Rejection, Recurrence, or Something Else?

Since the introduction of liver transplant as treatment for end-stage liver disease, thousands of lives have been saved. To prevent organ rejection, immunosuppression is given chronically and hence, patients are at increased risk for opportunistic infections related to immunosuppression, especially within the first year after the transplant. However, opportunistic infections can occur years after the transplantation. Disseminated nocardiosis is one of these infections, and although the common presentation includes involvement of skin, lungs, and central nervous system. We present an uncommon presentation of nocardiosis in which cholestasis and elevated liver enzymes predominate, mimicking organ rejection and liver disease recurrence. Infectious etiologies, including opportunistic microorganisms, must always be ruled out in patients presenting elevation in liver enzymes and cholestasis after liver transplant as early diagnosis can prevent complications such as re-transplantation and even death.

Granulomatous enterocolitis secondary to Yersinia in an 11-year-old boy from Puerto Rico, confirmed by PCR: a case report.

We report the case of an 11-year-old boy without any history of systemic illness and who developed fever and chills and experienced a 10 lbs. weight loss 3 weeks prior to admission. Two days before admission, he experienced abdominal pain that became localized to the right lower quadrant within 24 hours. A non-enhanced abdominal computed tomography scan revealed a 5 mm, amorphous, hyperdense, right lower quadrant calcification associated with a 9 mm fluid-filled structure. An exploratory laparotomy revealed thickening of the proximal vermiform appendix extending to the cecum, with mesenteric adenopathy. An en-bloc excision of the distal ileum, cecum, mesocolon, and vermiform appendix with an end-to-end ileo-ascending colon anastomosis was performed because of a suspected neoplasm. Pathologic examination of the specimen revealed granulomatous enterocolitis with associated acute suppurative appendicitis and a cecum abscess. Polymerase chain reaction analysis of a paraffin-embedded tissue block confirmed Yersinia enterocolitica DNA in the tissue, pointing to Yersinia as being the etiologic microbe. To our knowledge, this is the first case of yersiniosis to be reported in Puerto Rico.

Increase in the number of IBD surgical resections at the Puerto Rico Medical Center: A brief report.

OBJECTIVE: There is limited data describing Inflammatory Bowel Disease (IBD) among Hispanics. In Puerto Rico, three studies have been conducted that estimate the prevalence of this disease. Although still rare, these studies coincide that IBD has increased during the last decades. This study aims to describe the IBD surgical resections received in the Pathology Laboratory of the Puerto Rico Medical Center (PRMC) in two periods: 1966-82 and 2002-2008. METHODS: A retrospective review of the Surgical Pathology reports was done. A total of 133 reports complied with the inclusion criteria for IBD out of a total of 314,493 pathologies evaluated. For purposes of comparison, the data was grouped into 3 time periods: 1966-1973, 1974-1982 and 2002-08. RESULTS: The number of IBD cases per 100,000 pathologies for the period 1966-73 was 15.1, for the period 1974-82 was 19.1 and for the period 2002-08 was 125.0. The number of IBD resections increased 87.9% when comparing years 1966-73 with 2002-2008, with similar increases in Ulcerative Colitis and Crohn's Disease. Although this result was not statistically significant (p>0.05), a higher proportion of IBD patients had an IBD resection at a younger age in the 2002-2008 time period. A change in the sex profile of the patients was also identified; the majority of cases (75%) were females in the years 1966-1973 while 50.6% were males in 2002-2008. CONCLUSION: Our results confirm an increase in IBD surgical resections at the PRMC. Although not significant, a higher proportion of incident cases are occurring among younger age groups and the distribution of incident cases has become similar between males and females in recent years.

Surveillance for dysplasia in patients with ileal pouch-anal anastomosis for ulcerative colitis: an interim analysis.

