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Biomedical subjects

P Moerman

Publications and source records attributed to P Moerman.

At least 163 records · Page 9Linked to original sources
PubMed1985

Lung metastasis of malignant epithelioid hemangioendothelioma mimicking a primary intravascular bronchioalveolar tumor. A histologic, ultrastructural, and immunohistochemical study.

A 40-year-old woman presented with multiple nodules in both lungs and a small tumoral mass at the level of the left brachial artery. A pathologic diagnosis of pulmonary intravascular bronchioloalveolar tumor (IVBAT) and epithelioid hemangioendothelioma of the limb was established. Morphologic and immunohistochemical studies highly suggest that both tumors are manifestations of the same neoplastic process. In this case a metastatic nature of the IVBAT is suggested. The diagnosis of pulmonary IVBAT should be followed by a search for sclerosing vascular tumors elsewhere. Finally, the results substantiate the vascular origin of the IVBAT.

Adenocarcinoma, Bronchiolo-Alveolar↗
PubMed1985

Congenital muscular dystrophy associated with lethal arthrogryposis multiplex congenita.

Two unrelated patients with severe arthrogryposis multiplex congenita (AMC) who died perinatally, are presented. In both, postmortem examination revealed an intact nervous system and striking dystrophic muscle changes, consistent with congenital muscular dystrophy (CMD). Few similar cases have been reported before, but since the condition is not well known, it seems probable that in the past many have been labeled as mere multiple malformations. The possibility of an underlying muscular disorder, either primary myopathic or neurogenic should be considered in any patient with early lethal AMC. Our findings confirm that the fetal akinesia-arthrogryposis sequence is a nonspecific clinical syndrome resulting from various causes of muscular inactivity in utero. The main objective of this report is to provide reasonable guidelines on how to approach the problem of classification. We favor a pathogenetic approach, depending upon careful sampling of the central nervous system and skeletal muscles at autopsy.

Abnormalities, Multiple↗
PubMed1985

A new lethal chondrodysplasia with spondylocostal dysostosis, multiple internal anomalies and Dandy-Walker cyst.

We describe here a female infant, exhibiting lethal short-limbed dwarfism. The condition superficially resembled achondrogenesis. However, unlike achondrogenesis there was an associated severe spondylocostal dysostosis and major non-skeletal anomalies, particularly a cerebellar Dandy-Walker cyst, cardiovascular and urogenital malformations. The chondroosseous morphology was nonspecific. The case is believed to be unique. It is therefore suggested that this constellation of anomalies constitutes a "new" lethal syndrome, different from the delineated chondrodysplasias.

Abnormalities, Multiple↗
PubMed1985

Primitive neuroectodermal tumor: a newly recognized cause of early fetal death.

Reviews in the literature of fetal and neonatal neoplasms deal only with congenital tumors, that is, tumors presenting at or shortly after birth. Virtually nothing is known about neoplasms occurring in the embryonal period or early fetal life. We report five small macerated fetuses with a very primitive embryonal tumor of neuroectodermal origin. The tumor characteristically showed massive vascular permeation. Expulsion of the fetus occurred during the fourth or fifth month of pregnancy. It is suggested that this primitive neuroectodermal tumor is a "new" cause of spontaneous late abortion.

Blood Vessels↗
PubMed1985

[Polycystic renal diseases: morphology and genetic counseling].

The designation "polycystic kidneys" is vague and causes considerable confusion. This term comprises a number of affections, characterized by the presence of renal cysts. Cystic kidneys may be hereditary or sporadic. The defect may be isolated or occur as part of a syndrome. The identification of the exact nature of the lesion is therefore of paramount importance, not only for the prognosis of the propositus, but especially for the correct genetic counselling of the family. In this context, the perinatal autopsy represents an irreplaceable method of investigation. Since the pathogenesis of many, if not all, types of polycystic kidneys remains unknown, a practical classification must be based on gross and microscopic pathologic study, clinical features, and family history. Osathanondh and Potter (1964) distinguished four varieties, based on morphological findings, revealed by microdissection. Unfortunately, not all types of cystic kidneys are included in this classification. Moreover, their type III cystic kidney collects a number of clearly different entities. Nevertheless, once one is familiar with its deficiencies, the Osathanondh and Potter classification is still very useful.

Female↗
PubMed1984

Severe limb malformations in 4p deletion.

Severe limb anomalies with radial aplasia and hypodactylia are reported in a male newborn with 4p deletion syndrome. In apparent contradiction with previous reports, this finding indicates that the radial aplasia, as frequently observed in patients with ring chromosome 4, seems to be more likely related to the 4p deletion, rather than to the 4q deletion.

Abnormalities, Multiple↗
PubMed1984

[Prune belly syndrome, a secondary urethral functional obstruction due to prostatic hypoplasia].

The authors describe their experience of "prune belly syndrome" about 7 personal cases. The major signs are abdominal muscle aplasia or hypoplasia, cryptorchidix, and severe urinary tract malformations. The first evenment of the malformation sequence is a prostatic hypoplasia with functional obstruction of the urethra. Abdominal muscle aplasia is a secondary point of variable aetiology.

Cryptorchidism↗
PubMed1984

Pathogenesis of the prune-belly syndrome: a functional urethral obstruction caused by prostatic hypoplasia.

