North American Journal of Medicine and Science
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Intracranial Solitary Fibrous Tumor with Pseudopapillary Architecture: An Uncommon Tumor with Unusual Histopathology
Solitary fibrous tumor (SFT) is an uncommon soft tissue neoplasm first described in the pleura in 1931 and later recognized in other anatomical locations. Involvement of the central nervous system is rare; there are approximately 250 cases of central nervous system SFTs in the English literature. To the best of our knowledge, this is the first report of an intracranial SFT showing pseudopapillary architecture. The patient was a 73-year-old male who presented with recent onset altered mental status. Imaging studies showed a large left posterior parieto-occipital region intradural extra-axial mass with significant mass effect in the adjacent brain. Neuropathologic examination demonstrated a spindled mesenchymal neoplasm with variable cellularity and prominent collagen deposition. In areas, the tumor was discohesive, imparting a prominent pseudopapillary architecture. Entrapped brain parenchyma was present, indicating brain infiltration. The tumor cells were positive for CD34, CD99, vimentin, BCL-2, and STAT6; EMA was negative. Both the Ki-67 and mitotic indices were low, and anaplastic nuclear features were absent. To the best of our knowledge, this is the first example of an intracranial SFT with pseudopapillary architecture. Occasional extracranial SFTs showing papillary features have been reported. The significance of this architectural pattern is unclear and may be elucidated by future studies. [N A J Med Sci. 2016;9(4):181-186. DOI: 10.7156/najms.2016.0904181] Key Words: solitary fibrous tumor, central nervous system, intracranial, pseudopapillar
Out-of-School Sports Time and Children’s Body Weight Status: Evidence from a Longitudinal Survey
We used data from the Child Development Supplement (CDS) of the Panel Study of Income Dynamics in 2002 and 2007 to examine the relationship between the specific sport time spent during weekdays or weekends and American children’s body mass index (BMI). Time spent on out-of-school sports was recorded on a randomly selected weekday and a weekend day. Sports were further categorized as formal (organized sports such as sports games or lessons) or casual (any unorganized sports such as sports time in the neighborhood). Child’s height and weight were measured in person by interviewers. Body mass index was used to measure the child’s body weight status. We applied ordinary least square and fixed effects regressions to examine the cross-sectional and longitudinal relationships between out-of-school sports time and children’s body weight status. Children’s socio-demographics and parental socioeconomic status were controlled in the analyses. Double time spent on out-of-school sports during weekdays from 2002 to 2007 was associated with a reduction of BMI by 0.14 units, but the effects of time spent on out-of-school sports during weekends did not achieve statistical significance. For boys and girls, time spent on weekday casual (formal) sports was associated with a reduction of BMI by 0.18 and 0.17 units, respectively. Time spent on out-of-school sports during weekdays was more significant than during weekends in reducing BMI among US children
Sarcomatoid High Grade Urothelial Carcinoma with Rhabdomyosarcomatous and Other Differentiation: A Case Report with Review of Literature
It is not uncommon for high-grade urothelial carcinoma to show divergent differentiation with the most common being squamous followed by glandular. However, rhabdomyosarcomatous differentiation in urothelial carcinoma, which portends poor prognosis, is very rare. Here we described a case of bladder high grade urothelial carcinoma with rhabdomyosarcomatous as well as other histologic differentiation. The patient was a 91-year-old female with history of gross hematuria. She was found to have a large tumor protruding into the bladder lumen from the right lateral-posterior wall. The tumor was removed via transurethral resection. The tumor fragments measured 9.1 x 6.5 x 1 cm in aggregate and weighed 23.7 grams. Morphologically, the tumor demonstrated high grade urothelial carcinoma with an area showing abundant eosinophilic cytoplasm, large eccentric hyperchromatic nuclei, and prominent eosinophilic nucleoli, consistent with rhabdomyosarcomatous differentiation. Immunohistochemically, this area was positive for desmin and muscle-specific actin, supporting the above interpretation. There were also areas showing neuroendocrine, sarcomatoid, glandular and squamous differentiation. Scattered giant cells were also noted. Immunohistochemical stains for vimentin and synaptophysin/chromogranin were positive in the areas with sarcomatoid differentiation and neuroendocrine differentiation, respectively. Based on the overall morphological features and immunohistochemical patterns, this tumor was diagnosed as high grade urothelial carcinoma with rhabdomyosarcomatous, neuroendocrine, sarcomatoid, glandular and squamous differentiation. Similar cases in the literature were reviewed and compared with this case.
