A 53-year-old man presented with prunosus nodules and plaques on the trunk and extremities for half a month and with periorbital swelling for 4 days.Hematological examination in a local hospital showed thrombocytopenia,and pulsed corticosteroid therapy did not work.On physical examination,there were splenomegaly and multiple enlarged superficial lymphnodes.Serum calcium and 1-lactate dehydrogenase (LDH)were increased to 3.12 mmol/L and 853 U/L,respectively.Serum immunofixation electrophoresis evidenced the presence of a monoclonal immunoglobulin IgM (κ chain).Positron emission tomography (PET-CT) showed abnormal uptake in multiple lymph nodes,back wall of the pharynx,and spleen.The biopsy of a nodule in the neck revealed a diffuse infiltration of numerous atypical lymphoid cells in the subcutaneous fat tissue,which were medium-sized with round nuclei,obvious nucleoli and karyokinesis, Immunophenotyping of the abnormal lymphocytes indicated positive reactions for L26,CD79a,Bcl-2,cyclin D1,multiple myeloma oncogene 1 (partly),Ki-67 (＞80％),but negative for CD5,CD21,CD23,CD38,CD3,CD10,Bcl-6,CD45RO,terminal deoxynucleotidyl transferase (TdT),myeloperoxidase (MPO),CD30,anaplastic lymphoma kinase (ALK),CD117,or CD34.Fluorescence in situ hybridization (FISH) revealed the presence of a fusion gene (t(11:14) CCND1/IGH) in the abnormal lymphocytes.Based on the above findings,the diagnosis was made as blastoid mantle cell lymphoma with skin and periorbital involvement complicated by hypercalcemia.After treatment with rituximab injection,cyclophosphamide,vincristine,doxorubicin,dexamethasone,and intermittent treatment with intravenous high dose of methotrexate and cytarabine (R-Hyper-CVAD),serum calcium returned to a normal level three days later,and the patient made a quick and excellent recovery on the 6th day,with the regression of skin lesions and poriorbital swelling.Unfortunately,the patient eventually died of severe pulmonary infection one month later.

The nail-patella syndrome is a genetically determined autosomal dominant disease with variabIe expression affectiag tissues of both ectodermal and mesodermal origin. The condition in an individual is characterized chiefly by dysplasia of the fingernails, hypoplastic or absent patellae, deformities or luxation af the head of the radius, and the presence of illiac horns. It is now well established that the locus for the nail-patella syndrome and the locus for the ABO blood groups are closely linked. We report 2 cases of nail-patella syndrome affected a brother and a sister in a family. Our 2 cases, with blood group B, showed dystrophic nails and hypoplastir. patellae.
Animals ; Blood Group Antigens ; Congenital Abnormalities ; Ectoderm ; Head ; Horns ; Humans ; Mesoderm ; Nail-Patella Syndrome ; Nails ; Patella* ; Radius ; Siblings

OBJECTIVETo understand the status and problems of complementary feeding and to explore the strategy of improving complementary feeding for infants and young children in China.

METHODData of urban and rural children were selected from the China Food and Nutrition Surveillance System (CFNSS) dataset to describe the status of complementary feeding and growth of children. Logistic Regression was used to analyze the relationship between child growth and the complementary feeding pattern.

RESULTSApplications of foods that come from animals, vegetable/fruit and dairy product in complementary feeding were negatively correlated to the prevalence of stunting and underweight. Attributable risk (AR) of no application of vegetable/fruit in complementary feeding to stunting was 30.2%, to underweight 35.4%; AR of no application of foods from animal products in complementary feeding to stunting was 28.2%, to underweight 11.7%; and the AR of no application of diary products in complementary feeding to stunting was 27.4%, to underweight was 15.9%.

CONCLUSIONComplementary feeding contributed to the improvement of infants and young children growth in China. There is an urgent need to develop health education in the public on infants and young children complementary feeding, in the mean time, it is pressing to pay attention and take actions to develop and promote affordable, acceptable, and accessible fortified complementary food in rural areas of China.

