Segmental dyschromatosis with blue naevi and cherry angiomas: a new syndrome?

The dyschromatoses are a group of disorders characterized by the presence of both hyperpigmented and hypopigmented macules, many of which are small in size and irregular in shape. Localized dyschromatosis is a rare manifestation of dyschromatosis. Localized dyschromatosis showing segmental distribution may be a result of degeneration of localized cutaneous nerves. We report a case of localized dyschromatosis, with segmental distribution, with co-occurrence of blue naevi and cherry angiomas.

First Report of Fusarium mangiferae Causing Mango Malformation in China.

Mango (Mangifera indica L.) malformation caused by Fusarium mangiferae has been reported in many mango-growing regions of the world (3). The disease was also observed in Yunnan and Sichuan provinces of China (1). Typical symptoms in seedlings included loss of apical dominance, hyperplasia and hypertrophy of vegetative buds, shortened internodes, and leaves that were more brittle than those of healthy plants. Inflorescences were abnormally branched and thickened, with panicles producing as much as two to five times the normal number of flowers. Flowers in the malformed inflorescence were much more enlarged and crowded than the generally hypertrophied axes of the panicle, thus producing no fruit or aborting early. To identify the pathogen, samples of malformed and healthy mango seedlings were collected from the affected plantings. For isolation, portions of stems were cut into 3- to 4-mm segments, surface disinfested, dried, and then plated on potato dextrose agar and incubated at 25°C. Within 5 days, white, fluffy, aerial mycelium developed. With the aid of an inverted microscope, single conidia were transferred to carnation leaf agar (CLA) medium. After 10 days of incubation, morphological characteristics were found to be identical to those of F. mangiferae (4). Aerial mycelium was white with no pigmentation observed on potato sucrose agar. Pigmentation on rice medium was pink. On CLA medium, conidia grew in branched conidiophores with false heads bearing monophialides or polyphialides. No conidiospores in chains were observed. Microconidia were ovate to long and oval, 0 to 1 septate, and 3.1 to 10.2 × 1.5 to 2.2 µm. Macroconidia are falculate, 3 to 5 septate, and 18 to 38 × 1.8 to 2.4 µm. Chlamydospores were not observed. Pathogenicity studies were conducted with 7-month-old asymptomatic mango seedlings. These seedlings, except for the controls, were inoculated by injection of the isolated fungus in the axillary or apical bud position. A 1-ml spore suspension (1 × 106 spores/ml) was injected slowly into the stems using a microsyringe with three buds per seedling, for a total of 10 seedlings. Typical malformation symptoms developed within 3 to 4 months, and none of the plants inoculated with sterile water resulted in malformation symptoms. Reisolations from the induced malformed shoots yielded the same fungus, and no fungal growth was observed to be growing from the control plants. To confirm identity of the causal fungus, the gene encoding translation elongation factor 1 alpha (EF-1α) was amplified and sequenced (2). The EF-1α sequence was 660 bp long. The sequence (GenBank Accession No. HM068871) was 99.68% similar to sequences of FD_01167 in the Fusarium ID database. On the basis of symptoms, fungal morphology, the EF-1α region sequence, and pathogenicity testing, this fungus was identified as F. mangiferae. To our knowledge, this is the first report of F. mangiferae causing mango malformation in China. This report will establish a foundation for further study of F. mangiferae and effectively addressing the disease. References: (1) X. H. Chen. Pract. Technol. (in Chinese) 6:5, 1992. (2) D. M. Geiser et al. Eur. J. Plant Pathol. 110:473, 2004. (3) J. Kumar et al. Annu. Rev. Phytopathol. 31:217, 1993. (4) J. F. Leslie and B. A. Summerell. The Fusarium Laboratory Manual. Blackwell Publishing, Ames, IA, 2006.

Involvement of GRP78 in inhibition of HBV secretion by Boehmeria nivea extract in human HepG2 2.2.15 cells.

Previous studies showed that the root extract of Boehmeria nivea (BNE) can significantly suppress the production of hepatitis B virus (HBV) in vitro and in vivo. In this study, viral core and large-surface proteins accompanied with their encapsidated viral DNA were observed to accumulate within the cells. Notably, 78-kDa glucose-regulated protein (GRP78) was found to be suppressed by BNE, and stimulation of the GRP78 expression by thapsigargin could rescue virus production initially inhibited by BNE. The antiviral effect of BNE was reversible, which also coincided with the level of GRP78. Furthermore, we synthesized the GRP78 siRNA to knockdown the expression of GRP78 protein, and the production of supernatant HBV DNA was reduced simultaneously. Moreover, combined treatment of BNE and 3TC exhibited an additive anti-hepatitis B virus effect. In conclusion, the inhibitory effect of BNE on blocking assembled virion secretion might be via the reduction of GRP78.

