Nocardia bacteremia. Report of 4 cases and review of the literature

Laparoscopic colon surgery is gaining acceptance for benign conditions, but cannot yet be considered an established procedure for malignancy. The main reported benefit of the technique is the reduction in length of hospital stay. Other potential benefits such as cosmesis, improvement in quality of life, physiologic and immunologic advantages, as well as reduced complication rates have not been clearly demonstrated. Concerns about laparoscopic colon surgery for cancer including the possibility of inadequate resection, tumor staging, and altered tumor spread due to pneumoperitoneum have only been partially addressed by retrospective and experimental studies and require a prospective randomized trial for definitive resolution. Details of the trial currently underway sponsored by the National Institutes of Health are described. Although innovations in clinical practice and increased familiarity account for the expanding popularity of laparoscopic colon surgery, results from this and similar worldwide trials are needed before this approach can be recommended for cancer.     

Non-alcoholic steatohepatitis. Report of five cases and review of the literature

We describe three men and two women, aged 18-50, with an occasional finding of increased aspartate and alanine aminotransferase and gamma-glutamyl transpeptidase levels in the absence of any drug treatment and past or current alcohol abuse. Two patients were overweight (body mass index 29 and 32, respectively) and physical examination was normal in all but one case. Tests for hepatitis A, B and C, Epstein-Barr virus, cytomegalovirus, toxoplasma and autoimmune hepatitis were negative and metabolic diseases (Wilson's disease, haemochromatosis, alpha-l-antitrypsin deficiency) were excluded by specific tests. Ultrasound liver scan revealed massive steatosis in all patients. Liver histology showed diffuse steatosis and parenchymal inflammation in all cases, with concomitant fibrosis and Mallory bodies in three of them. Findings were consistent with non-alcoholic steatohepatitis, a rare condition with potential progression to cirrhosis in a minority of cases. This disease, for which no treatment is currently available, must be considered in all subjects with elevated aminotransferases, in the absence of known causes of liver damage.     

Active infective endocarditis in infants and childhood: ten-year review of surgical therapy

OBJECTIVE: The aim of this study was to determine whether or not continuous combined HRT used with GnRH-a for the treatment of endometriosis can prevent hypoestrogenic side effects associated with GnRH-a. METHODS: Forty premenopausal women with laparoscopically proven endometriosis entered the study. The patients were randomized into two groups. Group I (n = 19) received 3.75 mg i.m. leuprolide acetate (LA) every 4 weeks for 24 weeks. Group II (n = 21) received 3.7 mg LA combined with 1.25 mg oral conjugated equine estrogen (CEE) and 5 mg oral medroxyprogesterone acetate (MA). RESULTS: Total revised AFS score as well as total pelvic pain scores decreased significantly (P < .001) in both groups. However, a statistically significant difference of hot flushes and sweating was reported by women receiving LA + HRT as compared to those treated with LA alone (P < .001). Furthermore, the bone loss at the lumbar spine was 4.2% in group I compared to 0.9% in group II at the end of the study. CONCLUSIONS: This study suggests that 1.25 mg CEE + 5 mg MA is effective in preventing hypoestrogenic side effects caused by GnRH-a, while the treatment of endometriosis is not impaired.     

Male genitourinary tuberculosis. Report of 12 cases and review of the literature

The incidence of genitourinary tuberculosis is constant in western countries, despite dramatically falling prevalence of pulmonary tuberculosis. The kidney is especially involved in urinary tuberculosis, and genital tuberculosis mostly affects the epididymis in males and the fallopian tubes in females. Twelve cases of male genitourinary tuberculosis are presented and the literature is briefly reviewed.     

Carotid sinus syndrome. Report of five cases and review of the literature

Five cases of carotid sinus syndrome are presented. The syndrome is defined by spontaneous attacks of dizziness and fainting which can be reproduced by graded pressure on one carotid sinus. Three forms of the clinical syndrome, cardioinhibitory, vasodepressor, and cerebral, are discussed. The hyperactive carotid sinsu reflex, in which there is ventricular asystole lasting at least 3 seconds or a decrease of more than 50 mm Hg in systolic and diastolic blood pressure, should be differentiated from this syndrome. Treatment modalities include reassurance, drugs, radiotherapy, cardiac pacemakers, and surgical approaches. Carotid sinus syndrome should be considered in the differential diagnosis of unexplained syncope, arteriovenous block, or inappropriate sinus bradycardia.     

