Case A Research Articles

Calcium-alkali syndrome in post-surgical hypoparathyroidism

Milk-alkali syndrome or its up-to-date definition ‘calcium-alkali syndrome’ (CAS),1 is characterized by the triad of hypercalcemia, metabolic alkalosis and various degrees of renal failure, due to the intake of variable amounts of calcium and absorbable alkali. Its initial description dates back to the early 20th century when ‘alkali powders’ became a popular therapy for peptic ulcer disease.2 In 1923, two researchers from the Mayo Clinic, Leo Hardt and Andrew Rivers,3 described the adverse effects of this treatment, and in the three following decades communications about toxicity of Sippy's protocol were frequent.4 CAS has become a rare cause of hypercalcemia (<1% of cases) since proton pump inhibitor therapy was introduced in the 1990s.5,6 Nevertheless, it remains the third most common cause of hypercalcemia and the second most frequent cause of severe hypercalcemia among inpatients.5 The main reason for the rise of incidences of CAS is the increase of calcium carbonate use for prophylaxis and treatment of osteoporosis in post-menopausal women. Notably, any situation in which an overload of calcium and alkaline element coexist fosters a risk for CAS. We describe three patients who developed this syndrome in the setting of a post-surgical hypoparathyroidism. ### Case 1 A 71-year-old woman was admitted to our hospital with a 10-day history of nicturia, polyuria, fatigue, nervousness, insomnia and headache. Her past medical history included hypertension, treated with lisinopril and hydrochlorothiazide (20/12.5 mg/day), mild chronic renal failure (creatinine, 1.6 mg/dl) and primary hyperparathyroidism. She had undergone total thyroidectomy and parathyroid resection 2 years earlier and required a second intervention 1 year later because of a new mediastinal parathyroid adenoma. At the time she developed permanent post-operative hypothyroidism and hypoparathyroidism, and calcium carbonate (900 mg/day), calcitriol (1 µg/day) and levothyroxine (100 µg/day) were started. At admission laboratory results were as follows: serum creatinine, 2.4 mg/dl; urea, 104 mg/dl; …

Cerebrotendinous Xanthomatosis: A Treatable Disease With Juvenile Cataracts as a Presenting Sign

Cerebrotendinous Xanthomatosis (CTX) is an autosomal recessive disorder of bile acid synthesis1. At least 50 different causative mutations have been identified in the CYP27A1 gene encoding for a sterol 27-hydroxylase important in bile acid synthesis2. Sterol 27-hydroxylase deficiency leads to 5α-cholestanol accumulation in the blood and tissues of affected patients1, including brain, often leading to severe neurological dysfunction that can incapacitate patients by the 4th–5th decade of life1. CTX generally presents clinically in the 2nd–3rd decade; childhood signs and symptoms can include chronic diarrhea, juvenile cataracts, school failure1,3, and potentially xanthomas1. Eighty-five percent of CTX patients develop cataracts of multiple types2, reported to occur as young as age 5–6 years1, with cataracts and chronic diarrhea commonly presenting before neurologic disease3. For this reason, and since diarrhea is non-specific, the clinician may not consider a diagnosis of CTX when cataracts are identified. Treatment for CTX is available in the form of chenodeoxycholic acid (CDCA)1,4. The FDA recently reapproved CDCA (Manchester Pharmaceuticals, Inc). As treatment of CTX from the preclinical stage prevents the onset of disease complications4, the value of early diagnosis for this disorder cannot be overstated. We describe two cases of CTX affected children enrolled in an IRB approved study at OHSU with bilateral cataracts as presenting signs of disease. Case 1 A 9 year old female was referred to an ophthalmologist for decreased vision over several months. Past ocular history was unremarkable; full ocular exam 2 years prior was normal. Family history was unremarkable. Past medical history was significant for diarrhea and nonverbal learning disorder. On examination uncorrected distance visual acuity was 20/80 and uncorrected near acuity was 20/50 in each eye. Manifest refraction was 0.75+0.50X90 in each eye, with corrected distance acuity of 20/60 in the right and 20/80 in the left. Complete dilated exam was remarkable for the presence of cataract in each eye and described as a diffuse nuclear haze on slit lamp examination, with mild posterior capsular opacification (figure 1). Retroillumination highlighted posterior capsular findings (figure 2). Sequential bilateral cataract surgery with intraocular lens placement resulted in a corrected visual acuity of 20/20 in each eye. A metabolic workup indicated plasma 5α-cholestanol was markedly elevated at 3.7 mg/dl (normal < 0.2 mg/dl1), suggestive of CTX. Urine testing confirmed CTX with elevated bile alcohol glucuronides present (30.1 μg/ml). CDCA treatment was initiated and diarrhea resolved within one month, with accompanying weight gain. Plasma cholestanol dropped to 0.5 mg/dl within 7 months and after 6 years of treatment is currently stable at <0.2 mg/dl. No further clinical symptoms of CTX have developed. Figure 1 Diffuse nuclear haze and posterior sub capsular capsule opacification in right (A) and left (B) eye. Figure 2 Posterior sub capsular opacification in right (A) and left (B) eye. Case 2 An 11 year old male was referred to an ophthalmologist for cataract evaluation. High myopia was noted by a previous ophthalmologist at age 4. Family history was significant for high myopia and detached retina in mother and grandmother. Medical history included diarrhea from infancy, delayed language, autism, and seizures. Examination was limited by cooperation; however uncorrected distance visual acuity was at least 20/400 with both eyes open. Dilated examination revealed a refraction of −9.00 +1.00X090 in the right eye, and −9.50+1.25X090 in the left and was remarkable for bilateral cataracts. The lens opacities appeared visually significant with cortical flecks and distorted retinoscopic reflexes. Sequential bilateral cataract surgery was conducted with intraocular lens placement. The corrected visual acuity was 20/30+1 in the right and 20/40 in the left eye. Approximately 5 years later the patient was screened for a cholesterol disorder. Plasma 5α-cholestanol was elevated at 3.1–4.2 mg/dl, consistent with CTX. Elevated bile alcohol glucuronides in urine confirmed CTX. Diarrhea resolved within one month of initiating treatment. Plasma 5α-cholestanol dropped to 0.6 mg/dl within 6–8 months. After 8 months of treatment, the patient remains intellectually impaired, and continues to require an augmentative communication device. Although he still types single words to express wishes and needs, his spoken vocabulary has expanded modestly. His behavioral difficulties (making loud noises/head banging) also appear to be subsiding.