Intraperitoneal bladder rupture presenting as acute bloody ascites and oliguric acute renal failure in an alcoholic liver cirrhosis patient.

Intraperitoneal bladder rupture is a rare cause of acute abdomen with bloody ascites. We report herein the case of a patient who had alcoholic liver cirrhosis and multiple liver nodules, and experienced acute bloody ascites and oliguric acute renal failure in association with intraperitoneal bladder rupture. A 33-year-old male suffered from acute abdominal pain and oliguria following consumption of a large amount of alcohol and after blunt abdominal trauma. He was also found to have acute renal failure and newly onset bloody ascites that rapidly subsided following transurethral catheter drainage. Computed tomography cystography revealed intraperitoneal extravasation of contrast from the dome of the bladder, suggestive of intraperitoneal bladder rupture. The patient received surgical repair and was discharged with full recovery. This case shows that it is important for physicians to be aware of the possibility of intraperitoneal bladder rupture after alcohol consumption accompanied with abdominal blunt trauma. In particular, it has diagnostic complications for underlying liver tumors.

Aristolochic acid nephropathy due to herbal drug intake manifested differently as Fanconi's syndrome and end-stage renal failure--a 7-year follow-up.

Aristolochic acid (AA) may reduce glomerular or proximal tubular function, or both. We report a married couple taking AA-containing herbal drugs. The man developed Fanconi's syndrome (FS) whereas his wife reached end-stage renal failure (ESRF). He was a 36-year-old alcoholic cirrhotic patient who had taken the Chinese herbal drugs for 6 years, presenting with muscle weakness and laboratory findings of FS; the renal pathological findings were compatible with the diagnosis of aristolochic acid nephropathy (AAN). His 38-year-old wife, who took a lower cumulative amount of the same herbal drug for a shorter duration, developed advanced renal failure and severe anemia with pathological findings of extensive tubular atrophy, interstitial fibrosis but spared glomeruli. AA-I was detected in one of the herbal drugs. The wife has been on hemodialysis for 7 years, but the husband is still at the stage of slowly progressive chronic renal failure and persistent FS. None of their 5 children ever took the herbal drug, and none had renal problems during follow-up. It is important to trace the history of herbal drug intake in all the family members because of the possibility of sharing of drugs within a family. In addition to the effect of cumulative doses of AAs and the potentially higher susceptibility of females to AAN, the roles of liver cirrhosis and related vasodilators in the protection of the renal interstitium from fibrosis are questions that warrant further study.

Long-term exposure to retrovirally expressed granulocyte-colony-stimulating factor induces a nonneoplastic granulocytic and progenitor cell hyperplasia without tissue damage in mice.

Murine marrow cells infected with a retroviral vector (MPZen) bearing a granulocyte-colony-stimulating factor (G-CSF) cDNA insert were transplanted into lethally irradiated recipients to study the effects of autocrine production of G-CSF in normal hemopoietic cells. Most animals remained healthy with no evidence of tissue damage throughout the observation period (4-30 wk) despite high circulating G-CSF levels (range 2,000-26,000,000 U/ml). A dramatic neutrophilic granulocytosis was observed in all hemopoietic tissues with neutrophilic infiltration occurring in the lung and liver. Spleen, peritoneal, and peripheral blood cellularity increased approximately three-, two-, and eightfold, respectively, but total bone marrow cell counts remained unchanged. Progenitor cell numbers granulocyte-macrophage colony-forming cell (GM-CFC), granulocyte colony-forming cell (G-CFC), burst-forming unit-erythroid (BFU-E), colony-forming unit-erythroid (CFU-E) and mixed colony-forming cells (Mix-CFC) were elevated between 10-100-fold in the spleen, peritoneal cavity, and peripheral blood, but were unaffected or slightly depressed in the marrow. No tumors developed in syngeneic recipients transplanted with bone marrow or spleen cells from such mice, confirming the nonneoplastic nature of the hyperplasia induced by chronic G-CSF stimulation. These experiments also indicated the stable integration of MPZen vectors in infected cells, as evident from the continuous expression of the inserted gene for at least 6 mo, and from the ability of infected stem cells from the primary recipients to express the gene in lethally irradiated secondary recipients.