Prednisone as adjunctive therapy in the management of pulmonary tuberculosis. Report of 12 cases and review of the literature

A retrospective chart review was conducted over a 5-year period (1988 to 1993) in a tertiary inpatient care center on the effects of the addition of prednisone to the treatment regimens of 12 patients with pulmonary tuberculosis who continued to spike high temperatures and lose weight while showing bacteriologic response to effective antituberculosis therapy. After exclusion of other causes of fever, all patients were treated with 20 to 60 mg of prednisone daily until normalization of temperature and clinical improvement. Analyzed data included twice weekly sputum bacillary count, temperature record every 4 h, weekly patient weight, serum albumin level, liver function tests, and chest roentgenogram. The patients continued to spike temperatures of 38.3 degrees C to 40.5 degrees C (mean +/- SD = 39.6 degrees C +/- 0.6 degrees C) even after 18 to 53 days (mean +/- SD = 33.9 +/- 9.8 days) of antituberculosis therapy. Within 24 h after the addition of oral prednisone, temperature decreased in all 12 patients from a daily highest spike mean of 39.6 degrees C +/- 0.6 degrees C (SD) to 38.1 degrees C +/- 0.6 degrees C (SD) (p = 0.0022). The duration of required prednisone therapy was 20.1 +/- 9 days (mean +/- SD). During this period patients' appetites improved, and their weight increased from a mean (+/- SD) of 53.6 +/- 5.7 kg to 58.1 +/- 6.4 kg (p = 0.0022). The serum albumin level increased from a mean (+/- SD) of 2.51 +/- 0.4 g/dL to 3.21 +/- 0.4 g/dL (p = 0.0033). All the patients also showed clinical evidence of a decrease in toxic reactions associated with tuberculosis. There were no side effects from the addition of prednisone. This study shows the need for randomized controlled clinical trials to clarify the role of prednisone as adjunctive therapy in the management of pulmonary tuberculosis.     

Alstr?m syndrome. Report of 22 cases and literature review

OBJECTIVE: The authors report 22 cases of Alstr?m syndrome (AS), which is the largest series to date. Only 37 cases have been reported in the world literature since 1959. The authors review the clinical features and compare these with the overlapping condition of Bardet-Biedl syndrome. Their aim is to clarify the AS phenotype and to increase awareness of the early features. DESIGN: A retrospective case series. PARTICIPANTS: All patients (22) with a diagnosis of AS admitted to the authors' hospital in the past 10 years were included in this review. INTERVENTION: This is principally a review of ocular features, but other features are recorded and discussed. MAIN OUTCOME MEASURES: Features noted included age at onset of visual symptoms, presence of photophobia, visual acuity, and electroretinogram findings. Nonocular features recorded included cardiac status, weight and height, hearing, and presence of diabetes mellitus. RESULTS: Cardiomyopathy presenting in infancy has only been recognized recently to be a feature of AS. Of the authors' cases, 18 of 22 had infantile cardiomyopathy. In the authors' tertiary referral institution, there is an ascertainment bias toward younger patients and especially those with pathology that is other than ocular pathology. In addition, AS is difficult to recognize in childhood without the development of infantile cardiomyopathy. Alstr?m syndrome often is not recognized until diabetes mellitus develops in the second or third decade. Initially, a diagnosis of cone-rod dystrophy, achromatopsia, Leber's congenital amaurosis, or Bardet-Biedl syndrome may be made. In AS, there is a severe infantile retinal dystrophy. The electroretinogram is absent or attenuated with better preserved rod than cone function. The retinal dystrophy is progressive with the patient's visual acuity of 6/60 or less by 10 years of age and no light perception by 20 years of age. CONCLUSIONS: A diagnosis of AS should be considered in infantile cone and rod retinal dystrophy, particularly if the weight is above the 90th percentile (16 of 18 cases) or if there is an infantile cardiomyopathy (18 of 22 cases).     

Allergy to Anisakis simplex. Report of 2 cases and review of the literature

The metabolism of estradiol was investigated in postmenopausal women after 4 weeks' treatment with oral or transdermal unopposed estradiol. The urinary excretion of the metabolites was examined. With both administration routes, 2-hydroxyestrone, the main A-ring metabolite, and 16alpha-hydroxyestrone, the main D-ring metabolite, were excreted in higher amounts than estradiol and estrone. The ratio of 2-hydroxyestrone to 16alpha-hydroxyestrone remained the same for both administration routes. It has been suggested that dominance of D-ring metabolism, i.e. increase of 16alpha-hydroxyestrone production, is associated with an increased risk of breast cancer. The present study indicates that neither oral nor transdermal estradiol substitution shift this ratio to a higher level of possible risk. Oral estradiol substitution, however, in our study leads to higher metabolite concentrations which may be regarded as hazardous for women with diseases favoring D-ring metabolism.     

Primary cerebral melanomas. Report of six cases and a review of the literature

Six cases of primary malignant melanoma of the leptomeninges and brain are reported. Three were diagnosed histologically at surgery, three at autopsy. Four presented as solitary brain tumours which is unusual compared to other reported cases. In all six there was infiltration of the leptomeninges which must be regarded as the site of origin. Three of the cases have had a relatively long survival, two of them have died 2 years after the operation, and the third patient is still alive 18 months after surgery. It is suggested that the frequency of these tumours is underestimated on account of their resemblance to astroblastomas and poorly differentiated astrocytomas, and because biopsies are not always taken from small and atypical haematomas.     

