A boy with Leydig cell tumour and precocious puberty: Ultrasonography as a diagnostic aid

A 7 5/12 year-old boy developed pseudoprecocious puberty. The diagnosis of Leydig cell tumour was suggested by clinical and hormonal findings and greatly facilitated by ultrasonographic investigation of the testes. Surgical exploration was in keeping with the diagnosis and the tumour was removed. Orchidectomy was not necessary. It appears that ultrasonography may be very useful in diagnosing a testicular tumour. It facilitates surgical intervention since it permits localization of the lesion which is usually very small and limited to a small part of the testis, as in this case.     

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.     

Leiomyosarcoma of the prostate: a case report

A case of leiomyosarcoma of the prostate in a 26-year-old man is presented. The short duration of symptoms, the typical sensation at rectal palpation, and the sudden fast increase in growth are stressed. The bad prognosis justifies an agressive therapeutical approach.     

Interrupted right aortic arch in DiGeorge syndrome

The clinical and necropsy findings in four cases of interrupted right aortic arch and right descending aorta associated with DiGeorge syndrome (congenital absence or hypoplasia of the thymus and parathyroids) are described. All patients had a mirror image of type B interruption, namely a right aortic arch with reversed branching pattern and an interruption between the right common carotid and right subclavian artery. In two patients there was a doubly committed subarterial ventricular septal defect and in the two other patients there was a perimembranous septal defect. Three patients had a bicuspid aortic valve. In a consecutive series of 185 necropsies in infants and children with congenital heart disease there were no cases of interrupted right aortic arch that were not associated with DiGeorge syndrome. These observations and previous reports indicate that the concurrence of these two rare conditions is more than fortuitous. In patients with an interrupted aortic arch the clinician should be aware of the common association with DiGeorge syndrome. If the interruption is associated with a right-sided descending aorta it is highly probable that the patient has DiGeorge syndrome.     

The senile lung. Comparison with normal and emphysematous lungs. 2. Functional aspects.

Senile lungs are characterized by a homogeneous enlargement of the alveolar airspaces, without fibrosis or destruction of their walls. Study of the functional characteristics of excisea senile lungs showed an increase in minimal air and a shift to the left of the elastic recoil pressure-volume curves, less pronounced than in emphysematous lungs. Maximal expiratory volumes and flows were normal. Total lung capacity was not significantly increased, but this may be a consequence of preagonal edema. Comparison of normal, senile, and emphysematous lungs showed a close relationship between recoil pressures and mean linear intercept, Lm, and between forced expiratory volume in 1 s and diameter and density of the membranous bronchioles. It is concluded that airspace enlargement may precede emphysema and may be responsible for changes in lung elasticity. In this respect, senile lungs are an example of the functional changes caused by an isolated airspace enlargement.     

Ultrastructure and innervation of neuroepithelial bodies in the lungs of newborn cats

Neuroepithelial bodies (NEB) occur throughout the airway mucosa and alveolar parenchyma of kitten lungs. In the bronchi, they are often situated on top of a cartilage plate. They form compact corpuscles containing 10–20 corpuscular cells and appear covered with a layer of flattened Claracells. Kitten NEB are occasionally observed to display mitosis of the corpuscular epithelial cells. A prominent blood capillary lies at their basal pole. The corpuscular cells contain numerous dense core vesicles (DCV), whose number and diameter remain unchanged with age. Kitten NEB are innervated by nerve fibres that “loop” through the corpuscle and form morphologically afferent as well as efferent nerve endings. The nerve endings display afferent synaptic junctions with the corpuscular cells and sometimes run in clusters, so that they contact each other. Many nerve endings undergo spontaneous degeneration. We conclude that kitten NEB are well adapted to function as chemoreceptors and as endocrine or paracrine organs. Their chemoreceptor activity could be modulated by axon reflexes since their afferent nerve endings are often continuous with the efferent ones, as well as by interneural modulation since nerve endings often form clusters. In addition, kitten NEB innervation appears to involute rapidly soon after birth. This may indicate that their chemoreceptor function is only of primary importance during gestation and at birth. However, the secretory function of kitten NEB, as evidenced by the unchanged numbers and dimensions of their DCV, seems to remain steady throughout life.© 1993 Wiley-Liss, Inc.