Abdominal muscle deficiency, urinary tract abnormalities, and cryptorchidism are the three major features of the prune-belly syndrome, also referred to as triad syndrome or Eagle-Barrett syndrome. The etiology is unclear and the pathogenesis a subject of continuing debate. Clinical and pathologic experience with seven cases of prune-belly syndrome is reviewed. Findings indicate that the urogenital anomalies can be attributed to a functional urethral obstruction which in turn is the result of prostatic hypoplasia. The histology of the abdominal wall is that of atrophy-ie, the degeneration of already formed muscle--and not of primitive muscle. This observation supports the theory that the abdominal muscle hypoplasia is a nonspecific lesion, resulting from fetal abdominal distension of various causes. Transient fetal ascites may be an important feature of the prune-belly syndrome.

Abdominal Muscles↗
PubMed1984

Cystic hygroma and multiple pterygium syndrome.

We report the prenatal detection of a cystic hygroma in a 17 weeks gestation male foetus with multiple pterygium syndrome. Ultrasonographic study was performed in this pregnancy after the birth of a macerated, hydropic male foetus with large cystic nuchal hygroma in a previous pregnancy.

Abnormalities, Multiple↗
PubMed1983

Thrombosis of the right umbilical artery, presumably related to the shortness of the umbilical cord: an unusual cause of fetal distress.

This case report concerns a late pregnancy complication, clinically apparent as severe variable decelerations in the first stage of labor. Emergency cesarean section delivered a mildly asphyxiated full-term newborn infant. Examination of the umbilical cord revealed a thrombus of the right umbilical artery, near the fetal side, confirmed by histological examination. The total length of the umbilical cord, only 30 cm, was below the limit necessary for uncomplicated delivery of the fetus near term. Transient stretching during fetal descent is thought to be responsible for constriction of the umbilical arteries, blood flow sludging and thrombosis. Although only a few cases have been reported, thrombosis of the umbilical vessels has to be considered whenever the fetal heart rate pattern shows unexplained variable decelerations.

Adult↗
PubMed1983

5q- anomaly in a patient with disseminated teratoma.

A 5q- anomaly associated with other chromosome anomalies was found in the infiltrated bone marrow of a patient with a highly malignant teratoma originally located in the mediastinum. There was no evidence of a second malignancy, and it is likely that the 5q- anomaly was, indeed, associated with the malignant teratoma cells.

Adult↗
PubMed1983

Aberrant twinning (diprosopus) associated with anencephaly.

A case of Monocephalus diprosopus, associated with craniorachischisis and duplication of most of the foregut derivates is presented. The major part of the cardiovascular system remained single but the heart exhibited severe defects, including a complete persistent atrioventricular canal, transposition of the great arteries and atresia of the pulmonary valve. This report further supports the hypothesis that certain-types of incomplete twinning and neural tube defects may be caused by a single teratogenic mechanism.

Abnormalities, Multiple↗
PubMed1983

Partitioning of pulmonary impedance in excised human and canine lungs.

Partitioning of pulmonary resistance of 15 excised human and 5 canine lungs by means of a retrograde catheter demonstrated that the share of peripheral airways (with an ID of 2.4 mm or less) and of lung tissue in pulmonary resistance was markedly larger (44-96%) in humans than in dogs (41-59%). Similar percentages were found in patients with chronic obstructive pulmonary disease (COPD). The variations of resistance with volume during deflation and inflation of the lungs were due primarily to variations of peripheral resistance (Rp). The latter systematically increased at high and low lung volumes. Higher Rp values, with a more pronounced frequency dependence, were met in patients with COPD. A morphometrical study showed an inverse relationship between the value of Rp and the mean diameter of the terminal bronchioles, provided the airways density was taken into account.

Adult↗
PubMed1982

Decidual vasculopathy and extensive placental infarction in a patient with repeated thromboembolic accidents, recurrent fetal loss, and a lupus anticoagulant.

Evidence exists of an association between the presence of a "lupus" anticoagulant in plasma, recurrent fetal loss, and repeated thromboembolic accidents, also in the absence of systemic lupus erythematosus. Presented is an example of this association, with morphologic and biologic studies to elucidate its pathogenesis. In the case reported, the placenta showed massive infarction. In the spiral arteries of the basal plate of the placenta, lesions of intimal thickening, fibrinoid necrosis, acute atherosis, and intraluminal thrombosis were observed. The plasma of the patient contained a lupus anticoagulant and inhibited the formation of prostacyclin by rat aortic rings. Vascular production of prostacyclin is a major natural defense mechanism against thrombosis. Lack of generation of prostacyclin may account for the decidual vasculopathy and consequent placental infarction and for the generalized thrombotic tendency of some patients with lupus anticoagulant.

Abortion, Habitual↗
PubMed1982

The Meckel Syndrome. Pathological and cytogenetic observations in eight cases.

Eight new cases of Meckel syndrome, two of them occurring in the same family, are presented. Occipital encephalocele of varying extent, multicystic renal dysplasia not associated with urinary tract obstruction, and postaxial hexadactyly comprise the three basic features of this lethal syndrome with autosomal recessive inheritance. From our observations it appears that congenital hepatic fibrosis, abnormal external genitalia in male infants and a malformed tongue with lipomatous excrescences are also frequently occurring anomalies with important diagnostic value. The statement that the majority of cases of Meckel syndrome can be detected prenatally is further supported by two cases in the present series. The incidence of this syndrome may be much higher than previously thought.

Abnormalities, Multiple↗