Insulin-mediated pseudoacromegaly: a case report and review of the literature

A 23 year old female patient presented with oligoamenorrhea. She had excessive weight gain and had noticed hirsutism, enlargement of the jaw, increase in her ring and shoe size, increased sweating and darkening of her skin in flexural areas. Examination revealed a large framed woman with coarse facial features, large hands and feet, prognathism, acanthosis nigricans, hirsutism, acne and many skin tags. GH and IGF-1 were normal. MRI of pituitary showed a 7mm microadenoma, believed to be non-secretory with normal pituitary hormonal workup. She had marked elevation of serum insulin, elevated testosterone and mixed hyperlipidemia. The occurrence of acromegaloid manifestations is an unusual phenomenon seen in a subset of patients with insulin resistance. In vitro studies in fibroblasts obtained from such patients have revealed impairment of metabolic, but preservation of mitogenic insulin signaling. Insulin-mediated pseudoacromegaly is an unusual syndrome that combines severe insulin resistance and an acromegaloid phenotype. Physicians should consider this possibility while evaluating patients with similar clinical and laboratory features.     

PACHYDERMOPERIOSTOSIS

A case of fully developed idiopathic pachy- dermoperiostosis (idiopathic hypertrophic os- teoarthropathy) is reported. The main clinical manifestations are: skin thickening, espdcially over the face and cheeks, where the skin is drawn into thick folds; acral enlargement and gross clubbing of the fingers and toes; profuse sweating of the hands and feet and subperiosteal proliferation of long bone diaphysis shown in X-rays. Tufting t)f the distal phalanges and abnormalities of the sella turcica are not seen. It is important to recognize and distinguish the syndrome from pulmonary hypertrophic os- teoarthropathy, thyroid acropathy,. acromegaly and hypertrophic osteoarthropathy.     

Unusual presentation of progressive systemic sclerosis

A female 38 years old, housewife, presented to the Department of Dermatology and Venereology, Mymensingh Medical College Hospital (MMCH) on 08.04.07 with the complaints of i) pain and reduced movement of hand, knee, shoulder and neck joints for 1 year and 9 months ii) tightness of skin over face, neck, limbs and trunk for 1 year and 6 months iii) patchy depigmentation over same areas for 1 year and 3 months iv) deformity of hands with flexion contractures for 6 months and v) dysphagia to solid food for 3 months. She had no complaints of Raynaud's phenomenon. On general examination, she was ill looking, anemic and nutritionally poor. Examination of integumentary system showed smooth, shiny, thick, hard and hidebound skin with pigmentary alteration of 'salt and pepper' appearance over fingers, hands, limbs, face, neck and trunk. Hands appear claw like but more on the right side than the left and there were no other obvious changes suggestive of digital ischaemia (atrophy, ulceration, scarring, gangrene etc). Face has got suggestive features of scleroderma. Examination of the respiratory system showed restriction of chest movement and reduced expansibility of chest wall. No other abnormality was found on examination of other systems. Laboratory investigations showed histopathology typical of scleroderma. X-ray of hands and feet showed suggestive changes, lung function test-showed restrictive lung disease. Barium swallow x-ray of esophagus in supine position showed mild dilation of lower oesophagus. But serology was non-reactive (negative ANA, Negative RA test, Negative VDRL). So, she was diagnosed as a case of progressive systemic sclerosis (PSS) with some atypicality.     

微型游离腓动脉穿支皮瓣修复手足小创面27例效果观察

目的：探讨微型游离腓动脉穿支皮瓣修复手足小创面的临床价值。方法选取2009年3月至2013年12月于本院采用微型游离腓动脉穿支皮瓣进行修复治疗的27例手足小创面患者为研究对象,观察所有患者治疗后的皮瓣成活率、臃肿率、手足功能优良率及皮瓣部位感觉功能评估结果。结果患者的皮瓣成活率达到100.00%,手足功能优良率达到100.00%,皮瓣部位感觉功能持续改善,无一例皮瓣臃肿发生。结论微型游离腓动脉穿支皮瓣修复手足小创面的临床价值较高,患者治疗后的手足功能恢复更佳。     

