Neonatal cholestatic conditions requiring surgical reconstruction

Obstructive jaundice in infancy and childhood can arise from a variety of causes. Recognition of the importance of persistent jaundice beyond 2 weeks of age in the infant or elevation of the conjugated bilirubin more than 2 mg/dl will allow the timely evaluation of neonates with possible biliary atresia prior to the development of irreversible hepatic damage. Episodes of intermittent jaundice or persistent conjugated hyperbilirubinemia should be investigated at any age to exclude obstructive lesions of the extrahepatic biliary tract. In all cases, a rapid but carefully coordinated evaluation can be undertaken by a pediatric gastroenterology and surgical team, allowing optimum economy of effort and preservation of hepatic function. In nearly all cases with obstructive lesions of the extrahepatic biliary tree, satisfactory hepatic function can be preserved through appropriate surgical intervention. Results with biliary atresia continue to improve but are dependent on early diagnosis and treatment. In cases in which hepatic function deteriorates and hepatic failure becomes inevitable, orthotopic hepatic transplantation can be lifesaving and should be undertaken prior to the onset of irreversible hepatic decompensation. Although the terminology may be somewhat confusing and its etiology uncertain, the entity of choledochal cysts, taken within the context of its modern classification, accurately describes a group of cystic anomalies of the extrahepatic (and intrahepatic) biliary tree, which have many common features. The vast majority can be expeditiously dealt with by surgical excision and hepaticoenteral reconstruction. Two particular associations with choledochal cysts are important to note. When it occurs with infancy, choledochal cysts can be difficult to diagnose, but very important to define and treat expeditiously in order to prevent serious impairment of hepatic function. In all patients, but particularly in adults, the risk of associated carcinoma necessitates extremely careful definition of the biliary tree and urges one to advocate complete excision of the cyst at initial operation.     

Patellofemoral joint: kinematic MR imaging to assess tracking abnormalities

The patellofemoral joint was imaged with magnetic resonance (MR) in the axial plane while the knee was positioned from 0 degrees to 32 degrees of flexion (nine positions). These multiple sequential images obtained within the early phases of flexion of the knee were viewed in a "cine-loop" format, producing a kinematic study that clearly demonstrated the relationship of the patella to the trochlear groove. Four healthy subjects and one patient with known bilateral subluxing patellae were studied. The preliminary results suggest that kinematic MR imaging of the patellofemoral joint is potentially useful for the evaluation of patellar tracking abnormalities.     

Hyperbaric oxygen therapy in chronic stable multiple sclerosis: double-blind study

We carried out a randomized, double-blind, placebo-controlled trial of hyperbaric oxygen therapy (HBO) in patients with chronic stable MS. Eighty-two patients were treated in a multiplace hyperbaric chamber with gas supplied by mask. Forty-one patients received 20 consecutive daily treatments of 100% O2 followed by 7 "booster" treatments in the next 6 months; 41 control patients received "air" (12.5% O2 at 1.75 atmospheres absolute). There was no significant difference in treatment and control groups in the Extended Kurtzke Disability scores, Kurtzke Functional scores, magnetic resonance imaging, or evoked potentials after the initial 20 treatments or after the boosters. HBO is not effective in treating chronic stable MS.     

Marrow regeneration after mechanical depletion

The origin of marrow regeneration after mechanical depletion was reinvestigated in mouse chimeras. The results were compatible with the local origin of stem cells from remnants of incompletely removed marrow, but not with their origin from a common precursor of both bone and hemopoietic cell lines. In transplanted femurs depleted by a modified technique of in vivo evacuation of marrow, hemopoietic regeneration failed to occur. The presence of hemopoietic stem cells in the Haversian canals was thus excluded. The demonstration of ample hemopoiesis with minimal bone formation in nondepleted controls in which bone marrow initially became necrotic provided new evidence that osteogenesis was not a prerequisite of hemopoietic regeneration.     

Severe malabsorption in autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy syndrome successfully treated with immunosuppression

A 15 year old boy with autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy syndrome suffered recurrent episodes of severe intractable diarrhoea, steatorrhoea, and hypocalcaemia. The only treatment modality, which controlled the malabsorption syndrome, was immunosuppression with intravenous high dose methylprednisolone and oral methotrexate maintenance therapy.     

Toxic cardiogenic shock in a patient receiving weekly 24-h infusion of high-dose 5-fluorouracil and leucovorin

A 54-year-old man was treated with weekly 24-h infusion of high-dose 5-fluorouracil (2600 mg/m2) and leucovorin (100 mg/m2) for metastatic colon cancer. At first, he tolerated the treatment well and no significant toxicity was identified. After a total of eight courses of treatment, a stable disease was observed, but mild shortness of breath was found on occasion. The patient had no previous history of cardiac disease and the heart performance assessed by left ventricular ejection fraction before treatment was normal. Unfortunately, acute pulmonary edema with lethal cardiogenic shock occurred during the ninth course of treatment, in spite of intensive medical treatment. The chest X-ray showed extreme cardiomegaly. Repeated assessment of his heart function by echocardiogram and ventricular ejection fraction revealed a very poor cardiac performance. Toxic cardiogenic shock during weekly 24-h infusion of high-dose 5-fluorouracil and leucovorin is extremely rare. To the best of our knowledge, no case has been reported in the English literature. We report a case and the relevant literature about the incidence, clinical picture and possible pathophysiology on 5-fluorouracil-related cardioxicity is reviewed.