Effects of mesenchymal stromal cells play a role the oxidant/antioxidant balance in a murine model of asthma.

Asthma is a heterogeneous disease characterised by chronic airway inflammation. One of the most devastating consequences of this inflammatory process is the generation of reactive oxygen and nitrogen species responsible for oxidative stress. The aim of this study is to analyse the efficiency of treatment with human bone marrow-derived mesenchymal stromal cells (hMSC) in maintaining the oxidative balance in a murine model of allergic asthma by quantifying nitrotyrosine in lung tissues. After confirmation of asthma in the experimental model, samples of lung parenchyma were submitted to immunohistochemical assessment. Intravenous administration of hMSC reduced the levels of nitrotyrosine in the ASTHMA-hMSC group compared to those in the ASTHMA-SAL group. In conclusion, therapeutic administration of hMSC had a beneficial effect on oxidative stress, reducing the levels of nitrotyrosine in lung tissues in a model of allergic asthma.

Histopathologic and morphometric evaluation of the skin abnormalities induced by erbium:YAG and carbon dioxide lasers in 10 patients.

In the 1960s, carbon dioxide (CO2) laser therapy started to be applied to eliminate wrinkles, actinic scars, and acne because of its capacity of induce intracellular water vaporization. However, recent studies have shown the efficacy of the erbium laser in removing delicate and moderate scars. Furthermore, the postoperative lesions induced by the erbium laser seem to resolve faster and with less erythematous pattern compared with lesions induced by the CO2 laser. The purpose of this study was to determine the immediate pathologic alterations caused by single applications of CO2 and erbium lasers and their association in human skin shreds. Ten white female patients aged 30 to 63 years underwent rhytidectomy, and their respective shreds, which were prepared for excision, were tattooed with the CO2 laser, the erbium laser, or a combination of both in random order and number of applications, before final removal. This project was approved by the local ethical committee. After surgical removal, these tattooed shreds were fixed in 10% buffered formalin and submitted to histopathologic analysis. Morphometric studies demonstrated the normal skin thickness and thickness of the laser-treated area, and their subtraction resulted in the ablation damage values. Residual thermal damage corresponded to the thickness of the affected skin from the most superficial layer of tissue in the laser-treated area down to the deepest dermal area with basophilic degeneration of collagen fibers. Our results showed that two CO2 applications resulted in greater ablation and residual thermal damage when compared with only one CO2 application. The same was true in comparisons of one and two applications of the erbium laser. Both results were statistically significant (p < 0.05). When one isolated erbium and one isolated CO2 application were compared, ablation damage was greater in the former group, although with no statistical significance. One CO2 plus one erbium application compared with one isolated CO2 application showed similar ablation damage but greater residual thermal damage in the latter group (p < 0.05). These observations might contribute to our understanding of the lesions caused in the human skin by erbium and CO2 lasers and eventually help determine the ideal laser combination for the appropriate surgical treatment.

Pleomorphic xanthoastrocytoma: report of a case diagnosed by intraoperative cytopathological examination.

The authors present an unusual case of a 13-yr-old boy with a 3-mo history of seizures. A CT scan showed a contrast-enhancing mass located in the left temporal lobe. The patient underwent a stereotatic-guided craniotomy; intraoperative cytological diagnosis was performed by the smear technique, showing a pleomorphic xanthoastrocytoma. The tumor was totally resected. Definitive diagnosis was established by examination of paraffin-embedded material. Six months after the surgical intervention, the patient is doing well, with no radiological evidence of recurrence. The cytological differential diagnosis of giant cell lesions of the central nervous system is emphasized.

Accuracy of the smear technique in the cytological diagnosis of 650 lesions of the central nervous system.

The authors analyzed the results of 650 lesions of the central nervous system submitted to intraoperative cytological diagnosis by the smear technique. Cytological and paraffin section diagnoses were compared. The following statistical values were obtained: accuracy of 97.3%, sensitivity of 97.9%, specificity of 95%, positive predictive value of 99.1%, and negative predictive value of 89.6%. The authors comment on their main pitfalls using this cytological diagnostic procedure.

[The importance of muscle biopsy in the diagnosis of neuromuscular diseases]. / A importância da biópsia muscular no diagnóstico de neuromiopatias.

