OBJECTIVETo investigate a cerebral autosomal dominant arteriopathy with the subcortical infarcts and leukoencephalopathy (CADASIL) case with clinical manifestations of baldness, lumbago and Parkinson's symptoms.

METHODSClinical and imaging data of the patient were analyzed. The patient and his family members were also subjected to genetic testing.

RESULTSThe symptoms of the patient included recurrent stroke, dementia, and mood disturbance, in addition with lumbago, baldness and Parkinson's symptoms but no migraine. Cranial MRI of the patient showed bilateral symmetric leukoencephalopathy and multiple small subcortical lacunar infarcts. A point mutation in exon 11 of the NOTCH3 gene (R558C) was discovered in the proband and four asymptomatic relatives.

CONCLUSIONCADASIL is characterized by recurrent subcortical ischemic stroke, dementia, pseudobulbar palsy, and mood disturbance. Baldness, lumbago and Parkinson's symptoms may also be seen in such patients.

Alopecia ; etiology ; CADASIL ; complications ; diagnostic imaging ; genetics ; Humans ; Low Back Pain ; etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Mutation ; Parkinsonian Disorders ; etiology ; Receptor, Notch3 ; genetics

OBJECTIVETo evaluate a "silicone suture" technique for enhancing the effect of scalp reduction.

METHODSUnder the local anesthesia, when an incision was made in the midline of the lesion, the dissection was carried out underlying the sub-galea on both sides of the lesion, as far as the width of the lesion. A 3 mm silicone suture in diameter was placed in the galea beyond the lesion. After the first suture bite was anchored in the tissue at one end, the suture device was continued across the midline in such a way as a running, buried, horizontal mattress suture and it was brought out to the skin surface through the deep tissue at the another end of the lesion with a locker. The extra-tissue of the lesion was then excised and the wound was directly closed in layers. After one week of the operation, the silicone suture was gradually tightened in 2-3 times a week for about 3-5 weeks, until both sides of the lesion were approximately closed. The device was there after removed and the wound was directly closed in layers after the scar was excised.

RESULTSBetween October of 1999 and March of 2006, 12 scarring-scalp patients, 7 males and 5 females, were treated by using the silicone suture device without complications. The excised defects were 5-10.5 cm in width. The stretching period was 26.4 days in mean. With the following-ups over 3 months, no hypertrophic scar and widening scar cases appeared.

CONCLUSIONSThe silicone suture as an alternative device for tissue extension could be a safe, simple, effective and economical device. It could significantly enhance the efficiency of scalp reduction.

Adolescent ; Adult ; Alopecia ; etiology ; surgery ; Cicatrix ; complications ; surgery ; Female ; Humans ; Male ; Scalp ; surgery ; Silicones ; Sutures ; Young Adult

OBJECTIVETo investigate the relationship between the levels of antidesmoglein (DSG) 1, 3 antibodies in the sera of patients with paraneoplastic pemphigus (PNP) and alopecia.

METHODSSera from PNP patients, bullous pemphigoid patients, and normal healthy subjects were collected and 2 tissue samples from 2 healthy scalps were resected. Anti-DSG 1, 3 antibodies in the sera of PNP patients were detected by enzyme-linked immunosorbent assay (ELISA). Indirect immunofluorescent assay was used to detect whether the antibodies in the sera of PNP patients binds with the follicular epithelium of normal healthy scalp.

RESULTSAnti-DSG3 autoantibody was strongly positive and anti-DSG1 weakly positive in one patient, while both two antibodies were negative in the other patient. Their sera could bind to keratinocytes and follicular epithelium in human scalp. Immunofluorescent signals were found on the intercellular epidermal cell surface and outer root sheath of the follicular epithelium. However, the immunofluorescent signals in the section incubating with serum of bullous pemphigoid were only found on basal membrane zone. No signals were found in the section incubating with normal healthy serum.

CONCLUSIONAlopecia in PNP patients are correlated with the anti-DSG3.

Adult ; Alopecia ; etiology ; immunology ; Autoantibodies ; blood ; Desmoglein 1 ; immunology ; Desmoglein 3 ; immunology ; Female ; Humans ; Male ; Paraneoplastic Syndromes ; complications ; immunology ; Pemphigus ; complications ; immunology

OBJECTIVETo investigate the therapeutic effect of soft tissue expansion combined with follicular unit extraction( FUE) for burn cicatricial bald.

METHODS48 patients with burn cicatricial bald (> 25 cm2) were treated in three stages. The expanders were implanted on the first stage. After expansion for 8 weeks, the expanders were taken out and local flaps were transferred. One year later, follicular unit extraction( FUE) was applied on the bald area.

