Pemphigoid and pemphigus foliaceus successfully treated with topical corticosteroids

Six patients with pemphigoid and three patients with pemphigus foliaceus were successfully treated with topical corticosteroids. This was especially effective in cases of pretibial localized pemphigoid and in mild cases of bullous pemphigoid and pemphigus foliaceus with negative or low-titer circulating autoantibodies.     

Atherosclerosis, aortic stenosis and sudden onset central diabetes insipidus

This case report presents a thirteen year-old boy who was diagnosed as having Hypomelanosis of Ito. The developmental history includes severe failure to thrive, and moderate atypical autism as well as diverse clinical and neuropsychological symptoms are present. The pattern of neuropsychological functioning, which can be partially related to the neurophysiological findings, is discussed within the context of existing neuropsychological theories about autistic disorders.     

Acute myelomonocytic leukemia preceded by secondary amenorrhea and presenting with central diabetes insipidus: a case report

The antigenic components of Fasciola gigantica somatic extract were revealed by sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) and immunoblotting technique using sera from patients with F. gigantica infection, patients with clinically diagnosed fascioliasis, patients with other infections/illness and healthy adults. By SDS-PAGE, it was found that the somatic product comprised more than 22 polypeptides. Immunoblotting analysis revealed at least 13 components which were strongly recognized by sera of patients with fascioliasis. These antigenic components had molecular weights ranging from less than 14.4 to more than 94 kDa. One antigenic component, i.e. 38 kDa was found to give a consistent reaction with sera of patients with fascioliasis (100% sensitivity and 96.7% specificity). The finding suggests that the 38 kDa components may be a potential diagnostic antigen for fascioliasis.     

Persistent high MR signal of the posterior pituitary gland in central diabetes insipidus

We describe three cases of central diabetes insipidus, each with a different pathogenesis, in which unexpected hyperintensity of the posterior pituitary gland was seen on T1-weighted MR images obtained at the time of presentation. In the first case (idiopathic), the posterior pituitary signal persisted more than 10 years; in the second case (Langerhans cell histiocytosis), the signal disappeared within 3 months, despite early specific chemotherapy with etoposide; and in the third case (transient), the posterior signal disappeared within 1 year, but it was documented at the time of spontaneous reversal of polyuria and polydipsia.     

Acute myeloblastic leukemia associated with hyperleukocytosis and diabetes insipidus

Two cases with acute myeloblastic leukemia (AML M4-FAB) associated with diabetes insipidus (DI) are presented here. Both patients presented with hyperleucocytosis. One had a white blood cell count (WBC) of 150 x 10(9)/L and the second patient had 200 x 10(9)/L. One of these patients was a 40 year-old male and MRI of the hypophysis showed an infindibuler mass. This patient did not respond to remission induction chemotherapy and reinduction chemotherapy was given. The other patient was a 16-year-old male with a normal CT scan of the head. Both patients had DI with typical clinical and laboratory findings. The first patient died early on during reinduction chemotherapy and the second patient died of intracranial bleeding before induction chemotherapy was given. These findings are consistent with the data in the literature suggesting that the prognosis of AML associated with DI is poor and that these cases generally present with hyperleucocytosis.     

Metronidazole resistant trichomoniasis successfully treated with paromomycin

Thoracic infection by anaerobes are uncommon diseases and often presents difficulty in diagnosis. We report a case of thoracic infection due to Fusobacterium Nucleatum. A 60-year old man presented a lesion infiltrating from the lung to the thoracic wall. Fusobacterium Nucleatum was isolated on pus collected. Treatment by penicillin, metronidazole combined with surgical drainage was highly effective.     

Chronic eosinophilic pneumonia successfully treated with suplatast tosilate

STUDY OBJECTIVE: To review the epidemiology of hepatitis C virus (HCV) infection among injecting drug users (IDUs) in Australia, and consider needs for further research and prevention policies and programmes. DESIGN: (1) Review of the results of surveillance for HCV; (2) review of published literature on prevalence, incidence, and risk factors for HCV among IDUs; and (3) reconstruction of incidence rates from prevalence studies of HCV in IDUs. SETTING AND PARTICIPANTS: Field and clinic based studies of IDUs in Australia. MAIN RESULTS: HCV has been present at high prevalences (of the order of 60-70%) in populations of Australian IDUs since at least 1971. Duration of injecting and main drug injected were the main predictors of seropositivity, the latter possibly a surrogate for frequency of injecting and both together as surrogate for cumulative numbers of times injected. Risk of infection begins with first injection and continues as long as injecting does. Current incidence is approximately 15 per 100 person years, and up to 40 per 100 person years in some subpopulations. Incidence may have decreased through the 1980s as a result of behaviour change in relation to HIV, as it has for hepatitis B, but not significantly so. CONCLUSIONS: Control of HCV infection in Australia will depend on effectiveness of measures to control HCV spread among IDUs. This will be a greater challenge than the control of HIV in this population has been. Needs identified include improved surveillance, especially for recently acquired infection, better understanding of exact transmission modes, and urgent improvement in prevention strategies.     