Child Development ; Child, Preschool ; China ; Dairy Products ; Fruit ; Humans ; Infant ; Infant Nutritional Physiological Phenomena ; Meat ; Nutrition Surveys ; Vegetables

Purpose of this study was to investigate the biomechanical fates of the partial thickness tear of the rotator cuff in rabbit, and try to provide guideline of treatment for the partial thickness tear of the rotator cuff tendon. Infraspinatus tendons of fifty rabbits were used for study. The rabbits were divided into three groups, twenty for one-third resection(superficial partial resection group), twenty for two-thirds resection(deep partial resection group) and ten for control group. Extraarticular portion of the infraspinatus tendons of the rabbits were resected partially with one-third or two-thirds thickness for each experimental group. We sacrificed five rabbits of each experimental group at 3, 6, 9, and 12 weeks after the resections, and five rabbits for the control group at 6 and 12 weeks. Average maximal load per area of the infraspinatus tendons was measured by tension test. There was significant difference between the deep partial resection group, 5.3 and 6.3 N/emat 6 and 12 weeks respectively, and the control group, 13.2 N/mm(P<0.05). There was no difference between the control and superficial partial resection group, 12.8 and 14.1 N/mm at 6 and 12 weeks, respectively. Shallow partial tears might be healed spontaneously. Deep partial tears would not be healed naturally. When surgical treatment is indicated for deep partial tears of the rotator cuff, tendon repair, rather than debridement, would provide more predictable results.
Debridement ; Rabbits* ; Rotator Cuff* ; Shoulder ; Tendons

Richer's syndrome is a development of a high grade malignant lymphoma in a patient with preexisting chronic 1ymphocytic leukemia, small lymphocytic lymphoma or Waldenstrom's macroglobulinemia. A rare case of Richer's syndrome arising in the spleen of a 35-year-old-man was studied by morphology, immunohistochemistry and gene rearrangement study. He has had weight loss and night sweat for last 6 months. Hepatosplenomegaly and abdominal lymphadenopathy were noted on CT scanning. Especially an ovoid radiolucent mass was found within the image of splenomegaly. Lymph nodes and liver biopsy, bone marrow aspiration and splenectomy were done. In the lymph nodes, liver and bone marrow, well differentiated small lymphocytic infiltrations were found but, in the spleen, pleomorphic, large cells with occasional multinucleated giant cells formed a nodular mass surrounded by diffuse, extensive infiltration of small well differentiated lymphocytes. The two distinctive areas in the spleen had positive staining for B-cell marker (HLA-DR and L26), negative staining for T-cell marker (UCLH1), and positive staining for IgM heavy chain and kappa light chain by immuohistochemical study. so this case was diagnosed as a diffuse large cell 1ymphoma transformed from small lymphocytic lymphoma. We made an another effort to clarify their clonality. Gene rearrangement method usingcomplementarity.determining region 3(CDR3) of immunoglobulin heavy chain (IgH) gene and T-cell receptor gamma (TCRgamma) gene by polymerase chain reaction (PCR) technique was done. The two lymphomas in the spleen demonstrated the same rearrangement pattern in both IgH and TCRgamma gene. We think these findings strongly suggest that the large cell lymphoma has the same clonality with that of the small lymphocytic lymphoma.
Male ; Humans

