Literature Review and Profile of Cancer Diseases Among Afghan Refugees in Iran: Referrals in Six Years of Displacement.

BACKGROUND: There is a paucity of research on the profile of cancers among displaced populations, specifically Afghan refugees in Iran. This study illustrates the pattern of cancers in this population, and highlights the challenges of cancer care in displaced people with the intent that this data will facilitate appropriate allocation of resources to improve care in this population. MATERIAL AND METHODS: This was a retrospective cross-sectional study, in which we collected the demographics and profile of cancers among Afghan refugees from 2005 to 2010 from referrals to the United Nations High Commissioner for Refugees (UNHCR) offices in Iran. Accrued evidence by other studies published between January 1993 and July 2014 pertaining to cancer diagnoses in refugees from Afghanistan, Tibet, Syria, Jordan, and Iraq was reviewed. RESULTS: Cancer diagnoses accounted for 3083 of 23 152 total referrals, with 49% female and 51% male cases; 23.3% were 0-17 years of age, 61.2% were 18-59, and 15.5% were above 60. The most common health referral for females and males (0-17) was malignant neoplasms of lymphatic and hematopoietic tissue, accounting for 34.2%. In the age groups 18-59 and above 60 for both male and females it was malignant neoplasm of the digestive system, occurring in 26.3% and 48.7%, respectively. CONCLUSIONS: In the setting of humanitarian crises especially war, cancer diagnoses among refugees is a major health burden both on the host countries and the international community with serious implications considering the recent growing trend in the Middle Eastern countries. The prevalence of certain cancer diagnoses among refugees, like gastrointestinal, respiratory, breast, and genitourinary cancers necessitates a multidirectional approach, primarily aimed at prevention and early detection. International partnerships are essential for improvement in cancer surveillance service availability, and delivery of the standard of care, in an overall effort to reduce the human cost, monetary, and resource associated burdens of cancer. Recommendations to implement effective prevention and management goals as well as improved record keeping in the refugee setting and the acquisition of secure and sustainable funding sources should be implemented in collaboration with global humanitarian agencies like UNHCR.

Varied Clinical Manifestations of Amebic Colitis.

Invasive amebiasis is common worldwide, but infrequently observed in the United States. It is associated with considerable morbidity in patients residing in or traveling to endemic areas. We review the clinical and endoscopic manifestations of amebic colitis to alert physicians to the varied clinical manifestations of this potentially life-threatening disease. Copyright ©Most patients present with watery or bloody diarrhea. Less common presentations of amebic colitis include abdominal pain, overt gastrointestinal bleeding, exacerbation of inflammatory bowel disease, or the incidental association with colon cancer. Amebic liver abscesses are the most frequent complication. Rectosigmoid involvement may be found on colonoscopy; however, most case series have reported that the cecum is the most commonly involved site, followed by the ascending colon. Endoscopic evaluation should be used to assist in the diagnosis, with attention to the observation of colonic inflammation, ulceration, and amebic trophozoites on histopathological examination.

Incidence of new onset atrial fibrillation in patients with permanent pacemakers and the relation to the pacing mode.

BACKGROUND: Atrial fibrillation is a relatively common arrhythmia often seen in patients with permanent pacemakers. In this study we aimed to assess the incidence of atrial fibrillation in patients whose pacemakers were programmed to pace in the right ventricle (VVI) and compared it with patients whose pacemakers were programmed in non-VVI mode(i.e. AAI or DDD). MATERIAL AND METHODS: Records of the patients with permanent pacemaker or implantable-cardioverter-defibrillator were evaluated and analyzed. These patients had regular periodic follow-up evaluation over the last 10 years. (January 1, 2002 to December 31, 2012). Patient demographic, pacemaker data, pacing mode, review and analysis of arrhythmia log for occurrence of new atrial fibrillation and echocardiographic findings for left atrial size, mitral regurgitation, were analyzed and recorded. Left atrial size was classified as mild, moderate or severe enlargement, depending on the left atrial dimension. RESULTS: Average age was 68 years. There was no gender predominance (51% male). Mean follow-up duration was 6 years and 3 months. Hispanic population represented the majority of the patients (65.4%). Majority of the devices (80.0%) were programmed as DDD pacing mode. Fifty-five patients (52.8%) did not develop atrial fibrillation. 85.7% of the patients paced in VVI-mode had atrial fibrillation while atrial fibrillation occurred in 37.4% among patients paced in non-VVI-mode. This difference was statistically significant (P<0.0001). CONCLUSIONS: Right ventricular pacing in a VVI mode was associated with higher incidence of atrial fibrillation, mitral regurgitation and left atrial enlargement. Non-VVI based pacing demonstrated lower incidence of new onset atrial fibrillation.

