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Platybasia in 22q11.2 Deletion Syndrome Is Not Correlated with Speech Resonance

  • Spruijt, Nicole E.;Kon, Moshe;Molen, Aebele B. Mink Van Der
    • Archives of Plastic Surgery
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    • v.41 no.4
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    • pp.344-349
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    • 2014
  • Background An abnormally obtuse cranial base angle, also known as platybasia, is a common finding in patients with 22q11.2 deletion syndrome (22q11DS). Platybasia increases the depth of the velopharynx and is therefore postulated to contribute to velopharyngeal dysfunction. Our objective was to determine the clinical significance of platybasia in 22q11DS by exploring the relationship between cranial base angles and speech resonance. Methods In this retrospective chart review at a tertiary hospital, 24 children (age, 4.0-13.1 years) with 22q11.2DS underwent speech assessments and lateral cephalograms, which allowed for the measurement of the cranial base angles. Results One patient (4%) had hyponasal resonance, 8 (33%) had normal resonance, 10 (42%) had hypernasal resonance on vowels only, and 5 (21%) had hypernasal resonance on both vowels and consonants. The mean cranial base angle was $136.5^{\circ}$ (standard deviation, $5.3^{\circ}$; range, $122.3-144.8^{\circ}$). The Kruskal-Wallis test showed no significant relationship between the resonance ratings and cranial base angles (P=0.242). Cranial base angles and speech ratings were not correlated (Spearman correlation=0.321, P=0.126). The group with hypernasal resonance had a significantly more obtuse mean cranial base angle ($138^{\circ}$ vs. $134^{\circ}$, P=0.049) but did not have a greater prevalence of platybasia (73% vs. 56%, P=0.412). Conclusions In this retrospective chart review of patients with 22q11DS, cranial base angles were not correlated with speech resonance. The clinical significance of platybasia remains unknown.

Zinc Status of South Korean Women in the Second Trimester and the Effect of Those on Pregnancy Outcome (임신 중기의 아연 영양상태가 임신결과에 미치는 영향)

  • Lee Hyun-Sook
    • Journal of Nutrition and Health
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    • v.38 no.7
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    • pp.578-585
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    • 2005
  • Maternal zinc deficiency is relatively common worldwide, but its consequences for pregnancy outcome are not established. The purpose of this study was to examine the effect of zinc status in the second trimester on pregnancy outcome. Subjects were 248 pregnant Korean women (25-28 wk gestation). Cord blood was collected from subgroup of 69 women and pregnancy outcome data were obtained from 185 babies. Anthropometry measurement, dietary intake, and biochemical characteristics of pregnancy and cord serum substances were measured. The subjects were divided into quartiles on the basis of maternal serum Zn concentration; $ZnQ_1$ (< 25 percentile), $ZnQ_2$ ($26\~50$ percentile), $ZnQ_3$ ($51\~75$ percentile), and $ZnQ_4$ ($76\~100$ percentile). Zn groups were compared in terms of various maternal factors, concentrations of cord serum substances, and pregnancy outcome. Maternal serum Zn concentration was $118.4\;{\pm}\;35.5\;{\mu}g/dl$ as mean. Intake of Zn was lower than Korean RDA. The rate of Zn deficiency among all subjects was $8.5\%$. Maternal serum Zn levels belonged to normal range. Cord serum Zn level was about $154.7\%$ of maternal serum level. Intakes of energy, calcium, iron, folic acid, and riboflavin did not meet the Korean RDA for pregnant women by gestational age. The mean birth weight of neonates is 3083 $\pm$ 697 g, of whom $9.1\%$ were of low birth weight (< 2,500 g). Maternal serum Zn level was positively correlated with pre-pregnancy weight, pre-pregnancy body mass index, and vitamin C intake (p < 0.05). $ZnQ_1$ group had significantly lower maternal serum iron concentration and higher cord serum cholesterol than those in other groups. Maternal serum Zn level, cord serum Zn level, and dietary Zn intake were no related to the pregnancy outcome. The birth weight had a correlation with the maternal hemoglobin and albumin concentration. In conclusion, at this study, we could not find the association with maternal Zn status in 2nd trimester and pregnancy outcome.

