Objective To explore the clinical characteristics and treatment of methylmalonic acidemia(MMA) in order to improve our understanding of it.Methods We analyzed the clinical manifestations,laboratory examinations and treatments of 26 cases of methylmalonic acidemia in children.Results Twenty-four cases were involved in nervous system.Nine patients were involved in renal system.Eight cases of hematological involved.Liver enzyme elevated in 2 cases as well as the cardiac system were involved in 3 cases.One case was with pneumonia onset.The laboratory findings showed metabolic acidosis in 12 cases,hyperhomocysteinemia in 8 cases and remarkable elevation of urinary methylmalonic acid concentration in all cases.Some abnormalities in globus pallidus and cerebral white matter as well as diffuse cerebral atrophy were noted by the brain CT and MRI in 15 cases.Sixteen children have received therapy of vitamin B12,and supplementation of L-carnitine with restricted-protein diet.The follow-up for a period ranging from 3 months to 1.5 years( mean 8.5 months) of 15 cases with medical therapy showed a favorable outcome of nervous system improvement in 12 cases,however,2 patients died from severe metabolic acidosis.8 patients with renal involvement were normal in urine routine and renal function.Conclusion Methylmalonic acidemia has different clinical features,so early urine organic acids analysis by GC/MS method is essential Long-term and reasonable treatment after diagnosis is an effective way to improve the prognosis.

Objective To observe the food intolerance of irritable bowel syndrome with diarrhoea(IBS-D) patients and healthy physical examinees and the clinical effect of the treatment of IBS-D food rejectingtrend.Methods Sixty-eight cases IBS-D patients meeting the diagnostic criteria of RomeⅢ were selected and 40 cases of healthy as control group,tested 14 kinds of food specific IgG antibody by enzyme-linked immunosorbent assay,treated the IBS-D patients with food intolerance for 12 weeks by the application of food rejectingtrend and observed the change of main symptoms.Results Among the 68 cases of IBS-D patients,55 cases had at least one of food intolerance,the positive rate was 80.88%;the control group had 13 cases of food intolerance,the positive rate was 32.50%,the difference between the two groups was statistically significant(χ2=25.28,P<0.01).The food with the highest positive rates of two groups were both wheat,egg white/egg yolk,milk and soybean.After 12 weeks of diet treatment,the total score of the symptoms of IBS-D patients decreased from (8.25±2.97) points to (3.13±3.52) points(t=11.107,P<0.01),self scores of the degree 0and frequency of abdominal pain,frequency of diarrhea,degree of abdominal distension,stool shape and general feelings of distress were significantly improved compared with that before treatment(t=12.429,11.016,5.685,5.558,7.681,11.065,P<0.01).Among 54 cases IBS-D patients(1 patient lost to follow-up),61.11%(33/54) significantly improved,24.07%(13/54) improved,14.81%(8/54) invalid,the total effective rate was 85.19%(46/54).Conclusion There is correlation between food intolerance and IBS-D.According to the Results of the detection of food specific IgG antibody,food rejectingtrend treatment can effectively improve the symptoms of IBS-D.

Angiotensin-Ⅱ-induced apoptosis was widely involved in the pathogenesis of diabetes mellitus, cardiovascular diseases, etc. The mechanism of angiotensin-Ⅱ-induced apoptosis is complicated, including the receptor (AT1R and AT2R) level regulation, Fas/FasL pathway, and the regulation by p53 family, Bcl-2 family, Caspase family, Ang-(1-7), etc. Mitochondria damage and oxidative damage play important roles in the apoptosis process. This article reviews the previous researches on angiotensin-Ⅱ-induced apoptosis.

