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Biomedical subjects

P M Bale

Publications and source records attributed to P M Bale.

12 recordsLinked to original sources

A comprehensive microcomputer network program for histopathology.

For a small hospital with a limited budget, a stand-alone histopathology microcomputer network may be more valuable to the pathologist than one running off the hospital's main computer. In return for sacrificing the limited benefit of screen reading of pathology reports in the wards, one receives the great advantage of more rapid retrieval of disease data. Commercial relational database programs for microcomputers can now achieve nearly all the power of a minicomputer program, with more versatility. Using an application developed on such software, we now have 30,000 pathology reports entered, from which we can retrieve a list of pathology numbers, with patients' names, age, sex, disease, and site, for any diagnosis in 3-5 seconds. The application includes full biopsy reports for doctors and wards, autopsy final diagnoses, computer-assisted Snomed coding, outside consultations, literature abstracts, daybook printing, workload statistics and billing codes and charges, and a cytology module has been added for another hospital. Moreover, the interested pathologist can easily make his own alterations to menus, entry screens, fields, and screen listing or printout formats.

Computer Communication Networks

Sequestrated meningoceles of scalp: extracranial meningeal heterotopia.

Sequestrated meningocele of the scalp has seldom been reported and is difficult to diagnose. Clinically it resembles dermoid cyst, hemangioma, or alopecia; radiographs and computed tomographic scans reveal no cranial bone defect, and surgery discloses no communication with the cranial cavity. Histologically, the lesion is characterized by a loose arrangement of connective tissue in dermis and subcutis, associated with flattened cells around collagen fibers (meningothelial cells). Most examples are very vascular, sometimes mimicking angioma, and about one third contain small necrotic foci. The meningothelial nature of the lesion is shown by its architectural similarity to communicating meningocele, and its identical immunoperoxidase reactions with vimentin and epithelial membrane antigen. In the 12 cases reported herein, most lesions were small (1 to 1.5 cm), and all but one were noted at birth but usually not resected until the patient had reached age 1 to 4, and one not until the patient was 31 years of age. Five lesions were not midline. We have been unable on histologic grounds to determine whether meningoceles are communicating or sequestrated. Compared with 20 communicating meningoceles, the sequestrated lesions were usually smaller, found in slightly older patients, and much less likely to be associated with hydrocephalus. The local excision of scalp lesions in children should include a search for a small intracranial connection.

Adult

Nasal cerebral heterotopia: the so-called nasal glioma or sequestered encephalocele and its variants.

Twenty two nasal cerebral heterotopias were compared with 11 nasal encephaloceles. No histological feature was found that would allow a communication with the brain to be confidently identified or excluded. Even laminated cerebral cortex with neurones and ependymal canals, suggestive of encephalocele, were found in heterotopias. Distinction required radiological and surgical evidence. However, CT scan could be misleading, in one infant suggesting a cribriform plate defect when none was found at craniotomy. Three children had multiple extracranial glial lesions, two with both heterotopia and encephalocele in the same patient. In a few older children it was extremely difficult to identify brain tissue because of marked replacement by fibrous tissue (up to 95%), leading to one misdiagnosis as fibroma, and considerable fibrosis occurred also in five of six recurrences and in a longstanding small encephalocele. In two heterotopias, cellularity in places approached that of low-grade neoplastic glioma. One nasopharyngeal heterotopia contained multiple mesenchymal tissues suggestive of teratoma. Two midline nasopharyngeal encephaloceles showed adjacent epithelium, possibly vestiges of Rathke's pouch.

Brain

Rapid frozen section in pediatric pathology.

Frozen section examination in pediatrics differs from that in adult practice in two ways. First, there is a high proportion of undifferentiated small-cell cancers in which it it difficult to make a definitive diagnosis without additional information. Second, there are special categories of congenital disorders where one is seeking not neoplasia but the presence, absence, or size of normal structures. In 520 pediatric frozen sections, there was a comparatively high incidence of deferred (5.6%) or inaccurate diagnoses (3.5%). However, as there were 99 small-cell cancers, it is perhaps surprising that the number was not greater. In the nervous system (208 cases), it was sometimes difficult to distinguish between malignant ependymoma and medulloblastoma. In other neoplasms, e.g., soft tissues and bone, the most important requirement was adequate clinical and radiological information. In Hirschsprung's disease (132 cases), ganglion cell detection was 100% accurate. In measuring the diameter of bile ducts in the porta hepatis during surgery for biliary atresia (18 cases), it was sometimes difficult to recognize ducts that had lost their epithelial lining.

Bile Duct Diseases

Congenital cystic malformation of the lung. A form of congenital bronchiolar ("adenomatoid") malformation.

Of 41 cases of cystic lungs in children, 21 were found by microscopy to be due to congenital cystic malformation. In most of the remainder, chronic inflammation and fibrosis precluded differentiation from postinflammatory pneumatocele. A few were intermediate between cystic malformation and congenital lobar emphysema. The cystic malformations were 17 surgical and four necropsy specimens, and two thirds of the patients were under 1 year old. The condition was unilobar, and the cysts were thin-walled, up to 8 cm in diameter, multiple or multilocular, and microscopically resembled proliferated, dilated bronchioles communicating with alveoli. There was a wide range of size, shape, and number of cysts, and no sharp demarcation from adenomatoid malformation in stillborn infants. Thus, bronchiolar malformations fall into two overlapping clinicopathologic groups: (1) adenomatoid malformation in edematous stillborn and premature infants with perdominantly solid lobes showing more epithelial proliferation and immature terminal airways and (2) cystic malformation in term infants and children with predominantly cystic lobes and interspresed mature alveoli.