Leiomyosarcomas of the oral cavity. Report of four cases and review of the literature

Leiomyosarcomas (LMS) make up 7% of all soft tissue sarcomas. In the oral cavity, the LMS is rare due to the paucity of smooth muscle in that region. Four cases of intraoral LMS are reported, plus 34 cases found in the literature reviewed. There were 24 males and 14 females. The age range at presentation was 10 months to 88 years with no predilection for any particular age group. The commonest presenting symptom was a mass. The intraoral LMS occurred most commonly in the jaws (59% of cases). The mass was painful in 61% of cases. Follow-up of the case reports was assessed. Recurrence occurred in 36% of cases. Distant metastases occurred in 39% of cases, most commonly to the lungs. Cervical nodal metastasis was reported in 15% of cases. The 5-year survival rate determined on cases with adequate follow-up (n = 13) was 23% free of disease, 8% alive with disease and 69% who died of disease. In comparison with stage I and stage II tongue squamous cell carcinomas, the intraoral LMS is very aggressive. It is best treated surgically, early and aggressively.     

Cutaneous Cryptococcus infection and AIDS. Report of 12 cases and review of the literature

BACKGROUND: Cryptococcal infections occur in 6% to 13% of patients with acquired immunodeficiency syndrome (AIDS), most commonly infecting the central nervous system. Cutaneous lesions have been described morphologically as umbilicated papules, nodules, and violaceous plaques and can mimic molluscum contagiosum and Kaposi's sarcoma. Cutaneous lesions can present months prior to other signs of systemic infection. OBSERVATIONS: Cases of infection with cutaneous Cryptococcus and AIDS were reviewed and compared with cases reported in the literature. Among patients with Cryptococcus infection and AIDS seen at our institutions, 5.9% had skin lesions. All patients with cutaneous lesions had systemic involvement. Women were less commonly infected than men. There was no apparent predisposition associated with age, race, or human immunodeficiency virus infection risk factors. The median CD4 helper T-cell count was 0.024 X 10(9)/L (24/microL), and 44% (16/36) of the patients had previous opportunistic infections. Lesions were most commonly seen on the head and neck (78% [36/46]) and often mimicked molluscum contagiosum (54% [25/46]). The median serum and cerebrospinal fluid cryptococcal antigen titers were 1:32,768 and 1:512, respectively. Patients in our group did well with therapy (one death at 6 weeks, compared with 38% [13/34] mortality in the literature). There was no correlation between onset of lesions, number of lesions, CD4 helper T-cell count, or histopathologic characteristics. CONCLUSIONS: Disseminated Cryptococcus infection in AIDS presents with cutaneous lesions in up to 6% of cases. Clinicians need to be aware of the varied morphologic characteristics, since cutaneous lesions may present well in advance of other signs of systemic infection.     

Pseudotumor forms of demyelinating diseases. Report of three cases and review of the literature

INTRODUCTION AND CLINICAL CASES: We present three patients with unusual clinical findings studied in our hospital, after a period of follow-up of at least two years from the time of diagnosis of their demyelinating condition. We discuss the clinical onset, CT and magnetic resonance findings, neuropathological studies and posterior clinical course. Anatomopathological studies were done in two of the cases, since the diagnoses were not clear and the other paraclinical investigations did not clarify things. DISCUSSION: The presence of large space-occupying lesions or ring-like outlines in myelinating disorders may make it difficult to make a differential diagnosis from other conditions such as neoplasias and abscesses. This may lead to an erroneous initial diagnosis and even to the use of unsuitable, aggressive treatments. CONCLUSIONS: In young patients in whom radiological imaging suggests space-occupying lesions (single or multiple) one should consider the possibility of a primary demyelinating disease of the central nervous system with the appearance of a pseudo-tumour. In certain cases, stereotaxic biopsy should be considered if the diagnosis remains in doubt, rather than begin unsuitable treatment. The fundamental reason for the presentation of our cases is to emphasize that these pseudo-tumoral forms of demyelinating diseases should be considered in diagnosis.     