Rapid or normal gastric emptying as new supportive criteria for diagnosing cyclic vomiting syndrome in adults.

BACKGROUND: Cyclic vomiting syndrome (CVS) in adults is a disorder characterized by recurrent and stereotypic episodes of severe nausea, vomiting and abdominal pain separated by symptom-free intervals. Our goal was to investigate gastric emptying (GE) in CVS patients. MATERIAL AND METHODS: This was a retrospective study of 30 adult patients who met Rome III diagnostic criteria for CVS. Rapid GE was defined using two different predefined criteria as either <50% isotope retention or <65% isotope retention at 1st hour and/or <20% at 2nd hour. RESULTS: Of the 30 patients (25 had 4-hr GE) diagnosed with CVS, 22 were females and 8 males with a mean age of 39 years. Overall, 20 (80%) of the 25 CVS patients met the predefined criteria of <50% retention for rapid GE in the first hour. Fifteen (60%) met the 2-hour criteria for rapid emptying of <20% retention. Five (16.6%) patients of the 25 had a normal GE with a mean retention at the first hour of 65% (52-78%). Nine (36%) also met another predefined criteria of <35% retention for rapid GE in the first hour. Sixteen (64%) met criteria for normal GE. CONCLUSIONS: (1) In adult CVS patients, GE is either rapid or normal, clearly distinguishing this entity from gastroparesis. (2) Cyclic vomiting syndrome is an important new etiology to explain the finding of rapid GE on a radionuclide test. (3) We suggest that rapid gastric emptying should be added as supportive criteria for diagnosing CVS in adults.

Lessons from the profile of kidney diseases among Afghan refugees.

BACKGROUND: Due to a paucity of research on the profile of kidney diseases among refugee populations, specifically Afghan refugees in Iran, this study aimed to illustrate the pattern of kidney disease among Afghan refugees in Iran and create a database for evaluating the performance of future health services. MATERIAL AND METHODS: This was a retrospective cross sectional study, in which we collected the demographics and profile of kidney diseases among Afghan refugees between 2005 and 2010 from referrals to the United Nations High Commissioner for Refugees (UNHCR) offices in Iran. RESULTS: The total number of referrals in this group of diseases was 3193 out of 23 152 with 41.5% female and 58.5% male. Regarding age distribution, 10.5% were 0-14 years of age, 78% were 15-59, and 11.5% were ≥60. The most common health referral for females and males (0-14) was end-stage renal disease (ESRD), accounting for 34.6%. This was also the main reason of referrals for females and males aged 15-59, accounting for 73.5% and 66.6%, respectively, and in both sexes in the ≥60 age range it was 63.1%. CONCLUSIONS: The pattern of our renal clinic referrals may gradually change to ESRD, which is associated with a huge economic burden. The need to provide health insurance to everyone or reform the health care system to provide coverage for more of the population can be justified and would improve cost effectiveness.

Clinical profiles and outcomes of acute aortic dissection in a predominantly Hispanic population.

