Scalp and limb defects with cutis marmorata telangiectatica congenita: Adams-Oliver syndrome?

We report on a boy with congenital scalp and limb defects, consistent with a diagnosis of Adams-Oliver syndrome (aplasia cutis congenita with terminal transverse limb defects). An additional finding present in this child and in his mother was cutis marmorata telangiectatica congenita. Although this boy fits the diagnostic criteria for Adams-Oliver syndrome, his mother does not. We discuss whether this condition is highly variable, or heterogeneous.     

Bone marrow examination for the diagnosis of mycobacterial and fungal infections in the acquired immunodeficiency syndrome.

In a series of 342 bone marrow examinations from 314 patients with human immunodeficiency virus infection, 70 examinations (20%) detected opportunistic mycobacterial or fungal infections. One hundred eleven of the 314 patients had such infections, and, hence, 63% (70/111) were detected by bone marrow examination. Special stains for microorganisms detected 16 (32%) of 50 Mycobacterium avium complex infections, 10 (22%) of 45 Mycobacterium tuberculosis infections, eight (73%) of 11 Histoplasma capsulatum infections, and five (83%) of six Cryptococcus neoformans infections. Bone marrow cultures detected 36 (72%) of the 50 M avium complex infections, 13 (29%) of the 45 M tuberculosis infections, and 63% of the fungal infections. Marrow examination revealed infection in only one of the 70 specimens (1%) collected to evaluate thrombocytopenia alone or hematologic malignancy, but in 69 (25%) of 274 with fever, neutropenia, anemia, or miscellaneous other indications for marrow examination. Granulomas were detected in 102 (30%) of the biopsy specimens, including 71 (64%) of those in cases with mycobacterial or fungal infection. The granulomas showed caseous necrosis in nine cases, all in patients with tuberculosis, and the 27 cases with tuberculosis-associated granulomas tended to show large, tightly cohesive granulomas. The presence of granulomas correlated with opportunistic infection in 82 (80%) of 102 cases. Without granulomas, special stains were positive in only eight (3%) of 240 specimens. These results suggest that (1) bone marrow granulomas are a common and valuable histologic clue to opportunistic infection; (2) without them, special stains may not be a cost-efficient way to diagnose such infection; and (3) bone marrow examination can be a useful method of diagnosing opportunistic mycobacterial and fungal infections in patients with fever, anemia or neutropenia, and underlying human immunodeficiency virus infection.     

Fine-needle aspiration of a metaplastic breast carcinoma with extensive melanocytic differentiation: a case report

Metaplastic carcinomas of the breast are uncommon breast tumors with aberrant cellular differentiation, most commonly showing ductal, squamous, and mesenchymal components. A breast carcinoma composed of both epithelial and melanocytic differentiation is rare, with only four previously reported cases in the literature. We present the fifth reported case, where the diagnosis was suggested by fine-needle aspiration (FNA) and later confirmed after the surgical specimen was excised. Histologically, this neoplasm revealed multidirectional differentiation, consisting primarily of squamous and melanocytic cell types, with focal glandular and osseous metaplasia. Based on the morphologic, immunohistochemical, and ultrastructural findings, we conclude that such tumors fall within the spectrum of metaplastic carcinomas of the breast. We believe that this case will further contribute to the understanding of this enigmatic tumor.     

Balloon embolization to occlude a Blalock-Taussig shunt

Balloon embolization was used to successfully occlude a large residual Blalock-Taussig shunt. The use of an "upstream" nondetachable balloon catheter to reduce flow and turbulence during final positioning of the detachable balloon may have made the technique safer and more precise.     

