The Caenorhabditis elegans CED-9 protein does not directly inhibit the caspase CED-3, in vitro nor in yeast.

A genetically defined pathway orchestrates the removal of 131 of the 1090 somatic cells generated during the development of the hermaphrodite nematode Caenorhabditis elegans. Regulation of apoptosis is highly evolutionarily conserved and the nematode cell death pathway is a valuable model for studying mammalian apoptotic pathways, the dysregulation of which can contribute to numerous diseases. The nematode caspase CED-3 is ultimately responsible for the destruction of worm cells in response to apoptotic signals, but it must first be activated by CED-4. CED-9 inhibits programmed cell death and considerable data have demonstrated that CED-9 can directly bind and inhibit CED-4. However, it has been suggested that CED-9 may also directly inhibit CED-3. In this study, we used a yeast-based system and biochemical approaches to explore this second potential mechanism of action. While we confirmed the ability of CED-9 to inhibit CED-4, our data argue that CED-9 can not directly inhibit CED-3.

Use of developmental language scales in Chinese children.

The Reynell Developmental Language Scale (RDLS) and Symbolic Play Test (SPT) have been useful language tests for assessing the language age of children. Both tests have been validated in English-speaking children. However, there have been no studies conducted for Chinese children, whether Mandarin (Northerners) or Cantonese (Southerners) is used as the main dialect. As the Chinese population is the largest ethnic group in the world, and Chinese emigration occurred to nearly all parts of the world, it is essential to test whether these language tools can be applied for this ethnic group. The objective of this research was to study whether RDLS and SPT are useful in assessing the language age of Chinese children. Both RDLS (Chinese version) and SPT are conducted for 116 Chinese (Cantonese-speaking) children, aged 13-59 months, in Hong Kong. There is a significant positive correlation of the language age using RDLS and SPT with the chronological age of Chinese children. Both RDLS and SPT can be adopted in determining the language/mental age of Chinese (Cantonese-speaking) children.

The human secretin receptor gene: genomic organization and promoter characterization.

Secretin is the most potent regulator of pancreatic bicarbonate, electrolyte and volume secretion. In this report, the organization of the human secretin receptor (hSR) gene was characterized by overlapping genomic phage clones. The hSR gene consists of 13 exons and 12 introns with all the splice donor and acceptor sites conforming to the canonical GT/AG rule. By transient reporter gene assays, the wild-type promoter, containing 3.0 kb of the hSR gene 5' flanking region, was able to drive 5.8 +/- 0.6 and 6.6 +/- 0.2-fold (P < 0.01) increases in luciferase activities in pancreatic ductule-derived PANC-1 and BPD-1 cells, respectively. By subsequent 5' and 3' deletion analysis, a promoter element was identified within -408 to -158, relative to the ATG codon. This promoter element was found to be cell-specific since it could drive reporter gene expression in PANC-1 and BPD-1 cells but not in Hs 262.St, Hs 746T and alphaT3-1 cells. The study of the transcriptional control of human secretin and its receptor should shed light on the pathological developments of pancreatic cancer and autism in the future.

Second-trimester maternal serum alpha-fetoprotein and human chorionic gonadotrophin screening for Down's syndrome in Hong Kong.

Second-trimester maternal serum screening for fetal Down's syndrome is well established in many Western countries. Its usefulness and acceptability is unknown in the Asian countries. Between June 1994 and December 1996, we offered second-trimester serum AFP and hCG screening to pregnant women in Hong Kong who were less than 35 years old and without other risk factors for chromosome abnormalities. Each woman was assigned a risk of having a Down's syndrome term pregnancy by using a computer software program that took into account her age. weight, AFP and hCG MOMs. All those with a risk of one in 250 or greater were designated screen-positive, subject to the revision of gestation by ultrasound examination. 9,177 women with singleton pregnancies (93 per cent were Chinese) were screened. The uptake of screening was 75 per cent. 281 women (three per cent) were initially classified to be screen-positive. After revision of the gestation by ultrasound examination, 183 women (two per cent) were 'true' screen-positive and 164 (90 per cent) accepted the offer of amniocentesis. Eight of these pregnancies were affected by Down's syndrome and all the women elected pregnancy termination. The odds of being affected, given a positive screening result, were one in 23. Six Down's syndrome pregnancies were missed by screening programme. The detection rate was 57 per cent. The study showed that second-trimester serum screening for fetal Down's syndrome was feasible and accepted in the Hong Kong population.