The adjuvant role of thyroxine in the treatment of chronic hepatitis C infection

Thyroid disease (TD) is the commonest extra-hepatic complication in patients with chronic hepatitis C undergoing combination interferon-α and ribavirin treatment. About 5–10% develops TD of various aetiologies during the course of treatment.1,2 It had been suggested that patients who developed thyroid diseases tend to achieve sustained virological response more readily3 although a meta-analysis did not support this contention.4 Furthermore, the underlying pathogenetic mechanisms are poorly understood. The following 2 cases are presented to support this observation. In both cases, initial therapies were unsuccessful with normal thyroid function, whereas re-treatment with interferon-α (and ribavirin) in the presence of overt TD resulted in successful sustained viral clearance. Although anecdotal, these cases lend further evidence to the potential synergistic effect of thyroid hormone and interferon therapy. Further research is required to support this hypothesis. ### Case 1 A 48-year-old woman presented for ongoing management of her TD after her first course of interferon therapy. She had chronic hepatitis C of genotype 1 with cirrhosis. She underwent a first course of treatment with combination interferon-α (IFN-α) and ribavirin (RBV) for 48 weeks and did not achieve sustained virological response (SVR) at the end of treatment despite good compliance. She had regular monthly thyroid function tests throughout and they were entirely normal. Four weeks after completion, she developed Graves’ disease (GD) with hyperdynamic cardiovascular activity in the presence of a confirmed diffuse goitre. Her thyrotropin (TSH) was undetectable [reference range (RR), 0.4–4.0 mU/l]; free tetra-iodothyronine (fT4) was 28.7 (RR, 10.1–24.5 pmol/l), free tri-iodothyronine (fT3) was 6.9 (RR, 3.3–5.8 pmol/l). Her anti-thyroglobulin (anti-Tg) antibody was normal at 1:64 (RR, < 1 : 400), anti-thyroperoxidase (anti-TPO) titre 1 : 640 (RR, 1: 400), and thyrotropin stimulating immunoglobulin (TSI) titre 14 (RR, < 10 U/ml). The thyroid pertechnetate uptake scan showed diffuse uptake at 11%, (RR: 3–8%). The endocrinology team confirmed the diagnosis and began treatment …

Rituximab in anti-glomerular basement membrane disease

The role of rituximab has expanded beyond the treatment of non-Hodgkins lymphoma,1 to include successful management of rheumatoid arthritis,2 systemic lupus erythematosus,3 anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis4 and primary glomerulonephritis.5 Decrease incidence of side-effects or toxicity, coupled with clinical effectiveness, suggests that B-cell depleting agents may convey an advantage over traditional immunosuppressive agents. Rituximab therapy has previously been reported in anti-glomerular basement membrane (GBM) disease.6,7 Because of side-effects such as sterility and the risk of infection in the febrile patient with conventional therapy, we have used rituximab in anti-GBM disease to suppress antibody production. We report three cases of anti-GBM disease treated with rituximab along with steroids and plasma exchange. ### Case 1 A 54-year-old man with a history of hypertension and multiple sclerosis presented with severe renal failure (serum creatinine 1874 µmol/l) and anuria. His GBM antibodies were >680 U/ml. He was dialysis dependent from the outset and treated with i.v. pulsed methylprednisolone (PMP) 1 g daily for four days, i.v. cyclophosphamide 500 mg and plasma exchange. He developed overt pulmonary haemorrhage on Day 5, along with thrombocytopenia and leucopenia. Because of fears of infection and signs of marrow suppression, we switched cyclophosphamide (only one dose given) to rituximab (375 mg/m2 four times weekly doses). …

Non-compaction du ventricule droit : à propos de deux cas

BackgroundThe noncompaction of myocardium is a rare myocardiopathy. The isolated right ventricular involvement is exceptional. We report two cases of isolated noncompaction of the right ventricular myocardium. Case 1I., 2years old, investigated for a congenital cyanosis with severe functional repercussion. Her echocardiography revealed a complex congenital heart disease associated with isolate noncompaction of the right ventricle. Case 2A., 5years old, was referred for history of syncope for the 2 last years. The echocardiography showed a severe pulmonary hypertension and an isolate noncompaction of the right ventricle. ConclusionDue to the rarity of the isolate non-compaction of the right ventricle, it diagnosis remains difficult. The treatment and the outcomes of this disease are still unclear. An early diagnosis may help to reduce its complication.

