Cases from the Osler Medical Service at Johns Hopkins University

Presenting features

A healthy 44-year-old man presented to the Johns Hopkins Hospital after two episodes of nonpurulent hemoptysis, each consisting of approximately one-half cup of blood. The episodes were painless and were preceded by minimal coughing. The blood was bright red and without clots. He denied a recent history of upper respiratory tract infection, chest trauma, chest pain, or nasal/sinus disease. He had a chronic nonproductive “smoker’s cough.” He denied a recent history of fevers, chills, night sweats, hematuria, joint pains, epistaxis, or weight loss, and had no allergies. His past medical history was notable for neurogenic diabetes insipidus and diet-controlled diabetes mellitus. His only medication was intranasal desmopressin acetate. He smoked two packs of cigarettes daily for more than 20 years and rarely drank alcohol.On physical examination, he was afebrile, with normal heart rate and blood pressure. His oxygen saturation was 94% on room air, and his respiratory rate was 16 beats per minute. His lungs were clear to auscultation bilaterally, without evidence of crackles or wheezes. He had bilateral digital clubbing. His white blood cell count was 10,770/mm³, with a normal differential. His hematocrit was 46%, and his platelet count was normal. Blood chemistries and urinanalysis were normal. A posteroanterior and lateral chest radiograph obtained on admission demonstrated moderate diffuse interstitial fibrosis involving the upper and lower lung (Figure 1A and 1B ). What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Bartonella henselae infection of the liver and spleen

Presenting features

A 26-year-old man with human immunodeficiency virus infection (CD4 count, 3/μL) was admitted for evaluation of a 2-month history of fevers and fatigue and a 1-week history of nausea and abdominal pain. He had no previous history of opportunistic infections. His physical examination was notable for a temperature of 39°C and mild hepatomegaly without significant tenderness. There were no heart murmurs, and he did not have splenomegaly, lymphadenopathy, skin rash, or neurological changes. His white blood cell count was 2.9/μL, his platelet count was 132,000/μL, and his hematocrit was 22.4%. His alkaline phosphatase level was slightly elevated at 152 U/L, but his aminotransferase and bilirubin levels were normal. Evaluation included negative hepatitis serologies, negative blood and mycobacterial cultures, negative toxoplasmosis immunoglobulin (Ig) M and IgG, a nonreactive rapid plasma reagin, a negative purified protein derivative skin test, and negative serum cryptococcal and urine histoplasmosis antigens. A computed tomographic scan of the abdomen (Figure 1) revealed hepatomegaly with a patchy enhancing pattern, periportal edema, and multiple small lesions in the spleen.Upon further questioning, the patient reported that he had traveled extensively in Italy, France, and Spain while in the military, during which had he consumed unpasteurized milk products. He was currently working as a drill sergeant at a prison. He also had a pet cat and had recent contact with a friend’s kitten.What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University

Presenting features

A 41-year-old man with a past history of alcohol abuse, drug abuse, and smoking (>40 pack-years) presented with chronic epigastric pain and weight loss. During the preceding year, the epigastric pain had worsened progressively and was exacerbated by eating. For the past 3 months, his nausea and vomiting had worsened so that he was no longer able to tolerate solid food. Consequently, he had lost 50 lbs in 1 year. During the 3 weeks before admission, he had daily subjective fevers but no night sweats. He denied any melena, hematemesis, jaundice, or changes in stool color or consistency.His past medical history was remarkable for an episode of massive hematemesis, for which he was admitted to another hospital 3 months ago and found to have a bleeding gastric ulcer. Despite treatment with a proton pump inhibitor, his abdominal pain, nausea, and vomiting worsened after dis-charge. He stopped drinking alcohol after that hospitalization, and he stopped using intravenous drugs 1 month before this admission.On physical examination, the patient appeared wasted. His vital signs were notable for a normal blood pressure with resting tachycardia and an orthostatic increase in pulse rate. His jugular veins were flat. Cardiac and respiratory examinations were normal. The abdominal examination revealed guarding in the epigastric region with hypoactive bowel sounds and no rebound tenderness. His stool was guaiac negative. Laboratory studies revealed a total white blood cell count of 4180/mm³, a hematocrit of 33.1%, a platelet count of 239,000/mm³, a total protein level of 8.3 g/dL, an albumin level of 3.7 g/dL, and antibodies to human immunodeficiency virus (HIV). A computed tomographic scan of the abdomen showed thickening of the antrum with large gastric folds.What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Diagnosis: Polymorphic population of mature and immature hematopoietic cells consistent with extramedullary hematopoiesis

