[Congenital craniopharyngioma. Reflections on the histopathogenesis and coincidence with abnormalities].
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Biomedical subjects
Publications and source records attributed to F Pascu.
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Thirteen newborns with lethal short rib-polydactyly (SRP) have been reviewed, 11 with SRP type III (Verma-Naumoff) and 2 with SRP type II (Majewski). In the former group there were three sets of siblings. The excess of males with SRP type III (Verma-Naumoff) is confirmed in this present study. A high frequency of phenotypic females including sex-reversed constitutional males with SRP type I (Saldino-Noonan) is in marked contrast to these findings in SRP type III. Possible hypotheses include variable expressivity in non-Majewski short rib-polydactyly syndromes with sex-reversed and constitutional female cases tending to show more severe phenotypic expression both in terms of major anomalies and skeletal dysplastic effects.
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Extension of intensive care seems to have increased the risk of systemic candida infection. We report the incidence of systemic candida infection in 8 low-birth-weight infants (gestational age 27-32 weeks, birth weight 710-1,550 g). All infants required respiratory treatment. Various silastic catheters were inserted. Antibiotic therapy was started on the first day of life, usually a combination of ampicillin and gentamycin. Candida septicaemia was diagnosed at the age of 8-69 days of life based on blood and urine cultures, in two children at autopsy. There were no specific clinical symptoms in regard to candida infection. Sonographic technique revealed hydronephrosis in 3 infants due to candida mycelium. Antimycotic therapy included amphotericin B (dosage 0.44-1.0 mg/kg X day) and 5-fluorocytosine (80-100 mg/kg X day) as well as a monotherapy of 5-fluorocytosine (100-200 mg/kg X day). Four children were treated successfully. We like to advice a regular search for candida in urine, blood, tracheal secretion, stool and skin in low-birth-weight infants under intensive care conditions. If antimycotic therapy is started in time, therapy can be successful.
To investigate the effect of nutrition on tissue carnitine concentrations in infants of different gestational ages, specimens of muscle, heart, liver and kidney obtained at autopsy from 62 infants were analyzed for total acid-soluble carnitine content. Immature infants had smaller carnitine tissue reserves than term infants. Parenterally alimented premature infants (greater than 10 days old) had lower carnitine levels in heart, liver and kidney than those dying within 24 h after birth. Infants who received oral or intravenous carnitine had larger carnitine tissue reserves than those who did not. These data suggest that both gestational age and exogenous carnitine supply affect tissue carnitine reserves.
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This disease is discussed in the past mostly in the dermatological literature, the ENT specialist is little aware of its existence. The condition is characterized by subcutaneous, painless nodules occurring most frequently in the region of the ear, parotid gland, cheek and lateral neck. The lesion must be differentiated from disease of the lymph nodes, especially malignant tumours. The pathologic diagnosis is based upon a hyperplasia of angiolymphatic tissue and marked blood eosinophilia. The prognosis appears to be favourable. A case is presented together with the clinical picture and histopathological findings. The differential diagnosis and treatment are discussed.
Although nasal polypectomy is a frequently performed procedure in ENT Clinics, tissues taken may only seldom be histologically examined. We have recently uncovered an esthesioneuroblastoma during a routine polypectomy, and believe that even slightly changed "polyps" or fleshy or pinkish-gray appearing "polyps" seen with dicumarol therapy may mask tissue malignancy.
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The syndrome of pure gonadal dysgenesis (PGD) cannot always easily be distinguished from other disorders of gonadal development. Relations are evident with Turner's syndrome, females with hypoplastic ovaries, male pseudohermaphroditism, mixed gonadal dysgenesis and the vanishing testes syndrome. The case is reported of a 40 year old female with primary amenorrhea, alopecia, eunuchoid features, XY karyotype with normal breast development and sexual hair after estrogen therapy. On laparotomy streak ovaries were found at ovarian site. Pathohistological examination revealed on the left side wolffian duct remnants such as ductuli deferentes and epididymis besides sparse Leydig-(hilus-)cells and on the right side only a rudimentary fallopian tube with subendothelial accumulation of hyperplastic Leydig-(hilus-)cells. Serum-testosterone elevation above the normal female range (630 ng/dl) persisted following gonadectomy (151 ng/dl). Ectopic Leydig-(hilus-)cells were regarded responsible for the continuing testosterone production. The present case lies on borderline between PGD and mixed gonadal dysgenesis because remnants of wolffian duct derivatives suggest unilateral fetal testicular activity; classification as PGD however was justified in purely female body features and lacking evidence of testicular tissue.
A case of a dissecting aneurysm of the splenic artery is presented. Although aneurysms of the splenic artery are common, a dissecting aneurysm has been mentioned infrequently in the available literature. Clinical aspects, confirmation of the diagnosis by celiac arteriography and surgical therapy are described. The pathogenesis is discussed briefly. The necessity of clinical awareness in cases of obscure abdominal pain is emphasized.
A case of primary carcinoma of the Fallopian tube was discovered on routine mass screening of cervical smears. There were no symptoms and gynaecological findings were unremarkable. Atypical glandular epithelia in the smear may not, therefore, be adenocarcinoma of the cervix or body of the uterus, but could also be from an extrauterine carcinoma, especially if cervical scrapings are negative.
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