Spinal anaesthesia in a child with Job''s syndrome, pneumatoceles and empyema

We present a case of acute bowel obstruction in an immunocompromised child, who also had lobar pneumonia and a giant unilateral pneumatocele. She was successfully managed with subarachnoid anaesthesia for exploratory laparotomy to relieve a colonic obstruction. This proved to be a safe alternative to general anaesthesia with tracheal intubation in this patient and should be considered in infants and children in selected cases whenever a contraindication to general anaesthesia exists.     

BIOCHEMICAL CORRECTION IN THE SYNDROME OF HYPERTENSION AND HYPERKALAEMIA BY SEVERE DIETARY SALT RESTRICTION SUGGESTS RENIN-ALDOSTERONE SUPPRESSION CRITICAL IN PATHOPHYSIOLOGY

1. Plasma potassium and chloride concentrations were raised and plasma renin activity, aldosterone, bicarbonate and arterial pH were reduced in two brothers with the syndrome of hypertension and hyperkalaemia with normal glomerular filtration rate (Gordon's syndrome), on unrestricted or moderately restricted sodium diets. 2. These abnormalities were corrected in both patients within 10 days of severe sodium restriction. 3. Pressor sensitivity to cold and angiotensin II decreased on low sodium diet, associated with a fall in blood pressure. 4. Increasing distal tubular sodium delivery by infusion of normal saline increased fractional excretion of potassium when aldosterone had been stimulated by severely restricted sodium diet, but not when aldosterone levels were low on unrestricted sodium diet. 5. These findings are consistent with excessive sodium reabsorption as the primary renal lesion in Gordon's syndrome, leading to volume expansion and suppression of renin and aldosterone. Severe dietary sodium restriction leading to volume contraction, by stimulating renin and aldosterone and promoting kaliuresis, corrects the abnormalities.     

婴儿肝炎综合征63例临床分析

对我科1989-1990年中收治的婴儿肝炎综合征63例的临床表现、合并症、治疗等方面进行了分析。笔者认为婴儿肝炎综合征为一组临床症候群。病因诊断有其重要性。腹部B超检查有助于肝炎和胆道闭锁的鉴别,可作为常规检查方法。并认为用肾上腺皮质激素治疗有效。     

Bilateral Ulnar Agenesis: Case Report and Review of the Literature

Bilateral ulnar agenesis is a rare abnormality. A total of 36 cases are analyzed: 35 of these are documented in the literature and 1 stillborn male is presented in this study. Most patients had one of the three conditions: Al-Awadi/Raas-Rothschild syndrome, syndrome of ulnar aplasia with split hand/split foot deformity, or the Brachmann—de Lange syndrome. Fifty percent of all cases with bilateral ulnar agenesis were associated with lower limb defects and these cases, for the most part, also belonged to the aforementioned syndromes. Nonskeletal, internal organ malformations were identified in 34% of all patients. Nine patients presented with isolated bilateral ulnar agenesis. The Al-Awadi/Raas-Rothschild syndrome and the split hand/split foot deformity are heritable disorders. There was no evidence for genetic etiology in most of the other cases. Bilateral ulnar agenesis in our fetus was part of the Brachmann—de Lange syndrome with associated cardiac defect, diaphragmatic hernia, and umbilical artery agenesis.     

双胎输血综合征29对临床分析

目的：探讨新生儿双胎输血综合征的临床表现及其合并症等问题。方法：对过去10年间收治的29对双胎输血综合征患儿资料进行回顾性分析。结果：双胎输血综合征患儿有一般双胎儿的临床共性，且双胎输血综合征患儿中受血儿出现病理临床表现及合并症较供血儿多且严重。结论：双胎输血综合征对受血儿危害更大，故临床上一旦确诊为双胎输血综合征，应对受血儿进行严密监护，若出现临床表现，并发生红细胞增多症，应积极采取治疗措施，以减少严重并发症及后遗症的发生。     

A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

Image processing in digital radiography

Image processing is a critical part of obtaining high-quality digital radiographs. Fortunately, the user of these systems does not need to understand image processing in detail, because the manufacturers provide good starting values. Because radiologists may have different preferences in image appearance, it is helpful to know that many aspects of image appearance can be changed by image processing, and a new preferred setting can be loaded into the computer and saved so that it can become the new standard processing method.Image processing allows one to change the overall optical density of an image and to change its contrast. Spatial frequency processing allows an image to be sharpened, improving its appearance. It also allows noise to be blurred so that it is less visible. Care is necessary to avoid the introduction of artifacts or the hiding of mediastinal tubes.     

Anti-gal-C antibody in autoimmune neuropathies subsequent to mycoplasma infection

Four of 82 patients with Guillain-Barré syndrome (GBS) and 1 of 12 with multifocal motor neuropathy (MMN), who previously had had Mycoplasma pneumoniae infections, had serum antibody to galactocerebroside (Gal-C). Two patients with GBS without mycoplasma infection also had anti-Gal-C antibody, whereas none of the normal or the disease controls had it. As Gal-C is a major glycolipid antigen in myelin, anti-Gal-C antibody may function in the pathogenesis of autoimmune demyelinative neuropathies. Mycoplasma pneumoniae appears to be an important preceding infectious agent in autoimmune neuropathies with anti-Gal-C antibody. © 1995 John Wiley & Sons, Inc.     

Familial Sneddon's syndrome

We report the familial occurrence and apparent autosomal dominant inheritance of Sneddon's syndrome with variable clinical expression. The proband, a 40-year-old woman, presented with livedo reticularis and progressive neurological deterioration following a stroke. The diagnosis was confirmed by cerebral angiogram and skin biopsy, both showing the characteristic findings. Two of the patient's sisters were reported to have been similarly affected in the past. Her mother, two additional siblings and five of her seven children exhibited various vasospastic skin phenomena. Familial aggregation of this disorder may be common and a genetic basis may be involved in its pathogenesis.     

中医对干燥综合征治法和用药初探

干燥综合是一种全身性自身免疫病,目前西医对本病无疗法。因此探讨中医对本病的治法和用药,提高临床有效率是十分必要的。本文在中医辯证理论的指导下,提出了:益气养阴、养阴清热、琉风清热、养血活血、清热化湿、痹通络等六种治疗方法和用药初探,以供临床参考应用。