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

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

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*

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.

In view of the lack of health resources and medical treatment in Cangzhou City,especially at grass-roots level,Cangzhou medical college innovated the training mode for clinical medical students and actively explored the cooperation between school and hospital in the educational practice in an aim to improve the quality of talent training and to cultivate high-leveled specialized talents willing to stay in hospitals at grass-roots level and be useful.

Objective To evaluate the clinical value and safety of hysteroscopy in diagnosis of abnormal uterine bleeding.Methods The clinical characters and histopathological data of 272 cases of abnormal uterine bleeding were retrospectively analyzed.They received hysteroscopy in our hospital from Jan.2010 to Jan.2015.Results Histopathological diagnosis showed 75 cases were with endometrial polyps,including 5 cases misdiagnosed as submucosal fibroids,and the correct diagnostic rate was 93.3％ (70/75).50 cases were with submucosal fibroids,and the diagnostic rate was 90.9％ (50/55).97 cases were endometrial hyperplasia,including 7 cases misdiagnosed as atypical hyperplasia or cancer by hysteroscopy,and the correct diagnostic rate was 92.8％ (90/97).19 cases were atypical hyperplasia or endometrial carcinoma,and the diagnostic rate was 73.1％(19/26).Atrophic endometrium were 23 cases.8 cases had intrauterine device remained.Conclusions Premenopausal women are more likely to present with endometrial hyperplasia,submucosal fibroids,endometrial polyps,while endometrial cancer and precancerous lesions are rare.But in postmenopausal women endometrial polyps,atrophic endometrium,cancer and precancerous lesions are mainly included.Hysteroscopic has a good diagnostic value in endometrial diseases which should be combined with histopathological examination.

Age-related macular degeneration (AMD) has become a leading cause of irreversible visual loss in senior population with serious influence to their ability of living independently.Epidemiological researches have revealed various risk factors of AMD,some of which are not controllable such as age,heredity and race ;while others are modifiable such as lifestyle,eye conditions and other systemic diseases.However,the awareness of AMD risk factors is alarmingly low in public.Meanwhile,the understanding of AMD risk factors among ophthalmologists is also unsatisfactory.Therefore,the risk factors of AMD are reviewed here in order to improve the understanding of the ophthalmologists and better guide the clinical management of AMD.