Presenting features

A 40-year-old man with human immunodeficiency virus infection (CD4 count, 694/μL) and a history of sickle cell anemia was admitted for pain management after a right iliac wing fracture sustained in an assault. He denied recent fevers or respiratory symptoms. His medical history was remarkable for repeated vaso-occlusive crises and episodes of the acute chest syndrome, with resultant pulmonary hypertension and right-side heart failure. His physical examination was most notable for fever as high as 38.2°C, cervical and axillary adenopathy (2 cm), elevated jugular venous pressure, cardiomegaly, and hepatomegaly. His hematocrit was 19.5%, with a mean corpuscular volume of 91 fL; the platelet count was 176 000/μL; and the leukocyte count was 15 000/μL. He underwent a computed tomographic scan of the chest, abdomen, and pelvis (Figure 1), which revealed discrete lesions of low attenuation in the liver, the largest being 4.5 cm in diameter, and autoinfarction of the spleen.What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University

PRESENTING FEATURES: An 18-year-old white man was admitted to the Osler Medical Service with the chief complaint of back pain. Two weeks prior to admission, the patient developed diffuse and aching upper back pain. Over the next couple of days, he also developed severe anterior chest pain that was somewhat pleuritic in nature but diffuse and extending bilaterally into the shoulders. One week prior to admission, he developed intermittent fevers and night sweats. The patient denied any lymphadenopathy, pharyngitis, sick contacts, shortness of breath, rash, or bleeding. He was seen by a physician and told that he had thrombocytopenia. There was no history of recent or remote unusual bleeding episodes. His medical history was unremarkable except for a childhood diagnosis of attention deficit/hyperactivity disorder. He was not taking any medications and had no history of tobacco, alcohol, or illicit drug use. He had no risk factors for human immunodeficiency virus infection. Physical examination showed that he was afebrile and had normal vital signs. He was a well-appearing man who was lying still because of pain. HEENT examination was unremarkable. There was no pharyngeal erythema or exudates. His lungs were clear. His neck was supple and without lymphadenopathy. Examination of his back and chest revealed no focal tenderness. There was no hepatosplenomegaly, and his skin was without petechiae or rashes. Examination of the patient's joints showed pain on passive and active movement of his shoulders bilaterally, but no frank arthritis. There was no rash, petechiae, or echymoses. Chest radiograph and electrocardiogram were unremarkable. On admission, the laboratory examination was notable for a hematocrit level of 32.5%, with a mean corpuscular volume of 79 fL, and white blood cell count of 2.8 x 10(3)/microL. Platelet count was 75 x 10(3)/microL. A white blood cell differential revealed 7% bands, 53% polys, 34% lymphs, 5% atypical lymphocytes, 2% nucleated red cells, and a few young unidentified cells. His chemistry studies were unremarkable. What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Antiglomerular basement membrane disease

A 47-year-old Taiwanese man with no notable medical history was admitted with low-grade fevers and night sweats that had persisted for 5 to 6 weeks. An extensive investigation at another hospital could not determine the cause of the fevers, but documented acute renal failure with a blood urea nitrogen level of 60 mg/dL and a serum creatinine level of 5.6 mg/dL. He was admitted to the Johns Hopkins Hospital for further evaluation.The patient, who had been living in the United States for the past 20 years, reported no recent travel and no behaviors that are associated with transmission of human immunodeficiency virus. He was not taking any medications, and he denied using herbal or nutritional supplements. He had no recent weight loss. There were no specific complaints on review of systems. On physical examination, he was a thin, middle-aged man in no distress. Vital signs included a temperature of 37.5 degrees C, a blood pressure of 166/86 mm Hg, a pulse of 70 beats per minute, a respiratory rate of 16 breaths per minute, and 99% oxygen saturation on room air. Sclera were anicteric, and he had no palpable adenopathy. His lungs were clear, and his heart rate was regular without extra sounds. His abdomen was thin, nontender, and without masses or organomegaly. There was no edema or signs of embolism in the extremities. Laboratory studies revealed a white blood cell count of 14,200/mL(3), a hematocrit of 23.1%, and a platelet count of 456,000/mL(3). Blood chemistries were notable for a blood urea nitrogen level of 61 mg/dL and a serum creatinine level of 7.6 mg/dL. Levels of aminotransferases, total bilirubin, and alkaline phosphatase were within normal limits. Urinalysis revealed large hemoglobin, 1+ protein, numerous red blood cells, and 3 to 5 white blood cells. Numerous red blood cell casts were seen on microscopic examination of the urine sediment. The patient's erythrocyte sedimentation rate was >130 mm/h, and his C-reactive protein level was elevated at 12.6 mg/dL. Serologies were negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies; serum complement levels were normal. What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Diagnosis: P. carinii pneumonia and primary pulmonary sporotrichosis

