Volume 96  ·  Issue 16  ·  2016

The etiology of pulmonary hypertension involves many disciplines, which requires clinicians to have multi-disciplinary knowledge and flexible differential diagnosis thinking. For a patient with pulmonary hypertension, a standardized etiology screening process is very important. Respiratory doctors should consider cardiovascular disease factors. Cardiologists should not forget the causes of hypoxia and lung diseases. Pulmonary hypertension related to connective tissue and other related diseases should not be ignored. Clinicians should constantly accumulate experience, improve the diagnosis level, and reduce misdiagnosis and missed diagnosis. The diagnosis of pulmonary hypertension requires the clinician to perform a detailed physical examination based on the patient's symptoms, and make a comprehensive analysis of each examination to confirm whether the patient meets the hemodynamic diagnostic criteria of pulmonary hypertension, and to clarify the etiology, cardiac function and the degree of hemodynamic damage. The results of each examination need at least the evaluation and discussion of experts in cardiology, respiratory medicine, rheumatology and immunology, imaging and other departments. For patients with complex causes, multidisciplinary screening and co-diagnosis and treatment are particularly important.

chronic thromboembolic pulmonary hypertension (CTEPH) is a relatively common chronic disease. Thromboembolus blocks the proximal pulmonary artery and remodeling the distal pulmonary blood vessels, resulting in progressive increase of pulmonary vascular resistance and pulmonary artery pressure, which eventually leads to right heart failure and even death. It is often manifested as dyspnea, fatigue, and decreased exercise endurance. Its disability rate is high and the prognosis is poor.

Based on the "3+2" training mode of assistant general practitioners put forward in the "Guiding Opinions of the State Council on Establishing General Practitioner System" (Guofa [2011] No.23), Zhejiang Province issued the "Standardized Training Program for Assistant General Practitioners in Zhejiang Province (Trial)" and the "Standardized Training Standard for Assistant General Practitioners in Zhejiang Province (Trial)", which opened a precedent for the training of assistant general practitioners in China. Training qualified assistant general practitioners through the "3+2" model can gradually narrow the huge gap between the lack of rural health personnel and the construction goals put forward in the guiding opinions of the State Council. It is the key to improve the level of primary medical and health services, and it is also an effective measure to break through the bottleneck and promote the continuous deepening of the reform of medical and health system^[1]。 In 2012, the National Health and Family Planning Commission formulated and issued the Training Standards for Assistant General Practitioners (Trial) with reference to the Standardized Training Standards for Assistant General Practitioners in Zhejiang Province (Trial), which marked the national promotion of Zhejiang model. The principles, contents and implementation process of the standardized training practice of assistant general practitioners in Zhejiang Province are discussed as follows.

Interventional treatment of craniocerebral diseases includes intracranial aneurysm, cranial arteriovenous malformation, acute ischemic stroke and carotid stenosis, and deep brain stimulation in Parkinson's disease. This expert consensus aims to improve the level of anesthesia management for interventional treatment of craniocerebral diseases in China, reduce perioperative-related complications, and improve postoperative outcomes of patients. Due to the limited evidence of evidence-based medicine in this field, individualized anesthesia management needs to be implemented in clinical practice according to the specific conditions of patients and with reference to the consensus of this expert.

To investigate the mRNA expression level of plasminogen activator inhibitor-2 (PAI-2) in peripheral blood leucocytes and regulation by soluble guanylate cyclase (sGC) activator in pulmonary hypertension.

The human pulmonary arterial smooth muscle cells were treated with sGC activator Cinaciguat. The mRNA and protein expression levels of PAI-2 were detected with Real-time PCR and Western blot. The fresh blood samples of 8 patients with pulmonary arterial hypertension (PAH) (collected at the China-Japan Friendship Hospital from November 2014 to March 2015), 16 patients with chronic thromboembolic pulmonary hypertension (CTEPH) (collected at the China-Japan Friendship Hospital from November 2014 to March 2015), 24 age- and gender- matched healthy controls (collected at Beijing Hospital in March 2015) were treated with Cinaciguat for 8 hours. Then RNA of peripheral leukocytes was extracted and performed with reverse transcription and Real-time PCR to detect the mRNA level of PAI-2, which was compared between healthy controls and patients with pulmonary hypertension, before and after the treatment of Cinaciguat. At last, the correlation of PAI-2 mRNA level and the clinic severity of pulmonary hypertension were identified.