BACKGROUND: The risk of developing cancer in the ileal pouch of patients with surgery for ulcerative colitis has not been defined. Dysplasia in the pouch is quite rare. Although some suggest pouch surveillance based on previous histological assessments, there are no guidelines for surveillance of these patients. The aim of our study was to investigate that risk and identify time intervals for ileoanal pouch surveillance. METHODS: Endoscopy and biopsies of the ileal pouch were performed at 3, 6, and/or 12 months after ileal pouch-anal anastomosis (IPAA) became functional. Biopsies were evaluated by two pathologists using Riddel's criteria. Interim data analysis using descriptive statistics is reported. RESULTS: Thirty-eight patients have entered the study. Average patient age at 3, 6, and 12 months of surveillance was 39.1, 36.8, and 39.1 years, respectively. Average disease duration was 8.2 years. Ten of 38 cases (26%) had colonic dysplasia prior to surgery. Dysplasia within the pouch was reported in one patient 6 months after IPAA became functional. This patient demonstrated no dysplasia at 12 months or statistical divergence by age, duration of disease or history of colonic dysplasia prior to IPAA. No subgroup of patients with dysplasia was identified to calculate cumulative risk or perform comparative statistical analysis. CONCLUSION: A study with longer follow-up after IPAA should precede any attempt to recommend routine surveillance. However, the finding of dysplasia early after surgery underscores the importance of early pouch surveillance in our population, at least until definite predisposing variables are identified.

Clinicopathological factors associated to HER-2 status in a hospital-based sample of breast cancer patients in Puerto Rico.

Breast cancer is the most common female malignancy in Puerto Rico. Cases with human epidermal growth factor receptor 2 (HER-2) oncoprotein overamplification are associated with aggressive clinical behavior. Given the limited availability of information for Puerto Rico, we aimed to evaluate the prevalence and clinical correlates of HER-2 gene overexpression among a hospital-based female population of breast cancer cases. We analyzed data from 1,049 female patients with invasive breast cancer (diagnosed 2000-2005) at the I. González Martínez Oncologic Hospital and the Auxilio Mutuo Hospital. HER-2 status and other clinical characteristics were retrieved from the hospitals' cancer registries, from the Puerto Rico Central Cancer Registry, and from a review of medical and pathological records. Prevalence odds ratios were estimated with 95% confidence intervals, using logistic regression models to quantify the association between HER-2 status and different clinicophatological factors. The overall prevalence of positive HER-2 expression was 22.5%. In the multivariate logistic regression model, factors significantly associated with HER-2 positivity included a diagnosis age of < 50 years, having a tumor with negative progesterone receptor (PR) status, and having regional disease (p < 0.05). No significant differences in HER-2 positivity were observed by tumor histology or estrogen receptor (ER) status (p > 0.05). This is the most comprehensive epidemiological study to date on HER-2 status in Puerto Rico. The prevalence and correlates of HER-2 overexpression in this study are comparable to those observed in US populations. Study results will aid in the development of breast cancer control strategies in Puerto Rico.

Serrated adenomas.

Serrated adenomas are categorized as sessile serrated adenomas (SSAs) and traditional serrated adenomas (TSAs). SSAs are more prevalent in the proximal colon and lack classic dysplasia, whereas TSAs are more prevalent in the rectosigmoid and have cytologic dysplasia. Serrated adenomas may progress to colorectal adenocarcinoma through diverse molecular alterations. Colonoscopy is the only test for the early detection of serrated adenomas that allows inspection of the entire colon and same-session biopsy sampling or polypectomy, if necessary. If an endoscopic biopsy at the right colon reveals SSA without cytologic dysplasia or biopsy at the rectosigmoid reveals SSA or TSA, those polyps should be excised or surgically resected as necessary. Postpolypectomy surveillance for removed SSAs without dysplasia and TSAs must be performed at 5- and 3-year intervals, respectively, with colonoscopy to prevent recurrence and progression to colorectal adenocarcinoma.

Ventricular non-compaction cardiomyopathy.

