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Objective@#To describe the procedure for early corneal epithelial basement membrane(EBM) repair and regeneration in rabbits with corneal penetrating injury.@*Methods@#Forty-two New Zealand white rabbits were divided into modeling 1-, 3-, 5-, 7-, 14-, 21-, and 30-day groups using a random number table method, with 6 rabbits in each group; the right eyes were selected as the experimental eyes.Another 6 New Zealand white rabbits without any treatment were taken as the normal control group.A 2.0-mm trephine was used to ablate a full-thickness button of the central corneal tissue of each rabbit.The corneas were observed by slit lamp biomicroscopy at the respective time points after the trephined injury.Corneal epithelial fluorescein staining was used to evaluate re-epithelialization with Image J software and haze grading was evaluated with the Fantes classification.Hematoxylin-eosin staining was used to observe the healing process of the cornea.Transmission electron microscopy was conducted to assess the regeneration of the EBM and the reconstruction of the cornea.The study protocol was approved by the Ethics Committee of Guangxi Medical University (No.201811031). The use and care of the experimental animals complied with the Statement for the Use of Animals in Ophthalmic and Vision Research.@*Results@#The corneal epithelial fluorescein areas in modeling 1-, 3-, 5-, 7-, and 14-day group were (4.00±0.10), (3.11±0.10), (2.00±0.06), (0.90±0.04) and (0.67±0.03)mm2, respectively, with a significant difference among them (F=3 398.88, P<0.01). With the increasing of time after modeling, the corneal epithelium fluorescein area was gradually reduced, showing significant differences between any two groups (all at P<0.05), and the staining was disappeared at 21 and 30 days after modeling.The corneal haze grades were 3.44±0.53, 0.67±0.25, 1.33±0.50, 2.11±0.60, 2.44±0.53, 3.22±0.44 and 3.78±0.44 in modeling 1-, 3-, 5-, 7-, 14-, 21-, and 30-day group, respectively. The corneal opacity score gradually decreased during 1-5 days after modeling and gradually increased during 5-30 days after modeling, with a significant difference among them (F=51.182, P<0.01). Hematoxylin-eosin staining revealed that a fibrin clot formed in the wound area, and a single layer of epithelium covered the area at initial 3 days after modeling, and a large number of fibroblasts and some extracellular matrix were found at 5 days after modeling.At 21 and 30 days after modeling, the collagen fibers were tightly arranged in the anterior stroma.Transmission electron microscopy showed that the wound was filled with irregular collagen fibers and myofibroblasts.The stroma was remodeled at 21 days after modeling, and defective regeneration of the EBM was detected at 21 and 30 days after modeling.@*Conclusions@#Corneal fibrosis initiates after corneal penetrating injury in rabbits and gradually aggravates, the EBM regenerates defectively.
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Basement membranes are highly specialized forms of extracellular matrix and their formation is a prerequisite for normal tissue development and function.Basement membranes are important components of cornea.There are two types of basement membranes in cornea: epithelial basement membrane (EBM) and Descemet's basement membrane (DBM). After injury, the cornea assembles and regenerates basement membranes through binding interactions among laminins, type Ⅳ collagens, nidogens and proteoglycans.Defective or delayed regeneration of basement membranes leads to corneal stromal fibrosis, while reconstruction of basement membranes structure and function contribute to the remolding of corneal stroma with restoration of partial or full corneal transparency.The repair of corneal epithelium and endothelium, irregularity of injury surface and remnants of keratocytes all affect reconstruction of basement membrane's structure and function.This paper reviewes the components of corneal basement membranes and their functions, the associations of basement membranes and stromal fibrosis, basement membranes' coordinating modulation of stromal fibrosis and factors associated with the regeneration of basement membranes during the corneal wound healing.