The mRNA and protein expression levels of PAI-2 were promoted by Cinaciguat in human pulmonary arterial smooth muscle cells. The baseline mRNA level of PAI-2 in peripheral leukocytes was significantly lower in PAH patients compared to the healthy controls (0.201±0.152, 0.660±0.440, P=0.021). There was no significant difference in the mRNA expression level of PAI-2 between the CTEPH patients and controls (0.428±0.364, 0.769±0.682, P=0.152). After Cinaciguat treatment, the mRNA expression levels of PAI-2 were up-regulated in PAH patients and CTEPH patients (1.352±1.127, 1.203±1.008), there was no significant difference in the mRNA expression level of PAI-2 among the PAH patients, CTEPH patients and controls (P=0.130, P=0.534). The baseline mRNA level of PAI-2 was negative correlated with echocardiography-estimated systolic pulmonary arterial pressure (sPAP) (r=-0.744, P=0.034).

The mRNA expression level of PAI-2 is significantly down-regulated in peripheral blood leucocytes and up-regulated by sGC activator in pulmonary hypertension. PAI-2 could be used as potential biomarker of pulmonary vascular remodeling in PAH.

To summarize the clinical features of Takayasu arteritis (TA) patients with pulmonary hypertension due to pulmonary artery involvement.

Ninety-four TA patients with pulmonary artery involvement treated in Fuwai Hospital from Jun 1988 to Jun 2014 were retrospectively summarized. Patients were divided into two groups according to whether aorta and its main branches affected (APTA, n=48) or not (PTA, n=46). Clinical features and angiographic data were further analyzed.

Of all the patients, male to female rate was 1∶3.3, age ranged from 8 to 60 years with median age of 33.5 years when symptom onset. Main symptoms included dyspnea on exertion. Heart murmur and mid-systolic murmur in pulmonic area were commonly found by physical examination. All patients had pulmonary hypertension by echocardiographic examination. Angiographic data showed that multi-lobular and multi-segmental pulmonary branches were predominantly affected, followed by sub-segmental and peripheral branches. Both sides of pulmonary artery involvement were more common than one side involvement, while the right pulmonary arteries were more often affected than the left. Most of the clinical symptoms and signs between APTA group and PTA group were comparable. However, erythrocyte sedimentation rate and N-terminal pro-natriuretic peptide were significant higher in APTA group than that in PTA group when admission [14.0(5.0, 28.0) vs 8.5(3.0, 19.3) mm/1 h and (1 936±1 769) vs (1 627±1 153) ng/L, both P<0.05].

Dyspnea on exertion may be the main manifestation of TA with pulmonary artery involvement. All patients present with pulmonary hypertension and moderate to severe heart failure when symptom onset.

To explore the pathogenic genes of pulmonary arterial hypertension (PAH) and validate the association between OR2T3 gene and PAH.

Whole exome sequencing was performed in four patients and one healthy person as control in two pulmonary arterial hypertension pedigree; patient-specific variations were screened by bioinformatics methods and comparison between groups. To further analyze the association between these variations and PAH, Sanger sequencing was used to analyze the genotype of patient-specific variations of 30 patients with idiopathic PAH, 90 healthy people and 30 patients with chronic thromboembolic pulmonary hypertension.

The preliminary findings of whole exome sequencing were 57 variations may be associated with PAH; Among them, there were 6 AG heterozygotes due to OR2T3rs148748995 in the 30 idiopathic PAH patients, while no G allele carrier was found in other healthy people of two pulmonary arterial hypertension pedigree (AⅠ-1, AⅡ-3, BⅡ-1) and 90 normal control, and the difference was statistically significant (P<0.05). The variation also didn't exist in 30 chronic thromboembolic pulmonary hypertension patients.

OR2T3 gene may be the pathogenic gene of PAH and OR2T3rs148748995 could have a role in the development of PAH.

Mutation analysis of unrelated families with Duchenne/Becker muscular dystrophy (DMD/BMD) was performed to investigate the characteristic of DMD gene mutation, especially the distribution pattern of point mutation of DMD gene in Chinese population.

