A rare combination of recurrent pneumonia, diarrhoea, and visual loss in a patient after thymectomy: good syndrome.

This report describes a 68-year-old Chinese man who was diagnosed with Good syndrome 6 years after initial presentation when he underwent thymectomy. He presented with recurrent pneumonia, diarrhoea, weight loss, and visual symptoms. Extensive examination for anaemia and neutropenia was done, yet no conclusive diagnosis could be derived. During his last admission for pneumonia, his history of AB thymoma suggested the possibility of Good syndrome. Immunological testing revealed low T cells, absent B cells, and low immunoglobulin M and immunoglobulin G levels. Moreover, he had histologically identified cytomegalovirus pneumonia, cytomegalovirus colitis, and fundoscopic features of cytomegalovirus retinitis. He was treated with a 2-week course of intravenous ganciclovir, lifelong oral valganciclovir, and monthly immunoglobulin infusion. It took 6 years for the diagnosis to be established, therefore, early attention and vigorous search for such potentially treatable conditions in post-thymectomy patients presenting with recurrent infections is recommended.

Interactions of a very long chain fatty acid with model membranes and serum albumin. Implications for the pathogenesis of adrenoleukodystrophy.

Adrenoleukodystrophy (ALD) is an inherited disorder of fatty acid metabolism marked by accumulation of very long chain saturated fatty acids (VLCFA), especially the 26-carbon acid, hexacosanoic acid (HA), in membranes and tissues. We have studied interactions of 13C-enriched HA with model membranes (phospholipid bilayer vesicles) and bovine serum albumin (BSA) by 13C NMR spectroscopy to compare properties of HA with those of typical dietary fatty acids. In phospholipid bilayers the carboxyl group of HA is localized in the aqueous interface, with an apparent pKa (7.4) similar to other fatty acids; the acyl chain must then penetrate very deeply into the membrane. Desorption of HA from vesicles (t1+2 = 3 h) is orders of magnitude slower than shorter chain fatty acids. In mixtures of vesicles and BSA, HA partitions much more favorably to phospholipid bilayers than typical fatty acids. BSA binds a maximum of only 1 mole of HA at one binding site. Calorimetric experiments show strong perturbations of acyl chains of phospholipids by HA. We predict that disruptive effects of VLCFA on cell membrane structure and function may explain the neurological manifestations of ALD patients. These effects will be further amplified by slow desorption of VLCFA from membranes and by the ineffective binding to serum albumin.

Review of children hospitalised for ingestion and poisoning at a tertiary centre.

INTRODUCTION: The main aim of this study is to determine the pattern of referrals of poisoning to a tertiary university hospital. The information will be used for poison prevention programme planning, and for educating and awareness promoting to the public. MATERIALS AND METHODS: All patients (under 21 years of age) admitted to the paediatric wards between January 1997 and December 2002 with a discharge diagnosis indicating unintentional (UP) or intentional poisoning (IP) were identified through the computerised discharge information. RESULTS: Sixty males and 98 females accounted for 161 admissions over the 6-year period. Their mean (standard deviation, SD) age was 8.2 (6.2) years. Sixty per cent of admissions involved UP. Females accounted for 47% of the UP but 86% of the IP [odds ratio of females for IP, 7.05; 95% confidence interval (CI) 2.95 to 17.28]. When compared with UP, IP patients were significantly older [mean (SD): 14.9 (1.7) versus 3.6 (3.3) years]. In 70% of the admissions, the patients ingested a single substance. Tablets and pills, especially in the IP adolescents, were more commonly ingested than syrups. The spectrum of substances ingested was vast but paracetamol, cough or cold medicines, and common adult household medications and agents accounted for the majority of medications ingested. The substances ingested were obtained at home in 81 cases (50%) and as over-the-counter medication in 33 (20%). The majority (92%) of patients presented within 24 hours of ingestion. On admission, 63% of UP and 45% of the IP were asymptomatic. No active treatment was required in 65% of patients. In IP, nearly 30% of IP who ingested paracetamol had toxic levels and received N-acteyl cysteine. A history of previous poisoning was more common and subsequent follow-up was offered to 74%. CONCLUSION: Young boys were more at risk of unintentional ingestion whereas adolescent girls were more likely to ingest medications as a gesture of suicide. Paracetamol is a frequently ingested medicinal for which an antidote is available.