An Efficient Method for Aneurysm Volume Quantification Applicable in Any Shape and Modalities

  • Chung, Jaewoo;Ko, Jung Ho
    • Journal of Korean Neurosurgical Society
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    • v.64 no.4
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    • pp.514-523
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    • 2021
  • Objective : Aneurysm volume quantification (AVQ) using the equation of ellipsoid volume is widely used although it is inaccurate. Furthermore, AVQ with 3-dimensional (3D) rendered data has limitations in general use. A novel universal method for AVQ is introduced for any diagnostic modality and application to any shape of aneurysms. Methods : Relevant AVQ studies published from January 1997 to June 2019 were identified to determine common methods of AVQ. The basic idea is to eliminate the normal artery volume from 3D model with the aneurysm. After Digital Imaging and Communications in Medicine (DICOM) data is converted and exported to stereolithography (STL) file format, the 3D STL model is modified to remove the aneurysm and the volume difference between the 3D model with/without the aneurysm is defined as the aneurysm volume. Fifty randomly selected aneurysms from DICOM database were used to validate the different AVQ methods. Results : We reviewed and categorized AVQ methods in 121 studies. Approximately 60% used the ellipsoid method, while 24% used the 3D model. For 50 randomly selected aneurysms, volumes were measured using 3D Slicer, RadiAnt, and ellipsoid method. Using 3D Slicer as the reference, the ratios of mean difference to mean volume obtained by RadiAnt and ellipsoid method were -1.21±7.46% and 4.04±30.54%, respectively. The deviations between RadiAnt and 3D Slicer were small despite of aneurysm shapes, but those of ellipsoid method and 3D Slicer were large. Conclusion : In spite of inaccuracy, ellipsoid method is still mostly used. We propose a novel universal method for AVQ that is valid, low cost, and easy to use.

Cardiovascular Responses and Nitric Oxide Production in Cerebral Ischemic Rats

  • Shinl, Chang-Yell;Lee, Nam-In;Je, Hyun-Dong;Kim, Jeong-Soo;Sung, Ji-Hyun;Kim, Dong-Seok;Lee, Doo-Won;Bae, Ki-Lyong;Sohn, Uy-Dong
    • Archives of Pharmacal Research
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    • v.25 no.5
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    • pp.697-703
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    • 2002
  • We investigated that the role of nitric oxide (NO) on ischemic rats in brain and heart. Ischemia was induced by both common carotid arteries (CCA) occlusion for 24h following reperfusion. Then tissue samples were removed and measured NOx. In brain, NOx was increased by about 40% vs. normal and it was significantly inhibited by aminoguanidine, selective iNOS inhibitor. This result showed that NOx concentration was increased by iNOS. We investigated the role of $Ca^{2+}$ during ischemia. Nimodipine, L-type calcium channel blocker, didn't inhibit the increases of NOx concentration during ischemia. It suggested that increased NOx was due to calcium-independent NOS. MK-801, which N-methyl-D-aspartate (NMDA) receptor antagonist, didn't significantly prevent the increases of NOx. In heart, ischemia caused NOx decrease and it is inconsistent with NOx increase in brain. Aminoguanidine and nimodipine didnt affect on NOx decrease. But MK-801 more lowered NOx concentration than those of ischemia control group. It seemed that $Ca^{2+}$ influx in heart partially occurred via NMDA receptor and inhibited by NMDA receptor antagonist. The mean arterial pressure (MAP) in ischemic rats after 24h of CCA occlusion was decreased when compared to normal value, whereas the heart rates (HR) was not different between two groups. Aminoguanidine or MK801 had no effect on MAP or HR, but nimodipine reduced MAP. There was no difference the effects of aminoguanidine, nimodipine, or MK-801, on MAP and HR between normal rats and ischemic rats. In summary, ischemic model caused an increase of NOx concentration, suggesting that this may be produced via iNOS, which is calcium independent in brain. However in heart, ischemia decreased NOx concentration and NMDA receptor was partially involved. The basal MAP was decreased in ischemic rats but HR was not different from normal control, suggesting that increased NOx in brain of ischemic rat may result in the hypotension.

