The case report describes the presentation of a 19-year old female with tuberous sclerosis who presented with progressive dyspnoea over 2 days. Chest radiograph revealed bilateral pneumothorax. Computed tomography showed features of pulmonary lymphangioleiomyomatosis and bilateral renal angiomyolipomas. The coexistence of both conditions may cause devastating morbidity and mortality.

Objective To find out the situation of household consumption of iodized salt in Hebei province so as to provide scientific basis for prevention and control of iodine deficiency disorders(IDD). Methods According to the "national iodine deficiency disorders surveillance program (Trial)", the county (city, district) was taken as a unit, township (town) and administrative villages were selected in accordance with the principle of systematic sampling, then households were chosen by random sampling to collect their edible salt in Hebei province from 2007 to 2009. Salt iodine content was detected by direct titration method. Results A total of 48 675, 48 448 and 48 756 salt samples were collected from 2007 to 2009, respectively. The consumption rate of qualified iodized salt from 2007 to 2009 was 91.16%, 91.96% and 96.17%, respectively. There were 24.6%(41/167)and 18.0%(30/167)counties with consumption rate of qualified iodized salt under 90% in 2007 and 2008, respectively. The percentage of counties with consumption rate of qualified iodized salt above 90% was 100.0%(167/167) in 2009. In general there was a significant differences in frequency distribution of consumption rates of qualified iodized salt among the three years(H = 10.778, P ＜ 0.01 ), and the difference was found significant between 2007 and 2009 as well as between 2008 and 2009(all P ＜ 0.05), but was not significant between 2007 and 2008(P ＞ 0.05). Conclusions The consumption rate of qualified iodized salt at household level increases annually from 2007 to 2009. By 2009,the consumption rate of qualified iodized salt in each county is more than 90%, which has reached the national standard stipulated in "the evaluation programs for fulfilling the goal of eliminating IDD at county level".

AIM: To evaluate the cost of inpatient rehabilitation for children with moderate to severe traumatic brain injury (TBI). Secondary aim was to identify factors associated with high inpatient rehabilitation cost. METHOD: Retrospective review of a tertiary hospital's trauma registry was performed from 2011-2017. All patients aged 16 years or younger who sustained TBI with Glasgow Coma Scale ≤13 were included. Data on patient demographics, mechanism and severity of injury, hospital duration and inpatient rehabilitation cost were collected. We performed a regression analysis to identify factors associated with high rehabilitation cost. RESULTS: There were a total of 51 patients. The median duration of inpatient rehabilitation was 13.5 days (interquartile range [IQR] 4-35), amounting to a median cost of SGD8,361 (IQR 3,543-25,232). Daily ward costs contributed the most to total inpatient rehabilitation cost. Those with severe TBI had longer duration of inpatient rehabilitation that resulted in higher cost of inpatient rehabilitation. Presence of polytrauma, medical complications, post-traumatic amnesia and TBI post-non-accidental injury (NAI) were associated with higher cost of inpatient rehabilitation. CONCLUSION The cost of inpatient rehabilitation for paediatric patients post-TBI is significant in Singapore. Patients with TBI secondary to NAI had significantly higher cost of inpatient rehabilitation. Ways to reduce duration of hospitalisation post-TBI and early step-down care or outpatient rehabilitation should be explored to reduce cost.