RESULTS48 cases were followed up for 5 years with satisfactory cosmetic results. The VAS assessment of satisfaction on hair appearance after three-staged surgery was 8.2 ± 2.1.

CONCLUSIONSSoft tissue expansion combined with FUE has a reliable effect for burn cicatricial bald.

Alopecia ; etiology ; surgery ; Burns ; complications ; surgery ; Female ; Hair ; transplantation ; Hair Follicle ; transplantation ; Humans ; Male ; Surgical Flaps ; transplantation ; Tissue Expansion ; methods ; Tissue Expansion Devices

OBJECTIVETo study the effect of the technique of synchronously perforating and transplanting hair follicular-units in the treatment of cicatricial alopecia after burn.

METHODSOne hundred and sixty-six patients with 217 bald scar areas after burn were treated with above-mentioned technique from January 2002 to April 2008. Scalp strips, with conforming the necessity for grafting, were harvested from the occipital or temporal region. A series of follicular-units, each composing 1 - 3 hairs, were dissected from the strips under microscope or magnifying glass. Size-matching micro-slots were made in the scarred recipient area with 16 - 20 G needles to accept the grafts. The prepared follicular-unit was synchronously implanted into the bottom of the micro-slot as the needle being withdrawn. Patients who were not satisfactory with the density of hairs after I stage surgery underwent II stage surgery a half year later. Ten recipient areas with clear boundary in 10 patients were optionally chosen to observe the density of follicular-units and hair amount with naked eyes after I stage surgery. Survived transplanted hairs in above-mentioned 10 areas were counted to calculate hair survival rate at follow-up. Patients' postoperative satisfaction ratings were surveyed with questionnaire.

RESULTSIn one half of the patients, treatment was finished after I stage surgery, the other one half received 2 stages of surgery. The follicular-unit density reached 15 - 25 grafts/cm(2) with 40 - 70 hairs/cm(2) after I stage surgery. All patients were followed up for over 8 months. Grafted hairs grew well in a natural way. 96.5% mean hair survival rate was observed in the 10 recipient areas. From patients who received only I stage surgery, 61 patients (73.5%) were very satisfactory and 22 patients (26.5%) satisfactory with the results. From the other half of patients, 76 patients (91.6%) were very satisfactory and 7 patients (8.4%) satisfactory with the results.

CONCLUSIONSThe technique of perforating and transplanting follicular-unit hair synchronously is safe and effective with less surgery-induced injury and less bleeding. Hairs transplanted on cicatricial alopecia area with this technique grow well with high survival rate.

Adolescent ; Adult ; Aged ; Alopecia ; etiology ; surgery ; Burns ; complications ; Child ; Cicatrix ; complications ; Female ; Hair Follicle ; transplantation ; Humans ; Male ; Middle Aged ; Skin Transplantation ; methods ; Young Adult

Autoimmune hepatitis (AIH) is an immune-mediated chronic liver disease characterized by hepatocellular inflammation, necrosis, and fibrosis, which can progress to cirrhosis and fulminant hepatic failure. The standard treatment for AIH includes corticosteroids alone or in combination with azathioprine. Although most patients achieve remission using the standard regimen, some patients do not respond due to either drug intolerance or refractory disease; in such cases alternative immunosuppressive agents should be explored. The second-line therapies are cyclophilin inhibitors such as cyclosporine A or tacrolimus, and nowadays mycophenolate mofetil (MMF) is widely used if azathioprine-based therapies are not tolerated. Although these are recommended as an alternative to the first-line regimen, there is insufficient evidence for the efficacy of second-line therapies, with the evidence based mainly on expert opinion. Therefore, we report an AIH patient receiving the standard regimen in whom remission did not occur due to side effects to azathioprine, but was successfully treated with MMF in combination with corticosteroids as an alternative to the standard regimen.
Alanine Transaminase/analysis ; Alopecia/etiology ; Antibiotics, Antineoplastic/*therapeutic use ; Aspartate Aminotransferases/analysis ; Azathioprine/adverse effects ; Female ; Hepatitis, Autoimmune/*drug therapy/pathology ; Humans ; Liver/enzymology/pathology ; Middle Aged ; Mycophenolic Acid/*therapeutic use ; Pancytopenia/etiology ; Prednisolone/therapeutic use

OBJECTIVETo compare the efficacy and toxicity of chemotherapy combined with insterstitial (125)I seed implantation brachytherapy in unresectable staged IIIa/IIIb non-small cell lung cancer.

