Liver transplantation in tyrosinaemia type 1: the dilemma of timing the operation

Four children with tyrosinaemia type 1 received liver transplants. The metabolic disorder was corrected and all four had normal liver function on an unrestricted diet. Two children, transplanted at age five and seven years, proved to have occult hepatocellular carcinoma and both subsequently developed pulmonary metastases. One child was well 32 months after removal of a single pulmonary metastasis but the other child died with multiple metastases. The two younger children, transplanted at age 19 and 21 months, were well 28 and 44 months after operation, one after a second liver transplant. Our experience confirms the high risk of hepatocellular carcinoma in this disease and the potential value of early liver transplantation.     

Neurological crisis in hereditary tyrosinaemia and complete reversal after liver transplantation

A 19 month old Indian girl with tyrosinaemia developed a severe generalised neuropathy involving both phrenic nerves. Treatment with haemarginate failed to improve her condition. After liver transplantation the raised concentrations of the neurotoxin delta amino-laevulinic acid returned to normal and gradual but complete neurological recovery occurred over a period of 13 months.     

Homeopathy: A State of the Science Review With Recommendations for Practical Therapies in Midwifery Practice

A substantial proportion of adults and children in the United States use complementary and alternative health practices, including homeopathy. Many homeopathic therapies are readily available over the counter, and many individuals access and self-administer these therapies with little or no guidance from health care practitioners. In addition, patients and health care providers are often confused by terminologies associated with complementary practices and may be unable to distinguish homeopathy from naturopathy, herbalism, holistic medicine, Ayurveda, traditional Chinese medicine, or other forms of health care. Compared with European and Asian countries, education in the United States about complementary and alternative health practices is not typically found within traditional nursing, midwifery, or medical education curricula. Given this lack of education and the broad acceptance and popularity of homeopathy, it is necessary for health care practitioners to improve their knowledge regarding similarities and differences among therapies so they can fully inform and make appropriate recommendations to patients. The intent of this article is therefore to examine the state of existing science of homeopathy, distinguish it from other complementary methods, and provide midwives and women's health care providers with an introduction to common homeopathic therapies that may be recommended and safely used by persons seeking midwifery care. This review also presents the evidence base, pharmacology, manufacturing, and regulation of homeopathic therapies. We also consider controversies and misunderstandings regarding safety and efficacy of homeopathic remedies relevant to women and birthing persons. Examples of practical applications of homeopathic therapies for use in midwifery practice are introduced. Implications for practice and sample guidelines are included.     

Acute unilateral pulmonary nonventilation due to mucous plugs

Acute bronchial obstruction by mucous plugs may be manifested clinically by dyspnea, hypoxemia, and respiratory alkalosis mimicking pulmonary thromboembolism. In eight cases with complete interruption of ventilation to an entire lung, chest radiography failed to reveal the extent of the obstruction. Perfusion was substantially less affected than ventilation. Routine ventilation-perfusion scintigraphy with technetium-99m DTPA aerosol aids in the diagnosis of acute major bronchial obstruction in patients with suspected pulmonary thromboembolism.     

The effect of methimazole on thioamide bioactivation and toxicity.

The hepatotoxicity induced by administration of ethionamide and thionicotinamide (TNA) was shown to be decreased by pre-administration of methimazole (MMI). Pre-administration of MMI was also shown to decrease the levels of excretion of TNA S-oxide. This indicates that thioamide S-oxidation, mediated by the flavin-containing mono-oxygenase, may be linked to the initiation of hepatotoxicity induced by these thioamides. SK&F-525-A, the cytochrome P-450 inhibitor, did not affect either thioamide-induced toxicity or levels of excretion of TNA S-oxide; however, the role of the P-450 isoenzymes cannot be totally ruled out.     

Severe malaria in children in Papua New Guinea

The clinical features of severe falciparum malaria and risk factors for mortality were studied in 489 children admitted with malaria to Madang Hospital, Papua New Guinea. The most common severe manifestations of malaria were severe anaemia (22%) and coma (16%). Children with severe anaemia were younger than those with coma (median age 2.2 vs. 3.7 years) and had been ill for longer before admission (median 7 vs. 4 days, respectively). Although the clinical features of coma in Madang children with malaria resembled closely those reported in African children, mortality was lower (8% vs. 17-25%, respectively). Overall, 17 (3.5%) children died, most within 12 h of admission. A high level of plasma lactate (> or = 5 mmol/l) was common (20%) and was the major predictor of death in multiple regression analysis. Raised plasma creatinine and decreased plasma bicarbonate were also independent predictors of mortality. Coma was not predictive of death, although a high proportion of children with profound coma died. Investigation of the causes of acidosis in children with malaria is a high research priority. In view of the short time interval between admission and death in many children, emphasis must be placed on the prevention or early recognition and treatment of acidosis in the district health clinic as well as the central hospital.      

Concordance of endogenous cortisol and phospholipase A2 levels in gram-negative septic shock: a prospective study

Lipocortins, a group of corticosteroid-induced phospholipase-inhibitory proteins, are thought to play a prominent role in the mediation of the anti-inflammatory effects of steroids. The synthesis and release of these proteins may represent a major endogenous mechanism of regulation of extracellular phospholipase A2 (PLA2) activity. Because soluble PLA2 activity has been associated with circulatory collapse in hyperphospholipasemic conditions, such as septic shock and pancreatitis, we examined the relationship between circulating PLA2 activity and adrenocortical function. In a prospective study of 10 episodes of septic shock, serum PLA2 and cortisol levels correlated significantly in all survivors (p less than 0.0001), whereas such a correlation was absent in all nonsurvivors (p less than 0.07). No significant correlation of cortisol and adrenocorticotropic hormone (ACTH), or PLA2 and ACTH, was found in any patient, suggesting that the stimulus for cortisol release arises from outside the hypothalamic-pituitary axis. These data suggest that, in human beings, the regulation of soluble PLA2 activity may be mediated by adrenocortical hormones, perhaps through the intermediary action of lipocortins.