A total of 433 unrelated DMD/BMD families were collected at the Center of Prenatal Diagnosis of the First Affiliated Hospital of Zhengzhou University from March 2010 to December 2014. The deletions or duplications in 79 exons of DMD gene were screened using multiplex ligation-dependent probe amplification (MLPA). Any single-exon deletion detected by MLPA was further validated by PCR amplification. In the 117 unrelated Chinese families in which large-scale deletions and duplications had been excluded by MLPA, the point mutation in 79 exons of DMD gene were tested in the propositus using next-generation sequencing (NGS), and further verified the point mutation using Sanger sequencing.

In the 433 unrelated DMD/BMD families, 316 families with DMD deletions/duplications were identified by MLPA. Out of 57 single-exon deletions detected by MLPA, 3 were found as point mutations by PCR and Sanger sequencing, including 2 nonsense mutation (c.1729G>T [p.Glu577X], c. 3346A>T [p.Lys1116X]) and 1 frame-shift mutation (c.8605_8606delGT [p.Val2869ThrfsX25]). Direct sequencing with Ion PGM and Sanger sequencing in 117 families with negative results in MLPA detected 92 different point mutations in 96 families, including 46 novel mutations, 42 previously reported ones, and 4 possible polymorphisms (rs189143447, rs202008454, rs200213555, rs187617705). The 46 novel mutations consisted of 16 nonsense mutations (c.100A>T [p.Lys34X], c. 1201C>T [p.Gln401X], c. 1707C>A [p.Cys569X], c. 1831G>T [p.Glu611X], c. 1912C>T [p.Gln638X], c. 2213C>G [p.Ser738X], c. 3673_3673delA [p.Ile1225X], c. 3774C>A [p.Cys1258X], c. 4858G>T [p.Glu1620X], c. 5764A>T [p.Lys1922X], c. 6035T>G [p.Leu2012X], c. 6408G>A [p.Trp2136X], c. 7717C>T [p.Gln2573X], c. 7864G>T [p.Glu2622X], c. 8184_8185insT [p.Lys2729X], c. 8215C>T [p.Gln2739X]), 5 missense mutations (c.139G>A [p.Gly47Arg], c. 238G>C [p.Ala80Pro], c. 335G>T [p.Trp112Leu], c. 804A>C [p.Leu268Phe], c. 1149G>T [p.Glu383Asp]), 6 splice-site mutations (c.2293-3C>A, c. 2380+ 1G>T, c. 3277-1G>C, c.4519-7A>G, c. 5740-15G>T, c. 7661-1G>C), 16 small deletions (c.688_688delA [p.Met230CysfsX14], c.1760_1791del32 [p.Thr587IlefsX37], c. 2271_2271delA [p.Asp774ThrfsX22], c. 2281_2285delGAAAA [p.Glu761SerfsX10], c. 2527_2527delG [p.Glu843SerfsX3], c. 3405_3405delC [p.Asn1135LysfsX18], c. 4450_4450delC [p.His1484ThrfsX14], c. 4770_4770delA [p.Thr1590ThrfsX5], c. 4937_4937delA [p.Glu1646GlyfsX11], c. 5253_5256delATTA [p.Lys1751LysfsX2], c. 5654_5654delA [p.Gln1885ArgfsX6], c. 7441_7441delG [p.Glu2481AsnfsX13], c. 7860_7860delC [p.Ile2620IlefsX18], c. 8668-8668delG /c.8668+ 1-8668+ 1delG, c. 9009_9009delC [p.Thr3003ThrfsX18], c. 9021_9021delT [p.Ile3007IlefsX14]), and 3 small insertions (c.305_306insG [p.Gly102GlyfsX4], c. 3116_3117insA [p.His1039GlufsX11], c. 9197_9198insATCTC [p.Ser3066SerfsX25]). And 87.4% (83/95) of the pathologic point mutations disrupted the translational reading frame (46 nonsense mutations, 24 frame-shift mutations, and 13 splice-site mutations).

Inexpensive and efficient genetic/prenatal diagnosis of DMD/BMD may be plausible by MLPA analysis, NGS, and Sanger sequencing. Most of the mutations identified in this study led to a predictable premature stop codon or splicing defects, resulting in defective function of dystrophin.

To explorer the effect of improvement of diabetes on survival after radical pancreatic resection in patients with pancreatic cancer and diabetes.

Seventy-three patients who underwent radical resection for pancreatic cancer at Ruijin Hospital (Shanghai, China) between July 2007 and December 2011 and had preoperative diabetes were retrospectively analyzed. Clinical characteristics and overall postoperative survival were compared between patients with controlled/uncontrolled diabetes. Among patients with uncontrolled diabetes, comparison was made in clinical characteristics and overall postoperative survival between patients with long-term (≥2 years) diabetes and those with new-onset (<2 years) diabetes.

