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Goodpasture's syndrome

Goodpasture’s syndrome (also known as Goodpasture’s disease and anti-glomerular basement membrane disease or anti-GBM disease) was first described by Ernest Goodpasture in 1919. It is an rare condition characterised by rapid destruction of the kidneys and haemorrhaging of the lungs. Although many diseases can present with these symptoms, the name Goodpasture’s syndrome is usually reserved for the autoimmune disease produced when the patient’s immune system attacks cells presenting the Goodpasture antigen, which are found in the kidney and lung, causing damage to these organs. more...

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Signs and symptoms

Most patients present with both lung and kidney disease, however, some patients present with one of these diseases alone. The first lung symptoms usually develop days to months before kidney damage is evident.

Lung disease

Lung damage may cause nothing more serious than a dry cough and minor breathlessness and such mild symptoms may last for many years before more severe ones develop. At its most serious, however, lung damage may cause severe impairment of oxygenation so that intensive care is required. Deterioration between the two extremes may occur very rapidly, often at the same time as rapid deterioration in the kidney. The patient often does not seek medical attention until he or she begins coughing up blood. The patient may be anaemic due to loss of blood through lung haemorrhaging over a long period. In Goodpasture’s syndrome, unlike many other conditions that cause similar symptoms, lung haemorrhaging most often occurs in smokers and those with damage from lung infection or exposure to fumes.

Kidney disease

The kidney disease mostly affects the glomeruli causing a form of nephritis. It is usually not detected until a rapid advance of the disease occurs so that kidney function can be completely lost in a matter of days. Blood leaks into the urine causing haematuria, the volume urinated decreases and urea and other products usually excreted by the kidney are retained and build up in the blood. This is acute renal failure. Renal failure does not cause symptoms until more than 80% of kidney function has been lost. Symptoms include loss of appetite and sickness at first and then, when the damage is more advanced, breathlessness, high blood pressure and oedema (swelling caused by fluid retention).


Because of the vagueness of early symptoms and rapid progression of the disease, diagnosis is often not reached until very late in the course of the disease. Kidney biopsy is often the fastest way to secure the diagnosis and gain information about the extent of the disease and likely effect of treatment. Tests for anti-GBM antibodies may also be useful, combined with tests for antibodies to neutrophil cytoplasmic antigens, which are also directed against the patient’s own proteins.


As with many autoimmune conditions, the precise cause of Goodpasture’s Syndrome is not yet known. It is believed to be a type II hypersensitivity reaction to Goodpasture’s antigens on the cells of the glomeruli of the kidneys and the pulmonary alveoli, whereby the immune system wrongly recognises these cells as foreign and attacks and destroys them, as it would an invading pathogen.


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Black Hawk, Please Come Down: Reflections on a Hospital's Struggle to Survive in the Wake of Hurricane Katrina
From American Journal of Respiratory and Critical Care Medicine, 11/15/05 by deBoisblanc, Bennett P

On Monday, August 29, 2005, Hurricane Katrina, a category 4 storm, roared ashore along the Louisiana-Mississippi border packing 145-mile-per-hour winds. Katrina's 28-ft storm surge flattened coastline residences from Louisiana to the panhandle of Florida, killing over 1,000 persons. In New Orleans, hurricane protection levees subjected to unprecedented stress failed, and the city was flooded with up to 15 ft of water. Recovery will require a monumental effort from all sectors of society. Put in perspective. Hurricane Katrina will be the costliest natural disaster in U.S. history by a factor of five.

Despite 3 days of advance warning that Katrina would make landfall along the north-central Gulf Coast, hospital, city, state, and federal planners found themselves totally unprepared to deal with Katrina's early aftermath. That disaster officials were caught off guard seems particularly surprising when one considers that this worst-case scenario had been predicted for over 50 years-after all. New Orleans is a city largely below sea level and completely surrounded by water. As recently as June 2002, the New Orleans Times Picayune had published a five-part series on the vulnerability of the city to a major hurricane.

Although disaster drills do provide some insight into the weaknesses of a disaster plan, disaster planning remains largely theoretical. Under most disaster scenarios, hospitals become places of refuge for the injured and sick. What happens when a major hospital itself is incapacitated and must be evacuated? What becomes of its sickest and most vulnerable patients, those in intensive care units (ICUs)? Accounts of the remarkable evacuation of Charity Hospital's ICUs in the wake of Katrina have recently been chronicled in both primetime television and major print news media, and a brief report of this experience was posted on the American Thoracic Society's (ATS) website ( and in the ATS News (1). These accounts highlight the truly heroic performances of countless physicians, nurses, respiratory therapists, support staff, and even family members who unselfishly put themselves in harm's way to care for Charity's most vulnerable patients at a time when Charity Hospital had fallen off the government's disaster-management radar. Surely mistakes were made, and valuable lessons learned.

Charity Hospital is a 500-bed Level 1 trauma center in downtown New Orleans. On Sunday morning, August 28, emergency activation teams, including a staff critical care physician, residents, interns, nurses, and respiratory therapists, were assigned to each ICU area to support patients who could not be discharged prior to Katrina's expected landfall. At the peak of the storm, electrical power was lost, windows blew out, light fixtures fell from ceilings, and several floors of the hospital flooded, but by Monday night spirits were high in anticipation of beginning the recovery effort the next day. Serious problems first came to light early Tuesday morning when Charity's emergency electrical generators, located on the first floor, were submerged by rising floodwaters. To complicate matters, that very morning, additional mechanically ventilated patients were evacuated to Charity from a flooded nursing home.