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Objective To describe the procedure for early corneal epithelial basement membrane (EBM) repair and regeneration in rabbits with corneal penetrating injury.Methods Forty-two New Zealand white rabbits were divided into modeling 1-,3-,5-,7-,14-,21-,and 30-day groups using a random number table method,with 6 rabbits in each group;the right eyes were selected as the experimental eyes.Another 6 New Zealand white rabbits without any treatment were taken as the normal control group.A 2.0-mm trephine was used to ablate a full-thickness button of the central corneal tissue of each rabbit.The corneas were observed by slit lamp biomicroscopy at the respective time points after the trephined injury.Corneal epithelial fluorescein staining was used to evaluate re-epithelialization with Image J software and haze grading was evaluated with the Fantes classification.Hematoxylin-eosin staining was used to observe the healing process of the cornea.Transmission electron microscopy was conducted to assess the regeneration of the EBM and the reconstruction of the cornea.The study protocol was approved by the Ethics Committee of Guangxi Medical University (No.201811031).The use and care of the experimental animals complied with the Statement for the Use of Animals in Ophthalmic and Vision Research.Results The corneal epithelial fluorescein areas in modeling 1-,3-,5-,7-,and 14-day group were (4.00±0.10),(3.11±0.10),(2.00±0.06),(0.90±0.04) and (0.67 ± 0.03)mm2,respectively,with a significant difference among them (F =3 398.88,P ＜ 0.01).With the increasing of time after modeling,the corneal epithelium fluorescein area was gradually reduced,showing significant differences between any two groups (all at P＜0.05),and the staining was disappeared at 21 and 30 days after modeling.The corneal haze grades were 3.44±0.53,0.67±0.25,1.33±0.50,2.11±0.60,2.44±0.53,3.22±0.44 and 3.78±0.44 in modeling 1-,3-,5-,7-,14-,21-,and 30-day group,respectively.The corneal opacity score gradually decreased during 1-5 days after modeling and gradually increased during 5-30 days after modeling,with a significant difference among them (F =51.182,P＜0.01).Hematoxylin-eosin staining revealed that a fibrin clot formed in the wound area,and a single layer of epithelium covered the area at initial 3 days after modeling,and a large number of fibroblasts and some extracellular matrix were found at 5 days after modeling.At 21 and 30 days after modeling,the collagen fibers were tightly arranged in the anterior stroma.Transmission electron microscopy showed that the wound was filled with irregular collagen fibers and myofibroblasts.The stroma was remodeled at 21 days after modeling,and defective regeneration of the EBM was detected at 21 and 30 days after modeling.Conclusions Corneal fibrosis initiates after corneal penetrating injury in rabbits and gradually aggravates,the EBM regenerates defectively.
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Objective:To investigate the application value of Clavien-Dindo classification in evaluation of postoperative short-term complications of Da Vinci robotic-assisted or laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy. Methods:The retrospective cohort study was conducted. The clinicopathological data of 262 patients with gastric cancer who were admitted to the 940th Hospital of Joint Logistic Support Force of Chinese People′s Liberation Army from January 2016 to January 2019 were collected. There were 214 males and 48 females, aged (58±11) years, with a range from 17 to 81 years. Of 262 patients, 120 cases undergoing Da Vinci robotic-assisted total gastrectomy + D 2 lymphadenectomy + Roux-en-Y anastomosis were divided into robotic group, and 142 cases undergoing laparoscopic-assisted total gastrectomy + D 2 lymphadenectomy + Roux-en-Y anastomosis were divided into laparoscopic group. Observation indicators: (1) intraoperative and postoperative situations; (2) postoperative pathological examination; (3)complications; (4) stratified analysis; (5) follow-up. Follow-up using outpatient examination and telephone interview was performed to detect complications, tumor recurrence and survival of patients within postoperative 2 months. The follow-up was up to May 2019. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was analyzed using the t test. Measurement data with skewed distribution were represented as M (range), and comparison between groups was analyzed using the Mann-Whitney U test. Count data were described as absolute numbers or percentages, and comparison between groups was analyzed using the chi-square test or Fisher exact probability. Comparison of ranked data between groups was analyzed using the rank sum test. Results:(1) Intraoperative and postoperative situations: cases undergoing conversion to open surgery, the operation time, volume of intraoperative blood loss, the number of lymph node dissected, time to first flatus, time to initial fluid diet intake, duration of postoperative hospital stay of the robotic group were 1, (243±42)minutes, 100 mL(range, 100-150 mL), 38±15, (2.8±1.0)days, 