Edema and Related Medical Conditions

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Tuesday, February 27, 2007

Pulmonary oedema - A life threatening disease.

Pulmonary oedema - A life threatening disease.

Eur J Cardiovasc Nurs. 2007 Feb 23

Ekman I,
Ekstrand L,
Schaufelberger M.
Institute of Health and Care Sciences, The Sahlgrenska Academy at Goteborg University, Goteborg, Sweden.

AIM: The aim was to describe the health history of patients after pulmonary oedema and investigate how they perceive their condition and treatment.

METHODS: In part one of the study medical records of patients treated for acute pulmonary oedema (n=44) were reviewed regarding social status, health history, medication and cause of the pulmonary oedema. In part two, interviews were performed focusing on the patients' conceptions of the illness, current situation and effects of pulmonary oedema on daily life.

RESULTS: One-year mortality was 65% and all but 3 patients had a previous heart diagnosis. Analyses of the interviews yielded five categories: A suffocating feeling; trust in care providers; medication - an annoyance but also a saviour; dealing with existential issues alone or with relatives; concurrent diseases affecting daily life.

CONCLUSION: Patients' who experience a pulmonary oedema have several heart-related conditions and a very poor prognosis. Experiencing pulmonary oedema is an anxiety-provoking situation and patients should be regularly and carefully monitored.

PMID: 17321798 [
PubMed - as supplied by publisher]

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Tuesday, February 20, 2007

Hypogammaglobulinemia with facial edema.

Hypogammaglobulinemia with facial edema.

Adina Kay Knight*, Lloyd Mayer, Andrew G. Franks Jr., Charlotte Cunningham-Rundles
Funding: The authors received no specific funding for this article.
Competing Interests: Adina Kay Knight has received travel grants to attend meetings of the American College of Allergy, Asthma, and Immunology, the American Academy of Allergy, Asthma, and Immunology, the Clinical Immunology Society, the International Congress of Mucosal Immunology, and the Federation of Clinical Immunology Societies. Two years of fellowship were supported by the American Academy of Allergy, Asthma, and Immunology Clinical Fellowship Award. Charlotte Cunningham-Rundles has received research grants from the United States National Institutes of Health and USIDNet, an offshoot of the Immune Deficiency Foundation. She is a member of the US Food and Drug Administration Bodd Safety Advisory Board and National Institutes of Health review committees and of the Immune Deficiency Foundation and the Jeffrey Modell Foundation. LM and AGF declare that they have no competing interests.
Citation: Knight AK, Mayer L, Franks Jr. AG, Cunningham-Rundles C (2006) Hypogammaglobulinemia with Facial Edema. PLoS Med 3(12): e475


Published: December 26, 2006
Copyright: © 2006 Knight et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Abbreviations: IgA, immunoglobulin A; IgG, immunoglobulin G; IgM, immunoglobulin M; CVID, common variable immunodeficiency
Adina Kay Knight, Lloyd Mayer, and Charlotte Cunningham-Rundles are in the Department of Clinical Immunology, Mount Sinai School of Medicine, New York, New York, United States of America. Andrew G. Franks, Jr. is in the Department of Dermatology, New York University School of Medicine, New York, New York, United States of America.

*To whom correspondence should be addressed: Adina.Knight@mssm.edu

Description of Case

A 35-year-old man came to the office with right-sided facial swelling, which he had noted over the last two years. The swelling was worse in the mornings and decreased through the day; however there were no symptom-free days. He had not experienced fevers, chills, or changes in his vision. He had experienced multiple episodes of lower extremity cellulitis, left more frequent than right leg, beginning in childhood. Although most instances were treated with oral antibiotics, he required hospitalization for intravenous antibiotics at least four times. Previous evaluation had confirmed lymphedema by radionucleotide clearance. In addition, he had symptoms of allergic rhinitis and two episodes of otitis media as an adult, but he had no history of pneumonia or other significant respiratory or gastrointestinal infections.

