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Noonan syndrome (NS) is an autosomal dominant disorder with multisystem involvement. NS can be associated with bleeding disorders due to defects in platelet function or coagulation factors and diagnosis can be challenging. Factor XIII (FXIII) deficiency is uncommon in patients with NS. We present a case of NS who presented with bleeding in both thighs and was diagnosed to have deficiency in FXIII.

Prior studies in the USA and elsewhere have suggested a distinct seasonal pattern in the incidence of immune thrombocytopenic purpura (ITP) among children [Kuhne et al. 2001; Moussalem and Yassine, 2003; Watts, 2004; Zeller et al. 2005; Yong et al. 2010]. However, it remains unknown if such a seasonal trend exists among adults with ITP-related hospitalizations. We utilized the Nationwide Inpatient Sample (NIS) database to study the seasonal pattern of ITP-related hospitalizations among adults in the US. NIS is the largest inpatient database in the USA and is sponsored by the Agency for Healthcare Research and Quality. International Classification of Diseases ninth revision, Clinical Modification code 287.31 was used to identify all patients discharged with a principal diagnosis of ITP between 1 January 2005 and 31 December 2011. We stratified each hospitalization with a primary diagnosis of ITP by the month of presentation. Walter and Elwood modification of the Edwards test was used to determine the monthly variation in the frequency of hospitalizations. Statistical analysis was conducted using STATA-MP 13.0 (StataCorp LP, College Station, TX, USA). ITP accounted for a total of 69,981 hospitalizations between 2005 and 2011. No significant monthly variation in ITP-related hospitalizations was seen during the study period (Figure 1). There was a nonsignificant trend towards peak hospitalization during the month of September (peak/low ratio of 1.01, 95% confidence interval 1.0–1.03). Figure 1. Monthly frequency distribution of immune thrombocytopenic purpura (ITP)-related hospitalizations in the USA based on the Nationwide Inpatient Sample database 2005–2011. Our study shows a lack of a seasonal trend among ITP-related hospitalizations among adults in the USA. This is in contrast to childhood ITP, for which a distinct seasonal trend has been shown [Kuhne et al. 2001; Moussalem and Yassine, 2003; Zeller et al. 2005; Yong et al. 2010]. The seasonal pattern among children with ITP likely reflects the seasonality of viral infections such as influenza, which is responsible for the majority of cases of ITP in children [Yong et al. 2010]. Unlike in children, ITP in adults has an insidious onset and a chronic course with no preceding viral illness or other infection [Liu et al. 2013]. The large sample size and inclusion of different regions of the USA are the main strengths of our study. However, our study has several limitations. It was a retrospective study based on discharge-level patient data with limited clinical information. NIS is a deidentified administrative database and is prone to errors in coding of diagnoses that cannot be individually verified.

Thrombotic thrombocytopenic purpura (TTP) is uncommon in the postoperative setting, even more so after vascular surgery. We present a case of thrombocytopenia after aortofemoral bypass, which highlights the importance of a high index of suspicion for postoperative TTP to avoid life-threatening consequences.Keywords: postoperative thrombotic thrombocytopenic purpura; aortofemoral bypass; plasmapheresis(Published: 7 January 2013)Citation: Journal of Community Hospital Internal Medicine Perspectives 2012, 2: 19797 - http://dx.doi.org/10.3402/jchimp.v2i4.19797

We present the case of a 36-year-old woman who presented to our hospital with epigastric abdominal pain and tenderness. Laboratory evaluation identified high lipase, normal amylase, pseudohyponatremia, and relatively falsely low triglyceride levels (initial value of 2,329 mg/dl which on repeat was found to have corrected value of >10,000 mg/dl). The overall clinical picture was consistent with acute pancreatitis due to hypertriglyceridemia. The patient was commenced on IV insulin and eventually required plasmapheresis with good clinical outcome. This case highlights the importance of being cognizant of falsely low amylase and TG levels that can be present in patients with hypertriglycereidemic pancreatitisKeywords: hypertriglyceride; pancreatitis; epigastric pain(Published: 6 July 2016)Citation: Journal of Community Hospital Internal Medicine Perspectives 2016, 6: 31808 - http://dx.doi.org/10.3402/jchimp.v6.31808

