, metachromasia, collagenolysis, collagen necrosis, and lipomatosis were similar after 3 months and after 1 year. Granulation tissue, a sign that characterizes tissue repair, was seen only in those ligaments taken 3 months after anterior cruciate ligament surgery. Similar degenerative changes also were found in the ligaments of the knees with only a large medial arthrotomy (sham). In the contralateral control knees, the pathologic alterations were rare. Thus, open arthrotomy alone or with anterior cruciate ligament reconstruction seems to result in longstanding degenerative changes in all ligaments of the canine knee joint.

Animals↗

PubMed1995

Nuchal fibroma. A clinicopathological study of nine cases.

Nuchal fibroma apparently was first described in the second edition of Enzinger's and Weiss's text book in 1988. We have been unable to find any other accounts of this entity. We have reviewed the clinicopathological features of nine nuchal fibromas, eight of which were referred in consultation. Eight patients were men; the ages ranged from 19 to 53 years (median, 43). Patients were first seen with solitary, unencapsulated, subcutaneous swellings in the back of the neck or dorsal region measuring from 2.5 to 8 cm in maximum dimensions (median, 3.5). Macroscopically, lesions were firm, fibrofatty masses. Histologically, there were sheets of hypocellular dense collagen with interspersed mature fat, inconspicuous, small, thin-walled vessels, and entrapped nerve fibers. The picture suggested a fibrolipoma, a lipomatosis, scar tissue, or even elastofibroma, but the small amount of elastic tissue present lacked the beaded, nodular appearance of elastofibroma. Follow-up information was available in seven cases. In follow-up times from 6 months to 19 years (median, 6 years), one tumor was reexcised 4 months after an incisional biopsy and had not recurred 19 months later. None of the others recurred. The lack of a capsule, the entrapment of nerves, and the predilection for the nuchal or dorsal regions suggest that this hypocellular, benign, fibrofatty lesion is a distinct entity and not merely a lipoma.

Adipose Tissue↗

PubMedDate not supplied

[Protée syndrome associated with renal lithiasis and vesico-ureteral reflux].

BACKGROUND: Proteus syndrome is characterized by a range of various manifestations. The main ones are partial gigantism of hands and feet, nevi, hemihypertrophy, macrocephaly. Urinary tract abnormalities are exceptional. CASE REPORT: A 6 year-old boy was examined because he had presented numerous abnormalities from birth. His weight was 26 kg (+3 SD) and his height was 135 cm (+4 SD). The main abnormalities were ptosis, pterygium colli, nevi of the cervical area, plagiocephaly, frontal bossing, scoliosis, hemihypertrophy involving the skin, mucosa and bones, macrodactyly, varicose veins and lipomatosis. He underwent surgery at the age of 3 years for urinary lithiasis associated with an ureterovesical reflux on the left side, i.e. the side of hemihypertrophy. CONCLUSION: This case suggests that Proteus syndrome may be an example of ectomesodermal dysembryoplasy but it requires confirmation by a report of at least one other case of obstruction of the ureteropelvic junction on the same side as hemihypertrophy.

Abnormalities, Multiple↗

PubMedDate not supplied

Degenerative neuromuscular diseases with a late onset. Clinical, electrophysiological and morphological study.

This study carried out clinical, electrophysiological and morphological investigations (sural nerve and gastrocnemius muscle biopsies) in a group of 47 patients with neuromuscular disease of a certain or supposed degenerative origin and a late onset (over the age of 30 yrs.). It aimed the evidence of the eventual particularities of such diseases with a delayed onset. The equal involvement of sexes, the insidious onset, the clinical picture similar to that of the corresponding diseases with an onset at the usual age were observed. Regardless of the age, some interesting associations of the polyneuropathy with other diseases or its presence with in these diseases (Parkinsonism, Addison's disease, multiple symmetrical lipomatosis, etc.) were noticed, too. Electrophysiological examinations showed no particularities. Neither did the muscular morphological picture in most of the cases presenting neurogenic lesions with a moderate intensity. The sural nerve biopsy evidenced in 70% of the cases a moderately intense neuropathy of an axonal type ("dying back"), with or without secondary lesions of segmental demyelination and with the signs of a live regenerative activity.

Adult↗

PubMedDate not supplied

[Morphology of the minor salivary glands in Sjogren's disease].

