Childhood cardiac function after twin-to-twin transfusion syndrome – a 10-year follow up

Aim:  To perform a 10-year follow up of cardiac structure and function after twin-to-twin transfusion syndrome (TTTS) – a severe foetal circulatory complication associated with myocardial hypertrophy in the recipient twin.
Methods:  Cardiac dimensions, systolic and diastolic function as assessed by echocardiography including flow and tissue Doppler velocimetry in 22 healthy survivors of TTTS with a mean age of 9.6 (7.2–11.8) years.
Results:  The donor and recipient twin did not show any differences in end-diastolic ventricular size, interventricular septum thickness, diameter of right ventricular outflow tract, cardiac valves, coronary arteries or in systolic blood flow velocities. However, compared with the donors, the recipients had significantly lower E/A ratios because of lower E-waves in both mitral (−0.15 ± 0.10, p < 0.01) and tricuspid (−0.09 ± 0.07, p < 0.01) valves, indicating reduced early diastolic ventricular fillings compared with donors.
Conclusion:  At school age, twins surviving TTTS had a cardiac structure and function within normal range. There were no differences in heart structure or systolic ventricular function between twins but, compared with the donor twin, we found a reduced early diastolic function in the recipient.     

Evaluation of the anticonvulsant and muscle relaxant effects of the methanol root bark extracts of Annona senegalensis

ObjectiveTo investigate the potentials of the root bark of Annona (A.) senegalensis in the control of seizure and related hypnotic and motor incoordination effects in mice using experimental models.MethodsThe methanol extract (ME) of the root bark of A. senegalensis was studied in mice using pentylenetetrazole (PTZ) induced convulsions, phenobarbitone induced sleeping time and motor coordination test on rota-rod performance. Acute toxicity and lethality (LD₅₀) test as well as phytochemical analysis were also carried out.ResultsThe extract (200, 400, 800 mg/kg) exhibited a non-dose dependent significant (P <0.05) delay in the onset of both tonic and clonic phases of seizure induced by PTZ (60 mg/kg, s.c.) as well as offered a 100% protection (200 mg/kg) in mice from PTZ induced seizures. The extract significantly (P <0.05) decreased the latency and increased the duration of phenobarbitone induced sleeping time. At 200 mg/kg, the extract exhibited a significant (P <0.05) motor incoordination. The acute toxicity test revealed an oral LD₅₀ of 1 296 mg/kg, while the phytochemical studies showed the presence of alkaloids, resins, glycosides, carbohydrate, reducing sugar, flavonoids, terpenoids, saponins and tannins.ConclusionThe extract of A. senegalensis possessed anticonvulsant activity with pronounced hypnotic and muscle relaxant effects.     

CYP11β1 (11-beta-hydroxylase) deficiency in congenital adrenal hyperplasia

Objective : To review experience of CYP11β1 deficiency (previously known as 11β-hydroxylase) at the Royal Children's Hospital, Melbourne, Victoria.
Methodology : A retrospective case review was conducted from 1974 to 1995 with five cases identified.
Results : Age of presentation ranged from 1 day to 7 years. Presentation was with ambiguous genitatia at birth (two females), simple virilization (two males) and suspected early puberty in mid childhood (one female). Associated clinical features were hypertension (three cases) and tall stature with markedly advanced bone age (four cases). Biochemical abnormalities consistent with CYP11β1-deficiency were elevated urinary tetrahydro-11-deoxycortisol ( n = 5) and elevated serum 11-deoxycortisol ( n = 3). Additional abnormalities were elevated 17-hydroxyprogesterone ( n = 3), elevated androstenedione ( n = 4) and elevated dehydroepiandrosterone sulphate ( n = 4). The clinical features and investigations suggested CYP11β1-classical deficiency in four patients and CYP11β1-non-classical deficiency in one patient.
Conclusions : The five cases of CYP11β1-deficiency demonstrate a spectrum of clinical abnormalities, with diagnostic difficulties in two cases and delayed presentation in three cases. Prompt diagnosis of CYP11β1-deficiency is facilitated greatly by the availability of a gas chromatography-mass spectrometry instrument and is essential to avoid the long-term effects of hypertension and hyperandrogenism.     