BACKGROUND: Acute aortic dissection (AAD) is a life-threatening cardiovascular condition with high morbidity and mortality. We sought to assess clinical profiles as well as outcomes of AAD in a predominantly Hispanic population and to explore the relationship between this condition and uncontrolled/untreated hypertension in this community. MATERIAL AND METHODS: This was a single-center, retrospective, cross-sectional study of patients admitted with AAD over a 10 years period. RESULTS: Fifty-nine cases of AAD were included in the analysis. The group of Hispanics with AAD had more females (48.3%, p=0.002), more dyslipidemia and coronary artery disease (p=0.006 and 0.05, respectively), and a tendency to be older and have more hypertension and diabetes compared to non-Hispanics. Although 70.2% of all patients had hypertension, only 52.5% of them were being treated; of those, only 66.7% achieved optimal blood pressure control prior to presentation. Only 47.4% received beta-blockers for blood pressure control in the acute setting. Longer length of in-hospital stay was associated with older age, higher troponin and creatine kinase levels, and presence of hypertension. In-hospital death occurred in 10 (17%) patients and mortality was significantly associated with higher serum creatinine (p=0.01). CONCLUSIONS: Hispanic patients with AAD were more likely to be female, of older age, and have more cardiovascular risk factors in comparison to non-Hispanics. In addition, significant under-treatment of hypertension in this population and underutilization of beta-blockers for blood pressure control in the acute settings was evident. Better prevention and timely treatment may improve outcomes for this condition in this population.

Primary central nervous system amelanotic melanoma in a Hispanic male: Case report.

BACKGROUND: Primary melanotic neoplasms of the central nervous system (CNS) are uncommon; amelanotic melanomas in this region are extremely rare. Very few cases of amelanotic variation of primary melanoma in the CNS were reported on. General guidelines or recommendations to establish this diagnosis do not exist. CASE REPORT: A sixty-year-old male Hispanic patient presented with a 7-day history of numbness and dizziness. Initial laboratory work-up and physical examination were inconclusive. Cerebral radiological imaging showed a left frontal lesion. Further work-up after clinical deterioration revealed an increase in the lesion size consistent with hemorrhage and changes in T1WI. Biopsy and immunochemistry demonstrated the presence of amelanotic melanoma in the CNS without evidence of another primary lesion. CONCLUSIONS: Primary amelanotic melanoma of the CNS represents a challenge, clinically and diagnostically. Magnetic resonance imaging can be helpful in early stages. Final diagnosis is established with immunohistochemical testing. Physicians should be aware of the existence of this rare manifestation and difficulties faced while building this diagnosis.

Gastric pH and Therapeutic Responses to Exsomeprazole in Patients With Functional Dyspepsia: Potential Clinical Implications.

BACKGROUND: Therapy for functional dyspepsia remains a challenge. This study aimed to evaluate esomeprazole (E) versus placebo (P) regarding (1) the effectiveness in providing relief of abdominal pain or discomfort during 16 weeks of therapy in patients with functional dyspepsia having moderate or severe symptoms; (2) the effects on gastric acid suppression and (3) the relationship between symptom relief and gastric pH. METHODS: Enrolled patients were randomized to E (n = 38) or P (n = 35) in a double-blind, placebo-controlled trial. Outcomes were measured at four 4-week intervals. Drug dose titrated at each visit, based on relief of abdominal symptoms. The 24-hour gastric pH was monitored at baseline, 4 and 8 weeks. RESULTS: After 4 weeks, 71% of E patients (40mg) reported satisfactory symptom relief versus 34% of P patients (P < 0.001). When the dose for nonresponders (NR) was titrated to 40mg twice daily, the E relief rate increased to 82% versus 56% in P group (P < 0.05). During the next 4 weeks, with dose decreased by half in responders, E response rate declined to 69% versus 48% in P group (P < 0.10). When the dose was increased for NR during the last 4 weeks, E rate increased to 83% versus 57% in P group (P < 0.05). At 4 and 8 weeks for E responders and NR, patients׳ pH >4 value increased significantly compared to baseline. CONCLUSIONS: (1) Though E 40mg once daily is superior to P, some patients benefit from 40mg twice daily; (2) E, 40mg once daily, profoundly inhibits gastric acid secretion; (3) intragastric pH monitoring before and after therapy may help address the relationship between symptomatic relief and gastric acid secretion and (4) some patients respond to monitored titrated placebo therapy.

Clinical manifestations and endoscopic findings of amebic colitis in a United States-Mexico border city: a case series.