Potent contractile actions of prostanoid EP3-receptor agonists on human isolated pulmonary artery.

1. In 13 of 15 experiments, prostaglandin E2 (PGE2) and sulprostone (a prostanoid EP1/EP3-receptor agonist) contracted isolated rings of human pulmonary artery at low concentrations (> or = 5 and > or = 0.5 nM respectively). Tissue was obtained from patients undergoing surgery mainly for carcinoma of the lung. Characterization of the receptors involved was complicated by loss of sensitivity to the contractile PGE action over the experimental period. In contrast, contractile responses to KCl, phenylephrine and the specific thromboxane (TP-) receptor agonist, U-46619, did not decrease with time. 2. The relative contractile potencies for seven PGE analogues, measured during the first few hours after setting up the preparations, were as follows: sulprostone > misoprostol = gemeprost > or = PGE2 > or = GR 63799X > 17-phenyl-omega-trinor PGE2 > or = 11-deoxy PGE1. This ranking indicates that an EP3-receptor is involved. 3. The contractile action of sulprostone was not blocked by the TP-receptor antagonists, EP 169 and GR 32191, and the EP1-receptor antagonist, AH 6809. 4. In two experiments, PGE2 (50 nM) reduced basal tone and sulprostone was a weak contractile agent. Phenylephrine-induced tone was also inhibited by PGE2 (EC50 = 5-20 nM), 11-deoxy PGE1 and butaprost (a selective EP2-receptor agonist); the latter prostanoids were about 2 and 4 times less potent than PGE2 respectively. Interactions with phenylephrine were different in experiments where PGE2 alone was contractile: PGE2 induced contraction superimposed on the phenylephrine response and 11-deoxy PGE1 induced either further contraction or had no effect. Butaprost produced relaxation at high concentrations;this may not be an EP2 action since preparations were highly sensitive to relaxant actions of prostacyclin (IP-) receptor agonists (cicaprost and TEI-9063).5 The study has shown that in the majority of experiments on the human isolated pulmonary artery,the contractile EP3 system outweighed the relaxant EP2 system. However, in two experiments the reverse was true. It is not clear to what extent these differences are due to disease processes affecting the tissues.The findings are discussed in relation to the adverse cardiovascular responses occasionally encountered during treatment of postpartum haemorrhage with sulprostone, and more generally to the clinical use of EP-receptor agonists in man.

Video-assisted thoracoscopic thymectomy for myasthenia gravis.

Video-assisted thoracoscopic surgery (VATS) provides a new approach to thymectomy. From June 1993 to December 1994, we performed a total of eight thymectomies for myasthenia gravis (MG). There were four male and four female patients with ages ranging from 9 to 76 years. Three of the eight patients had associated thymoma. We believe that complete thymectomy was accomplished in all cases by examination of the thymic bed and resected specimen. There was no mortality or intraoperative complications. The median postoperative hospital stay was 5 days (range, 2 to 37 days). One patient required ventilatory support postoperatively. Clinical improvement was observed in all patients after a mean follow-up of 10 months (range, 2 to 21 months). Compared with a comparable historical group of patients with MG who underwent transsternal thymectomy, the VATS group was associated with significantly less analgesic requirement and shortened hospital stay. We conclude that VAT thymectomy is technically feasible and is associated with a favorable postoperative course compared with the transsternal approach. We believe that complete thymectomy can be achieved by this approach. Further investigation with long-term follow-up is needed to further clarify the role of VAT thymectomy in thoracic surgery.

Thoracoscopic management of malignant pleural effusions.