Lip Pits: An Uncommon Clinical and Histologic Finding Associated with Underlying Genodermatoses and Developmental Anomalies
We describe the clinical and histologic features of lip pits in a patient with Van der Woude syndrome (VdWS; OMIM 119300), an autosomal dominantly inherited disorder characterized by varying combinations of paramedian lip pits, cleft lip with or without cleft palate, cleft palate alone and/or hypodontia. VdWS is the most common single-gene cause of cleft lip and cleft palate. Mutations in the interferon regulatory 6 gene (IRF6) are detectable in over 70% of cases. Lip pits can be seen in other disorders such as the popliteal pterygium syndrome. Pits are usually divided into three categories 1) upper lip 2) lower lip and 3) commissural. Biopsy findings can be subtle and may be overlooked without the provision of relevant historic information. No treatment is needed for mild cases, however, surgical excision is helpful in more severe cases, both to alleviate discomfort and for cosmetic reasons
Benign and Malignant Prostatic Urethral Polyps: Two Case Reports and Review of Literature
Polypoid mass in the prostatic urethra is uncommon, yet the disease entities are so heterogeneous that the spectrum ranges from congenital malformations, benign polyps, to various malignancies. Prostatic urethral polyp (PUP) is a rare benign overgrowth of prostatic parenchyma protruding into prostatic urethra, and correspondingly, prostatic ductal carcinoma is a rare carcinoma originated from the primary duct of the prostate and occasionally presents as a polyp in the prostatic urethra. Due to the location of the polyps, varieties of symptoms, including dysuria, hematuria, urinary tract infections and obstruction leading to urinary retention, can be encountered and diagnostic confusion might be appreciated. Here we report two cases of polypoid mass located in the prostatic urethra: one case of benign PUP and another one of malignant prostatic ductal carcinoma protruding into prostatic urethra. The origins of both lesions were proven to be prostatic epithelium by using immunohistochemical identification of prostate-specific antigen (PSA). Further histological and immunohistochemical studies established the diagnoses of PUP and prostatic ductal carcinoma respectively. Their mimickers, including prostatic acinar adenocarcinoma, villous adenoma of urinary bladder, and papillary urothelial cell carcinoma, were also reviewed.
Clinical Practice Guidelines: the More, the Better?
Clinical practice guidelines are supposed to be evidence based and unbiased. High quality guidelines have the potential to promote the use of effective clinical services, minimize undesirable practice variation, and reduce the use of unnecessary services. Unfortunately, most of the guidelines produced thus far are flawed and untrustworthy. High quality guidelines may still have the intrinsic limitation of being too disease-focused rather than patient-focused, and lack applicability and validity when dealing with patients with multiple comorbidities or diseases. When applicable, clinical practice guidelines may serve as a relative guidance, rather than the absolute standard. Physicians need to be critical and vigilant when faced with a plethora of guidelines as following flawed practice guidelines may result in harm to patients. The use of clinical practice guidelines as the “standard of care” as well as for pay-for-performance based on guideline adherence is unjustified.