BACKGROUND: Invasive amebiasis is not frequently seen in the United States. It is associated with considerable morbidity in patients residing in or traveling to endemic areas. We report a case series of patients with amebic colitis in a United States-Mexico border city to alert physicians to the varied clinical manifestations. CASE PRESENTATION: Nine patients were diagnosed with amebic colitis. Mean age was 56 (38-83), 6 were males, and all were Hispanic. Common symptoms were diarrhea (56 %), hematochezia (33 %) and abdominal bloating (11 %). The diagnosis of amebic colitis was established in the following ways: 8 patients by colonoscopy with biopsy, 1 by surgery for colonic obstruction. The diagnosis of amebic colitis was confirmed in 8 patients (89 %) by amebic trophozoites present in histopathologic sections. One patient was diagnosed with amebic colitis based upon clinical symptoms, colitis on colonoscopy and visualization of amebic trophozoites on stool examination. In the 8 patients in whom colonoscopy was done, 6 (75 %) had inflammation with rectosigmoid involvement and 5 (62.5 %) had ulcerations. Infection resolved after treatment with metronidazole in most patients; however, one patient developed a liver abscess and another had a colonic perforation and later developed a liver abscess. CONCLUSION: The occurrence of amebic colitis in this United States-Mexico border city hospital population was low, but in some cases potentially life-threatening. Physicians should be alert to the less common presentations of amebic colitis, such as overt gastrointestinal bleeding, exacerbation of inflammatory bowel disease, and the incidental finding of association with colon cancer, or a surgical abdomen. Rectosigmoid involvement was typically found on colonoscopy.

Cardiovascular safety profile and clinical experience with high-dose domperidone therapy for nausea and vomiting.

INTRODUCTION: Domperidone is a dopamine receptor antagonist with peripheral prokinetic and central antiemetic properties. Prolongation of the QTc interval with chronic use of oral domperidone in standard doses has been reported in the literature. Our goal was to investigate cardiac toxicity in patients receiving 2-fold greater doses than in previous reports. METHODS: A retrospective chart review was conducted of patients with nausea (N) and vomiting (V) receiving domperidone from 2009 to 2013 under an Investigational New Drug (IND) protocol. Patient demographics, indications for therapy, clinical outcomes, cardiac symptoms and electrocardiogram tracings were reviewed. Prolonged QTc was verified if >470 milliseconds in females (F) and >450 milliseconds in males (M). RESULTS: A total of 64 patients, 44 female (37% Hispanic, 60% white, 3% African American), were taking domperidone for diabetic gastroparesis 45%; idiopathic gastroparesis 36%; chronic N&V 8%; dumping syndrome 5%; cyclic vomiting 5% and conditioned vomiting 1%. Mean duration of therapy was 8 months (range, 3 months to 4 years). Doses ranged from 40 to 120 mg/d with 90% receiving 80 to 120 mg compared with the standard dose of 40 mg. Of note, 73% of subjects benefited from treatment with reduced nausea and vomiting. Thirty-seven patients had follow-up electrocardiograms available, and they showed that the mean QTc at baseline was 424 milliseconds ± 28.4 (SD) compared with 435 milliseconds ± 27.2 (SD) at follow-up (not significant). Ten of these patients had prolonged QTc at F/U ranging from 453 to 509 milliseconds, without any cardiovascular complaints. There was no relationship between prolonged QTc and daily dose of domperidone, body mass index or age. CONCLUSIONS: Our data indicate that at very high dosing, the prokinetic/antiemetic agent domperidone has a low risk of adverse cardiovascular events while exhibiting good clinical efficacy.

Comparative Outcome of Computed Tomography-guided Percutaneous Radiofrequency Ablation, Partial Nephrectomy or Radical Nephrectomy in the Treatment of Stage T1 Renal Cell Carcinoma.