Controlled diabetes after radical pancreatic resection was observed in 38.4% (28/73) in the patients with pancreatic cancer and diabetes. The median follow-up time was 44.5 months (95CI: 31.7-57.2 months). Patients in whom diabetes was controlled had well-differentiated tumors and longer overall survival compared to patients with uncontrolled diabetes [median overall survival (95%CI) 41.0 (34.2-47.7) months vs 17.0 (14.8-19.2) months, P<0.001]. Among the 45 patients with uncontrolled diabetes, there was no differences in clinic characteristic and overall survival between new-onset (n=21) and long-term diabetes (n=24) [median overall survival (95%CI): 17.8 (15.7-20.1) months vs 15.8 (10.5-21.1) months, P=0.198].

Good postoperative control of diabetes after radical pancreatic resection in patients with pancreatic cancer and diabetes may predict favorable survival.

To evaluate the clinical predictive factors associated with pathologic complete response (pCR) after neoadjuvant chemoradiotherapy in rectal cancer.

A retrospective analysis was performed on clinical data of 87 patients with rectal cancer, who underwent neoadjuvant chemoradiotherapy followed by radical surgery from April 2007 to May 2015. All patients received pelvic intensity-modulated radiotherapy (50 Gy/25 fractions) with concurrent fluorouracil- based chemotherapy and then underwent radical surgery 4-8 weeks later. The clinical factors associated with pCR or non-pCR were analyzed by Logistic regression.

Eighty-three patients had completed treatment, of which 15 patients (18.1%) achieved pCR after neoadjuvant chemoradiotherapy. Univariate analysis showed non-diabetes(P=0.033), pre-chemoradiotherapy plasma glucose(≤6.1 mmol/L)(P=0.033), pre-chemoradiotherapy CEA(≤5 μg/L)( P=0.026), pre- chemoradiotherapy plasma lymphocytes count(≤1.5×10⁹/L)(P=0.042), pre- chemoradiotherapy plasma platelet(≤240×10¹²)(P=0.043), tumor pathological type(adenocarcinoma)(P=0.036)were significantly correlated with pCR after neoadjuvant chemoradiotherapy in rectal cancer. Logistic regression revealed that non-diabetes, pre-chemoradiotherapy plasma glucose, pre-chemoradiotherapy CEA(≤5 μg/L), pre-chemoradiotherapy plasma lymphocytes count(≤1.5×10 ⁹/L), tumor pathological type(adenocarcinoma) were independent predictive factors of pCR after neoadjuvant chemoradiotherapy in rectal cancer.

Patients with non-diabetes, normal plasma glucose, normal CEA, lower plasma lymphocytes count and adenocarcinoma were associated with pCR after neoadjuvant chemoradiotherapy for rectal cancer.

To explore the therapeutic effect and quality of life in elderly patients of muscle-invasive bladder cancer by transurethral resection combined with intravesical instillation therapy.

From January 2005 to January 2015, 136 cases were diagnosed with T2 bladder cancer by transurethralplasmakinetic therapy or transurethral laser therapy. The data of 136 cases were analyzed retrospectively.

In transurethralplasmakinetic therapy group, the overall survival rate was 73.3%, the cancer-specific survival rate was 85.6%, the average survival time were 65 months. In transurethral laser therapy group, the overall survival rate was 73.9%, the cancer-specific survivalrate was 87.2%, the average survival time were 70 months. Two methods also can improve the patient's quality of life.

For the aged with invasive bladder cancer (T2), we could try the transurethral resection combined with intravesical instillation therapy.

To evaluate the effects of intubating laryngeal mask airway (ILMA) in prediction of extubation responses in patients with hypertensive cerebral hemorrhage during general anesthesia recovery period.

A total of 120 patients with hypertensive cerebral hemorrhage aged 47-71 years, with Glasgow Coma Scale (GCS)11-15 scores and American Society of Anesthesiologists (ASA) physical status Ⅰ or Ⅱ, undergoing craniotomy surgery from December 2012 to December 2014 in the Affiliated First Municipal Hospital of Fujian Medical University were randomly divided into 2 groups (n=60): tracheal intubation (Group T) and intubating laryngeal mask airway (Group I), by using a random number table. Variations of invasive arterial blood pressure and responses of endotracheal extubation were compared between two groups before and after extubation.