BACKGROUND: Each kind of human cell has its own characteristic morphological and functional property. In the skin, epidermal cells, including keratinocyte and melanocyte, also have their own functional characteristics. Thus, it is expected that there are some different responses to external stimuli, such as ionizing radiatio,, free radicals, and cytokines between these cells. OBJECTIVE AND METHODS: To im estigate whether there are different effects of UV light on the viability of cultured human ker tinocytes and rnelanocytes. Cultured human keratinocytes and melanocytes are irradiated by UVB at 5, 25, 50, and 100mJ/cm, and examined by Methylthiazole tetrazollium assay at 0, 1, 3, 6, 24, 48, and 72 hours after UVB exposure. RESULTS: 1. The effects on viability according to the doses of UVB are as follows: 1) In the keratinocytes, the viability was increased in most of the UVB exposure groups within 24 hours after UVB exposure, and was significantly increased at 25, 50, and 100mJ/cm of UVB at 3 hours after UVB exposur.(p<0.05). However, the viability was significantly decreased at relatively high doses of UVB (50, 100mJ/cm) from 48 hours after UVB exposure (p<0.05). 2) In the melanocytes, the viability was decreased in all of the UVB exposure groups within 3 hours, and was significantly decreased in all of the UVB exposure groups at, 1 hour after UVB exposure (p<0.05). The viability was increased from 6 to 24 hours, which was significantly decreased at 100mJ/cm of UVB from 48 hours after UVB exposure (p<0.05). 2. The effects on viability according to the time after UVB exposure at the same dose of UUB In both cells, the viability was increased as time went by. The slopes of the viability curve gradually decreased according to the increment of UVB doses. CONCLUSION: The viability of keratinocyte was decreased at 50mJ/cm of UVB which melanocyte did not show decrease. Melanocyte was more easily damaged than keratinocyte in relatively earlier time period after UVB exposure. These results suggest that the change of viability in cultured keratinocyte and melanocyte after UVB exposure at the dose of less than 100mJ/cm is related to the time course after UVB exposure as well as to the UVB dose.
Cytokines ; Free Radicals ; Humans* ; Keratinocytes* ; Melanocytes* ; Skin ; Ultraviolet Rays

Vitiligo is an acquired systemic disease of the skin characterized by circumscribed patches of complete pigment loss due to destruction of melanocytes. A 28-year old male patient presented with generalized depigmented patchs. We performed microscopic studies of cultured melanocytes from this patient and compared them with those of normal neonatal foreskin. Phase contrast microscopic findings revealed no difference between the two groups of melanocytes, but transmission electron microscopic findings showed dilated circular rough endoplasmic reticulum in cultured melanocytes from our vitiligo patient. We could observe the innate cellular structural aberration of melanocytes from the vitiligo subject.
Adult ; Endoplasmic Reticulum, Rough ; Foreskin ; Humans* ; Male ; Melanocytes* ; Skin ; Vitiligo*

Child ; Child Health Services ; trends ; Delivery of Health Care ; organization & administration ; Education, Medical ; Hospitals, Pediatric ; economics ; organization & administration ; Humans ; Pediatrics ; trends ; Specialization ; Urban Population

Pituitary stalk interruption syndrome(PSIS) is a rare disease characterized by different degrees of anterior pituitary hormone deficiency.The typical imaging finding is a thin or absent pituitary stalk,often associated with an ectopic posterior pituitary and a hypoplastic anterior pituitary.The cause is still unkown.Dynamic contrastenhanced MRI helps to confirm the diagnosis.Replacement of multiple anterior pituitary hormones is the key of the treatment.Doctors should have a thorough understanding of the disease and give patients efficacious treatment and a better prognosis would be obtained.

Objective To analyse the clinical characteristics of male hypogonadotropic hypogonadism.Methods Clinical data of 87 cases of male hypogonadotropic hypogonadism were retrospectively analyzed.Results Mean age was(18.8?4)years old,and 25 patients were diagnosed as Kallman syndrome.The level of serum LH,FSH,and T was low,and LH responded poorly to GnRH stimulation.Most patients' bone age was delayed,and all patients' CT or MRI of pituitary was normal.Conclusion According to the above clinical features,and testosterone replacement or hCG and/or plus hMG are the choice of treatment.

Objective To explore the changes and their correlation of event-related potential(ERP)and the plasma levels of monoamine neurotransmitter in the patients with epilepsy.Methods ERP and the plasma levels of noradrenalin(NE),dopamine(DA) and 5-hydroxytryptamine(5-HT) were tested in 50 patients with epilepsy,and the results were compared with the normal controls.Results The latency of N2,P3 of ERP and the amplitude of P3 in the epilepsy group were significant longer and lower than those in the normal control group(all P

Participating in the international conferences helps to get the latest progress in cer-tain areas. It provides important channels of communication and platform for cooperation. The article summarized the categories of international conferences, access to it, way to apply for it, how to apply for financial support, visa and passport, as wellas preparations before. The author's experience attend-ing the seventh Asia-Pacific conference neurochemistry(APSN) held in Singapore, may offer some help for reference.