Malignant pleural effusion is a common condition and often presents a challenge for treatment. We report our experience from a single institution with the use of video-assisted thoracoscopic surgery (VATS) in the management of malignant effusions. From September 1992 to April 1995, 69 patients (31 men, 38 women; age range, 38 to 76 years) underwent diagnosis and/or treatment of malignant effusions; these included 46 pleural biopsies, 34 talc insufflations, and 16 limited decortications. There was no mortality and there were no intraoperative complications. Postoperative complications occurred in seven patients (10%). Specific histologic diagnoses were obtained in all but 6 patients (87%). Malignant effusion was confirmed in 25 of 46 cases (54%). Thoracoscopic talc insufflation with or without additional decortication was successful in 32 of 34 cases (94%) in controlling recurrence of effusion after a mean follow-up of 6 months among the survivors (22 patients died during the follow-up period without effusion reaccumulation). We conclude that VATS not only provides an accurate diagnosis but also allows effective therapeutic procedures to be performed for malignant effusions that are associated with an acceptable morbidity.

Video-assisted thoracoscopic anatomic lung resections. The initial Hong Kong experience.

We report our combined experience on video-assisted thoracoscopic (VAT) anatomic lung resections from two major hospitals in Hong Kong over a 17-month period. From August 1993 to December 1994, 82 cases of major lung resections were attempted using the VATS approach, of which 60 were successfully completed (55 lobectomies, 2 bilobectomies, 2 pneumonectomies, and 1 segmentectomy). Of these 60 cases, there were 43 men and 17 women with a mean age of 66 years (range, 37 to 85 years). The final pathologies were 52 primary lung cancers (37 adenocarcinoma, 11 squamous cell carcinoma, 2 bronchoalveolar carcinoma, 1 adenosquamous carcinoma, and 1 undifferentiated carcinoma); 1 pulmonary metastasis (from nasopharyngeal carcinoma); and 7 benign lesions (3 tuberculosis, 1 bronchiectasis, 1 sclerosing hemangioma, 2 organizing pneumonia). There was one postoperative death (mortality rate, 1.8%). Complications include persistent air leak over 10 days (four), wound infection (two), supraventricular tachycardia (three), and recurrence of tumor over the utility thoracotomy scar (one). All the patients were followed up from 8 weeks to 19 months (mean, 10 months). The mean duration of chest drainage was 5.4 days (range, 2 to 25 days). The mean hospital stay was 7.2 days (range, 4 to 35 days). The average postoperative parenteral narcotic (meperidine hydrochloride [Pethidine]) requirement by patient-controlled analgesia was 275 mg (range, 75 to 800 mg). This compared favorably with an age- and sex-matched historic group of patients who underwent posterolateral thoracotomy and had a hospital stay of 10.4 days (statistically non-significant) and narcotic requirement of 950 mg (statistically significant by paired t test). We conclude that VAT anatomic lung resection is technically feasible. However, there are some specific complications associated with major lung resection through minimal access. Refinement of our present technique and attention to details are important to improve our results.

Thoracoscopic talc insufflation versus talc slurry for symptomatic malignant pleural effusion.

BACKGROUND: Talc has been generally accepted to be the most effective sclerosant for chemical pleurodesis, although the optimal route of administration remains unclear. METHODS: We designed a prospective, randomized study to compare video-assisted thoracoscopic talc insufflation with bedside talc slurry in the treatment of malignant pleural effusion. From September 1993 to November 1995, 57 patients were recruited and randomized to either video-assisted thoracoscopic talc insufflation under general anesthesia (n = 28) or talc slurry by the bedside (n = 29). Patients with poor general condition (Karnofsky score less than 30%), poor pulmonary function (forced expiratory volume in 1 second less than 0.5 L), or trapped lungs were excluded from this study. Five grams of purified talc was used for either video-assisted thoracoscopic talc insufflation or talc slurry. RESULTS: There was no statistically significant difference between the two groups of patients with respect to age, sex ratio, chest drainage duration, postprocedural hospital stay, parenteral narcotics requirement, complications, or procedure failure (ie, recurrence). CONCLUSIONS: Video-assisted thoracoscopic talc insufflation has not been shown to be a superior approach compared with talc slurry in our study. Because the former demands more resources, we advocate that talc slurry should be considered as the procedure of choice in the treatment of symptomatic malignant pleural effusion in patients who do not have trapped lungs.

