SUMMARY Following hydramnios, five female arthrorgypotic siblings were born around the 30th week of gestation, labour being further complicated by hydrocephalus. Full autopsy on two babies showed a diffuse destructive process in the brain and spinal cord, resulting in hydren‐cephaly in the forebrain, and gliosis elsewhere, the aqueduct of Sylvius being stenosed to produce hydrocephalus. Striking microscopic changes were the glomeruloid vessels in the brain, spinal cord and retina, and the intracytoplasmic inclusions in pericytes; focal necrosis with calcification was a third important finding. Other systems, apart from the deformed limbs and the small suprarenals, were normal. Investigation yielded no evidence of infection in the parents or babies. Various intra‐uterine infections known to produce malformations, including hydrencephaly, are considered and rejected. The condition, apparently not previously described, is thought to be of genetic origin, possibly a defect in the vascularization of the central nervous parenchyma. RÉSUMÉ Hydrocéphalie‐hydrencéphalie congénitales dans une fratrie de cing A la suite d'un hydramnios, cinq soeurs atteintes d'arthrogrypose sont nCes aux environs de la 30 éme semaine de gestation. Le travail fut compliquépar l'hydrocéphalie. Une autopsie complète de deux bébés révéla un processus destructif diffus du cerveau et de la moelle épiniére avec hydrencéphalie du cerveau antCrieur et gliose ailleurs. L'hydrocCphalie Ctait le résultat d'une stéose de l'aqueduc de Sylvius. Trois importantes découvertes ont CtC des modifications microscopiques frappantes des vaisseaux glomtrulaires du cerveau, de la moelle tpinitre et de la rCtine, des inclusions intracytoplasmiques dans les pkricytes et des ntcroses focales avec calcification. A l'excep‐tion. A l'exception de membres dCformCs et de surrtnales petites, les autres organes étaient normaux. L'anamnèse n'a apporté aucune indication d'infection chez les parents ou les bébés. Des infections intra‐utérines variées connues pour produire des malformations, incluant l'hydrencéphalie ont été envisagées et rejettes. Le tableau, qui ne semble pas avoir été décrit auparavant, parait être d'origine gCnktique, se traduisant peut être par un défaut de vascularisation du parenchyme du système nerveux central. ZUSAMMENFASSUNG Nach bestehendem Hydramnion wurden funf weibliche Geschwister mit Arthrogryposis um die 30 igste Schwangerschaftswoche geboren. Die Geburt wurde weiter durch Hydro‐cephalie kompliziert. Eine vollständige Autopsie an zwei Babies zeigte einen diffusen, destruktiven Prozeß im Gehirn und im Rückenmark, der Hydrencephalie im Vorderhirn und Gliosis an anderer Stelle zur Folge hatte. Der Hydrocephaus war bedingt durch eine Stenose des Aqueductus Sylvii. Drei wichtige Befunde waren besonders auffallend: Mikroskopische Veränderungen der glomeruloiden Gefäße im Gehirn, Rückenmark und in der Retina, Einschlußkörperchen im Cytoplasma der Pericyten und fokale Nekrose mit Verkalkungen. Außer deformierten Extremitäten und kleinen Nebennieren gab ...
Despite the suggestions of several writers on the importance of convulsive seizures in infancy as an aetiological factor in the development of cerebral palsies, little attention is given to this association in the standard texts in general medicine and pathology, or even in the more special treatises on diseases of the nervous system. It is not altogether surprising, therefore, that convulsions occurring in the course of febrile illnesses are often not treated with the gravity and urgency they deserve. Because convulsions are so frequent an accompaniment of relatively innocuous infections and in most cases leave the patient apparently normal, the paediatrician may regard both a convulsion of purely febrile origin and the resulting signs of nervous disease as common manifestations of some other intracranial process.The following five cases are presented in the hope that the potentially lethal nature of convulsions will be more widely acknowledged. Materials and MethodsThe pathological material was obtained from necropsies performed at the Adelaide Children's Hospital during the years 1954 to 1956 inclusive. Before immersion of the intact brain pieces were removed for fixation in formolammonium bromide, and in two cases samples were kept for animal inoculation. After prolonged fixation complete coronal slices of the cerebral hemispheres and smaller pieces of brain-stem and cerebellum were embedded in celloidin and paraffin, and stained by Weil's method for myelin sheaths, with Nissl's stain, and by haematoxylin, counterstaining with both eosin and van Gieson's mixture. Frozen sections were used for the metallic impregnation of neurofibrils and astrocytes using Bielschowsky's and Cajal's methods, respectively.Case Reports Case 1. The patient, previously a healthy boy of 2 years, developed measles two days before admission. He had not been very sick but had been coughing a lot. On the day of admission he was found in his cot at 7.45 a.m. in a convulsive attack, blue and frothing at the mouth. This continued until 9.30 a.m. when the convulsion was controlled by ether anaesthesia in the Casualty Department of the Adelaide Children's Hospital where his colour was described by the Admitting Officer as 'almost black'.On examination in the ward his temperature was 103°F., he was slightly cyanosed, with a cold skin on which there was a fading measles rash. The pupils were equal and reacted sluggishly to light. The fundi oculorum were normal. The fauces were moderately reddened, the right tympanic membrane was dull and slightly bulging. Air entry into the lungs was good but there were many rhonchi. The abdomen was normal. Reflexes were absent in the limbs. Slight neck stiffness was present for an hour after the convulsions had ceased. SPECIAL INvEsTIGAIoNs. The cerebrospinal fluid was under a pressure of 100 mm. of water, contained 28 mg. protein per 100 ml. and no cells. The white blood count was 24,000 per c.mm., of which 81% were polymorphonuclear leucocytes. COURSE. In the evening of admission he went pale, with a blood...