[Effect of TSC2 gene expression downregulation by lentivirus induced RNA interference on U937 cell line and its mechanism].

Objective: To investigate the effect of biology and mTOR pathway activity of down-regulated TSC2 gene expression on U937 leukemia cells. Methods: Gene expression was down-regulated by lentivirus induced RNA interference on TSC2 high expressed U937 cell line; the proliferation, apoptosis and differentiation were detected by CCK-8 assay, colony formation assay and flow cytometry; the gene expression level and protein kinase activity were detected by qRT-PCR and Western blot. Results: Down-regulated expression of TSC2 gene promoted U937 cell proliferation and colony formation ability (P<0.05) . The proportion in G(0)/G(1) phase of TSC2 down-regulated U937 cell was much lower than that of the control cells [ (52.53±3.75) % vs (75.10±4.33) %, t=6.829, P=0.002], the S phase [ (22.43±1.00) % vs (15.47±1.20) %, t=-5.581, P=0.019] and G(2)/M phase [ (25.03±4.34) % vs (14.33±0.91) %, t=-5.413, P=0.013] was remarkably higher than that of the control cells (P<0.05) . There were no statistically significant differences in cell apoptosis and differentiation (P>0.05) . Down-regulation of TSC2 led to the increased activity of mTOR, 4EBP1 and S6K1, but did not influence the activity of AKT. The expressions of proliferation related cyclinD1, c-myc and PTEN were also up-regulated after TSC2 silenced, but the expressions of P27KIP and BCL-XL were not changed. Conclusion: Downregulation of TSC2 could promote the proliferation of U937 cells through up-regulation of mTOR activity.

Kaposiform hemangioendothelioma arising from the internal auditory canal.

Kaposiform hemangioendothelioma is a rare vascular tumor and locally aggressive endothelial-derived spindle cell neoplasm, which occurs almost exclusively in infants and adolescents. Radiologically, hemangioendothelioma, including Kaposiform hemangioendothelioma, is seen as a highly vascularized well-enhancing tumor, but no characteristic findings differentiate Kaposiform hemangioendothelioma from other soft-tissue tumors, particularly when the tumor is too small to have any locally aggressive features or identifiable large vessels. We present a case of Kaposiform hemangioendothelioma in the internal auditory canal that had no differential features on initial MR images and rapidly grew into a huge mass in a few months.

Decreased B cells and IgA-secreting B cells partially explain the high prevalence of IgA deficiency in dialysis patients.

AIMS: We previously reported 2 hemodialysis (HD) patients with recurrent infections and selective immunoglobulin A deficiency (IgAD). We further demonstrated that serum IgA levels were lower and the prevalence of IgAD was higher in uremic patients. The exact mechanisms of IgAD in uremic patients largely remained unclear. In some patients, it was caused by anti-IgA antibody neutralization and subsequent destruction. We performed the present study to survey if there is any defect in IgA production. MATERIALS AND METHODS: 288 patients were initially included for examination of serum immunoglobulins. 16 normal persons, 16 dialysis patients without IgAD, and 12 dialysis patients with IgAD were enrolled after the initial examination. Blood was drawn into heparinized tubes. WBC counts and lymphocyte percentage were examined by a CBC counter. Lymphocytes were separated by the Ficoll-Paque method. Flow cytometry was utilized to isolate the B cell and IgA-secreting B cell after staining with CD 19 phycoerythrin and FITC-conjugated rabbit anti-human IgA antibody. RESULTS: There is no significant difference between WBC counts or total lymphocyte counts of these 3 groups. However, we found a lower percentage of total lymphocyte counts in dialysis patients, either with or without IgAD. The total B cell numbers were lower in dialysis patients with IgAD. In addition, there were fewer IgA-secreting B cells in dialysis patients with IgAD. CONCLUSION: Decreased B cell and IgA-secreting B cell counts are seen in uremic patients with IgAD. This, in turn, indicates that there might be a defect of IgA production in some patients, rather than IgA destruction by anti-IgA antibodies as seen in some other patients. Further study is needed to investigate the mechanisms of decreased B cells and IgA-secreting B cells.

Endothelin-1 regulates parathyroid hormone expression of human parathyroid cells.

