Ambiguous genitalia in a child.
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Biomedical subjects
Publications and source records attributed to H J Wigger.
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Calcified caval thrombus should be considered in any infant or child where calcifications are noted in the high right retroperitoneal area on plain x-rays of the abdomen. Although typically bullet-shaped in configuration, the calcium distribution in the neonate may be atypical or incompletely developed, suggesting neuroblastoma. Definitive diagnosis can be made by inferior vena cavagram. As no deaths or complications have been attributed to the lesion in the cases thus far reported, no specific treatment is recommended.
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Extracardiac rhabdomyomas are rare benign lesions of striated muscle origin. A review of the world literature revealed about 27 acceptable cases of benign, adult-type rhabdomyoma with a distinct male predominance. In most of the reported cases, the tumor was located in the head and neck region, usually submucosal or deeper. The case reported here is unusual because the tumor was subcutaneously located in the neck. The tumor was fairly well circumscribed, multilobulated, and easily shelled out. Light microscopy showed large round to elongated cells with granular, highly eosinophilic cytoplasm, often with peripheral vacuolation. Cross striations were infrequent. The nuclei were often peripheral and had prominent nucleoli. Electron microscopic studies confirmed the tumor's myogenic origin. Myofibrils with Z band material, abundant mitochondria, and glycogen particles were observed.
The distribution and development of angiotensin converting enzyme (EC 3.4.15.1) were studied in fetal and newborn rabbits, using fixed tissues embedded in paraffin and stained with a fluorescein-conjugated antibody specific for the enzyme. The enzyme was found at the luminal plasma membrane of endothelial cells from the second third of gestation to the neonatal period. In addition, two types of epithelial cells also contained the enzyme, renal tubular and intestinal. The vascular enzyme may play a role in the regulation of its vasoactive peptide substrates, angiotensin and bradykinin, at term.
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Potter renal nonfunctional syndrome is an association of facial and limb anomalies, pulmonary hypoplasia, and fetal renal anomalies which lead to marked oligohydramnios, including renal agenesis (true Potter syndrome), renal cystic dysplasia, and obstructive uropathies. Some infants survive long enough to develop severe respiratory distress secondary to pulmonary hypoplasia. The underlying renal disease is often noted only at autopsy. We studied four infants, only one of whom had clinical signs of the renal nonfunction syndrome. Portable voiding cystourethrography revealed a tiny bladder in three infants with cystic dysplasia kidneys (two of these infants had reflux into unused ureters). Bladder hypertrophy and vesicoureteral reflux secondary to posterior uretral valves were noted in the fourth infant.
The definition, history, and clinical presentation of the prune belly syndrome are outlined. Review of autopsy material and survivors reveal three groups of patients, and the author describes the choices of management available for both groups. Highlighted is the role of the radiologist.
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Desquamative interstitial pneumonia in a young infant followed a rapidly fatal course and developed morphologic features of honey-combing, giant cell pneumonia, and pulmonary alveolar proteinosis. The rapid and fatal progression of the disease in spite of steroid therapy and the polymorphous histologic appearance of the lungs at autopsy raise doubt that the disease is a relatively benign and uniform entity and invalidate the classical initial histologic features as prognostic indicators.
Soft tissue tumors with the characteristics of Ewing sarcoma of bone have thus far only been studied by light microscopy. A pelvic tumor of this type in a 13-year-old girl was examined by electron microscopy. Comparison of its ultrastructural features with those of reported cases of bony Ewing sarcoma reveal much similarity. It is believed that they are probably identical and that the tumor cells are of immature mesenchymal type. However, their site of origin and the direction of their potential differentiation remain obscure.
The unusual occurrence of a fibrous histiocytoma of subcutaneous soft tissues, bones, and viscera is described in a newborn infant. The clinical and radiologic picture simulated a congenital fibromatosis, but histologic, electronmicroscopic and tissue culture studies indicated a malignant tumor with a bimodal cell population resembling immature fibroblasts and histiocytes. Although less mature and more uniform, it essentially parallels the findings in the adult malignant fibrous histiocytomas.
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The effects on hepatic growth of fat-free TPN and semistarvation have been studied in newborn beagle puppies. Our data indicate that liver growth during TPN is characterized by normal deposition of protein and minerals, with increased glycogen and decreased lipid content. While the lipid pattern of TPN livers is normal, the phospholipid fraction contains an abnormal fatty acid pattern, consistent with the development of essential fatty acid deficiency. The exact physiologic significance of this is unknown, but it is tempting to speculate that changes in integrity of the hepatic cell membrane as a result of this abnormal fatty acid pattern might account for some of the clinical abnormalities of hepatic function during TPN.
Despite the fact that necrotizing enterocolitis is considered a disease of premature infants, 20% of all affected infants at Babies Hospital over the past 20 years were products of term gestations. Two distinct subgroups of such infants were noted (1) five infants with congenital heart disease and/or congestive heart failure (e.g.hypoplastic left heart syndrome), all but one of whom developed the disease in the first week of life; (2) eight infants who developed the disease at a much later age after a protracted period of diarrhea. This histopathologic features of the disease in term infants are the same as those in premature infants. Further, the pathogenesis of the disease in term infants does not appear to differ basically from that in premature infants. These facts, lead away from the concept of NEC as a disease of simple etiology.
The fetal rhabdomyomatous nephroblastoma is considered to be a predominantly monophasic mesenchymal variant of Wilms' tumor, which has not been seen in patients older than four years. It acts less aggressively than a Wilms tumor despite its much larger size. Its better prognosis appears to be related to either the absence of or the insignificant amounts of neoplastic epithelium. The bilaterality of this tumor in one-third of the cases, however, may negatively affect the overall prognosis, because complete resection may be impossible or because renal failure ensues.
Peliosis hepatis, which has previously only been reported in adults, occurred in a chronically ill 11-year-old boy with cystic fibrosis. As in cases in adults, chronic illness, long-term anabolic steroid therapy, and right ventricular congestive failure may well have been the causative factors in this case. Histologically, the phlebectatic and parenchymal types, which were presumed to be separate, were found conjointly in this case. This is compatible with its origin from severe right ventricular congestive failure. The rarity of peliosis, however, indicates that one or more as yet unknown factors might be operative in its development.
The ultrastructure of a fetal mesenchymal hamartoma of the kidney shows undifferentiated mesenchymal cells, whose morphological characteristics conform to those of secondary mesenchyme. In contrast to primary mesenchyme or mesoblast, which may form epithelial structures, secondary mesenchyme is incapable of doing so. While Wilms' tumor may be considered a tumor of mesoblast, the fetal mesenchymal hamartoma is believed to originate from secondary mesenchyme or some of its more mature derivatives.