On physical examination he had no evidence of wasting or malnutrition. He had normal tonsils and no cervical lymphadenopathy; there was mild right facial swelling with induration and trace erythema from the eye to mid-cheek. The right facial skin was slightly warmer than the left, but it was non-tender to palpation. Tympanic membranes and chest examination were normal. His legs were moderately swollen, left greater than right, with a “woody” or indurated texture to the left leg; there were no rashes or other skin lesions. He had no hepatosplenomegaly.

Why Investigations are Indicated in This Patient?

Frequent infections raise the possibility of immunodeficiency. A targeted immunologic evaluation should be guided by the clinical symptoms as well as the relative frequency of known immunodeficiencies.

Immune defects in the humoral system are most common and screening can be performed with tests for serum immunoglobulin levels and titers of specific antibody. A suggestion of immunoglobulin deficiency arises if there is a low total protein on standard chemistry panels, as the immunoglobulins make up a considerable portion of serum proteins. Clinical symptoms of immunoglobulin deficiency include increased frequency or severity of sino-pulmonary and other bacterial infections.

Cellular immune deficiencies are suggested by opportunistic and viral infections. An initial step in the evaluation of these is a complete blood count (a low lymphocyte number can be missed if only total white cells are counted) followed by a lymphocyte panel enumerating CD4 and CD8 T cells as well as B cells and natural killer cells. It is important to obtain the lymphocyte evaluation with a standard complete blood count to allow for the calculation of absolute numbers of cells and not just percentages, since normal relative percentages may be preserved despite very low cell numbers. More subtle defects in T cell function may be investigated by examining lymphocyte proliferative responses to mitogens and soluble antigens.

Other rarer immune function defects, such as neutropenia or neutrophil dysfunction (e.g., chronic granulomatous disease leading to recurrent skin or organ abscesses), complement defects (systemic bacterial infections or meningitis), and IL-12 and interferon gamma axis dysfunction (mycobacterial infections), are less likely in this adult patient without clinical history or infections characteristic of these conditions.

Laboratory data included low serum immunoglobulin G (IgG) 268 mg/dl (694–1618) and immunoglobulin M (IgM) 18 mg/dl (48–271,) but normal serum immunoglobulin A (IgA) 119 mg/dl. Electrolytes, kidney, and liver function tests were normal. Serum calcium was 7.8 mg/dl (normal 8.5–10.4), total protein was 4.8 g/dl (normal 6.0–8.3), albumin was 2.9 g/dl (normal 3.7–5.1), and calculated globulin was 1.9 g/dl (normal 2.2–4.2). The urinalysis was normal with no protein detected. Further studies revealed normal white blood cell count (7.8 × 103/μl), hemoglobin, hematocrit, platelet count, and normal numbers of neutrophils, monocytes, eosinophils, and basophils. He had reduced numbers of lymphocytes 0.5 × 103/μl (normal 1.0–4.5 × 103/μl), consisting of reduced T cells 293 (750–2500/cu mm), CD4 T cells 238 (480–1700/cu mm), and CD8 T cells 40 (180–1000/cu mm), an increased CD4/CD8 ratio 6.10 (1.00–3.00), slightly low numbers of natural killer cells, 90 (135–525/cu mm), and normal numbers of B cells 85 (75–375/cu mm).

What Is the Differential Diagnosis?

Decreased immunoglobulin levels can result from reduced production or increased loss (Table 1). Primary causes of hypogammaglobulinemia are the genetic B or T cell defects. Secondary causes of reduced immunoglobulin production can be malignancy (lymphoma, thymoma, leukemia, multiple myeloma), selected medications (carbazepine [1], oxcarbazepine [2], immunosuppressive agents [3], and others), or infections such as Epstein-Barr virus, perinatally acquired HIV, or starvation. Increased nonselective loss of immunoglobulin can occur in rare states of high catabolism or with protein loss through protein-losing enteropathy (Table 2), drainage of ascites, or chylothorax (lymph fluid in the pleural space).

Lymphedema may predispose to recurrent cellulitis in affected limbs, however, given the significant hypogammaglobulinemia found here, an intrinsic defect in the immune system leading to an inability to eliminate infections should be investigated.