The entrepreneur and the bully: case study

Mady, an entrepreneur, builds a retirement home in 2002. During the start up, a funding shortage necessitates her bringing in a management company. As part of the funding agreement, the management company assumed the role of hiring. Poor hiring decisions by the management company unionise the organisation and burden the organisation with problem employees. Case A documents the events confronting the entrepreneur. Case B documents the actions taken by the entrepreneur to solve her problems with a bully.

Delayed diagnosis of tooth aspiration in three multiple trauma patients with mechanical ventilation

Tooth aspiration is a critical and rare complication in trauma patients. It can remain undetected and misdiagnosed. Here we present three mechanically ventilated multiple trauma cases with delayed diagnosis of bronchial tooth aspiration. Case 1 A 23-year-old man was severely injured in a car accident and had multiple trauma, with an injury severity score (ISS) of 50 and a head abbreviated injury score (AIS) of 5. Three days later he was transferred to our hospital and chest CT showed a tooth-like foreign body in the right bronchial tree, which was confirmed to be a bicuspid ( according to the Palmer notation method) by flexible bronchoscopy. As the tooth was psilate and firmly embedded, many attempts with either flexible or rigid bronchoscopy could not extract it. The patient died of sepsis 70 days after the accident. Case 2 A 26-year-old man was presented to a local hospital with multiple trauma (ISS 54, head AIS 5) following a motorcycle accident. On the next day when he was transferred to our hospital, a chest film and fibreoptic bronchoscopy confirmed a lateral incisor () in the left lobe. We failed to extract the tooth, but fortunately it was expectorated via the tracheotomy tube following a vigorous cough on day 12. Case 3 A 25-year-old man was presented with multiple trauma (ISS 41, head AIS 4) after a motorcycle accident. He was transferred to our hospital 9 hours later. Chest CT and flexible bronchoscopy confirmed a central incisor () in the right bronchial tree. It was extracted by flexible bronchoscopy on day 8 (Figure ​(Figure11). Figure 1 CT images of lung and maxillofacial bones, inspired tooth in case 3. (a-d) CT scan on day 1 showed the tooth-like foreign body (white/black arrow) in the right bronchus. (e) Reconstructed CT images on day 2 showed fracture of mandible and left zygomatic ... Early diagnosis of tooth aspiration in coma patients is difficult because aspiration is rarely considered in the absence of an acute clinical presentation. Thus, an endobronchial tooth can remain undetected for a long time and result in serious complications [1]. In all three cases reported here, tooth aspiration was missed by the local hospitals. Doctors should suspect that any tooth that has been avulsed and not found as possibly aspirated in trauma patients, especially for those in a coma status. The medical history, clinical signs and radiological findings should be carefully checked. CT is more accurate than chest radiography in detecting endobronchial foreign bodies. When negative chest radiography or doubtful clinical findings are presented, bronchoscopy should be performed to confirm the diagnosis [2]. Both flexible and rigid bronchoscopy can be used for the diagnosis and removal of teeth [3]. Tracheotomy may facilitate the removal of an aspirated tooth.

Membranous nephropathy after use of UK-manufactured skin creams containing mercury

Membranous glomerulonephritis (MGN) is the commonest cause of the nephrotic syndrome in adults.1,2 The majority of cases are idiopathic (primary MGN), however ∼30% are secondary to systemic diseases such as SLE, epithelial malignancies, medications or exposure to heavy metals.3 Recently, an autoantibody has been identified in >70% of cases of idiopathic MGN with specificity for the M-type phospholipase A2 receptor (PLA2R).4 Skin-lightening creams, which may contain mercury salts, are widely used in ethnic subgroups where paler skin is seen as a desirable attribute.5 With prolonged use, mercury toxicity can occur, leading to the development of autoimmune phenomena and renal dysfunction.6 We describe two cases of biopsy-proven MGN associated with elevated serum and urine mercury levels in females who used skin-lightening creams containing mercury that had been manufactured in the UK. Neither patient had antibodies against the PLA2R providing further evidence for the specificity of this antibody for primary MGN. ### Case 1 A 44-year-old woman originally from Pakistan was referred for investigation of the nephrotic syndrome. She had peripheral oedema, an albumin of 32 g/l, a plasma creatinine of 95 µmol/l (eGFR59 ml/min/1.73 m2) and a urine protein–creatinine ratio of 1419 mg/mmol (normal <30). Her history included peptic ulcer disease and vitamin D deficiency, which had been diagnosed 4 months before the …

Psychological changes after receiving comprehensive interventional therapy and the effect of psychological intervention on carcinoma patients