OBJECTIVE: Parathyroid cells synthesize and release endothelin-1 (ET-1). ET-1 displays an in vitro inhibitory effect on basal parathyroid hormone (PTH) secretion and also counteracts PTH hypersecretion stimulated by low calcium. Such effects are further demonstrated in vivo, independent of the changes in calcitonin. We propose that ET-1 may regulate the pathogenesis of uremic hyperparathyroidism. However, this was not directly demonstrated in human parathyroid glands. DESIGN: Hyperplastic parathyroid glands are obtained from the surgical operation for uremic hyperparathyroidism. Cells are isolated by enzyme digestion and treated with ET-1, and are assessed for PTH mRNA expression. PTH in the plasma and the medium is measured by a newly developed method to detect the whole PTH (1-84). PATIENTS: Uremic patients with severe secondary hyperparathyroidism and ultrasonography-proved hypertrophy of parathyroid glands received elective surgical approaches under general anesthesia. The resected glands were immediately taken to the laboratory for fresh isolation. MEASUREMENTS: Following ET-1 treatment, PTH mRNA expression is evaluated by RT-PCR method. ET-1 is detected with radioimmunoassay kit and PTH is measured by a new commercially available Duo PTH kit. RESULTS: ET-1 exhibited a dose-dependent inhibitory effect (from 10(-12) - 10(-7) M) on PTH mRNA expression of parathyroid cells, either in the basal or in the low-calcium-stimulated states. Release of PTH into the medium is also gradually inhibited by the increase in ET-1 concentrations. CONCLUSIONS: Our results demonstrate that ET-1 attenuates PTH mRNA expression in freshly isolated human parathyroid cells, and PTH release is also decreased. This result is consistent with our previously reported in vitro and in vivo experiments.

[Discovery of a novel spliceosome of ABL gene (ABL(Δexon7+35INS)) and its association with TKIs resistance in chronic myeloid leukemia].

OBJECTIVE: To explore whether the ABL(Δexon7) and ABL(35INS) spliceosome contributed to TKIs resistance. METHODS: Screening ABL(Δexon7) and ABL(35INS) in 74 normal people and 76 CML patients (53 patients in remission and 23 patients with TKIs resistance) by using polyacrylamide gel electrophoresis combined with cloning sequencing. RESULTS: A novel spliceosome ABL(Δexon7+ 35INS) (ABL(Δexon7) and ABL(3)5INS existed at the same time) was identified and the mutation was detected in 8 (10.8%) of 74 normal people, 4 (7.5%) of 53 remission patients and 2 (8.7%) of 23 resistant patients. While 47 (63.5%) cases expressed ABL(Δexon7) and 8 (10.8% ) cases expressed ABL(35INS) in 74 healthy people, 30 (56.6%) cases expressed ABL(Δexon7) and 5 (9.4% ) cases expressed ABL(35INS) in 53 remission patients, 12 (52.2%) cases expressed ABL(Δexon7) and 3(13.0%) cases expressed ABL(35INS) in 23 resistant patients. Three kinds of spliceosome in all groups had no statistical difference. CONCLUSION: ABL(Δexon7+ 35INS), ABL(Δexon7) and ABL(35INS) may be not uncommon in ABL gene and were unrelated to resistance in CML with TKIs treatment. ABL(35INS) were often accompanying with exon 7 deletion.

[Frequency and clinical features of ASXL2 gene mutation in acute myeloid leukemia patients with AML1- ETO fusion gene positive].