Doppler evaluation of homograft valved conduits in children

To assess the flow characteristics of homograft valved conduits in the immediate postoperative period, 69 children with 71 homograft conduits underwent 2-dimensional and Doppler echocardiographic examination at 1 to 40 days (mean 8) after surgery. Of the 71 conduits studied, 19 were aortic and 52 were pulmonary homograft valved conduits. Two aortic homograft valved conduits were inserted in the aortic position, whereas all remaining homografts were placed in the pulmonary position. On the immediate postoperative echocardiogram, 25 (35%) of the conduit valves had no regurgitation and 44 (62%) had 1+ (mild) regurgitation. Two pulmonary valved conduits (3%) in the pulmonary position had 2+ (moderate) regurgitation and right ventricular dimensions greater than 95% for body surface area. The peak velocity across the homograft valve was normal (less than 1.3 m/s) in 58 valves (82%). In the remaining 13 valves, peak velocity ranged from 1.4 to 2.6 m/s. No homograft valve had a peak velocity greater than 2.6 m/s in the immediate postoperative period. To assess the fate of homograft valved conduits in the intermediate-term follow-up period, 38 children with 38 conduits had a repeat echocardiogram at 6 to 25 months (mean 15 +/- 6) after surgery. Of the 38 conduits examined, 10 (26%) had no regurgitation, 25 (66%) had 1+ regurgitation and 3 (8%) had 2+ regurgitation. Progression of the amount of regurgitation occurred in 11 (29%) patients. At the follow-up examination, peak velocity was less than or equal to 1.4 m/s across 34 conduit valves, between 1.4 and 2.6 m/s across 3 valves and greater than 2.6 m/s across 1 valve.(ABSTRACT TRUNCATED AT 250 WORDS)     

Covid-19 in liver transplant recipients: the French SOT COVID registry

BackgroundNotwithstanding the ongoing coronavirus disease-2019 (Covid-19) pandemic, information on its clinical presentation and prognosis in organ transplant recipients remains limited. The aim of this registry-based observational study was to report the characteristics and clinical outcomes of liver transplant (LT) recipients included in the French nationwide Registry of Solid Organ Transplant Recipients with Covid-19.MethodsCOVID-19 was diagnosed in patients who had a positive PCR assay for SARS-CoV-2 or in presence of typical lung lesions on imaging or specific SARS-CoV-2 antibodies. Clinical and laboratory characteristics, management of immunosuppression, treatment for Covid-19, and clinical outcomes (hospitalization, admission to intensive care unit, mechanical ventilation, or death) were recorded.ResultsOf the 104 patients, 67 were admitted to hospital and 37 were managed at home (including all 13 children). Hospitalized patients had a median age of 65.2 years (IQR: 58.1 ? 73.2 years) and two thirds were men. Most common comorbidities included overweight (67.3%), hypertension (61.2%), diabetes (50.7%), cardiovascular disease (20.9%) and respiratory disease (16.4%). SARS-CoV-2 infection was identified after a median of 92.8 months (IQR: 40.1 ? 194.7 months) from LT. During hospitalization, antimetabolites, mTOR inhibitor, and CNIs were withdrawn in 41.9%, 30.0% and 12.5% of patients, respectively. The composite endpoint of severe Covid-19 within 30 days after diagnosis was reached by 33.0% of the adult patients. The 30-day mortality rate was 20.0%, and 28.1% for hospitalized patients. Multivariate analysis identified that age was independently associated with mortality.ConclusionIn our large nationwide study, Covid-19 in LT recipients was associated with a high mortality rate.     

New insights into the target site and mode of action of the antifungal protein of Aspergillus giganteus

The antifungal protein (AFP) secreted by Aspergillus giganteus exerts growth inhibitory effects on various filamentous fungi. In order to obtain more information on the mode of action of AFP, we used transmission electron microscopy in this study to compare the cellular ultrastructure of the AFP-sensitive Aspergillus niger and of the AFP-resistant Penicillium chrysogenum upon AFP treatment. Furthermore, AFP was localized by immunogold staining in both fungi. Severe membrane alterations in A. niger were observed, whereas the membrane of P. chrysogenum was not affected after treatment with AFP. The protein localized predominantly to a cell wall attached outer layer which is probably composed of glycoproteins, as well as to the cell wall of A. niger. It was found to accumulate within defined areas of the cell wall, pointing towards a specific interaction of AFP with cell wall components. In contrast, very little protein was bound to the outer layer and cell wall of P. chrysogenum. For future applications of AFP as an antimycotic drug, the mode of action of the protein was further characterized. The protein was found to act in a dose-dependent manner: it was fungistatic when applied at concentrations below the minimal inhibitory concentration, but fungicidal at higher concentrations. Using an in vivo model system, we were able to finally show that AFP indeed prevented the infection of tomato roots (Lycopersicon esculentum) by the plant-pathogenic fungus Fusarium oxysporum f. sp. lycopersici.     