Non-compaction of the left ventricle is an extremely rare cardiomyopathy resulting from a defective morphogenesis of the endomyocardium. It results in an architecturally aberrant ventricular wall consisting of two layers: a compacted layer and a loose interwoven meshwork with prominent trabeculae and deep intertrabecular recesses that communicate with the left ventricular cavity. This report describes the case of a 25-year-old man with dilated cardiomyopathy, due to non-compaction of the left ventricle, presenting with a transient ischemic attack.

MSH-2 and MLH-1 protein expression in Muir Torre syndrome-related and sporadic sebaceous neoplasms.

BACKGROUND: Muir-Torre Syndrome (MTS) is a rare autosomal-dominant disorder characterized by the predisposition to both sebaceous neoplasm and internal malignancies. MTS-associated sebaceous neoplasms reveal mutations in DNA mismatch repair (MMR) genes and microsatellite instability. A significant part of MTS patients represents a phenotypic variant, the hereditary nonpolyposis colorectal cancer (HNPCC). A strong correlation between microsatellite instability and immunostaining has been demonstrated. The early recognition of sebaceous neoplasm as part of MTS, and their differentiation from sporadic sebaceous neoplasm may have an important application in a clinical setting. The absence of MLH-1 or MSH-2 expression by immunostaining identifies tumors with mismatch repair deficiency. OBJECTIVES: Our aim is to determine whether an immunohistochemical approach, targeting DNA repair proteins MSH-2 and MLH-1 in MTS-related sebaceous neoplasm and their sporadic counterparts, can be used for their identification. METHODS: We examined 15 sebaceous neoplasms (including 6 internal malignancy- associated sebaceous neoplasms and 8 sporadic sebaceous neoplasms) from 11 patients for the expression of MSH-2 and MLH-1 by immunohistochemistry. RESULTS: Four of 5 internal malignancy-associated sebaceous neoplasms showed loss of expression of MSH-2 or MLH-1. Correlation of the immunostaining pattern of the sebaceous neoplasms and the patients' positive history of colon carcinoma was 80%. Seven of 8 sporadic sebaceous neoplasms showed a positive expression of MSH-2 and MLH-1. The prevalence for loss of expression of MMR proteins in sebaceous neoplasms was 38.5%. MMR immunostaining had 87.5% specificity and 80% sensitivity. LIMITATIONS: This study is limited by a small sample size, and by bias selection due to the use of non nationwide data-base as the resource of cases. CONCLUSIONS: Our findings demonstrate that immunohistochemical testing for internal malignancy-associated sebaceous neoplasms is a practical approach to confirm a suspected inherited MMR gene defect, and an accurate method to distinguish between sporadic and MTS-associated sebaceous lesions.

Ileal perforation and reactive hemophagocytic syndrome in a patient with disseminated histoplasmosis: the role of the real-time polymerase chain reaction in the diagnosis and successful treatment with amphotericin B lipid complex.

The following case illustrates an ileal perforation and reactive hemophagocytic syndrome (RHS) resulting from disseminated histoplasmosis in a patient with Human Immunodeficiency Virus (HIV) from Puerto Rico. Although the diagnosis was established by histopathologic findings and a positive bone marrow culture, Histoplasma capsulatum-specific real-time Polymerase Chain Reaction (PCR) allowed to confirm the diagnosis from formalin-fixed, paraffin-embedded tissue. Interestingly, the Histoplasma antigens in both serum and urine samples were falsely negative. Amphotericin B lipid complex (Abelcet), followed by oral itraconazole, led to a successful response and resolution of symptoms. A short review of the clinical signs and symptoms, diagnostic tests, and therapeutic options for disseminated histoplasmosis is done, with emphasis on the role of Histoplasma-specific real-time PCR as a molecular diagnostic tool and the efficacy of treatment with one of the lipid formulations of amphotericin B.

Serum vitamin D and colonic vitamin D receptor in inflammatory bowel disease.