3 days(range, 3-4 days), 11 days(range, 9-13 days), respectively. The above indicators of the laparoscopic group were 2, (244±38)minutes, 100 mL(range, 100-150 mL), 34±14, (3.2±1.0)days, 4 days(range, 3-5 days), 10 days(range, 9-13 days), respectively. There were significant differences in the number of lymph node dissected, time to first flatus, time to initial fluid diet intake between the two groups ( t=2.068, -3.030, Z=-3.370, P<0.05), and there was no significant difference in cases undergoing conversion to open surgery, the operation time, volume of intraoperative blood loss, duration of postoperative hospital stay between the two groups ( χ2=0.000, t=-0.158, Z=-1.824, -0.088, P>0.05). (2) Postoperative pathological examination: cases with well differentiated tumor, moderately differentiated tumor, poorly differentiated tumor, signet ring cell carcinoma or other types of tumor, cases in stage T1b, T2, T3 or T4a (pT staging), cases in stage N0, N1, N2, N3a or N3b (pN staging), cases in stage ⅠB, ⅡA, ⅡB, ⅢA, ⅢB or ⅢC (pTNM staging) of the robotic group were 6, 50, 55, 9, 10, 22, 63, 25, 42, 19, 19, 24, 16, 17, 22, 23, 20, 23, 15, respectively. The above indicators of the laparoscopic group were 4, 42, 84, 12, 6, 18, 81, 37, 39, 27, 32, 19, 25, 13, 19, 28, 39, 16, 27, respectively. There was no significant difference in the above indicators between the two groups ( Z=-1.880, -1.827, -0.140, -1.460, P>0.05). (3) Complications: cases with complication classified as grade Ⅰ, grade Ⅱ, grade Ⅲa, grade Ⅲb, grade Ⅳa, grade Ⅳb of Clavien-Dindo classification, cases with death, cases with overall complications, cases with severe complications of the robotic group were 9, 6, 3, 2, 2, 0, 0, 22, 7, respectively. The above indicators of the laparoscopic group were 12, 15, 9, 6, 3, 1, 1, 47, 20, respectively. There were significant differences in cases with overall complications, cases with severe complications between the two groups ( χ2=7.309, 4.790, P<0.05), and there was no significant difference in cases with complication classified as grade Ⅰ, grade Ⅱ, grade Ⅲa, grade Ⅲb, grade Ⅳa, grade Ⅳb of Clavien-Dindo classification, cases with death between the two groups ( χ2=0.080, 2.730, 1.042, 0.704, 0.000, 0.000, 0.000, P>0.05). (4) Stratified analysis: of the patients with overall complications in robotic group, cases of male or female, cases aged ≥65 years or <65 years, cases with body mass index (BMI) ≥24 kg/m 2 or <24 kg/m 2, cases with tumor diameter ≥5 cm or <5 cm, cases with or without abdominal surgery, cases with tumor located at upper stomach or middle stomach, cases in Ⅰ-Ⅱ grade or Ⅲ grade of American Society of Anesthesiologists (ASA) classification, cases with well differentiated tumor or undifferentiated tumor, cases in stage Ⅰ-Ⅱ or stage Ⅲ (pTNM staging), cases with operation time ≥250 minutes or <250 minutes, cases with volume of intraoperative blood loss ≥150 mL or <150 mL, cases with the number of lymph node dissected ≥25 or <25 were 15, 7, 14, 8, 11, 11, 16, 6, 4, 18, 19, 3, 15, 7, 7, 15, 8, 14, 12, 10, 12, 10, 14, 8, respectively. The above indicators of patients with overall complications in the laparoscopic group were 33, 14, 17, 30, 16, 31, 36, 11, 11, 36, 27, 20, 31, 16, 13, 34, 14, 33, 24, 23, respectively. Of the patients with overall complication, there were significant differences in cases of male, cases aged ≥65 years or <65 years, cases with BMI<24 kg/m 2, cases with tumor diameter≥5 cm, cases without abdominal surgery, cases with tumor located at middle stomach, cases in Ⅰ-Ⅱ grade or Ⅲ grade of ASA classification, cases with well differentiated tumor, cases in stage Ⅲ (pTNM staging), cases with operation time ≥250 minutes, cases with volume of intraoperative blood loss <150 mL, cases with the number of lymph node dissected ≥25 between the two groups ( χ2=6.683, 4.207, 6.761, 7.438, 4.297, 6.325, 9.433, 3.970, 4.850, 4.911, 3.952, 3.915, 6.865, 4.128, P<0.05) and there was no significant difference in cases of female, cases with BMI≥24 kg/m 2, cases with tumor diameter <5 cm, cases with abdominal surgery, cases with tumor located at upper stomach, cases with undifferentiated tumor, cases in stage Ⅰ-Ⅱ (pTNM staging), cases with operation time < 250 minutes, cases with volume of intraoperative blood loss ≥150 mL, cases with the number of lymph node dissected <25 between the two groups ( χ2=0.277, 1.052, 1.996, 1.552, 2.172, 2.594, 2.244, 3.771, 1.627, 3.223, P>0.05). (5) Follow-up: 262 patients were followed up postoperatively for 2 months. During the follow-up, no patient was diagnosed with tumor recurrence, and one patient in the laparoscopic group died of severe infection. Conclusions:The Clavien-Dindo classification can be used in evaluating postoperative short-term complications of Da Vinci robotic-assisted or laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy. Compared with laparoscopic-assisted total gastrectomy with D 2 lymphadenectomy, Da Vinci robotic-assisted total gastrectomy with D 2 lymphadenectomy has the advantages of minimally invasiveness, low incidence of overall and severe complication.
