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.     

Brachial plexus injury during cardiac catheterisation in children. Report of two cases

The arm is particularly vulnerable to neurological injury, due to the intimate anatomical relationship between the brachial plexus and the traction zones. Severe injuries of the brachial plexus can be caused by compression, traction or laceration. Fortunately, many deficits are superficial and permanent neurovascular deficits are rare. Nevertheless, it is important to identify the probable cause of the injury since the prognosis for recovery directly depends on the underlying nature of the neurological deficit. Two anaesthetised children who suffered brachial plexus injury during cardiac catheterisation are reported. The first, with Ebstein's anomaly and significant pulmonary valve stenosis, presented, after the procedure, with a right arm motor deficit with proximal predominance. The second patient, with tetralogy of Fallot and pulmonary atresia, presented difficulty in left arm abduction and external rotation on awakening. The risk factors for brachial plexus lesions during anaesthesia are discussed. These include improper positioning, anaesthetic agents, extreme variations of body mass index and anatomical anomalies. Prevention, evolution and treatment of the brachial plexus injury are also considered. With proper care by the cardio-radiologist and anaesthesiologist the frequency of this injury can be reduced.     

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.     

Association between inflammatory bowel disease and sarcoidosis. Report of two cases and review of the literature

BACKGROUND: Common etiopathogenic factors may explain the association of systemic sarcoidosis with inflammatory bowel disease. METHODS: We report two cases of such an association: one of sarcoidosis that developed 2 years after proctocolectomy for ulcerative colitis and one of sarcoidosis and Crohn's colitis. Factors like increased cellular immunity or circulating immunocomplexes or autoantibodies may have a role. Exogenous agents or familiarity may also be involved. CONCLUSIONS: It is postulated that the association between sarcoidosis and inflammatory bowel disease (both ulcerative colitis and Crohn's disease) does not occur by chance alone and that the two conditions may share some genetic or immunologic alterations. The two diseases, however, follow an independent clinical course.     

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.     

Vascular and visceral injuries associated with lumbar disc surgery: medicolegal implications

Symptomatic perforation of the anterior annulus fibrosus/anterior longitudinal ligament during surgery for herniated lumbar disc disease is one of the more solemn and sobering complications experienced by neurosurgeons or orthopedic surgeons. This complication frequently results in the death of the patient. Its occurrence is probably more common than the medical community would expect. The authors report 21 cases since 1985 in which an injury to an intra-abdominal vessel or viscera occurred. In all cases litigation resulted and a settlement or verdict was rendered. A review of the literature is presented and the medicolegal implications of symptomatic ventral perforations of the annulus fibrosus/anterior longitudinal ligament are discussed.     

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.     

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.     

Calcification of the basal ganglia and Fahr disease. Report of two clinical cases and review of the literature

gamma-Aminobutyric acidA (GABAA) receptors are linked to ion channels which mediate many aspects of neural inhibition. Although the effects of phosphorylation on GABAA receptor function have been widely studied, the actual role of phosphorylation in the regulation of these receptors still remains controversial. In recent reports, we have described the effects of phosphorylating/dephosphorylating enzymes on the regulation of GABAA receptors in a rat cortical slice preparation (Shaw et al., Mol. Neuropharmacol., 2 (1992) 297-302; Shaw and Lanius, Dev. Brain Res., 70 (1992) 153-161; Pasqualotto et al., Neuroreport, 4 (1993) 447-450) and predicted that ionic co-factors are involved in mediating the regulation of GABAA receptors by kinases and phosphatases. In the present report, the effects of chloride, sodium, potassium, and calcium were examined alone and in the presence of cAMP-dependent protein kinase (protein kinase A) or alkaline phosphatase. The results showed a decrease in [3H]SR 95531 (GABAA receptor antagonist) binding after incubation with chloride alone; this decrease was further enhanced in the presence of protein kinase A. Both effects could be blocked by a protein kinase A inhibitor. Conversely, an increase in [3H]SR 95531 binding was observed after incubation with sodium alone; this increase was further enhanced in the presence of alkaline phosphatase. In both cases these increases in binding could be blocked by sodium orthovanadate, a phosphatase inhibitor. Potassium was ineffective under all conditions; calcium showed enzyme-independent effects at low concentrations only. These results suggest the existence of a novel chloride-dependent protein kinase which may have significant sequence homology to protein kinase A, and a novel sodium-dependent phosphatase.(ABSTRACT TRUNCATED AT 250 WORDS)     

Typical and atypical fractures of the odontoid process in young children. Report of two cases and a review of the literature

The most common injury to the odontoid process in children under the age of seven years is a fracture through the synchondrosis with or without anterior displacement of the odontoid process, but this is not the only type of fracture of the odontoid process in this age-group. Fractures above and below the synchondrosis and fractures with posterior displacement were described. Typical clinical features of these fractures are: (1) major and blunt trauma, (2) neck pain and resistance to active and passive head movements; and (3) no or only slight neurological deficits. Conservative treatment had excellent results in the majority of cases. Nevertheless, there are a few specific indications for surgery.     

Spinal anesthesia for lumbar disc surgery: review of 576 operations

Neurologic complications accompanying spinal anesthesia were examined in 576 lumbar disc operations on 507 patients. The single serious complication did not seem attributable to the choice of anesthetic method. Minor neurologic complications, with the exception of spinal headache, could be explained by surgical manipulation. The authors conclude that spinal anesthesia is safe for surgical operations on the laterally herniated lumbar disc.     

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.     

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.     

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).     

Renal myxoma. A report of two cases and review of the literature

Renal myxomas are rare neoplasms. Seven cases have been reported, of which only two are convincingly diagnosed as myxoma; the remaining cases exhibit features of sarcoma, fibroepithelial polyp, or myxolipoma. We report two additional cases; one in a 52-year-old man and another in a 68-year-old woman. They were discovered incidentally by radiological examination. The resected kidney in both patients contained a well-demarcated gelatinous intraparenchymal tumor, which consisted of occasional slender spindle cells scattered in an abundant myxoid stroma, closely resembling myxomas of other sites. The tumor cells showed immunoreactivity for vimentin but not for S-100 protein, epithelial membrane antigen (EMA), CAM 5.2, HHF-35, or smooth muscle actin. Ultrastructural features were of fibroblast-like cells with an elaborate network of cytoplasmic processes. The differential diagnosis of myxoid tumors of the kidney includes myxoid variants of renal sarcomas and carcinomas, renomedullary interstitial cell tumors, and fibroepithelial polyps. It is important to recognize the existence of a renal myxoma, to avoid confusing this benign tumor with the malignant neoplasms with secondary myxoid features that may involve the kidney.     

Familial erythrophagocytic lymphohistocytosis. Report of two cases and clinicopathologic review

This paper deals with the kinetic inhibition of six cephalosporinases (cephalosporin amido-beta-lactamhydrolase, EC 3.5.2.8) by carbenicillin. In previous cases, the inhibition has appeared usually to be competitive and slowly reversible. This makes it possible to measure the two terms of the ratio Ki=k5/k4;k4 and k5 being respectively the velocity constants of formation and destruction of the enzyme-inhibitor complex. A program was prepared which made it possible to obtain these constants from only one experiment. With ampicillin and cloxacillin, we verified that the reaction is faster, and that only Ki can be measured. These facts suggest that special precautions should be taken in order to obtain signigicant values for the constants governing inhibition.