Bilateral hyperplastic nephromegaly, nephroblastomatosis, and renal dysplasia in a newborn: a variety of universal nephroblastomatosis

A preterm boy was born at 34 weeks. Prenatal ultrasonography showed oligohydramnios, fetal ascites, large kidneys, and small thorax. He died 21 h after birth of respiratory insufficiency. Autopsy revealed Potter's-like facies, hypoplastic lungs, ascites, and bilateral nephromegaly (renal weight almost 10 times normal). The kidneys were finely nodular externally, solid, and cerebriform on cut section. Histologically, they showed a diffusely distorted architecture of jumbled lobules, hyperplasia of cortical-type tissue with inconspicuous proximal tubules, relative hypoplasia of medullary tissue, tubulointerstitial dysplasia, and perilobar nephrogenic rests. The renal features represent a variety of the universal or panlobar (also called pancortical or infantile) type of nephroblastomatosis. To our knowledge, this is only the third such case reported. In the brain, each lateral ventricle contained a yellow gelatinous mass. Histologically, the masses consisted of a pseudomyxoid matrix with delicate fibers and focal adipocyte clusters, all confined within choroid plexus. We consider these lesions fibrolipomatous hamartomas.     

Hippocampal lesions and associated learning in the pigeon.

The performance of pigeons with hippocampal lesions was compared with that of unoperated and neostriatal-lesioned control Ss in 3 experiments. In Experiment 1, hippocampal-lesioned birds were retarded in the acquisition and the maintenance levels of autoshaped responding. However, the deficit was attenuated following the addition of a response contingency to the autoshaping schedule. In Experiment 2, the hippocampal-lesioned birds showed impaired performance on a differential reinforcement of low rates of responding schedule. From the high levels of responding in Experiment 2, underresponding was observed in hippocampal-lesioned birds relative to control Ss on return to the autoshaping schedule in Experiment 3. Results are interpreted in terms of impaired classical conditioning in hippocampal-lesioned birds. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Pathomorphology of kidney lesions induced by non-steroidal anti-inflammatory drugs

Pathomorphological findings on nephrobiopsies from two groups of patients with NSAID-induced kidney lesions are provided. In the first group (9 patients) the disease developed in patients of different age after short-term use of NSAID and was clinically manifested by acute renal failure with extrarenal allergic symptoms (skin, rash, eosinophilia). Morphological features were acute tubulo-interstitial nephritis without glomerular lesions. In the second group (23 patients) the disease developed after longterm use of NSAID in older patients with a compromised renal blood circulation presenting clinically with nephrotic syndrome, and morphologically by acute tubulo-interstitial nephritis with minimal change (lipoid nephrosis).     

Ultrasonic diagnosis of space-occupying lesions of the kidney (author's transl)

In 140 patients who were suspected of having space-occupying lesions of the kidney the ultrasound findings were analysed. There were no false negative results. Out of 4 false positives two could be explained by nontumourous focal renal disease. In a further 10 cases a space-occupying lesion could not be excluded with certainty on the grounds of the ultrasound investigation alone. Out of a total of 96 space-occupying lesions (48 solid tumours, 48 cystic processes) 83 were correctly diagnosed sonographically.     

Incidence of ligament lesions and associated degenerative changes in the elderly wrist

As the carbohydrate lacto-N-fucopentaose III (CD15 antigen or X-determinant) and its sialylated derivative sialyl-Lewis X are involved in the adhesion of cells rolling along the surface of endothelial cells, experiments were done to study the presence of these molecules on human eosinophils from patients with the idiopathic hypereosinophilic syndrome. Normal-density eosinophils from some patients showed higher levels of expression for lacto-N-fucopentaose III than light-density eosinophils. In contrast, sialyl-Lewis X was highly expressed by light-density eosinophils. Activation of normal-density eosinophils with calcium ionophore A23187 resulted in increased expression of these molecules for a short time. Monoclonal antibodies to these carbohydrates stimulated eosinophils to secrete eosinophil cationic protein, but not eosinophil peroxidase, and acted as costimulatory signals for C3b-induced degranulation of eosinophil cationic protein. It was suggested that CD15 and sialyl-Lewis X might contribute to eosinophil-mediated tissue injury in patients with the idiopathic hypereosinophilic syndrome.     

Obsessive-compulsive disorder associated with brain lesions: clinical phenomenology, cognitive function, and anatomic correlates

We studied the behavioral, cognitive, and neuroimaging characteristics of obsessive-compulsive disorder (OCD) in 13 patients with focal brain lesions (acquired OCD) and compared their clinical features and the severity of obsessive and compulsive (OC) symptoms with patients with idiopathic OCD. Both OCD groups were further compared with matched normal controls on a series of neuropsychological tests. Patients with acquired OCD had a negative familial history and later age at onset of OCD symptoms than patients with idiopathic OCD. The two OCD groups showed relatively similar clinical phenomenology, severity of OC symptoms, and profile of neuropsychological deficits. Compared with normal control subjects, both OCD groups showed cognitive deficits affecting attention, intellectual function, memory, word retrieval, and motor and executive functions. Eight of the 13 patients with acquired OCD had abnormal neurologic examinations, whereas only 3 of the 13 patients with idiopathic OCD had abnormal neurologic examinations. Neuroimaging in the acquired OCD group disclosed a variety of lesions involving exclusively the cerebral cortex (frontal, temporal, or cingulate regions), the basal ganglia, or both. These results suggest that acquired and idiopathic OCDs may share a common pathophysiologic mechanism, and that structural damage to specific frontal-limbic-subcortical circuits plays an important role in the pathogenesis of acquired OCD.     