Purpose: The updated American Society of Clinical Oncology/College of American Pathologists guideline for estrogen receptor (ER) testing recommends that breast cancer with ER expression in 1–10% of tumor cells should be reported as ER-low positive (ERlow), although limited data are available on the overall benefits of endocrine therapy. We investigated the clinicopathological characteristics and clinical outcomes of ERlow breast cancer and to compare them with those of ER-negative (ERneg) and ER-high (> 10% of tumor cells, ERhigh) breast cancers. Methods: Consecutive patients with invasive breast cancer who underwent curative surgery between November 2007 and December 2014 were included. Clinicopathological characteristics and disease-free survival (DFS) of ERlow tumors were compared with those of ERneg and ERhigh tumors. Results: Of the 2,309 cases included, 46 (2%), 643 (27.8%), and 1,620 (70.2%) were ERlow , ERneg , and ERhigh , respectively. ERlow tumors were associated with no special type of histology (p = 0.011), advanced pT (p = 0.017), pN (p = 0.009) and anatomic stages (p < 0.001), high grade (p < 0.001), negative/low progesterone receptor (PR) status (p < 0.001), human epidermal growth factor receptor 2 positivity (p < 0.001), high Ki-67 (p < 0.001), and recurrence (p = 0.006) compared to ERhigh tumors. DFS was significantly dependent on ER status, and ERlowtumors showed poorer DFS than ERhigh tumors (p = 0.001), however, there was no significant survival difference between ERlow and ERneg tumors. Furthermore, DFS in ERhigh patients was affected by hormone therapy (p < 0.001), while it was not affected in ERlow patients. Conclusion Patients with ERlow breast cancer have clinicopathological characteristics that differ from those with ERhigh tumors. Although this study was limited by the small sample size of the ERlow group, no benefit from hormone therapy was observed in the ERlow group compared with the ERhigh group.

OBJECTIVETo analyze the early clinical and radiographic outcomes of Hoffa fractures treated by a standard protocol of open reduction and internal fixation using headless compression screws combined with back buttress plate in a consecutive series of 8 Chinese patients.

METHODSOpen reduction and internal fixation was performed on all patients. The fractures were anatomically reduced and held temporarily by K-wire. If the ends of fractures were atrophic, autologous bone graft from the ipsilateral iliac crest was packed between the ends. Then the fracture fragments were fixed with AO 6.5 mm headless compression cannulated screws. At least two screws were used to provide rotational stability. One pre-contoured reconstruction plate was placed on the nonarticular surface posteromedially or posterolaterally as back buttress plate.

RESULTSAll the patients were followed up for at least 12 months (range 12-25 months). All fractures achieved anatomical reduction and healed clinically and radiographically. At recent follow-up, the mean flexion degree was 120.6° (range 110°-135°) and the mean extension degree was 2.5° (range 0°-5°). The average visual analogue scale score was 1.6 points (range 0-3). Six patients were assessed as excellent and 2 as good according to the hospital for special surgery knee score system. There were no superficial or deep infections, or hardware breakages. No patient had giving way or locking of the knee, though some had intermittent pain and swelling after strenuous exercise. Injury mechanism had significant influence on the functional outcome (P=0.046).

CONCLUSIONHeadless compression screws combined with back buttress plate and/or autologous bone grafting to treat old Hoffa fracture is one of effective measures. It would be conducive to not only fracture healing but also early exercise and functional recovery.

Adult ; Bone Plates ; Bone Screws ; Female ; Femoral Fractures ; surgery ; Fracture Fixation, Internal ; methods ; Humans ; Male ; Middle Aged

