Tuesday, July 15, 2008

Legal Action

bacterial infections

Samantha and Pete Sabatino together with their three children were looking forward to their holiday in Florida.

But during the charter flight, six-year-old Emily complained of a funny smell then vomited violently.

In the following fortnight, other family members became ill.

Mum Samantha says she suffered severe flu-like symptoms and breathing problems for the rest of the holiday.

'Fume events'

She said: "I just felt absolutely paralysed and couldn't move I had wheezing and lots of crackling in the chest… Just plain, plain plain awful. I really thought something serious was wrong."

She suspected the 'funny smell' was toxic fumes on the plane.

The air breathed on airliners is drawn in past the engines.

It can become polluted by any leaks of engine oil.

'Fume events' are rare but there are no accurate figures of just how many occur each year.

As they waited to fly home, Samantha made a list of 31 passengers who complained they too had been unwell following the flight out.

Legal action

"I just couldn't believe it.

"I was absolutely astonished that so many people had got sick and they all said their symptoms had started from the plane journey out."

Fourteen of the passengers on that Florida flight are now planning legal action.

They too believe toxic fumes had contaminated the air on board.

Doctors say Samantha didn't have an infection and she's discounted food poisoning.

She says the affected passengers ate different meals, and all she herself had was bread.

And her family are still suffering.

She said: "There has not been one week where all the children have been at school for a full week.

"One's fainted at school, one's been sent home with headache and nausea. The list is endless."

Acute exposure

The airline concerned has told Panorama there was no fume event on that flight.

That will now be argued out in court.

Samantha and her fellow passengers have found an American attorney specialising in chemical exposure cases.

Stuart Calwell says the case will focus on his claim that passengers have been damaged by breathing in chemicals contained in jet engine oil.

He said: "Samantha and her fellow passengers exhibit unequivocally signs and symptoms of acute exposure.

"What else explains it? There is no evidence of a viral infection, bacterial infections.

"Most other causes that would produce the long lasting complaints that Samantha and her fellow passengers have have been excluded."

Panorama: Something in the Air, BBC One 8.30pm Monday 21 April 2008.
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Thursday, July 10, 2008

Fluoroquinolone Resistance among Streptococcus pneumoniae in Hong Kong Linked to the Spanish 23F Clone


Fluoroquinolone Resistance among Streptococcus pneumoniae in Hong Kong Linked to the Spanish 23F Clone

from Emerging Infectious Diseases

Pak Leung Ho, Wing Cheong Yam, Terence K.M. Cheung, Wilson W.S. Ng, University of Hong Kong, Hong Kong Special Administrative Region (SAR), China; Tak Lun Que, Tuen Mun Hospital, Hong Kong SAR, China; Dominic N.C. Tsang, Queen Elizabeth Hospital, Hong Kong SAR, China; Tak Keung Ng, Princess Margaret Hospital, Hong Kong SAR, China; Wing Hong Seto, University of Hong Kong, Hong Kong Special Administrative Region (SAR), China

Abstract and Introduction

Abstract Serotypes 6A/B, 19F, and 23F accounted for 73% of 140 mucosal isolates of Streptococcus pneumoniae from Hong Kong. In pulsed-field gel electrophoresis analysis, a group of related patterns was shared by 14 of 15 ciprofloxacin-resistant and 12 of 16 ciprofloxacin-susceptible isolates. These strains exhibited capsular switching and were highly similar to the Spanish 23F clone.

IntroductionStreptococcus pneumoniae, the most important cause of community-acquired pneumonia worldwide, particularly affects young children, elderly persons with chronic cardiopulmonary conditions, and immunosuppressed patients of all ages. Widespread emergence of antimicrobial resistance has become a concern in recent years. In many countries, rates of resistance to penicillin are >40%. Among penicillin-resistant S. pneumoniae, 60% to 90% are also resistant to the macrolides, tetracyclines, chloramphenicol, clindamycin, and cotrimoxazole. For this reason, newer fluoroquinolones with expanded activity against gram-positive bacteria have been recommended by the Infectious Disease Society of America as initial treatment of choice for community-acquired pneumonia[1].

Although resistance to the newer fluoroquinolones remains rare in most countries, the percentage of nonsusceptible S. pneumoniae has increased from <0.5% for ofloxacin to 5.5% for levofloxacin (MIC >/=4 µg/mL) from 1995 to 1998 in Hong Kong[2]. Almost all strains of fluoroquinoline-resistant S. pneumoniae were isolated from respiratory tract specimens. Knowledge of the serotype distribution of S. pneumoniae, particularly strains with the emerging resistance pattern, is important for development of conjugate vaccines.