Staphylococcal kidney abscesses in rats treated with corticosteroids

A heavy growth of Staphylococcus aureus was isolated from kidney abscesses in rats from 2 inbred strains treated with corticosteroids. The S. aureus phage types which caused the abscesses are endemic in the rat breeding colonies. One strain of rat was more sensitive to the action of the corticosteroids.     

Primary intrasellar mixed germ-cell tumor with precocious puberty and diabetes insipidus

A case-control study using data from the Baltimore-Washington Infant Study (BWIS) examined possible paternal risk factors in the etiology of isolated membranous ventricular septal defects (VSD). There were 641 total VSD case infants and 3,549 randomly selected control infants ascertained between 1981 and 1989. Isolated membranous VSDs were identified in 499 cases. Socio-demographic factors (such as parental age and race), social habits, and medical conditions were analyzed by multiple logistic regression in order to estimate adjusted odds ratios (OR) and 95% confidence intervals (CI). Paternal age was not found to be a risk factor per se, but small positive associations were found for some social habits and maternal factors. Significant associations were found for paternal marijuana use (OR 1.36, 95% CI 1.05-1.76), African-American race of the infant (OR 1.34, 95% CI 1.09-1.65), and for cocaine use among older fathers (OR 3.92, 95% CI 1.30-11.86). These associations support a multifactorial etiologic hypothesis for isolated membranous VSDs and point to some interesting parental behavioral and medical considerations which may contribute to risk for this common birth defect.     

Torsade de pointes with sotalol overdose treated successfully with lidocaine

In a newborn girl with a history of connatal liver damage, histological examination of a liver biopsy sample taken during the seventh week of life revealed incipient destruction of bile ducts. Very high titres of antimitochondrial antibodies were later detected in the plasma. As the hepatic injury tended towards fibrosis, the histological diagnosis became primary biliary cirrhosis. Autoantibodies against E1 alpha, E2, and E3 subunits and protein X component of pyruvate dehydrogenase complex, and against citrate synthase were detected on western immunoblotting in a 1 in 1000 dilution of the patient's serum. The patient died of her illness at 11 years of age. In liver specimens obtained at autopsy human immunoglobulin deposition was detected on the surface of almost all hepatic cells by immunohistology. As there is a physical and functional interaction between pyruvate dehydrogenase and citrate synthase within the mitochondria, the presence of autoantibodies against certain proteins in the patient suggests that in this form of the disease the molecular recognition and then the autoimmunisation process could be directed against a mitochondrial enzyme cluster containing both pyruvate dehydrogenase and citrate synthase.     

Endocrinological aspects of Langerhans cell histiocytosis complicated with diabetes insipidus

Langerhans cell histiocytosis (LCH) is a rare disorder and may be complicated with hypopituitarism and diabetes insipidus (DI) due to invasion of the hypothalamic-pituitary area. In this study, 10 patients with complete (4) and partial (6) type central DI were found among 125 LCH patients in our hospital records. The water deprivation test, followed by the pitressin test, was performed to confirm DI. Hypothalamic-pituitary endocrine function tests were carried out on these 10 patients at the initial diagnosis and during follow-up. All patients revealed growth hormone insufficiency in the insulin hypoglycemic tolerance test. Four patients had impairment of cortisol secretion, demonstrated by insulin hypoglycemic stimulating test results. Two patients had poor response in the thyrotropin releasing hormone stimulating test. Two patients had only partial responses in the luteinizing hormone releasing hormone test. Four patients had hyperprolactinemia. All patients underwent surgical treatment followed by chemotherapy and/or radiotherapy. One patient completely recovered from the endocrine disorder, 3 patients required smaller doses of desmopressin, and one patient had normal adrenal, thyroid, and gonadal function. Hypothalamic-pituitary disorders in LCH should not be neglected. Treatment of LCH can partially or completely reverse associated endocrine disorders. Therefore, endocrine studies and hormone replacement should be mandatory for patients with LCH.     

Molecular biological studies on patients with nephrogenic diabetes insipidus]

Nephrogenic diabetes insipidus is a rare, mostly X-linked recessive disorder characterised by renal tubular resistance to the antidiuretic effect of arginine vasopressin. The gene responsible for the X-linked nephrogenic diabetes insipidus, the G-protein-coupled vasopressin V2-receptor, has been localised on the Xq28 region. In this study four patients were investigated with molecular genetic methods. Diagnosis was based on clinical symptoms and lack of increase of urinary osmolality after administration of the arginine vasopressin, or the synthetic vasopressin analogue DDAVP. Three different mutations (C112R, N317K, W323S) were found in three patients, while no mutation was detected in the fourth patient. Since earlier histiocytosis X has been diagnosed in this patient, this patient has probably central diabetes insipidus. Although the main symptoms of the disease can be found in all patients, there are significant differences in the seriousness of the symptoms as well as in some other symptoms. The explanations might be the different mutations in the V2-receptor gene and the various other genetic and environmental factors; these findings provide further evidence that X-linked nephrogen diabetes insipidus results from defects in the V2-receptor gene.