Nosologic and anatomy pathological study of flat adenoma and associated lesions

Flat adenomas can be dysplastic from onset, whereas for polyps the risk of malignant transformation increases over time and with the size of the polyp. A dominant autosomal syndrome characterized by the development of flat adenomas has been reported. In a retrospective study of operative specimens from high-risk patients who had surgery for colorectal cancer, the author found that flat adenomas, which had been overlooked at initial evaluation, were a potentially useful phenotype. The mucosa adjacent to the flat adenomas showed hyperplasia and increased proliferation reminiscent of the morphologic abnormalities in aberrant crypts described by Pretlow; the latter have been found to occur rapidly in rats exposed to carcinogens and have also been demonstrated in patients at high risk for colon cancer. Aberrant crypt abnormalities and flat adenomas may be premalignant lesions.     

Morphometric analysis of the kidney lesions in mixed connective tissue disease (MCTD)

We examined histopathological changes of the kidney in patients with mixed connective tissue disease (MCTD) including those of glomeruli, arteries and interstitium by morphometric method. All specimens examined were collected from 25 autopsy cases diagnosed as MCTD according to the criteria for this disease proposed by the MCTD committee sponsored by the Japanese government. Clinical evidence of renal dysfunction such as proteinuria was present in 16 out of 25 cases (64%). Histopathologically, membranous type glomerular lesion was found most frequently (40%), followed by membrano-proliferative (6.7%) and mesangioproliferative types (6.7%). Nine cases had no glomerular lesion. Severe arterial lesion such as necrotizing angiitis was not found in our kidney specimens. However, morphometry revealed a high incidence of intimal thickening in the renal arteries of these patients as compared to control cases, showing this to be one of the most common features of MCTD with clinical importance. This type of arterial lesion, also seen in kidneys in other types of collagen diseases, may suggest an etiology common to them. The severity of the renal interstitial lesion in MCTD was intermediate between that of systemic lupus erythematosus (SLE) and progressive systemic sclerosis (PSS), poly-or dermatomyositis (PM/DM).     

Rhabdoid tumor of the kidney associated with a tumor of the posterior fossa

This article reports our use of intraoperative sonography to guide in real time, the removal of radiolucent foreign bodies from five patients. Two of these patients had undergone previous unsuccessful attempts at surgical removal in the operating room. The technique is cost effective, readily available, and can be very helpful in locating difficult-to-find radiolucent foreign bodies at the time of surgery.     

Morphologic and immunohistochemical characterization of the hepatic lesions associated with European brown hare syndrome

Liver lesions were studied in 40 free-living adult European brown hares (Lepus europaeus) and varying hares (Lepus timidus) of both sexes that had died in Sweden with the viral infection European brown hare syndrome (EBHS). The lesions were characterized by their histopathologic, immunohistochemical, and electron microscopic findings. Periportal to massive coagulation necrosis was a distinctive feature of EBHS. Lytic necrosis, inflammation, fatty degeneration, and cholangitis occurred variably. Accumulation of basophilic granules in the cytoplasm of hepatocytes was commonly observed; these lesions corresponded ultrastructurally to mitochondrial calcification. Viral antigen was revealed in the cytoplasm and nucleus of hepatocytes and in the cytoplasm of macrophages.     

Neuronal degeneration in the gerbil brainstem is associated with spongiform lesions

Spongiform lesions arise in dendrites and glia in the brainstem of domestic Mongolian gerbils. Most pronounced within the cochlear nucleus (CN), this disorder is dynamic and progressive; the lesions increase in number, size, and extent with age. It has not been clear whether these spongioid lesions either cause or are associated with significant neural degeneration. In contrast, feral Mongolian gerbils (wild-trapped in Tuva) and their offspring show few spongiform lesions. The Tuvan gerbils provide an appropriate within-species control. We compared degeneration in the brainstem of domestic and Tuvan gerbils using the amino-cupric-silver (ACS) stain of de Olmos et al. [(1994) Neurotoxicol. Teratol., 16:545-561]. Positive histologic controls were provided by cerebellar stab wounds in domestic gerbils and by unilateral kainic acid injections into the CN of Tuvan gerbils. The ACS stain revealed extensive degeneration of axons, terminals, dendrites, and neurons in the brainstem of domestic gerbils. Neurodegeneration was most pronounced in the CN and was coextensive with spongiform lesions. Neurodegeneration was also seen in the trapezoid body, lateral lemniscus, and inferior colliculus, but was less pronounced than in the CN. The cerebellar stab wounds resulted in silver-stained Purkinje cells restricted to the stab wound local region. Kainic acid produced extensive neuronal and spongiform degeneration of the injected CN that was very similar to that spontaneously occurring in domestic gerbils. In contrast, the non-injected CN of Tuvan gerbils showed no neuronal or spongiform degeneration with the ACS stain. We conclude that, in domestic gerbils, the naturally occurring spongiform lesions of the CN and the accompanying neurodegeneration are both results of a common mechanism, most probably excitotoxic.     