What Additional Laboratory Data or Investigations Would Be Helpful in Making a Diagnosis in This Patient?

The ability to make specific antibody after vaccination challenge can assist in differentiating between decreased production and increased loss of immunoglobulins. Defects in specific IgG antibody production are characteristic of primary immune defects such as X-linked agammaglobulinemia, hyper-IgM syndrome(s), and common variable immunodeficiency (CVID). Patients with hypogammaglobulinemia due to protein loss would be expected to respond normally to vaccinations in generating plasma B cells producing specific IgG antibody, though there may be lower serum immunoglobulin levels due to the general loss of proteins including IgG antibodies.

This man was first assumed to have a primary immune deficiency such as CVID, a syndrome most often associated with sino-pulmonary infections. The diagnosis of CVID is made by documenting decreased serum IgG, and IgA and/or IgM and poor specific antibody production [
4]. Significant improvement in infections is expected when IgG is replaced by immunoglobulin therapy [5]. CVID was considered a likely diagnosis, since it is often made during the second or third decade of life, however it is usually associated with a history of recurrent upper respiratory infections [6], not noted here.

However, other genetic immune defects and secondary causes, such as drug-induced, malignancy-associated hypogammaglobulinemia, or immunoglobulin loss, must be excluded. X-linked agammaglobulinemia and hyper-IgM were considered improbable due to the patient's age, mild clinical history, normal numbers of B cells, normal serum IgA, and normal tonsillar tissue.

Specific IgG antibody titers to varicella, rubella, and tetanus were found to be in a range considered to be protective, though the patient lacked sufficient antibody to measles, mumps, and 12 serotypes of pneumococci. After vaccination with measles, mumps, and rubella (MMR) and pneumococcal vaccine, he developed protective specific antibody titers to measles and mumps and nine out of 14 pneumococcal serotypes. The preserved specific antibody production in response to pneumococcal, measles, and mumps vaccination, with the protective titers of antibody to varicella, rubella, and tetanus demonstrated normal B cell function, and excluded CVID.

How Did We Investigate the Possibility of a Protein-Losing Enteropathy?

Normal B cell function, low albumin, and the intermittent history of significant lymphedema suggested the possibility of protein loss as the explanation for the hypogammaglobulinemia [
7]. Normal urinalysis and renal functions eliminated nephrotic loss, which would have been an extremely unusual etiology for this degree of hypogammaglobulinemia.

To evaluate gastrointestinal protein loss, stool alpha-1 antitrypsin was determined and found to be elevated at 625 mg/dl (normal <55>
8,9]. However, the remaining T cells are generally able to prevent opportunistic infections. Intestinal lymphangiectasia was thus consistent with the patient's reduced numbers of T cells and low levels of serum immunoglobulins.

What was the Original Diagnosis

Based on the presence of excess alpha-1 antitrypsin in the stool, the patient was diagnosed with protein-losing enteropathy. Conditions leading to protein-losing enteropathy can be congenital or acquired. Our patient has had symptoms suggestive of lymphatic dilatation since childhood and congenital lymphangiectasia was considered as a leading diagnosis. The most likely type of congenital lymphangiectasia would be hereditary lymphedema type I (Milroy disease) (Table 3). Hereditary lymphedema type I is the result of a defect in the FLT4 gene, encoding vascular endothelial growth factor receptor-3 [10]. This is an autosomal dominant mutation with variable penetrance and expression in affected members of a family. Although our patient has no known affected family members, relatively mild disease may have been overlooked. However, the degree of facial swelling our patient experienced is not generally seen in this condition.

Targeted evaluation for secondary causes of intestinal lymphangiectasia was conducted. Cardiac history and echocardiography were unremarkable in our patient, excluding cardiac disease as a secondary cause of his protein-losing enteropathy. Upper and lower endoscopies by his local gastroenterologist did not reveal any visible anomalies indicative of Crohn disease, ulcerative colitis, or other lesions, suggesting that our patient's protein loss was likely due to either a diffuse process or one occurring in the non-visualized small bowel.