Second-trimester maternal urinary gonadotrophin peptide screening for fetal Down syndrome in Asian women.

Maternal urinary gonadotrophin peptide (UGP) was recently found to be elevated in second-trimester Down syndrome pregnancies. There is controversy about its screening efficacy. Data in the Asian population are scanty. We measured the UGP levels in 29 Down syndrome pregnancies and 297 controls and expressed them as a ratio of the urine creatinine concentration. Median UGP MOM was elevated to 3.44 (95 per cent confidence interval 1.99-5.13) in cases, compared with controls. UGP values were widely scattered in cases and controls with extensive overlap such that only 35 per cent of Down syndrome cases were expected to be detected at a 5 per cent false-positive rate. The data of the present study and previous reports show that there is a wide variability of gestation-specific UGP median values. UGP alone does not appear to be an effective screening marker for fetal Down syndrome.

Burst-shear flexion-distraction injuries of the lumbar spine.

Seven cases of a previously undescribed lesion of the lumbar spine consisting of a burst fracture of the vertebral body associated with a posterior subluxation of the adjacent lower level facet joints are described. The lesion is due to a flexion-distraction mechanism. All seven cases reported involve a burst fracture (four upper burst, one burst-split, and two complete burst fractures). The dislocation line goes through the upper end-plate, through the posterior wall of the fractured vertebra, through the spinal canal, and through the caudal facet joints. The caudal disk is not destroyed primarily, but is involved in cases of burst-split or complete burst fractures. The treatment is surgical: reduction of the posterior subluxation, reduction of the burst fracture with anterior distraction (e.g., AO internal fixator or any other pedicle system allowing anterior distraction and reduction of the burst fracture), transpedicular bone grafting of the burst fracture if necessary, and fusion of the destroyed motion segment(s).

A comparison of results of extensor tendon repair followed by early controlled mobilisation versus static immobilisation.

To compare the functional results of early controlled mobilisation and static immobilisation following repair of extensor tendons, we conducted a comparative study between two centres. In one, a consecutive series of tenorrhaphy patients was treated post-operatively by the dynamic splinting technique. In the other, a consecutive group was treated by static splinting. All patients treated by dynamic splinting were graded excellent within six weeks following surgery; no tendon ruptures occurred and no secondary corrective tendon surgery was required. After static splinting, 40% were graded excellent, 31% good, 29% fair, and none poor; six fingers treated by static splintage subsequently required tenolysis. Following surgical repair of extensor tendons of the hand, patients treated by early controlled motion regain better flexion function in terms of grip strength and pulp-to-palm distance. Dynamic splinting is a more effective technique than static splinting in the prevention of extensor lag.

Pigmented villonodular synovitis presenting as a large lateral knee mass. Case report and review of the literature.

Pigmented villonodular synovitis is characterized by synovial villonodular lesions with hemosiderin pigmentation and a stromal infiltrate of histiocytes. Analogous lesions may occur in joints, bursae, and tendon sheaths. Although the etiology and natural history of pigmented villonodular synovitis remain unknown, it should be considered a benign entity. It usually presents as either diffuse monoarticular involvement with chronic pain and swelling, or a more localized nodular lesion, typically involving the fingers or knees. Although treatment involves excision of the involved tissue, due to its benign behavior, radical surgical procedures are not indicated. A case report of a large, extra-articular mass is described, with histologic and clinical changes consistent with localized PVS.