Pathological lesions associated with Anoplocephala perfoliata at the ileo-caecal junction of horses

The intestinal tracts of 20 horses, killed at a local abattoir and of unknown age, sex and previous clinical history were examined for the presence of Anoplocephala perfoliata attached to the ileo-caecal junction. Four horses had no tape-worms, nine had one to 20 tapeworms attached to the mucosa and seven had more than 100 attached to the mucosa. The histological changes of thickening, ulceration and eosinophil infiltration of the mucosa at the ileo-caecal junction were more severe when more than 100 parasites were present.     

Microvasculopathy is associated with the number of cerebrovascular lesions in hereditary cerebral hemorrhage with amyloidosis, Dutch type

BACKGROUND AND PURPOSE: Microvascular changes such as microaneurysms and fibrinoid necrosis have been found in the presence of cerebral amyloid angiopathy (CAA). These CAA-associated microvasculopathies (CAA-AM) may contribute to the development of CAA-associated hemorrhages and/or infarcts, hereafter referred to as "cerebrovascular lesions." Hereditary cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D) is an autosomal dominant form of CAA, in which the amyloid angiopathy is pathologically and biochemically similar to sporadic CAA associated with aging and Alzheimer disease. To determine the significance of CAA-AM for CAA-associated cerebrovascular complications, we investigated the association between CAA-AM and cerebrovascular lesions in HCHWA-D patients. METHODS: In a previous autopsy study we semiquantitatively scored CAA-AM in 29 HCHWA-D patients. In the present study we reviewed clinical charts and autopsy protocols of these same patients. We investigated whether CAA-AM was associated with age at death, number of cerebrovascular lesions, duration of clinical illness, hypertension, and atherosclerosis. RESULTS: An association was found between CAA-AM and the number of cerebrovascular lesions (P = 0.009). The presence of microaneurysmal degeneration was most strongly associated with the number of cerebrovascular lesions (P < 0.001). In addition, we found an association between atherosclerosis and the CAA-AM score (P = 0.047). Hypertension was not associated with CAA-AM. CONCLUSIONS: Our findings support previous reports suggesting an important role of secondary microvascular degenerative changes in CAA-associated cerebrovascular lesions and suggest an aggravating effect of systemic atherosclerosis, but not hypertension, on the evolution of CAA-AM. These findings may be of relevance to understanding cerebrovascular complications of sporadic or Alzheimer disease-associated CAA.     

The structure of bovine periodontal ligament with special reference to the epithelial cell rests

The aim of this study was to determine the structure of the bovine periodontal ligament, with special reference to epithelial cell rests (ECR) and their cytokeratin content. Periodontal ligament was obtained from bovine molar teeth and studied at both the light microscopic and electron microscopic levels. Cytokeratin content was determined using immunohistochemistry against a number of cytokeratin antibodies and specificity tested against bovine and human oral mucosa. Collagen fibril diameters and the area of a fiber bundle occupied by collagen were determined using a digital planimeter with a digitizing tablet. The majority of periodontal fibroblasts possessed considerable quantities of roughened endoplasmic reticulum, indicating rapid synthesis and secretion of collagen, but no intracellular collagen profiles were present. Endothelial cells showed Weibel-Palade bodies. Collagen fibril diameters showed a unimodal distribution with a mean collagen fibril diameter of 55.3 nm. The mean percentage area of the extracellular matrix occupied by collagen was 42%. Structurally, ECR were unusual in exhibiting large numbers of microvilli and conspicuous amounts of cytokeratin filaments. Bovine ECR showed a positive reaction to the pancytokeratin MNF116 (which reacts with the cytokeratins 5, 6, 8, 17, and probably 19), to PCK-26 (which reacts with the type II cytokeratins 1, 5, 6, and 8) and to cytokeratin 13. There was no reaction for cytokeratins 1, 4, 10, 11 and 18. Structurally, bovine periodontal ligament showed features common to other species. However, ECR in terms of both structure and cytokeratin content showed features indicative of important species differences which may have relevance when considering the etiology of radicular cysts.     

Insulinoma associated with liver lesions: value of MR imaging

We report on a middle-aged woman who presented with clinical and biochemical findings of insulinoma. Preoperative evaluation by ultrasound, CT, and angiography located the pancreatic lesion but also revealed two focal liver lesions. The latter were interpreted as metastases. MR imaging with injection of superparamagnetic iron oxide particles not only localized the insulinoma but proved to be the only noninvasive technique capable to exclude presence of liver metastases preoperatively. This reversed management to minimal laparoscopic surgery. Recent literature of preoperative imaging evaluation of insulinoma and focal liver lesions is discussed.