The passive immunization of pregnant female rats to S-100 protein often leads to ultra-structural abnormalities in the brain glial structures of the offspring of these rats and induces signs of delayed development in the fetal brain. Additionally passive immunization of pregnant animals with certain antigens induces permanent Ag-specific changes in the immune response of their offspring. The purpose of this study was to investigate serum immunoreactiviy (SIR) to S-100 in cerebral-palsied and developmentally-delayed children as well as in their healthy parents and to evaluate its significance related to radiologic findings of brain MRI and single photon emission computed tomography (SPECT). The subjects were children with cerebral palsy and delayed development that had abnormal findings on brain MRI or Brain SPECT. SIR to S-100 protein was measured by ELISA method in the patients, their healthy parents, 20 normal adult controls and 22 normally developed children. The SIR to S-100 protein was significantly higher in the cerebral-palsied and developmentally-delayed children when compared to that of the normal control group children. Increased SIRs were detected in healthy mothers but not in their fathers. There was no difference of SIR between the cerebral-palsied and developmentally-delayed children or any significant difference of SIRs according to the findings of the brain MRI or to developmental quotients. But, the SIRs to S-100 protein were higher in the group of more abnormal findings on brain SPECT.
Adolescence ; Adult ; Cerebral Palsy/immunology* ; Cerebral Palsy/blood ; Child ; Developmental Disabilities/immunology* ; Developmental Disabilities/blood ; Female ; Human ; Male ; Nerve Tissue Protein S 100/blood* ; Parents* ; Reference Values

PURPOSE: Neurosonography, cerebral doppler and electrophysiological study are valuable in predicting neurodevelopmental outcome. The purpose of this study was to analyze characteristics of neonatal neurosonographic findigs, cerebral doppler and electrophysiological findings in infants withmotor disturbance. METHODS: The subjects were 33 infants hospitalized at Yonsei Severance Medical Center NICU from January, 1990 to December, 1992. 19 of these patients underwent neonatal cranial ultrasonogram and cerebral doppler and were diagnosed with cerebral palsy(CP) after Vojta treatment. 14 patients alose received Vojta treatment after discharge but showed only delayed development(DD). The cases were studied retrospectively and color doppler sonograpy was performed within 3 days of birth, 5-7 days after birth and weekly thereafter, and the cerebral blood flow velocity of the anterior cerebral artery was measured and comparison between normal pre-term and full-term infants done. Electroencephalogeraphy(EEG) and brainstem auditory evoked potential(BSAEP) were performed before discharge, with follow-up studies done only in cases showing abnormal findings, whereas visual evoked potential(VEP) was performed after discharge interhemispheric fissure was seen in 7 cases of CP and 1 cases of DD, showing a singnificatn frequency in CP, whereas there was no difference in the linear echogenecity of the basal ganglia. 4) Abnormal EEG was seen in 12 cases(63%) of CP group and 4 cses(29%) of DD group. The follow up EEG was normalized in all DD patients but CP patients conhtinues to show significant EEG abnormalities (7 cases out of 9). BSAEP and VEP abnormalities showed no significant difference between the 2 groups. 5) There was no significant difference in the average resistance index(RI) which was calculated from the cerebral blood flow velocity measured by cerebral doppler among normal newborns, CP and DD patents group. But average RI of CP patients was significantly lower than that of normal newborns, indicating an increase in cerebral blood flow in early life with severe neurologic sequelae. CONCLUSIONS: Neurosogography, cerebral doppler and electrophysiological studies performed in the perinatal period of children with motor disturbance show characteristic abnormal finding, with significant differences among childrem diagnosed with CP and DDD.
Anterior Cerebral Artery ; Basal Ganglia ; Blood Flow Velocity ; Brain Stem ; Cerebral Palsy ; Child ; Dichlorodiphenyldichloroethane ; Electroencephalography ; Follow-Up Studies ; Humans ; Infant* ; Infant, Newborn ; Parturition ; Retrospective Studies ; Ultrasonography