PRESENTING FEATURES: A 53-year-old man who had human immunodeficiency virus (HIV) presented to the Johns Hopkins Hospital with a 3-month history of increasing dysphagia, cough, dyspnea, chest pain, and an episode of syncope. His past medical history was notable for oral and presumptive esophageal candidiasis that was treated with fluconazole 6 months prior to presentation. Three months prior to presentation, he discontinued his medications, and his symptoms of dysphagia recurred. During that time he developed intermittent fevers and chills, progressively worsening dyspnea on exertion, and a cough productive of white sputum. He also reported a 40-lb weight loss over the past 3 months. On the day prior to presentation, he had chest pain and shortness of breath followed by weakness, dizziness, and a brief syncopal episode. He denied orthopnea, paroxysmal nocturnal dyspnea, lower extremity edema, jaundice, hemoptysis, hematemesis, melena, hematochezia, or diarrhea. There was no history of alcohol use, and he stopped smoking tobacco approximately 1 month previously. He smoked cocaine but denied injection drug use. The patient had never been on antiretroviral therapy and had never had his CD4 count or viral load measured. On physical examination, the patient was a thin, cachectic man who appeared older than his stated age. His vital signs were notable for blood pressure of 102/69 mm Hg, resting tachycardia of 102 beats per minute, resting oxygen saturation of 92% on room air, normal resting respiratory rate, and a temperature of 38.1 degrees C. His oropharynx was clear, with no signs of thrush or mucosal ulcers. His pulmonary examination was notable for diminished breath sounds in the lower lung fields bilaterally. Cardiac, abdominal, and neurologic examinations were normal. His skin was intact, with no visible petechiae, rashes, nodules, or ulcers. Laboratory studies showed a total white blood cell count of 3.2 x 10(3)/microL, with a total lymphocyte count of 330/microL, hematocrit of 30.2%, a serum sodium level of 129 mEq/L, and a serum lactate dehydrogenase level of 219 IU/L. The patient had an absolute CD4 count of 8 cells/mm3 and a HIV viral load of 86,457 copies/mL. His arterial blood gas on room air had a pH of 7.51, a PCO2 of 33 mm Hg, and a PO2 of 55 mm Hg. Electrocardiogram and serial serum cardiac enzymes were normal. A chest radiograph showed bilateral upper lobe patchy infiltrates with left upper lobe consolidation. Computed tomographic (CT) scan of the chest with contrast showed bilateral ground glass infiltrates with focal consolidation (Figure 1) and no evidence of pulmonary embolism. Induced sputum was negative for Pneumocystis carinii, fungi, or acid-fast bacilli. A bronchoalveolar lavage was performed. What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University. Zieve syndrome

Presenting features

A 47-year-old African American man was admitted to the Osler Medical Service with a chief complaint of light-headedness. He was a heavy drinker and consumed 2 pints of fortified wine every day. On the day of admission, he had been at home consuming alcohol when he stood up, became lightheaded, and immediately had to sit down. He denied any loss of consciousness, dyspnea, hematemesis, coffee-ground emesis, lower abdominal pain, bright red blood per rectum, or melena, but he recalled a burning epigastric discomfort. The patient's medical history was notable only for a 20-year history of hypertension and recently diagnosed type 2 diabetes mellitus. He was not taking any medications. He had a normal hematocrit of 43% 6 months prior to admission.Physical examination showed a supine heart rate of 115 beats per minute and blood pressure of 165/90 mm Hg, without orthostatic changes. There was scleral icterus and a jaundiced palate, but no other stigmata of end-stage liver disease. His abdominal examination was unremarkable; there were no masses, tenderness, or hepatosplenomegaly. Rectal examination revealed guaiac-negative stool. The chest radiograph and electrocardiogram were unremarkable.On admission, the laboratory examination was notable for the following values: hematocrit, 22.8% with a mean corpuscular volume of 86.7 fL and a red cell distribution width of 25.7%; absolute reticulocyte count, 177,500/mm³; total bilirubin, 4.2 mg/dL; direct bilirubin, 2.3 mg/dL; albumin, 3.3 g/dL; lactate dehydrogenase, 481 U/L; aspartate aminotransferase, 106 U/L; and alanine aminotransferase, 44 U/L. The prothrombin and activated partial prothrombin times were normal. There was no evidence of iron, vitamin B₁₂, or folate deficiency. The patient's haptoglobin level was severely depressed (<6 mg/dL) with a negative direct Coomb test and normal glucose-6-phosphate dehydrogenase activity. A peripheral blood smear was compatible with hemolysis and demonstrated normocytic, normochromic erythrocytes with moderate poikilocytosis, as well as rare spherocytes and target cells Figure 1).On the second day of hospitalization, a lipid panel revealed hypercholesterolemia with a total cholesterol level of 300 mg/dL. Due to the patient's complaint of burning epigastric pain and long history of alcohol consumption, esophagogastroduodenoscopy was performed and revealed grade 1 nonbleeding esophageal varices.What is the diagnosis?     