Intravascular papillary endothelial hyperplasia of the mandibular lingual mucosa

In 1923, Masson1 described an unusual papillary endothelial proliferation that he named as "hemangioendotheliome vegetant intravasculaire". In 1976, Clearkin and Enzinger2 coined the term intravascular papillary endothelial hyperplasia (IPEH) that is now in use. This term refers to the presence of endothelium lined papillary projections within a vascular space. Characteristically, the papillary structures are composed of a single layer of swollen or plump endothelial cells without cellular pleomorphism, mitotic activity or necrosis.     

The Angiographic Findings in a Haemophilic Pseudotumour of Bone

The angiographic findings in a patient with haemophilic pseudotumour of the femur are presented. The examination showed the position of the displaced femoro-poplited vessels, enabling these structures to be avoided at operation. Under anti-haemophilic globulin cover no complications occurred. It is suggested that angiography may be safely employed provided the coagulation defect is controlled. The examination may be useful in differentiating other bone lesions and in defining more clearly the extent of a haemophilic pseudotumour.     

Chest wall lipoblastoma in a seven-month-old girl: a case report

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infanthood and early childhood. It is typically located in the extremities and the trunk and less frequently in the head-neck region, mediastinum, and retroperitoneum. However, cases of pleural, thoracic wall, and pulmonary lipoblastoma have also been reported in the literature. Lipoblastoma is a tumor with good prognosis despite its potential for local invasion and rapid growth. Our patient is a 7-month-old girl who was brought to our clinic for a rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the mass was removed by thoracic wall resection and the resultant defect covered with an absorbable polyglactine mesh. The excised mass was diagnosed pathologically as a benign lipoblastoma. In the postoperative period, the thorax wall was stable, and after 24 months of follow-up no recurrence was observed. In the literature, 4 types of thoracic wall lipoblastomas have been reported, but thoracic wall resection has not been applied in any benign lipoblastoma case. To the best of our knowledge, the only benign lipoblastoma case in which a thoracic wall resection was carried out, with its clinical and histopathologic specifications alongside that in the literature, is presented here for the first time.     

Ancestry reported by white adults with cutaneous melanoma and control subjects in central Alabama

Background  

We sought to evaluate the hypothesis that the high incidence of cutaneous melanoma in white persons in central Alabama is associated with a predominance of Irish and Scots descent.     

Histopathological effects of cyphenothrin on the gills of Lebistes reticulatus

We observed the histopathological effects of several doses of cyphenothrin. Adult guppies (Lebistes reticulatus) were placed in water containing 34.8, 46.5, 53.5 or 60 micrograms cyphenothrin/L for 96-h and their gills examined. The most common changes at all cyphenothrin doses were the lifting of the epithelial layer from gill lamellae and some necrosis. Degeneration of secondary lamellae due to edema, the shortening of secondary lamellae, and club-shaped lamellae were also observed. The 96-h LC50 for cyphenothrin to Lebistes reticulatus was 48.7 micrograms/L.     

Suspected poisoning in children. Study of the incidence of true poisoning and poisoning scare in a defined population in North East Bristol

The distinction between true and suspected poisoning in children has not been made clear in previous work on childhood poisoning. A study of suspected poisoning in children under 15 years of age in a defined population of North East Bristol from November 1970 to July 1973 carried out by the Health Education Council Medical Research Division included 53,000 child-years at risk. The number of suspected poisonings was 3-4/1000 population aged under 15 years per year, with a higher incidence in younger age groups. Detailed investigation of the circumstances of the accidents carried out by a multidisciplinary team showed that at least 65%, and possibly as many as 78% were poisoning scares and not true poisoning. The evidence used by the casualty doctor and by the parents to diagnose poisoning was explored, and in many cases was circumstantial. Children with fathers in nonmanual occupations were over-represented. This may reflect differences in patterns of utilization behaviour rather than true differences in incidence.