PURPOSE: Neurosonography, cerebral doppler and electrophysiological study are valuable in predicting neurodevelopmental outcome. The purpose of this study was to analyze characteristics of neonatal neurosonographic findigs, cerebral doppler and electrophysiological findings in infants withmotor disturbance. METHODS: The subjects were 33 infants hospitalized at Yonsei Severance Medical Center NICU from January, 1990 to December, 1992. 19 of these patients underwent neonatal cranial ultrasonogram and cerebral doppler and were diagnosed with cerebral palsy(CP) after Vojta treatment. 14 patients alose received Vojta treatment after discharge but showed only delayed development(DD). The cases were studied retrospectively and color doppler sonograpy was performed within 3 days of birth, 5-7 days after birth and weekly thereafter, and the cerebral blood flow velocity of the anterior cerebral artery was measured and comparison between normal pre-term and full-term infants done. Electroencephalogeraphy(EEG) and brainstem auditory evoked potential(BSAEP) were performed before discharge, with follow-up studies done only in cases showing abnormal findings, whereas visual evoked potential(VEP) was performed after discharge interhemispheric fissure was seen in 7 cases of CP and 1 cases of DD, showing a singnificatn frequency in CP, whereas there was no difference in the linear echogenecity of the basal ganglia. 4) Abnormal EEG was seen in 12 cases(63%) of CP group and 4 cses(29%) of DD group. The follow up EEG was normalized in all DD patients but CP patients conhtinues to show significant EEG abnormalities (7 cases out of 9). BSAEP and VEP abnormalities showed no significant difference between the 2 groups. 5) There was no significant difference in the average resistance index(RI) which was calculated from the cerebral blood flow velocity measured by cerebral doppler among normal newborns, CP and DD patents group. But average RI of CP patients was significantly lower than that of normal newborns, indicating an increase in cerebral blood flow in early life with severe neurologic sequelae. CONCLUSIONS: Neurosogography, cerebral doppler and electrophysiological studies performed in the perinatal period of children with motor disturbance show characteristic abnormal finding, with significant differences among childrem diagnosed with CP and DDD.
Anterior Cerebral Artery ; Basal Ganglia ; Blood Flow Velocity ; Brain Stem ; Cerebral Palsy ; Child ; Dichlorodiphenyldichloroethane ; Electroencephalography ; Follow-Up Studies ; Humans ; Infant* ; Infant, Newborn ; Parturition ; Retrospective Studies ; Ultrasonography

BACKGROUND: We compared the yields of mobilized PBSCs from single day of normal volume leukapheresis (NVL) in children and adults, and factors affecting the yields, to understand differences in mobilization efficiency between adults and small children with healthy marrows.METHODS: This study involved 18 adult volunteer donors and 47 small children weighing less than 20 kg who participated in a clinical trial of cell therapy in children with cerebral palsy. Donor factors analyzed to identify predictors of the yield of apheresis included age, gender, weight and complete blood cell count (CBC) with differential counts as well as equipment parameters.RESULTS: The yields of total nucleated cells (TNCs) and CD34⁺cells in the apheresis products of the children were significantly lower than in those from healthy adults. However, the efficiency of recovery of PBSCs (total CD34⁺ cell counts/TNCs) was significantly higher in small children (0.48±0.30%) than in adults (0.10±0.05%) (P < 0.05). Multivariable analysis of adult donor factors showed that the processed volume and flow rate of apheresis were significantly associated with the yield of TNCs (P < 0.05, for both), but not of CD34⁺cells. However, in multivariable analysis of child donor factors, body weight and circulating WBC count on the day of apheresis were significantly associated with the yield of TNCs (P < 0.05, for both) and of CD34⁺cells (P < 0.05, for both).CONCLUSION: The predictors of PBSC yields from a single day of NVL in adults and small children are different. Also mobilization is more effective in small children than in adults.
Adult ; Blood Cell Count ; Blood Component Removal ; Body Weight ; Bone Marrow ; Cell- and Tissue-Based Therapy ; Cerebral Palsy ; Child ; Hematopoietic Stem Cell Mobilization ; Humans ; Leukapheresis ; Tissue Donors ; Volunteers

BACKGROUND AND OBJECTIVES: The transplantation of human umbilical cord blood cells (hUCBCs) has been shown to attenuate the unregulated activation of microglia in a rat model of cerebral palsy (CP). To investigate whether hUCBCs transplantation is also anti-inflammatory in humans, we performed a clinical trial in patients with CP. METHODS AND RESULTS: Allogeneic or autologous hUCBCs and erythropoietin (EPO) were intravenously injected into human patients with CP (mean age of approximately 38 weeks), and patients were analyzed for their motor function and social behavior. Blood samples were tested for cytokine levels. The most surprising finding in the study was that the cytokine levels were dependent on the donor cell source (allogeneic or autologous). Interestingly, the allogeneic treatment group demonstrated significantly decreased levels of pro-inflammatory factors, such as IL-1alpha, IL-6, TNF-beta, and RANTES, and showed a statistically significant improvement in motor and social behavior compared to the autologous treatment group. CONCLUSIONS: Given that inflammation plays a pivotal role in CP, our results suggest that allogeneic hUCBCs therapy may be an appropriate strategy for CP treatment. In addition, prior to transplantation, a detailed analysis of the amount of proinflammatory cytokines in cord blood may be needed to avoid exacerbating inflammatory responses.
Animals ; Cerebral Palsy ; Chemokine CCL5 ; Cytokines ; Erythropoietin ; Fetal Blood ; Humans ; Inflammation ; Interleukin-6 ; Lymphotoxin-alpha ; Microglia ; Rats ; Social Behavior ; Tissue Donors ; Transplants ; Umbilical Cord

