Multiresistant Acinetobacter baumannii causing malignant otitis externa: a case report.

Abstract: Malignant otitis externa is an infection of the skin and soft tissue of the ear canal, spreading to the nearby structures. It causes severe otalgia and otorrhea, and can lead to ominous consequences such as cranial nerve damage and meningitis. The main etiologic agent is Pseudomonas aeruginosa and treatment relies on broad-spectrum intravenous antibiotics. We report a rare case of a woman suffering from Malignant otitis externa caused by Acinetobacter baumannii and requiring the use of colistin.

Potential use of acupuncture in the treatment of first bite syndrome.

OBJECTIVES: First bite syndrome (FBS) is an early postoperative pain syndrome characterized by the sudden onset of pain in the parotid region at the first bite of each meal. The etiology is not certain, hence a standardized therapy does not exist. METHODS: A 50-year old woman referred to us complaining of a swelling in the right parotid region. Fine-needle aspiration biopsy (FNAB) was diagnostic for pleomorphus adenoma of the deep lobe of the parotid gland. A 50-year old man presented with a mass in the right side of the neck, FNAB was diagnostic for parapharyngeal space neurinoma. The first patient was submitted to total parotidectomy with facial nerve preservation, the second to extracapsular dissection of the tumor. A week after surgery both patients developed FBS. A qualitative/quantitative description of pain was obtained by means of a self-coded questionnaire. The score ranges from 8 to 44, corresponding to the lowest and the highest discomfort possible, respectively. Acupuncture was used to treat these 2 patients. The treatment protocol comprised 6 sessions, one per week, lasting 30 minutes each. RESULTS: Our questionnaire was administered before and after treatment and the score dropped from 33 to 25 in the female, from 30 to 15 in the male patient. CONCLUSION: FBS is a complication of upper cervical surgery with a high morbidity rate. We describe the first two cases of FBS that were successfully treated with acupuncture in our ENT department. We believe that this procedure may represent a valid therapeutic alternative in the future.

Cefixime for the prophylaxis of urinary tract infections in children with malformative uropathies: an open study.

Urinary tract infections are often associated with urinary anomalies. An appropriate pharmacologic treatment may prevent, or may at least limit, any kidney damage due to pyelonephritis. The antibiotic prophylaxis plays a role as significant as early surgical therapy, taking into consideration also the present limitative trend for a softer therapeutic regimen. In the past few years a greater bacterial resistance has emerged against some commonly administered antibiotics. Cefixime (3rd generation cephalosporin) has been used on a wide series of patients suffering from urinary infections associated with urinary tract anomalies. A few significative results emerge from the present study. In conclusion, cefixime's effectiveness long-term prophylaxis of urinary infections associated with anomalies.

Sentinel lymph node identification in breast cancer patients.

PURPOSE: To evaluate the predictive value of sentinel lymph node biopsy versus axillary node dissection on lymph node status in patients with T1-T2 breast cancer. MATERIAL AND METHODS: Twenty-nine patients with T1 and 12 with T2 breast carcinoma and clinically N0 axillary lymph nodes, underwent lymphoscintigraphy following the administration of 99mTc-human albumin nanocolloids. The tracer was injected subdermally, over the tumor mass, in the 34 patients with palpable lesions and peritumorally (n=3) or intratumorally (n=4), under stereotactic or ultrasound guidance, in the 7 patients with non-palpable lesions. Anterior and lateral planar images were acquired 15 min after the injection of the tracer and repeated every 30 min up to 3 hr until identification of sentinel lymph node. At the end of the scintigraphic study, sentinel node skin projection was marked using a dermographic pen. Eighteen hours after lymphoscintigraphy, sentinel lymph node was identified and removed during surgery by hand-held gamma probe, then, the remaining axillary lymph nodes were dissected. All surgical specimens underwent histologic examination. Sentinel lymph nodes free of metastasis at histology, underwent additional examination with immunohistochemistry using monoclonal antibodies against cytokeratin and EMA to search for micrometastases. RESULTS: Sentinel lymph node was identified in the 34 patients injected subdermally and in the 3 patients injected peritumorally, while it remained undetected in the 4 patients injected intratumorally except for one case in which it was isolated by radioguided surgery but not scintigraphically. Sentinel nodes resulted free of metastases both at histology and immunohistochemistry in 32 cases and metastatic in 6. In the 32 patients with non-metastatic sentinel lymph nodes the other axillary nodes were also free of metastases. Among the 6 metastatic sentinel lymph nodes, in 3 cases they were the only metastatic nodes of the axilla while in the other 3 cases metastases were spread to other axillary nodes. CONCLUSIONS: In agreement with previous studies, our results showed that sentinel lymph node radioguided biopsy is a simple and reliable method for predicting axillary lymph nodes status and for avoiding axillary dissection in early breast cancer patients with sentinel node free of metastases.