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Objective To investigate the application of damage control surgical treatment in the patients with severe abdominal infection and its nursing experience. Methods Toally 10 patients with severe abdominal infection were treated with damage control measures, including damage control surgery, improvement of pathological and physiological status of patients and surgical re-repairing, and the corresponding nursing measures including rapid completion of preoperative preparation, prevention of complications, nursing care to abdominal double cannula drainage, enteral nutrition and disease observation and mobilization the subjective initiative of patients. Results The average hosptitalization time was 21 days (14~29 days). There were 2 cases of anastomotic leakage and 1 case of pulmonary infection who all were cured after 14~29 d. Conclusion Damage control surgery is an effective treatment strategy for patients with severe abdominal infection and the corresponding nursing plays a positive role in improving the treatment effect.
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Objective To explore the relationship of insulin resistance with serum 25-hydroxyvitamin D levels and arterial stiffness in elderly patients.Methods A total of 162 elderly inpatients (aged 60 years or over) were recruited from 2012 to 2014.Levels of fasting serum insulin,fasting serum glucose,creatinine and vitamin D were determined.Insulin resistance (IR) was evaluated by the homeostasis model assessment of insulin resistance (HOMA-IR).The patients were divided into the following three groups according to HOMA-IR:the low IR group with HOMA-IR less than 2 (n=78),the median IR group with HOMA-IR between 2 and 6 (n=43),and the high IR group with HOMA-IR greater than 6 (n=41).Arterial stiffness was assessed by carotid-femoral pulse wave velocity (cfPWV).Arterial stiffness and serum vitamin D levels were compared among the three groups.Results cfPWV was increased and the serum 25-hydroxyvitamin D level was decreased in the high insulin resistance group compared with the low insulin resistance group[(13.2± 5.7) μg/L vs.(17.8±6.2) μg/L,(14.3±5.2) m/s vs.(11.9±3.0) m/s].Multiple liner regression analysis showed that IR was negatively correlated with the serum 25 hydroxyvitamin D level (r=-0.63,P＜0.05) and positively correlated with arterial stiffness (r=0.45,P＜0.05) after adjustment for age,sex and other confounders.Conclusions Elderly patients with high insulin resistance may have lower levels of serum vitamin D and higher arterial stiffness.
