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[Recovery after treatment for cirrhotic ascites : a study of 90 cases. Frequency of arterial hypertension (author's transl)].

Thirty patients with alcoholic cirrhosis, ascitic during 13.6 +/- 13 months (mean +/- S.D.) were cured of ascites and followed up during 2 to 9 years (4.3 +/- 2.7 years). Twenty six were compared with a same number of cirrhotics, matched for age and sex, who died during the year after the first admission. Many biological data show statistical difference. Nevertheless no valuable prognosis can be predicted in an individual case. The clinical improvement is associated with major, sometimes total biological recovery. Other complications of cirrhosis (gastro-intestinal bleeding, hepatoma) may occur (7 cases with 5 deaths) or alcoholic hepatitis if alcohol withdrawal is stopped (3 cases, 2 deaths). Some associated diseases look unexpectedly frequent: diabetes (4 cases), obesity (9), nodular lipomatosis (14 cases) whose frequency looks higher than that can be calculated for a similar group of healthy subjects.

Adult↗

[Weight parameters of various internal organs].

The weight of some organs [brain, heart, lungs, kidneys, liver] was ascertained in a group of 1,598 persons who died a violent death, and a comparison was made with values obtained in a group of persons who died of cardiac insufficiency. The figures found were correlated according to sex with age, obesity, lipomatosis, physical size, the presence and stage of atherosclerosis. --Within the violent death group, a correlation was also made between a subgroup with minimal or no presence of atherosclerosis and one with ascertained atherosclerosis. The average weight of the brain was found to be 1419.6 g in men, 1266 g in women; the heart--394.7 or 343 g; kidney--149.0 g or 124.1 g; lungs 1280.0 or 973.4 g; liver--1770.9 g or 1522.0 g.

Adult↗

Structural and ultrastructural changes of lymph nodes in primary lymphoedema.

Optical and electronmicroscopic investigations of the inguino-iliac lymph nodes and lymphatic vessels of the lower limb suggested a pathogenic role of lymph node structural alterations in primary lymphoedema. Most of the investigated lymph nodes showed an extensive fibrosis frequently associated with lipomatosis ectasy of medullary sinuses, a.o., estimated as primary lesions appearing on a genetically propensic ground or by developmental anomalies. Alteration of the lymphatic vessel intima, proliferation, muscle hypertrophy, subintimal fibrohyalinosis, a.o., occurred in consequence of the impeded lymphatic drainage by the primary lymph node fibrosis.

Adolescent↗

[Differential diagnosis of adnexal tumors using computer tomography].

A correct differential diagnosis of pelvic tumors is sophisticated in gynecology. The present effort concerns with introducing of computertomography in our diagnostic facilities. The ultrasound method being of value in detection of early developing ovarian tumors and showing their size as well as the extent of tissue transformation is however unprecise in comparison with CT-method which allows to evaluate the anatomical relations and tissue differences. The latter one allows differentiation of cystic tumor from dermoid cyst as well as from solid tumor. Density measurement scrutinizes with more precision endometriosis, lipomatosis and tubo-ovarial abscesses. The CT-diagnosis has been proving useful especially in differentiation between ovarian and intestinal tumors.

Adnexal Diseases↗

Influence of dietary fat on spontaneous lesions of Syrian golden hamsters.

Dietary corn oil was consumed by Syrian golden hamsters at levels of 4.5 g [low fat (LF)] or 18 g [high fat (HF)]/385 kilocalories (kcal) from 3 to 7 weeks of age followed by a diet containing 9 g [medium fat (MF)]/385 kcal for life. In other groups MF diet was given from 3 to 7 weeks of age and followed by either LF or HF for life. A separate group was fed MF continuously after 3 weeks of age. Spontaneous lesions, which were altered by these dietary protocols, are reported. An HF diet fed after 8 weeks increased the incidence of flank organ hyperplasia and prostatitis, but it decreased prostatic fibrosis in males. Consumption of HF diets after 8 weeks by females increased survival and resulted in an elevated incidence of thyroid adenomas, ovarian cell hyperplasia, vaginal papillomas, and adrenal cortical cell adenomas. In age-adjusted data the increase in ovarian cell hyperplasia and adrenal cortical cell adenomas was shown to be due to HF diet and not to be a consequence of extended survival. Periodontitis and calcification of cardiac tissues decreased in hamsters fed HF diets after 8 weeks, but cell vacuolization and hyperplasia of the anterior pituitary gland and epithelial hyperplasia in the forestomach were increased. Salivary gland adenocarcinomas were observed only in hamsters fed HF diets. Feeding HF levels, either during weeks 3-7 or after week 8, decreased osteofibrosis and otitis media and increased urinary bladder epithelial hyperplasia, adrenal cortical cell lipomatosis, and bone chondrosis. Calcification of gastric and renal arteries decreased as dietary fat levels increased either before or after 8 weeks of age in males and only when fed after 8 weeks in females. Colitis and focal glandular hyperplasia of the colon mucosa were increased in both sexes by an HF diet being given before or after 8 weeks of age.