Histochemical, immunohistochemical and histomorphometrical changes in sialadenitis have been studied on 33 biopsies of labial salivary glands (LSG) from patients with primary Sjogren's syndrome. The control group consisted of 10 biopsies from persons without oral cavity inflammatory diseases. A direct relation was established between xerostomia stage and the degree of LSG parenchyma substitution for the inflammatory cell infiltrate. Dependence of LSG tissue damage on local immunopathologic reaction was detected. With a highly active inflammatory reaction the changes were characterized by cellular hypersensitivity including inflammatory cell infiltrate (T-lymphocytes, macrophages, neutrophils), increased vascular permeability, destructive and proliferative changes of the intralobular ductuli and acini. Reduction in inflammatory activity was accompanied by a decrease in the number of macrophages and neutrophils, an increase in the number of plasma cells and mast cells, higher compensatory hypertrophy of the serous cells of semicircular mixed acini, secretion products status, as well as intralobular sclerosis and lipomatosis.

Adult↗

PubMedDate not supplied

Thymectomy in over 50 years-old patients with myasthenia gravis: long and short-term results in 21 patients.

Thymectomy is considered to be an important method for the treatment of myasthenia gravis in older patients when the improvement cannot be achieved with the use certain drugs such as anticholinesterasic, corticosteroids and immunosuppressive agents. In our series of 281 thymectomized patients, 13 were male and 8 female, varying their age between 50 and 73 years. The duration of the disease prior to the thymectomy varied between 30 days and 15 years; in 12 of them (57%) it lasted already 2 years. Eleven patients had the severe and eight the moderate clinical form of the disease. Nine patients were treated with corticosteroids before submitted to thymectomy. In 11 patients the histopathological changes in the thymus were evaluated: eight had thymomas, one atrophic changes, one lipomatosis and one a normal thymus. The short-term follow-up revealed important improvement in 12 patients, slight improvement in four and worsening in two; two patients died. It is concluded that thymectomy may be indicated in older patients when their condition worsens in spite of the use of corticosteroids and immunosuppressive agents, in the presence of serological markers or computerized tomography image indicating thymoma, and intolerance for corticosteroids.

Aged↗

PubMed1993

[Proteus syndrome].

We report the case of a 4-year old boy presenting with a polymalformative syndrome made of cutaneous and subcutaneous lesions associated with visceral and skeletal lesions. The cutaneous lesions consisted of 3 verrucous hamartomas, one haemangioma and one lymphangioma. The subcutaneous lesions were melting of panniculus adiposus, plantar tumefaction and diffuse lipomatosis of the peritoneal and retroperitoneal cavities. The skeletal lesions were uneven lower limbs and dorsal scoliosis. Atrophy of the intestinal villi was also found. The diagnosis of Proteus syndrome was made.

Child, Preschool↗

PubMed1977

[Acute emphysematous cholecystitis. One case (author's transl)].

Acute emphysematous cholecystitis is a rare condition. Its preoperative diagnosis is based upon radiological studies which should include a film in the dorsal decubitus position, a lateral and a film in lateral decubitus with the ray horizontal. The presence of air within the gall bladder lumen; and in a part or all of the wall. Additional findings: the presence of air in the peribiliary space; the presence of gas within the biliary tract, rarely reported, is of special interest in the case reported here. Gangrenous cholecystitis must be differentiated essentially from: 1 degree an internal biliary fistula 2 degrees air in a neighbouring organ 3 degrees lipomatosis.

Acute Disease↗

PubMedDate not supplied

Shwachman syndrome: CT and MR diagnosis.

The MR and CT features of the pancreas are described in an 18-year-old woman. Lipomatosis of the pancreas found on MR and CT and the clinical findings pancytopenia, short stature, and recurrent infections are typical for Shwachman syndrome.

Adolescent↗

PubMedDate not supplied

[Myosonographic findings in inflammatory muscular diseases].

Inflammatory myopathies are rare diseases with an annual incidence of about 1-8 per 1 million persons. However, they are of special importance in myology as they can be treated successfully. There are auto-immune mediated and infectious forms of myositis. From the clinical and myopathological view, one can distinguish acute and chronic forms: the former presenting with distinct inflammatory reactions in the muscle tissue and often causing myalgia. Muscle atrophy, the latter presenting with marked muscle weakness, often with clear muscle atrophy and mesenchymal abnormalities in the biopsy. Ultrasound well documents muscle atrophy, sometimes compensatory hypertrophy, as well as mesenchymal abnormalities such as lipomatosis and fibrosis. In very acute myositis, as in pyomyositis muscle edema and abscesses can be depicted. Typical findings in autoimmune mediated myositis are reported based on our own experiences in 75 patients and according to the literature. The degree and distribution of muscle atrophy and mesenchymal abnormalities visualized by ultrasound can give clues to the type and prognosis regarding muscle function. Additionally, ultrasound can be used for searching the optimal biopsy site.