The implications of total electrical power failure in a modern ICU are difficult to comprehend. From Tuesday morning on there was no reliable electrical power for life-support systems, such as mechanical ventilators, suction machines, bedside monitors, intravenous fluid pumps, and dialysis machines. There was no air conditioning. Batteries on transport monitors and infusion pumps worked for a few hours, but then could not be recharged. There was no radiology or laboratory capability.

Patients who required positive-pressure ventilation were at greatest risk in our ICU. Gas-driven transport ventilators were able to be used for several lower acuity patients, but proved to be inadequate for patients who required high positive end-expiratory pressure or high minute ventilation. For these higher acuity patients, nurses, respiratory therapists, residents, and even family members took turns hand ventilating with bag-valve devices in total darkness. As if sensing that the evolving crisis in the ICU might be a cause for patient anxiety, caregivers rapidly assumed ownership of individual patients. One respiratory therapist, a single mother still mourning the death of her only child, hand-ventilated a 23-year-old man with Goodpasture's syndrome for 6 hours before she allowed others to relieve her.

Water pressure was lost soon after the electrical power failure. Charity found itself not only caring for 340 patients but also providing refuge for approximately 1,000 support staff and their families. Toilets soon overflowed with liquid and solid waste, driving some refugees to use garbage cans or stairwells. Within 24 hours, the hospital became a sanitation nightmare. Waterless hand cleanser, traded like cigarettes in a prison, was used for more than just hand hygiene. The basement morgue, also an early victim of the flooding, could not be used to store the deceased. Patients who died were either moved to a hallway or left in their ICU beds. The water surrounding the hospital very quickly filled with sewage, dead animals, and medical waste.

The first casualty of a disaster is always communication. Cell phone networks began to fail and most hospital phone lines were dead. There was no television, no Internet, and no e-mail. Morale hit a low point when portable radio news reports from the governor's office informed us that we had already been evacuated. Some minor problems with communication infrastructure were expected. But as hundreds of helicopters buzzed overhead, we were surprised to find ourselves unable to communicate with local police, the National Guard, the Federal Emergency Management Agency, or even between and within buildings of our own institution.

Peering from Charity's rooftop, we could see thousands of persons stranded in buildings and on top of bridges. These homeless, hungry, thirsty, and desperate persons posed an immense security risk. The heat, humidity, and darkness of the hospital's interior became unbearable for some, whereas others were stricken with fears of personal safety or of being forgotten. Tempers flared and work quality suffered. When Wednesday morning arrived without any sign of outside rescue, it became clear that further waiting was not a tenable strategy.

On Wednesday evening, we began an air evacuation of our ICUs that stretched into Friday afternoon. After taping brief medical records to each patient's forearm, the patients were sedated and then ferried through the floodwaters to a makeshift ICU on the rooftop of Tulane Hospital's parking garage. There, two dozen residents, nurses, and respiratory therapists cared for 50 of our sickest patients over a period of 36 hours, using flashlights, bag-valve masks, and bottled drinking water for irrigating endotracheal tubes.

In a battlefield-like environment, one might have been tempted to predict that expressions of compassion would be rare. Somewhat surprisingly, just the opposite occurred. Armed with little technology, caregivers gave with their hearts and their hands. Everywhere on that rooftop, respiratory therapists, nurses, and residents sat on the bare concrete holding, petting, and whispering to patients frightened by the roar of helicopters overhead. In one particularly profound scene, a 91-year-old woman with a hip fracture stroked the forearm of a resident who in turn ventilated and consoled another patient with respiratory failure (Figure 1). We quickly ran out of sedatives and analgesics, but unexpectedly, anxiety reactions were rare. It was intriguing that the crisis had the effect of breaking down traditional divisions of labor, creating a community of shared caregiving. It was particularly moving to watch senior faculty jump into the rotations of suctioning and hand-ventilating.

Leadership is often borne under duress, and from every corner of our hospital, I witnessed young physicians, nurses, and allied health professionals rising to meet unique challenges. As civilians, this was as close as many of us will ever get to a wartime experience. The resourcefulness of these young professionals will forever deeply humble me. Their heroism seems even more impressive when one considers that it did not come at the cost of compassion. In those few days, I witnessed so many profoundly moving acts of kindness that I have trouble describing them without becoming teary-eyed. Since Katrina, I have received hundreds of e-mails from friends and colleagues around the country offering succor. We, the employees of just one of the many hospitals ravaged by Katrina, are appreciative of their concern, but let all be assured that our experience was a triumphant one. Instead, let us all turn our focus to our many brothers and sisters who have not fared as well.


1. deBoisblanc B. Hurricane Katrina: one ATS member's ordeal. ATS News 2005;31(10), October. pp 3-7.

Bennett P. deBoisblanc

Louisiana State University Health Sciences Center, New Orleans, Louisiana

Correspondence and requests for reprints should be addressed to Ben deBoisblanc, M.D., 1901 Perdido Street, Suite 3205, New Orleans, LA 70112 [temporary address: 3918 Villanova Street, Houston, TX 77005]. E-mail:

Am J Respir Crit Care Med Vol 172. pp 1239-1240, 2005

DOI: 10.1164/rccm.2509004

Internet address:

Copyright American Thoracic Society Nov 15, 2005
Provided by ProQuest Information and Learning Company. All rights Reserved

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