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PubMed1987

[Hemosiderin findings in the liver, spleen and lung in newborn infants and infants].

The content and distribution of hemosiderin in the liver, spleen and lungs of stillborn children, newborns and infants were determined histologically by Perl's method (Prussian blue reaction). We describe a study of 59 deaths up to the age of 2 years. The hemosiderin content depends on age and decreases with increasing age. No relevant differences were found in cases of sudden infant death syndrome (SIDS). In the liver hemosiderin is localized preponderantly in the periphery of the lobules. The spleen shows a diffuse siderosis of the red pulp. Minimal intrapleural, septal and perivasal depositions of hemosiderin can be found in the lung tissue. The significance of these findings is discussed.

Female↗
PubMedDate not supplied

MR of the kidneys, liver, and spleen in paroxysmal nocturnal hemoglobinuria.

The magnetic resonance (MR) findings in the liver, kidneys, and spleen in eight patients with paroxysmal nocturnal hemoglobinuria (PNH) were retrospectively reviewed to determine whether characteristic features could be demonstrated. Eight patients underwent abdominal MR examinations by gradient echo sequences (seven patients), spin-echo sequences (seven patients), and inversion recovery (one patient). Signal intensities of the kidneys, liver, and spleen were visually evaluated. Autopsy and liver biopsy correlation were available in one case each. Renal signal intensity was decreased in all eight patients by either gradient-echo or T2-weighted sequences and in the single inversion recovery sequence. Hepatic signal intensity was decreased in three of eight patients on spin- and gradient-echo images. Splenic signal intensity was decreased in three of eight patients on spin- and gradient-echo images, and in two of these was manifest as focal low signal spots (Gamna-Gandy bodies). While the signal intensity in the renal cortex is typically decreased in patients with PNH, signal intensities in the liver and spleen are variable. Low signal intensity in the kidneys is due to hemosiderin deposition resulting from intravascular hemolysis, whereas low signal intensity in the liver or spleen may be due to either transfusion siderosis, or as a consequence of hepatic or portal venous thrombosis.

Adolescent↗
PubMed1981

[Morphology and diagnosis of Zellweger syndrome. A contribution to combined cytochemical-finestructural identification of peroxisomes in autopsy material and frozen liver tissue with case report].

A female newborn, the second child of healthy non consanguineous parents, exhibited muscular hypotonia, areflexia, apathy, seizures, hepatomegaly and failure to thrive since birth. The peculiar skull shape was lacking. In the urine pipecolic acid and trihydroxycoprostanoic acid were excreted. At the age of seven weeks she died of bronchopneumonia. Lightmicroscopy revealed malformations and deficiency of myelinisation in the brain, renal cysts and fatty metamorphosis in the enlarged liver, which showed only minimal siderosis. Ultrastructurally no peroxisomes could be found in liver and kidney. No peroxisomes were detected by histochemical demonstration of catalase in frozen liver tissue which was taken immediately after death and stored for three months. Absence of peroxisomes is pathognomonic for the cerebro-hepato-renal syndrome of Zellweger and occurs in the liver irrespective of duration and degree of liver damage. It is best demonstrated by enzymehistochemical electron microscopy. With this method peroxisomes can be visualized even 30 h post mortem. In deep frozen normal liver tissue the activity of catalase remains very stable and enables the identification of peroxisomes even after a 12 months period of storage. In the cerebro-hepato-renal syndrome of Zellweger, frozen liver tissue should be stored for biochemical and diagnostic enzymehistochemical studies.

Autopsy↗
PubMed1977

[Hairy cell leukemia in the spleen (author's transl)].