HNPCC-Lynch syndrome and idiopathic inflammatory bowel disease. A hypothesis on sharing of genes.

Colon cancer occurring in patients with Lynch Syndrome and in Inflammatory Bowel Disease (IBD) share many features. There is some evidence to support the assumption that multiple genetic factors play an important role in the pathogenesis of idiopathic IBD and Lynch Syndrome. In our previous study, providing detailed medical, genetic and pathologic findings on 202 hereditary non polyposis colorectal cancer (HNPCC) relatives we found in the colonic mucosa features indicating an IBD though all the screened subjects of the family denied symptoms of IBD. Some studies have reported that the rate of undetected IBD ranges from 27 to 38%. Finally, a member of this family, considered not at risk for cancer by genetic analysis results, developed a clinically manifested IBD. The morphological aspects of the disease were not discussed in our previous study. It is possible that many members of this family inherit a major gene giving liability to the disease and are carriers of a subclinical form of IBD with a minimal morphological marker which becomes manifest in some members when other factors intervene. A possible genetic model linking the two diseases can be suggested: IBD needs two major genes for susceptibility (s) and clinical development (D). Both can be present in IBD and Lynch Syndrome, but in the latter a third gene plays a suppressor role on the development gene (D). In conclusion, we hypothesize that the IBD developing gene may be considered as protective against HNPCC, and this condition may result in a selective genetic advantage.

[A seroepidemiological study on the level of immunological coverage in a nomadic population in Rome]. / Studio sieroepidemiologico sul livello di copertura immunitaria in una comunita nomade a Roma.

Immunization status to the three types of poliovirus, to tetanus, diphtheria and measles was evaluated in a Gypsy population living at a Roman camp. Information about demographic data and history of immunization was collected from 149 subjects and a blood sample was obtained from 86 individuals to determine antibody titres to the above mentioned infectious agents. Among the responders, only 20.8% had received at least one dose of oral polio vaccine (OPV), tetanus and diphtheria vaccine, while none was vaccinated against measles. In spite of a low immunization coverage, serological data showed high prevalence of antibodies to the three types of poliovirus (81.4% to polio type 1:94.2% to polio type 2:62.8% to polio type 3) and to measles (76.7%), while antibodies to tetanus and to diphtheria were detected respectively only in 3.5% and 0% of the individuals tested. High levels of antibodies to polio were found also among unvaccinated subjects. For these, a statistically significant positive correlation between age and number of "contact doses" from vaccinated family members was observed (r = 0.70; CI 95%: 0.27-0.90). In conclusion, this study uncovers very low levels of immunization to poliovirus, tetanus, diphtheria and measles in the study Gypsy population, and shows the effects of the secondary spread of the OPV, which probably contributed to reduce the risk of contracting the disease in unvaccinated individuals.

Linkage analysis identifies gene carriers among members of families with hereditary nonpolyposis colorectal cancer.

BACKGROUND & AIMS: Uncertainty about genetic risk in hereditary nonpolyposis colorectal cancer (HNPCC) may lead to unnecessary screening. The aims of this study were to show how gene linkage findings can elucidate who is at risk and requires intensive screening and how cancer control can be enhanced by screening high-risk family members. This information can be useful given the public health magnitude of HNPCC. METHODS: An extended family with HNPCC was studied using formal linkage analysis with DNA extraction from blood samples, followed by genotyping with polymerase chain reaction technique for microsatellite markers. Sixty-one blood relatives of a family with HNPCC, 5 of whom had colorectal cancer, and 12 unrelated family members underwent DNA sampling for genetic analysis. RESULTS: Linkage analysis showed that all 5 affected individuals had a haplotype with the same alleles 10/7/9, which was also detected in 13 first-degree healthy gene carriers and absent in the remaining 43 non-gene carriers. In the asymptomatic subjects screened, one incidental colorectal cancer and four adenomas were detected in 3 of 6 gene carriers. An adenoma was found in 1 of 17 noncarriers; the remaining 16 noncarriers have undergone 67 unnecessary colonoscopies. CONCLUSIONS: Linkage analysis can differentiate gene carriers from non carriers. Colorectal cancer screening should be restricted to gene carriers.