Animals↗

Receptors for IgA on human thymus cells in myasthenia gravis.

Specific surface receptors for the Fc region of human immunoglobulin A (Fc alpha R) were identified on the thymus cells of all 15 patients affected by myasthenia gravis. A large variation of the percentage of Fc alpha R bearing thymus cells was recorded (9 . 5-66 . 0%; mean value 27 . 9 +/- 17 . 8) the high values being associated in most cases with the thymus hyperplasia and the low values with a lipomatosis of the organ. Even the lowest percentage of Fc alpha R bearing cells recorded in myasthenia gravis are considerably higher than those found in normal subjects (0 . 4-4 . 1; mean value 1 . 8) demonstrating that the expression of Fc alpha R on the thymus cells is the consequence of the disease. No attempts were made to identify the cell population bearing Fc alpha R but indirect results indicate that the cells belong to E-rosette forming population, and, in some cases, to a null cell population devoid of the ability to form E and EAC rosettes, Fc alpha R can bind various IgA ligands and the binding is confined to a small fraction of IgA (not more than 5%) considered as cytophilic IgA.

Absorption↗

Human myocardium and plasma digoxin concentration in patients on long-term digoxin therapy.

Digoxin concentration of 14 left ventricular papillary muscles and 36 right atrial appendages of 45 patients undergoing cardiac surgery were determined by the NEN 125J-radioimmunoassay. Blood specimens for digoxin assay were withdrawn immediately before operation, 12 to 16 hr after the last digitalis dose. Mean papillary muscle digoxin concentration was 76.1 ng/g +/- 25.5; mean ratio to plasma digoxin level, 36.8:1. The mean concentration of right atrial appendages was 41.8 ng/g; mean ratio, 29.9:1. Most of the tissue specimens were examined histologically using Hematoxylin-Eosin and van Gieson stains. No clear relation between tissue digoxin concentration and hisological alteration, i.e., the degree of fibrosis or lipomatosis, could be found, although most severe alterations were found in right atrial appendages corresponding to a higher variation in tissue concentrations and a poorer correlation to plasma digoxin (r = 0.47, respectively for papillary muscle r = 0.73).

Adult↗

Radiology and pathology of fat.

Conventional roentgenograms usually reveal a fatty tumor by its characteristic radiolucency. Exceptions to this presentation are the intrathoracic fatty tumors which are more often radiopaque than radiolucent. Obesity and steroids may cause focal, excessive accumulation of fat which can cause a mass effect and become symptomatic from pressure changes. Tubular widening of the superior mediastinum is characteristic mediastinal lipomatosis. Liposarcomas may or may not appear radiolucent. When a calcified retroperitoneal mass appears at least partially radiolucent, a liposarcoma must be suspected. Intrarenal fatty masses may be confused by overlapping bowel gas. Nephrotomograms, prior to the injection of contrast medium, will reveal the presence of fatty process. Obliteration of juxtavisceral or juxtamuscular fat is an important finding for localizing a disease process. CT is the most informative imaging method in establishing the presence of a fatty mass and for localizing a pathological process which infiltrates the juxtavisceral fat.

Adipose Tissue↗

[Myocardial fibrosis in cystic fibrosis. A new case report (author's transl)].

We report the case of a 9 month-old infant in whom acute heart failure was the presenting symptom of cystic fibrosis. Post-mortem examination showed lesions of myocardial fibrosis associated with typical lesions of cystic fibrosis in the pancreas. Hypoproteinemia and anemia were present and earlier could have suggested the correct diagnosis before the episode of acute heart failure. The analogy of the histologic myocardial changes in cystic fibrosis and in congenital lipomatosis of the pancreas suggests a common pathogeny related to the deficiency of the exocrine secretion of the pancreas.