Diagnosis, Differential↗

PubMed1996

[Pathology of the heart in AIDS. Study of 73 consecutive necropsies].

PURPOSE: To study the incidence and the etiology of the cardiac lesions in AIDS patients. METHODS: The autopsy protocols and the filled slides of the heart from 73 consecutive AIDS patients were reviewed. There were, at least, 2 slides of each heart stained by haematoxylin-eosin; when indicated, Ziehl-Nielsen, Gram and Gomori Grocott stains were used. RESULTS: No cause of death was assigned to the heart. There was involvement of the heart in 66 (90%) cases. Marked atrophy of cardiac fibers with or without lipomatosis was observed in 38 patients. Interstitial infiltrates of myocardium were present in 38 necropsies and in 13 of these cases a probable pathogen was demonstrated: cryptococcus neoforms in three cases and mycobacteria tuberculosis, atypical mycobacteria, toxoplasma' gondii, trypanosoma cruzi and cytomegalovirus in two cases each. Bacterial endocarditis was found in 4 autopsies and Kaposi sarcome in one. The pericardium was involved in 22 cases; in 12 there was only non specific mononuclear infiltration. CONCLUSION: Autopsy examination of the heart from AIDS patients revealed frequent pathologic involvement.

Acquired Immunodeficiency Syndrome↗

PubMed1996

Oculocerebrocutaneous syndrome: a case report, a follow-up, and differential diagnostic considerations.

Oculocerebrocutaneous syndrome consists essentially of orbital cysts and microphthalmia/anophthalmia, focal dermal defects, skin appendages and malformations of the central nervous system. Up to now, about 20 sporadic cases have been reported. Limited information is available on the clinical spectrum and on the natural history. We present the long-term follow-up of a 19 year-old patient who was one of the first two patients described by Delleman and Oorthuys in 1981. Furthermore, we report on a new case presenting with bilateral anophthalmia and orbital cysts, typical skin lesions, a complex brain malformation and cleft lip palate. This infant died due to the severe cerebral malformations at the age of 10 months. Encephalocraniocutaneous lipomatosis, focal dermal hypoplasia and microphthalmia with linear skin defects (MLS) syndrome are discussed as the most important differential diagnostic entities.

Adult↗

PubMed1997

[Polychlorinated biphenyls in human subepicardial fat].

A considerable relation between myocardial fatty infiltration (lipomatosis) and ischemic lesion of myocardium was proved by authors else where. This time, polychlorinated biphenyls (PCB) wee studied in subepicardial fat as well as in subcutaneous fat in thorax and abdomen of deceased person after sudden and violent death. For the time being, most published results concerned the abdominal subcutaneous fat. All the published results showed high concentrations of PCB were proved in subcutaneous fat tissue from thorax and abdominal area and specially in subepicardial fat. Topical relation of PCB deposits and heart muscle was direct in all analyzed case because of fatty infiltration of myocardium. Obviously, a direct toxic effect of PCB on myocardium can take place in stress lypolysis e.g. associated with heart infarct (in some animals hydropericardium and impairment of parenchymatous organs were observed) and prognosis of patients with ischemic lesion can get worser.

Adipose Tissue↗

PubMedDate not supplied

[Difficult laparoscopic cholecystectomy].

The study analyses different situations encountered throughout the authors personal operative experience, susceptible to generate technical and tactical difficulties during laparoscopic cholecystectomy such as: postoperative perivisceritis, obesity, difficult liver mobilization, lipomatosis, acute and scleroatrophic cholecystitis, "porcelain gallbladder". They also point out different causes capable of generating important bleeding during the operation: cirrhosis, accidental adhesion tearing, anatomical arterial variations. Above all, the study presents the possible technical and tactical solutions which they applied when dealing with the already mentioned critical situations.

Acute Disease↗

PubMedDate not supplied

[Gastrointestinal problems in elderly patients].