The aim of this work was to investigate guar gum/ethylcellulose mix coated pellets for potential colon-specific drug delivery. The coated pellets, containing 5-fluorouracil as a model drug, were prepared in a fluidized bed coater by spraying the aqueous/ethanol dispersion mixture of guar gum and ethylcellulose. The lag time of drug release and release rate were adjustable by changing the ratio of guar gum to ethylcellulose and coat weight gain. In order to find the optimal coating formulation that was able to achieve drug targeting to the colon, the effect of two independent variables (the ratio of guar gum to ethylcellulose and the coat weight gain) on drug release characteristics was studied using 3 x 4 factorial design and response surface methodology. Results indicated that drug release rate decreased as the proportion of ethylcellulose in the hybrid coat and the coat weight gain increased. When the ratio of guar gum to ethylcellulose was kept in the range of 0.2 to 0.7, and the coat weight gain in the range of 250% to 500%, the coated pellets can keep intact for about 5 h in upper gastrointestine and achieve colon-specific drug delivery. The pellets prepared under optimal conditions resulted in delayed-release sigmoidal patterns with T(5%) (time for 5% drug release) of 5.1 - 7.8 h and T(90%) (time for 90% drug release) of 9.8 - 16.3 h. Further more, drug release was accelerated and T(90%) of the optimum formulation pellets decreased to 9.0 - 14.5 h in pH 6.5 phosphate buffer with hydrolase. It is concluded that mixed coating of guar gum and ethylcellulose is able to provide protection of the drug load in the upper gastrointestinal tract, while allowing enzymatic breakdown of the hybrid coat to release the drug load in the colon.
Cellulose ; administration & dosage ; analogs & derivatives ; Colon ; metabolism ; Drug Delivery Systems ; Fluorouracil ; administration & dosage ; chemistry ; Galactans ; administration & dosage ; Mannans ; administration & dosage ; Plant Gums ; administration & dosage

PURPOSE: To examine the acetabular development after the surgical treatment of a developmental dislocation of the hip (DDH). MATERIALS AND METHODS: The serial radiographs of 27 hips were evaluated. The measurement included: acetabular index, CE angle, Smith's h/b, c/b ratio, medial joint space, and center-head distance discrepancy (CHDD). All the patients were followed up until they were at least 10 years old. The overall results were evaluated using the modified Severins classification (group I & II, satisfactory; group III & IV, unsatisfactory). RESULTS: In the satisfactory group, the average acetabular index at 1 and 3 years after the pelvic osteotomy was <25 degrees. In the satisfactory group, the average CHDD 1 and 3 years after surgery was <6%. Eighty-two percent of hips with a CHDD <6% at postoperative 1 and 3 years were in the satisfactory group, whereas 81% of hips with CHDD >6% were in the unsatisfactory group. CONCLUSION: The most reliable factors for predicting normal acetabular development after surgery were an acetabular index <25 degrees and a CHDD <6%. If the hips do not meet the criterion of a CHDD <6% after the osteotomy of one component (either the pelvic or femur) then the other component must be osteotomized.
Acetabulum* ; Child ; Classification ; Dislocations* ; Hip* ; Humans ; Joints ; Osteotomy

A pathologic rupture of the flexor pollicis longus tendon secondary to Kienbock disease is extremely rare with only three cases being reported in the literature. We encountered a case of a pathological rupture of the flexor pollicis longus tendon secondary to longstanding Kienbock disease. The treatment included tendon ball insertion after excising the collapsed lunate and a flexor pollicis longus tendon reconstruction with autogenous palmaris longus tendons. Satisfactory results with a restoration of the active motion of the interphalangeal joint of the thumb, maintenance of the preoperative active range of motion of the wrist and markedly reduced pain was achieved after a one year follow up. We describe this case with a review of the relevant literature.
Follow-Up Studies ; Joints ; Osteonecrosis ; Range of Motion, Articular ; Rupture* ; Tendons* ; Thumb ; Wrist