Fifteen spleens with hairy cell leukemia are studied with immunofluorescent (7 cases), electron microscopic (5 cases), and usual histologic methods. The findings are: enlargment of the spleen (weight always superior to 400 g), diffuse red pulp infiltration by ambiguous cells with regular repartition of nuclei and clear spaces between them, hairy aspects of the cytoplasmic membrane which are especially observed on semi-thin and ultra-thin sections, presence of particular cytoplasmic inclusion bodies (polysome lamellae complex). The cellular infiltration is accompanied by some vascular modifications: pseudo angiomatosis lesions, nodular formations ressembling splenomas. An important hypertrophy of the splenic macrophages with erythrophagocytosis and siderosis is also observed. These two phenomena partly explain the anemia.

Erythrocyte Aging↗
PubMed1983

Morphological feedback effect on the nucleoli of the neurons in the nucleus arcuatus (infundibularis) to hypophyseal hypogonadism in juvenile haemochromatosis.

Case report of a man of 22 with juvenile haemochromatosis and hypogonadotropic hypogonadism. There is evidence of siderosis of the basophil cells of the hypophysis, atrophy of the testicles with disappearance of leydig cells and alterations in the nucleoli of the neurones with enlargement, vacuolation and multiplication limited to the nucleus arcuatus tuberis. The nucleolar changes in this hypothalamic centre are interpreted as a feedback effect. They are comparable to matching alterations in the hypertrophy of the nucleus subventricularis (Sheehan and Kovács 1966) in post-partum hypopituitarism.

Adult↗
PubMed1994

The pathology of the adrenal glands in sudden infant death syndrome (SIDS)

The sudden infant death syndrome (SIDS) is at present based on unknown pathogenetical mechanisms but in industrial nations is the most common cause of death in infancy after the perinatal period. Studies of a large number of adrenal glands in this syndrome have not been reported. Therefore, we evaluated 146 SIDS cases (85 males, 61 females, aged 14-465 days) and 24 control cases (17 males, 7 females, aged 18-623 days) by light microscopy, morphometry and immunocytochemistry (anti-chromogranin A and anti-S100 protein). Our data revealed a normal maturation of the adrenal glands in SIDS cases. Necroses, extensive hemorrhages or inflammation were not found. A focal lipid depletion of the zona fasciculata was seen in 92% of the adrenal glands of the SIDS and control cases. We found a siderosis (in 33% of the SIDS cases and 4% of the control cases) and calcium deposits (13% and 12% respectively) due to hyperemic involution of the fetal zone. The medulla, including the sustentacular cells (S 100 protein-positive cells) and chromaffin cells (chromogranin A-positive cells) was unchanged. Our results indicate that the few morphological alterations of the adrenal glands in SIDS cases are the effect of the underlying disease and not the cause of the sudden death.

Adrenal Cortex↗
PubMed1980

A non-transferrin-bound serum iron in idiopathic hemochromatosis.

The nature of iron in the serum of patients with idiopathic hemochromatosis has been studied utilizing an isotope labeling method and results have been compared with those from normal individuals and patients with other forms of liver disease. Between 2 and 4% of a tracer dose of 59Fe added to normal serum was retained by DEAE Sephadex and has been designated non-transferrin-bound. Alcoholic liver disease, chronic active hepatitis, and iron deficiency have no effect on this fraction. In idiopathic hemochromatosis 34.6 +/- 3.9% of the added iron was not bound to transferrin at diagnosis, representing approximately 700 microgram Fe/liter serum. Treatment lowers this fraction before serum iron concentration falls to normal. The majority of the non-transferrin-bound iron is of low molecular weight and is not bound to albumin. The presence of this fraction may contribute significantly to the development of tissue siderosis.

Binding Sites↗
PubMed1982

Hepatic iron stores and markers of iron overload in alcoholics and patients with idiopathic hemochromatosis.