Most of the neuromuscular diseases have similar clinical presentation affecting mainly young patients. Clinical observations, serum enzymes, electromyographic studies and muscle biopsy are required for correct diagnosis. The authors show the results of morphological observations of a series of 124 muscle biopsies studied between 1988 and 1992 using conventional paraffin embedded material, frozen sections of muscle tissue stained by several histochemical techniques, electron microscopic observations and, in some cases, morphometric analysis of the smaller diameter of at least 100 random muscle fibres. Neurogenic atrophy was present in 27% of the cases (n = 33), dystrophic muscle was diagnosed in 26%, mitochondrial myopathy in 7% while inflammatory and metabolic myopathies were less frequent. In the group of muscle dystrophy most cases were of Duchenne type showing great variation in fibre size ranging from 10 to 110 microns (mean = 30 to 70 microns). There was also muscle necrosis and fatty changes. Neurogenic biopsies showed fibre atrophy with clustering of nuclei and group atrophy. Electron microscopic observations in cases of Werdnig-Hoffmann showed tiny fibres with less than 5 microns in great dimension and also with redundant basal laminae. Cases of mitochondrial myopathy showed the classical ragged red fibres and many different mitochondrial abnormalities under the electron microscope. Many cases (33%) showed only minor structural abnormalities or end-stage alterations with marked fatty infiltration and fibrosis so that a definitive diagnosis failed to be achieved. The correct selection and handling of muscle biopsies is important for adequate diagnosis in neuromuscular pathology.

[Anatomo-pathological and ultrastructural features of mucopolysaccharidosis. Case report]. / Achados anatomo-patológicos e ultraestruturais na mucopolissacaridose. Relato de caso.

The mucopolysaccharidoses (MPS) are lysosomal storage diseases in which a specific enzyme defect causes glycosaminoglycans storage in tissues. The authors present a necropsy case of a 10 years old boy with clinical and laboratorial diagnosis of MPS. The necropsy revealed thickening of meninges, cardiac valves and hepatomegaly. The microscopical examination of the brain showed finely vacuolated histiocytes around blood vessels and meninges. Systemic deposits of vacuolated histiocytes in cardiac valves and liver were also detected. The ultrastructural examination of the brain, liver and spleen showed filamentous material accumulated in vacuolated histiocytes and hepatocytes and features neuronal storage disease.

[Neuronal ceroid-lipofuscinosis. Report of 4 cases with study by rectal histochemistry, conjunctiva electron microscopy and necropsy]. / Lipofuscinose ceróide neuronal. Relato de quatro casos com estudo por histoquímica retal, microscopia eletrônica de conjuntiva e necrópsia.

The authors present the neuropathological and clinical findings of four cases of ceroid lipofuscinosis or Batten's disease. In two cases the autopsy findings showed brain atrophy and nerve cells packed with cytoplasmic granules compatible with lipofuscin. One case was diagnosed by histochemical techniques in frozen sections of rectal biopsy which showed myoenteric ganglion cells with cytoplasmic acid phosphatase positive material as well as numerous macrophages filled with lipofuscin in the mucosae. The fourth case diagnosed by electron microscopy of conjunctival biopsy which showed cytoplasmic inclusions of membranous, curvilinear and fingerprint types.

Sacrococcygeal chordoma in a 9-year-old boy.

A case of sacrococcygeal chordoma in a 9-year-old boy is presented. The symptoms at presentation were pain in both legs and sacrococcygeal region for the last two years that increased in the last four weeks irradiating mainly to the left leg. X-ray and CT scan examinations of the lumbar region revealed an expansive process in the coccygeal region with multiple calcifications and a partially eroded coccyx. There was no invasion of the retroperitoneum and regional lymph nodes. A biopsy was performed and showed cords and nests of cells with large cytoplasm, sometimes vacuolated, nuclei with moderate pleomorphism and clumped chromatin. Immunohistochemistry with avidin-biotin peroxidase technique showed positivity for CK, S-100 protein, CEA, vimentin and to EMA. Chordomas are a distinctly uncommon neoplasm in the first two decades of life, specially in the sacrococcygeal region. They have an aggressive behavior. Treatment of choice is complete resection.

[Electron microscopy study in neurodegenerative diseases in childhood]. / Estudo por microscopia eletrônica em doenças neurodegerativas na infância.

Neurodegenerative diseases are a group of disorders in which there is storage of abnormal material in cells throughout the body due to an enzyme defect. The authors present the experience in the diagnosis of the neurodegenerative diseases in infancy by electron microscopical study of skin, conjunctival and rectum material of 89 patients and 2 necropsy cases. The age of the patients ranged from 49 days to 13 years with speak age of incidence in first year of life (n = 28). Fifty patients were female and 39 were male. The most frequent sites of biopsy were the skin and conjunctival. Of the total 89 patients, 15 had a definitive diagnosis (16.8%) including 4 cases of gangliosidosis, 3 cases of mucopolysaccharidosis, a case of Gaucher's disease, a case of Niemann-Pick disease, 3 cases of neuronal ceroid lipofuscinosis and 3 cases of storage disease which could not be specified. The authors studied all these patients within clinic and ultrastructural aspects and concluded that electron microscopy is an important method in diagnosis of storage diseases but with a low sensitivity as a single "screening" test for patients with progressive encephalopathy.