Objective To study the psychological changes and the effect of psychological intervention and to discuss the influence of psychological rehabilitation on carcinoma patients after comprehensive interventional therapy.Methods 100 carcinoma patients were divided into two groups randomly:the psychological intervention group(50 casea) received both psychological intervention therapy and comprehensive intervention therapy;the control group (50 cases) received only comprehensive interventional therapy;Curative effects were assessed with the Symptom Checklist90(SCL-90) ,the Self Rating Depression Scale(SDS) and the Self Rating Anxiety Scale(SAS) for each patient. Results For the psychological intervention group, SCL-90 scores after therapy was significantly lower than that before therapy ( P < 0. 05 ), and were also significantly lower than that ( without anxiety agent) of the control group. The scores of SDS and SAS in post-therapy patients were significantly lower than that of pre-therapy patients in the psychological intervention group. The scores of SDS had no statistical difference between post-therapy patients and pre-therapy patients in the control group. The scores of SAS in post-therapy patients was significantly lower than that of pre-therapy patients in the control group. Both scores of SDS and SAS in the control group were significantly higher than that of the psychological intervention group after therapy. Conclusion Psychological intervention for carcinoma patients after comprehensive interventional therapy played an promotional role in psychological rehabilitation in the community. Key words: Cancer; Interventional therapy; Anxiety; Depression; Community; Psychological intervention

Free Communications

Background: Persulfate salts are potent oxidizing agents in hair bleach products that accelerate the bleaching process. Ammonium and potassium persulfates may cause delayedtype and immediate skin reactions. Also allergic asthma and rhinitis have been described. Objectives: Ammonium and potassium persulfates may cause delayed-type and immediate skin reactions. Also allergic asthma and rhinitis have been described. Methods: Case 1A 36-year-old female hairdresser suffered from contact urticaria after exposure to bleaching agents for ten years. She also experienced asthma and rhinitis when she worked in the hair salon for the same period. Several minutes after accidental contact with bleaching powder she developed an urticarial reaction on the neck, facial angioedema, and dyspnoea. We performed prick-to-prick tests with serial dilutions of freshly prepared persulfates starting with 0.1%. Prick-to-prick tests with ammonium persulfate 1.0% aqua and potassium persulfate 1.0% aqua showed wheals of 8 mm (positive control 6 mm). Ten minutes after the prick-to-prick tests, she suffered from asthmatic complaints which cleared after inhalation of b2-sympathicomimetics. Patch testing with ammonium persulfate 2.5% petrolatum and potassium persulfate 2.5% aqua resulted in wheals at the test site after 20 minutes. Prick tests with ammonium and potassium persulfate 1.0% aqua were negative in four controls.Case 2A 48-year-old woman showed pruritus of the scalp and facial angioedema which started several minutes after application of a hair bleaching product. Forty-five minutes after application she fainted with increased heart rate and low blood pressure. She was treated with clemastine, dexamathasone, and saline and transferred to a hospital. Prick tests with ammonium persulfate 2.0% aqua and potassium persulfate 2.0% aqua showed wheals of 4 mm (positive control 7 mm). Intradermal testing with ammonium persulfate 0.1% aqua and potassium persulfate 0.1% aqua showed wheals of almost 20 mm. Several hours after the intradermal tests, she became increasingly dizzy which cleared after treatment with fexofenadine. Patch tests with ammonium persulfate 2.5% petrolatum and potassium persulfate 2.5% aqua were negative after 20 minutes. Intradermal tests with ammonium and potassium persulfate 0.1% aqua were negative in three controls. Results: We presented two cases of immediate type hypersensitivity to persulfates with an anaphylactic reaction in one patient. These reactions have never been adequately documented. Conclusion: It seems that the reactions are immunologic and based on release of IgE. In patients suspected of allergic reactions to persulfates, prick and patch tests should be performed. Because severe respiratory symptoms and anaphylaxis may occur, we recommend to start with prick-toprick testing.

Takotsubo cardiomyopathy in association with endogenous and exogenous thyrotoxicosis

### Case 1 A 79-year-old woman from Sri Lanka, with a history of hyperthyroidism, presented with chest pain and breathlessness following an episode of extreme anxiety after missing her connecting flight. She had been unable to take carbimazole during the preceding 3 months due to local shortages. Examination revealed signs of acute heart failure, with an electrocardiogram (ECG) showing anterior T-wave inversion, a 12-h cardiac Troponin I of 4.1 ng/ml and thyroid function tests demonstrating thyrotoxicosis (free thyroxine [fT4] 40.4 pmol/l, thyroid-stimulating hormone [TSH] 0.04 mU/l). Coronary angiography showed only minor disease in the left anterior descending and circumflex arteries. Ventriculography revealed classical apical ballooning and left ventricular dysfunction. Cardiovascular magnetic resonance (CMR) imaging on Day 3 confirmed this finding on cine imaging and without evidence of delayed gadolinium hyperenhancement (Figure 1). Her symptoms resolved within 4 …

Low field MRI on the diagueqs of nasopharynged carcinoma(NPC)