OBJECTIVE: To investigate frequency and clinical features of additional sex combs-like 2 (ASXL2) gene mutation in acute myeloid leukemia (AML) patients with AML1-ETO fusion gene and to analyze the relationship between ASXL2 gene mutation and c- kit gene mutation. METHODS: Mutation analysis of exon 11 and 12 of ASXL2 gene in 59 de novo AML patients was performed by using polymerase chain reaction (PCR) followed by sequence analysis. The clinical features, survival curve and c-kit gene mutation in ASXL2 gene mutation positive and negative patients were compared. RESULTS: In a total of 59 AML patients with AML1-ETO fusion gene positive, 11.9% (7/59) patients harboured ASXL2 gene mutations. The hemoglobin levels of patients with mutated ASXL2 gene [56.2 (38.0- 72.0) g/L] were significantly lower than those with wild type ASXL2 [69.0(37.2-154.0) g/L] (P=0.038). Differences were not observed in white blood cell counts, platelet counts, the proportion of acidophilic cell, and the proportion of primitive cell in the marrow between patients with mutant ASXL2 and ones without mutant ASXL2 (P>0.05). None of all 59 patients suffered from liver, spleen, central nervous system metastases in both groups. Moreover, enlarged lymph nodes was similar between patients with mutant ASXL2 and ones without mutant ASXL2 (P=0.859). Immunophenotypic analysis: in positive group CD33 positive expression was significantly lower than that of negative group (P=0.033). cCD3 was not expressed in both groups. Expression levels of CD117, cMPO, HLA-DR, CD34, CD38, CD13, CD44, CD15, CD64, CD11b, CD56, CD19, cCD79a and CD7 were similar between patients with mutant ASXL2 and ones without mutant ASXL2 (P>0.05). All of 59 patients were in remission (P=0.577). Overall survival was similar between patients with mutant ASXL2 and ones without mutant ASXL2 (P=0.631). The mutation rates of c- kit in positive group and negative group were 14.3% and 29.4%, without statistical significance (P= 0.697). CONCLUSIONS: ASXL2 mutation may be a new event that can cooperate with AML1-ETO to induce leukemia. Patients in AML1- ETO positive AML with ASXL2 mutation show specific clinical characteristics of hemoglobin levels and expression level of CD33. ASXL2 gene mutations and c-kit gene mutations may not have a specific correlation between them.

Evaluation of right ventricular early diastolic filling by cine nuclear magnetic resonance imaging in patients with hypertrophic cardiomyopathy.

Numerous studies have established abnormalities in systolic and diastolic function of the left ventricle in hypertrophic cardiomyopathy. A consistent feature of this disease is reduced diastolic function of the left ventricle, but little information is available regarding right ventricular function in this disease. Cine nuclear magnetic resonance (NMR) imaging has been found to be effective for measuring right ventricular volumes and therefore was used to assess early diastolic filling of the right ventricle in patients with hypertrophic cardiomyopathy. Right ventricular time-volume curves were obtained from cine NMR images in 10 patients with hypertrophic cardiomyopathy and 8 normal subjects. Right ventricular volume was calculated with use of Simpson's algorithm at approximately 18 phases of the cardiac cycle and, from the curve, peak filling rate and filling fraction during the first third of diastole were determined. In patients with hypertrophic cardiomyopathy, peak filling rate tended to be less (176 +/- 46 vs. 305 +/- 50 ml/s, p less than 0.01) and filling fraction decreased (39.5 +/- 13.8 vs. 74.5 +/- 13.3%, p less than 0.01) in comparison with values in normal subjects. Thus, analysis of right ventricular time-volume curves obtained by using cine NMR imaging demonstrated diastolic dysfunction of the right ventricle in hypertrophic cardiomyopathy.

7-84 parathyroid hormone fragments are proportionally increased with the severity of uremic hyperparathyroidism.

AIMS: Recent progress in PTH assay has revealed that the intact PTH assay kit in current use does not differentiate between the truncated 7-84 PTH molecule and the 1-84 PTH molecule. In our series, we examined the effectiveness of a new PTH assay as a noninvasive method of evaluating severity of uremic hyperparathyroidism. METHODS AND MATERIALS: Two hundred and seventy hemodialysis (HD) patients recruited from three HD centers were included and divided into subgroups according to the conventional iPTH assay results. Pre-dialysis blood samples were collected and subjected to two different PTH assays: "intact" PTH assay (iPTH) and "whole" PTH (wPTH) assay. Two biochemical markers of bone remodeling were also examined. RESULTS: In all cases, PTH levels determined by the wPTH assay were in the average 32.3% lower than those determined by the iPTH assay. The difference of the results of the two PTH assay methods, which indicated the portion of 7-84 PTH fragments of the total PTH molecules measured with the iPTH assay, was gradually increased while the severity of uremic hyperparathyroidism increased. Biochemical markers of bone formation/resorption showed a similar change. CONCLUSION: The portion of the 7-84 PTH fragments and markers of increased bone turnover increased in proportion to the severity of uremic hyperparathyroidism. This finding disproves the hypothetical role of 7-84 PTH fragments alone as the noninvasive marker of low-turnover bone disease in HD patients.

Effects on spleen colony-forming unit self-renewal after retroviral-mediated gene transfer of multi-colony-stimulating factor, granulocyte-macrophage colony-stimulating factor, or granulocyte colony-stimulating factor.