AIM: To determine serum vitamin D levels and colonic vitamin D receptor (VDR) expression in inflammatory bowel disease (IBD) and non-IBD patients and correlate these with histopathology. METHODS: Puerto Rican IBD (n = 10) and non-IBD (n = 10) patients ≥ 21 years old scheduled for colonoscopy were recruited. Each patient completed a questionnaire and provided a serum sample and a colonic biopsy of normal-appearing mucosa. For IBD patients, an additional biopsy was collected from visually diseased mucosa. Serum vitamin D levels were measured by ultra-performance liquid chromatography and mass spectrometry. Hematoxylin and eosin stained tissue sections from colonic biopsies were classified histologically as normal or colitis (active/inactive), and scored for the degree of inflammation present (0-3, inactive/absent to severe). Tissue sections from colonic biopsies were also stained by immunohistochemistry for VDR, for which representative diagnostic areas were photographed and scored for staining intensity using a 4-point scale. RESULTS: The IBD cohort was significantly younger (40.40 ± 5.27, P < 0.05) than the non-IBD cohort (56.70 ± 1.64) with a higher prevalence of vitamin D deficiency (40% vs 20%, respectively) and insufficiency (70% vs 50%, respectively). Histologic inflammation was significantly higher in visually diseased mucosa from IBD patients (1.95 ± 0.25) than in normal-appearing mucosa from control patients (0.25 ± 0.08, P < 0.01) and from IBD patients (0.65 ± 0.36, P < 0.05) and correlated inversely with VDR expression in visually diseased colonic tissue from IBD patients (r = -0.44, P < 0.05) and from IBD patients with Crohn's disease (r = -0.69, P < 0.05), but not in normal-appearing colonic tissue from control patients or IBD patients. Control and IBD patient serum vitamin D levels correlated positively with VDR expression in normal colon from control and IBD patients (r = 0.38, P < 0.05) and with patient age (r = 0.54, P < 0.01). CONCLUSION: Levels of serum vitamin D correlate positively with colonic VDR expression in visually normal mucosa whereas inflammation correlates negatively with colonic VDR expression in visually diseased mucosa in Puerto Rican patients.

Immunohistochemical expression of SP-NK-1R-EGFR pathway and VDR in colonic inflammation and neoplasia.

AIM: To determine the expression of neurokinin-1 receptor (NK-1R), phosphorylated epidermal growth factor receptor (pEGFR), cyclooxygenase-2 (Cox-2), and vitamin D receptor (VDR) in normal, inflammatory bowel disease (IBD), and colorectal neoplasia tissues from Puerto Ricans. METHODS: Tissues from patients with IBD, colitis-associated colorectal cancer (CAC), sporadic dysplasia, and sporadic colorectal cancer (CRC), as well as normal controls, were identified at several centers in Puerto Rico. Archival formalin-fixed, paraffin-embedded tissues were de-identified and processed by immunohistochemistry for NK-1R, pEGFR, Cox-2, and VDR. Pictures of representative areas of each tissues diagnosis were taken and scored by three observers using a 4-point scale that assessed intensity of staining. Tissues with CAC were further analyzed by photographing representative areas of IBD and the different grades of dysplasia, in addition to the areas of cancer, within each tissue. Differences in the average age between the five patient groups were assessed with one-way analysis of variance and Tukey-Kramer multiple comparisons test. The mean scores for normal tissues and tissues with IBD, dysplasia, CRC, and CAC were calculated and statistically compared using one-way analysis of variance and Dunnett's multiple comparisons test. Correlations between protein expression patterns were analyzed with the Pearson's product-moment correlation coefficient. Data are presented as mean ± SE. RESULTS: On average, patients with IBD were younger (34.60 ± 5.81) than normal (63.20 ± 6.13, P < 0.01), sporadic dysplasia (68.80 ± 4.42, P < 0.01), sporadic cancer (74.80 ± 4.91, P < 0.001), and CAC (57.50 ± 5.11, P < 0.05) patients. NK-1R in cancer tissue (sporadic CRC, 1.73 ± 0.34; CAC, 1.57 ± 0.53) and sporadic dysplasia (2.00 ± 0.45) were higher than in normal tissues (0.73 ± 0.19). pEGFR was significantly increased in sporadic CRC (1.53 ± 0.43) and CAC (2.25 ± 0.47) when compared to normal tissue (0.07 ± 0.25, P < 0.05, P < 0.001, respectively). Cox-2 was significantly increased in sporadic colorectal cancer (2.20 ± 0.23 vs 0.80 ± 0.37 for normal tissues, P < 0.05). In comparison to normal (2.80 ± 0.13) and CAC (2.50 ± 0.33) tissues, VDR was significantly decreased in sporadic dysplasia (0.00 ± 0.00, P < 0.001 vs normal, P < 0.001 vs CAC) and sporadic CRC (0.47 ± 0.23, P < 0.001 vs normal, P < 0.001 vs CAC). VDR levels negatively correlated with NK-1R (r = -0.48) and pEGFR (r = -0.56) in normal, IBD, sporadic dysplasia and sporadic CRC tissue, but not in CAC. CONCLUSION: Immunohistochemical NK-1R and pEGFR positivity with VDR negativity can be used to identify areas of sporadic colorectal neoplasia. VDR immunoreactivity can distinguish CAC from sporadic cancer.