We studied the serotype distribution of recent isolates of drug-resistant pneumococci from Hong Kong, including isolates with resistance to the fluoroquinolones. To understand better the emergence of fluoroquinolone-resistant S. pneumoniae in this locality, we used pulsed-field gel electrophoresis (PFGE) to compare strains.

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Sunday, July 06, 2008

Worsening of Hypertension by Cyclo-oxygenase-2 Inhibitors

Worsening of Hypertension by Cyclo-oxygenase-2 Inhibitors

from Journal of Clinical Hypertension

Case 2

A 73-year-old female was seen in the Hypertension Clinic for a change in blood pressure control. Her sitting blood pressure was 176/94 mm Hg, which was similar to the readings she had been getting at home. When last seen 3 months previously, her sitting blood pressure had been 124/72 mm Hg while on Maxzide® 25 mg once daily and atenolol 50 mg, one and one-half tablet daily. Review of her health status showed that her primary care physician had recently started her on Celebrex® 200 mg/day. No other medication use or dietary indiscretions were found. The Celebrex® was discontinued and, upon her return to the clinic 4 weeks later, her sitting blood pressure was 132/80 mm Hg.

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Classification and Treatment of Urticaria: A Brief Review

Urticarial Vasculitis

Urticarial vasculitis represents 5% to 10% of cases of chronic urticaria, characterized histologically by the presence of vasculitis on skin biopsy. Clinically it tends to last longer than "ordinary" urticaria and be more painful and itchy. Lesions often occur at pressure points, and may resolve with residual purpura. Extra-cutaneous manifestations include transient and migratory arthralgias (50%), gastrointestinal symptoms (20%), and pulmonary obstructive disease (20%), particularly in smokers. Renal disease with proteinuria or hematuria occurs in 5% to 10% of patients, but progression to severe renal disease is uncommon. The clinical course is usually benign, on average lasting 3 years. Urticarial vasculitis may be associated with systemic lupus erythematosus, Sjögren's syndrome, hypocomplementemia, or cryoglobulinemia.  Printer- Friendly Email This

Dermatol Nurs.  2005;17(5):361-364.  ©2005 Jannetti Publications, Inc.
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Saturday, July 05, 2008

Fragile X-Associated Tremor


Summary and Introduction


Background: A 76-year-old man presented with an 8-year history of balance problems and a 2-year history of short-term memory loss. He had also been experiencing long-term problems with impotence and episodes of urinary incontinence, and had been managed for hypertension for 25 years. His medical history was otherwise unremarkable. Three of his grandchildren had been diagnosed with fragile X syndrome.
Investigations: Neurological examination, cognitive and neuropsychological testing, nerve conduction studies, MRI, and genetic testing.
Diagnosis: Fragile X-associated tremor/ataxia syndrome (FXTAS) resulting from a premutation (CGG repeat) expansion of the FMR1 gene.
Management: Explanation of the genetic ramifications of premutation carrier status for the FMR1 gene, and symptomatic treatment for the clinical difficulties experienced by the patient.Introduction

A 76-year-old man presented to a movement disorders clinic with a history of gait ataxia that had commenced at the age of 68 years. His wife had noted at that time that his gait would drift to one side, and he began to fall on a regular basis while jogging. He had used a walking stick intermittently since the age of 72 years, from which age he had also noticed a marked reduction in his stamina levels, although he could still walk a mile when necessary. After reaching the age of 74 years, he experienced several falling episodes—two resulting in fractured ribs. When he was aged 75 years, he first noted tremor in his left hand during walking; the tremor did not occur at other times.

Memory problems became increasingly apparent to the patient from the age of 74 years, and he experienced occasional episodes of confusion—for example not knowing whether to move forward at a red traffic light. At the age of 75 years his primary health-care provider commenced him on donepezil 5 mg daily, which the patient felt helped his memory and decreased his episodes of confusion.

