Solitary hyperplastic polyp of the stomach.
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Biomedical subjects
Publications and source records attributed to S Gosseye.
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Some problems concerning the diseases due to deficiencies of the T immune system in infancy and childhood are reviewed. The relationships between SCID and Nezelof syndrome and the pathogenesis of this group of diseases are particularly discussed.
The clinical and pathologic findings of a case of cystic partially differentiated nephroblastoma in a 5-month-old boy is reported. We believe that it probably represents the differentiated counterpart of nephroblastoma. After nephrectomy, the child was given a short course single cytotoxic therapy with vincristine for 6 months.
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Isolated rupture and thrombosis of the renal artery is a rare form of traumatic injury : two cases are added by the authors to the seventy reports found in the English and French literature. Clinical signs calling for the diagnosis like hematuria or high blood pressure may be absent. To make an early diagnosis, every patient suffering from acute blunt trauma of the abdomen should be submitted immediately to intravenous urography. Angiography should follow immediately when no secretion is produced on one or both sides. If a lesion of the renal artery is discovered, surgical repair can be attempted when the delay does not exceed 12 to 24 hours from the time of the accident. The best technical modalities include segmental resection with thrombectomy, aortorenal by-pass or eventually renal autotransplantation.
A 2 1/2-year-old child died after an illness of three days duration, characterized by a sudden onset, vomiting, coma and a striking hyperammonemia. At autopsy, the liver was practically normal. This is a further example of Reye's syndrome with only minimal fatty infiltration of the liver. It appears that the classical massive and diffuse fatty degeneration of the liver is not an essential feature of the syndrome. The evidence for this is discussed.
The authors report the occurrence of an intralobar pulmonary sequestration in a neonate with symptoms of congestive heart failure, due to a fistula effect in the sequestrated area. The venous drainage was directed mainly to the superior caval vein via azygos vein and accessorily to the left atrium. The patient became asymptomatic after lobectomy performed at the age of 11 days.
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Using an immunoperoxidase technique the localization of hCG, hPL, SP1, PAPP-A and PP5 within villous and extravillous trophoblast has been studied. The villous syncytiotrophoblast appears to be the sole significant source of hCG, SP1, PAPP-A and PP5 but hPL is also present in the infiltrating extravillous trophoblast. Within the interstitial extravillous trophoblast the proportion of cells staining positively for hPL increases progressively as the cells extend deeper into the uterine tissues but all the vascular extravillous trophoblast within the lumina of the spiral arteries stains positively for hPL at all levels within the decidua and inner myometrium. It is not clear why the synthetic capacity of the infiltrating trophoblast is limited to the production of hPL. We suggest that this might be indicative of selectivity of a specific subpopulation of cytotrophoblastic cells. It is unlikely that the physiological effects of infiltrating trophoblast are mediated by hPL, this substance being probably only a marker of a cell population with a particular invasive capacity.
Sinusoidal dilatation is a hallmark of peliosis hepatis, a rare vascular disorder that can be either drug induced or of infectious origin. Here we report a patient with peliosis hepatis of unknown etiology. An hepato-pulmonary syndrome developed which was reversible following liver transplantation.
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A 9-year-old boy presented with a small subcutaneous tumor of the trunk and diffuse bone marrow involvement. The first histological diagnosis given was undifferentiated malignancy possibly of neural crest origin and chemotherapy was started immediately using vincristine, cyclophosphamide, cisplatin, and teniposide (OPEC). Complete response was achieved after four courses of chemotherapy. Histological slides were then reviewed and the final diagnosis of alveolar rhabdomyosarcoma (RMS) was retained. Moreover, chromosome analysis of malignant cells in the bone marrow revealed a translocation involving chromosomes 2 and 13:t(2;13) (q35;q14). This specific karyotype finding has been recently reported in a few cases and could be specific for alveolar RMS. The patient had a relapse 7 months after diagnosis and died 4 months later.
We report the occurrence of a leiomyoma of the suprarenal gland in a 10-year-old girl with ataxia-telangiectasia (A-T). Muscle cell tumors are very uncommon in this gland as they are in A-T. Possible reasons for developing nonhematologic tumors in this syndrome are reviewed. A defect in DNA repair mechanisms probably favors, in young children, the expression of tumors normally expected in the aged.
Cellular (or atypical) congenital mesoblastic nephroma (CMN) is a potentially aggressive form of the benign classical congenital mesoblastic nephroma. We report here a case of cellular CMN in a 5-month-old boy treated preoperatively with chemotherapy with an excellent response allowing a complete surgical resection.
A firm, painless tumor of the temporal region was excised in a 3-year-old girl. The diagnosis of nonossifying fibromyxoid tumor was established on pathologic examination, which showed that most of the cells formed cords and tubulelike structures in a myxoid background. Bone and osteoid were lacking. The child is free of recurrence 4 years after surgery.
Defects of neuronal migration were detected in the brains of five unrelated infants with Potter syndrome (oligohydramnios sequence). These consisted of abnormal lamination of cerebral cortex, white matter heterotopias, and meningeal and molecular zone neuronal-glial ectopias. Besides, various other brain anomalies were sometimes found. They comprised one or more of the followings: abnormal gyration patterns (gyral fusion, cerebellar microgyria), cerebellar granule and Purkinje cell heterotopias, brain stem heterotopias, adysplasia of basal ganglia, gliosis, mineralization, and hydrocephalus. Detailed investigations, using standard neuropathologic stains, immunohistochemical and Golgi methods, and a new electron microscopic histochemical technique that we applied to study the developing human brain, suggest that migration defects of neurons are caused by an abnormality in their glial guides, the radial glial fibers, during the period of cortical histogenesis. We hypothesize that abnormally and precociously induced radial glial transformation into astrocytes is the pathogenic mechanism for the defects in neuronal migration. The etiological factor(s) that precipitates such abnormal glial transformation seems to be heterogeneous. Its relation to the Potter anomaly is discussed.
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