Objective The MRI manifestdtions of NPC were analysed and low-field MRI on the diagnosis of NPC was disscussed.Methods The MRI manifestations cf 83 casea that were confirmed by biopsy were analysed retrospectively. Results The MRI of NPC manifese mainly as follow:The mucosa thickened or solf tissue mass formed on the top of posterior, left and right nasopharyngeal wall,Pharyngeal recess became supperficial and disappearing, And pharyngeal ostium of eustachian tube became dull or bstructive, The pharyngeal space displaced or narrowed and disappeared. unilteral or bilateral otomastoiditis can be seen and can be accompanied by the destruction of skull base, carernous sinus and orbit can be violated, and temporal fossa or pterygopalatine fossa can be involed .The T1WI signal of tumorous tissue was homogeneous or slightly low, the T2WI signal was slightly high or higher on the enhamced scanning, the amount(or ratio) of serer,moderate and mild enhanced cases were respectively 22(31.0%), 41(57.7%), 8( 11.3% ).Conclusions Low-fild MRI can imdicate muticfaceted clearly and accurately the location,size, shape, violating scope of NPC and the extent of involting adjacent tissue and structure, So the low-fild MRI is the best imaging examination on the diagnosis of NPC. Key words: Nasopharyngeal carcinoma(NPC); Low-fild MRI; Diagnosis

The Hoarse Patient: Asking the Right Questions

Case 1 A man, age 64 years, reports that he has had hoarseness and throat pain for the preceding three months. His symptoms have been constant and gradually worsening in intensity. He has lost approximately 15 lb (6.8 kg) and reports a 60 pack-year history of smoking, in addition to consuming three to four alcoholic drinks daily for the preceding 40 years. How would you treat this patient?

Dynamic Response of Unlined Circular Tunnels Subjected to Internal Explosion

Due to the development of urban areas, underground structures are increasingly needed. Therefore, not only it is likely to have two tunnels located close to each other, but also it happens frequently.Because of the complexity of realistic problems, closed form analytical solutions are not accessible for them. However, latest advances in computational techniques have made numerical approaches more practical for realistic problems. Boundary element method (BEM) is one of such approaches. This technique formulates the problem in terms of boundary values. The main advantage of BEM, especially in comparison with finite element and finite difference methods, is that the discretization is only applied to the boundary, thus reducing the volume of modeling and the number of unknown variables. Moreover, the radiation condition at infinity is exactly satisfied in this method which is very striking for wave propagation problems. First the formulation of boundary element method in the time domain is presented and then a parametric study on the dynamic response of unlined circular tunnels subjected to internal explosion is done. Depth of tunnels, vertical and horizontal distance between two tunnels, diameter of tunnels, and other parameters related to geometry are important factors which can affect displacements and stresses of tunnels under explosive loading. In the present study, effect of two parameters has been investigated: changing the depth of the tunnel that is subjected to internal explosion (case A) and changing the horizontal distance between two shallow tunnels subjected to explosion (case B). Radial displacements and circumferential stresses are the results obtained. Case A- Tunnel depth: The tunnel behavior in depth of H=50R is alike to behavior of same tunnel in infinite depth. Also, radial displacement in upper point of tunnel in depth of H=2R is about 2.7 times of the same tunnel in infinite depth. Circumferential stress in points placed on horizontal diameter of tunnel in depth of H=2R is about 1.8 times of the same tunnel in infinite depth. Case B- Tunnels distance: Two tunnels with horizontal distance of greater than L=50R don’t affect each other. When horizontal distance parameter of two tunnels is L=2.5R then the maximum normalized displacement of the side point of the loaded tunnel, is approximately 2.15 times of the same tunnels with distance of infinite. Also, when horizontal distance of two tunnels is L=2.5R, circumferential stress in lower point of the loaded tunnel, which is normalized with static stress, is approximately 1.2 times of the same tunnels with distance of infinite. Normalized results calculated here display the importance of the geometric parameters on the behavior of unlined tunnels subjected to internal explosion and provide suitable criteria for displacement design of shallow tunnels that are subjected to internal explosion. The accuracy of the present study for the computation of radial displacements and circumferential stresses is verified by solving several problems.

Pheochromocytoma in Pregnancy

Hypertensive disorders in pregnancy remain among the most understudied areas despite the recent advancement in medical care and management.1 Although most of this is ascribed to a pregnancy-specific disorder, preeclampsia, there is a paucity of data and few recommendations about another potentially disastrous hypertensive disorder, pheochromocytoma, a catecholamine producing tumor, with a reported incidence of <0.2 per 10 000 pregnancies.2 Despite its rarity, untreated pheochromocytomas carry a risk of mortality for both mother and fetus, as high as 58%.3 This may be attributed to several factors, such as the failure to detect the condition because of its extreme rarity, the tendency of these tumors to have varied presentations, and the fact that pregnancy may preclude certain imaging modalities and radioisotope testing.2,4,5 The enlarging uterus may also trigger tumor activity, in addition to the tendency for gravidas to undergo operative procedures on short notice.6 Thus, it is imperative that physicians who manage patients with pheochromocytoma familiarize themselves with special considerations in relation to pheochromocytoma during pregnancy. Focus should be directed toward understanding the indications of when women with chronic or de novo hypertension during gestation should undergo the special tests used to diagnose pheochromocytoma and how to manage the disease once diagnosed. This report surveys 6 pheochromocytomas managed at our institution, reviews the literature of pheochromocytomas, and presents recommendations on how to better suspect, detect, and manage these disorders in pregnant populations. We reviewed 6 cases of pheochromocytoma managed at the University of Chicago Medical Center between 1984 and 2009. Table 1 briefly summarizes their presentation and management. Two of these cases (cases 1 and 4) were published previously in detail but are presented to provide a more complete spectrum of presentation. View this table: Table 1. Summary of Cases ### Case 1 A 31-year–old black woman presented during gestational week 21 …