Uterine fibroid, one of the most common tumors in women, is estrogen dependent, which commonly regresses after menopause. Fibroid degeneration after menopause, therefore, is rare. Here the authors report a case of 56-year-old postmenopausal woman who presented with acute abdominal pain, low grade fever, and leukocytosis as a result of fibroid degeneration. Keywords: post-menopause; uterine fibroid; degeneration; acute abdomen (Published: 3 February 2015) Citation: Journal of Community Hospital Internal Medicine Perspectives 2015, 5 : 25917 - http://dx.doi.org/10.3402/jchimp.v5.25917

Fulminant hepatic failure (FHF) is a relatively rare manifestation of Hodgkin’s lymphoma. Clinical features, laboratory findings, and imaging of the liver are usually inconclusive, and liver biopsy may be required for confirmation. We present a case of an FHF in a woman 1 week after the diagnosis of Hodgkin’s lymphoma. Chemotherapy could not be instituted due to hepatic encephalopathy and other complications. Autopsy revealed diffuse infiltration of the liver parenchyma. This case illustrates the importance of early diagnosis and institution of chemotherapy, which may reverse the liver failure.Keywords: failure; acute liver; fulminating hepatic failure; Hodgkin’s disease; Hodgkin’s lymphoma(Published: 25 November 2014)Citation: Journal of Community Hospital Internal Medicine Perspectives 2014, 4: 25821 - http://dx.doi.org/10.3402/jchimp.v4.25821

Background : Acute mountain sickness (AMS) can occur in anyone going to a high altitude. Non-steroidal anti-inflammatory drugs (NSAIDs) have been studied for the prevention of AMS with mixed results. In this systematic review, we analyze all existing data on the use of NSAIDs to prevent AMS using the Lake Louise Scoring System (LLSS) in different randomized clinical trials (RCTs). Methods : Electronic literature searches for relevant studies were identified through MEDLINE, EMBASE, SCOPUS, and Cochrane library up to June 2013. RCTs involving NSAIDs compared to placebo in patients undergoing ascent to a height of at least 3,800 m were included. Odds ratios (OR) were calculated and combined using fixed-effect model meta-analysis if I 2 =0%. Differences between groups were calculated using the inverse variance of the standard mean differences. Between-study heterogeneity was assessed using the I 2 statistics. Results : In three clinical trials involving 349 patients, AMS using LLSS occurred in 26.92% of patients on NSAIDs and 43.71% on placebo (OR 0.43; CI [confidence interval] 0.27–0.69, I 2 =0%, p= 0.0005), NNT=6. Minor outcome of end point Spo2 was not significant in the two groups (IV=0.74; 95% CI −0.20–1.69, I 2 =81%, p= 0.12). Similarly, a change in Spo2 from baseline was also not significant in the two groups (IV=0.05; 95% CI −0.28–0.37, I 2 =44%, p= 0.78). Conclusion : NSAIDs might be a safe and effective alternative for the prevention of AMS. However, further larger population studies and studies comparing NSAIDs to acetazolamide and dexamethasone in the future may provide further data to its relative efficacy. Keywords: altitude sickness; acute mountain sickness; anti-inflammatory agents; non-steroidal; ibuprofen; headache To access the supplementary material for this article, please see Supplementary files in the column to the right (under Article Tools) (Published: 29 September 2014) Citation: Journal of Community Hospital Internal Medicine Perspectives 2014, 4 : 24927 - http://dx.doi.org/10.3402/jchimp.v4.24927