Cardiomyopathies↗

[Sympathetic ganglionitis in the hereditary syndrome of QT prolongation (Romano-Ward syndrome); intracardiac registration of after-potentials (author's transl)].

A 20-year-old woman, admitted to an intensive care unit in ventricular fibrillation, had marked QT prolongation in the ECG after successful defibrillation. Family study revealed ECG signs of QT prolongation, as seen in the Romano-Ward syndrome, in four generations. Syncopal attacks only occurred in the third generation, affecting four siblings. In the described patient the intraventricular ECG revealed additional depolarizations (after-potentials) during and following repolarization (ST-T) in the surface ECG. The patient died later as a result of irreversible hypoxic cerebral damage. Invasive ECG studies in the mother also revealed after-potentials. In the patient, morbid-anatomical examination revealed, in addition to fibrosis and lipomatosis around the sinus and AV nodes, focal round-cell ganglionitis in both sympathetic trunks, but predominantly on the right. These infiltrations have not previously been described in this disease and could be of significance in the pathogenesis of the prolonged QT syndrome.

Adult↗

Adult GM2 gangliosidosis masquerading as slowly progressive muscular atrophy: motor neuron disease phenotype.

Ultrastructural and biochemical studies were performed on postmortem material of a 67-year-old woman presenting with proximal muscle weakness in the legs, slurred speech, and mental subnormality. The symptoms began at age 19 and showed extremely slow progression, mimicking progressive muscular dystrophy. A brother suffered from a similar chronic neuromuscular disease, and two sisters died at an early age from unknown "nervous" diseases. Autopsy disclosed abundant lipid accumulation in CNS neurons and severe cerebellar cortical atrophy of the granule cell type. Skeletal muscle showed a terminal stage of denervation atrophy with severe lipomatosis; intrafusal fibers of muscle spindles contained lipid deposits. Complex lamellar cytoplasmic inclusions often resembling membranous cytoplasmic bodies or stacked membranes were seen in cells of the brain. In addition, there were various lipopigment bodies, fingerprint profiles, rare polyglucosan bodies, rodlike structures, and filamentous sheaves, particularly in substantia nigra. Accumulation of gangliosides GM2 and GA2 in the cerebral cortex was demonstrated by thin-layer chromatography. Determination of hexosaminidase activity was not possible (formalin-fixed material). This observation, in addition to the cases reported by Navon et al. [1981] and Johnson [1982], is suggested to represent a new phenotype of adult-onset GM2 gangliosidosis referred to as motor neuron disease phenotype, which can be differentiated from other adult-onset lipidoses and motor neuron disorders. Our paper emphasizes the importance of ultrastructural demonstration of lamellar inclusions for the differential diagnosis of ceroid lipofuscinosis, and the value of biochemical studies in the diagnostic clarification of atypical neuromuscular disorders.

Adult↗

[Primary exocrine pancreatic insufficiency in the adult. Presentation of a case].

The authors report the case of a 37-year-old female complaining of chronic diarrhea since childhood. She had gross steatorrhea and azotorrhea . Pancreatic function tests using secretin and cholecystokinin-pancreozymin stimulation, and analysis of pure pancreatic juice collected under secretin stimulation proved exocrine pancreatic insufficiency. Oral glucose tolerance test was normal. Intestinal absorption tests, small bowel X-ray films and jejunal histology were normal. Brush-border specific enzyme activities were increased. In vitro palmitic acid uptake and esterification in the jejunal mucosa were decreased. There was no pancreatic calcification. Biliary tract was normal. Retrograde pancreatography, pancreatic ultrasonography and tomodensitometry showed no morphologic abnormality. No cause of exocrine pancreatic insufficiency could be detected. A son and a niece of the proband had chronic diarrhea and growth failure. The relationship of the described case with primary lipomatosis of the pancreas in childhood and hereditary exocrine pancreatic insufficiency in CBA/J epi mice is discussed.

Adult↗

[Spinal epidural lipoma as a cause of paraplegia].