Background: Community-acquired pneumonia (CAP) is the most important cause of hospitalisation in Malaysia and the 6th most important cause of mortality in patients aged 65 years and above. CAP is a lower respiratory tract infection that includes signs and symptoms like cough, fever, dyspnoea, the presence of new focal chest signs and new radiographic shadowing with no prior cause. To assist clinical judgement in deciding whether to admit the patient for in-ward treatment or otherwise, the severity of CAP is most commonly graded using the CURB-65 score as the components are more readily accessible in the Accidents and Emergency Department. We believe that cardiopulmonary diseases, immunosuppressive diseases like HIV infection or diabetes mellitus and other co-morbidities may affect the severity of CAP and are thus aspects of a patients’ history that should play a more significant role in influencing a clinician’s judgement of CAP severity. The general objective of the study is therefore to identify the relationship between co-morbidities and initial severity assessment of a patient admitted for community acquired pneumonia. The 3 specific objectives are i) to determine if presence of co-morbidities affects initial severity assessment in a patient admitted with CAP ii) To identify which co-morbidities affects initial severity assessment and iii) to determine whether having multiple co-morbidities increases initial severity assessment. Methodology: A retrospective study was carried out from the month of February 2013 to July 2013 at Hospital Tuanku Ja’afar, Seremban (HTJS). Patients admitted to the four Medical wards – 6A, 6B, 7A, and 7B – from July 2012 to December 2012 and have been diagnosed with CAP were chosen. A checklist was used as a survey instrument. Using statistical analysis, the severity of CAP in patients was compared in patients with different factors like gender, different co-morbidities and the number of co-morbidities. Results: A total of 63 patients in the control group had no co-morbidities and 54 patients were of low risk, 7 patients had moderate risk, and 2 patients had high risk CAP. Of the remaining 337 patients in the sample population, 124 patients had one co-morbidity, while 213 patients had multiple co-morbidities. Among those with a single co-morbidity, 100 patients had low risk, 19 patients had moderate risk, and 5 patients had high risk CAP. For the group with multiple co-morbidities, 135 patients had low risk, 58 patients had moderate risk, and 20 patients had high risk CAP. This study found that the presence and number of co-morbidities present in a patient affected the severity of CAP. Co-morbidities like diabetes mellitus, hypertension and asthma had significant correlation to the severity of CAP in patients. The gender of the patient had no significant correlation to the severity of CAP. Conclusion: The presence and number of co-morbidities present in a patient increases the severity of CAP. Hypertension, diabetes mellitus, and asthma are comorbidities that are prerequisites for increased caution and alert when judging the severity of CAP in patients. Comparison of patients with single and multiple comorbidities showed that patients in the latter group present with higher severity scores (p-value = 0.004).
Morbidity

Objective To evaluate the accordance of chronic kidney disease (CKD) staging between the CKD-EPI2009 equation, the CKD-EPI2012 equation and the modification of diet in renal disease (MDRD) equation and compare the predictive value of common cardiovascular disease. Methods A total of 11151 adults from Jurong area, Jiangsu province, were surveyed from September to November in 2015 and their serum creatinine and cystatin C were detected. The glomerular filtration rate (GFR) was estimated by three equations. Results In the individuals with history of chronic renal insufficiency, the results of CKD staging of CKD-EPI2009 equation and CKD-EPI2012 equation were all consistent with that of MDRD equation (P<0.001), and the consistence between CKD-EPI2012 equation and CKD-EPI2009 equation was even higher. In the people without history of CKD, the results of CKD staging of CKD-EPI2009 equation and CKD-EPI2012 equation were also highly consistent with the results of MDRD equation (P<0.001) and Ka ppa values were 0.662 and 0.654 respectively whilst the results of CKD staging estimated by CKD-EPI2012 equation and MDRD equation were only moderately consistent (Ka ppa=0.436, P<0.001). In the whole observational population, the CKD staging results of MDRD equation, CKD-EPI2009 equation and CKD-EPI2012 equation had a good consistency evaluated by Band-Altman method. The consistency of CKD staging between CKD-EPI (2009, 2012) equation and MDRD equation was higher in ≥70 years old group than that in<70 years old group as well as in males than in females. For predicting hypertension, the AUCs of CKD-EPI equations calculated GFRs were significantly higher than that of MDRD equation;the AUCs of CKD-EPI2012 equation calculated GFR for predicting stroke and coronary heart disease were higher than that of MDRD equations whereas no significant difference in GFR prediction result was found between CKD-EPI2009 equation and MDRD equation. Conclusion MDRD equation and CKD-EPI equation for GFR estimation have high consistency in CKD staging whilst the predictive value of chronic cardiovascular disease by CKD-EPI equation estimated GFR was higher than that of MDRD equation.