Liver iron concentrations were determined in 60 alcoholics with liver disease of varying severity, 15 patients with untreated idiopathic hemochromatosis, and 16 control subjects with biliary tract disease. Mean liver iron concentrations (microgram/100 mg dry weight) were significantly greater in the alcoholics (156.4 +/- 7.8 (SEM); P less than 0.05) and in patients with idiopathic hemochromatosis (2094.5 +/- 230.7; P less than 0.01) than in control subjects (53.0 +/- 7.0). Liver iron concentrations of greater than 140 micrograms/100 were found in 17 alcoholics (29%) and in all 15 patients with idiopathic hemochromatosis. Liver iron concentrations greater than 1000 micrograms/100 mg were found in all patients with idiopathic hemochromatosis but in none of the alcoholics. In the alcoholics no relationship existed between liver iron concentrations and the amount of alcohol consumed daily, the length of the drinking history, the amount of beverage iron consumed daily, or the severity of the liver disease. Serum ferritin concentrations reflected iron stores in patients with hemochromatosis and in alcoholics with minimal liver disease. However, in alcoholics with significant liver disease serum ferritin concentrations did not reflect iron stores accurately, although with normal values iron overload is unlikely. Serum iron concentration and percentage saturation of total iron-binding capacity were of little value in assessing iron status in either alcoholics or patients with hemochromatosis. Measurement of the liver iron concentration clearly differentiates between alcoholics with significant siderosis and patients with idiopathic hemochromatosis.

Alcoholism↗
PubMed1990

Deposition of lipopigment--a new feature of human splenic sinus endothelium (SSE). Ultrastructural and histochemical study.

Lipopigment (LP) deposition was studied in a series of 36 control and 79 pathological spleens. In the control group the LP deposition in SSE was rudimentary and did not display age-dependence. A varying degree of lysosomal and cytoplasmic siderosis was a frequent finding in haemolytic anemia without any significant LP induction. In the acquired secondary storage syndrome and in some inherited lysosomal enzymopathies, the amount of LP in splenic sinus endothelium (SSE) was significantly increased and in some instances its deposition reached very high values. As deposition was not accompanied by any detectable lysosomal lipid storage phenomenon in pulpar histiocytes, the pigmentogenesis is thought to be by a process resembling that for lipofuscin. In ceroid-lipofuscinosis group the SSE affection was of low degree, as seen in other viscera. The LP deposition seems thus to be a prominent, albeit variable feature of human SSE lysosomal pathology and may represent a monotonous response to various stimuli connected with increased demands on the SSE lysosomal system. Only in some lysosomal enzymopathies, typically in sphingomyelinase deficiency was SSE LP deposited progressively and concurrently with the stored lipid. LP deposition was accompanied by an increase in lysosomal enzyme activities but lacked the alkaline phosphatase induction in SSE described in lipid and mucopolysaccharide storage diseases. This and several other features which are reviewed clearly distinguish SSE from the pulpar histiocytes with which they have been often identified.

Endothelium↗
PubMed1989

Multiple nodular foci in the liver associated with chronic hepatic porphyria after previous treatment of breast cancer.

More than 7 years after the diagnosis and treatment of breast cancer (T1N1aM0), multiple nodular foci were observed in the liver of a 40-year-old woman at ultrasonographic examination. The lesions were confirmed by CT scan, but CT-guided liver biopsy revealed only non-specific alterations. At subsequent peritoneoscopic examination, bluish-brown foci were indeed visible on the liver surface, but guided liver biopsies again failed to corroborate the suspected metastases. Instead, histology showed mild portal fibrosis, moderate steatosis and siderosis of hepatocytes, as before. Only the intense red fluorescence of part of the biopsy material under Wood's light suggested the diagnosis of chronic hepatic porphyria or porphyria cutanea tarda, here presumably as a consequence of prolonged alcohol consumption. Subsequent porphyrin studies in urine, faeces and plasma yielded the typical constellation of latent porphyria cutanea tarda (chronic hepatic porphyria type C). The activity of erythrocyte uroporphyrinogen decarboxylase was normal, which argued against a genetic predisposition. After 1 year of strict alcohol abstinence and low-dose chloroquine treatment the "nodular foci" in the liver were no longer visible on ultrasonogram and CT scan; only proton-weighted NMR imaging (SE 1500/30) still showed ill-defined areas of higher signal intensity. The renal excretion of porphyrins had decreased considerably. The levels are now consistent with the diagnosis of subclinical chronic hepatic porphyria type A. Modern non-invasive imaging techniques are tremendously useful, but they have their pitfalls. Focal liver lesions may present serious diagnostic problems, especially when they are found in a patient with a history of carcinoma at an extrahepatic primary site. A rare example is described.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

"Porcelain liver" appearance due to Glisson's capsule phleboliths.

