Two cases of encephalo-angiomatosis are reported. The clinical presentation and certain neurological findings and investigations confirm the diagnosis of a disease which is relatively rare among Blacks. The case reports were considered valuable because of the rareness of the condition. Up to 1962 only 145 cases had been reported in ...

Angiolymphoid hyperplasia has become a well-recognized entity in adults. Kimura's disease is a similar and possibly identical disease occurring in Oriental children. This is a case report of angiolymphoid hyperplasia with eosinophilia occurring in a 12-year-old Caucasian boy with elevated levels of serum IgE. The condition responded to intralesional triamcinolone. ...

Two cases of alpha chain disease are described. The clinicopathological features of the two patients were very similar, and similar to the numerous cases described in the literature, presenting the gastrointestinal form of the disease. In both patients the pathological protein was not noticeable in the electrophoretic pattern. The diagnosis ...

The Authors report their own results concerning plasma hormonal dosages in Benign Breast Disease (BBD) and Premenstrual Mastodynia (PM). Dosages were realized during the second phase of the cycle, 4 to 8 days following ovulation, according to the basal body temperature. 171 Patients were studied: fibro-cystic disease 82 cases; BBD ...

Sixteen consecutive cases of Cushing's disease were treated by radical transphenoidal hypophysectomy. Surgical removal was not possible in two cases. Of the other 14, there was total clinical and biochemical remission of the disease in 13 and partial remission in one. Some form of replacement therapy has been necessary in ...

Cunninghamella, a zygomycete in the order Mucorales, is an extremely rare cause of human infection. Of the five reported cases of human disease caused by this fungus, none involved rhinocerebral infection. Here, the authors document what appears to be the first case of rhinocerebral mucormycosis caused by Cunninghamella bertholletiae in ...

Between 1977 and 1981, the Fish Pathology Laboratory of the Ontario Veterinary College received 239 cases from trout farms of southern Ontario, 51 (21.3%) of which had diseased gills. Branchial lesions in 86.3% of these 51 cases were characterized by marked lamellar epithelial hyperplasia with epithelial hypertrophy and lamellar fusion. ...

The occurrence of both gastrointestinal carcinoid and von Recklinghausen's disease in the same patient is uncommon. There seems to be a predilection for ampullary carcinoids in these patients. This article reports the sixth case, including a review of the literature and a brief discussion of the implications of this finding. ...

Seven cases of ulceration of the small intestine are described and the relationship to coeliac disease is discussed. Evidence for coeliac disease is found in all cases but is less strong in some than in others, and coeliac disease was proved in only two cases. The ulcers were examined histologically ...

Specimens from confirmed cases of Creutzfeldt-Jakob disease (CJD) were examined for evidence of spiroplasma infection. No spiroplasmas or other mycoplasmas were cultivated from brain tissue of 18 cases and no antibodies to several recognised Spiroplasma spp. were detected in sera from 15 patients. These negative cultural and serological results provide ...

Lactic acidosis is a rare metabolic complication of cancer. An analysis of the cases reported in the English and French literature shows that all the patients have extensive neoplastic disease. Metastatic hepatic lesions are present in the large majority of cases, suggesting that alteration of liver function is part of ...

Human adjuvant disease is an immunologically mediated disorder manifested by arthritis, arthralgias, skin lesions, malaise, pyrexia, and weight loss. Clinically, it often resembles rheumatoid arthritis and Reiter's syndrome. While scattered cases of adjuvant disease have been reported following injections with silicone fluid of unknown purity, paraffin, and petroleum jelly, no ...

A case of Menetrier's disease (giant hyperplasia of the gastric mucosa) in a 2-day-old infant is presented. The lesion was primarily confined to the gastric antrum. It resulted in outlet obstruction and necessitated partial gastrectomy. The pertinent literature is critically evaluated, and probably only 2 of the 9 previously reported ...

Caffey's disease or infantile cortical hyperostosis is a rare condition affecting infants in the first five months of life. Varying forms of its presentation are illustrated by the four case reports described. The aetiology, pathology, differential diagnosis and management are discussed. It is possible that Caffey's Disease is becoming more ...

Nodular regenerative hyperplasia of the liver (N.R.H.) is an uncommon clinicopathological entity characterized by diffuse regenerative hepatocytic nodules without fibrosis. Grossly the lesion is often mistaken for cirrhosis. The etiology and pathogenesis are unknown. We report two new cases of N.R.H. diagnosed by liver biopsy. Peritoneoscopically the liver showed a ...

Reports of vertical deviation in Graves' disease in recent years have always been associated with restrictive phenomena and positive forced duction testing, usually related to a tight inferior rectus. Two cases of medically proven Graves' diseased of ten-year duration are presented with increasing vertical disparity. Forced duction testing showed no ...