Two cases (a 56-year-old woman and a 57-year-old man) with spinal epidural lipomas are described. In the discussion, the origin, the development and the biological valence of this spinal growth are dealt with. Besides, the age of the manifestation of the neurological symptoms, the localization, the clinical symptoms, diagnosis and differential diagnosis as well as therapy and prognosis of these tumours, which are rarely found in the spinal canal, are discussed. With the exclusion of the lipomatosis combined with lumbosacral malformations of the spinal column, the literature contains reports on about 66 cases of epidural and intradural spinal lipomas described until now.

Female↗

[Age-related radio-morphologic changes in the extremity muscles].

The authors examined the limb musculature of 133 individuals of various ages using a special soft tissue X-ray technique. In individuals under 45 years of age the broad striping indicative of group fibre necrosis in the musculature or of secondary interstitial lipomatosis was not observed at all. In older individuals, apart from a decrease in the volume of the calf musculature, diffuse striping was also characteristic, but not the rule. In certain cases fatty tissue proliferation was also observed in patches. Pathological changes in the musculature of the upper limbs due to old age were considerably rarer and less varied. The authors discuss the possible reasons and explanations for the pathological changes.

Adipose Tissue↗

Muscular changes in carcinomas of the breast.

14 samples of the Musculus pectoralis major from patients with early stages of breast cancer and 3 samples from patients with nonmalignant mammary diseases were stored in liquid nitrogen and investigated histologically, histochemically and histometrically. Main changes are lipomatosis, fibre necrosis, occasionally perifascicular atrophy, metabolic changes (e.g. accumulation of lipids), and alterations of the activity of oxidative enzymes. Histometrically we found an atrophy of type 2-fibres. There may be relations between the degree of the muscle changes and the severity of the tumour disease. The paraneoplastic muscle disease is a "concomitant myopathy" and may be regarded as a "functional myopathy". In its development we can differentiate a phase of compensation and a phase of decompensation.

Atrophy↗

[Perforation of the cardiac wall during central venous catheterization - muscular defects as a contributing factor (author's transl)].

Two cases of perforation of the cardiac wall during central venous catheterization are reported. One patient, a women, died 6 weeks after perforation of the anterior wall of the right ventricle; the other, also a woman, died immediately following perforation of the posterior wall of the right atrium by a polyethylene catheter. Both cases prove that even a seemingly correct technique and the use of soft catheters cannot entirely exclude the possibility of perforation. Relative weakness of the cardiac muscle due to lipomatosis could have been a pre-disposing factor in one case, and thinning of the atrial wall between the pectinate muscles in the other case. There is also the possibility that the spaces between the trabeculae carneae act as a trap for the catheter. Radiological control of the placement of the catheter should also be performed in those cases where the catheter had to be withdrawn because of malposition in order to make sure of its correct extracardial location.

Aged↗

[Bisalbuminemia and pseudobisalbuminemia: clinical significance (author's transl)].

Two cases of bisalbuminaemia are hereby studied: a congenital case and an acquired or transitory case. In the first one, this hereditary disturbance is studied on four affected members of one Spanish family, being associated in two of them with multiple lipomatosis. This kind of association is considered as casual. As in most of the informed cases, ours belongs to the slow kind. The second case deals with a bisalbuminaemia of transitory character or pseudobisalbuminaemia which, appeared in a woman in the course of an acute pancreatitis of biliolithiasic origin, with a pancreatic pseudocyst and pleural effusion, the evolution in the inflamatory pancreatic process being advantageous in spite of the prsence of bisalbuminaemia. Briefly revised, are exposed the causes of this interesting and rare alteration in the electrophoretic fractioning of plasmatic proteins: congenital, and adquired causes due to administration of betalactamines and during the course of acute pancreatitis.

Aged↗

[Ultrasonic patterns of giant hypertrophy of the gastric mucosa (Ménétrier's disease) (author's transl)].

Since it can display the gastric folds, as well as the gastric wall and the exogastric area, sonography is a reliable way of diagnosing the giant hypertrophy of the gastric mucosa (Ménétrier's disease). The characteristic pastterns are: --a considerable thickening of the gastric folds and wall, --a high sonolucency, --the presence of the intra-mural cysts, --some "hilly" deformations in the exogastric part of the stomach, --occasionally, a mesenteric lipomatosis.

Aged↗