Extreme lymphocytopenia associated with toxic shock syndrome

Galus MA, Stern J (Medical University of South Carolina, Charleston and Pennsylvania Hospital, Philadelphia, USA). Extreme lymphocytopenia associated with toxic shock syndrome (Case Report). J Intern Med 1998; 244 : 351–354. We report an unusual case of extreme lymphocytopenia associated with staphylococcal toxic shock syndrome. A young, sexually promiscuous man presented with high fevers, productive cough, nausea with occasional vomiting, mild headache and right midthoracic and shoulder pain. His initial lymphocyte count was 72. Such a presentation in an inner city hospital, where about 15% of admissions are AIDS-related, put the diagnosis of AIDS high on the differential list. However, the CD4:CD8 ratio was 1.7, which was inconsistent with AIDS diagnosis. Further work-up led to diagnosis of toxic shock syndrome due to staphylococcal epidural abscess. The patient's lymphocyte count normalized with antibiotic treatment. A 30-year-old man presented because of high fevers, chills, productive cough, nausea with occasional vomiting and mild headache for 3 days. He also complained of right midthoracic back pain and right shoulder pain which started after he lifted a heavy object a few days earlier. He had no diarrhoea. His primary physician treated him with amoxicillin, darvocet and flexeril without significant improvement. Past medical history was significant for three episodes of meningitis in childhood. He denied intravenous drug use, but admitted to having sex with many partners. He looked ill, his temperature was 40 °C, blood pressure was 98/50 mmHg, with a pulse of 160 min^?1 and respiratory rate of 24 min^?1. After intravenous fluids, his blood pressure rose to 120/70 mm Hg with a pulse of 130 min^?1. There was a faint diffuse macular erythematous rash on the trunk and extremities. There was no nuchal rigidity. Cardiac examination revealed no gallops or murmurs. There were crackles at the right lung base. Examination of the abdomen was benign. There was tenderness to palpation of the right paraspinal muscles at midthoracic level, but no pain along the extent of the cervical and thoracic spinous processes. Neurological examination was non-focal. The results of blood tests are listed in Table 1. Chest X-ray showed increased interstitial markings. A sputum Gram stain showed Gram-positive cocci in clusters, and sputum culture grew normal flora. Lumbar puncture revealed 43 leucocytes mm^?3 with 84% of polymorphonuclear neutrophils and 16% of monocytes, glucose 64 mg dL^?1, protein 59 mg dL^?1 and no organisms were seen. No India ink examination was performed. The patient was started on broad antibiotic coverage. Chest X-ray repeated after intravenous fluid administration revealed bibasilar infiltrates and generalized increase in interstitial markings, suggesting opportunistic infection. Increased alveolar–arterial gradient persisted. Cerebrospinal fluid, blood and throat cultures showed no growth of bacteria after 24 h. Liver function tests showed slightly elevated transaminases, total bilirubin and alkaline phosphatase. Empiric therapy for Pneumocystis carinii pneumonia was begun. The patient underwent bronchoscopy which revealed acute inflammation but no Pneumocystis carinii or acid-fasting bacilli. Rapid respiratory panel was also negative after 24 h. HIV test and RPR were non-reactive. CD4 count was 43, and CD8 25 with a ratio of 1.7. Therapy for Pneumocystis carinii was discontinued, and broad antibiotic coverage was continued. On the fourth day of hospitalization, the patient started to feel better, but was still febrile. Although unlikely, doxycycline was added to cover Rocky Mountain spotted fever, leptospirosis and ehrlichiosis. A skin biopsy revealed slight chronic inflammation and telangiectasia consistent with a viral exanthem. An abdominal ultrasound was normal. Hepatitis panel was negative and rubella titres were negative. There was a low level of rubeola IgG antibodies; mono-spot and rapid respiratory panel were negative for viral infections. By the sixth day of hospitalization, clinically the patient improved further. He was feeling much better, had more energy, no shorthness of breath, only a low-grade temperature, and his rash had cleared. He still complained of some right midthoracic pain and right shoulder pain. He developed desquamation of skin of the face and palms, which he insisted was an old recurrent problem secondary to an occupational exposure preceding this illness. Ehrlichia antibodies were negative, cold agglutinin titre was low, parvovirus IgG antibodies were positive with a negative IgM; R. typhi < 1:64, R. rickettsii < 1:64. His white cell count remained persistently elevated at 14 000 mm^?3, but left shift with bandaemia resolved. The plan was to complete a 10-day antibiotic course and discharge the patient home. Despite clinical improvement, his alkaline phosphatase rose to 850 units L^?1, total bilirubin of 2.3 mg dL^?1, with normal transaminases. A repeat abdominal ultrasound was unremarkable. Following antecubital placement of heplock on the 10th day in hospital, the patient started to complain of numbness and weakness of the right hand. Neurological examination revealed weakness in the right upper extremity in a multiple nerve distribution. The neurologist suggested that the weakness might be factitious; however, he recommended an MRI of the cervical spine. The MRI revealed an epidural abscess at the level of C7–T1 and T1 osteomyelitis. The patient underwent emergent laminectomy and drainage of 10 mL of pus. Cultures grew methicillin-susceptible Staphylococcus aureus. The patient was discharged to home in a stable condition to complete a prolonged course of intravenous antibiotic therapy as an outpatient. Weakness in his hand improved after a neurosurgical procedure. Just before discharge the patient gave additional history of recent contact dermatitis of the palms for which he was receiving weekly intramuscular steroid injections.     