Macromastia: how much of it is fat?

A total of 25 patients who underwent bilateral breast reduction were included in this study. Each patient's age, weight, height, and amount of breast tissue removed from each breast were recorded. The body mass index was calculated for each patient. On the day of the operation, tissue samples (two each) were taken from the central, lateral, and preaxillary areas of the breast. One of the samples was weighed, placed in a closed glass container, and heated for 10 minutes in a microwave oven at full power. The liquid fat was separated from the solid residue, and the percentage of fat was calculated. The other sample from each area was examined grossly, and representative sections, corresponding to the distribution of fat and connective tissue, were submitted for evaluation. In these samples, the percentage of fat, gland, and connective tissue was estimated using low-magnification light microscopy. In this group of patients (who had an average age of 34 years and who were significantly overweight as determined by a mean body mass index of 28), it was found (using the microwave method) that there was a mean fat percentage of 61 percent in the central breast area, 74 percent in the lateral breast area, and 73 percent in the preaxillary area. Upon microscopic examination, the pathologist reported that fat accounted for 64 percent of the central breast area, 92 percent of the lateral breast area, and 94 percent of the preaxillary area. On average, the central breast area in macromastia patients had only seven percent gland and 29 percent connective tissue. The lateral and preaxillary areas of the breast had one to three percent gland and five percent connective tissue. The two methods had a significant (p < 0.05) positive correlation in the central breast area, but in the lateral and preaxillary regions, the correlation was poor. In the microscopic examination, there was a tendency to overestimate the amount of fat. Both methods of evaluation used in the study concur that the enlarged breast of macromastia consists primarily of fat and that the glandular element is rather small.

Epstein-Barr virus in biopsies from patients with Hodgkin and non-Hodgkin lymphoma at the University of Puerto Rico immunohistochemistry laboratory.

OBJECTIVES: We aimed to determine the Epstein-Barr Virus (EBV) presence rate in our laboratory's lymphoma tissue biopsies for comparison with that reported in literature. BACKGROUND: The presence of EBV has been established in Hodgkin lymphoma (HL), endemic Burkitt Lymphoma and some non-Hodgkin lymphomas (NHL). It has been linked to geographic, ethnic and socioeconomic factors, with a lower rate in developed countries. METHODS: We used the immunoperoxidase technique to determine the rate of the EBV LMP-1 in eighty-seven biopsies diagnosed as lymphoma. Tissue slides were stained using the Ventana Automated Slide Stainer with the DAKO EBV LMP-1 primary antibody and the results were analyzed with the SYSTAT program. RESULTS: We found an LMP-1 positive rate of 50% for 22 cases of HL and 35% for 63 cases of NHL. Among HL, 5 were children and 16 were adults, with LMP-1 positive rates of 60% and 50% respectively. Among NHL, 3 were children and 59 were adults, with equal LMP-1 positive rates of 33%. The sex LMP-1 positive rates for HL were 42% for 12 males and 60% for 10 females. Among NHL, the sex LMP-1 positive rates were 39% for 38 males and 28% for 27 females. NHL was further subdivided into subtypes and LMP-1 primary antibody positive rates were reported. CONCLUSIONS: We found a similar presence rate of EBV in the HL biopsies to that of developed countries, but a similar presence rate of EBV in NHL biopsies to that of developing countries.