The use of extradural block (EDB) in healthy parturients for normal delivery and operative procedures is now a widespread and established practice. However, clinical reports of its use in those parturients with severe cardiovascular disease are rare. Here we present two case reports of patients with severe cardiovascular disease delivered ...

A case of abdominal aortic aneurysm with Bechçet's disease and a review of two similar cases previously reported are presented. This case and additional review of other reported major vascular complications of Behçet's disease lead us to conclude that the pathogenesis of the abdominal aneurysm is related to involvement of ...

Occasional normal anatomic variations may produce radiographic appearances that closely simulate disease and visceral enlargement may cause unusual displacements of adjacent organs. Two cases are reported in whom prominent splenic lobulation caused flattening of the upper pole of the left kidney. The appearance simulated that produced by a supra-renal mass. ...

Examination of two siblings who had histories of progressive decline in speech, intelligence, and coordination disclosed vertical supranuclear ophthalmoplegia, hepatosplenomegaly, and signs of diffuse CNS dysfunction. Niemann-Pick "foam cells" were found in the bone marrow of both patients. The features of these cases correlate in appearance and clinical findings with ...

Malignant Histiocytosis (MH) is a disease being recognized with greater frequency. A case is reported together with a literature review to determine the frequency of this occurrence. Of the 108 case reports of MH reviewed, 43 (39%) had head and neck involvement described, with 22 (20%) having extranodal disease. Nine ...

A case of cystosarcoma phylloides is presented here showing the natural history of this disease both clinically and pathologically from 1962 through 1979, and demonstrating the management of this disease by surgery and by chemotherapy with the use of adriamycin at 25 mg/m2 daily, times three days, every 28 days, ...

An unusual case of simultaneously occurring acute syphilitic hepatic and renal disease is presented and a concise literature review of both the pathological and clinical findings in this disease is included. Hepatic and renal biopsies were useful in excluding other diagnostic possibilities and in establishing the correct diagnosis in the ...

Sternocostoclavicular hyperostosis is a recently recognized disease entity of unknown cause. Although the condition bears similarities to Paget's disease, the limited and unique anatomic, clinical, and histologic findings support the hypothesis that this is a separate entity. The case reported satisfies the criteria for sternocostoclavicular hyperostosis and to our knowledge ...

In a case of hepatoerythropoietic porphyria (HEP) with unusual features, the patient had onset of photosensitivity in infancy, followed by spontaneous resolution of photosensitivity by the age of 7 years. Seven other cases of HEP have been found in the medical literature; the disease has systemic complications, mainly liver disease ...

This review of 69 infants (up to two years old) with full preoperative workup, all with urinary calculus disease, covers a fifteen-year period and shows a high incidence of this disease in Turkey when compared with other studies in the literature. It also presents cases of urolithiasis in infancy which, ...

Nodular sclerosis (NS) Hodgkin's disease was pathologically subdivided by cellular composition and degree of fibrosis in a series of 49 children admitted to the Istituto Nazionale Tumori of Milan between 1967 and 1977. NS showed lymphocytic predominance (LP) in 26 cases, mixed cellularity (MC) in 16 cases, and lymphocytic depletion ...

Nine cases of peliosis hepatis are reported: five of these were associated with the administration of androgen or anabolic androgenic steroids and a sixth with large doses of medroxy-progesterone acetate. In five instances, neoplasm was present as an underlying disease. Antemortem evidence of liver disease was detected in six of ...

The current concepts of histiocytosis X are discussed, and the literature concering this rare disease of obscure aetiology reviewed. Two case reports of histiocytosis X presenting in similar sites in young children of similar ages, but with markedly different courses, are presented. The importance of early recognition of the disease, ...

We report the management of a case of malignant trophoblastic disease which presented initially as a haematemesis and melaena from a gastric metastasis. The patient had had a tubal sterilization 4 years previously. The management of trophoblastic disease in general is also discussed, and the importance of chemotherapy is stressed.

Two cases of Hodgkin disease, lymphocyte depletion type, with bronchial involvement are described. One patient revealed involvement of the right upper lobar bronchus, the other patient of the left main bronchus. The correct diagnosis was made by fiberbronchoscope examination with cytologic brushing and biopsy. That bronchial involvement by Hodgkin disease ...

A case of immunoblastic lymphadenopathy has been presented which had the characteristic clinical and histological features. The patient did not respond to chemotherapy and succumbed to the disease. Instead of the usual polyclonal gammopathy associated with this condition, he showed a fall in IgM levels, IgG and IgA being within ...

Six of 41 presumed cases of Van Buchem disease described in the literature fit uniform diagnostic criteria. Segregation analysis of these 6 cases, in addition to another the authors report, supports a recessive mode of inheritance. Genetic heterogeneity is confirmed by the demonstration of a dominantly-inherited phenotype resembling Van Buchem ...

