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Biomedical subjects

Ben Zion Garty

Publications and source records attributed to Ben Zion Garty.

11 recordsLinked to original sources
PubMed2006

Successful treatment of Wolman disease by unrelated umbilical cord blood transplantation.

Wolman disease is a rapidly fatal lysosomal storage disease caused by the complete absence of lysosomal acid lipase activity. We report the cure of an infant with Wolman disease following transplantation of unrelated HLA-mismatched umbilical cord blood-derived stem cells. Umbilical cord blood was chosen as the stem-cell source because of its immediate availability and reduced tendency to cause graft-versus-host disease. The transplantation resulted in restoration of normal acid lipase levels before the onset of permanent end-organ damage. Four years after transplantation, the patient is thriving and has normal levels of acid lipase in peripheral blood cells. To our knowledge, this is the first report of a successful unrelated cord blood transplant in a patient with Wolman disease. Umbilical cord stem cells transplantation can restore acid lipase levels in Wolman disease, and if performed early, can cure the disease.

Brain↗
PubMed2006

Facial paralysis as a presenting symptom of leukemia.

Facial paralysis may occur as a complication of central nervous system leukemias in children, but it is rarely a presenting symptom. This report describes an 8-month-old child who presented with peripheral facial palsy, failure to thrive, anemia, and otitis media. Antibiotic and steroid treatment led to an improvement in the clinical condition, but not the paralysis. At readmission 3 weeks later, physical examination revealed bluish, firm, palpable masses on the scalp and facial areas, and laboratory and imaging studies confirmed the diagnosis of acute myeloid leukemia. This case should alert physicians to consider hematologic malignancies in children with facial paralysis.

Acute Disease↗
PubMed2004

Recurrent parotitis in selective IgA deficiency.

Recurrent parotitis is an uncommon condition in children. In most cases the etiology is unknown, although the disease is occasionally associated with viral infections, autoimmune disorders and immunodeficiency. We describe, for the first time, a child with recurrent parotitis and isolated immunoglobulin A (IgA) deficiency, without autoimmune disease. As IgA is the main immunoglobulin secreted into the mucosal surfaces, including that of the respiratory and gastrointestinal tracts, and into the saliva, the lack of IgA may be involved in the pathogenesis of recurrent parotitis. We recommend that IgA and other immunoglobulins be tested in all cases of recurrent parotitis.

Child↗
PubMed2003

Heat stroke in familial dysautonomia.

A 14-month-old female with familial dysautonomia was referred to the pediatric department with high fever (41.6 degrees C), watery diarrhea, and vomiting. A few hours later, signs of encephalopathy appeared. Laboratory tests revealed elevated levels of lactate dehydrogenase (3500 U/L), aspartate aminotransferase (640 U/L), alanine aminotransferase (320 U/L), and creatine kinase (28,420 U/L). The diagnosis was heat stroke. Impaired autonomic nervous system function may be another risk factor for the development of heat stroke in young children.

Autonomic Nervous System↗
PubMed2002

Large T-cell lymphoma in a 13-year-old girl with hyperimmunoglobulinemia E syndrome.

Large T-cell lymphoma was diagnosed in a 13-year-old girl with hyperimmunoglobulinemia E syndrome (HIES). Her past medical history included severe dermatitis, recurrent pneumonia, urinary tract infections, mucocutaneous herpetic infections, fungal skin infections, and staphylococcal sepsis. The diagnosis of HIES, based on the clinical features and a serum IgE level of >20000 IU/ml, was established when the girl was 6 years old. This is the eighth case of lymphoma in a patient with HIES reported in the English-language medical literature. HIES has not usually been considered a predisposing factor for malignancy, but in view of the rarity of HIES and the young age of the patients, this association seems to be more than coincidental. A link between lymphoma and Epstein-Barr virus (EBV) infection in patients with HIES has been proposed. Serological tests for EBV in our patient were positive 6 years prior to the development of the lymphoma; however, examination for EBV DNA in the lymph node biopsy failed to detect EBV in the tumor.

Adolescent↗
PubMed2002

Topical treatment of tinea capitis in a neonate.

Tinea capitis is the most common fungal skin infection in children. Given that this infection invades the hair shaft and the pilosebaceous unit, systemic antifungal therapy is the gold standard of treatment. Despite the neonate's increased susceptibility to infections, tinea capitis is rare in this population. We present the case of a 16-day-old infant with tinea capitis caused by Microsporum canis and effectively treated with topical bifonazole 1%.

Administration, Topical↗
PubMedDate not supplied

Acute hemorrhagic edema of infancy associated with herpes simplex type 1 stomatitis.

Acute hemorrhagic edema of infancy is a benign leukocytoclastic vasculitis occurring in children younger than 2 years. The etiology is unknown. Viral or bacterial infections, immunizations, and the use of several medications, mainly antibiotics, may be involved in the pathogenesis. We report the first instance of this disease associated with herpesvirus type 1 stomatitis.

Acyclovir↗