In the months before his visit to the clinic, the patient experienced sleep disturbance that was found to improve with the prescription of trazodone 50 mg daily. He also experienced restless leg syndrome, for which his primary care physician prescribed gabapentin 100 mg daily, which appeared to improve his symptoms. The patient had experienced problems with impotence for many years, and during the year before his visit to the clinic he had occasional episodes of urinary frequency and mild urinary incontinence. He also experienced episodes of light-headedness when standing up rapidly; he had had hypertension for 25 years, which was managed with hydrochlorothiazide and atenolol. When questioned, the patient denied any numbness or tingling in his extremities, thyroid disease, diabetes, migraine, weakness, swallowing problems, or bowel incontinence. His past medical history was unremarkable apart from an ongoing high serum cholesterol level that was treated with a statin, and partial loss of hearing.

His family history revealed that two of his three daughters had children; five in total. All three of his granddaughters had full-mutation alleles (>200 CGG repeats) of the fragile X mental retardation 1 (FMR1) gene (Online Mendelian Inheritance in Man® [OMIM] 309550; Johns Hopkins University, Baltimore, MD), with mental impairment ranging from learning disabilities to mild mental retardation. Of his two grandsons, one was a carrier of the premutation allele (55–200 CGG repeats) with learning disabilities, and the other was without a fragile X mutation. One of his daughters, the mother of two of the full-mutation children, suffered from premature ovarian failure (POF)—the cessation of menses prior to the age of 40 years. Genetic testing of the patient was subsequently carried out using the standard FMR1 DNA test, which confirmed his status as a premutation carrier (78 CGG repeats).

Neurological examination demonstrated a significant bilateral ataxia on heel-to-shin movements, and a broad-based gait. The patient could not tandem walk, and the pull test was positive. Although the patient had no rest tremor, a mild tremor was present in his left hand while walking, and his walking arm swing was decreased bilaterally, with greater decrease on the left. He displayed no other features of parkinsonism, such as increased tone. Tremor was absent with finger-to-nose testing, but the patient had a slight postural tremor in the left hand. His palmomental reflex and snout reflex were positive. Deep tendon reflexes were symmetrical—2+ in the upper extremities, 3+ at the knees, but barely detectable in the ankles. Vibration sense was absent in the toes and ankles and present in the right knee, but was decreased by 50% in the left knee as well as in the right and left forefingers. Pinprick sensation and temperature and position sense were normal in the upper and lower extremities.

Cognitive testing at the age of 72 years using the Wechsler Adult Intelligence Scale®—Third Edition (WAIS-III; The Psychological Corporation, San Antonio, TX) had demonstrated a full-scale IQ of 111 (verbal 108; performance 113). At the time of the patient's presentation at the age of 76 years, his full-scale IQ was found to have diminished to 103 (verbal, 112; performance, 92). Additional neuropsychological testing at this time revealed memory problems on the Rey Auditory–Verbal Learning Test (RAVLT), with scores ranging from the 6th to the 18th percentiles for age. The patient also demonstrated executive function deficits on the Stroop color–word test with deficits in inhibition (T score of 31), and on the Behavior Dyscontrol Scale 2 (BDS2; score 11 = severely impaired). These results indicated significant deficits in his executive function and short-term memory.

An MRI scan at the age of 72 years had displayed mild brain atrophy, which was found to have progressed to moderate atrophy by the time of the patient's presentation at the age of 76 years. There was an increased T2 signal intensity in the subcortical and periventricular white matter, which was accompanied by characteristic involvement of the deep cerebellar white matter and middle cerebellar peduncles (MCPs) at both age 72 years (Figure 1) and age 76 years.

Figure 1.  (click image to zoom)

MRI of the patient at the age of 72 years. (A) Axial T2-weighted image demonstrating symmetric, increased signal within the middle cerebellar peduncles. (B) Axial fluid-attenuated inversion recovery (FLAIR) image revealing increased signal within the periventricular cerebral white matter. (C) Sagittal T1 image demonstrating mild atrophy of the caudal pons, mild cerebral volume loss and thinning of the corpus callosum. Images courtesy of J Brunberg.      

Nerve conduction velocity studies showed a slowing of motor conduction velocity across the elbow (40 m/s; normal >48 m/s) and a reduction of sensory nerve action potential amplitude in the right ulnar nerve (2 mV; normal >7 mV) that was consistent with entrapment neuropathy. In the lower extremities, there was evidence of a mild motor and sensory neuropathy as demonstrated by absence of the right sural nerve action potential, temporal dispersion of the compound muscle action potential in the left tibial nerve on proximal nerve stimulation, and bilateral absence of tibial H-reflexes.