Surveillance in hereditary nonpolyposis colorectal cancer: an international cooperative study of 165 families. The International Collaborative Group on HNPCC.

During its second meeting at Amsterdam in 1990, the International Collaborative Group on Hereditary Non-Polyposis Colorectal Cancer (ICG-HNPCC) decided to carry out a pilot study on colorectal cancer surveillance in HNPCC. The objectives of the study were to ascertain in each of the participating centers the number of HNPCC families, the recommended screening procedures, the age at diagnosis of colorectal cancer (CRC), and the occurrence of interval cancers. Nine centers in seven countries including Denmark, Finland, Italy, Japan, The Netherlands, Switzerland, and the United States participated. Data were derived from a total of 165 families. With respect to screening, half of the centers advise colonoscopy as the only procedure. The interval between the consecutive examinations varies from one to three years. In the majority of the centers, screening begins at 20 to 25 years. Lifelong screening is recommended by three centers, while the rest advise discontinuation at age 60 to 75 years. The family material included 840 patients with colorectal cancer. The mean age at diagnosis was 45 years, and about 15 percent were diagnosed at age 60 or later. A total of 682 high-risk relatives are being followed. After the follow-up of 1 to 10 years in these families, only six cases of interval cancers were encountered.

Cancer family syndrome: cytogenetic investigations, in vitro tetraploidy, and biomarker studies in a large family.

Fifty-five members of a family with the cancer family syndrome (CFS) were investigated for the following potential biomarkers for cancer proneness: (1) cytogenetics of peripheral blood lymphocytes and skin fibroblasts; (2) in vitro tetraploidy of dermal fibroblast monolayer cultures; (3) quantitative serum immunoglobulin determinations; (4) study of genetic linkage with respect to eight blood group markers including Kidd. Biological specimens were obtained from 14 patients affected with cancer, 21 subjects at risk, and 20 healthy subjects. None of the markers tested in this family, in order to identify a biomarker for the status of CFS gene carrier, was found to be useful. Our search for linkage to other biological markers (DNA RFLPs and NK cells) is in progress.

Adenocarcinoma of the small bowel in lynch syndrome II.

Adenocarcinoma of the small bowel is rare and accounts for about 1% of all gastrointestinal tract cancer. This disorder has been identified in association with Crohn's disease, celiac disease, Peutz-Jegher's syndrome, and familial adenomatous polyposis. We report adenocarcinoma of the small bowel in nine patients from eight Lynch syndrome II extended pedigrees. Each affected patient was in the direct genetic lineage or manifested multiple primary cancers (stomach, colon, endometrium, and ovary) consonant with the tumor spectrum of Lynch syndrome II. The average age of onset for small bowel cancer was 47 years (range 31 to 56 years), versus the general population peak occurrence after the sixth decade. We conclude that small bowel cancer may be an integral component of the tumor spectrum of Lynch syndrome II.

Depressed level of natural killer cells in cancer family syndrome.

Individuals from kindred with cancer family syndrome (CFS) have an increased genetic risk for the development of adenocarcinoma of the colon as well as of several other organs. Previous studies have suggested that this high occurrence of adenocarcinoma in this as in other hereditary neoplastic syndromes may be correlated to an underlying abnormality in immunological tumor surveillance. In attempt to define a marker that might identify individuals within CFS kindred at risk of developing cancer, we determined natural killer (NK) cell number and NK cell function in affected and healthy members of a CFS family. We studied 13 cancer-affected patients, 20 unaffected but "at-risk" subjects, 20 healthy subjects and 26 normal individuals matched to the patients with colon cancer on the basis of sex and age. We determined the number of NK cells and their function concurrently, using a monoclonal antibody and a 51Cr-release assay with K562 as target cells. We found that the number of NK cells was significantly (P = 0.00004) reduced in cancer patients as compared with healthy subjects and normal controls. Of the 20 at-risk individuals 9 had levels lower than the norm, while 11 showed normal-values. Consequently, the mean percentage of NK cells of this group does not differ either from that of normal subjects or from that of cancer patients. Mean NK cell function was lower in cancer patients than in healthy members of the CFS family but the differences were not statistically significant. Therefore, the mean NK cell function per single cell, expressed as a ratio between cytotoxicity (LU) and the number of NK1-positive cells, resulted paradoxically in an increase when compared with that of normal subjects. The possible mechanisms for this dichotomy were examined.

Hereditary nonpolyposis colorectal cancer--Lynch syndromes I and II.