Imported from Cuba?

A 21-year-old man presented to the emergency room complaining of fever, chills, diarrhea and nausea with vomiting of two days'' duration. He had a past medical history of remote jejunal dysplasia, which was surgically corrected when he was a child. He was taking no regular medications. He had been well until two days earlier, when he developed a sudden onset of fever to 39.9°C, chills and severe nausea with frequent bouts of vomiting. He remembered feeling more tired than usual for three days before the onset of his fever, but nothing more specific. He saw a physician the day after the onset of fever, and the physician prescribed oral cotrimoxazole double-strength, of which he took one dose. Due to persistence of the above symptoms, he presented to the emergency room. He had travelled to Cuba for a seven-day vacation at a resort, and returned home 14 days before the onset of symptoms. He had experienced one day of mild diarrhea while in Cuba, but two of his friends experienced two to three days of diarrhea without fever at the same time while there. He denied abdominal pain, headache, cough, shortness of breath, rash, urinary symptoms or other problems. He had no risk factors for HIV infection. On examination, he was toxic and experiencing rigors, but was awake and alert. His temperature was 39.8°C, pulse was 107 beats/min and respirations were 30 breaths/min. The examination was normal except for evidence of dehydration and marked splenomegaly without tenderness. A chest radiograph and urinalysis were normal. The patient''s hemoglobin level was 167 g/L, his platelet count was 138x10⁹/L and his leukocyte count was 9.5x10⁹/L with a marked shift to the left. The patient''s creatinine level was 139 μmol/L, urea level was 6.8 mmol/L, total bilirubin level was 48 μmol/L (44% conjugated), alanine aminotransferase level was 113 U/L and gamma glutamyl transpeptidase level was 83 U/L. Other liver function tests were normal. Blood and urine cultures were obtained. An abdominal ultrasound confirmed the presence of splenomegaly with a tiny splenic cyst and an otherwise normal examination (including a normal liver). Infectious enteritis with sepsis was diagnosed and the patient was started on intravenous ciprofloxacin. The following day, two sets of blood cultures that were taken while the patient was in the emergency room showed the presence of Gram-negative rods, which were identified the next day as Salmonella species (serogroup C1), susceptible to ampicillin, cotrimoxazole, fluoroquinolones and ceftriaxone. After three days of taking parenteral ciprofloxacin, the patient felt subjectively better but continued to have afternoon and evening fevers of more than 40°C, accompanied by rigors and extreme exhaustion. His platelets decreased daily, to a nadir of 49x10⁹/L, and his leukocytes decreased to 3.8x10⁹/L. HIV serology was negative. He had no other new complaints, and the examination was unchanged. Repeat blood cultures were negative. A diagnostic procedure was performed to explain the persistent sepsis.What is your diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University

Presenting features

A 50-year-old Guatemalan man with no significant past medical history was admitted to the Barker Medical Service with weight loss over the past two weeks and fever for two days. His physical examination was remarkable for cachexia. His laboratory studies at presentation were notable for pancytopenia with a white blood cell count of 0.7 × 103/ml, hematocrit of 25% and platelet count of 8000. His admission chest radiography was normal. He had urine and blood cultures sent before beginning broad-spectrum antibiotic coverage with piperacillin/tazobactam and gatifloxacin. His peripheral blood smear is shown in Figure 1. What is the diagnosis?     

Churg–strauss syndrome: importance of colonoscopy and endoscopic biopsy in establishing diagnosis

A 31‐year‐old Japanese man was admitted with complaints of left lower abdominal pain and diarrhea. He had a history of bronchial asthma since he was 26 years old. On admission, his eosinophil count was 4100/mm ³ and increased as high as 12 000/mm ³ . A neurological examination disclosed mononeuritis multiplex with paresthesia in both lower and upper extremities. Because of hematochezia, colonoscopy was performed and disclosed multiple colonic ulcers and erosions from the rectum to the ascending colon. Endoscopic biopsy showed fibrinoid necrosis of small‐sized vessels with moderate infiltration of eosinophils in the mucosa and submucosa. He was suspected of being a case with Churg–Strauss syndrome (CSS; allergic granulomatous angiitis), and prednisolone of 30 mg/day was administered. After the administration of prednisolone, his eosinophil count decreased dramatically. His symptoms also improved gradually, except for numbness in the extremities. Three weeks later, colonoscopy revealed improvement of the mucosal appearance, but the colonic ulcers and erosions were still present. Endoscopic biopsy showed exravascular granuloma with moderate infiltration of eosinophils in the submucosa. Colonoscopy and endoscopic biopsy may be important in establishing the diagnosis of CSS with intestinal symptoms.     

