Acquired C1-esterase inhibitor deficiency: Three case reports and commentary on the syndrome

Acquired C1-esterase inhibitor deficiency is a rare syndrome which usually presents with episodes of angioedema. Most patients have an underlying lymphoproliferative disorder. It is important to gastroenterologists, haematologists, immunologists and dermatologists, as these are the more likely specialties to which the patient will present. Recognition of the syndrome is important not only for diagnostic purposes but for investigating and treating any associated underlying lymphoproliferative disorders. In some instances the angioedema may precede the lymphoma by many years. Optimal patient management requires that both angioedema and the underlying lymphoma be recognized and treated.     

A prospective study of symptomatic bacteremia following platelet transfusion and of its management

BACKGROUND: The danger of bacteremia due to contaminated platelets is not well known. There are also no established guidelines for the management of febrile reactions after platelet transfusion. STUDY DESIGN AND METHODS: To determine the risk of symptomatic bacteremia after platelet transfusion, 3584 platelet transfusions given to 161 patients after bone marrow transplantation were prospectively studied. Platelet bags were routinely refrigerated for 24 hours after transfusion. Septic work-up was initiated for a temperature rise of more than 2 degrees C above the pretransfusion value within 24 hours of platelet transfusion or a temperature rise of more than 1 degree C that was associated with chills and rigor. Diagnosis of bacteremia after platelet transfusion was made only when the pairs of isolates from the blood and the platelet bags were identical with respect to their biochemical profile, antibiotic sensitivity, serotyping, or ribotyping. RESULTS: Thirty-seven febrile reactions, as defined above, occurred. Bacteremia subsequent to platelet transfusion was diagnosed in 10 cases. There was a 27-percent chance (95% CI, 15–43%) that these febrile reactions represented bacteremia that resulted from platelet transfusion. For a subgroup of 19 patients with a temperature rise of more than 2 degrees C, the risk of bacteremia was 42 percent (95% CI, 23–64%). Septic shock occurred in 4 of the 10 bacteremic patients. A rapid diagnosis was possible because the involved bacteria were demonstrated by direct Gram stain of the samples taken from the platelet bags of all 10 patients. CONCLUSION: Significant febrile reactions after platelet transfusion are highly likely to be indicative of bacteremia. Routine retention of platelet bags for subsequent microbiologic study was useful in the investigation of these febrile reactions. Empiric antibiotic therapy is indicated.     

Iron deficiency in children with early chronic kidney disease

Iron deficiency (ID) contributes to the development of anemia in patients with chronic kidney disease (CKD). The frequency of ID in children with early CKD has not previously been reported. This was a retrospective chart review of children with CKD stages 2 and 3 followed at the CKD clinic of Children’s Hospital of Michigan. ID was defined as low ferritin and transferrin saturation <20%. Patients on iron supplements were considered as iron-deficient cases. There were 50 patients included in the study (72% male) with a mean age of 10.31 (±5.21). The mean glomerular filtration rate (GFR) was 55.4 ml/min/1.73 m² (±14.61). ID was present in 42% of patients, out of whom almost half (42.9%) presented with anemia. Females had a higher frequency of ID (64.3%). The frequency of ID with anemia increased from 4.3% to 29.6%, (p = 0.03) in stage 2 to stage 3 CKD, respectively. The frequency of ID without anemia also increased with progression of CKD from stage 2 to stage 3, however, the difference was not statistically significant. ID is frequent in patients with early CKD. Monitoring of iron tests and treatment of ID is important in this population of patients.     

Substance use and other psychiatric disorders in first-degree relatives of opioid-dependent males: a case-controlled study from India