Renal cell carcinoma (RCC) accounts for 3% of all cancers in adults. The indications for Radiofrequency Ablation (RFA) for renal carcinomas include T1a (tumor 4 cm or less, limited to the kidney), elderly patients, renal impairment, comorbidities, poor surgical candidate, and multiple bilateral renal masses. We retrospectively reviewed medical records, specifically investigating the indications, complications and outcomes of RFA and nephrectomy for treatment of RCC in a tertiary medical center with a predominantly Hispanic patient population. Forty-nine patients with RCC were evaluated. Nine patients had RFA, 9 had partial nephrectomy and 31 had radical nephrectomy. All patients among the 3 groups had stage T1N0M0 RCC at diagnosis. Tumor recurrence was observed in 2 (22%) patients that had RFA, one (11%) patient that had partial nephrectomy and no patients that had radical nephrectomy. One patient had recurrence of the tumor at the opposite kidney pole from the initial RFA site 4 years later. This particular patient did not have any tumor recurrence at the site of the initial RFA. A second RFA was performed on the recurrent tumor with no recurrence upon subsequent follow up visits. The second patient had recurrence of the RCC on 1 year follow that was discovered to be sarcomatoid RCC, which is an aggressive type with a poor prognosis. Our results support the clinical utility of RFA in patients with stage T1 RCC who are poor surgical candidates or those with reduced renal function. The clinical utility of RFA as an equally effective approach when compared to partial nephrectomy in patients with stage T1 RCC that meet strict indications for the procedure. The treatment choice should be individualized and based on the characteristics of the renal tumor such as size, location and histological type of RCC. We conclude that RFA presents a safe treatment choice for patients with RCC if long term follow up is maintained.

West nile virus encephalitis induced opsoclonus-myoclonus syndrome.

West Nile virus (WNV) is an arthropod borne neurotropic single stranded RNA flavivirus with <1% developing presenting with neurological disease. Immunocompromised and elderly patients are more prone to developing WNV meningitis or encephalitis. Definitive diagnosis of WNV meningoencephalitis is a combination of clinical suspicion and cerebrospinal fluid (CSF) serology. Forty-eight year old Caucasian female presented with a sudden onset of altered mental status after being found unresponsive. She was confused with intermittent bouts of alertness/lethargy and unintelligible responses to questioning. Her medical problems included endometrial cancer that was in remission after undergoing a total abdominal hysterectomy with bilateral salpingectomy and postoperative chemotherapy with paclitaxel and carboplatin. Pertinent physical examination revealed muscle strength that was significantly decreased, nuchal rigidity and +2 pitting edema of both lower extremities. Computed tomography and magnetic resonance imaging of the brain were negative for any intracranial pathology. CSF analysis was consistent with aseptic meningitis with all CSF serology being negative except for positive WNV antibody. A few days after being admitted she developed involuntary random movements of her eyes and generalized jerking movements (myoclonus). This was determined to be opsoclonus myoclonus syndrome (OMS) induced by the WNV meningoencephalitis. She then received five consecutive days of plasmapheresis with a significant improvement in her neurological status. Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder associated with chaotic multidirectional eye movements, myoclonus and less frequently cerebellar ataxia. OMS affects as few as 1 in 10,000,000 people per year. The pathogenesis is not fully understood with the majority of cases of opsoclonus-myoclonus syndrome being idiopathic. According to current medical literature there have only been two previous case reports of opsoclonus myoclonus syndrome associated with WNV encephalitis.

Recurrent Skin and Lung Infections in Autosomal Dominant Hyper IgE Syndrome with Transactivation Domain STAT3 Mutation.

Background. Hyper IgE is a rare systemic disease characterized by the clinical triad of high serum levels of IgE (>2000 IU/mL), eczema, and recurrent staphylococcal skin and lung infections. The presentation of hyper IgE syndrome is highly variable, which makes it easy to confuse the diagnosis with that of severe atopy or other rare immunodeficiency disorders. Case Report. A 23-year-old Hispanic presented with history of frequent respiratory and gastrointestinal infections as a child and multiple episodes of skin and lung infections (abscess) with Staphylococcus aureus throughout his adult life. He had multiple eczematous lesions and folliculitis over his entire body, oral/esophageal candidiasis, and retention of his primary teeth. The IgE was elevated (>5000 IU/mL). Genetic mutation analysis revealed a mutation affecting the transactivation domain of the STAT3 gene. Conclusion. The hallmark of hyper IgE syndrome is serum IgE of >2000 IU/mL. Hyper IgE syndrome is a genetic disorder that is either autosomal dominant or recessive. A definite diagnosis can be made with genetic mutation analysis, and in this case, it revealed a very rare finding of the transactivation domain STAT3 mutation. Hyper IgE syndrome is a challenge for clinicians in establishing a diagnosis in suspected cases.

Outcomes of the use of fully covered esophageal self-expandable stent in the management of colorectal anastomotic strictures and leaks.