A retrospecive clinical observation was done on 325 cases of hypertension admitted to Department of internal Medicine of Seoul Red Cross Hospital from January, 1979 to December, 1982. The following results have been obtained. 1) The observed patients were 157 males and 168 females, and the prevalence rate of malignant hypertension among 325 hypertensions was 16 cases (4.9 percent). Among 16 cases of malignant hypertension 9 cases were male and 7 cases were female. Most frequently in 50, 60 and 40 years of age in this order each sex. 2) The average admitted dates are 7 days. 27 cases (8.3percent) were readmitted above 2 times after one discharged. 3) The most freqeuntly observed duration of Known hypertension 37.9 percent was 6-10 years and the next frequently observed duration 37.4 percent was 3-5 years. 4) The highest percentage of malignant hypertension had systolic blood pressure between 190-209mmHg and diastolic blood pressure between 150-169mmHg. 5) The highest percentage of seasonal number was spring (31.1 percent) and the next autumn, summer, winter in this order and the prevalence rates of hypertention are increased average 1.9% of each year. 6) The 78.5 percent of the patients were found that their blood pressure were abnormaly high when they were examined with related symptoms on admission, and they major symptorms were headache and dizziness. 7) The physical examination on admission, tachycardia (56percent), tachypnea (15.1percent), semicoma to coma (12.9 percent) in this order. 8) The status of treatment in hypertension on admission was as follows; imtermittent treatment 54.5 percent, no treatment 31 percent and continuous treatment 14.5 percent in this order. 9) In the chest X-ray on admission, the most frequent finding was cardiomegaly, and normal finding was the next frequency. The pulmonary Tbc was noted in 12.6 percent. 10) According to the degree of fundoscopic abnormality by K-W classification stage III was 68.8 percent and stage IV was 31.2 percent. 11) Electrocardiogram abnormalities were 44.9 percent, and 87.5 percent of malignant hypertension revealed electrocardiographic abnormalities and left ventricle hypertrophy was observed in 70.5 percent. 12) Hypercholesterolemia 78 percent, Hypernatremia 69.8 percent, Hypokalemia 22.8 percent, serum chloride 34.5 percent (<95m Eg/l), BUN 53.8 percent (>20mg/dl) and creatinine 45.8 percent (>3mg/dl) on admission. 13) Proteinuria was observed in 87.5 percent of malignant hypertension. 14) Marked improved for clinically was 24 percent and mortality rate was 5.2 percent, most common cause of death are cardiovascular accident (64.7percent) and renal failure (23.5percent).
Blood Pressure ; Cardiomegaly ; Cause of Death ; Classification ; Coma ; Creatinine ; Dizziness ; Electrocardiography ; Female ; Headache ; Heart Ventricles ; Humans ; Hypercholesterolemia ; Hypernatremia ; Hypertension ; Hypertension, Malignant ; Hypertrophy ; Hypokalemia ; Internal Medicine ; Male ; Mortality ; Physical Examination ; Prevalence ; Proteinuria ; Red Cross ; Renal Insufficiency ; Seasons ; Seoul ; Tachycardia ; Tachypnea ; Thorax

Patients with myelodysplastic syndromes (MDS) become dependent on blood transfusions and develop into transfusional iron overload, which is exacerbated by increased absorption of dietary iron in response to ineffective erythropoiesis. However, it is uncertain whether there is an association among iron accumulation, clinical complications, and decreased likelihood of survival in MDS patients. Thereby our current understanding of the effects of transfusion dependency and iron overload in MDS are discussed. Particular emphasis should be placed on further characterizing the role of redox-active forms of labile iron and oxidative stress in iron overload, decreased life expectancy and increased risk of leukemic transformation in MDS patients with iron overload.
Humans ; Iron ; metabolism ; Iron Overload ; Myelodysplastic Syndromes ; metabolism ; physiopathology ; Oxidative Stress

OBJECTIVETo investigate the role of gap junction in ischemic preconditioning (IPC).

METHODSSprague-Dawley rats were subjected to a 30 min coronary artery occlusion followed by 4 h of reperfusion (I/R). Rats were divided into seven groups: I/R, IPC/R, IPC/R + 5-hydroxydecanoic acid (mitochondrial ATP sensitive potassium channel antagonist), I/R + diazoxide (mitochondrial ATP sensitive potassium channel agonist), I/R + 5-hydroxydecanoic acid + diazoxide, I/R + 18beta-glycyrrhetinic acid (gap junction blocker) and I/R + 18beta-glycyrrhetinic acid + 5-hydroxydecanoic acid. Hemodynamics and myocardial infarct size were measured and connexin43 phosphorylation and subcellular distribution were determined by quantitative immunoblotting and confocal immunofluorescence.

RESULTSInfarct size was reduced in IPC/R, I/R + diazoxide and I/R + 18beta-glycyrrhetinic acid group (13.34% +/- 7.87%, 11.02% +/- 2.24%, and 15.03% +/- 11.35%, respectively; P < 0.001 vs. I/R group: 45.81% +/- 7.91%). 5-hydroxydecanoic acid abolished the cardioprotective effects of IPC and diazoxide (46.57% +/- 5.36% and 47.36% +/- 3.17%; P > 0.05 vs. I/R) but not the effects of glycyrrhetinic acid (14.60% +/- 7.36%; P < 0.001 vs. I/R). Phosphorylation of connexin43 was significantly increased, dephosphorylation and connexin43 intracellular redistribution significantly decreased (Cx43 size in the cellular membrane 1.00% +/- 0.35% and 0.83% +/- 0.31%, P < 0.001 vs. I/R: 0.19% +/- 0.06%) by IPC and diazoxide and these effects could be abolished by 5-hydroxydecanoic acid.