Fine needle aspiration (FNA) biopsy of palpable breast masses: comparison of conventional smears with the Cyto-Tek MonoPrep system.

BACKGROUND: One of the limitations preventing the widespread use of fine-needle aspiration (FNA) is that it requires skill to obtain an adequate sample and well prepared smears. In this study, a new monolayer technique, the Cyto-Tek MonoPrep (MP) system, which obviates the need for smear preparation, was evaluated against conventional smear (CS) preparation for palpable breast lesions. METHODS: A total of 44 paired CS/MP breast FNA specimens were studied. The authors blindly analyzed the CS and the MP slides separately, then by a side-by-side evaluation. The two methods were compared with respect to diagnostic concordance, cellularity, cell preservation, background debris, and time needed to prepare and diagnose each case. RESULTS: An exact diagnostic correlation was present in 34 of 44 (77%) cases. The 10 noncorrelating cases were caused by decreased cellularity in the MP cases; nonetheless, 7 of these were correctly assigned to the right general diagnostic category, whereas the remaining 3 cases had insufficient cells. In addition to overall lesser cellularity on MP, fibroadenoma cases had smaller epithelial sheets and absence of stroma compared with CS. Both methods had comparable cellular preservation and diagnostic evaluation time, but background debris and preparation time were greater for MP. CONCLUSION: CS are favored over MP for the preparation of breast FNA specimens in centers with specialized FNA services because of their higher diagnostic yield, ease of preparation, and availability for immediate cytologic evaluation. However, in settings where specimens are collected sporadically by unskilled clinicians, the MP system may prove to be useful as an alternative or an adjunct to CS. Cancer (Cancer Cytopathol) Copyright 1999 American Cancer Society.     

Breast-Conservation Treatment Outcomes: A Community Hospital's Experience

Abstract:  In the United States, the majority of early breast cancer patients choose breast-conserving treatment in the community setting, yet there is a paucity of literature describing outcomes. In this paper, we describe our experience with breast-conserving treatment in a small community hospital. Our hospital tumor registry was used to identify breast cancer cases diagnosed at our hospital between 1997 and 2003. We limited our study to those women with initial attempts at breast-conserving surgery (BCS) who had follow-up oncology treatment at on-campus affiliated oncological services. We looked at factors that influence survival for early stage 0–II disease such as tumor and patient characteristics, completeness of local surgical tumor excision, and adjuvant treatment. We also evaluated the percentage of cases in which the initial BCS did not achieve adequate surgical oncological results and the number and type of subsequent surgeries that were required to achieve this goal. There were 185 cases with a median patient age of 55 and a median follow-up time of 53 months. Most tumors were stage 0–I (68%) or stage II (23%). A single surgery was deemed sufficient to achieve the desired oncological outcome in 54% of cases; the remaining cases (46%) required additional surgeries. A final margin of 5 mm or greater was successfully achieved in 81% of cases. Ninety-two percent of the patients underwent radiotherapy, 65% received hormonal therapy, and 49% underwent chemotherapy. One hundred and sixty one patients had successful breast-conserving surgeries (87%) and 24 patients (13%) ultimately required mastectomy. There were four loco-regional recurrences and 19 deaths during the study period. Our disease-free survival rate for early-stage cancer (stage 0–II) was 91% at 5 years. Our study shows that high-quality patient outcomes for breast-conserving treatment can be achieved in the community setting.