Growth inhibitory activity of lemnabourside on human prostate cancer cells.

Induction of apoptosis and androgen ablation are two major approaches for treating human prostate carcinoma. In a study of the bioactive components of the soft coral Nephthea chabroli, we found that lemnabourside is a 5alpha-reductase inhibitor, as shown by its ability to inhibit the conversion of testosterone into the more potent dihydrotestosterone in rat prostate homogenate. The compound also inhibited the incorporation of tritiated thymidine into human prostate androgen-dependent carcinoma LNCaP cells, and thus blocking the cell proliferation (IC50 = 37.5 microM). The expression of prostate marker genes, including 5alpha-reductase, prostate-specific antigen, prostatic acid phosphatase and androgen receptor, and the anti-apoptotic bcl-2 gene were markedly reduced, but the transcription of apoptosis-related caspase 3 gene showed a dose-dependent increase in lemnabourside-treated LNCaP cells. Immunofluorescent microscopy and flow cytometric analysis further demonstrated apoptotic changes in these cells. Taken all results together, a relatively weak 5alpha-reductase inhibitory activity on LNCaP cells (EC50 > 250 microM), and a similar growth inhibitory activity on both androgen dependent- and independent-prostate cells (IC50 approximately 37.5 microM) indicated that caspase-3 apoptosis pathway is one of the possible antiproliferative activities mediated by lemnabourside.

Echocardiography as a tool for determining the incidence of congenital heart disease in newborn babies: a pilot study in Hong Kong.

We studied, with echocardiography as the main tool, the incidence of congenital heart disease in newborn babies in Hong Kong. The population examined was the 20,928 babies who were born alive in the Prince of Wales Hospital from January 1987 to December 1989. All had a thorough physical examination by a paediatrician after birth, and an estimated 95% of the babies who were discharged from hospital received another routine physical examination at one of the five local Maternal and Child Health Centres within two months of birth. All babies with either suspected congenital heart disease or multiple congenital abnormalities were referred to the paediatric cardiologists in the Prince of Wales Hospital for further cardiovascular assessment that included echocardiographic examination. In all, 492 babies had Doppler and cross-sectional echocardiographic studies. Various abnormalities of the cardiovascular system were diagnosed in 216 babies. Almost all the babies who died within two months of life underwent autopsy. This proved the presence of congenital cardiac malformations in 15 babies, of whom 10 had correct echocardiographic diagnoses during life. One had a patent arterial duct which was missed by the echocardiography. The other four babies did not have echocardiographic examination while alive, either because of early death or absence of clinical suspicion. In total, congenital cardiac malformations were confirmed in 221 babies by echocardiographic examination and autopsy. Excluding 82 premature babies with patency of the arterial duct, and 6 babies with transient tricuspid regurgitation, there were 133 cases of structural cardiac malformation in the studied population, giving an incidence of 6.35 per thousand live births.(ABSTRACT TRUNCATED AT 250 WORDS)

A case report of a large thymic carcinoid.

Thymic carcinoid is a rare tumour. A case of giant thymic carcinoid with extensive necrosis is reported in a 44-year-old Chinese male. Diagnosis was difficult to make before the operation and even intraoperatively. Subtotal excision was performed followed by adjuvant radiation therapy. The patient remained disease-free at the last follow-up 12 months after surgery. Complete excision remains the mainstay of treatment for this disease.

Subretinal fluid is common in experimental non-arteritic anterior ischemic optic neuropathy.

