Kabuki make-up syndrome associated with West syndrome

A Japanese boy with Kabuki make-up syndrome associated with West syndrome is reported. He developed periodic tonic spasms at 6 months of age while his electro-encephalogram also revealed hypsarrhythmia. Although only a few previously reported cases of Kabuki make-up syndrome have been associated with epilepsy, it is likely that epileptic seizures are another primary feature of Kabuki make-up syndrome.     

Society stress and peptic ulcer perforation

To examine the relationship between society stress and peptic ulcer perforation, time-trend analysis was performed on the annual incidence of perforated peptic ulcer per 100 000 population in Hong Kong during the years 1962–85, when Hong Kong, as a developing city, went through significant socio-economic and political changes, and the trend was correlated with specially designed and validated society stress scores estimated annually during the same period. The society stress scores were derived independently by two expert panels blinded to the purpose of the study, one selecting and categorizing negative news events for Hong Kong during this period, and the other weighing the categories and scoring the impact of the news on Hong Kong. The incidence of perforation increased significantly during the years and manifested three distinct peaks, which coincided with the worst economic recession in Hong Kong, the influx of mainlander Chinese and Vietnamese boat people, and the Sino-British negotiation on the sovereignty of Hong Kong after 1997. Both linear and autoregression analysis, the latter taking into consideration point fluctuations in rates, showed that perforation rates correlated significantly with the society stress scores (r= 0.57, P < 0.002). The peak effects and the significant correlations indicate that an association exists between society stress and peptic ulcer perforation, and suggest that chronic society stress plays an important role in the aetiology of this condition, although the relatively low r value also suggests the presence of other aetiological factors.     

THE INTERRELATIONSHIP BETWEEN BLEEDING,PERFORATION, AND STENOSIS IN DUODENAL ULCERATION

The interrelationship of gastrointestinal bleeding, acute perforation, and pyloroduodenal stenosis was examined in a hospital series of 1,457 patients with duodenal ulcers. The proportion of each complication among the total number of ulcer patients after various periods of ulcer symptoms was determined. There was little variation in the proportion with bleeding as the duration of symptoms increased, whereas the proportion with perforation decreased and the proportion with stenosis increased with increasing duration of ulcer dyspepsia. The association of bleeding with perforation (26 patients) was more common than the association of bleeding with stenosis (12 patients), and more so than the association of perforation with stenosis (4 patients). To explain the paradoxical relationship of perforation and stenosis it was proposed that perforation occurred when the pathological process in ulceration was such that there was more tissue destruction than tissue repair, and that stenosis would result if there was more tissue repair than tissue destruction.     

Decreased serum carnitine is independently correlated with increased tissue accumulation levels of advanced glycation end products in haemodialysis patients

Aim: There is accumulating evidence that advanced glycation end products (AGE) play a role in cardiovascular disease (CVD) in patients with haemodialysis (HD). Carnitine deficiency is frequently observed in HD patients, which may also contribute to CVD. In this study, we examined whether carnitine deficiency was independently associated with increased tissue accumulation levels of AGE in HD patients. Methods: One hundred and twenty‐nine HD patients underwent determinations of blood chemistries including serum level of carnitine. Tissue AGE levels were evaluated by measuring skin autofluorescence with an AGE‐reader. Results: Serum carnitine levels were significantly lower, while skin AGE levels were significantly higher in HD patients compared with healthy controls (P < 0.001). In univariate analysis, β₂‐microglobulin (β₂‐MG) and carnitine (inversely) were correlated with skin AGE levels. Multiple stepwise regression analysis revealed that carnitine levels were one of the independent determinants of skin AGE levels (P = 0.024). When β₂‐MG‐adjusted skin AGE levels were stratified by serum carnitine levels, a statistical significance and dose‐response relationship were observed (P = 0.043). Furthermore, skin AGE levels were one of the independent determinants of serum carnitine levels as well (P = 0.012). Conclusion: The present study demonstrated that decreased carnitine levels were independently associated with increased skin AGE levels in HD patients. Since carnitine is reported to inhibit the formation of AGE in vitro, our study suggests that supplementation of carnitine may be a therapeutic target for preventing the accumulation of tissue AGE and subsequently reducing the risk of CVD in HD patients.     