OLLUSCUM sebaceum is a common M lesion in South Australia and certainly occurs elsewhere with a greater frequency than the few reports in the literature would indicate. The discrepancy is partially due to the reluctance of many clinicians and pathologists to recognize its separate identity but is mainly the result of indecision in histological differentiation from squamous cell carcinoma so that most of these lesions therefore are not allowed to prove their benign habit by being left to disappear spontaneously. FIG. I. Shows large lesion on the radial edge of the graft, with the two other smaller lesions on the proximal and ulnar borders.The following case of a patient with multiple skin lesions over many years is of interest because of the appearance of typical growths of molluscum sebaceum around the donor and recipient sites of skin grafts following removal of two "squamous cell carcinomas" of the back of the hand. This resulted in clinical diagnosis of malignant recurrence at the original site, and of operative implantation on to the donor area. CASE REPORTThe patient, a man aged 76, had been attending the Radiotherapy Uepartment for twelve years with multiple and recurrent skin tumours of various parts of the dorsum of both hands which had been treated by surgery and irradiation. He presented in October, 1953, with two red nodules which had grown in two months on the right hand. One was in the midst of a scar of a previous lesion, healed following radiation therapy, on the dorsum of the hand; it measured 1.5 cm. in diameter. The other was a smaller lesion over the head of the fifth metacarpal bone and measured just over a centimetre in diameter. Both , had bulging smooth raised edges and a central crust. These two lesions were excised, that on the back of the hand being removed (to include the scar of the previous treatment) with a large area of surrounding skin, which was approximately oval and measured 8 x 5 cm. The deficiencies were covered by split skin grafts taken from the right thigh. The pathologist reported on the two lesions as welldifferentiated squamous cell carcinomas. FIG. 11. Biopsy from the edge of the large lesion shown in Fig. I. (x 6.5)The grafts took well, but two months later the patient returned with further lumps, 3 on the donor site on the right thigh and 3 on the recipient site on the dorsum of the right hand. Those on the right hand (Fig. I ) were all on the junction of the graft with the native skin, measuring 1.3 cm., 0.8 cm. and 0.5 cm. in diameter respectively. The lesions were raised, rounded, smooth nodules, the largest being tomato-red and having a central horny plug suggestive of molluscum sebaceum. The small graft over the head of the metacarpal bone showed
Maruyama, Yoshiharu (Cornell University, Ithaca, N.Y.) and Martin Alexander. Localization of enzymes in the mycelium and microconidia of Fusarium oxysporum. J. Bacteriol. 84:307-312. 1962-Extracts prepared from mycelium and microconidia of Fusarium oxysporum f. cubense were fractionated into a soluble and four particulate fractions by differential centrifugation, and the distribution of several enzymes in the isolated cell constituents was examined. Succinic dehydrogenase, cytochrome oxidase, and a large amount of the reduced diphosphopyridine nucleotide (DPNH) cytochrome c reductase and reduced triphosphopyridine nucleotide cytochrome c reductase were associated with one of the particulate fractions prepared from the hyphae; fumarase and DPNH oxidase activities were largely found in the soluble and in a second particulate fraction. The highest recovery and concentration of diphosphopyridine nucleotidase was observed to be bound to a third type of hyphal granule. Aldolase, aconitase, glucose-6-phosphatase, and uricase were recovered entirely with the soluble mycelium constituents. Similar enzyme-distribution patterns were observed in microconidia. Several enzymatic activities of the mycelial extracts were compared with those in the extracts of microconidia.
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