Introduction: LEUKOCYTE ADHESION DEFICIENCY (LAD) is a rare functional LEUKOCYTE disorder, which is divided into two separate types: LAD-1 and LAD-2. LAD-1 results from lack of b2 integrin molecules (CD11 & CD18) on the LEUKOCYTE cell surface. These molecules are essential for LEUKOCYTE ADHESION to endothelial cells and chemotaxis.The present case report is about a 42-month-old girl with recurrent otitis, pneumonia and gingivitis. On physical examination, patient was pale and malnourished. Multiple desquamated erythematous plagues were found on her body and extremities. Blood investigations revealed persistent leukocytosis with normal serum Immunoglobulin profile and complement. The diagnosis of LAD1 was made based on Flow cytometry finding; showing decreased in CD11 and CD18 markers of PMN a.Conclusion: When a patient has persistent leukocytosis and recurrent infections, investigation for the primary immune DEFICIENCY, specially LEUKOCYTE ADHESION DEFICIENCY must be considered.

Objective: LEUKOCYTE ADHESION DEFICIENCY (LAD) disorders, LAD-1 and LAD-2 are rare disease of LEUKOCYTE function. LAD-1 results from a lock of LEUKOCYTE cell surface expression of β2 integrin molecules (CD11 & CD18) that are essential for LEUKOCYTE ADHESION to endothelial cells and chemo taxis. Case Presentationt: Our patient a 23-month-old male infant was brought with recurrent skin infections. On examination, the infant was pale with multiple scars in his body. Investigations revealed persisting leukocytosis, serum Immunoglobulin profile and complement were normal. Flow cytometry showed decreased in CD11 and CD18 marker of PMN a diagnosis of LAD1 was made.Conclusion: The accompaniment of persistent leukocytosis and recurrent skin infections will guide us to the primary immune DEFICIENCY specially LEUKOCYTE ADHESION DEFICIENCY.

Introduction: LEUKOCYTE ADHESION DEFICIENCY syndrome (LADS) is a rare inherited immunoDEFICIENCY in which the function of LEUKOCYTEs, particularly the phagocytes are disturbed. Case report: In the present sudy, we report three cases with LADS (2 male infants and 1 female child) who had a history of delay in separation of umbilical cord. One infant admitted for skin infection and two patients admitted in hospital following bacterial and fungal infections in nose and finger.All cases had a remarkable leukocytosis, neutrophilia and decreased of CD11 and CD18. Conclusion: LADS is a congenital syndrome causes recurrent bacterial and fungal infections. Usually all patients have a history of delayed separation of umbilical cord.

Background: LEUKOCYTE ADHESION DEFICIENCY syndrome type 1 (LAD-1) is a rare autosomal recessive primary immunoDEFICIENCY disorder of neutrophil phagocytic function characterized by the DEFICIENCY of one or several surface integrins which altered ADHESION and cause recurrent infection. Some reports shows association of LADS with malignancy.Case presentation: We describe a case of a 7-day-old boy who presented with an omphalitis, sepsis, icter and erythematus rashes and characteristic history of recurrent infections, marked leukocytosis and delayed separation of umbilical cord. The diagnosis is based primarily on flow cytometric analysis of neutrophils for the surface expression of CD11, CD18 and CD15s. He developed lymphadenopathy and abdominal mass with diagnosis Nonhodgkins lymphoma (NHL). Our patient represents the first clinically and histopathologically documented association between LADS and NHL. Data could support to the role of tumor genesis of b2 integrins in the human.

Progressive periodontal disease in LEUKOCYTE ADHESION DEFICIENCY children may lead to severe systemic infections and even death. A five-year-old Iranian male child with LEUKOCYTE ADHESION DEFICIENCY I was first seen in December 2005 at the Pediatric Dentistry Department of Shahid Beheshti Medical University and diagnosed with periodontitis as a manifestation of systemic disease. The treatment approach was based on assessing plaque index, oral prophylaxis, periodic supra and subgingival scaling, in addition to strict oral hygiene instruction with a chlorhexidine prescription and restoration of decayed teeth. The patient attended two dental visits at a one month interval. At the second session, an improvement was seen in the plaque index. Gingival inflammation and bleeding were decreased. Unfortunately he did not regularly attend treatment sessions and at the following examination, progression of periodontitis and bone destruction occurred. The present case emphasizes the need for cooperation between medical and dental professionals, parents and the pediatric patient in order to achieve treatment goals in controlling oral infection in these patients.