The Relationship between Recurrent Abdominal Pain and Depressive Trends in School-Aged Children (학동기 소아에서 반복성 복통과 우울 성향과의 관계)

  • Ahn, Young-Joon;Moon, Kyung-Rye
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.5 no.2
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    • pp.166-173
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    • 2002
  • Purpose: Recurrent abdominal pain (RAP) is common, affecting between 7% and 25% of school-aged children and young adult. This study was designed to assess the relationship between RAP and depressive trends in school-aged children. Methods: In 1999, We surveyed 1,181 elementary school children (566 boys and 615 girls) whose ages ranged from 9 to 12 years. The degree of depression was compared between the group with RAP and normal control group without RAP according to Korean Children's Depression Inventory (CDI) of Kovacs and Beck. Results: The number of the group with RAP was significantly higher in female (25.7%) than in male (18.9%). The most common site of abdominal pain was periumbilical. The mean depression score in the group with RAP was higher than that of the control group. The depression score was significantly higher in the group with RAP who has frequently pain and additional symptoms. The depression score was significantly higher in the group with RAP who prefered to play alone, did not enjoy going to school and had few friends than in the group with RAP who had a lot of friends, had active and meticulous characters. The depression scores were not significantly different according to the duration of abdominal pain, the onset time of pain, and the day of pain in the group with RAP. Conclusion: The depression score of the group with RAP was higher than that of normal group. So, there is relatively a specific association between childhood RAP and depression in school aged children. It is good for physicians to consider psychological support in the treatment of childhood RAP.

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Analysis of High-Resolution Manometry Results in LPRD Patients Who Do Not Response to PPI Medication (양자펌프 억제제에 반응을 보이지 않은 인후두 역류질환 환자에서 고해상도 식도 내압검사의 분석)

  • Lee, Chang-Hee;Lee, Hyun-Sub;Jin, Sung-Min;Lee, Sang-Hyuk
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.23 no.1
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    • pp.43-47
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    • 2012
  • Background and Objectives : Laryngopharyngeal reflux disease (LPRD) originates from regurgitation of gastric contents to the laryngopharynx, and it is relatively common disease in otolaryngology. Proton-pump inhibitor (PPI) medication is frequently using treatment method in present time, but controversies exist regarding treatment for it. High-resolution manometry (HRM) system is a newly developed device that using 36 channels and pressure topography plotting program, checking the function and status of esophagus. The aim of this study was to evaluate clinical usefulness of HRM in LPRD patients who do not response to PPI medication. Materials and Method : From July 2009 to July 2010, a total of 99 patients who were suspected LPRD according to symptoms (Reflux Symptom Index >13) and laryngoscopic findings (Reflux Finding Score >7) were retrospectively enrolled in this study. Patients were consisted of 31 men and 68 women, the mean age was 51.8 years, the mean BMI was 22.46. Patients have taken PPI medication for 2 months, after medication, they performed HRM. Comparative analysis was performed with results of the difference in symptoms. Results : In 99 patients, 37 patients (37.4%) show not improvement of LPRD symptoms after PPI medication. Among them, 18 patients (48.6%) showed abnormal findings that were classified as Peristaltic dysfunction (38.9%), Diffuse esophageal spasm (11.1%), Relaxation impairment of LES (11.1%), Achalasia (5.6%), Hypotensive LES (11.1%), Relaxation impairment of UES (11.1%), Nutcracker esophagus (5.6%), Decreased resting pressure (5.6%). Other 62 patients (62.6%) show improvement of LPRD symptoms, 23 patients (37.1%) showed abnormal findings. Most common finding was Peristaltic dysfunction (43.4%) More prevalent abnormal findings of HRM were found in patients who revealed no response to PPI medication (48.6% vs. 37.1%), but it does not reach the statistical significance. Conclusion : Although statistical significances is not showed, the difference observed in the frequency of HRM abnormal finding between response and no response for PPI medication. It has been estimated that HRM may be used to determine differential diagnosis in patients with LPRD. Further studies in lager population containing normal controls will be needed to prove clinical usefulness.

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Analysis of High-Resolution Manometry Results in LPRD Patients who do not Response to PPI Medication (양자펌프 억제제에 반응을 보이지 않은 인후두 역류질환 환자에서 고해상도 식도 내압검사의 분석)