Hydroquinone neuropathy following use of skin bleaching creams: case report

A 30-year old black woman presented with gradual onset of weakness of the legs associated with burning sensation in the feet for two months. She had been using two hydroquinone based skin bleaching creams (MGC by M. G. C. International, MEKAKO by Anglo Fabrics BOLTON Ltd) for about four years. Her BP was 80/40 mm Hg supine with un-recordable diastolic pressure on standing. She had decreased power (Grade 3/5), loss of deep tendon reflexes and impairment of deep sensation in the lower limbs. A complete blood count, urinalysis, serum electrolytes, serum creatinine and uric acid were all normal. Oral GTT, VDRL and brucella tests were negative. Chest and abdominal radiographs did not show any abnormalities. A diagnosis of peripheral neuropathy with autonomic neuropathy possibly due to hydroquinone toxicity was made and she was advised to stop using hydroquinone based skin bleaching creams. Four months later she was asymptomatic, her BP was 120/80 mmHg supine and standing, and neurological examination was normal. The case raises the question of whether hydroquinone based skin bleaching creams could be a cause of peripheral neuropathy and underscores the need for research on hydroquinone based skin bleaching creams and neuropathy particularly in black women involved in the sale and/or use of skin bleaching creams.     

Staphylococcal toxic shock syndrome in a small infant

Non menstrual staphylococcal toxic shock syndrome is rare in small infants. This is a 4-month-old infant presented to us with a picture of bronchiolitis and few postuler skin lesions, treated with antistapylococcal antibiotics in addition to other supportive medications. On the 4th day of therapy the patient developed sunburn like erythroderma, hypotension, and high grade fever. The dose of antibiotics was increased to the maximum possible dose, in addition to other supportive medications. The patient improved and developed extensive desquamation in both hands and feet on the 14th day of hospitalization, which confirms the diagnosis.     

恶性黑色素瘤80例临床病理分析

本文总结我室活检诊断恶黑80例,对其临床和病理进行详细分析。结果表明,恶黑主要发生在41-60岁年龄组,占54％,男性:女性1.8:1。皮肤恶黑的发生部位主要在手、脚、头面部、粘膜恶黑发生在鼻腔和眼部,恶黑的发病一般认为与日光照射、损伤、遗传等因素有关。恶黑组织结构复杂,细胞形态多样。多形的瘤细胞排列成肉瘤样,巢状、血管外皮瘤样、腺样等类型。同时也讨论了恶黑的分型、核分裂、浸润深度之间的关系。结节型核分裂最多且浸润深,预后就最差。     

Primary hypertrophic osteoarthropathy: clinical, radiologic, and scintigraphic characteristics

BACKGROUND: Our aim was to establish clinical and imaging characteristics of primary hypertrophic osteoarthropathy (PHO). METHODS: A group of 76 patients (71 men and 5 women) with primary hypertrophic osteoarthropathy was examined. Extensive history and status of the locomotor system were determined in all patients. Radiologic and scintigraphic examinations of the bones, thermography, capillaroscopy, gastroscopy, and histologic analysis were also performed. RESULTS: Family history was positive in all 55 patients (100%) summoned for a check-up. Finger clubbing of the hands and feet as well as positive Schamroth test were found in all patients, while hyperhydrosis was found in 65 (85.5%), seborrhea in 39 (51%), and involvement of the joints in 52 (68.5%) patients. Histologic analysis in 18 (23.7%) patients predominantly showed periarticular edema and moderate cellular activity. Periostosis of the long bones was also found in all patients, while scintigraphy, performed in 44 patients, was positive in 18 (33.5%) patients in the active phase of the disease. Thermography, performed in 10 patients, ranged from hypothermia to thermal amputation of feet and hands. Capillaroscopy of hands and feet in 47 (61.8%) patients showed changes in small blood vessels. Gastroscopy indicated abnormalities (ulcer and hypertrophic gastritis) in 21 patients (27.6%). CONCLUSIONS: PHO has special clinical and radiographic manifestations (finger clubbing and periostosis of the long bones) that are associated with some minor manifestations. Development of the disease is gradual (to 20 years) and requires a special diagnostic assessment.     

Harlequin fetus--a case report

A one-day-old male premature baby was born to a G4P4 mother with the gestation age of 30 weeks. His birth weight was 2000 gm. He was noted to be covered with a thick, armor-like skin which is split by deep fissures and makes the feature of Ectropion and eclabium. His nose was flattened, only the nostrils were visible, and the external ears were underdeveloped. The hands and feet appeared to be swollen and crumpled. The genital organ was covered with thick fissured skin and it was impossible to distinguish sex by external appearance. Three days after delivery, the patient died of respiratory distress. He was found normal through general laboratory and chromosome study. However, dermatohistology showed hyperkeratosis, focal parakeratosis and hypogranulosis. Pathology showed PDA (patent ductus arteriosus), enlarged thymus and congestion of internal organs. Its etiology and pathogenesis somehow remain unknown. We hereby reported this harlequin fetus, the most severe type of lamellar ichthyosis, and discussed its incidence, pathogenesis, pathohistology, prognosis and the recent development in treatment.     