METHODSSeventy six patients with staged IIIa/IIIb non-small cell lung cancer were included in this study. Among them 37 cases were of the study group, treated with NP/GP scheme synchronization chemotherapy combined with (125)I seed implantation brachytherapy, while 39 cases in the control group were given NP/GP scheme chemotherapy. The cumulative survival time and median survival time of the two groups were compared by Kaplan-Meier analysis. The difference of mean survival time between the two groups was analyzed by log-rank method.

RESULTSThe study group and the control group achieved a total response rate of 56.8% and 30.8%, local control rate of 78.4% and 56.4%, respectively, showing a statistically significant difference (P < 0.05). The 1-year survival rates of the study group and control group were 66.7% and 45.3%, and the median survival times 15.4 and 11.5 months, respectively, with a significant difference between the 2 groups (P < 0.05). The total chemotherapy toxicity rate of the two groups showed no significant difference (P > 0.05).

CONCLUSIONThe (125)I seed implantation brachytherapy combined with concurrent chemotherapy shows a low complication rate, acceptable toxicity, and good therapeutic effectiveness, and is an effective and satisfactory therapeutic modality in the management of locally advanced non-small cell lung cancer.

Alopecia ; etiology ; Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Brachytherapy ; adverse effects ; Carcinoma, Non-Small-Cell Lung ; drug therapy ; pathology ; radiotherapy ; Cisplatin ; administration & dosage ; Combined Modality Therapy ; Deoxycytidine ; administration & dosage ; analogs & derivatives ; Diarrhea ; etiology ; Female ; Follow-Up Studies ; Humans ; Iodine Radioisotopes ; adverse effects ; therapeutic use ; Leukopenia ; etiology ; Lung Neoplasms ; drug therapy ; pathology ; radiotherapy ; Male ; Middle Aged ; Neoplasm Staging ; Remission Induction ; Survival Rate ; Vinblastine ; administration & dosage ; analogs & derivatives ; Vomiting ; etiology

OBJECTIVETo investigate the time to progression (TTP) and overall survival in patients with previously untreated metastatic or relapsed esophageal squamous cell carcinoma treated with chemotherapy (paclitaxel plus cisplatin) combined with radiotherapy versus chemotherapy alone, and also to evaluate the efficacy and toxicity of the regimen.

METHODSIn this prospective and non-randomized study, 47 patients with definite measurable lesion and having no previous chemotherapy were enrolled. All patients were treated with paclitaxel 175 mg/m2 by 2-hour iv infusion on d1 and cisplatin 75 mg/m2 by iv infusion on d1, which were repeated every 21 days. After 2-6 cycles of chemotherapy, those who gained CR, PR or SD were non-randomly assinged into radiotherapy group (group A) or non-radiotherapy group (group B).

RESULTSTotally, 47 patients were enrolled into this study, and all of them were valuable for response. One patient achieved complete response (CR), 19 partial response (PR), 24 stable disease (SD), and 3 were found to have disease progression (PD). The overall response rate of chemotherapy was 42.6% (95% CI, 0.28-0.58). Twenty-one of these 47 patients were sequentially treated with radiotherapy. The median survival and TTP was 13 months and 10 months in the group A , versus 11 months and 5 months in the group B (P < 0.024, P < 0.015), respectively. The most common toxicities were neutropenia and alopecia. There were no grade 4 clinical toxicity and treatment-related death in this series. Systemic adverse effects frequently occurred during radiotherapy were esophagitis and fatigue, which were tolerable.

CONCLUSIONThe combined therapy using chemotherapy (paclitaxel + cisplatin) followed by radiotherapy is effective, tolerable, and statistically superior to chemotherapy alone in patients with metastatic or relapsed esophageal squamous cell carcinoma.

Adolescent ; Adult ; Aged ; Alopecia ; etiology ; Antineoplastic Combined Chemotherapy Protocols ; adverse effects ; therapeutic use ; Carcinoma, Squamous Cell ; drug therapy ; radiotherapy ; secondary ; Cisplatin ; administration & dosage ; Combined Modality Therapy ; Esophageal Neoplasms ; drug therapy ; pathology ; radiotherapy ; Fatigue ; etiology ; Follow-Up Studies ; Humans ; Liver Neoplasms ; drug therapy ; radiotherapy ; secondary ; Lung Neoplasms ; drug therapy ; radiotherapy ; secondary ; Lymphatic Metastasis ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neutropenia ; etiology ; Paclitaxel ; administration & dosage ; Prospective Studies ; Radiotherapy ; adverse effects ; methods ; Radiotherapy, High-Energy ; adverse effects ; Survival Analysis