  • Lee, Chang-Hee;Lee, Hyun-Sub;Jin, Sung-Min;Lee, Sang-Hyuk
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.22 no.2
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    • pp.151-155
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    • 2011
  • Background and Objectives : Laryngopharyngeal reflux disease (LPRD) originates from regurgitation of gastric contents to the laryngopharynx, and it is relatively common disease in otolaryngology. Proton-pump inhibitor (PPI) medication is frequently using treatment method in present time, but controversies exist regarding treatment for it. High-resolution manometry (HRM) system is a newly developed device that using 36 channels and pressure topography plotting program, checking the function and status of esophagus. The aim of this study was to evaluate clinical usefulness of HRM in LPRD patients who do not response to PPI medication. Subjects and Method : From July 2009 to July 2010, a total of 99 patients who were suspected LPRD according to symptoms (Reflux Symptom Index >13) and laryngoscopic findings (Reflux Finding Score >7) were retrospectively enrolled in this study. Patients were consisted of 31 men and 68 women, the mean age was 51.8 years, the mean BMI was 22.46. Patients have taken PPI medication for 2 months, after medication, they performed HRM. Comparative analysis was performed with results of the difference in symptoms. Results : In 99 patients, 37 patients (37.4%) show not improvement of LPRD symptoms after PPI medication. Among them, 18 patients (48.6%) showed abnormal findings that were classified as Peristaltic dysfunction (38.9%), Diffuse esophageal spasm (11.1%), Relaxation impairment of LES (11.1%), Achalasia (5.6%), Hypotensive LES (11.1%), Relaxation impairment of UES (11.1%), Nutcracker esophagus (5.6%), Decreased resting pressure (5.6%). Other 62 patients (62.6%) show improvement of LPRD symptoms, 23 patients (37.1%) showed abnormal findings. Most common finding was Peristaltic dysfunction (43.4%) More prevalent abnormal findings of HRM were found in patients who revealed no response to PPI medication (48.6% vs. 37.1%), but it does not reach the statistical significance. Conclusion : Although statistical significances is not showed, the difference observed in the frequency of HRM abnormal finding between response and no response for PPI medication. It has been estimated that HRM may be used to determine differential diagnosis in patients with LPRD. Further studies in lager population containing normal controls will be needed to prove clinical usefulness.

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Carotid artery intima media thickness in obese children : relation with cardiovascular risk factors (비만 소아에서 경동맥 내중막 두께와 심혈관질환 위험인자와의 상관관계)

  • Lee, Jin;Kwon, Hyo Jin;Park, Moon Ho;Jang, Ki Young;Lee, Kee-Hyoung;Lee, Kwang Chul;Son, Chang Sung;Lee, Joo Won
    • Clinical and Experimental Pediatrics
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    • v.50 no.11
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    • pp.1091-1096
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    • 2007
  • Purpose : The of common carotid artery intima media thickness (IMT) is an acknowledged noninvasive marker for early atherosclerotic changes. We investigate whether common carotid IMT is different between obese and normal-weight children and also evaluate the relationships IMT with cardiovascular risk factors. Methods : We collected the clinical data (age, sex, pubertal stage, body mass index) and measured blood pressure, glucose, insulin, lipid profiles and adiponectin in 49 obese children (mean age, 12 years) and 24 nonobese children as controls. The control group was composed of 24 nonobese children of the same age, sex and pubertal stage. We measured the carotid IMT of all subjects by B-mode ultrasound with a 7.5-MHz linear transducer and analyzed. Results : Obese children demonstrated a significantly thicker intima media (mean, 0.34 mm, peak, 0.42 mm) compared to the control group (mean, 0.31 mm, peak, 0.38 mm, P<0.01). IMT was significantly correlated to the BMI (r=0.431, P<0.01), age (r=0.317, P<0.01), total cholesterol (r=0.377, P< 0.01), triglyceride (r=0.253, P<0.05) and low-density lipoprotein cholesterol (r=0.289, P<0.05). Serum adiponectin was significantly lower in obese children than in controls (11.2 ng/mL vs. 14.7 ng/mL, P<0.05) and negatively related with IMT (r=-0.267, P<0.05). Conclusion : Obesity is associated with increased carotid artery IMT in children. Our results suggest vascular changes in obesity seem to occur already in childhood and vascular ultrasonography may helpful for screening cardiovascular complications in obese children.

Shape and Incidence of Rib Variations in Chest Radiographs (단순흉부촬영상 관찰되는 늑골 변이의 양상 및 빈도)