The following article contains a short review on gastrointestinal problems of the elderly. The diseases of the esophagus occurring in the elderly are not much different from those in younger patients. Clinically relevant in the stomach are above all bleeding ulcerations and the gastric carcinoma occurring more frequently in advanced age. The pyogenic liver abscess is diagnosed primarily in the elderly and is at a rule the consequence of an infection of the gall bladder and other abdominal sites. The hepatocellular carcinoma does not grow rapidly in the elderly, but its accompanying unfavourable survival rate at five years is also approximately 5 per cent. In the case of symptomatic cholelithiasis, older high risk patients do especially profit from minimally invasive laparoscopic surgical procedures. Today, bile duct calculi are preferably treated by endoscopic papillotomy and following extraction of the calculi. The pancreas is subjected to atrophy, lipomatosis and fibrosis at the advanced age. However, these changes are rarely of clinical relevance. A frequent problem in clinical practice is that of constipation, from which 35% of patients suffer above the age of 65 years. Another typical symptom of the elderly is the incontinence, the different causes are being discussed. In advanced age, gastrointestinal hemorrhages are mostly occurring above the Treitz's ligament. Hemorrhages of the lower gastrointestinal tract occur mostly in the form of diverticle bleedings and those of angiodysplasias in the elderly. The diverticulosis is also a disease observed in over 50 per cent of patients above 70 years, but it is symptomatic in only part of the patients. When suspecting an inflammatory bowel disease in the elderly, the possibility of a mesenterial ischemia must always be considered as differential diagnosis. The classical chronic inflammatory bowel diseases can, however, also occur at advanced age. The colon carcinoma is one of the most frequent lethal causes in the Western countries 90 per cent of the cases of colon carcinoma are found in patients older than 50 years of age. Intensive attention is therefore required in this age group.

Aged↗

PubMed1976

[Clinico-morphologic characteristics of X-linked pseudohypertrophic myopathy].

A clinical, genealogical, EMG and morphological study of the development of pseudohypertrophic myopathy linked with X-chromosome allowed one to distinguish 2 variants: 1) the malignant (Duchenne form) and 2) benign (Baker form). Although both forms have similar clinical symptoms, the Baker form has a later onset, a more favourable development and more preserved motor functions. The activity of fructosodiphosphataldolase and creatinphosphokinase in the blood serum of these patients was significantly lower than in Duchenne' myopathy. In the muscular bioptates of patients with Duchenne's myopathy as the disease progresses there is a gradual smoothening of the diameter of preserved elements at the expense of almost complete disappearance of hypertrophysed filaments. In Baker's myopathy besides expressed processes of muscular sclerosis and lipomatosis there are also hypertrophysed filaments.

Adolescent↗

PubMedDate not supplied

[Cystic fibrosis in adolescent patients].

Summary--An autopsy observation of mucoviscydosis in a female who died at the age of 17 with a prevalent affection of the bronchopulmonary system is reported. Pronounced cystic fibrosis with atrophy and lipomatosis of the pancreas was found. The cause of death was respiratory failure in spite of intensive antibacterial therapy. Bullous emphysema with frequent pneumothorax in adults with this disease considerably worsens the prognosis and requires urgent therapy which in this case was not conducted because of late hospitalization.

Adolescent↗

PubMed1998

Radioprotection of salivary glands by amifostine in high-dose radioiodine therapy.

UNLABELLED: Salivary gland impairment after high-dose radioiodine treatment is well recognized. Because differentiated thyroid cancer has a good prognosis, reduction of long-term side effects is important. This study investigated the radioprotective effects of amifostine in animals and humans receiving high-dose radioiodine therapy. METHODS: Quantitative salivary gland scintigraphy was performed in five rabbits before and up to 3 mo after high-dose radioiodine therapy applying 1 GBq 131I. Three animals received 200 mg/kg amifostine before high-dose radioiodine therapy, and two served as controls. All animals were examined histopathologically. Quantitative salivary gland scintigraphy also was performed in 17 patients with differentiated thyroid cancer before and 3 mo after high-dose radioiodine therapy with 6 GBq 131I. Eight patients were treated with 500 mg/m2 amifostine before high-dose radioiodine therapy, and nine served as controls. RESULTS: In two control rabbits, high-dose radioiodine therapy significantly reduced parenchymal function by 63% and 46% in parotid and submandibular glands, respectively. In contrast, there was no significant decrease in parenchymal function in amifostine-treated animals. Histopathologically, lipomatosis was observed in control animals but was negligible in amifostine-treated animals. Similar findings were observed in differentiated thyroid cancer patients. In nine control patients, high-dose radioiodine therapy significantly (p < 0.01) reduced parenchymal function by 37% and 31% in parotid and submandibular glands, respectively. Three patients exhibited Grade I (World Health Organization) xerostomia. In contrast, there was no significant decrease in parenchymal function in amifostine-treated patients and no incidence of xerostomia. CONCLUSION: Parenchymal damage in salivary glands induced by high-dose radioiodine therapy can be reduced significantly by amifostine. This may increase the quality of life of patients with differentiated thyroid cancer.

Adenocarcinoma, Follicular↗