The de Lange Syndrome: Report of Three Cases

Three cases of de Lange's syndrome are described. This condition is characterized by generally severe mental retardation, reduced stature, mild microcephaly, hypertrichosis, various anomalies of hands and feet, and a peculiar facies. The most outstanding features of the latter are the low forehead, profuse, generally confluent eyebrows, abundant long eyelashes, eyes that frequently slant downwards and outwards in antimongoloid fashion, pug nose with prominent anteverted nostrils, increased distance between nose and vermilion border of upper lip, slight reduction in size of chin, and often abnormally low-placed ears. The etiology of de Lange's syndrome is at present unknown.     

手足皲裂420例临床分析

目的寻求手足皲裂有效而且治疗费用低廉的治法。方法对420例手足皲裂的患者,采用内服与外治相结合,并进行统计和观察。结果420例患者经过3周的治疗,取得了良好的治疗效果。结论传统简便的“皲裂灵”外搽,配合口服复合维生素B片、浓缩鱼肝油丸和维生素E丸,仍不失为治疗手足皲裂的效、便、廉的治疗方法。     

Turner-mongolism polysyndrome. Review of the first eight known cases.

To date, a total of eight cases of the Turner-mongolism polysyndrome have been recognized. The clinical manifestations included retarded growth (resulting in a small and infantile appearance), with shield-like chest, poorly developed breasts, absent body hair, brachycephaly, short neck with foldings and low hairline, oblique eyes with epicanthal folds, squat nose, scrotal or normal tongue, abnormal hard palate (high or cleft), short hands and feet, frequent cubitis valgus, normal clitoris (may be either hypoplastic or peniform), mental retardation, and the XO/G+ karyotype, mosaic for XO in most instances. At this time, a single cause for all cases of the double aneuploidy is not known.     

尿毒症肾性骨病致退缩人综合征及面容改变1例并文献复习

目的报道并分析1例尿毒症透析患者合并严重肾性骨营养障碍致退缩人综合征、发生面容改变的病因及临床诊治特点。方法分析患者的临床及影像学资料并结合相关文献,探讨本病的临床特点及防治思路。结果该患者确诊尿毒症规律血液透析1年后出现血全段甲状旁腺激素（PTH）升高至2 208pg/L,曾行罗盖全2.0μg,2次/周冲击治疗4周,后停药。2年后颜面及双手关节逐渐变形并加重,伴脊柱左侧弯,严重骨痛及皮肤瘙痒。第6年出现＂右侧股骨自发性骨折＂,自透析以来身高缩短8cm。入我院后给予高通量血液透析、纠正钙磷代谢紊乱、甲状旁腺次全切除术后,患者骨痛及皮肤瘙痒明显缓解,PTH显著下降至160pg/L。结论通过早期干预慢性肾脏病钙磷代谢紊乱,合理补充活性VitD3,控制甲状旁腺激素在目标水平,减少重度肾性骨营养障碍的发生,提高患者生活质量。     

自身增强型可吸收材料治疗手足部骨折

目的评价自身增强型可吸收内固定材料治疗手足部骨折的效果.方法应用BIOFIX公司生产的自身增强型可吸收内固定材料对33例手、足部闭合性骨折实行内固定治疗,术后石膏外固定3周,观察治疗效果.结果随访9月-3年,33例骨折全部愈合,平均骨愈合时间松质骨8.8周,管状骨为11周,功能完全恢复,无并发症.结论自身增强型可吸收内固定材料可以替代金属材料治疗手足部骨折.     

One case of seminal fluid induced anaphylaxis

To the editor:Hypersensitivity to human seminal fluid is an uncommon phenomenon,which can manifest as localized or systemic clinical symptoms.Seminal fluid induced anaphylaxis is rarely reported in China till now.In this paper,we reported a Chinese patient with a rather severe attack of seminal fluid hypersensitivity.A 39-year-old woman was admitted to our Allergy Department for Angioedema and Urticaria after sexual intercourse with her husband,accompanied with itchy of the skin,nose and eyes,wheezing,dyspnea,sneezing,running nose,stuffy nose,without abdominal pain,vomiting and dizzy.She received artificial abortion several times and gave birth to the first child 3 years ago without birth control for 7years.After delivery 6 months,she had problem with the intercourse.The symptom attacked 4-5 times 3 years recently and condom could prevent the attacks.The patient ever went to the emergency room twice,blood pressure was 90/60 mmHg and lower than usual blood pressure 120/80 mmHg,her symptoms were relieved gradually after glucocorticoid and antihistamine was accepted.     