The genetic ramifications of premutation carrier status for the FMR1 gene were explained to the patient, and he was continued on his current medications although the dose of his donepezil was increased to 10 mg per day. He was also subsequently offered a trial of amantadine 100 mg twice daily for ataxia. Both the patient and his wife felt that the donepezil was helpful; follow-up has been scheduled to assess improvement in his symptoms with medication.  Printer- Friendly Email This

Nat Clin Pract Neurol.  2007;3(2):107-112.  ©2007 Nature Publishing Group
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Thursday, July 03, 2008

“CRAB” Findings in a Patient With Fatigue and Poor Appetite




52-year-old African-American female.Chief Complaint

None; the patient was seen for follow-up of a recent stem cell transplant. The patient denied any major symptoms (eg, bone pain, poor appetite); however, she reported minimal fatigue and poor appetite that had only recently returned to normal.History of Present Illness

Two years prior to this most recent clinic visit, the patient had presented to the emergency department with flu-like symptoms and was found to have normocytic/normochromic anemia. The work-up that followed included serum (SPE) and urine (UPE) protein electrophoresis, immunofixation electrophoresis (IFE), a bone marrow biopsy and examination, and imaging studies of the axial skeleton. SPE revealed the presence of an M-spike in the gamma-region of the gel at a concentration of 6.4 g/dL. IFE, performed on the patient's serum, demonstrated the presence of an IgG kappa monoclonal immunoglobulin (Figure 1A). SPE of a sample from the patient's 24-hour urine collection demonstrated an M-spike, subsequently identified by IFE as IgG kappa. Microscopic examination of the patient's stained bone marrow biopsy demonstrated the presence of aberrant plasma cells constituting 64% of all cells observed in the bone marrow. Imaging studies of her axial skeleton revealed lytic lesions in both humeri and femurs, and in the skull, a compression fracture of the T12 vertebra, and a plasmacytoma in her left-side sixth rib. Laboratory testing revealed normal serum electrolytes, calcium, and creatinine levels. The patient started a prolonged chemotherapeutic regimen that included 3 cycles of vincristine, adriamycin, and dexamethasone (VAD), combination therapy with Thalidomide and Decadron, and Decadron maintenance therapy. Each regimen provided only a short-term reduction in the size of her serum Mspike and in her urine free light chain excretion rate. As a prelude to an autologous stem cell transplant, the patient underwent a repeat bone marrow biopsy, SPE, and UPE. The bone marrow biopsy demonstrated 27% aberrant plasma cells, while the SPE again showed an M-spike consistent with IgG kappa (0.38 g/dL); however, the UPE of a sample from a 24-hour urine collection was negative for any intact or free light chain immunoglobulins (data not shown). The patient underwent stem cell mobilization followed by bone marrow myeloablation using high-dose melphalan therapy and stem cell transfusion. She tolerated the procedure well, requiring only administration of packed red blood cells and platelets to maintain an adequate cell count of these formed elements while engraftment was occurring.

Figure 1.  (click image to zoom)

Patient's prior (A) and most recent (B) SPEP (top panel), densitometric tracing/report (middle panel), and IFE (bottom panel), demonstrating the presence of an M-spike in the gamma-region (A; top and middle panels) of the IgG kappa type (A; bottom panel), and the presence of M-spikes in the gamma-region (B; top and middle panels) consisting of the IgG kappa (2 distinct subypes), IgG lambda, and free lambda light chain types (B, bottom panel). IFE, immunofixation electrophoresis; ELP, electrophoresis lane; G, immunoglobulin G (IgG) antiserum lane; A, immunoglobulin A (IgA) antiserum lane; immunoglobulin M (IgM) antiserum lane; k, free kappa light chain antiserum lane; L, free lambda light chain antiserum lane.      

Past Medical History

Hypertension, community-acquired pneumonia, and hip repair with insertion of bilateral intramedullary nails. The patient had no known drug allergies and was currently taking: Bactrim DS (1 tablet po bid every Saturday and Sunday); Acyclovir (400 mg bid); Protonix (40 mg qd); Norvasc (5 mg qd); potassium chloride (20 mEq qd).Family/Social History

The patient's father died of colon cancer; she had no family history of any hematologic malignancies. She denied use of alcohol, tobacco, or drugs of abuse.Physical Examination

The patient appeared comfortable and in no acute distress. No abnormal findings were noted on complete physical examination.Principal Laboratory Findings, Post-Stem Cell Transplant

Table 1  Printer- Friendly Email This

Lab Med.  2005;36(8):482-485.  ©2005 American Society for Clinical Pathology
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