There were no significant differences in ages, sex ratio, preoperative GCS, operation time and the time from the end of operation to Train of Four stimulation (TOF) becoming to 4 between the two groups. The mean arterial pressure (MAP) at the time point of extubation (T₃), 1 min (T₄), 3 min (T₅), 5 min (T₆) after extubation were (136±20), (130±16), (128±12), (125±10) mmHg in Group T, and heart rate(HR) at these four time points were (105±11), (96±8), (92±7), (86±6) bpm, respectively. While in group I, MAP were (108±10), (106±8), (105±9), (106±7) mmHg, and HR were (75±8), (76±7), (68±5), (67±6) bpm, respectively. MAP and HR of the two groups at these four time points had significant differences (T₃: t=10.91, 17.20; T₄: t=13.72, 14.69; T₅: t=12.54, 13.35; T₆: t=13.39, 11.27; all P<0.01). During recovery, the incidences of extubation responses of group T and group I were 92% and 2%, respectively, and the difference had statistically significant (χ²=94.05, P<0.01). The incidences of coughing of group T and group I were 97% and 0, respectively, and the difference had statistically significant (χ²=148.69, P<0.01).

Compared with tracheal intubation, ILMA can decrease the incidence of extubation responses and coughing in patients with hypertensive cerebral hemorrhage during general anesthesia recovery period, and avoid cere-brovascular accidents.

To investigate the clinical application of spontaneous acrosome reaction (AR) rate of sperm in predicting the outcome of in-vitro fertilization and embryo transfer (IVF-ET).

The spontaneous AR rate of the sperm of patients who underwent IVF-ET treatment in our center during the period from November to December 2014 were studied. The cut-off value from 6% to 12% were set and analyzed its association between the IVF-ET outcomes (including fertility rates, normal fertilization rates and high-quality embryo rates). For those who underwent fresh embryo transplantation, the rates of chemical pregnancy and clinical pregnancy were calculated, and compared the spontaneous AR rates and quantity of acrosomal enzyme according to the pregnancy outcome.

There were 202 patients in this study and the mean spontaneous AR rate was 5.99%±5.18%. For patients with the spontaneous AR rate ≥9% versus <9%, the fertility rate, normal fertilization rate and high-quality embryo rate were 81.33% vs 83.85%, 60.53% vs 60.99%, and 51.10% vs 59.67%, respectively, with statistically significant difference in the high-quality embryo rate ( P=0.02). For patients who underwent fresh embryo transplantation, when comparison was made between those with spontaneous AR rate ≥8% and those <8%, the rate of chemical pregnancy and clinical pregnancy were 48.57% (17/35) vs 69.64% (78/112) and 37.14% (13/35) vs 63.39% (71/112), respectively, both with statistically significant difference ( P=0.02 and P<0.01). The patients with clinical pregnancy had lower spontaneous AR rate than those without clinical pregnancy (5.41%±3.87% vs 7.40%±6.79%,P=0.04), while the quantity of acrosomal enzyme showed no significant difference [(131.79±68.50) vs (153.62±59.59) μU/10 ^6,P=0.06]. Logistic regression analysis demonstrated association between spontaneous AR rates and clinical pregnancy (OR=0.93, 95%CI: 0.87-0.99, P=0.03).

The spontaneous AR rate of sperm may have clinical significance in predicting the outcome of IVF-ET, as it is reversely correlated with IVF high-quality embryo rate and pregnancy rate. The quantity of acrosomal enzyme may not have significant predictive value for the outcome of IVF.

To explore changes in the proteomics of lung tissue in early stage of burn-blast combined injury in rats.

According to a random digital table, a total number of 18 Sprague Dawley (SD) rats were divided into burn-blast combined injury group (n=9) and blast injury group (n=9). Lung protein samples were collected at 6 h post injury. 2-DE was performed to separate proteins. After silver staining, the protein of differential expression were analyzed by PQ Quest and then identified by matrix-assisted laser desorption/ionization time of fight mass spectrometry (MALDI-TOF-MS). The features of the changes of proteomics of rat lung after burn-blast combined injury were studied by biological spectrometry, protein bank and reference article analysis technique. At same time, pathologic changes of the lung were monitored after injury.

