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BackgroundAlthough therapeutic hypothermia improves the outcome of neonatal hypoxic-ischemic encephalopathy (HIE), its efficacy is still limited. This preliminary study evaluates the safety and feasibility of the combination therapy with erythropoietin (Epo), magnesium sulfate and hypothermia in neonates with HIE.MethodsA combination therapy with Epo (300 U/kg every other day for 2 weeks), magnesium sulfate (250 mg/kg for 3 days) and hypothermia was started within 6 h of birth in neonates who met the institutional criteria for hypothermia therapy. All patients received continuous infusion of dopamine. Vital signs and adverse events were recorded during the therapy. Short-term and long-term developmental outcomes were also evaluated.ResultsNine patients were included in the study. The mean age at first intervention was 3.9 h (SD, 0.5). Death, serious adverse events or changes in vital signs likely due to intervention were not observed during hospital care. All nine patients completed the therapy. At the time of hospital discharge, eight patients had established oral feeding and did not require ventilation support. Two patients had abnormal MRI findings. At 18 months of age, eight patients received a follow-up evaluation, and three of them showed signs of severe neurodevelopmental disability.ConclusionThe combination therapy with 300 U/kg Epo every other day for 2 weeks, 250 mg/kg magnesium sulphate for 3 days and therapeutic hypothermia is feasible in newborn patients with HIE.Trial registrationISRCTN33604417retrospectively registered on 14 September 2018.

A 62-year-old man with neurofibromatosis type 1 presented with slowly progressive weakness in the right leg and impaired bladder function. Physical examination revealed bilateral brisk deep tendon reflexes and extensor plantar reflex. He had dozens of café au lait spots and cutaneous neurofibromas. His 12-year-old granddaughter presented with toe walking and weakness in the right hand. She showed several (>6) café au lait spots and axillary freckling, but no cutaneous neurofibromas or Lisch nodules. Physical examination of his asymptomatic 39-year-old son also revealed brisk deep tendon reflexes. He had six café au lait spots but no other cutaneous signs of neurofibromatosis. An ocular examination revealed Lisch nodules. All of them satisfied the NIH diagnostic criteria for neurofibromatosis type 1. In all three family members, spine MRI showed an intradural extramedullary tumour compressing the spinal cord at C1eC2 (figure 1), which necessitated neurosurgical removal. Upon histological evaluation, the tumours were found to be neurofibroma. In the proband, MRI of the cervical spine showed this single tumour alone, and other spinal regions were not examined. In his granddaughter and son, a spine MRI revealed multiple paraspinal tumours on both sides at almost all levels of the vertebral column from C1 to the sacrum. At follow-up visits, the proband's motor impairment persisted, his son remained asymptomatic, and his granddaughter had slight neurological sequelae.Familial spinal neurofibromatosis is a variant of neurofibromatosis type 1. 1 Multiple symmetrical tumours on the nerve roots with minimal cutaneous signs of neurofibromatosis type 1 are considered typical for this entity. 1 To our knowledge, the granddaughter represents the youngest person afflicted by familial spinal neurofibromatosis with symptomatic spinal tumour. 1 2 REFERENCES 1. Upadhyaya M, Spurlock G, Kluwe L, et al. The spectrum of somatic and germline NF1 mutations in NF1 patients with spinal neurofibromas. Neurogenetics 2009;10:251e63. 2. Kluwe L, Tatagiba M, Fünsterer C, et al. NF1 mutations and clinical spectrum in patients with spinal neurofibromas. J Med Genet 2003;40:368e71.Figure 1 T2-weighted sagittal MR images of the cervical spine show an intradural extramedullary tumour (arrowheads) compressing the spinal cord at C1eC2 in each of three family members across three generations.

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The immune responsiveness in streptozotocin (SZ)-induced diabetic mice was studied using the immune responses to sheep red blood cells (SRBC) as an indicator system. In SZ-diabetic mice, the weights of such lymphoid organs as the thymus and spleen were significantly decreased with time after SZ administration, whereas the weight of liver was markedly increased. In SZ-diabetic mice, the level of delayed type hypersensitivity (DTH) to SRBC was not lower than that in normal controls in most cases, although the level of DTH was significantly depressed, on occasion, in SZ-diabetic mice. In contrast, antibody-forming activity, measured as the number of plaque-forming cells (PFC), was markedly decreased in SZ-diabetic mice. It seems that antibody production is more profoundly depressed than is DTH in SZ-diabetic mice. The transfer of normal thymus and bone marrow cells into SZ-diabetic mice caused only a partial restoration of PFC activity. When normal spleen cells were transferred into diabetic irradiated mice, proliferation of spleen cells and production of splenic PFC was greatly reduced as compared with normal irradiated mice. Treatment with insulin completely reversed such depression in the transfer system. These findings suggest that the chronic insulin-deficient diabetic state caused a depression and delay in the proliferation and differentiation of lymphoid cells.