Introduction. Colorectal anastomotic leak or stricture is a dreaded complication leading to significant morbidity and mortality. The novel use of self-expandable metal stents (SEMS) in the management of postoperative colorectal anastomotic leaks or strictures can avoid surgical reintervention. Methods. Retrospective study with particular attention to the indications, operative or postoperative complications, and clinical outcomes of SEMS placement for patients with either a colorectal anastomotic stricture or leak. Results. Eight patients had SEMS (WallFlex stent) for the management of postoperative colorectal anastomotic leak or stricture. Five had a colorectal anastomotic stricture and 3 had a colorectal anastomotic leak. Complete resolution of the anastomotic stricture or leak was achieved in all patients. Three had recurrence of the anastomotic stricture on 3-month flexible sigmoidoscopy follow-up after the initial stent was removed. Two of these patients had a stricture that was technically too difficult to place another stent. Stent migration was noted in 2 patients, one at day 3 and the other at day 14 after stent placement that required a larger 23 mm stent to be placed. Conclusions. The use of SEMS in the management of colorectal anastomotic leaks or strictures is feasible and is associated with high technical and clinical success rate.

Bird fanciers' lung induced by exposure to duck and goose feathers.

PATIENT: Female, 60 FINAL DIAGNOSIS: Bird fanciers' lung Symptoms: Cough productive • hypoxia • short of breath • substernal chest pain MEDICATION: - Clinical Procedure: - Specialty: - OBJECTIVE: Rare disease. BACKGROUND: Hypersensitivity pneumonitis (HP) is a group of inflammatory interstitial lung diseases caused by hypersensitivity reactions from repeated insults of inhalation of fine particulate organic dusts derived from environmental sources. Bird fanciers' lung (BFL) is the most common form of HP, with an estimated prevalence of 0.5-7.5% and is observed in individuals who develop a hypersensitivity response to avian droppings or antigens on bird feathers. CASE REPORT: A 60-year-old woman presented to our care with shortness of breath with exertion. She was hypoxic with oxygen saturation of 70% on room air. The CTA of the chest revealed a diffuse bilateral ground glass density in the lung parenchyma with a mosaic attenuation pattern. On further questioning she explained that she collected many duck and goose feathers she found on the ranch and placed them in a vase at home. Transbronchial lung biopsy revealed non-caseating granulomas, aggregates of epithelioid macrophages, and patchy mononuclear cell infiltration with lymphocytes and fibrotic tissue. The patient clinically improved and was discharged home on the 6(th) hospital day with prednisone 20 mg daily, with clinical improvement noted on subsequent follow up visits. CONCLUSIONS: There is no specific clinical manifestation; abnormal laboratory test results help establish a definitive diagnosis. The best diagnostic tool is the correlation of symptom onset with the environmental exposure. The prognosis is excellent after a single episode of HP, but continuous re-exposure carries the risk of progressive pulmonary impairment.

A complicated case of an immunocompetent patient with disseminated nocardiosis.

Nocardia species are aerobic, gram positive filamentous branching bacteria that have the potential to cause localized or disseminated infection. Nocardiosis is a rare disease that usually affects immunocompromised patients and presents as either pulmonary, cutaneous or disseminated nocardiosis. Forty-two year-old hispanic male presented to our care with bilateral lower extremity weakness, frontal headache, subjective fever, nausea, and vomiting. Brain computed tomography (CT) revealed multiple hyperdense lesions with vasogenic edema in the frontal, parietal and left temporal lobes. Chest CT demonstrated bilateral cavitary nodules in the lung and right hilar lymphadenopathy. Brain magnetic resonance imaging revealed multiple bilateral supratentorial and infratentorial rim enhancing lesions involving the subcortical gray-white matter interface with vasogenic edema. Patient was started on empiric therapy for unknown infectious etiology with no response. He eventually expired and autopsy findings revealed a right hilar lung abscess and multiple brain abscesses. Microscopic and culture findings from tissue sample during autopsy revealed nocardia wallacei species with multidrug resistance. The cause of death was stated as systemic nocadiosis (nocardia pneumonitis and encephalitis). The presence of simultaneous lung and brain abscesses is a reliable indication of an underlying Nocardia infection. An increased awareness of the various presentations of nocardiosis and a high index of clinical suspicion can help in a rapid diagnosis and improve survival in an otherwise fatal disease. This case highlights the importance of obtaining a tissue biopsy for definitive diagnosis on the initial presentation when an infectious process is considered in the differential diagnosis and early treatment can be initiated.