Mesenchymal chondrosarcoma of the orbit. Report of three new cases and review of the literature

BACKGROUND: Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all sites, long-term survival is approximately 30%. Only seven cases of orbital mesenchymal chondrosarcoma have been reported. METHODS: The records of three cases of orbital mesenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical Center, and the seven previously reported cases of this tumor were reviewed to determine clinical characteristics and appropriate therapy. RESULTS: Nine of the 10 patients were female; age of onset ranged from 10 to 35 years. Of eight patients with at least 2 years of observation, five survived 5 or more years after resection, or after resection plus adjuvant therapy. Two patients died of metastatic disease 2 and 5 years, respectively, after the initial treatment, and one died of pneumonia 6 months after surgery. Review of the histology of the three cases treated at Columbia-Presbyterian Medical Center did not identify distinct histologic types that might guide therapy. Presenting symptoms were typical of symptoms of an orbital mass: proptosis, pain, diplopia, change in visual acuity, ptosis, and tearing. CONCLUSION: The small number of reported cases of mesenchymal chondrosarcoma of the orbit prevents definitive conclusions, but it appears that resection is adequate therapy in some cases. Extraskeletal mesenchymal chondrosarcoma of the orbit may have a better prognosis than tumors in other sites.     

Endodermal cysts of the posterior fossa. Report of three cases and review of the literature

The pathogenesis of endodermal cysts of the posterior fossa is still incompletely understood. The authors reviewed three new cases and those reported in the literature to clarify the clinical, pathological, radiological, and surgical characteristics of these lesions. A total of 49 cases were reviewed. Details on demographic profiles, clinical characteristics, histopathological and radiological features, and surgical methods were collected. These cysts have a predominance in male patients (61%) and can occur at any age (birth-77 years). In patients with posterior fossa endodermal cysts there is a bimodal age distribution and headache is the most frequent complaint. On immunohistopathological examination, endodermal cysts were reactive for epithelial membrane antigen and for keratin immunostains whenever the latter were tested. The cysts were reactive for carcinoembryonic antigen in nine of 11 cases. Endodermal cysts were located anterior to the brainstem in 51% of cases and in the fourth ventricle in 21% of cases. They frequently appeared hypodense on computerized tomography scans, and in five cases, the lesion was missed. The cyst's appearance on magnetic resonance imaging is variable. Resection was complete in 19 cases and partial in 11; marsupialization was achieved in two cases. Three recurrences have been reported. Total excision with preservation of neurological function should be the goal. Cranial base approaches are helpful for surgical access in selected examples of these lesions.     

Subacute bacterial endocarditis due to Actinobacillus actinomycetemcomitans. Report of a case with a review of the literature

A case of subacute bacterial endocarditis due to Actinobacillus actinomycetemcomitans is reported. The patient was successfully treated first by a combination of gentamicin and ampicillin and then, because of severe allergic reactions, ampicillin was replaced by co-trimoxazole; symptoms did not recur and blood cultures remained sterile. A synoptic table is presented of 19 reported cases of infection caused by A. actinomycetemcomitans not connected with actinomycosis, with particular regard to their clinical features, treatment, and outcome.     

Salmonella colitis. Report of two cases and literature review (author''s transl)

Uterine activity was measured for a 30-minute preinjection period and a 30-minute postinjection period for six drugs commonly used in labor. Uterine activity was quantitated by measuring the area below the uterine pressure curve with an on-line technic utilizing a voltage control oscillator. Uterine activity readings were obtained every 2.5 minutes and reported in uterine activity units (UAU). Unmedicated labor demonstrated a relatively static increase in UAU per unit of time in labor. On comparing total UAU before and after injection, only magnesium sulfate was noted to cause a decrease in total UAU. Utilizing 10-minute summations of UAU, the pre- and postinjection periods were compared to the calculated expected UAU for the 30 minutes following the preinjection period (assuming no drug had been used). For each of the other drugs, the positive slope of the calculated expected UAU shifted to a negative slope of the actual observed UAU following drug administration.     

Breast hamartoma with invasive ductal carcinoma. Report of two cases and review of the literature

Two cases of well-defined masses also containing clinical and radiographical abnormalities suggestive of malignancy, subsequently found to be invasive ductal carcinomas in breast hamartomas are described. The patients were 53 and 78 years old. Both presented with a generally soft palpable breast lump, containing a firm area which in one case invaded and ulcerated the skin. Mammography demonstrated two typical hamartomas: one containing a spiculated opacity, the other irregular opacities with suspicious calcifications, suggesting the presence of carcinomas in these benign lesions. The cut surface of these well-circumscribed masses measured 5 cm and 7 cm. The microscopic appearance was characteristic of breast hamartoma (sharp circumscribed "pseudocapsule" surrounding breast fibrocystic changes with variable amounts of adipose tissue) with the firm area in each case corresponding to invasive ductal carcinoma. In one case the invasive ductal carcinoma was confined to the hamartoma, whereas in the other malignant tumor, cells extended beyond the surrounding breast tissue and infiltrated the skin. These findings raise the question of secondary involvement of a hamartoma by invasive carcinoma. Breast hamartomas are probably underrecognized lesions. In our view, these findings do not justify a more aggressive approach towards the management of breast hamartomas.