C57BL/6 mice were established to constitutively produce multi-colony-stimulating factor (multi-CSF), granulocyte-macrophage colony-stimulating factor, or granulocyte colony-stimulating factor by transplantation with post-5-fluorouracil-treated syngeneic marrow cells infected with retroviral vectors bearing the corresponding growth factor complementary DNAs. At 2-4 weeks after transplantation, these mice had a 2-7-fold increase in spleen colony-forming unit (CFU-S) numbers when compared to control animals transplanted with MPZipNeo-infected marrow cells. Most of the CFU-S in the former animals were located in the spleen, whereas those of control mice were found in the marrow. The increase in total CFU-S content in recipients of CSF-infected mice was not due to an increase in self-renewal ability but rather to the recruitment of more primitive cells, as there were no differences in CFU-S content of spleen colonies generated from marrow cells of either group. Neither were there any differences in the cellular composition of these spleen colonies or in the size or distribution of cell types in secondary spleen colonies generated from these spleen colonies, suggesting the inability of any of the three CSFs to alter the differentiation pattern of CFU-S.

In vivo effect of endothelin-1 on plasma calcium and parathyroid hormone concentrations.

We have previously reported an in vitro inhibitory effect of endothelin-1 (ET-1) on parathyroid hormone (PTH) secretion. In the present experiment, ET-1 was infused into rabbits to study the in vivo effect of ET-1 on the changes in calcium, magnesium, PTH and calcitonin concentrations. Femoral arteries and veins of anesthetized male rabbits were cannulated to monitor vital signs, blood sampling and infusion of the agents being studied. Infusion of ET-1 (1, 5, 10 and 20 ng/kg per min) induced a dose-dependent decline in plasma ionized calcium concentrations from 6.68+/-0.26 to 5.50+/-0.46 mg/dl (P<0.05) and a decrease in calcitonin concentrations from 48.6+/-6.5 to 32.5+/-4.7 pg/ml. PTH concentrations increased from 58.3+/-10.2 to 159.4+/-22.1 pg/ml. In a separate experiment, calcium gluconate solution was simultaneously infused to keep calcium concentrations steady, thereby proving a calcium 'clamp'. In normal calcium concentration, ET-1 infusion gradually decreased PTH concentrations from 71.4+/-8.6 to 38.0+/-6.2 pg/ml. We further infused sodium citrate solution to decrease the calcium concentration (2.0 mg/dl less) and calcium gluconate solution was infused to keep calcium concentrations steadily less than normal. PTH concentrations were initially stimulated by the induction of hypocalcemia (68.1+/-11.2 to 135.6+/-8.5 pg/ml), but decreased by ET-1 infusion (135.6+/-8.5 to 85.1+/-15.2 pg/ml). Plasma magnesium concentrations did not change significantly throughout the entire study and calcitonin concentrations were not significantly changed during the calcium clamp studies. Serum phosphate and 1,25-(OH)(2) vitamin D(3) concentrations were also measured, but they also did not change significantly. In conclusion, ET-1 exhibited an in vivo acute hypocalcemic action, independent of calcitonin. It also directly decreased PTH secretion if serum calcium concentrations were kept steady. The above findings are consistent with the results of our previous in vitro experiment.

Fatal outcome after ingestion of star fruit (Averrhoa carambola) in uremic patients.

Clinical outcome of dialysis patients after eating star fruit (Averrhoa carambola) varies, but it may be fatal. In the past 10 years, 20 such patients were treated in our hospital when they developed clinical symptoms after eating the fruit or drinking star fruit juice. Their initial presentations included sudden-onset limb numbness, muscle weakness, intractable hiccups, consciousness disturbance of various degrees, and seizure. No other major events that might be responsible for these symptoms could be identified. Eight patients died, including one patient with a serum creatinine level of 6.4 mg/dL who had not yet begun dialysis. The clinical manifestations of the survivors were similar to those who died except for consciousness disturbance and seizure. Death occurred within 5 days despite emergent hemodialysis and intensive medical care. The survivors' symptoms usually became less severe after supportive treatment, and these patients subsequently recovered without obvious sequelae. The purpose of this article is to report that patients with renal failure who ingest star fruit may develop neurological symptoms and also run the risk for death in severe cases. Mortality may also occur in patients with chronic renal failure not yet undergoing dialysis.

Unusual short rib-polydactyly syndrome.

We present a case of lethal short rib-polydactyly syndrome (SRPS) that cannot be categorized into the existing classification. A nosologic discussion is presented. To our knowledge, situs inversus totalis, as in our case, has not been described before in any SRPS.