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Objective To investigate the relationship of BPH in patients with newly diagnosed type 2 diabetes mellitus with age,weight,blood glucose levels.Methods Totally 141 cases with type 2 diabetes and BPH in elderly patients were discussed.Glucose level,the international prostate symptom score (IPSS),prostate volume (PV) and maximum urinary flow rate (Qmax) were determined.Results The levels of IPSS(10.9±2.5 vs.16.4±3.0,P＜0.01; 10.9±2.5 vs.19.4±3.3,P＜0.05) and PV(43.3±7.7 vs.47.9±10.5,43.3±7.7 vs.48.1±8.6,both P＜0.05) were increased in age group of 70-79 years and age group ≥ 80,Qmax in age≥ 80 years was decreased (12.0±2.6 vs.8.5±1.4,P＜0.05) compared with age group of 60-69 years.In contrast to BMI ≤ 24 kg/m2 group,IPSS score,PV and HOMA-IR were increased in BMI ≥ 28 kg/m2 (9.8±3.4 vs.18.8±4.1,P＜0.01; 41.1±10.8 vs.49.1±11.4,1.87±1.1 vs.3.89±2.0,P＜ 0.05).IPSS and PV were higher (12.6±5.7 vs.16.9±4.2,42.9±9.7 vs.48.3±10.6,P＜0.05),Qmax was lower (11.7±1.9 vs.9.0±1.6,P＜0.05) in patients with HbA1c ≥7％ versus HbA1c＜7％.Conclusions Age,obesity,high blood glucose and insulin resistance can promote the progress of BPH in the elderly newly diagnosed type 2 diabetic patients.Therefore,control of weight,and lowering the glycosylated hemoglobin may inhibit the progress of BPH.
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Objective To investigate the clinical and molecular pathological features of limb-girdle muscular dystrophy 2A (LGMD2A) of Chinese patients. Methods Thirty cases of LGMD with excluding LGMD2B were included in this study. The muscle specimens were performed by a standard series methods of histochemistry, enzymohistochemistry, immunohistochemistry and Western blot. The clinical and molecular pathological features of LGMD2A were retrospective analyzed. Results Five cases with no or only trace expression of calpain-3 protein were diagnosed as calpainopathy (LGMD2A) by Western blot analysis. The age of onset of these 5 patients ranged from 10 to 45 years and the duration of the disease were about 2-10 years. Proximal muscles weakness and atrophy of lower limbs were predominantly involved. In all patients,symptoms progressed slowly. The ambulation could be retained for many years but running and jumping were impaired early. The serum creatine kinase level was elevated moderately to markedly. Electromyography showed myopathic patterns in all cases. Two siblings had similar symptoms indicating autosomai recessive inherited pattern. Pathologically, there was marked variation in fibre size and most small fibres were round. Some necrotic and regenerating fibers were seen. Fibres with centrally placed nuclei can be found frequently. No infiltrations of inflammatory cells were seen. Lobulated fibers were observed in 2 patients by NADH-TR stain. The expression of dystrophin, caveolin-3, α-, β-, γ- and δ-sarcoglycan protein were normally staining of 5 LGMD2A patients' specimens by immunohistochemistry. Two patients had reduced staining of dysferlin by immunohistochemistry study. Conclusions Clinical and pathological characteristics of our 5 LGMD2A patients are consistent with typical muscular dystrophy features reported in other countries. Identification of calpian-3 deletion by Western blot is essential for the diagnosis of calpainopathy.
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Objective To explore the correlation between lower urinary tract symptoms (LUTS) with prostate volume and peak flow rate in aging staff men with benign prostatic hyperplasia (BPH). Methods A total of 180 elderly patients were randomly enrolled. They were diagnosed with BPH by rectal touch and transected ultrasound from April 2008 to December 2008. The international prostate symptom score (IPSS), prostate volume (PV) as well as peak flow rate (QMAX)were analyzed respectively. Results IPSS were ( 9. 1 ± 0. 7 ) scores, ( 12. 1 ± 0. 7 ) scores and (14.0±1.3) scores in 60-69 years old group, 70-79 years old group and more than 80 years old group. PV were (40. 6±1.9) ml, (42. 4±1.9) ml and (48. 7±2.8) ml in corresponding groups, and PV was elevated along with aging (F= 5. 705, 2. 983, P＜0. 05). QMAX were ( 14.7 ± 0. 6) ml/s,(14.0±0. 5) ml/s and (12.6±0.9) ml/s, and QMAX was decreased along with aging (F=2. 131, P＞0. 05). Along with aggravation of LUTS, PV (ml) increased (39. 2±18. 1 vs. 45.7±16.9 vs. 47. 9± 16. 5) and QMAX (ml/s) decreased ( 15.0 ± 4.8 vs. 13. 5 ± 5.06 vs. 11.5 ± 4. 7, F= 3. 427, 4. 742, P ＜0.05). Conclusions The LUTS of patients with BPH is aggravated with aging, at the same time, the degree of LUTS increases with PV and decreases with QMAX. If get active treatment of drugs if available, they may improve their quality of life.