[Primary pediatric tumours of the central nervous system. Anatomopathological study of 623 cases]. / Tumores pediátricos primários do sistema nervoso central. Estudio anatomopatológico de 623 casos.

Tumours of central nervous system (CNS) represent the second most frequent malignancy in children under 15 years of age but are the commonest cause of death. The authors present the epidemiologic and histopathologic analysis of 623 primary tumours of CNS occurring during the period 1990 to 1996 in paediatric patients. In this period 3318 biopsies of CNS were analyzed. In this total were included 623 paediatric tumours (18 7%). The age of patients ranged from 5 months to 15 years, 325 tumours occurred in males and 298 in females. The majority affected the posterior fossa. The majority of paediatric neoplasias were of glial origin (n = 277). The most frequent tumours were: astrocytoma (27.9%), medulloblastoma (9.95%), craniopharyngioma (5.93%), ependymoma (4.97%) and glioblastoma (3.37%).

[Malformations of the central nervous system: analysis of 157 pediatric autopsies]. / Malformações do sistema nervoso central : análise de 157 necrópsias pediátricas.

The malformations of the central nervous system affect about 5 to 10 children per 1000 births. We studied the central nervous system malformations in 5837 pediatric autopsies performed in the Sector of Anatomic Pathology, Hospital de Clínicas-UFPR, between 1960 and 1995. There were 157 central nervous system malformations (2.69%), the commonest were neural tube defects (61%): 47 cases of anencephaly and 45 cases classified in the group of myeloencephaloceles. The anomalies of the prosencephalic evagination corresponded to 8% of all central nervous system malformations, with seven cases of holoprosencephaly. Posterior fossa malformations occurred in 3%, with three cases of Arnold-Chiari. In the present study, the mortality rate due to central nervous system malformations was higher in the neonatal period.

[Infantile desmoplastic ganglioglioma: a clinical, histopathological and epidemiological study of five cases]. / Ganglioglioma desmoplásico da infância. Estudo clínico, histopatológico e epidemiológico de cinco casos.

Infantile desmoplastic gangliogliomas are rare tumors of the central nervous system, composed by a mixture of glial and neuronal cells and a fibrous stroma, which affect mainly young patients and arise from the surface of the cerebral hemispheres. We present five cases of infantile desmoplastic ganglioglioma: three were male and two were female. The ages ranged from three months and seven years (mean 2.62 years). The symptoms reflected the growth and topography of the tumors affecting the parietal (n = 3), parieto-occipital (n = 1), occipital (n = 1) lobes. Immunohistochemistry was performed in two cases with similar results to those reported in the literature.

[Meningiomas. Epidemiological and anatomopathological study of 340 cases]. / Meningiomas. Estudo epidemiológico e anátomo-patológico de 304 casos.

The authors have retrospectively reviewed all tumors of central nervous system (CNS) operated at the most important neurosurgery hospitals of Curitiba in a 5-year period (1990-1994) and found 304 (22.4%) cases of meningioma. Age mean of the patients was 48.5 years, with a range of 3 to 90 years. A marked female preponderance (68.7%) was noted. The most common tumor location was brain (n = 280) and the remaining cases occurred in spinal cord (n = 10), cerebellum (n = 9) and cranial nerves (n = 5). Histologically, there were 294 (96.7%) meningiomas of the classical type, six malignant or anaplastic, three atypical and one papillary. Two hundred and sixty seven classical meningiomas were from the meningotelial subtype, ten psamomatousos, five fibroblastic, five microcystic, five transicional and two angiomatous. The authors conclude that meningiomas are one of the most common group of primary neoplasias of CNS and the definition of malignancy in those tumors is beset by frequent discordance between histologic and biologic features.

[Brain hypoxic-injury of hemorrhagic pattern in newborns: analysis of 1028 autopsies from 1960 to 1995]. / Injúria hipóxico-isquêmica de padrão hemorrágico em encéfalos de neomortos do Hospital de Clínicas de Curitiba. Análise de 1028 casos de necrópsia entre 1960 e 1995.