How Is Lymphangiectasia Treated?

Treatment for intestinal lymphangiectasia includes treatment of the underlying pathology if a secondary cause is present (
Table 4). Our patient's history is not consistent with any of the secondary causes of protein-losing enteropathy and he has chronic lymphedema of the face and extremities as well. Congenital intestinal lymphangiectasia can be surgically resected if there is an isolated segment of affected bowel. Most often there is diffuse intestinal involvement precluding resection. Other modalities have been used, including an extremely low fat diet to reduce the lymph flow to the intestinal mucosa, dietary medium chain triglycerides, or medications including heparin and octreotide [11–14]. However, our patient's normal intestinal biopsy (no dilated lymphatics characteristic of lymphangiectasia) suggested these treatments were unlikely to be effective.

What was the Final Diagnosis

Since his history and biopsy findings were not consistent with congenital or acquired lymphangiectasia, an inflammatory etiology was considered; thus a diagnostic trial of topical oral steroids (budesonide) was given. After several weeks of use, the patient experienced a significant decrease in facial and extremity lymphedema. Laboratory evaluation also revealed a decrease in stool protein loss with decreased stool alpha-1 antitrypsin levels (Figure 1) suggesting an inflammatory component to his protein-losing enteropathy. Based upon the response to steroids, an inflammatory gastrointestinal process was considered to be the underlying cause of his protein-losing enteropathy and subsequent hypogammaglobulinemia, but no specific etiology was identified.

With good specific antibody production to vaccination challenge and no history of serious infections, replacement immunoglobulin therapy with intravenous immunoglobulin is not indicated. Continued close surveillance and a low threshold for the use of antibiotics would be prudent as he is at higher risk of recurrent cellulitis due to his lymphedema.


The differential diagnosis of hypogammaglobulinemia includes evaluation for both primary and secondary causes, since the treatment for these conditions is different. Protein-losing conditions can lead to hypogammaglobulinemia and loss of T cells, but immune function is generally preserved and immunoglobulin replacement therapy is not usually required.

Acknowledgments and References:

Plos Journals

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Thursday, February 15, 2007

Acetazolamide proves effective in pulmonary edema

Acetazolamide proves effective in pulmonary edema

Drug Controls High-Altitude Illness

NEW YORK, NY -- February 1, 2007 --

Acetazolamide, a drug used to manage fluid retention in heart failure, controlled the serious effects of pulmonary edema, the accumulation of fluid in lung tissue from high altitude, as well as improved brain oxygenation, during a randomized, double-blind and placebo-controlled study.

The results appear in the first issue for February 2007 of the American Journal of Respiratory and Critical Care Medicine.

Marc J. Poulin, PhD, DPhil, of the Departments of Physiology, Biophysics and Clinical Neuroscience at the University of Calgary in Canada and seven associates showed that acetazolamide had complex effects on ventilation, pulmonary vascular resistance and cerebral blood flow in optimizing brain oxygenation during simulated high-altitude tests on nine subjects.

The researchers concluded that the drug could be a valuable means of preventing or treating high-altitude pulmonary edema."Acute mountain sickness is one of three major high-altitude-related diseases seen in people who rapidly ascend to altitudes higher than 3,000 meters or 9,843 feet," said Dr. Poulin. "The other two diseases are high-altitude pulmonary edema and high-altitude cerebral edema. Worldwide, an increasing number of sea-level residents--altogether several million a year–visit areas higher than 2,500 meters. Many of them develop symptoms of acute mountain sickness. Among these symptoms are insomnia, headache, lightheadedness, fatigue, breathlessness, lack of appetite and nausea.

Acute mountain sickness can precede the more serious problem of high-altitude pulmonary edema."The authors found that among those susceptible to high-altitude pulmonary edema, acetazolamide decreases lung edema, facilitates the diffusion of oxygen and improves the ventilation/perfusion ratio in the lung.

They also noted that those with the lowest mountain sickness scores after being exposed to a simulated altitude of slightly over 16,000 feet showed the lowest rate of fluid retention.