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@#ObjectiveTo evaluate the significance of flexion priority strategy and the principle of joint mobilization technique in the rehabilitation of posttraumatic stiffness of the elbow. Methods24 patients with posttraumatic stiffness of the elbow were divided into 2 groups, 12 patients in each group. The treatment group was treated with flexion priority strategy of joint mobilization technique only in the treatment of flexion contracture. In order to decrease the pain caused by joint mobilization training of extension and achieve the restoration of flexion as early as possible, the rehabilitation of extension was altered to self-exercise under the consultation of doctors combined with gentle passive traction by the therapist. The control group was treated with joint mobilization technique of both flexion and extension once a day. All the patients were measured the flexion range, extension range, and flexion-extension arc after 6 weeks of rehabilitation. Results6 weeks after rehabilitation, the treatment group got 124° of flexion (range 95°～135°), much better than the control group of 95° (range 80°～110°) （P<0.01）. There was no significant difference of extension degrees between the treatment group (15°, range 10°～35°) and the control group (16°, range 10°～30°) （P＞0.05）. The final arc of flexion and extension in the treatment group had an increase of 53°, significantly greater than 30° in the control group（P<0.01）. ConclusionFlexion priority strategy of elbow rehabilitation can significantly restore the flexion function of posttraumatic elbow stiffness.
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Objective To explore the relationship between cognitive impairment and arterial stiffness in elderly patients. Methods A total of 142 elderly patients were enrolled. Cognitive function was assessed by mini-mental state examination (MMSE) and arterial stiffness was assessed by pulse wave velocity (PWV). A full score on the MMSE was 30, and cognitive impairment was defined as a score less than 24. All subjects underwent the measurement of PWV and MMSE . The subjects were divided into 2 groups: 93 were assigned to the normal cognitive function group (MMSE score 24), and the remainders (n=49) were assigned to the cognitive impairment group (MMSE score 24). Results The PWV was significantly increased in the cognitive impairment group than in the normal cognitive function group [(13.3±2.4) m/s vs. (11.8±2.2) m/s, t=3. 775, P=0. 000]. Logistic regression analysis showed that the PWV was also independently and significantly associated with the MMSE score. Conclusions The increase of arterial stiffness is an important risk factor for impaired cognitive function in elderly patients.
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12 m/s group,n=70;cfPWV≤12 m/s group,n=49). Carotid atheromatous plaque was assessed by Doppler echocardiograph;blood pressure and hemoglobin A1c were measured. Results After adjustment for body mass index (BMI),sex,stroke and fasting blood glucose,logistic multivariable analysis showed that cfPWV associated with age,systolic blood pressure(SBP),pulse pressure,coronary artery disease,diabetes,carotid atheromatous plaque and hemoglobin A1c. Conclusion Arterial stiffness in elderly patients with essential hypertension was strongly positively associated with age,SBP,pulse pressure,coronary artery disease,diabetes,carotid atheromatous plaque and hemoglobin A1c.
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Objective To investigate the features of clinical symptoms and pathological changes of mitochondrial encephalomyopathy. Methods 16 cases of mitochondrial encephalomyopathies were analyzed on clinical features, histochemical changes and ultrastructure observations of muscles.Results The average proportion of RRFs was 5.9% in 16 cases and central nuclears were present in 11 cases. There were blue fibers on SDH/CCO double stain sections in 12 out of 13 cases. The distribution of blue fibers was concordent with that of RRFs. There were crystal include body in mitochondria of 4 cases. Conclusions The diagnosis of mitochondrial encephalomyopathy could be made accoding to the presence of blue fibers on SDH/CCO double stain sections and the mitochondria encephalomyopathy could be distinguished from other myopathies.