After removing death drain during the experiment, each group contained 8 rats and the results were analyzed statistically. Well focused and distinct 2-DE maps with good reproducibility were obtained, means of 736±47 and 782±30 protein spots were detected from the blast injury group and burn-blast combined injury group and the matching rates were 91%. From the two groups, 14 differential protein spots expressions were analyzed by MALDI-TOF-MS, of which 10 proteins were up-regulated and 4 proteins were down-regulated in burn-blast combined injury group. 12 different expression proteins were identified in the lung through 2-DE, mass spectrometry and protein date base, including heat shock 27 protein 1, heat shock 70 protein 1, carbonic anhydrase 2, cytochrome c oxidase, ATP synthase subunit alpha, Ca²⁺ -transporting ATPase et al, which took part in stress reaction, metabolism, immune response and cytoskeleton.

Burn-blast combined injury could induce dramatically changes of proteomics in lung tissue at early stage. The mechanism probably involves several proteins associated with oxidative stress, energy metabolism, immune response and cytoskeleton.

To investigate the effect of miR-34b-3p on the proliferation, migration and tube formation of senescent endothelial cell.

Primary human umbilical vein endothelial cells (HUVECs) were cultured in vitro, and population doubling levels (PDLs) were calculated by passage. The young endothelial cell was defined as PDL8. The senescent endothelial cell was defined as PDL44. Reverse transcription quantitative real-time polymerase chain reaction (RT-qPCR) was applied to detect the expression of miR-34b-3p in PDL8 and PDL44 HUVECs. miR-34b-3p mimic and inhibitor were transfected into PDL8 and PDL44 HUVECs. Then, cell counting kit-8 (CCK-8), transwell and tube formation assays were used to determine the proliferation, migration and tube formation of HUVECs, respectively.

miR-34b-3p was significantly up-regulated approximately 4.3 times in PDL44 HUVECs than that in PDL44 HUVECs (t=-4.528, P<0.05). The proliferation, migration, total tube length and branch points of miR-34b-3p in PDL8 HUVECs group were significantly higher approximately 1.2 (0.67/0.57), 1.2 (106/86), 1.4 (10 605/7 735) and 1.3 (41/31) times than that in PDL44 HUVECs group, respectively (t=3.237, 3.564, 5.165, 3.487, P<0.05 orP<0.01). Overexpression of miR-34b-3p had significantly inhibited proliferation, migration, total tube length and branch points approximately 2.2 (0.67/0.30), 2.3 (106/46), 1.6 (10 605/6 652) and 1.9 (41/22) times in PDL8 HUVECs, respectively (F=145.898, 53.026, 41.997, 36.341, all P<0.01). Repression of miR-34b-3p had significantly increased proliferation, migration, total tube length and branch points approximately 1.4 (0.77/0.57), 2.3 (198/86), 1.7 (13 073/7 735) and 2.3 (71/31) times in PDL44 HUVECs, respectively (F=14.815, 42.970, 167.063, 258.340, all P<0.01).

The high expression of miR-34b-3p in senescent HUVECs could impair the proliferation, migration and tube formation of senescent endothelial cell.

To report the first clinical experience of living donor liver transplantation(LDLT) in patient with complicated hepatic cystic echinococcosis in China.

First Affiliated Hospital of Xinjiang Medical University successfully implemented LDLT in the treatment of one patient with complicated hepatic cystic echinococcosis after four times liver surgery and secondary congestive cirrhosis on May 9, 2015. The clinical data of the patient has been retrospectively analyzed.

LDLT procedure was successfully performed in a patient with four previous liver intervention due to his recurrence. The patient was lack of right lobe, while with significant hypertrophy in left lobe. The reoccurred lesion compressed the retrohepatic vena cava, left hepatic vein, portal vein, that led to the stricture of retrohepatic vena cava, left hepatic vein orifice. The phlebography displayed remarkable stricture in retrohepatic vena cava and rich collateral circulation by which the venous blood flow back to the right atrium via paravertebral vein. The orifice of left hepatic vein shaped as hair-like so that the stenting was impossibly. After the multi-disciplinary team discussion, the liver transplantation was proposed, while the autotransplantation was considered as first line option, and with the backup of living related donor. The laparotomy showed the hugely enlarged liver with nodular changes. The frozen section was tested twice and showed as spotty necrosis with pseudo-lobe formation. Although the enlarged left lobe about 2 000 g weight, the liver colored as grey with cotton-like texture, the plan for autotransplantation was abandoned regarding the high risk for post-operative liver failure. Therefore, LDLT was performed. The donor was her brother who donated right lobe with 685 g weight. The operation time was 1 005 min with anhepatic time 335 min. Total of 12 units red blood cell suspension was transfused. The coagulating function was back to normal in 3 days after operation. The patient was discharged at 30 days after operation, while the routine blood test and biochemical markers had back to normal levels. The patient had taken tacrolimus and methylprednisolone with normal life. The liver functions of transplanted liver were normal in 90 days after surgery. The patient was able to self-activity and regular outpatient follow-up.