Chorea, Hyperglycemia, Basal Ganglia Syndrome (C-H-BG) in an uncontrolled diabetic patient with normal glucose levels on presentation.

PATIENT: Female, 66 FINAL DIAGNOSIS: Chorea • hyperglycemia • Basal Ganglia Syndrome (C-H-BG) Symptoms: Hemibalism • hemichorea MEDICATION: - Clinical Procedure: - Specialty: Endocrinology and Metabolic. OBJECTIVE: Challenging differential diagnosis. BACKGROUND: Hemichorea-hemiballism (HCHB) is a spectrum of involuntary, continuous non-patterned movement involving 1 side of the body. Possible causes of HCHB include hemorrhagic or ischemic stroke, neoplasm, systemic lupus erythematosus, HHNK, Wilson's disease, and thyrotoxicosis. This case illustrates the need to be aware of hyperglycemia as a cause of hemiballism/hemichorea, which is now referred to in the medical literature as C-H-BG (chorea, hyperglycemia, basal ganglia) syndrome. CASE REPORT: A 66-year-old Hispanic woman presented to our care with hemiballism/hemichorea of the right arm and leg of 1 week duration. She had been admitted 3 months prior with toxic metabolic encephalopathy secondary to hyperosmolar hyperglycemic non-ketotic syndrome with a blood glucose level of 984 mg/dL. Her blood glucose level was normal but hemoglobin A1C was 12.2%. A brain MRI revealed an asymmetric T1 hyperintensity of the left putamen. This specific finding was compatible with hyperglycemia-induced hemichorea hemiballism syndrome. The hemiballism/hemichorea slowly improved over the course of the hospitalization with strict glycemic control. At the 3-month follow-up visit she had no involuntary movements of her extremities, and she had well controlled blood glucose levels and a hemoglobin A1C of 9.0. CONCLUSIONS: In a patient with normal glycemic levels but a history of uncontrolled diabetes, C-H-BG syndrome should be on the top of the differential list when the characteristic MRI findings of a hyperintensity in the basal ganglia are observed. This is a rare disease that deserves attention because it is reversible with correction of hyperglycemia. Thus, prompt recognition and treatment is essential to avoid adverse outcomes.

Hepatitis C- and HIV-induced porphyria cutanea tarda.

PATIENT: Male, 47 FINAL DIAGNOSIS: Porphyria cutanea tarda Symptoms: Chills • cough dry • thumb swelling MEDICATION: - Clinical Procedure: - Specialty: Metabolic Disorders and Diabetics. OBJECTIVE: Challenging differential diagnosis. BACKGROUND: Porphyria cutanea tarda (PCT) is the most common type of the porphyria. It occurs due to the deficiency of enzyme uroporphyrinogen decarboxylase (UROD), which is the fifth enzyme in the biosynthesis of heme and catalyzes the conversion of uroporphyrinogen to coproporphyrinogen. The risk factors for PCT include hereditary hemochromatosis, hepatitis C infection, ethanol abuse, estrogen use, HIV, smoking, chlorinated polycyclic aromatic hydrocarbons, and hemodialysis. CASE REPORT: A 47-year-old Hispanic man presented with right thumb swelling, redness, and pain for approximately 1 week. Past medical history included HIV/AIDS, hepatitis C infection, alcohol abuse, heroin abuse, and CMV retinitis. Skin examination revealed blistering and hypo/hyper pigmented lesions over the dorsal aspects of the hands and other sun-exposed areas. Serum porphyrins were discovered to be elevated. The quantitative urine porphyrins revealed elevation of uroporphyrins, heptacarboxyl-porphyrins, hexacarboxy-porphyrins, pentacarboxyl-porphyrins and coproporphyrin. Genetic mutation of UROD was not detected. Due to the classic cutaneous lesions, laboratory findings, and associated risk factors, we were able to confirm our suspicion of the sporadic (type 1) form of PCT. CONCLUSIONS: A strong correlation has been demonstrated between the sporadic (type 1) form of PCT and hepatitis C virus (HCV) infection in multiple studies. The mechanism through which HCV infection may cause or trigger PCT is unknown. PCT has been described for many years, but still eludes the differential diagnosis in a patient with cutaneous findings. The uniqueness of our case is the possibility that combined risk factors have an effect on PCT.