By directly acting on smooth muscle cells, acetazolamide appears to cause dilation (enlargement) of vessels involved in cerebral blood flow."

Acetazolamide has been shown to prevent high-altitude pulmonary edema in rats and the agent could prove to be an efficient therapeutic means in the prevention of the disease," said Dr. Poulin. "It could be an alternative to calcium channel blockers, phosphodiesterase inhibitors or inhaled nitric oxide."

Study participants took either 250 milligrams of acetazolamide or a placebo every eight hours for three days. On the fourth test day, the researchers measured the subjects' responses to ventilation, pulmonary vascular resistance and cerebral blood flow during simulated high-altitude tests.

Each treatment period was separated by a 10-day washout to overcome any potential crossover effects from acetazolamide.

In the test group were six men and three women who were slightly over age 28. All were non-smokers with no history of cardio-respiratory disease. They were told to abstain from caffeine, alcohol or strenuous exercise throughout the test period.

SOURCE: American Thoracic Society


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Friday, February 09, 2007

Effectiveness of CT for Clinical Stratification of Occupational Lung Edema.

Effectiveness of CT for Clinical Stratification of Occupational Lung Edema.
Ind Health. 2007 Jan

Masaki Y,
Sugiyama K,
Tanaka H,
Uwabe Y,
Takayama M,
Sakai M,
Hayashi T,
Otsuka M,
Suzuki S.

Division of Environmental Medicine, National Defense Medical College Research Institute.
We treated two occupational lung diseases in different situations during military training. The purpose of this study is to investigate the availability of CT scanning for the evaluation of inhalation pulmonary edema. Two soldiers suffered severe lung edema after using a spray for the daily maintenance of their firearms. Four soldiers suffered severe dyspnea after undertaking drills in a narrow zone where numerous smoke bombs had been used. We evaluated these patients from several aspects. CT scans of the chest of spray-induced patients revealed bilateral infiltration predominantly in the upper lung fields.

The patients received steroid pulse treatment and gradually recovered. CT scans of the chest of smoke-induced patients revealed bilateral ground-glass attenuation with peripheral lung sparing. The patients gradually recovered with steroid therapy. In accordance with previous studies, CT scans of the chest in our patients demonstrated that the periphery of the lungs remained normal, except in cases of serious injury.

When differential diagnosis is required, we consider that CT scans of the chest are particularly useful; CT findings are useful in determining the severity of lung injury as well as the diagnosis of inhalation pulmonary edema.


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Tuesday, February 06, 2007

Perthes syndrome associated with bilateral optic disc edema.

Perthes syndrome associated with bilateral optic disc edema.
Can J Ophthalmol. 2006 Dec;41

Esme H,
Solak O,
Yurumez Y,
Ermis S,
Yaman M,
Fidan F.

Case report: Perthes syndrome, or traumatic asphyxia syndrome, results from a severe crush injury of the thorax. It manifests itself with facial and upper chest petechiae, subconjunctival hemorrhages, cervical cyanosis, and occasionally neurological symptoms. A patient who had been incarcerated under a block of marble presented with severe traumatic asphyxia syndrome complicated by pulmonary contusion, fractures of the right clavicle and the 8th and 9th ribs, minimal right hemothorax, liver contusion, and bilateral optic disc edema.

Comments: To our knowledge, we present the first case of Perthes syndrome with bilateral optic disc edema.

Full Text Article

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Perthes syndrome. The classical symptom triad as a rarity in trauma surgery practice


Gosling T,
Schmidt U,
Herzog T,
Tscherne H.
Unfallchirugische Klinik, Medizinischen Hochschule Hannover, Carl-Neuberg-Str. 1, 30625 Hannover.

Perthes syndrome or traumatic asphyxia is a condition characterised by subconjunctival hemorrhage, cervicofacial petechiae and cyanosis caused by severe compression of the chest. Diagnosis is made by history and clinical examination. Associated injuries such as intrathoracic or abdominal lesions can be life-threatening and must be strictly assessed. This report demonstrates an illustrative case of this rare injury.


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