Successful Treatment of Refractory Idiopathic Thrombocytopenic Purpura and Neutropenia with the Monoclonal Antibody,Rituximab

We describe a 22-year-old male with idiopathic autoimmune thrombocytopenia whose diagnosis was made at age of eight. He underwent splenectomy at age ten and ITP recurred at age 21 with episodes of infection and severe neutropenia (absolute count around 170/μl). He showed no response to immunoglobulin, corticosteroids, danazol, cyclosporine and azathioprine. Anti-CD20 antibody was administered at a dose of 375 mg/m² once a week for 2 weeks. After the second infusion of rituximab, the platelet count increased from 4,000 to 516,000/mm³ and neutrophils count raised from 180 to 545/mm³. The response improvement persisted during follow up for 9 months (neutrophil count 4,390/mm³). This observation indicates that B-cells may play a central role in the pathogenesis of ITN. Anti-CD20 antibody therapy may be an efficient treatment for the patients with chronic or recurrent ITN.     

Mediastinal Mass Causing Spinal Cord Compression

A 4-year-old Cambodian male presented to the emergency room with 2 weeks of gradually increasing leg weakness until he could no longer stand. He was also reported to have a deformity on his back, intermittent fevers and cough. His physical exam was notable for a 2 cm × 1 cm bony protrusion at his T4 vertebrae, and 2/5 strength and positive Babinski reflexes in his lower extremities. A chest x-ray showed a 3.2 cm × 2.9 cm mass in the middle mediastinum extending to the posterior mediastinum. A purified protein derivative test was positive. A computed tomography scan showed findings consistent with pulmonary tuberculosis and a paravertebral mass with amorphous calcifications, which involved destruction of the T4-T5 vertebrae and evidence of cord compression. These findings were all consistent with tuberculous spondylitis (Pott''s disease).A 4-year-old male presented to a Cambodian emergency department with gradually increasing leg weakness for 2 weeks until he could no longer stand. He was also reported to have a deformity on his back for the past 2 months, and intermittent fevers and cough for several months. He retained control of his bowel and bladder function. On physical exam he was mildly tachypneic. His skin exam revealed a 2 cm × 1 cm bony protrusion at his T4 vertebrae (Figure 1). His neurological exam was significant for 2/5 strength, and positive Babinski reflexes in his lower extremities. He had normal sensation.Open in a separate windowFigure 1.Gibbus deformity representing wedged vertebrae as a result of vertebral body collapse.Workup included anteroposterior (Figure 2) and lateral chest x-rays, which showed a 3.2 cm × 2.9 cm mass in the middle mediastinum extending to the posterior mediastinum. The patient was promptly started on anti-tuberculosis (TB) treatment. A purified protein derivative test was positive. Three sputum samples were analyzed with the Xpert Mycobacterium tuberculosis/rifampin resistance (MTB/RIF) test and were all negative. A computed tomography scan done at an outside facility showed findings consistent with pulmonary tuberculosis as well as a paravertebral mass with amorphous calcifications, which involved destruction of the T4-T5 vertebrae and evidence of cord compression. These findings were all consistent with tuberculous spondylitis (Pott''s disease). After 1 month of treatment with anti-tuberculosis medication in conjunction with physical therapy, the patient had significantly improved. He remained afebrile, his cough had decreased in frequency, and he was able to stand with support.Open in a separate windowFigure 2.Anteroposterior chest x-ray demonstrating a mediastinal mass.Despite significant progress to reverse the TB epidemic, it was estimated that there were 500,000 cases of TB in the pediatric population and 64,000 deaths in 2011.¹ Tuberculosis can spread hematogenously to vertebral bodies, causing pain and eventual bony destruction.² A Gibbus deformity is a late finding in Pott''s disease³ and clinicians should keep TB high on the differential when presented with this finding in any TB-endemic area. Although microbiological confirmation is preferred, TB can be very difficult to isolate in children in the developing world due to lack of effective diagnostic tools, paucibacillary samples, and the difficulty of obtaining high-quality specimens from children. Even the new Xpert MTB/RIF test has only been shown to have 79.5% sensitivity for detection of confirmed TB from sputum, gastric, cerebrospinal fluid, and pleural fluid samples in a pediatric population from Vietnam.⁴Current management always includes medical management and can also include surgical management for patients with acute neurological deterioration.⁵ In a resource-limited setting without a neurosurgeon, it is important to promptly initiate TB treatment without waiting for the results of diagnostic studies, advanced imaging, or biopsy.     