CONCLUSIONIschemic preconditioning could reduce myocardial infarction size by activating mitochondrial ATP sensitive potassium channel and modulating connexin43 phosphorylation and internalization.

Animals ; Connexin 43 ; metabolism ; Gap Junctions ; physiology ; Ischemic Preconditioning, Myocardial ; Male ; Myocardial Infarction ; metabolism ; pathology ; Phosphorylation ; Rats ; Rats, Sprague-Dawley

OBJECTIVETo explore the effect of in-home fortification of complementary feeding on intellectual development of Chinese children aged below 24 months.

METHODSOne thousand and four hundred seventy eight children aged 4-12 months were recruited and divided into study groups (formula 1 group and formula 2 group) and control group. In two study groups, in addition to the usual complementary food, children were fed with a sachet of fortified food supplement each day. Protein and micronutrients were provided in formula 1 group. Formula 2 group had the same energy intake as the formula 1 group . In addition to measurement of physical growth and detection of hemoglobin level, Development Quotient (DQ) or Intelligence Quotient (IQ) was assessed.

RESULTSThe DQ of children aged below 24 months was 97.2, 95.5, and 93.8 in formula 1 group, formula 2 group and control group, respectively, and the differences were statistically significant (P < 0.05). The DQ of children in formula 1 group, formula 2 group, and control group was 92.7, 90.4, and 88.3 respectively in the first follow up showing statistically significant differences (P < 0.05). And, DQ of children in formula 1 group, formula 2 group and control group were 96.7, 94.5, and 93.7 respectively in the second follow up, showing statistically significant differences (P < 0.05). Full-IQ of children in the formula 1 group was 3.1 and 4.5 points higher than that in formula 2 group and in control group respectively. Verbal IQ of children in the formula 1 group was 2.1 and 5 points higher than that in formula 2 group and control group respectively. Performance IQ was 2.5 and 3.1 points higher than that in formula 2 group and control group respectively. All above mentioned comparisons were statistically significant.

CONCLUSIONFortification of complementary feeding showed persistent effect on intelligence development of young children which could persist to 6 years of age. The critical time for correction of anemia could be under 18 months.

Asian Continental Ancestry Group ; Child Development ; China ; Dietary Supplements ; Female ; Humans ; Infant ; Infant Nutritional Physiological Phenomena ; Intelligence Tests ; Male

BACKGROUND AND OBJECTIVES: The purpose of this study is to investigate whether Ginkgo biloba extract (EGb 761) can suppress IL-1beta-induced MUC5AC gene expression in NCI-H292 human airway epithelial cells and to discover what its possible mechanism is. MATERIALS AND METHODS: NCI-H292 human pulmonary mucoepidermoid carcinoma cell line was used. MUC5AC mRNA and protein were measured using the RT-PCR and Western blot analysis. The activation of mitogen activated protein kinases (MAPKs) were determined by means of the Western blot analysis. RESULTS: MUC5AC was induced by treating the NCI-H292 cells with 10 ng/ml of IL-1beta for 24 hours. A pre-treatment of 200 microgram/ml of EGb 761 significantly suppressed the IL-1beta induced MUC5AC expression. The inhibition of MUC5AC gene expression by EGb 761 was noted to be suppressed via both the extracellular signal-regulated kinase (ERK) and the p38 MAPK pathways in a kinase-specific inhibitor study. CONCLUSION: EGb 761 suppresses IL-1beta-induced MUC5AC gene expression in the human airway epithelial cells. Therefore, it may be considered as a possible anti-hypersecretory agent.
Blotting, Western ; Carcinoma, Mucoepidermoid ; Cell Line ; Epithelial Cells* ; Gene Expression* ; Ginkgo biloba* ; Humans* ; Interleukin-1beta ; Mitogen-Activated Protein Kinases ; Mucins ; p38 Mitogen-Activated Protein Kinases ; Phosphotransferases ; RNA, Messenger