R-R interval variation and sympathetic skin response in fibromyalgia

BACKGROUND: This study proposed to assess the autonomic nervous system (ANS) functions in fibromyalgia (FM) by using two electrophysiological tests, sympathetic skin response (SSR) and the heart rate variability named R-R interval variation (RRIV). METHODS: Sympathetic skin response and RRIV were studied in 29 female patients with FM and 22 healthy age-matched female controls. R-R interval variation at rest (R%), during deep breathing (D%), the difference between D% and R% (D-R) and the ratio of D-R% (D/R) were determined. Pain threshold was measured using a mechanical algometer. RESULTS: R-R interval variation at rest (R%) and D/R did not show significant difference between patients and controls, whereas D% and D-R were significantly lower in patients compared to controls. SSR latencies of patients' hands and feet had no significant difference compared to controls' hand and feet SSR latencies. SSR latencies of patients' hands correlated significantly with control point score, total myalgic score, Hamilton Anxiety Rating Scale (HARS) and Hamilton Depression Rating Scale. Sympathetic skin response latencies of patients' feet correlated only with HARS. CONCLUSIONS: Analysis of heart rate variability may be useful and complementary to clinical examination in patients with symptoms of dysfunction in cardiovascular reflex pathways.     

糖尿病患者骨关节改变的临床探讨(附100例分析)

本组连续无选择性对100例糖尿病人进行双手,足及膝关节X线正位和斜位摄片,并与血糖、血脂、钙磷代谢及其他并发症之间的关系进行探讨。其中有程度不等的骨质疏松者76例(76％),其骨关节改变的发生率与病程、微血管病变及糖尿病性神经病变有密切关系;与患者年龄也有一定关系;而与糖尿病类型、血脂、碱性磷酸酶、血钙、磷的浓度以及24小时尿钙、磷浓度无显著相关。     

Fractures of Facial Bones

A detailed review was made of 1025 consecutive patients with “face-bone” fractures admitted to four Montreal hospitals over the five-year period 1958-1962, inclusive. In addition a survey was carried out of the other general hospitals in Greater Montreal in order to obtain admission figures for facial-bone fractures and for total hospital cases. In the study group the common causes of face-bone fractures were found to be fights, traffic accidents, falls, and athletic pursuits. Very few of these injuries occurred in industrial settings. A distinctly vulnerable group is made up of males between ages 16 and 35 years. In order of frequency of occurrence these injuries involve the nose, lower jaw, cheekbone, upper jaw, and zygomatic arch. The experience throughout metropolitan Montreal indicated that more persons with face-bone fractures require hospital treatment each year, but the increase is approximately parallel to the upward trend of total hospital admissions.     

Retinal pigment epithelial change and partial lipodystrophy

Cuticular drusen and retinal pigment epithelial changes were found incidentally in a 27 year old Lebanese woman during assessment of partial lipodystrophy. Her vision was normal despite involvement of both maculae. The patient had hypocomplementaemia, but serum C3 nephritic factor was absent and renal function was normal. She had impaired glucose tolerance and a continuous infusion of glucose with model assessment (CIGMA) test revealed low normal tissue insulin sensitivity and high normal pancreatic beta cell function. Mild fasting hypertriglyceridaemia (2.0 mmol/l) may have been secondary to impaired insulin sensitivity. Endocrine function was otherwise normal apart from a completely absent growth hormone response to adequate hypoglycaemia. The simultaneous occurrence of partial lipodystrophy and retinal pigmentary epithelial and basement membrane changes appears to be a newly recognized syndrome.     

新疆地方性慢性砷中毒119例五年随访结果

本文报告1987年4月对新疆奎屯地方性慢性砷中毒119例五年随访结果。1982年4月曾进行首次调查,1984年10月起当地改良饮用水,其含砷量降至0.045mg/L(原为0.6mg/L),含氟量0.095～1mg/L(原为3.45rag/L)。随访表明,病情好转及痊愈者共88例,占74％,主要表现为砷色素异常的色素沉着消失或减轻,砷角化全部消退者较少。神经系及心血管系症状有所加重。阵发性手足麻木多见,司能与神经炎有关。此外,雷诺氏现象及手足发绀、心电图异常较常见。发现皮肤癌4例,基底细胞上皮瘤3例,表皮内鳞癌l例,迄今尚未见内脏恶性肿瘤病例。本组2例死亡,死因与砷中毒的关系有待进一步研究。本组伴发氟中毒18例,与砷中毒有无协同或拮抗作用尚待观察。