Use of Pedometer to Assess the Requirement and Consumption of Calories by Service Dogs

The assessment of the type and amount of food given to dogs, the diet formulation and the evaluation of the intake of calories according to the physical work of the dog, are essential conditions to maintaining the health status and performance of the service dogs. The housing and climate conditions also play a major role in maintaining the health status of the dogs and in expressing their genetic potential. The research used service dogs, German Shepherd breed, aged 1.6 to 9 years, which were observed and monitored during their performance of specific duties, without taking them out of the environment where they live and work. The requirement of energy and the amount of food required each day are calculated function of the body weight (BW) and of the intensity of the effort. The BW categories that were surveyed were: normal weight (40 kg BW, case A), over weight (50 kg BW, case B), and under weight (35 kg BW, case C). BMI evaluation (body mass index) was done according tot the international specifications, between 1 – emaciated, to 9 – very obese. The analytical methods evaluated the chemical structure of the given food; the amount of dry commercial food required for maintenance according to the physical work; the consumption of calories of the dog function of the walked distance (expressed in meters and steps) during the physical work. The consumption of calories during the physical work was calculated with a Dista T300 pedometer, which recorded the walked distance in terms of meters and steps, and the consumption of calories during the physical work. Caloric consumption=0.14*caloric necessary+43.61*(travelled) distance-7.57 In this case, the caloric necessary and the (travelled) distance influence the caloric consumption by 99,86% and the result will be estimated with 4.51 error. Instead, if entries 1 and 3 are eliminated (because of the low number of kilometers, which statistically speaking could be considered extreme values), we can observe a decrease of the two factors’ influence, but with a growth in the precision of the estimates. Caloric consumption=0.05*caloric necessary+55.8*(travelled) distance-115.23 In this case, the caloric necessary and the (travelled) distance influence the caloric consumption by 98,42%. The daily consumption of food of the dogs monitored with the pedometer was 593 Kcal in average, which requires an additional 167 g food per day, to the requirement for maintenance of 529 g food/day, giving a total of 696 g (40 kg average dog BW; 3.55 Kcal/g dry commercial food). The results revealed that the permanent monitoring of the consumption of calories in the dogs making constant physical work, but different in terms of intensity and duration, may be of use for the fast adjustment of their feed requirement, which must meet the daily requirement of calories.

Chest Pain in Children and Adolescents

1. Surendranath R. Veeram Reddy, MD* 2. Harinder R. Singh, MD* 1. *Division of Cardiology, The Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University School of Medicine, Detroit, Mich After completing this article, readers should be able to: 1. Enumerate the most common causes of chest pain in pediatric patients. 2. Differentiate cardiac chest pain from that of noncardiac cause. 3. Describe the detailed evaluation of a pediatric patient who has chest pain. 4. Screen and identify patients who require a referral to a pediatric cardiologist or other specialist. 5. Explain the management of the common causes of pediatric chest pain. ### Case 1 During an annual physical examination, a 12-year-old girl complains of intermittent chest pain for the past 5 days that localizes to the left upper sternal border. The pain is sharp and stabbing, is 5/10 in intensity, increases with deep breathing, and lasts for less than 1 minute. The patient has no history of fever, cough, exercise intolerance, palpitations, dizziness, or syncope. On physical examination, the young girl is in no pain or distress and has normal vital signs for her age. Examination of her chest reveals no signs of inflammation over the sternum or rib cage. Palpation elicits mild-to-moderate tenderness over the left second and third costochondral junctions. The patient reports that the pain during the physical examination is similar to the chest pain she has experienced for the past 5 days. A detailed cardiovascular and other organ system examination yields normal results. What is the most likely cause of this patient's chest pain? What will you recommend for this patient? Does she need to see a pediatric cardiologist? ### Case 2 A 17-year-old boy is playing soccer on a Saturday afternoon and has a syncopal event on the field. He regains consciousness within a few seconds, does not require resuscitation, and is taken to the emergency department. The patient informs the physician that he developed sudden midsternal chest pain and lightheadedness prior to passing out. His vital signs are normal for age, and he is …

IgM Myeloma: Report of Two Cases of a Rare Entity with Unusual Immunophenotype.