LEUKOCYTE ADHESION DEFICIENCY type 1 (LAD 1) is an autosomal recessive hereditary disorder resulting from DEFICIENCY of CD18, characterized by recurrent bacterial infections. We report two consanguineous patients with LEUKOCYTE ADHESION DEFICIENCY type 1(LAD1). These two infant boy patients were referred to us, within a short period of time, with the complaints of recurrent infections at the age of 38 and 75 days -old, respectively. Parents of two patients were first cousins and their grandmothers also were first cousins. The history of delayed umbilical cord separation was shown in both patients. Patient 1 had history of omphalitis, conjunctivitis, skin lesion of groin area and abscess formation of vaccination site, and had infective wound of eye-lid at the last admission. Patient 2 had history of omphalitis and soft tissue infection of right wrist at the last admission. Laboratory findings showed marked leukocytosis and low CD18 levels (6.6% in Patient 1 and 2.4 % in Patient 2). In Patient 1 recurrent infections were treated with antibiotic regimens and received bone marrow transplantation but Patient 2 died because of septicemia, generalized edema, ascites and progression to acute renal failure at 4 months of age. Due to considerable rate of consanguineous marriages in parents of LEUKOCYTE ADHESION DEFICIENCY patients, sequence analysis especially for prenatal diagnosis in subsequent pregnancies and genetic counseling is recommended.

The hallmarks of LEUKOCYTE ADHESION DEFICIENCY (LAD) are defects in the LEUKOCYTE ADHESION process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of LEUKOCYTEs from the blood vessels to sites of infection, which requires ADHESION of LEUKOCYTEs to the endothelium. Over last 20 years, three distinctive defects in the LEUKOCYTE ADHESION cascade, involving several precise ordered steps such as rolling, integrin activation and firm ADHESION of the LEUKOCYTEs have been described. While LAD I and II are clearly autosomal recessive disorders, the mode of inheritance of LAD III is still not clear. LAD I is due to structural defects in the integrin molecule, preventing firm ADHESION to occur. We present a case of a male neonate referred with continuation of leukemoid reaction and multiple non healing ulcers, gingivitis and periodontitis in spite of good antibiotic therapy.

LEUKOCYTE ADHESION DEFICIENCY type 1 (LAD-1) is a rare autosomal recessive immunoDEFICIENCY disorder, characterized by recurrent bacterial and fungal infections without pus formation. Herein, we report a case of LAD-1 that developed into gastrointestinal cytomegalovirus (CMV) disease and manifested with persistent abdominal pain and bloody diarrhea. Although the presence of concurrent gastrointestinal CMV infection with LAD-1 is a rare condition, this case highlights the need for further research to evaluate the complex mechanisms between LAD-1 and CMV occurrence.

Objective: LEUKOCYTE ADHESION DEFICIENCY (LAD) is a rare, autosomal recessive inherited disorder; LAD-I which is the most common type, occurs due to mutations in the CD18 gene. This mutation down-regulates the expression of ß2 integrin LEUKOCYTE cell surface molecules, which are necessary for the ADHESION of LEUKOCYTEs to endothelial cells, trans endothelial migration, and chemotaxis. The major symptoms are recurrent severe bacterial infections without pus formation, recurrent or progressive necrotizing soft tissue infections, marked leukocytosis and severe progressive periodontitis accompanied by alveolar bone loss, periodontal pockets, and partial or total early loss of primary and permanent teeth.Cases: Herein, we report a case of moderate LAD-I in a nine year-old boy with severe alveolar bone loss and aggressive periodontitis. For several years, approximately every month, the patient was followed up to evaluate the status of primary and permanent dentition. During these visits, scaling and root planing were performed and severely mobile teeth were extracted. The patient is now under regular follow up.Conclusion: This case confirms the need for interactions between the medical groups to identify and manage medically compromised children with rare diseases. It is important to include LAD in the differential diagnoses of children presenting with periodontal disease. Early correct diagnosis of LAD has various benefits for patients.