Mean Corpuscular Hemoglobin Concentration in Hemoglobin CC, SC, and AC
Point mutation in the hemoglobin beta chain generates many deviants from the normal format hemoglobin A, which contains 2 alpha chains and 2 beta chains. Among the numerous deviants, Hemoglobin C (HbC) and Hemoglobin S (HbS, sickle cell hemoglobin) are 2 of the most common variants with clinical significance. Mean corpuscular hemoglobin concentration (MCHC) is of a measurement of the concentration of hemoglobin in a given volume of packed red blood cells. It is one of the red cell indices measured daily in every blood specimen, which provides clues to the clinicians about the quality of the red cells and the associated diseases. Previous studies have suggested that patients with HbCC and HbSC, and up to 45% patients with HbAC present with high MCHC. However, in our practice we found that the MCHC values in these patients showed a broad spectrum. To clarify this discrepancy, 388 peripheral blood specimens with HbCC (homozygous C), HbSC (compound heterozygous S and C), or HbAC (heterozygous C) were pulled out from hospital’s medical records. We found about 50% of the HbCC patients and 20% of HbSC patients had elevated MCHC, and in HbAC patients, elevation of MCHC was rare. Therefore, our study sheds new light on the interpretation of MCHC in daily practice
Sudden Onset Complex Tic Associated with Streptococcal Infection in a Neonate: The First Case of Neonatal PANDAS
Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal Infections (PANDAS) is defined by acute onset of neuropsychiatric symptoms, most notably simple or complex motor or vocal tics, with an abrupt onset before adulthood associated with a Group A Streptococcal infection. This disorder is most commonly diagnosed in childhood presumably because of the high incidence of Group A Streptococcal infections during this time in development. This is the first report of a neonate with PANDAS. The neonate presented with clinical and laboratory evidence of a non-invasive Group A Streptococcal infection and concomitantly developed a complex tic. The complex tic waned as the Group A Streptococcal antibody titers decreased suggesting a temporal relation between the two. Infection and other triggers should be considered when abrupt changes in behavior or development occur early in life. Such an increased index-of-suspicious of PANDAS or Pediatric Acute-Onset Neuropsychiatric Syndromes (PANS) early in life may lead to improved neurodevelopmental outcomes
Autism Spectrum Disorder Related TCM Symptoms and TCM Herbs Prescriptions: A Systematic Review and Meta-Analysis
The purpose of this research is aimed at finding effective classical herbs prescriptions and providing references for further research on the autism treatment by consulting modern literature, ancient books and monographs. We took full advantage of CiteSeer, CNKI, PubMed, VIP and other literature databases as the major methods in this study to review the modern literature of autism during 1989-2014 and summarized them. We have also sorted out many prescriptions which used to treat autism in modern literature. In the traditional Chinese medicine (TCM) terms, the highest frequency of symptoms used to diagnose autism are "dullness", "mutistic", "soliloquy", "five kinds of retardation", "five weaknesses", "fetal toxicity" and "infantile metopism". By consulting ancient books and monographs, we found TCM associated with treatment of the aforementioned autism descriptors used by TCM. The results of this research is as following: Collecting over 4,706 research articles about autism spanning approximately 26 years, we reviewed the research of autism with respect to TCM and western medicine regarding its etiology, symptoms and treatment. We found the highest frequency TCM terms used to describe diagnostic symptoms of autism are "dullness", "mutistic", "soliloquy", "five kinds of retardation", "five weaknesses", "fetal toxicity" and "infantile metopism". Those descriptors were used as keywords to search related prescriptions from ancient books and monographs. More than 300 prescriptions were obtained. Finally, we verified clinical applications of these prescriptions, noting the frequency of use as a single medicine. We also collated pharmacological effects of the prescriptions, and the frequency of usage in the treatment of autism symptoms. The conclusion has been reached that TCM emphasizes a holistic treatment strategy with comprehensive aftercare. In addition, ancient physicians recorded many prescriptions about treatment of autism related symptoms in the ancient books and monographs, and some prescriptions are still applied in clinical autism treatment in current practices. In conclusion, seeking effective prescriptions and medicine from the perspective of TCM is of great importance.
Parathyroid Adenoma Arising from Autotransplanted Parathyroid Tissue in Sternocleidomastoid Muscle: A Case Report with Review of the Literature
Total parathyroidectomy followed by autotransplantation in patients with renal hyperparathyroidism to prevent hypoparathyroidism is a relatively common surgical procedure. However, parathyroid adenoma arising in autotransplanted parathyroid tissue in patients with secondary hyperparathyroidism is very rare. To date, less than 20 such cases have been reported in the English literature. Here we report a case of a 47-year-old African-American male with a history of end stage renal disease on dialysis undergoing a total parathyroidectomy and autotransplantation in 2001, presenting with severe hyperparathyroidism and enlarged transplanted parathyroid tissue 6 years later. The palpable mass at the site of autotransplantation was excised. Grossly, the cut surface of the mass appeared brown-tan. Microscopically, the mass was well circumscribed and composed of sheets of small, round and relatively uniform cells, morphologically consistent with chief cells. No adipose tissue or oxyphil cells were found inside the mass. Cellular atypia was not identified. Based on the above morphological features and the patient’s history, the diagnosis of parathyroid adenoma arising from autotransplanted tissue was established. This case illustrates that parathyroid adenoma arising from autograph can cause hyperparathyroidism