Hepatic CE is an infectious disease, and liver transplantation could be the last choice for complicated case, especially in combination with severe complications, like Budd-Chiari syndrome.

The newborn male, born 2 h, was born by caesarean section at term. The main cause was multiple scalp defects in the parieto-occipital region after birth and was admitted to hospital 2 h. Personal history and family history are not special. Admission physical examination: The body temperature was 37.0 ℃, and three irregular scalp defects were seen near the midline of the parieto-occipital region, with the extent of about 3.0 cm ×1.0 cm, 4.0 cm ×1.5 cm and 1.5 cm ×1.5 cm, respectively, with local scab, redness of the base, blood and fluid oozing on the surface, and no hair coverage. CT of the head: partial defect of the top skull. Diagnosis: Congenital skin agenesis: (1) neonatal scalp defect with infection; (2) Skull defect (Figure 1)。 After admission, the scalp defect was washed with normal saline and Kangfuxin liquid, and the recombinant bovine basic fibroblast growth factor and mupirocin ointment were applied externally. Finally, the scalp defect was covered with petrolatum gauze, put into an incubator, and vitamin K was introduced to prevent bleeding, cefmenoxime was used to prevent infection, pediatric compound amino acid nutritional support, immunoglobulin was used to enhance immunity, and oxiracetam was used to nourish cranial nerves. After 13 days of treatment, the scalp defect of the child was basically healed, and he was discharged. Follow-up after 1 week of discharge, the healing area of the scalp defect of the child was replaced by scar tissue. Follow-up after 3 months of discharge showed that the extent of the skull defect was reduced.

Patient, male, 83 years old (bladder cancer diagnosed at 63 years old). In 1995, he was diagnosed with bladder cancer in Peking Union Medical College Hospital and underwent partial cystectomy. The pathological report showed that bladder papillary transitional cell carcinoma grade II. Four local recurrences occurred in June 1997, January 2002, January 2003 and May 2004, all of which underwent local electrical resection. In October 2008, the patient developed low back and leg pain. Bone scan performed in the local hospital showed that multiple nuclear radiation concentrations throughout the body, and multiple bone metastases were considered. The first dose of zolephosphate 4 mg intravenous drip was started on 30 October 2008, and then infused once every two months on average. Bone pain was relieved after administration. The last dose was on 17 May 2015, with a total of approximately 38 doses and a total dose of 152 mg.

In recent years, population studies have found that iron deficiency is more common in patients with pulmonary hypertension (PH), and may aggravate the progression of the disease. The research on targeted treatment with iron supplement is also gradually being carried out, and some progress has been made. However, the mechanism of iron deficiency and the occurrence and development of PH needs to be further explored. The effect of iron deficiency on PH and the related research status and possible mechanism of iron therapy are described below.

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with an incidence rate of about 1~2/1 million. Children under 10 years old and adults aged 40~50 years old are the two peak age groups of adrenocortical carcinoma. In recent years, the diagnosis and treatment of ACC has undergone major changes, gradually shifting to individualized and precision medicine^[1]。 In order to further improve the diagnosis and treatment of adrenocortical carcinoma, the research progress related to adrenocortical carcinoma in recent years is reviewed as follows.

neutral lipid storage disorders (NLSDs) are autosomal recessive diseases caused by the intracellular deposition of triglycerides (TG) in different tissues. Its onset is caused by mutation of adipose triglyceride lipase (ATGL), also known as patatin-like phospholipase domain protein 2 (PNPLA2) gene, which leads to NLSDs with myopathy and myocardial vascular disease, which is mainly muscle damage^[1]or Dorfman-Chanarin syndrome with ichthyosis and fatty liver as the main manifestations caused by mutations in the comparative gene identification-58 (CGI-58), also known as α/β hydrolase domain-containing protein 5 (ABHD5) gene^[2]。