Interventional stent implantation in a child with patent ductus venosus and pulmonary hypertension

 We report on the rare case of a 4-year-old boy with patent ductus venosus and pulmonary hypertension presenting with progressive fatigue, tachypnoea at rest and tachycardia. Cardiac catheterisation revealed suprasystemic pressure in the pulmonary arteries with severely elevated pulmonary vascular resistance. In order to reduce the diameter of the ductus venosus, a stent was implanted interventionally, which closed, as expected, spontaneously 2 years later. Pulmonary arterial pressure and pulmonary vascular resistance decreased significantly and the general condition of the boy improved dramatically. Conclusion To the best of our knowledge, this represents the first report of successful interventional stent occlusion of a patent ductus venosus associated with severe pulmonary hypertension. The future will tell whether this intervention is curative or represents a bridging procedure for subsequent liver transplantation. Received: 21 September 2000 / Accepted: 14 March 2001     

Glykogenose 1non-a

Diagnosis. Glycogen storage disease type 1non-a results from a mutation in a putative glucose-6-phosphate translocase gene that has been characterized recently. Whereas the diagnosis required a liver biopsy earlier, the diagnosis can now primarily be attempted by molecular studies. If the molecular studies show negative results in cases clinically suggesting the diagnosis, enzymatic studies from biopsy specimen are still necessary. Clinical manifestation. Clinical manifestations of the disease are hypoglycemia, lactic acidemia, hyperlipidemia, hyperuricemia, neutropenia and impaired neutrophil function, resulting in recurrent bacterial infections and oral and intestinal mucosa ulceration. Therapy. The metabolic component of glycogen storage disease type 1non-a is successfully treated by continuous application of carbohydrates. The immunologic component may be treated with G-CSF that causes a rise of the neutrophils thus preventing recurrent infections. Glycogen storage disease type 1a, an important differential diagnosis, however, lacks the neutropenia.     

Gender differences in the association between pre-adolescent smoking initiation and emotional or behavioural problems

Background  

Emotional and behavioural problems are a risk factor for the initiation of smoking. In this study, we aimed to assess this relationship beyond clinical cut-off values of problem behaviour.     

Hydrosyringomyelia and its management in childhood

Syringomyelia, once regarded as a degenerative disease of adults, is now recognized to be a disorder usually associated with the Chiari malformation and occurring in patients of all ages. We have reviewed 47 patients with syringomyelia treated on the Neurosurgical Service at the Hospital for Sick Children during the years 1977 to 1985. Twelve of these patients had a Chiari I malformation, 30 had a Chiari II malformation, and 5 had an acquired Chiari malformation. Thirty-one of these patients were treated by decompression of the Chiari malformation and plugging of the obex, 5 were treated by a simple posterior fossa decompression, 9 were treated by shunting of the syringomyelic cavity, and 2 were treated by a combined decompression of the posterior fossa and shunting of the syrinx. The Gardner procedure (decompression of the Chiari malformation and plugging of the obex) was the procedure most commonly used in managing our group of patients and resulted in improvement in over 70% of patients.     

Simultaneous occurrence of multiple primary carcinomas within the abdominal cavity

A case is reported of the occurrence in the same patient of two simultaneously growing primary carcinomas, the one a papillary cyst adenocarcinoma of the ovary, the other an adenocarcinoma of the sigmoid colon, grade 3, with metastasis, together with a leiomyoma uteri.     

Ambulance visits for severe hypoglycaemia in insulin-treated diabetes.

AIM: To determine, in insulin-treated diabetes the incidence and risk factors for severe hypoglycaemia requiring ambulance visits. METHODS: A cross-sectional, questionnaire survey was made of patients with type 1 diabetes, who received help for severe hypoglycaemia from Ambulance Association personnel, during the period 1/6/95 to 31/5/96. RESULTS: The ambulance service made 386 emergency visits to 247 persons with type 1 diabetes. Of these, 128 respondents (52%) completed a questionnaire detailing personal and diabetes history, usual diabetes care practices and hypoglycaemia management. Two or more visits for severe hypoglycaemia were made to 26.3% of patients, who reported a longer duration of diabetes than those who required only one visit (28 vs 20 years, p<0.03). Self-blood-glucose monitoring was performed by 98.4% of respondents and 66.4% self-adjusted insulin doses. Intensively treated patients (> or = 3 insulin injections daily) reported less awareness of hypoglycaemia than standard therapy patients (< or = 2 insulin injections daily) (p<0.05). Fifty-four per cent of respondents had glucagon available for emergency use, but those who lived alone and in general practitioner care only (27%) were less likely to have glucagon (p<0.05) compared to those with companions and in shared-care arrangements (62%). Hypoglycaemia management was influenced by the availability of glucagon. Oral glucose was used by 82% before injecting glucagon, whereas 40% of patients without glucagon called for the ambulance when severe symptoms were present even before initiating treatment with oral glucose. CONCLUSION: This survey determined the minimum frequency of severe hypoglycaemia requiring the ambulance at 1.6 episodes patient(-1) year(-1). Precipitating factors and a lack of coping skills and behaviours that might prevent severe hypoglycaemia and ambulance calls were identified.     

Spontaneous clostridial myonecrosis. A collective review and report of a case

A collected series from the English literature up to 1984 of spontaneous clostridial myonecrosis (SCM) is presented in order to reveal possible common denominators. SCM was associated with malignancy (colonic cancer and leukaemia), diabetes, and injections in descending order. C. perfringens was isolated in more than 70% of the cases followed by C. septicum in 27 and C. novyi in 7%. The pathogenesis is still speculative but is thought to be due to bacteraemia especially from the gastrointestinal tract, due to growth of dormant spores in tissue following antecedent trauma or as an infection descending along the ilio-psoas sheath from a gastrointestinal focus. The symptoms are pain in an oedematous, discoloured, crepitant area with haemorhagic bullae and often shock. The diagnosis is based on the clinical findings and Gram-positive rods in the exudate. Treatment instituted promptly constitutes of surgical debridement, antibiotics and hyperbaric oxygen. The prognosis of SCM is worse than for other cases of clostridial myonecrosis and survival was in this collected series only 19%.     

Parents' concerns about children are highly prevalent but often not confirmed by child doctors and nurses

Background  

The aim of this study was to assess the prevalence in the general population of parents' concerns about the development of their child, to identify groups at risk and to assess the association between parents' concerns and professional judgement.