Alopecia ; chemically induced ; diagnosis ; Antirheumatic Agents ; administration & dosage ; adverse effects ; Arthritis, Rheumatoid ; drug therapy ; Biopsy ; methods ; Cyclosporine ; administration & dosage ; Dermatologic Agents ; administration & dosage ; Drug Hypersensitivity Syndrome ; diagnosis ; etiology ; physiopathology ; therapy ; Humans ; Male ; Middle Aged ; Prednisolone ; administration & dosage ; Skin ; pathology ; Sulfasalazine ; administration & dosage ; adverse effects ; Symptom Flare Up ; Treatment Outcome ; Vitiligo ; chemically induced ; diagnosis

Systemic lupus erythematosus (SLE) associated macrophage activation syndrome (MAS) is clinically severe, with a high mortality rate and rare neuropsychiatric symptoms. In the course of diagnosis and treatment, it is necessary to actively determine whether the neuropsychiatric symptoms in patients are caused by neuropsychiatric systemic lupus erythematosus (NPSLE) or macrophage activation syndrome. This paper retrospectively analyzed the clinical data of 2 cases of SLE associated MAS with neuropsychiatric lesions, Case 1: A 30-year-old female had obvious alopecia in 2019, accompanied by emaciation, fatigue and dry mouth. In March 2021, she felt weak legs and fell down, followed by fever and chills without obvious causes. After completing relevant examinations, she was diagnosed with SLE and given symptomatic treatments such as hormones and anti-infection, but the patient still had fever. The relevant examinations showed moderate anemia, elevated ferritin, elevated triglycerides, decreased NK cell activity, and a perforin positivity rate of 4.27%, which led to the diagnosis of "pre-hemophagocytic syndrome (HPS)". In May 2021, the patient showed mental trance and babble, and was diagnosed with "SLE-associated MAS"after completing relevant examinations. After treatment with methylprednisolone, anti-infection and psychotropic drugs, the patient's temperature was normal and mental symptoms improved. Case 2: A 30-year-old female patient developed butterfly erythema on both sides of the nose on her face and several erythema on her neck in June 2019, accompanied by alopecia, oral ulcers, and fever. She was diagnosed with "SLE" after completing relevant examinations, and her condition was relieved after treatment with methylprednisolone and human immunoglobulin. In October 2019, the patient showed apathy, no lethargy, and fever again, accompanied by dizziness and vomiting. The relevant examination indicated moderate anemia, decreased NK cell activity, elevated triglycerides, and elevated ferritin. The patient was considered to be diagnosed with "SLE, NPSLE, and SLE-associated MAS". After treatment with hormones, human immunoglobulin, anti-infection, rituximab (Mabthera), the patient's condition improved and was discharged from the hospital. After discharge, the patient regularly took methylprednisolone tablets (Medrol), and her psychiatric symptoms were still intermittent. In November 2019, she developed symptoms of fever, mania, and delirium, and later turned to an apathetic state, and was given methylprednisolone intravenous drip and olanzapine tablets (Zyprexa) orally. After the mental symptoms improved, she was treated with rituximab (Mabthera). Later, due to repeated infections, she was replaced with Belizumab (Benlysta), and she was recovered from her psychiatric anomalies in March 2021. Through the analysis of clinical symptoms, imaging examination, laboratory examination, treatment course and effect, it is speculated that the neuropsychiatric symptoms of case 1 are more likely to be caused by MAS, and that of case 2 is more likely to be caused by SLE. At present, there is no direct laboratory basis for the identification of the two neuropsychiatric symptoms. The etiology of neuropsychiatric symptoms can be determined by clinical manifestations, imaging manifestations, cerebrospinal fluid detection, and the patient's response to treatment. Early diagnosis is of great significance for guiding clinical treatment, monitoring the condition and judging the prognosis. The good prognosis of the two cases in this paper is closely related to the early diagnosis, treatment and intervention of the disease.
Humans ; Female ; Adult ; Rituximab/therapeutic use* ; Macrophage Activation Syndrome/etiology* ; Retrospective Studies ; Lupus Erythematosus, Systemic/drug therapy* ; Methylprednisolone/therapeutic use* ; Lupus Vasculitis, Central Nervous System ; Fever/drug therapy* ; Erythema/drug therapy* ; Hormones/therapeutic use* ; Anemia ; Alopecia/drug therapy* ; Triglycerides/therapeutic use* ; Ferritins/therapeutic use*