A case of multiple Glisson's capsule phleboliths is reported in a 29-year-old-woman who had portal vein obstruction with spontaneous shunting resulting in hepatic siderosis and cirrhosis. The phleboliths were visible as multiple linear calcifications on plain film and computed tomography. Diagnosis was established by surgical liver biopsy. The potential mechanisms of this unique entity is discussed.

Adult↗
PubMed1983

The EOG in unilateral eye disease: injuries.

In unilateral injuries, the tap to the eye by a penetrating foreign body is sufficient to depress the electro-oculogram (EOG). Total recovery is time-consuming. During the recovery period, complications such as siderosis and detachment may arise, acting with predilection on the light-sensitive EOG component. Blunt traumata and possibly surgical opening of the eyeball predominantly decrease the light-insensitive component of the EOG.

Adolescent↗
PubMed1997

Pseudotumoral presentation of nodular regenerative hyperplasia of the liver: imaging in five patients including MR imaging.

Nodular regenerative hyperplasia (NRH) of the liver is a condition characterized by multiple monoacinar regenerative nodules in the absence of fibrous septa. When these nodules become confluent they may be seen with sonography or CT. The appearance of these pseudotumoral pattern of NRH has been scarcely described with MRI. We present the imaging findings of five patients with NRH and a pseudotumoral form at sonography. Sonography depicted hyperechoic lesions in four patients and hypoechoic lesions in another. Computed tomography showed hypodense lesions with little contrast enhancement in two patients. Three patients showed subtle focal liver lesions on MRI: isointense in one, mildly hypointense in another, and minimally hyperintense in a patient with siderosis. The dynamic behavior at MRI was similar to the normal liver parenchyma. Hyperechoic lesions on sonography or hypodense lesions on CT, barely or not seen on MRI, can be indicative of NRH in an appropriate clinical setting.

Adolescent↗
PubMed1988

Experimental study of iron effect on the liver function.

The effect of iron on the liver function was studied in rats. A total of 40 rats were divided into four groups. Group 1 was given iron; Group 2, carbon tetrachloride; Group 3, a combination of iron and carbon tetrachloride; and Group 4 was the control. The changes in liver function were evaluated by using hepatobiliary and liver scintigraphy as the index of hepatocyte function and reticuloendothelial system function, respectively. Determination of liver CT number and a histological study were made at the same time. The administration of iron activated the reticuloendothelial system function per unit of liver weight. However, because of the decrease in liver weight, the total reticuloendothelial system function did not change at all. In the group given iron and carbon tetrachloride, liver cirrhosis and siderosis in the reticuloendothelial system occurred. Dysfunction in the reticuloendothelial system was more severe in this group than in the group given carbon tetrachloride only, by hepatocyte dysfunction was more mild. It is doubtful that the administration of iron after liver dysfunction had developed, which caused acceleration of fibrosis and reduction of liver blood flow, led to the enhancement of the reticuloendothelial system dysfunction.

Animals↗
PubMed1999

Evaluation of cardiac functions in patients with thalassemia major.