Abstract 4901 IntroductionPlasma cell myeloma is characterized by clonal proliferation of plasma cells and serum and/or urine paraprotein, most commonly IgG, IgA or light chain only. IgM, IgD, IgE and non-secretory variants are infrequent. IgM myeloma comprises less than 0.5% of myelomas. These should not be confused with B-cell non-Hodgkin lymphoma showing plasmacytoid differentiation and an IgM paraprotein, particularly lymphoplasmacytic lymphoma. Two cases are reported herein. Case 1A 57 year old female with known MGUS, type II diabetes, rheumatoid arthritis, underwent total thyroidectomy for papillary thyroid carcinoma. Two months later she required excision of a cervical lymph node, identified by radioactive iodine scanning, revealing metastatic papillary thyroid carcinoma. However, during pre-operative evaluation, cytopenias were noted (hemoglobin 7.8 g/dL, platelets 86 × 109L), and renal insufficiency (creatinine 2.1 mg/dL). Subsequent laboratory findings included a serum IgM kappa paraprotein of 6158 mg/dL, normal IgA, decreased IgG of 423 mg/dL, elevated beta-2 microglobulin (7.7 mg/L), elevated serum viscosity (3 cp), mildly decreased serum calcium (8.2 mg/dL). A skeletal survey was negative. Pathologic findings: Peripheral blood showed pancytopenia, marked RBC rouleaux, and occasional atypical plasmacytoid cells (5%). Bone marrow biopsy sections revealed marked hypercellularity with a predominance of plasmacytoid cells. By immunohistochemistry (IHC), these were CD138+, CD117+. MUM1+, weak cytoplasmic kappa+, weak CD79a+, CD45-, CD19-, CD20-, PAX 5-, CD5-, CD10-, CD23-, CD56-, cyclin D1-. Flow cytometry (FC) of peripheral blood also revealed abnormal cells, of comparable phenotype (CD45-, CD19-, CD20-, CD38+, CD138+, CD56-), though CD117 and cytoplasmic light chain expression were not evident. Clinical course: Management included plasma exchange for serum hyperviscosity, pulse steroids and thalidomide. Despite these interventions, her condition worsened, including a progressive decline in renal function, requiring hemodialysis. Eventually she declined further treatment, and expired two months after diagnosis. Case 2An 83 year old male patient presented with fatigue, dyspnea, diffuse pain, paresthesias involving hands and feet of two weeks duration. Past history was remarkable for COPD, lower extremity DVT, and hyperlipidemia. Physical examination revealed no lymphadenopathy or organomegaly. Laboratory studies revealed pancytopenia, IgM kappa paraprotein of 4664 mg/dL, normal serum IgG, diminished IgA, elevated serum creatinin (1.7 mg/dL), normal serum calcium, elevated beta-2 microglobulin (4.9 mg/L) and elevated serum viscosity (3.4 cp). A skeletal survey was negative. Pathologic findings: Peripheral blood revealed pancytopenia with marked RBC rouleaux. Marrow aspirate smears showed abundant abnormal plasma cells, many showing numerous, azurophilic, crystalline cytoplasmic inclusions, Auer rod-like. Bone marrow biopsy was hypercellular and heavily infiltrated by abnormal plasma cells. By IHC, these were CD138+, MUM1+, cyclin D1+, CD56+, cytoplasmic kappa+, CD20+ (sub-set), weak CD117+. FC of marrow aspirate yielded comparable results (CD56 co-expression, bright CD38+ and CD138+, CD19-, CD20-, CD45-). Conventional cytogenetics revealed a normal male karyotype. Interphase FISH revealed t(11; 14), trisomies 11, 13, 14 and 17, with 13q14 and 17p13 deletions. Clinical course: The patient rapidly deteriorated, and expired shortly after hospital admission. ConclusionWe describe two cases of IgM myeloma. The diagnosis in each case was supported by morphology and immunophenotype and, in one case, by interphase FISH. These add to our limited knowledge regarding the biology of this disorder. Recently, it has been suggested that such cases are of relatively uniform immunophenotype, in particular lacking either CD56 or CD117 expression (Feyler S et al, BJH 140, 547-551, 10/2007). Our cases show that the spectrum of immunophenotypic findings in IgM myeloma is broader than has been suggested. In addition, one of the two cases showed Auer-rod like crystalline cytoplasmic inclusions in malignant plasma cells, also a rare finding in this setting. DisclosuresNo relevant conflicts of interest to declare.

Blastic Plasmacytoid Dendritic Cell Neoplasm: Report of Three Cases.