Crohn's disease associated to chordoma: a case report.

Crohn's disease is an inflammatory bowel disease characterized by remissions and exacerbations. Immunosuppressants are frequently used to induce and maintain remission in these patients. The use of the immunomodulator azathioprine has been associated to malignancies. Chordomas are rare, locally aggressive tumors arising from remnants of the notochord. A specific trigger for this tumor has not been identified and association to any medication has not been reported. The purpose of this report is to present the first case reported in the literature of Crohn's disease associated to a chordoma. The patient to be presented was on azathioprine therapy, among other medications. A review of literature revealed that Crohn's disease and chordoma have abnormalities in chromosomes 1 and 10. Inflammatory bowel disease and chordoma also have abnormalities in chromosomal regions 1p, 3p, and 7q. Despite these findings, a direct genetic relationship between these diseases is speculative.

Prevalence of steroid receptors and HER 2/neu in breast cancer biopsies of women living in Puerto Rico.

OBJECTIVE: The purpose of this study is to determine the prevalence rate of estrogen and progesterone receptors and HER 2/neu in the breast cancer biopsies analyzed in the Laboratory of Immunohistochemistry of the University of Puerto Rico School of Medicine in the year 2000. This data may serve as a reference point for future studies of the epidemiological aspects of breast cancer among women living in Puerto Rico. BACKGROUND: Determination of estrogen receptor (ER) and progesterone receptor (PR) on biopsy specimens of breast carcinoma prior to treatment is standard practice in the management of breast carcinoma. ER and PR are used to identify patients who are likely to respond to endocrine therapy. The prevalence of ER, PR and Her2/neu among USA women is 77%, 55% and 10-34%, respectively. One of the major clinical roles for testing HER 2/neu expression is to determine eligibility for treatment with Trastuzumab. METHODS: Retrospective analysis of 309 breast cancer biopsies was done. Paraffin embedded blocks of breast cancer tissue biopsies were received from different hospitals and Pathology Laboratories located throughout the island specifically for routine analysis of steroid receptor (ER/PR) and/or HER 2/neu expression. Immunostaining was performed in a Ventana Medical Systems automated instrument. RESULTS: Positive nuclear staining for ER and PR were seen in 65.9% (203/308) and 51.8% (159/307), respectively. In the HER2/neu test, 27.8% (46/165) gave a strong and complete membranous staining (score 3+). CONCLUSIONS: There is a lower prevalence of estrogen receptor in the breast cancer biopsies of women living in Puerto Rico than their USA counterparts, but similar prevalence of progesterone receptor status and HER 2/neu protein over expression.

Retinal hemorrhages and intermediate uveitis in a patient with drug reaction with eosinophilia and systemic symptoms syndrome: a case report.

PURPOSE: To report a case of drug reaction (or rash) with eosinophilia and systemic symptoms syndrome in association with intraretinal hemorrhages and intermediate uveitis. METHODS: Single case report. RESULTS: A 22-year-old Hispanic woman developed a facial rash and blurry vision after the use of oral trimethoprim-sulfamethoxazole for a urinary tract infection. Fundus examination revealed bilateral +2 vitritis and intraretinal hemorrhages in all four quadrants. An oral mucosal biopsy revealed V-shaped coagulative necrosis, intraepithelial and superficial acute and lymphoplasmacytic inflammation, consistent with drug hypersensitivity reaction. CONCLUSION: Drug reaction (or rash) with eosinophilia and systemic symptoms syndrome can present as cutaneous rash, mucosal lesions, eosinophilia, intermediate uveitis, and intraretinal hemorrhages. In such cases, vitreoretinal manifestations may be considered as diagnostic criteria instead visceral involvement.