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Objective To investigate the clinical and pathological features of distal myopathy with rimmed vacuoles(DMRV). Methods Clinical manifestations and pathological features of biopsied muscle specimens were summarized and analysed retrospectively. Muscle specimens were collected from biceps brachii (3 cases), tibialis anterior (2 cases), quadriceps femoris (1 case)and gastrocnemius (3 cases) respectively. 6 cases were observed by electronic microscope. Results The onset of disease was ranged in age from 15 to 33 years, averaging 25 years. It is more common in women than in men in a ratio of 2∶1. Distal muscle weakness and atrophy of the lower extremities was predominant in early stage. Varied involvement of proximal and trunk muscles, with sparing of the facial, extraocular, bulbar, intercostal and diaphragm muscles was recognized in the advanced stage. The striking and common pathologic finding was the presence of rimmed vacuoles in muscle fibers with little evidence of necrotic or regenerative processes. Electronic microscopic study showed an accumulation of myeloid structure in 5 cases, cytoplasmic inclusion bodies in 4 cases and intranuclear filamentous inclusions in 2 cases. A perforated nuclear envelope and extrusion of filaments in adjacent cytoplasm were found in an enlarged nucleus filled with tubulofilementous inclusion.Conclusion The clinical and pathological findings of DMRV occurred in China is basically similar to those reported by Japanese. Ultrastructural study suggested that sarcoplasmic filamentous inclusions might originate from perforated nuclei with inclusion body, which predicts that the rimmed vacuoles are likely to be the end products of nuclear disintegration and focal myofibrillar degeneration.
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Objective To investigate the clinical and pathological features of riboflavin-reactive lipid storage myopathy.Methods Clinical material of 4 patients with riboflavin-reactive lipid storage myopathy were analyzed retrospectively.Results All the patients were subacute onset and presented proximal and axial muscle weakness accompanied by intolerance to excise.Amyotrophy and weakness involved in neck and paraspinal muscle were found in 3 cases,and chewing muscle weakness in 2 cases.Electromyogram showed myogenic changes in 2 cases and reduced conductive velocity of tibial nerves in 1 case.Muscle Biopsy study showed markedly increased lipid droplets in muscle fibers.The ragged red fibers,succinate dehydrogenase strongly reactive vessels and COX deficiency fibers which supported mitochondrial myopathy were not detected.Electromicroscope observation revealed that the structure and quantity of mitochondria were normal.All the patients had a dramatic response to riboflavin treatment.Two cases were cured and the other two were improved significantly.Two cases relapsed 1 year and 5 years later,respectively,and riboflavin was still effective for them.Conclusions Riboflavin-reactive lipid storage myopathy is a myopathy characterized by preferential involvement of neck,paraspinal and chewing muscles.The distinct pathological features are lipid accumulation in muscle fibers without any abnormalities of mitochondrial structure and quantity.Riboflavin alone is effective for this myopathy and this is distinguished from other myopathies.
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Objective To investigate the clinical and pathological features of adult form nemaline myopathy. Methods Biopsied muscle specimens from two patients were observed using enzymatic histochemical method and electron microscope. Results These two patients were found with an initial onset from neck muscle weakness at the age of 52 and 36 years respectively, followed by trunk and limb muscle involvement to a variant degree. Type Ⅰ fiber atrophy and numerous granules (rod) were found in both patients in light microscopy. In case 1, the centralized nuclei were seen almost in all normal sized muscle fibers. Electron microscopic observation showed marked myofibril disorganization and numerous rod-like formations. Two inclusion-carrying nuclei were detected in case 1. The intranuclear inclusion showed a lattice pattern of Z-disc line or sarcoplasmic rod. Conclusions Adult form nemaline myopathy is clinically nonspecific. Rod-like structures might be found either in sarcoplasma or in nucleus. Centralized nuclei might coexist with rods in adult form nemaline myopathy. As compared to the child form, the adult one might often show a marked muscle fiber atrophy.
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Objective To investigate the clinical,genetic and pathological features of familial chronic progressive external ophthalmoplegia (CPEO) type of mitochondrial myopathy.Methods Clinical manifestations, family histories and pathological findings of 21 patients with CPEO type of mitochondrial myopathy from 3 families constellations were analyzed retrospectively.Results All the patients had ptosis and movement disorder of eyeball, with or without myasthenia. An autosomal dominant pattern of transmission was deduced from one family and a maternal transmission appeared most likely in the other two families. The striking and common pathologic findings were presence of ragged red fibers and cytochrome C oxidase (COX) deficiency fibers under microscope. Ultrastructural alterations included subsarcolemmal accumulation of mitochondria, increase of mitochondria with abnormal shape, disarrangement of cristae and paracrystaline inclusion bodies.Conclusions The clinical and pathological features between generations and families seem to be similar. It is suggested that different genetic mode of CPEO may lead to similar clinical and pathological features.