Aviva Mimouni-Bloch1, Hadassa Goldberg-Stern2, Rachel Strausberg3, Amichai Brezner4, Eli Heyman5, Dov Inbar6, Sara Kivity2, Alex Zvulunov7, Ignacio Sztarkier7, Rami Fogelman8, Aviva Fattal-Valevski9. 1. The Pediatric Neurology and Developmental Unit, Loewenstein Rehabilitation Hospital, Raanana, Israel; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. Electronic address: aviva100@bezeqint.net. 2. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Epilepsy Service, Schneider Children's Medical Center, Petach Tikva, Israel. 3. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Pediatric Neurology Unit, Schneider Children's Medical Center, Petach Tikva, Israel. 4. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Rehabilitation, Sheba Medical Center, Ramat Gan, Israel. 5. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Department of Child Neurology and Rehabilitation, Assaf Harofeh Medical Center, Zerifin, Israel. 6. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Child Development Center, Schneider Children's Medical Center, Petach Tikva, Israel. 7. Soroka Medical Center, Beer-Sheva, Israel; Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer-Sheva, Israel. 8. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Pediatric Cardiology Unit, Schneider Children's Medical Center, Petach Tikva, Israel. 9. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; Pediatric Neurology Unit, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
Abstract
BACKGROUND: In 2003, several hundred Israeli infants risked thiamine deficiency after being fed a soy-based formula deficient in thiamine. Approximately 20 patients were seriously affected, and three of them died. We report the clinical presentation of acute encephalopathy in 11 children and the long-term sequelae of eight children who initially survived. PATIENTS: In the acute phase, six had bulbar signs, five had ophthalmologic signs and two had phrenic neuropathy. Three of the five patients with cardiac involvement had cardiomyopathy and died in the acute phase. One patient presented with a complete atrioventricular block. RESULTS: In the long-term, one patient, who was in a chronic vegetative state, died after 6 years. Seven children exhibited mental retardation and motor abnormalities, six developed severe epilepsy, two early kyphoscoliosis, and one patient remained with a complete atrioventricular block. CONCLUSIONS: Infants who survive severe infantile thiamine deficiency have serious residual motor and cognitive sequelae as well as epilepsy.
BACKGROUND: In 2003, several hundred Israeli infants risked thiamine deficiency after being fed a soy-based formula deficient in thiamine. Approximately 20 patients were seriously affected, and three of them died. We report the clinical presentation of acute encephalopathy in 11 children and the long-term sequelae of eight children who initially survived. PATIENTS: In the acute phase, six had bulbar signs, five had ophthalmologic signs and two had phrenic neuropathy. Three of the five patients with cardiac involvement had cardiomyopathy and died in the acute phase. One patient presented with a complete atrioventricular block. RESULTS: In the long-term, one patient, who was in a chronic vegetative state, died after 6 years. Seven children exhibited mental retardation and motor abnormalities, six developed severe epilepsy, two early kyphoscoliosis, and one patient remained with a complete atrioventricular block. CONCLUSIONS:Infants who survive severe infantile thiamine deficiency have serious residual motor and cognitive sequelae as well as epilepsy.
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