Hypoxic-ischaemic injury of the central nervous system (CNS) in newborns is a very prevalent entity affecting 1 to 6 children per 1000 births. This injury may induce severe neurological sequelae. We present the analysis of 1028 consecutive cases of hypoxic-ischaemic CNS injuries of haemorrhagic pattern detected in autopsies performed at the Division of Anatomic Pathology, Hospital de Clínicas, University of Paraná, Brazil, from 1960 to 1995. The prevalence of these lesions was high (49.73%) amongst all autopsied newborns. The main types of haemorrhage were microscopical intra-parenchymal haemorrhages, intraventricular and periventricular haemorrhages and subarachnoid foci of bleeding. Our results emphasize that premature children constitute a high risk group for CNS haemorrhage needing special preventive therapeutic procedures to avoid neurological complications.

[Congenital pulmonary extralobar intra-abdominal sequestration]. / Seqüestro pulmonar extralobar intra-abdominal congênito.

Pulmonary sequestration is a rare congenital anomaly characterized by nonfunctioning embryonic pulmonary tissue that has no communication with the normal tracheobronchial tree. Pulmonary sequestration comprises 0.15% to 6.4% of all congenital pulmonary malformations and is divided into intralobar and extralobar types. The extralobar sequestration is rare, and comprises only 25% of all sequestrations. It is frequently associated with diaphragmatic hernia, eventration and other congenital anomalies. The authors report a case of extralobar retroperitoneal pulmonary sequestration which presented as adrenal tumour. The diagnosis was done by anatomo-pathological examination and no associated malformation was detected.

[Pilocytic astrocytoma in childhood: presentation of six cases]. / Astrocitoma pilocítico na infância: apresentação de seis casos.

OBJECTIVE: To present 6 cases of pilocytic astrocytomas whose clinic, epidemiological and histopathological aspects were studied. METHODS: Between 1990 and 1994 the records of the laboratories of pathology of the main hospitals in Curitiba were analysed and all cases of pilocytic astrocytomas were studied with special attention to age of onset, sex and site of the lesions. The biopsies, which were reviewed, have been processed according to conventional techniques and in a few cases electron microscopy and immunohistochemistry was further done. RESULTS: Five patients were females and 1 was male. Medium age was 4,5 years (range 1-8). The pilocytic astrocytomas affected optic chiasm (n=3), midline of cerebral hemisfere (n=2) and third ventricle (n=1). The symptoms reflected the location and extension of the tumour. The commonest signs and symptoms were those of intracranial hypertension, visual disturbances, hydrocephalus, among others. The patients were treated with surgical ressection and chemotherapy. Three patients died after a medium survival of 45 days, and three remain alive. CONCLUSIONS: The authors emphasize that pilocytic astrocytoma, mainly the juvenile variant, affect mostly the midline brain structures of infants through signs of visual disturbance. These lesions are low grade astrocytomas and should be treated predominantly by total surgical ressection.

Expression level of quiescin sulfhydryl oxidase 1 (QSOX1) in neuroblastomas.

Neuroblastoma is the most common extracranial solid malignant tumor observed during childhood. Although these tumors can sometimes regress spontaneously or respond well to treatment in infants, genetic alterations that influence apoptosis can, in some cases, confer resistance to chemotherapy or result in relapses and adversely affect prognosis for these patients. The aim of this study was to correlate immunohistochemical expression of the protein QSOX1 (quiescin sulfhydryl oxidase 1) in samples obtained from untreated neuroblastomas with the patients' clinical and pathological prognostic factors and clinical course. Neuroblastoma samples (n=23) obtained from histology blocks were arrayed into tissue microarrays and analysed by immunohistochemistry. The cases were classified according to the following clinical and pathological prognostic factors: age at diagnosis greater or less than/equal to 18 months; location of the lesion at diagnosis (abdominal or extra-abdominal); presence or absence of bone-marrow infiltration; tumor differentiation (well or poorly differentiated); Shimada histopathologic classification (favourable or unfavourable); state of the tumor extracellular matrix (Schwannian-stroma rich or poor); amplification of the MYCN oncogene; and clinical course (dead or alive with or without relapses/residual lesions). Twelve of the cases were female, 9 children were over 18 months old, 9 cases presented with extra-abdominal tumors and 9 cases exhibited tumors with unfavourable histologies. Fifteen patients underwent bone-marrow biopsy, and 4 of these were positive for metastasis. Nine patients died. The higher immunohistochemical expression of QSOX1 was more common in well-differentiated samples (P=0.029), in stroma-rich samples (P=0.029) and in samples from patients with a high prevalence of relapses/residual disease. The functions of QSOX1 include extracellular matrix maturation and the induction of apoptosis. Therefore, QSOX1 may be involved in neuroblastoma differentiation and regression and may thus function as a biomarker for identifying risk groups for this neoplasm.