This paper reports a peculiar case seen in a 36-year-old woman who every summer since the age of 30 has developed numerous solitary vesicles or crusted papules with intense pruritus in the seborrheic zones. There are no general symptoms and no hereditary relations. The individual rash disappears completely in a ...

We reviewed the published reports of skeletal cryptococcosis and added three cases to the fifty-six in the literature. Eight of the patients in the reported cases probably did not have primary skeletal cryptococcosis. The potential toxicity of antifungal drugs in current use and the apparent effectiveness of surgical treatment for ...

In a female infant with severe combined immunodeficiency disease extraneous HLA specificities were found which could neither have been inherited from the father nor the mother. The case is reported in detail and a survey of the literature with similar observations is given. The different explanations of this phenomenon are ...

A case report in which argyria is mistaken for cyanotic heart disease is described. A discussion of argyria, a benign cosmetic condition resulting from either ingestion or the local application of silver salts or colloidal silver, is presented. It is suggested that by obtaining comprehensive drug histories, maintaining patient profiles, ...

A case of neurofibromatosis involving the larynx is added to the 19 previously reported cases; furthermore, it represents the only reported case of plexiform ganglioneurofibroma of the larynx in Von Recklinghausen's disease. The reported cases of neurofibromatosis with laryngeal involvement in the world literature are discussed and summarized. The main ...

Few cases of pachyonychia congenita are reported in the dental and medical literature because of the rarity of the disease. This article presents a review of the literature and adds a new case history. Examination of a 4-year-old boy revealed the presence of the disease, which was also present in ...

A review of the last seventy-eight consecutive cases of Hodgkin's disease seen at our hospital revealed that two presented several hematologic cytopenias. These two cases and a similar case first seen at an associated hospital revealed pancytopenia in two and leukopenia in the third. All responded to splenectomy with hematologic ...

During the last five years (1970-1974) 42 cases of pseudotuberculosis were diagnosed among the Antwerp Zoo animals. The prevalence of disease, and the distribution frequency of gross lesions in the visceral organs were studied. The susceptibility of different animals and the influence of some factors to the disease are discussed. ...

Two-year disease-free survival is considered highly predictive of therapeutic success with neuroblastoma and death from neuroblastoma greater than 5 years after diagnosis is a rare event. We report of an unusual case of death from neuroblastoma 20 years after diagnosis; a literature review and discussion of this rare phenomenon of ...

Three cases of "shock kidneys" were demonstrated by excretory urography. A dense, prolonged nephrogram and decreased excretion of the contrast material during the hypotensive episode were seen. In addition, our cases show bilaterally small kidneys without evidence of underlying chronic renal disease, which we consider to be a normal physiologic ...

The authors describe a case of subarachnoid hemorrhage secondary to moyamoya disease in an adult Negro. To the authors' knowledge, this is the first such case reported. Moyamoya, the Japanese word for "puff of smoke," describes the characteristic angiographic appearance of the collateral arterial network associated with internal carotid artery ...

Maggot infestation in a series of 100 cases pertaining to the E.N.T. domain is reported. The Entomological aspects were studied in 20 cases. The clinical features of myiasis have been described. This disease commonly occurs in diseased nasal and ear cavities which allow maggots to grow. The use of maggot ...

We have described an unusual case of multifocal eosinophilic granuloma ("Hand-Schueller-Christian disease") in a middle-aged woman. The case underscores the varied and subtle nature of the disease presentation and the extent to which many organ systems may become involved. Unusual features of her case include atypical bone roentgenograms, cutaneous anergy, ...

Five cases of Nocardia infection are briefly described, four due to Nocardia asteroides and one to N. brasiliensis. The latter represents the first reported case of N. brasiliensis infection in Israel. In three of the patients, the infections were associated with an underlying disease process and with prolonged high-dosage therapy ...

Exaggerated onychodermal bands and a unilateral racket thumb nail occurred in a healthy 36-year-old primiparous female. Each condition is described and the relationship of exaggerated onychodermal bands to chronic renal disease (systemic disease) is reviewed. We are reporting this patient since we have been unable to find a similar reported ...

The diseases, lesions, congenital disorders, and parasites of both Insectivora and prosimian shrews were reviewed. Preliminary baseline data provide encouraging prospects for using these animals as research models because few spontaneous diseases and lesions have been reported. It must be pointed out, however, that throughout the literature reviewed, clinical histories ...

Seven cases of osteochondritis dissecans following Legg-Calvé-Perthes disease are presented and the literature is reviewed. This complication could not be predicted during the early stages of the Legg-Calvé-Perthes process. A high index of suspicion is necessary to recognize the lesion. Conservative treatment is suggested.