The clinical feature of febrile episodes

Institutionalized elderly patients are at risk of nosocomial infection because of their compromised status by aging. To clarify the relationship between fever and disease in elderly patients, we analyzed 1,105 febrile episodes, the etiology of which were already diagnosed, of 443 patients (136 men, 307 women). All patients who were 65 years of age or older and who had been admitted to the hospital for more than 7 days had fevers above 37.5 degrees C recorded. The etiologies of the 1,105 febrille episodes were respiratory tract infection in 381 (34.5%), urinary tract infection in 263 (23.8%), other diseases in 164 (14.8%) and in 297 (26.9%) unknown. The episodes were categorized into two groups by the degree of initial fever: group A, 559 episodes (50.6%) of 37.5-38.0 degrees C and group B, 546 episodes (49.4%) of above 38.0 degrees C. Of the episodes, 41.0% were one-day fevers, 21.4% two-day fevers, and 14.0% three-day fevers. The frequency of two-or-more-day fevers was significantly higher in group B (69.0%) than in group A (49.2%) (p < 0.001). In group B, respiratory tract infection (44.3%) was more frequent than urinary tract infection (16.1%) (p < 0.001). Of the respiratory tract infections, 63.5% were in group B and, in contrast, 66.5% of the urinary tract infections were in group A. The white blood cell count and C-reacting protein levels were significantly higher in group B than in group A (p < 0.001). The degree of initial fever is an important predictive marker of severity of disease in elderly patients.     

Causes and empirical treatment of fever in HIV-infected adult outpatients, Abidjan, Côte d'Ivoire

OBJECTIVES: In Abidjan, HIV prevalence has been estimated at 20% in outpatients attending community clinics. Documenting causes of fever in HIV-infected adult outpatients may help to improve care in these centres with limited facilities. DESIGN: Prospective study. METHODS: We describe all diagnoses and treatments made during febrile episodes in HIV-infected adults participating in the ANRS 059 trial and followed up in a dedicated outpatient centre. RESULTS: Causes of fever could be identified in half of the 269 febrile episodes. Bacterial diseases were the leading identified cause of fever in all CD4 cell count strata (53, 56 and 43% of identified causes in episodes with CD4 count < 200 x 106/l, 200-499 x 106/l, and >or= 500 x 106/l, respectively), followed by malaria (5, 22, and 38%, respectively). Among febrile bacterial diseases, respiratory tract infections and enteritis accounted for 62% of organ involvement, and Streptococcus pneumoniae and non-typhi Salmonella represented 69% of isolated bacterial strains. In these bacterial episodes, an early empirical antibacterial treatment was associated with shorter duration of hospitalization and fever. In the 19 episodes leading to death (7%), the two leading diagnoses were atypical mycobacteriosis (26%) and acute unexplained fever (21%). Death was associated with the absence of antimalarial treatment in the group of acute unexplained fevers. CONCLUSIONS: African HIV treatment guidelines should take into account the predominant role of bacterial infections and malaria in HIV-infected adult outpatients. Reports from other African settings would be useful to compare experiences in algorithms of empirical early antibacterial and antimalarial treatments.     

Fever in renal transplant recipients: causes, prognostic significance and changing patterns at the University of Minnesota Hospital

During a three year period in which 433 renal transplants were performed, 194 episodes of fever were documented in allograft recipients hospitalized at the University of Minnesota. Viral infections were responsible for over half of the febrile episodes, and 98 (51 percent) of the fevers were associated with cytomegalovirus (CMV), either occurring alone or in conjunction with allograft rejection or another systemic infection. Bacterial infections, fungal infections and rejection were other important causes of fever, accounting for 14 percent, 5 percent and 13 percent of the febrile episodes, respectively. Most fevers occurred in the first four months after transplantation; although about two thirds of these fevers were due to CMV, only 17 percent of fevers that occurred more than one year after the renal transplant were due to CMV. Bacterial and fungal infections and malignancy were important causes of these fevers. Of the febrile illnesses associated with transplant nephrectomy or death, a majority occurred in patients with CMV disease. Secondary bacterial and/or fungal infections were observed in a large majority of patients with lethal CMV disease. During the third year of this study there was a significant decrease in the proportion of febrile episodes due to CMV.     