Primary cardiac sarcomas are rare malignant tumors of the heart. Clinical features depend on the site of tumor and vary from symptoms of congestive heart failure to thromboembolism and arrhythmias. Echocardiography is helpful but definitive diagnosis is established by histopathology. Surgical resection is the mainstay of treatment, and the role of chemotherapy and radiotherapy is unclear. We report a case of primary cardiac sarcoma which presented with signs and symptoms of acute left-sided heart failure. Keywords : heart neoplasms; primary cardiac sarcoma; heart failure; left-sided heart failure (Published: 31 July 2014) Citation : Journal of Community Hospital Internal Medicine Perspectives 2014, 4 : 23057 - http://dx.doi.org/10.3402/jchimp.v4.23057

Acquired methaemoglobinaemia is a potentially fatal impairment in oxygen delivery if not recognised early and treated appropriately. Benzocaine used as an anaesthetic for endoscopic procedures is an uncommon but clinically important precipitant of acquired methaemoglobinaemia. We present a case of an elderly woman who developed perioral cyanosis and desaturation 20 min after a transoesophageal echocardiogram. Further evaluation led to the diagnosis of benzocaine-induced methaemoglobinaemia and timely treatment with intravenous methylene blue was initiated.

A 42-year-old homosexual man with HIV AIDS presented with headache, neck pain and a new bilateral palmoplantar rash for 2 weeks. On examination, he had a temperature of 39.6°C and neck stiffness was noted. Maculopapular rash with some scaling was present on bilateral distal extremities, especially in the palmar (figure 1) and plantar surfaces (figure 2). Genital lesions were absent. A complete blood count was unremarkable. CD4 cell count was 287/μL. An MRI of the brain was …

Left ventricular non-compaction (LVNC) is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non-compacted endocardial layer with marked trabeculations. The diagnosis is primarily based on echocardiographic demonstration of a spongy myocardium. Here, we present a young male with LVNC presenting with left heart failure and multiple left ventricular thrombi. We also review the presentation, diagnosis and management of this condition.

Haemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disorder resulting from a highly stimulated immune response with uncontrolled accumulation of lymphocytes and macrophages in multiple organs. Both the inherited and acquired forms of this disease exist; the latter can sometimes occur secondary to different malignancies. In this report, we present a middle-aged Hispanic man who presented with features of septic shock during the course of chemotherapy for squamous cell carcinoma of the neck. Despite aggressive treatment for septic shock, he rapidly deteriorated and died after 30 h of admission. Autopsy findings confirmed a diagnosis of HLH. HLH should be recognised as a serious adverse event during chemotherapy for different malignancies including squamous cell carcinoma of the neck.

Although rarely reported in the literature, adenomatous polyp and adenocarcinoma can occur as a late complication in an interposed colonic segment. We describe a case of a late stage adenocarcinoma in a colonic interposition performed for benign oesophageal stricture.

Papillary fibroelastoma of the aortic valve is an uncommon benign tumor of the heart that can present with embolic events. We report a case of 54-year-old lady with exertional chest pain and prior history of ST segment elevation myocardial infarction who was subsequently found to have a fibroelastoma of the aortic valve. The absence of angiographically significant coronary artery disease and resolution of anginal symptoms post-surgery in our patient points to the possibility of fibroelastoma causing these anginal symptoms. Although uncommon, fibroelastoma are being recognized more frequently with the help of transesophageal echocardiography. Hence, in the absence of significant coronary artery disease, we emphasize the importance of consideration of papillary fibroelastoma of the aortic valve as a cause of angina. We also discuss the key aspects of the fibroelastoma including presentation, diagnostic modalities and treatment options.

Hypertriglyceridaemia is the third most common cause of acute pancreatitis in the USA. The treatment approach for hypertriglyceridaemia to date has largely been conservative including weight loss, exercise and avoidance of medications that raise triglyceride levels. This approach, however, is not practical in cases of acute pancreatitis due to severely elevated triglycerides. A small number of case reports have been published supporting the treatment of acute pancreatitis due to severely elevated triglyceride levels with insulin and heparin. We report a case of acute pancreatitis in a young woman due to a triglyceride level of 15 215 mg/dl who was successfully treated with insulin and heparin.