Skin biopsies from children and adolescents with various syndromes of somatomedin deficiency revealed changes in structure and in distribution of the dermal elastin fibers as compared with a group of seven healthy control subjects. In 18 patients with isolated growth hormone deficiency (IGHD) and in 11 with multiple pituitary hormone deficiencies (MPHD), the number of elastin fibers was reduced; the individual fibers were shorter and slimmer than usual, and frequently arranged in groups dispersed in various directions. Skin biopsies from six patients with Laron-type dwarfism (LTD) revealed mostly thickened elastin fibers, frequently arranged in irregular bundles, but the number of elastin fibers was normal in prepubertal patients and reduced only in postpubertal patients. The reduction of elastin fibers in these patients was less prominent than in IGHD. The finding of more numerous elastin fibers in LTD than in IGHD is suggestive of a direct nonsomatomedin-mediated effect of human growth hormone on skin elastogenesis.

Extension of a benign intravenous leiomyomatous tumor from the pelvis into the right atrium resulted in severe tricuspid stenosis in a 28-year-old woman. Absence of the Budd-Chiari syndrome and other abdominal manifestations led to a misdiagnosis of a primary right atrial tumor, with a fatal outcome during operation. In our search of the English literature, this was only the second case found in which extensive intravenous leiomyomatosis interfered with the tricuspid valve mechanism. In this report, the clinical picture, etiology, and treatment are discussed, along with the literature on this rare entity.

A patient with primary leiomyosarcoma of the pulmonary artery diagnosed at autopsy is reported. The literature dealing with this subject is reviewed.

A patient underwent right nephroureterectomy for gross hematuria of one year's duration for a suspected transitional cell carcinoma of the upper calyces. Two renomedullary nodules were found. Histopathology revealed both lesions to be mixed tumors: renal cell carcinoma closely associated with angiomyolipoma. We believe that this combination of renomedullary tumors has not been reported previously.

We describe a patient with heavy posttransfusion iron overload, to whom continuous high-dose i.v. desferrioxamine was administered shortly before his death. The administration of nearly 500 g of this agent was followed by urinary excretion of 5.4 g of iron. The chelator therapy lost its efficiency in the last of five courses and the patient eventually died as a result of cardiac and hepatic damage related to iron overload. The theoretical and practical implications of these observations are discussed.

A histopathological and ultrastructural analysis of the early stage of limb dysmorphogenesis from 1 hr to 48 hr, following citral administration is presented as a model for studying the reaction to injury of undifferentiated embryonic tissues. Early lesions such as cloudy swelling, hydropic degeneration, enlargement of the extracellular space and retraction of protoplasmic extensions were observed 1 hr after treatment, affecting both epiderm and mesenchyme of the limb. Few pyknotic cells were encountered during the early stages. Cell necrosis and autophagocytosis became more conspicuous at 12 hr post-injection. About the same time the first signs of healing were observed, charcterised by heterophagocytosis and return of mitotic activity. The epiderm being more differentiated, regenerates ad integrum and more rapidly than the mesenchyme. The evolution of these pathological events, which proceed in a spatio-temporal cascade, seem to reflect the pharmacokinetic gradient of the cell-teratogen interactions. The type of limb malformation will depend mainly on the extent of injury and/or healing of the mesenchymal mass, rather than on the injury and/or healing of the surface ectoderm. It is concluded that the reaction to chemical injury of undifferentiated tissues partially mimics the destructive and repair process in adult tissues, but differs in lacking some basic constituents such as oedema, monocyte exudation and granulation tissue formation.

Mature female rats treated with Citral (3-7-dimethyl-2,6-octadienal) either topically for 60 or 100 days or by 6 i.p. injections (at 4-5 day day intervals) showed a marked decrease in the number of normal follicles per section, because oocytes tended to degenerate although the follicular cells remained normal. The reproductive performance after Citral treatment was impaired: there was a reduction in implantation number and litter size and an increased post-implantation fetal wastage. None of the young survived after 100 days of topical Citral treatment. It is suggested that Citral directly affects the oocytes.

A 16-year old patient with Fanconi's anemia developed pre-leukemia and pulmonary infiltration which was found upon autopsy to be pulmonary alveolar proteinosis. The question whether the pulmonary alveolar proteinosis was due to the marked leukopenia and an eventual defective phagocytic ability of the leukocytes is raised.

Nineteen cases of cysts of the external female genitalia were diagnosed over a two-year period, giving an incidence of six per 1,000 female infants. The embryology, morphology, and differential diagnosis are discussed. No treatment was given and follow-up at two months of age revealed complete resolution of the cysts, thereby establishing their benign course in the newborn period.

Microphthalmos was indeed experimentally in 3-day-old chick embryos by intraamniotic injection of citral. Various degrees of this particular microphthalmic syndrome were observed. Severe cases were always accompanied by destructive changes in the ipsilateral part of the head. The corneal epithelium lost its continuity and the lens showed, even in the early stages, severe degenerative changes with spherophakia. The neural retina seemed to be affected later and showed hyperplasia and fold formation, while the retinal pigment epithelium was only mildly affected. The mesenchyme behaved differently according to its location: the superficial corneal and ciliary mesenchyme underwent hyperplasia, while the deeper choroidal mesoderm showed edematous changes only. This temporal and spatial distribution of pathological changes in the eye structures favors the assumption that citral acts locally. The experimental microphthalmic syndrome is not only due to the direct effect of the exogenous teratogenic factor on the ocular anlagen but is also the result of subsequent adaptive changes of these structures to the new environmental condition.

Administration of STZ prior to mating, induced significant impairment in glycogen metabolism in term rat placentae. Glycogen retention was observed in the treated placentae, while glycogen synthetase activity was reduced. The glycogenolytic pathway seemed to proceed mainly through amyloglucosidase enzyme whose activity increased threefold. Glucose-6-phosphatase showed a moderate activation, mainly in the labyrinth zone, while phosphorylase was slightly inhibited. These changes were accompanied by a decrease in placental insulin levels. The possible defensive role played by the placenta in cases of maternal hyperglycemia was postulated.