OBJECTIVE: To investigate the effect of intravenous infusion of peripheral blood mononuclear cells (mPBMC) mobilized by granulocyte-colony stimulating factor (G-CSF) on upper extremity function in children with cerebral palsy (CP). METHODS: Fifty-seven children with CP were enrolled. Ten patients were excluded due to follow-up loss. In total, 47 patients (30 males and 17 females) were analyzed. All patients' parents provided signed consent before the start of the study. After administration of G-CSF for 5 days, mPBMC was collected and cryopreserved. Patients were randomized into two groups 1 month later. Twenty-two patients were administered mPBMC and 25 patients received normal saline as placebo. Six months later, the two groups were switched, and administered mPBMC and placebo, respectively. Quality of Upper Extremity Skills Test (QUEST) and the Manual Ability Classification System (MACS) were used to evaluate upper motor function. RESULTS: All subdomain and total scores of QUEST were significantly improved after mPBMC and placebo infusion, without significant differences between mPBMC and placebo groups. A month after G-CSF, all subdomain and total scores of QUEST were improved. The level of MACS remained unchanged in both mPBMC and placebo groups. CONCLUSION: In this study, intravenously infused mPBMC showed no significant effect on upper extremity function in children with CP, as compared to placebo. The effect of mPBMC was likely masked by the effect of G-CSF, which was used in both groups and/or G-CSF itself might have other neurotrophic potentials in children with CP.
Cerebral Palsy* ; Child* ; Classification ; Follow-Up Studies ; Granulocyte Colony-Stimulating Factor ; Humans ; Infusions, Intravenous* ; Male ; Masks ; Parents ; Peripheral Blood Stem Cell Transplantation ; Upper Extremity*

Excessive oral and maxillofacial bleeding causes upper airway obstruction, bronchotracheal and gastric aspiration and hypovolemic shock. Therefore, the rapid and correct bleeding control is very important for saving lives in the emergency room. Despite the conventional bleeding control methods of wiring (jaw fracture, wound suture and direct pressure), continuous bleeding can occur due to the presence of various bleeding disorders. There are five main causes for excessive bleeding disorders in the clinical phase; (1) vascular wall alteration (infection, scurvy etc.), (2) disorders of platelet function (3) thrombocytopenic purpura (4) inherited disorders of coagulation, and (5) acquired disorders of coagulation (liver disease, anticoagulant drug etc.). In particular, infections can alter the structure and function of the vascular wall to a point at which the patient may have a clinical bleeding problem due to vessel engorgement and erosion. Wound infection is a frequent cause of postoperative active bleeding. To prevent postoperative bleeding, early infection control using a wound suture with proper drainage establishment is very important, particularly in the active bleeding sites in a contaminated emergency room. This is a case report of a rational bleeding control method by rapid wiring, wound suture with drainage of a rubber strip & iodoform gauze and wet gauze packing, in a 26-year-old male cerebral palsy patient with active oral and maxillofacial bleeding injuries caused by a traffic accident.
Adult ; Airway Obstruction ; Blood Platelets ; Cerebral Palsy ; Drainage ; Emergencies ; Glycosaminoglycans ; Hemorrhage ; Humans ; Hydrocarbons, Iodinated ; Infection Control ; Male ; Mentally Disabled Persons ; Purpura, Thrombocytopenic ; Rubber ; Scurvy ; Shock ; Sutures ; Wound Infection

Tracheoinnominate artery fistula is a rare, fatal complication of tracheostomy, and prompt diagnosis and management are imperative. We report the case of tracheoinnominate artery fistula after tracheostomy in a 14-year-old boy with a history of severe periventricular leukomalacia, hydrocephalus, cerebral palsy, and epilepsy. The tracheoinnominate artery fistula was successfully treated with a stent graft insertion via the right common femoral artery. Endovascular repair of the tracheoinnominate artery fistula via stent grafting is a safe, effective, and minimally invasive treatment for patients in poor clinical conditions and is an alternative to traditional open surgical treatment.
Adolescent* ; Angioplasty ; Arteries ; Blood Vessel Prosthesis ; Brachiocephalic Trunk ; Cerebral Palsy ; Diagnosis ; Epilepsy ; Femoral Artery ; Fistula* ; Humans ; Hydrocephalus ; Infant, Newborn ; Leukomalacia, Periventricular ; Male* ; Stents* ; Tracheostomy*

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.
Blood Coagulation Factors ; Brain ; Cerebral Palsy* ; Child ; Hematoma ; Humans ; Intracranial Hemorrhages* ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans ; Rehabilitation ; Skin ; Stroke

Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.
Blood Coagulation Factors ; Brain ; Cerebral Palsy* ; Child ; Hematoma ; Humans ; Intracranial Hemorrhages* ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans ; Rehabilitation ; Skin ; Stroke