It is known that a blood transfusion is necessary for survival in patients with thalassemia, but it may cause myocardial dysfunction due to myocardial siderosis as in other organs. The aim of this study was to evaluate myocardial perfusion by means of stress thallium scanning (MPS) and left ventricular functions by rest radionuclide ventriculography (RNV). Twenty-one patients at ages 9-16 (mean 12.1 +/- 3.2) who have been diagnosed with thalassemia for 4-15 years (mean 12.7 +/- 4.8) were included in the study. They had blood transfusions 78-318 times (mean 162.1 +/- 71). MPS and RNV was performed within two days after the any transfusion. MPS showed ischemia in 3 patients and normal perfusion in 18 patients. RNV revealed normal systolic parameters (wall motion, EF, PER, TPE) but diminished diastolic parameters (TPF, PFR) compared with normal values (p < 0.05). We conclude that ischemia or fixed defects may be seen in stress MPS as a result of cardiac involvement in patients with thalassemia. But, RNV is an important and preferable test for the early detection of subclinic cardiomyopathy. RNV may therefore show diastolic abnormalities before the systolic abnormalities show up.

Adolescent↗
PubMed2000

Epidemiological situation and treatment of patients with thalassemia major in Germany: results of the German multicenter beta-thalassemia study.

At present, about 300 patients in Germany suffer from thalassemia major. In 1990, a multicenter study was introduced to identify all thalassemic patients in Germany as well as to establish a uniform therapy protocol, including follow-up diagnostic procedures. After 6 years of study, the data of 203 patients were analyzed. The majority originate from endemic regions around the Mediterranean Sea. The median age of the patients is 13.8 years (range 1-37.5 years). At present, about 20% of the patients are older than 21 years. Regarding transfusion therapy, a shortening of the average transfusion interval to 3 weeks in most cases occurred. Throughout the entire period, median baseline hemoglobin concentrations of 10.0 g/dl were observed. The evaluation of serum ferritin levels revealed considerable differences, depending on the patients' age. Thalassemic patients in the first decade of life generally presented with good therapeutic results; serum ferritin levels were below 1800 ng/ml in 76/102 patients (75%) upon entry into the study. In contrast, 51/98 patients (52%) older than 10 years had ferritin levels above 2500 ng/ml. More than half of all treated patients presented with siderotic complications such as cardiac disease in 20/157 (13%), liver disease in 32/157 (21%), impaired glucose metabolism in 22/157 (14%), hypogonadism in 39/66 (59%), and hypothyroidism in 38/157 (24%) who were under treatment at the time of first survey. Since the situation concerning siderosis and the lack of compliance proved to be particularly difficult with adolescent patients, further efforts should concentrate on this age-group.

Adolescent↗
PubMed1995

[Histological processing of iliac crest biopsies based on decalcification and paraffin embedding with reference to osteolytic and hematologic diagnosis].

A survey is given of methods involving decalcification and paraffin embedding of iliac crest biopsy for osteological and haematological diagnostic procedures. In order to avoid shrinkage, loss of antigens, and fading of ferritin iron and enzymes, a fixative has been designed that is composed of an aqueous solution of calcium acetate (10(-1) M), glutaraldehyde (0.5%), and formaldehyde (1%; CGF). CGF-fixated specimens are decalcified in an aqueous solution of 10% di-sodium ethylene-diaminotetraacetate (EDTA) neutralized by tris[hydroxy]methylaminomethane and embedded in paraffin. Tissue prepared in this manner allows histochemical detection of naphthol AS-D chloroacetate esterase in the neutrophilic cell line and in tissue mast cells, tartrate-resistant acid phosphatase in hairy cells and certain other low malignant B-cell lymphomas, in Gaucher cells, and in osteoclasts, and a specific platelet esterase in megakaryocytes and leukaemic megakaryoblasts. A broad panel of antigens is well preserved. Beside haemosiderin, cytosolic ferritin can be detected by Perls' reaction in acute phase-stimulated macrophages. Emphasis is placed on the diagnostic impact of plasma cell siderosis and lysosomal sideroblastocytosis in haemochromatosis and in alcoholism respectively. A technique is presented to discriminate mineralized and non-mineralized bone even after decalcification.

Bone Diseases↗