HNPCC is an autosomal dominantly inherited disorder with proclivity to early onset colorectal cancer in the absence of multiple polyps of the colon. There is a predilection for proximal colonic location (70 per cent) and an excess of synchronous and metachronous colorectal cancers. HNPCC is subdivided into Lynch syndrome I, which is restricted to site-specific colon cancer susceptibility, and Lynch syndrome II, which shows all of the features of Lynch syndrome I, but in addition, patients are at inordinately increased risk for carcinoma of the endometrium, ovary, and other anatomic sites. The frequency of HNPCC is conservatively estimated to be 4 to 6 per cent of the total colorectal cancer burden. Because of the fact that the family history is underreported almost uniformly in medical practice, we believe that the true frequency of this disease may be much greater. Heterogeneity may be extant with respect to tumor association, in that in certain Lynch syndrome II kindreds, carcinoma of the pancreas, kidney, breast, and other anatomic sites may predominate. Knowledge of the natural history of HNPCC predicates surveillance and management strategies. Thus, because of the early onset of and proximal predilection for colorectal cancer, we recommend initiation of colonscopy at age 25 and annually thereafter. We also recommend guaiac testing of the stool at least twice a year. In the case of Lynch syndrome II, in addition to colonscopy, we recommend intensive surveillance for the endometrium, including aspiration biopsies. Other targeted organs, depending on the tumor spectrum in the family, should be given priority attention. Because of an excess of synchronous and metachronous colorectal cancer in HNPCC, subtotal colectomy with ileorectal anastomosis is the treatment of choice for initial colorectal cancer. In women presenting with initial colorectal cancer who have completed their families, consideration should be given to prophylactic hysterectomy and bilateral salpingo-oophorectomy at the time of surgery for colorectal cancer. Needed are biomarkers of acceptable sensitivity and specificity for the genotype, because HNPCC lacks premonitory physical signs. We believe that increased knowledge about colorectal cancer etiology and carcinogenesis can be attained through the study of families prone to the Lynch syndromes.

Differential diagnosis of hereditary nonpolyposis colorectal cancer (Lynch syndrome I and Lynch syndrome II).

Increasing recognition of the statistical burden posed by HNPCC (5 to 6 percent of all colorectal cancer) mandates that physicians have a better understanding of the genetics, natural history, and distinction between the hereditary site-specific variant (Lynch syndrome I) and the Cancer Family Syndrome (Lynch syndrome II). The authors report detailed cancer (all sites) family histories on two prototype families with Lynch syndrome I (Family R) and Lynch syndrome II (Family N), which have been under investigation for more than two decades. Emphasis is placed on shared clinicogenetic features; namely, early age of onset of colonic cancer (approximately age 44), multiple primary colonic cancer (24 percent of cases showed metachronous colonic cancer), predominance of proximal colonic cancer location (approximately 65 percent in the proximal colon), and vertical transmission consonant with an autosomal dominantly inherited factor. An increased predilection for extracolonic cancer, particularly endometrial carcinoma, occurs in Lynch syndrome II and is the primary basis for distinction from Lynch syndrome I. Surveillance and management programs must be wholly responsive to these natural history features.

New phenotypic aspects in a family with Lynch syndrome II.

Increasing attention has been given to hereditary nonpolyposis colorectal cancer (HNPCC). This report provides medical genetic/pathologic findings on an HNPCC kindred from southern Italy that shows criteria consistent with Lynch syndrome II. An international collaborative effort led to extension of this kindred with disclosure of a potentially new spectrum of phenotypic findings: an excess of gastric carcinoma; complete intestinal metaplasia and chronic atrophic gastritis restricted to the antrum; an apparent excess of colonic mucosal macrophagia, which by special stain appeared to be positive for mucin, with a constant content of both sialo and sulfomucin, a lack of iron, and an inconstant positivity for lysozyme obtained by immunoperoxidase technique; and findings of crypt atrophy of the colonic mucosa. During the relatively short period of investigation of this family, an intensive educational and surveillance program has been mounted in the interest of improving cancer control through direct application of knowledge of natural history and the risk factor evidence through pedigree assessment.

Lymphocyte subpopulations in Lynch syndrome II: an immunocytochemical study on gastrointestinal biopsies. Preliminary report.

Gastrointestinal biopsies from 18 members of a family with Lynch Syndrome II were evaluated and immunocytochemical studies were made to characterize the phenotypic expression of the tissue's immune populations. The intestinal findings suggest polyclonal B-cell activation related to the T-helper distribution. Our evaluation provides no specific information so far on the management of patients with Lynch Syndrome II.