Abstract 4703The World Health Organization (WHO) recently published a revised, updated edition of the WHO Classification of Tumours of the Haematopoietic and Lymphoid Tissues, including new criteria for the recognition of some previously described neoplasms as well as clarification and refinement of the defining criteria for others. It also adds entities – some defined mainly by genetic and immunophenotypic features – that have only recently been characterized. Particularly, the diagnosis and classification of acute leukemias of ambiguous lineage is debated; among these: “blastic NK-cell lymphoma” and “agranular CD4+/CD56+ hematodermic neoplasms”. Both of them are now known to be, in virtually all cases, a tumor derived from precursors of a specialized subset of dendritic cells, plasmacytoid dendritic cells, and so are myeloid-related neoplasms defined as blastic plasmacytoid dendritic cell neoplasm (BPDCN). This is a clinically aggressive neoplasm that is usually characterized at onset by solitary or multiple skin lesions, often with associated regional lymphadenopathy, and frequently by involvement of the PB and BM. Leukemic cells show submembranous cytoplasmic vacuoles and pseudopodia-like extensions of agranular cytoplasm. The blasts in such cases do not express myeloperoxidase or nonspecific esterase, and are characterized by the expression of CD4, CD43, CD56, CD123, BDCA-2/CD303, TCL1, and CLA; CD7 and CD33 are not uncommonly expressed as well, and TdT is expressed in about 30% of cases. There is no expression of CD34 or CD117. Here we report three cases with clinical data, cytological and immunophenotypic findings strongly suggesting the diagnosis of BPDCN. Case 1An 80 year-old-man was admitted to our institution on December 2006. He referred the occurrence of skin lesions since January 2005, when a diagnosis of extranodal B-cell non-Hodgkin lymphoma was made and treatment with conventional chemotherapy was performed, but without achieving any response. At our evaluation he presented leukocytosis (144 × 109/L) associated with purplish, firm nodules on the trunk, arms and face. Peripheral blood and bone marrow aspirate showed the presence of blast cells with a lymphoid appearance, granular periodic acid-Schiff (PAS) positivity and a high expression of CD33, CD4, and CD56. He was treated with AML-like therapy, but died of disease progression. Case 2A 79-year old woman was admitted in December 2006 with a 2-month history of anemia, splenomegaly, and weight loss of 10 kg in the last year. Laboratory tests were as follows: Hb, 41 g/L; leukocytes, 2.5 × 109/L (with 10% of blast cells); platelets, 43 × 109/L. No lymphadenopathy or skin lesions were present. Bone marrow examination revealed 41% of small to medium-sized blast cells without Auer rods or granula and negative reactivity to myeloperoxidase, esterase and PAS. She was treated with an AML-like protocol; she achieved partial response, but died after three months, of disease progression. Case 3A 69-year-old man was admitted to our Institution for cytopenia in June 2009. He referred the occurrence of brownish-purple firm nodules on the trunk since April 2009. At our evaluation he presented pancytopenia; bone marrow aspiration was performed and revealed infiltration by 65% of blasts with reticulated chromatin, evident nucleoli, a vacuolated cytoplasm and pseudopodia-like expansions. The blasts were negative for myeloperoxidase, monocyte esterase and PAS staining. Skin biopsy revealed a dermal infiltration by the same blastic-cell BM population. He underwent AML-like therapy and, although the skin lesions disappeared, 30% blastic bone marrow infiltration persisted.Morphological revision of these cases, selected for their peculiar immunophenotype reported in the following Table, revealed the same cytological features and cytochemical reactivity in cases 2 and 3; case 1 had a lymphoblastic-like morphology and showed PAS positivity, but the lack of cCD3 was not consistent with the diagnosis of ALL. All the cases were FLT3-ITD+. We suggest that a correct modern panel of MoAb with a careful morphological examination could help to pose the diagnosis of BPDCN, which typically affects older patients and is characterized by poor prognosis.CaseCD4CD56HLA-DR7.1cMPOcCD3cCD79aCD34CD117CD33CD3031++++-----+nt2++++-----++3++++-----++ Disclosures:No relevant conflicts of interest to declare.

Foreign Body Ingestion and Aspiration

1. Marisa C. Louie, MD* 2. Stuart Bradin, DO* 1. *Pediatric Emergency Medicine Division, Department of Emergency Medicine, University of Michigan, Ann Arbor, Mich After completing this article, readers should be able to: 1. Recognize the presenting history, signs, and symptoms of patients who have swallowed or inhaled foreign bodies. 2. Discuss the long-term complications of gastrointestinal and airway foreign bodies. 3. Describe appropriate management strategies for patients who have common esophageal and gastric foreign bodies. 4. Identify the risks associated with ingestion of button batteries and recognize when emergent removal is necessary. 5. Maintain a high level of suspicion for aspirated foreign bodies, recognizing that delays in diagnosis can lead to increased complications. ### Case 1 A 3-year-old boy is brought to his pediatrician's office by his mother after an older sibling saw him swallow two small pieces from a magnetic building set. He is playful and smiling. There is no history of choking or distress. Radiography reveals two small rodlike objects in his stomach. ### Case 2 A 10-year-old girl who has developmental delay is brought to the emergency department after possibly swallowing a calculator battery 2 hours ago. She is asymptomatic. On the radiograph, a small round object is seen in the lower esophagus. ### Case 3 A 2-year-old boy is brought into an urgent care facility for new-onset wheezing. He has a low-grade fever and cough but no rhinorrhea. He has mild tachypnea with wheezing on the right side. Albuterol treatments and steroids are started. In spite of several treatments, he continues to wheeze. These three cases are examples of a common pediatric problem: foreign body ingestion and aspiration. Because toddlers explore the world with their mouths, tend to eat and run, and have a less developed chewing ability, they are the most common age group seen for foreign body complaints. Many studies report a slight male predominance. This review addresses foreign body ingestion and aspiration. ### Background Foreign body ingestion is a frequent reason for pediatric visits. Coins are common culprits, followed by small toys …