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Objective: Hyperphenylalaninemia (HPA) is an inborn error of metabolism in which the hydroxylation of phenylalanine (Phe) to tyrosine is disturbed. Accumulation of Phe leads to severe mental and psychomotor retardation. (1)H magnetic resonance spectroscopy ((1)HMRS) is a novel non-invasive method to quantitate the brain metabolites besides Phe concentration in HPA patients. And it could be acquired conveniently on clinical MRI routine scanners. This study aimed to investigate the correlation between blood Phe ([Phe](blood)) and [Phe](brain), the characteristics of blood-brain Phe metabolism and its impacts on mental retardation., Method: Totally 32 untreated patients diagnosed with HPA were studied, including 18 boys and 14 girls (age ranging from 33 days to 13 years). The patients were divided into two groups: elder than 4 months old (n = 22) and younger than 4 months old (n = 10). (1)HMRS were performed in all patients. [Phe](brain) were measured by absolute [Phe](brain) using Creatinine as an internal reference. [Phe](blood) were measured and developmental quotient (DQ) or intelligence quotients (IQ) were evaluated., Result: (1) [Phe](brain) measured by (1)HMRS ranged from 0.0640 to 0.6296 (M = 0.1542) while the [Phe](blood) was from 0.3804 to 2.5140 mmol/L (M = 1.5210 mmol/L) in all the 32 cases of HPA patients. (2) There was a positive linear correlation (r = 0.6103 (P < 0.01)) between [Phe](blood) and [Phe](brain). And there were interindividual differences in [Phe](brain) in several patients. (3) Variable mental retardation were observed in 23/32 cases in this study. (4) There was a negative correlation between [Phe](blood) and [Phe](brain) to the mental retardation (r(blood) = -0.5045, r(brain) = -0.6471 (P < 0.01)) in 22 cases of the HPA patients older than 4 months. And [Phe](brain) had more significant correlation with mental development than [Phe](blood)., Conclusion: The [Phe](blood) could correspondingly represent the [Phe](brain) in most HPA patients. The Phe concentration could reflect the degree of mental retardation substantially in 22 cases with HPA older than 4 months. And the [Phe](brain) could more accurately illustrate it. (1)HMRS can be used to quantitate intracerebral Phe concentrations non-invasively in HPA patients. Preliminary findings suggest that interindividual variations in the kinetics of Phe uptake and metabolism do exist. (1)HMRS has great clinical significance in understanding the mechanism of HPA patient's mental retardation, providing proper objective standards for better diagnosis and treatment of HPA patients.

Objective: To investigate the stability, predictability and safety of angle-supported phakic intraocular lens (IOL) implantation in high myopia., Methods: Seventy-six myopic eyes of 40 patients treated with anterior chamber angle-supported phakic IOL (Phakic6H) implantation were followed. The average myopia diopter was (-15.89 +/- 3.78) D (-9.50 to -26.25 D) and the best corrected visual acuity (BCVA) was (0.82 +/- 0.20) (0.5 to 1.0). The average follow-up time was 12 months. All the treated eyes were observed for anterior segment changes, uncorrected visual acuity (UCVA), BCVA, refraction, intraocular pressure (IOP) and corneal endothelium preoperatively and postoperatively. All data were analyzed in statistics., Results: One year after the surgery, the average UCVA was (1.00 +/- 0.35) (0.3 to 1.5) and BCVA was (1.18 +/- 0.29) (0.5 to 1.5). The average residual refraction was (-0.40 +/- 0.64) D (-2.00 to +0.50 D). In 96.1% of eyes, the average residual refraction was within +/- 1.0 diopter. The average corneal endothelial count before and one year after the surgery was (3174 +/- 248)/mm(2) and (3067 +/- 320)/mm(2) (P < 0.01) respectively. The intraocular pressure before and one year after the surgery was (16.12 +/- 2.32) mmHg and (15.29 +/- 3.38) mmHg (P < 0.05) respectively. Two patients (4 eyes) complained of halo and glare. One patient's (2 eyes) pupils were oval and another one (2 eyes) showed decentered phakic IOL. Macular hemorrhage occurred in one patient (2 eyes) after eight months of surgery., Conclusions: Angle-supported phakic IOL implantation is effective for treatment of high myopia and the results are predictable. It deserves further clinical investigation. The effectiveness and safety of this kind of phakic IOL should be further observed for a long period of time.

Six cases of rare infundibuloma are reported. Infundibuloma, implying pilocytic astrocytoma which stems from the neurohypophysis, often encroaches upon or injures the neural pathway from the nerve nucleus beneath the hypothalamus to pituitary adenohypophysis. Therefore, various hormones, stimulating or inhibitory, produced by the hypothalamic nerve nuclei could not reach the target cells of adenohypophysis, resulting in different clinical symptoms of dysendocrisiasis. The tumor is difficult to diagnose by X-ray, CT or histopathology because of its miniature size. Regional tomography should be done besides endocrine tests. Specimens should be collected as much as possible during the operation for pathological confirmation. Transoro-nasosphenoidal microsurgery could achieve a good result.

42 patients of midline malignant histiocytosis are reported. The differential diagnosis should be made carefully, for its clinical sign and pathologic picture are similar to some neoplasms, inflammatory or allergic diseases. The 3, 5 and 10 year survival rates are 52.4%, 38.1% and 21.4% respectively. The survival rate of those in the prodromal stage is the highest. The authors suggest that this disease probably belongs to the malignant tumor of mononuclear macrophage system and be a special type of malignant histiocytosis. It may be designated as the midline malignant histiocytosis.