Complex dorsal dislocation of the second carpometacarpal joint.

Isolated dislocation of the carpometacarpal joint of the index finger is a rare injury. This paper reports a case of an isolated dorsal dislocation of the second carpometacarpal joint not reducible by closed means. At open reduction, the extensor carpi radialis brevis was found to be interposed in the joint, blocking reduction.

Flexor tendon repair and rehabilitation in zone II open sheath technique versus closed sheath technique.

A comparative prospective study of the surgical management of the tendon sheath after repair of flexor tendons in zone II is reported. The study included only patients with lacerations of both flexor tendons and no other associated injuries. A modified Kessler suture was used to repair the profundus tendon and the superficialis tendon was repaired with a horizontal mattress suture. In 48 fingers the flexor tendon sheath was left open and it was closed in the second group of 42 fingers. When it was impossible to close the tendon sheath, a vein patch was taken from the dorsal veins of the hand. Both groups of patients were treated with the same regimen of controlled motion rehabilitation and supervised by the same hand therapist. Results were evaluated by the Strickland formula for total active motion of the proximal and distal interphalangeal joints. There was no statistical difference between the results of open sheath versus closed sheath in these two groups of patients treated postoperatively with the same controlled motion rehabilitation program.

Aneurysms of the upper extremity.

Thirty aneurysms in the upper extremity in 28 patients over the last 10 years are reviewed. Analysis showed that false aneurysms develop from penetrating trauma, while true aneurysms tend to arise in parts of the arterial tree exposed to blunt trauma. Penetrating injury to vessels should be thoroughly explored and repaired. Arterial aneurysm should be included in the differential diagnosis of masses in the upper extremity, especially after trauma. Three-phase radionuclide scanning is a useful tool for evaluating lesions of the distal arterial tree. When an aneurysm is suspected, early treatment is advised. Treatment options of resection and ligation versus reconstitution of vessel flow should be based on preoperative and intraoperative evaluation of circulatory status.

Mycotic aneurysms of the upper extremity, report of two cases.

Two cases of mycotic aneurysm of the brachial artery secondary to illicit drug injection are described. Review of the literature shows an increased incidence of mycotic aneurysms secondary to intravenous/intra-arterial drug injections. There is a greater propensity for these aneurysms to rupture, thus early diagnosis and adequate antibiotic and surgical treatment is advised. Resection of the aneurysm and ligation of the vessel is the procedure of choice if adequate distal circulation exists.

Trapezium implant arthroplasty: evaluation of a semiconstrained implant.

Twenty-five patients who presented with symptoms of disabling pain secondary to arthritis at the base of thumb had 29 arthroplasties with silicone rubber trapezium implants. Stabilization was achieved with tenodesis using a strip of abductor pollicis longus as described by Eaton. All patients had relief of pain after surgery. We examined 23 hands in 19 patients for follow-up. Average follow-up was 31 months. Assessment of functional status and measurements of prehensile capabilities showed that all patients had improved after surgery. Range of motion of the thumb was measured (radial abduction averaged 40 degrees; 80% had full opposition). Average key pinch was 8 pounds. Stability of the implant was assessed with standard posteroanterior, lateral, and stress views of the trapeziometacarpal joint. Comparisons were made with previously reported results for Eaton and Swanson trapezium implants. The tenodesis effect may provide a more stable, although possibly less mobile, CMC joint of the thumb.

True aneurysms of the hand resulting from athletic injury. Report of two cases.

We report two cases of true aneurysms arising in the vasculature of the hand after blunt athletic trauma. This relatively rare tumor of the hand should be included as part of the differential diagnosis of masses arising in the hand after athletic injury.

Ocular findings in anencephaly.

We found an upper eyelid notch and posterior pole lens in a newborn anencephalic infant. An analysis of the development of the affected tissues helped localize the insult at the fifth to tenth week of gestation, probably close to the fifth week.