A novel treatment for chronic pancreatitis

Background Hereditary pancreatitis is an important cause of chronic pancreatitis, which may result in endocrine and exocrine failure. This may necessitate simultaneous pancreas and kidney transplant (SPK). Bladder drainage of the exocrine secretions may cause problems. Aim To report one such case and its surgical correction. Methods A 20-year-old male with insulin-dependent diabetes mellitus secondary to idiopathic chronic pancreatitis had a SPK with bladder drainage. Urological and metabolic complications secondary to the drainage of pancreatic secretions, rich in proteolytic enzymes required convertion from bladder to enteric drainage. Results He was able to discontinue his pancreatic enzyme supplements, ceased to have steatorrhoea and gained weight. He was referred to the €pean Registry of Hereditary Pancreatitis and Familial Pancreatic Cancer (€PAC), hereditary pancreatitis was confirmed by genetic analysis. Conclusion Enteric-drained pancreas transplantation is a successful treatment for exocrine as well as endocrine pancreatic failure and should be considered as a treatment option in patients with chronic pancreatitis.     

Multiple-organ pathology in septic shock in patients with hemoblastoses

A prospective study of 20 patients with hemoblastosis and septic shock (SS) was carried out by invasive monitoring of the central hemodynamics and oxygen transport, evaluation of biochemical and coagulological parameters, and assessment of the severity of clinical condition by the APACHE II and SOFA scores. Septic shock was effectively treated in 12 patients, 5 of them were discharged from the department (group 1) and 7 died in intensive care wards from various complications (group 2). Eight patients died during the first 2 days from SS resistant to therapy (group 3). Group 2 patients were in need of a longer inotropic support than group 1 patients (5.8 +/- 1.6 vs. 2.7 +/- 0.8 days, p < 0.01). The deficit of bases was more expressed in groups 2 and 3 in comparison with group 1 (-11.3 +/- 3 and -2.7 +/- 9.1 mmol/liter vs. 1.4 +/- 4.4 mmol/liter) and left ventricular stroke index (LVSI) and oxygen delivery were lower. LVSI and base deficit were in linear correlation (rho = 0.4, p < 0.05). XIIa-dependent fibrinolysis was suppressed in all patients, which was more pronounced in group 3 in comparison with groups 1 and 2 (135 +/- 47.4 vs. 103 +/- 27 and 88.3 +/- 42.3). According to SOFA score, the severity of cardiovascular disorders during day 1 of SS was the same in all groups, while starting from day 2 it decreased in patients who survived. Acute respiratory failure was lower in group 1 only on day 1 according to SOFA. More pronounced (according to SOFA) hepatorenal failure was observed in group 2 in comparison with other patients. Organ involvement in hemoblastosis was detected at autopsy in 8 out of 13 cases. Hence, the need in prolonged cardiovascular support of SS patients is associated with development of polyorgan involvement. Fibrinolysis suppression is a frequent early manifestation of hemostasis disorders. Specific neoplastic organ involvement was observed in 61.5% patients with hemoblastosis who died from SS and its complications.     

Diminished concentrations of insulin-like growth factor I in cystic fibrosis

Cystic fibrosis is frequently accompanied by a catabolic condition with low body mass index caused by a number of disease complications. Insulin-like growth factor-I (IGF-I) is an anabolic hormone and an important marker of nutritional status, liver function, and linear growth. Available data on IGF-I in cystic fibrosis are sparse and conflicting. From 1990-3, 235 of our 240 patients (114 males, 121 females, median age 16.2 years, ranged 0.1-44.0 years) had IGF-I measured once by radioimmunoassay. IGF-I was significantly reduced compared with a healthy Scandinavian control population: mean (-2 SD to +2 SD) IGF-I SD score was -0.97 (-3.7 to 1.7) in males and -0.67 (-3.2 to 1.9) in females. Height SD score was -0.95 (-3.3 to 1.4) in males and -0.81 (-3.2 to 1.6) in females. In patients who were still in the growth period a significant correlation of IGF-I SD score to height SD score (r = 0.28, p < 0.001) was found. The low IGF-I concentrations may reflect the catabolic state of many patients with cystic fibrosis and play a part in their abnormal growth pattern.     

Physiological anticoagulants and activated protein C resistance in childhood stroke

Immunological and functional protein S, protein C and antithrombin III levels and anticoagulant responses to activated protein C were measured in 24 patients with stroke in childhood. No hereditary deficiencies were found. The protein S levels in healthy controls of younger age did not differ from the adult levels. For optimal screening of protein S deficiency, measurements using functional as well as immunological assays are recommended. Appropriate criteria for the diagnosis of the deficiencies must be carefully applied if unnecessary anxiety and inappropriate treatment of children are to be avoided.     

Evaluation of attempted prevention of unexpected infant death in very high-risk infants by planned health care

Three hundred and ninety-six babies born in Sheffield between 1982 and 1990 identified as being at "very high risk" of unexpected infant death by means of a scoring system, received an intensive programme of health care including a case discussion between a paediatrician, the GP and the health visitor held in the family doctor's surgery, weekly visits from the health visitor and informal hospital admission. Significantly fewer sudden unexpected infant deaths occurred in this group than were expected by logistic regression anlysis or occurred in the best available control group with comparable scores ( p = 0.024). Problems in evaluation include identification of an adequate control population, ethical difficulties in introducing a controlled study when the programme is already perceived as effective, and the calculation of "expected death rates". The results of this study indicate that very energetic programmes of intervention may prevent some deaths in vulnerable infants.     

Fibromatosis of bone in children

Radiographs, computed tomograms, and radionuclide bone scans were obtained preoperatively in three children with fibromatosis involving the bones and soft tissues of the extremities. Two of the children had identical scar-like bone lesions of the proximal tibia, which, to the authors' knowledge, have not been reported before in this disease. The lesions recurred in two children.