Interleukin-2 treatment for persistent cryptococcal meningitis in a child with idiopathic CD4(+) T lymphocytopenia

We report a 16-year-old male patient who presented with headache, behavior changes, and fever. His cerebral spinal fluid and blood cultures grew Cryptococcus neoformans. His laboratory evaluation was negative for human immunodeficiency virus infection but flow cytometry revealed low CD4(+) count of 39 cells/mm(3) and CD4:CD8 ratio of 0.43. He was initially treated with antifungal agents with only partial clinical improvement, and he was discharged to home on oral fluconazole and prophylactic co-trimoxazole. After discharge, he continued to have persistent headache and recurrent episodes of vomiting. He was readmitted several times because of worsening of meningitis symptoms and received prolonged courses of multiple antifungal therapy, with clearance of infection from the central nervous system. He was subsequently placed on prophylactic therapy with fluconazole. His peripheral CD4(+) cell count remained low after resolution of his meningitis. Eight months after the initial diagnosis, recombinant IL-2 therapy was initiated and within a few months, his CD4(+) cell count started to increase. Treatment with rIL-2 and prophylactic antifungal therapy continued and he has been asymptomatic for almost 20 months so far. This case is the first reported pediatric idiopathic CD4(+) T-lymphocytopenia case with cryptococcal meningitis that was successfully treated by the addition of rIL-2 therapy to antifungal therapy.     

Cases from the Osler Medical Service at Johns Hopkins University. Pseudoachalasia due to esophageal adenocarcinoma

Presenting features

A 69-year-old man presented with nausea and vomiting. He was in good health until 2 years before presentation, when he first noted occasional difficulty eating solid foods. He could chew food without difficulty, but sometimes he had the sensation that the food was lodged in his chest. This sensation would persist for several hours; occasionally, he would need to induce emesis for relief.These episodes of chest discomfort became more frequent over time. Two weeks before presentation, he had to induce emesis after every meal and began having difficulty ingesting both liquids and solids. On the day of his presentation, he awoke at 3:00 am feeling hungry. He drank some juice, after which he had emesis and chest pain, which led him to believe that he was having a myocardial infarction.His past medical history included hypercholesterolemia and gastroesophageal reflux disease attributed to a hiatal hernia. His only medication was simvastatin, which he stopped taking 1 month before presentation because he thought that it was con-tributing to his difficulty in swallowing. He had not smoked for more than 30 years. He previously drank one beer a day but recently stopped drinking as well. A review of systems was notable for an unintentional weight loss of 20 lbs during the past 6 months.On admission, his vital signs were normal. His chest, cardiac, and abdominal examinations were unremarkable. His electrocardiogram and laboratory tests were normal. A barium swallow (Figure 1 and Figure 2) demonstrated tapering of the distal esophagus.What is the diagnosis?     

Cases from the Osler Medical Service at Johns Hopkins University: cardiac valvular lesions in Wegener's granulamatosis

Presenting features

A 65-year-old man presented to the emergency department with hemoptysis and hematuria. During the preceding 3 weeks, he had developed fevers, weakness, and myalgias that were increasingly debilitating. He subsequently developed frank hemoptysis and gross hematuria over 3 days. He denied contact with persons who were ill, recent travel, or dental procedures. His past medical history was unremarkable except for elevated serum cholesterol levels, for which he had been taking simvastatin for many years.On presentation, he was acutely ill, with a temperature of 38.8°C, a pulse of 90 beats per minute, a blood pressure of 132/88 mm Hg, and a respiratory rate of 18 breaths per minute. Examination of the head and neck revealed episcleritis and no oral ulcers. Examination of the heart was normal. Chest examination demonstrated bilateral coarse breath sounds with diffuse rhonchi. Skin examination revealed palpable purpura over the extensor surface of the right elbow.Laboratory studies included the following values: serum urea nitrogen, 158 mg/dL; creatinine, 11.7 mg/dL; total leukocyte count, 16.1 × 10³/mL; and erythrocyte sedimentation rate, >130 mm/h. Urinalysis was notable for 35 red blood cells per high-power field. A chest radiograph showed patchy infiltrates in the right upper and lower lobes. An electrocardiogram (ECG) revealed normal sinus rhythm at a rate of 94 beats per minute with normal intervals.The patient was admitted to the hospital with a presumptive diagnosis of vasculitis. He was treated with broad-spectrum antimicrobial agents, prednisone, and cyclophosphamide, in addition to beginning hemodialysis. Antimicrobial therapy was discontinued when blood cultures remained negative at 48 hours. The clinical diagnosis of Wegener’s granulomatosis was confirmed by an antinuclear cytoplasmic antibody titer of 1:80 and antiproteinase 3 (PR3) antibodies of 143 units (positive >30 units). A skin biopsy specimen demonstrated a small vessel vasculitis consistent with the diagnosis.The patient’s symptoms improved gradually, and he was discharged on day 13 with outpatient hemodialysis. During the next 3 days, his strength increased, and he was able to walk longer distances. On the fourth day after discharge, he suffered a ventricular tachycardic arrest at the initiation of hemodialysis. He was admitted to the intensive care unit after a prolonged resuscitation effort, but he remained unresponsive. His ECG was unchanged, and troponin I assay did not suggest myocardial infarction. Blood cultures remained negative. A transthoracic echocardiogram demonstrated normal left ventricular function and no valvular abnormalities. After 10 days, he showed no neurologic improvement. The family decided to withdraw ventilatory support, and he died quickly.What is the diagnosis?