  • Joo, Ji-Seon;Bae, In-Young;Kim, Sung-Tae;Kwak, Seung-Min;Cho, Chul-Ho;Cho, Seung-Wook;Park, Chan-Sup
    • Tuberculosis and Respiratory Diseases
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    • v.48 no.1
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    • pp.45-53
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    • 2000
  • Background: The literature on variations of rib is limited. Very little has been written in the radiological journal of this country on the subject. It seemed of interest to investigate the nature and incidence of congenital variations in a series of routine chest roentgenograms. The topic of rib variations has not been covered extensively in the radiological journals in Korea. This has presented an opportunity to investigate the nature, type, shape and incidences of congenital rib variations in normal Korean adults from a series of routine roentgenograms. Methods: Chest radiographs of 5,000 adults, who visited our hospital for a routine check-up or for employment physical examinations from January 1996 to September 1998, were consecutively reviewed. The sex distribution consisted of 2,827 male males and 2,173 female females(ratio of 1.3:1) with the age range aged between 19 and 65 years(mean age: 34.6 years) were included. The chest PAs were analyzed for the presence, type, location, and shape of the rib variations. From this data the incidence of each type of variations was calculated. Results: Seventy-six of the 5,000 adults(1.52%), 63 male(2.23%) and 13 female(0.6%), showed 88 cases of rib variation(Table 1). The most common variation was the bifid rib(n=35), followed by hypoplasia of the rib(n=22), flaring of the rib(n=18), bridging of the ribs(n=7), cervical ribs(n=3), and fusion of between ribs(n=3)(Table 2). The bifid rib(Table 1) was found most frequent in the right fourth rib(12/35, 34.3%), followed by the left fifth rib(6/35, 17.1%) and right third rib(6/35, 17.1%). Hypoplasia of the rib was common in first rib(20/22, 90.9%). Flaring of the rib was common at fourth rib(8/18, 44.4%, right and left combined), and bridging between ribs was common between first and second rib(3/7, 42.9%). Conclusion : The percentage of incidence of rib variations in adults was 1.52%. Bifid rib was the most common variation, followed by hypoplasia, flaring, bridging, cervical rib, and fusion of ribs, in decreasing order.

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A Pressure Adjustment Protocol for Programmable Valves

  • Kim, Kyoung-Hun;Yeo, In-Seoung;Yi, Jin-Seok;Lee, Hyung-Jin;Yang, Ji-Ho;Lee, Il-Woo
    • Journal of Korean Neurosurgical Society
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    • v.46 no.4
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    • pp.370-377
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    • 2009
  • Objective : There is no definite adjustment protocol for patients shunted with programmable valves. Therefore, we attempted to find an appropriate method to adjust the valve, initial valve-opening pressure, adjustment scale, adjustment time interval, and final valve-opening pressure of a programmable valve. Methods : Seventy patients with hydrocephalus of various etiologies were shunted with programmable shunting devices (Micro Valve with $RICKHAM^{(R)}$ Reservoir). The most common initial diseases were subarachnoid hemorrhage (SAH) and head trauma. Sixty-six patients had a communicating type of hydrocephalus, and 4 had an obstructive type of hydrocephalus. Fifty-one patients had normal pressure-type hydrocephalus and 19 patients had high pressure-type hydrocephalus. We set the initial valve pressure to $10-30\;mmH_2O$, which is lower than the preoperative lumbar tapping pressure or the intraoperative ventricular tapping pressure, conducted brain computerized tomographic (CT) scans every 2 to 3 weeks, correlated results with clinical symptoms, and reset valve-opening pressures. Results : Initial valve-opening pressures varied from 30 to $180\;mmH_2O$ (mean, $102{\pm}27.5\;mmH_2O$). In high pressure-type hydrocephalus patients, we have set the initial valve-opening pressure from 100 to $180\;mmH_2O$. We decreased the valve-opening pressure $20-30\;mmH_2O$ at every 2- or 3-week interval, until hydrocephalus-related symptoms improved and the size of the ventricle was normalized. There were 154 adjustments in 81 operations (mean, 1.9 times). In 19 high pressure-type patients, final valve-opening pressures were $30-160\;mmH_2O$, and 16 (84%) patients' symptoms had nearly improved completely. However, in 51 normal pressure-type patients, only 31 (61%) had improved. Surprisingly, in 22 of the 31 normal pressure-type improved patients, final valve-opening pressures were $30\;mmH_2O$ (16 patients) and $40\;mmH_2O$ (6 patients). Furthermore, when final valve-opening pressures were adjusted to $30\;mmH_2O$, 14 patients symptom was improved just at the point. There were 18 (22%) major complications : 7 subdural hygroma, 6 shunt obstructions, and 5 shunt infections. Conclusion : In normal pressure-type hydrocephalus, most patients improved when the final valve-opening pressure was $30\;mmH_2O$. We suggest that all normal pressure-type hydrocephalus patients be shunted with programmable valves, and their initial valve-opening pressures set to $10-30\;mmH_2O$ below their preoperative cerebrospinal fluid (CSF) pressures. If final valve-opening pressures are lowered in 20 or $30\;mmH_2O$ scale at 2- or 3-week intervals, reaching a final pressure of $30\;mmH_2O$, we believe that there is a low risk of overdrainage syndromes.