Bronchi

Epignathus, double pituitary and agenesis of corpus callosum.

Two infants from unrelated families died on the 1st day of life with epignathus, duplication of the entire pituitary, infundibulum and sella, and widening or separation of midline structures of the head including absent corpus callosum. We suggest that some infants surviving surgery for large epignathi may have relatively symptomless absent corpus callosum or double pituitary.

Abnormalities, Multiple

Familial hepatic venoocclusive disease with probable immune deficiency.

Five infants from three families died between the ages of 2 and 7 months with venocclusive disease of the liver. No dietary, toxic, or other extrinsic cause was uncovered. In one family the first infant was breast-fed; the second one received no breast milk. In two of the families the parents were cousins. All infants had some evidence of immune deficiency, including hypogammaglobulinemia in at least three, multiple infections especially Pnumocystis carnii and enteroviruses, and lymphoid tissues devoid of germinal centers and mature plasma cells. Other findings in some of the infants, not previously recorded in venoocclusive disease, were microcephaly, multiple small cerebral softening, and left atrial endocardial fibrosis. A congenital cause for venoocclusive disease is suggested in these cases.

Australia

Necropsy findings in childhood leukaemia, emphasizing neutropenic enterocolitis and cerebral calcification.

In 50 necropsies on leukaemic children, the major cause of death was infection. In patients dying during therapy for induction or reinduction of remission, the most frequent infection was a distinctive neutropenic enterocolitis or typhlitis. This was seen in 46% of the whole series and was a major factor in the death in 38%. Other infections were predominantly bacterial pneumonia in patients in relapse, and viral disease, e.g. measles pneumonia, in those in remission. One patient treated for meningeal leukaemia showed an unusual linear calcification of the cortical grey matter.

Autopsy

Teratomas in childhood.

Of 109 teratomas in children 86 were benign and 23 malignant. Sacro-coccygeal and pelvic teratomas predominated 52 cases, and these fell into three groups, post-sacral, dumb bell and pre-sacral. The 34 purely posterior tumours were always congenital and benign whilst the incidence of malignancy in dum bell and pre-sacral teratomas increased as the tumour became more internal. Other sites affected in order of frequency were: the gonads, head and neck, retroperitoneal anterior mediastinum and the central nervous system. Malignant teratomas were carcinomas usually containing glandular, capillary and clear cell areas, and metastases were similar. Immature tissues in benign teratomas were usually neural or connective tissue. They did not give rise to neuroblastomas or sarcomas and did not indicate a worse prognosis. Only two originally benign teratomas later developed malignancy.

Child, Preschool

Rhabdomyosarcoma in childhood.

Fifty-four rhabdomyosarcomas in children affected mainly the pelvis and scrotum, 22 cases, head and neck, 19, and limbs and limb girdles, 11. Rhabdomyosarcomas of the female genital tract occurred only in children under 2 years, and those in the lower eyelid presented in the first year of life. A leiomyosarcoma-like appearance, and an undifferentiated small cell sarcoma sometimes mimicking Ewing's tumour, were patterns giving rise to diagnostic difficulty. Many fine gradations from undifferentiated embryonal to almost purely differentiated "pleomorphic" examples, made microscopic classification arbitrary. However, the histological pattern had no bearing on prognosis in children in this series. Of the 46 cases adequately followed, 43 are dead. Two of the 3 long-term survivors had paratesticular tumours.

Adolescent

Teratomas in childhood.

Of 107 teratomas in children, 86 were benign and 21 malignant. Sacrococcygeal and pelvic teratomas predominated (51 cases) and these fell into 3 groups: post sacral, dumb-bell and presacral. The 34 purely posterior tumours were always congenital and benign, whilst the incidence of malignancy in dumb-bell and presacral teratomas increased as the tumour became more internal. Malignant teratomas were carcinomas, usually containing glandular, papillary and clear-cell areas, and metastases were similar. Immature tissues in benign teratomas were usually neural or connective tissue. They did not give rise to neuroblastomas or sarcomas, and did not indicate a worse prognosis. Only 2 originally benign teratomas later developed malignancy.

Adolescent

Male pseudohermaphroditism in XY children with female phenotype.

Twenty-two children with female external genitalia, testes, and 46,XY karyotype, most presenting with inguinal lumps or hernias, underwent bilateral gonadal excision (21 patients) or biopsy. On clinical and biochemical grounds, 13 were classed as androgen resistance (AR), 6 as testosterone biosynthetic defect, 2 as XY gonadal dysgenesis, and 1 as 5 alpha-reductase deficiency. The main pathological difference between the groups was that in AR the gonocytes were present in normal numbers, whereas in testosterone biosynthetic defect, gonocytes, though present in infancy, were rare or absent by 12 years. In all groups, Leydig cells, normally not recognizable between 1 year and puberty, were often present and could not always be attributed to a gonadotropin stimulation test. In four cases the tubules were more heterogeneous than those seen in control testes, but tubular atrophy and increased stroma were indistinguishable from those in age-matched cryptorchid controls. Small Sertoli cell nodules were found in three children, but no carcinoma in situ was identified. The epididymis, said to be absent in adult cases of AR, was consistently present but often cystic or atrophic. Oviduct-like structures were found in three cases of AR, and smooth muscle resembling round ligament in four. Because of the difficulty in distinguishing the types of male pseudohermaphroditism microscopically, we recommend that XY female children should undergo thorough endocrinologic investigation before orchidectomy.

Adolescent