Interferon-alpha-induced sarcoidosis in a patient being treated for hepatitis C.

PATIENT: Female, 43 FINAL DIAGNOSIS: - SYMPTOMS: Diarrhea • generalized weakness • headache • lightheadedness • nausea • rash • short of breath • vomiting MEDICATION: - Clinical Procedure: - Specialty: Pulmonology. OBJECTIVE: Rare diseae. BACKGROUND: IFN-alpha-2b in combination with ribavirin is now the standard of care for the treatment of hepatitis C. Sarcoidosis is a chronic multisystem granulomatous disorder characterized by noncaseating granulomas in the involved organs. The pathologic hallmark of sarcoidosis is the presence of noncaseating granulomas in the interstitium that typically involve the lymphatics. CASE REPORT: A 43-year-old woman presented to our care with 2-week history of nausea, vomiting, diarrhea, shortness of breath, migraine headache, maculopapular rash, generalized weakness, and lightheadedness. She had been treated for hepatitis C with telaprevir, ribavirin, and interferon-alpha-2b for 6 months. Chest radiograph showed bilateral diffuse prominence of bronchovascular markings. CT of the chest revealed bilateral diffuse centrilobular nodules with associated intralobular septal thickening, thickening of the central peribronchovascular interstitium, nodularity of the major fissures, and mediastinal lymphadenopathy. These findings were suspicious for atypical pulmonary sarcoidosis, possibly interferon-induced. The pathology of the mediastinal lymph node biopsy revealed noncaseating granulomatous inflammation consistent with the diagnosis of pulmonary sarcoidosis. Pathology of the skin punch biopsy showed giant-cell granulomatous inflammation without necrosis. The patient was started on prednisone 40 mg daily with a steroid tapering course for 8 weeks. CONCLUSIONS: The management of IFN-induced sarcoidosis includes the discontinuation of IFN therapy with or without the administration of systemic corticosteroids. With the increasing prevalence of HCV in the United States, it is likely that more IFN-alpha-induced sarcoidosis will be encountered by clinicians.

Multicentric Castleman's Disease and Kaposi's Sarcoma in a HIV-Positive Patient on Highly Active Antiretroviral Therapy.

Castleman's disease is a group of rare lymphoproliferative disorders. The plasmablastic multicentric Castleman's disease is frequently discovered in HIV-infected individuals in association with Kaposi sarcoma (HHV-8). Thirty-five year old male presented to our care with the main compliant of severe back pain for one week. His past medical problems include acquired immune deficiency syndrome diagnosed 12 years prior and Kaposi sarcoma, currently on highly active antiretroviral therapy (HAART). Radiographic imaging revealed hepatomegaly and diffuse lymphadenopathy. The HIV viral load was <20 polymerase chain reaction copies/mL, absolute CD4 count was 453 cells/mcL (490-1740 cells/mcL) and CD8 count was 4142 cells/mcL (180-1170 cells/ mcL). Excisional biopsy of the left supraclavicular lymph node was performed with pathological findings of HHV8+ Kaposi sarcoma in the background of multicentric Castleman's disease (plasmacytic variant). No evidence of transformation into large B-cell or plasmablastic lymphoma was noted. He was discharged on HAART and follow up to receive chemotherapy with cyclophosphamide, adriamycin, vincristine plus prednisone was started and rituximab plus prophylaxis for pneumocystis carinii. Multicentric Castleman's disease has become more relevant in recent years due to its association with HIV and HHV-8 (Kaposi sarcoma) and its potential to progress into plasmablastic B-cell lymphoma. The progression of MCD to B-cell lymphoma is a concern, especially in patients with HIV infection because it precludes the worst outcome and a high mortality, despite treatment. The most intriguing part of this case is that MCD occurred in a HIV-positive on HAART. This case signals a warning that a high suspicion for MCD can be justified even in those HIV-positive patients on HAART because the possibly of progression to plasmablastic B-cell lymphoma.