Breast cancer molecular subtypes and survival in a hospital-based sample in Puerto Rico.

Information on the impact of hormone receptor status subtypes in breast cancer (BC) prognosis is still limited for Hispanics. We aimed to evaluate the association of BC molecular subtypes and other clinical factors with survival in a hospital-based female population of BC cases in Puerto Rico. We analyzed 663 cases of invasive BC diagnosed between 2002 and 2005. Information on HER-2/neu (HER-2) overexpression, estrogen (ER), and progesterone (PR) receptor status and clinical characteristics were retrieved from hospitals cancer registries and record review. Survival probabilities by covariates of interest were described using the Kaplan-Meier estimators. Cox proportional hazards models were employed to assess factors associated with risk of BC death. Overall, 17.3% of BC cases were triple-negative (TN), 61.8% were Luminal-A, 13.3% were Luminal-B, and 7.5% were HER-2 overexpressed. In the multivariate Cox model, among patients with localized stage, women with TN BC had higher risk of death (adjusted hazard ratio [HR]: 2.57, 95% confidence interval [CI]: 1.29-5.12) as compared to those with Luminal-A status, after adjusting for age at diagnosis. In addition, among women with regional/distant stage at diagnosis, those with TN BC (HR: 5.48, 95% CI: 2.63-11.47) and those HER-2+, including HER-2 overexpressed and Luminal-B, (HR: 2.73, 95% CI:1.30-5.75) had a higher mortality. This is the most comprehensive epidemiological study to date on the impact of hormone receptor expression subtypes in BC survival in Puerto Rico. Consistent to results in other populations, the TN subtype and HER-2+ tumors were associated with decreased survival.

Mismatch repair protein expression and colorectal cancer in Hispanics from Puerto Rico.

Colorectal cancer (CRC) is a leading cause of morbidity and mortality and alterations in mismatch repair (MMR) genes, leading to absent protein (negative) expression, are responsible for approximately 20% of CRC cases. Immunohistochemistry is a tool for prescreening of MMR protein expression in CRC but the literature on its use on Hispanics is scarce. However, Hispanics represent the second leading ethnicity in the United States (US) and CRC is a public health burden in this group. Our objectives were to determine the frequency of MMR protein-negative CRC and to evaluate its association with clinical and pathological characteristics among Hispanics from Puerto Rico, for the first time to our knowledge. A retrospective observational study of unselected CRC patients from the Puerto Rico Medical Center from 2001 to 2005 was done. MLH1 and MSH2, the most commonly altered MMR genes, protein expression was evaluated using immunohistochemistry, with microsatellite instability (MSI) and BRAF gene analyses in the absence of MLH1 protein expression. One-hundred sixty-four CRC patients were evaluated: the overall MMR protein-negative frequency was 4.3%, with 0.6% frequency of co-occurrence of MLH1-protein negative expression, MSI-high, and normal BRAF gene. MMR protein-negative expression was associated with proximal colon location (P = 0.02) and poor histological tumor differentiation (P = 0.001), but not with other characteristics. The frequency of MMR protein-negative CRC in Hispanics from Puerto Rico was lower than reported in other populations. This finding may explain the lower CRC incidence rate among US Hispanics as compared to US non-Hispanic whites and blacks.

Acute fatty liver of pregnancy. Case report and review of the literature

A case of acute fatty liver of pregnancy is presented. This case is characterized by many of the reported diagnostic features of AFLP, including the abrupt onset of symptoms with progressive liver failure, coagulopathy and encephalopathy, during the third trimester of pregnancy. Autopsy findings confirmed the diagnosis. The literature is reviewed, emphasizing the clinical and laboratory features helpful in the diagnosis of this entity