Success and complication rates of coronary angioplasty in patients with and without previous myocardial infarction

The primary success rate and incidence of major complicationshave been retrospectively assessed in a consecutive series of224 patients undergoing percutaneous transluminal coronary angioplasty(PTCA) in one centre. The patients have been divided into threegroups; those with angina and no previous myocardial infarction(Group I; N =130), those with angina and a previous transmuralmyocardial infarction (TMI) (Group 2; N=59), and those withangina and a previous non-transmural myocardial infarction (NTMI)(Group 3; N=26). The three groups were well matched for age,gender and angiographic severity of stenosis. The primary success rate in Group 1 was 90% compared to 64%in Group 2. The success rate in Group 3 lay in between at 77%.The lower success rates in Groups 2 and 3 were mainly due toan increase in the frequency of major complications. Acute coronaryocclusion occurred in seven patients in Group I, nine patientsin Group 2 and four patients in Group 3. In all these patientsin Groups 2 and 3 the outcome of acute occlusion was a procedure-relatedclinical myocardial infarction despite immediate re-angioplastyand/or emergency coronary arlery bypass grafting whereas onlyfour patients in Group I sustained an acute infact. In this series of patients undergoing coronary angioplasty forsymptom limiting angina, previous myocardial infarction appearsto be a risk factor for a lower success rate mainly due to anincrease in the frequency and severity of major complications.     

Abnormal gastro-oesophageal reflux in Chinese with atypical chest pain

Although atypical chest pain has been well described in the Western population, its frequency in Chinese is unknown. Over a period of 42 months, we studied 521 Chinese patients with chest pain and identified 108 patients (20.7%) whose pain was not related to cardiac causes, as determined by exercise ECG or cardiac catheterization. Using 24 h ambulatory pH monitoring and baseline oesophageal manometry, 28.7, 19.4 and 5.6% of these patients were found to have abnormal reflux parameters, abnormal manometric findings or both, respectively. There were significantly more patients complaining of chest pain during the study in the gastro-oesophageal reflux disease (GERD) group than in the non-GERD group (16/31 vs 20/77; P< 0.001). The lower oesophageal sphincter pressure was lower in those with abnormal reflex parameters than in those with normal reflux parameters (12.7±5.4 vs 17.8±5.8 mmHg; P< 0.05). There was no significant difference in symptoms, such as heartburn (54.8 vs 42.9%), regurgitation (38.7 vs 35.1%) and dysphagia (19.4 vs 24.7%), among the two groups. Non-specific changes were the most frequent baseline motility pattern. In conclusion, atypical chest pain and gastro-oesophageal reflux disease are not uncommon in Chinese and this deserves special emphasis as the continuation of anti-anginal drugs may aggravate their condition.     

Hypertrophic Pulmonary Osteoarthropathy and Nasopharyngeal Carcinoma in a Young Chinese Patient

This paper describes the case history of a young Chinese male who developed hyper-trophic osteoarthropathy associated with pulmonary metastases less than one year after successful treatment by radiotherapy of a poorly     

The Straight Back Syndrome

The straight back syndrome, consisting of loss of normal upperthoracic spinal curvature associated with cardiac murmurs andradiographic cardiom is considered a form of ‘pseudoheartdisease’ which has been attributed to squashing of theheart in the reduced AP diameter of the chest. During an 18-month period 31 patients referred to a cardiologistwere found to have a straight back. Forty-five relatives weresubsequently examined and 27 were found also to have a straightback. Palpitations and chest pain were the commonest symptoms.On the lateral chest radiograph the distance from the middleof the anterior border T₈ to a vertical line connecting T₄ andT₁₂ was found to be significantly reduced compared to controls,and a value less than 1·2 cm is indicative of a straightback. Of the 58 subjects with the syndrome, 39 (67 per cent) had clinicaland/or echocardiographic evidence of mitral valve prolapse.Respiratory function testing revealed no significant abnormality. HLA typing showed no association with any particular HLA antigenbut indicates that the straight back syndrome is inherited asan autosomal dominant condition and suggested that the antigenicdeterminants may be located on chromosome 6. We conclude that the straight back syndrome should no longerbe considered a form of pseudoheart disease and patients shouldbe investigated for associated mitral valve prolapse and theirrelatives screened.