PURPOSE: Anterior ischemic optic neuropathy (AION) is an important cause of acute vision loss for which several animal models exist. It has been associated with subretinal fluid in a previous study on patients but not yet so in animal models. PATIENTS AND METHODS: A patient presented with acute non-arteritic AION (NAION) and underwent ophthalmic evaluation and testing including fluorescein angiography and spectral-domain optical coherence tomography (SD-OCT). On the basis of the patient's findings, we used SD-OCT circular and volume scans to analyze retinal changes in a murine model of NAION. RESULTS: One week after left eye vision loss, the patient had clinical and imaging findings consistent with NAION. On SD-OCT, there was prominent peripapillary retinal thickening consistent with intra-retinal edema and sub-foveolar fluid. Inspired by the findings in human AION, we looked for similar changes in murine NAION using SD-OCT. The circular scan did not adequately detect the presence of subretinal fluid. Using the 25-line scan, which covered a larger part of the posterior pole, we found that 100% of murine AION resulted in subretinal fluid at day 1. The subretinal fluid resolved by week 1. CONCLUSION: This study detailed a case of clinical NAION associated with intra-retinal and subretinal fluid. We also found that subretinal fluid was common in murine photochemical thrombosis model of AION and could be found far away from the optic disc.

One hundred consecutive cases of video-assisted thoracoscopic surgery for primary spontaneous pneumothorax.

We reviewed our experience on video-assisted thoracoscopic surgery (VATS) from our first 100 cases of primary spontaneous pneumothorax (PSP) performed at our institution from September 1992 to January 1994. Apical bullae were identified in 87% of cases. Mechanical pleurodesis with Marlex mesh was performed on all patients. Excision with endoscopic staple cutter was performed in 69 cases; an endoloop was used in five cases; ablation with an argon beam coagulator (ABC) was done in six cases; excision with endoscopic suturing occurred in seven cases; and mechanical pleurodesis alone was used in 13 cases. The overall median postoperative chest tube duration was 2 days (range 1-25 days) and hospital stay 4 days (range 1-30 days). Complications occurred in 8 cases (8%): 1 wound infection; 1 chest wall bleeding; and 6 persistent air leaks which lasted for more than 10 days (one of which eventually required an axillary thoracotomy for control). Procedure failure with recurrence occurred in three cases (3%) at a mean follow-up of 17 months (range 8-24 months). The ABC group alone was responsible for one recurrence and two persistent leaks. We conclude that with the VATS approach in the treatment of PSP, staple resection of apical bullae is quick and most reliable but costly. Endoloop and hand suturing are applicable to selected patients with small and localized bullae and should be further studied, while our limited experience does not favor ABC as the primary treatment modality.

Video assisted thoracoscopic staple resection of a giant bulla.

Video assisted thoracoscopic surgery (VATS) for spontaneous pneumothorax has been well described. However, there are few reports on the use of this technique on giant bullae. We report a 49 year old man with a symptomatic giant bulla who underwent a successful thoracoscopic staple resection. His chest drains were removed on the second postoperative day and he was discharged on the third postoperative day with virtually no discomfort. We conclude that thoracoscopic staple resection of a giant bulla is feasible technically and is an attractive alternative to the conventional open technique.

Video-assisted thoracoscopic lobectomy: a word of caution.

Advances in video-assisted thoracoscopic (VAT) surgery allow it to assume an increasingly important therapeutic role. Two successful VAT lobectomies (left upper lobe and right lower lobe) were performed in patients with stage I adenocarcinoma. An 8 cm access minithoracotomy for hilar dissection and subsequent specimen delivery was used. Routine mediastinal node sampling was performed thoracoscopically as would be done conventionally. Video-assisted thoracoscopic lobectomy of the right upper lobe was attempted in another patient but he required emergency conversion to open thoracotomy because of bleeding from mechanical failure of the vascular staple-cutter. Although VAT lobectomy is feasible technically, attention to detail is important when mechanical devices are used to minimize the chance of malfunctioning. Once disaster occurs the surgeon should be capable of dealing with the consequences.