Find information on thousands of medical conditions and prescription drugs.

Leukocyte adhesion deficiency syndrome

Leukocyte adhesion deficiency, abbreviated LAD, is a rare autosomal recessive disorder characterized by immunodeficiency resulting in recurrent infections. The disorder is often divided into two separate genotypes called type I and type II, with type II being associated with fewer infections but more developmental delay. more...

Home
Diseases
A
B
C
D
E
F
G
H
I
J
K
L
Amyotrophic lateral...
Bardet-Biedl syndrome
Labyrinthitis
Lafora disease
Landau-Kleffner syndrome
Langer-Giedion syndrome
Laryngeal papillomatosis
Laryngomalacia
Lassa fever
LCHAD deficiency
Leber optic atrophy
Ledderhose disease
Legg-Calvé-Perthes syndrome
Legionellosis
Legionnaire's disease
Leiomyoma
Leiomyosarcoma
Leishmaniasis
Lemierre's syndrome
Lennox-Gastaut syndrome
Leprechaunism
Leprophobia
Leprosy
Leptospirosis
Lesch-Nyhan syndrome
Leukemia
Leukocyte adhesion...
Leukodystrophy
Leukomalacia
Leukoplakia
LGS
Li-Fraumeni syndrome
Lichen planus
Ligyrophobia
Limb-girdle muscular...
Limnophobia
Linonophobia
Lipodystrophy
Lipoid congenital adrenal...
Liposarcoma
Lissencephaly
Lissencephaly syndrome...
Listeriosis
Liticaphobia
Liver cirrhosis
Lobster hand
Locked-In syndrome
Loiasis
Long QT Syndrome
Long QT syndrome type 1
Long QT syndrome type 2
Long QT syndrome type 3
LSA
Lung cancer
Lupus erythematosus
Lyell's syndrome
Lygophobia
Lyme disease
Lymphangioleiomyomatosis
Lymphedema
Lymphoma
Lymphosarcoma
Lysinuric protein...
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

Epidemiology

LAD is a rare disease; its estimated prevalence is 1 in 100,000 births. There is no described racial or ethnic predilection.

Clinical manifestations

LAD was first recognized as a distinct clinical entity in the 1970s. The classic descriptions of LAD included recurrent bacterial infections, defects in neutrophil adhesion, and a delay in umbilical cord sloughing. The defects in adhesion result in poor neutrophil chemotaxis and phagocytosis.

Patients with LAD suffer from bacterial infections beginning in the neonatal period. Infections such as omphalitis, pneumonia, gingivitis, abcesses, and peritonitis are common and often life-threatening due to the infant's inability to properly destroy the invading pathogens.

Molecular defect

The inherited molecular defect in patients with LAD is a deficiency of the beta-2 integrin subunit of the leukocyte cell adhesion molecule, which is found on chromosome 21. This subunit is involved in making three other proteins (LFA-1, CR3, and Mac-1) This basically means that a gene which creates a protein does not function properly. This results in the lack of important molecules which help neutrophils make their way from the blood stream into the infected areas of the body (ie the lungs in pneumonia). Those neutrophils which do manage to make it to the infected areas have a difficult time "swallowing" the bacteria leading to infection (this is known as impaired phagocytosis). Therefore, the infection is allowed to spread unimpeded and cause serious injury to important tissue.

Diagnosis

Typically diagnosis is made after several preliminary tests of immune function are made, including basic evaluation of the humoral immune system and the cell-mediated immune system. Specific diagnosis is made through monoclonal antibody testing for CR3, one of the three complete proteins which fail to form properly as a result of beta-2 integrin subunit deficiency.

Treatment

Once the diagnosis of LAD is made, bone marrow transplantation is the current standard of care. However, some progress has been made in gene therapy, an active area of research.

Read more at Wikipedia.org


[List your site here Free!]



Complement deficiencies
Complement deficiencies are a group of disorders in which there is a reduced level of specific proteins, complement, involved in proper immune functioning.
Recruited Inflammatory Cells Mediate Endotoxin-induced Lung Maturation in Preterm Fetal Lambs
Rationale: Chorioamnionitis is paradoxically associated with a decreased incidence of respiratory distress syndrome in preterm infants. In preterm lambs, ...
New anticoagulant drugs
This article about new antleoagulant drugs is pan of the seventh American College of Chest Physicians Conference on Antithrombotic and Thrumbolytic Therapy: Evidence-Based Guidelines. The limitatio
NO and Neutrophils during Sepsis: NO says "Yes" to Sequestration but "No" to Migration
The biology of nitric oxide (NO) has become a very interesting story in inflammatory pulmonary disease, with many complexities. Its role in the initiation ...
Neutrophils and the pathogenesis of COPD - chronic obstructive pulmonary disease
Key words: [[alpha].sub.1]-antitrypsin deficiency; chemoattractant; COPD; elastase; emphysema; interleukin-8; leukotriene [B.sub.4]; neutrophil Abbreviations: ...
Protein C Levels in Severe Sepsis
In this issue of CHEST (see page 915), Yan and colleagues demonstrate that 90% of a population of 70 patients who met the standard clinical criteria ...
Malnutrition impairs CD11b/CD18 expression on circulating polymorphonuclear neutrophils and subsequent exudation into inflammatory sites in the early phase
ABSTRACT. Background: The effects of malnutrition on polymorphonuclear neutrophil (PMN) exudation are not well understood. The purpose of this study was ...
nitric oxide connection: Hyperbaric oxygen therapy, becaplermin, and diabetic ulcer management, The
ABSTRACT Clinical experience with adjunctive hyperbaric oxygen therapy in the treatment of diabetic ulcers has shown that wound hyperoxia increases wound ...

Home Contact Resources Exchange Links ebay