To assess the prevalence of substance use and other psychiatric disorders in first-degree relatives of males with opioid dependence compared to normal controls. DESIGN: Observational, case-control study using family history method. SETTING: A drug addiction treatment centre in northern India. PARTICIPANTS: First-degree relatives of 100 male probands with opioid dependence and no comorbidity (n=493) and those of 50 matched probands from normal population (n=254). Measurement Family interview of probands and family members, using the Family Interview for Genetic Studies. The main outcome measure was relative risk (expressed as odds ratio after controlling for confounding variables using logistic regression) of familial aggregation of psychiatric and substance use disorders. FINDINGS: First-degree relatives of opioid-dependent males were more likely to have a psychiatric disorder than those of normal controls [adjusted odds ratio (OR) 4.47; 95% confidence interval (CI) 1.97-10.11; P<0.001], especially for opioid use disorders in the brothers (adjusted OR 6.55; 95% CI 1.44-29.88; P=0.015) and for alcohol use disorders in the fathers of the probands (adjusted OR 5.64; 95% CI 2.39-13.24; P<0.001). Other disorders (major depression, chronic psychosis and obsessive compulsive disorder) did not have significant aggregation in the first-degree relatives of opioid-dependent subjects. CONCLUSIONS: This study provides further evidence for the higher rates of alcohol and opioid dependence in first-degree relatives of opioid-dependent patients. The exact pattern of this familial aggregation may be influenced by the gender of the relatives and their relation to the proband.     

Effectiveness of a Multidisciplinary Clinic in Managing Children with Chronic Kidney Disease

Background and objectives: Long-term outcome of patients with chronic kidney disease (CKD) correlates with adequacy of predialysis care. This is best provided in a multidisciplinary clinic that integrates the services of a nephrologist with other staff. There is limited data about such clinics in children. The Children''s Hospital of Michigan established a Chronic Renal Insufficiency (CRI) clinic in 2002 to provide comprehensive care to children with CKD. These children receive care from a nephrologist, nurse clinician, transplant coordinator, dietician, social worker, and psychologist. The objective of the study was to compare outcome variables between patients from the CRI clinic and a general nephrology clinic.Design, setting, participants, & measurements: This was a retrospective chart review of 44 patients with CKD stages 2 to 4, who were managed in the general nephrology clinic (1996–2001, n = 20) or the CRI clinic (2002–2007, n = 24) for 1 yr before starting renal replacement therapy (RRT). Laboratory parameters, growth, and dialysis access type at time of RRT were compared between the two cohorts.Results: At RRT, patients from the CRI clinic had better hemoglobin, lower parathyroid hormone and calcium phosphorus product than patients followed in the general nephrology clinic. More patients from the general nephrology clinic had an unplanned initiation of dialysis compared with patients from the CRI clinic (50% versus 10.5%, P < 0.05).Conclusions: This indicates that children followed in a multidisciplinary clinic have better outcome variables and are more likely to achieve K/DOQI targets at initiation of dialysis. They are better prepared for dialysis with electively planned catheter insertion or functioning arteriovenous grafts/fistulae.The incidence of chronic kidney disease (CKD) in children is not well defined and is usually extrapolated from the data available from end-stage renal disease (ESRD) registries. According to the United States Renal Data Service Annual Data Report 2007, the prevalence of CKD in the United States is 13.8% to 15.8% of the adult population (1). Pediatric ESRD patients constitute less than 2% of the total ESRD population (1). In adults, the prevalence of CKD stages 1 to 4 has increased from 10.0% in 1988–1994 to 13.1% in 1999–2004 (2). A part of this increase in prevalence is attributed to a higher prevalence of diabetes, hypertension, and obesity. Because of its high morbidity and mortality, CKD is now recognized as a major public health problem. In a longitudinal study from a large managed care organization, Keith et al. noted that the mortality risk over a mean follow-up period of 3 yr for CKD stages 3 and 4 was 24.3% and 45.7%, respectively, compared with 10.2% in patients without CKD (3). Although mortality rates are significantly lower in pediatric patients with ESRD as compared with adults, the age-specific mortality rate is still 30 to 150 times higher than in children without kidney disease (4). Early evaluation of patients with CKD by nephrologists helps prevent or slow the progression of renal disease, allows timely treatment of renal and extrarenal complications, and facilitates preparation for renal replacement therapy or pre-emptive renal transplant in those approaching ESRD (5,6). In addition to expert medical care by a nephrologist, patients with progressive moderate to severe CKD need dietary, social, and psychological support to ensure optimum growth and development (7–10).The National Kidney Foundation Kidney Disease Outcomes Quality Initiative (NKF-K/DOQI) has developed comprehensive guidelines for care of CKD patients (11–13). However, despite these efforts, the morbidity associated with ESRD remains high. One of the reasons could be that most such patients, adults as well as children, are seen in general nephrology clinics that do not offer the comprehensive care required by patients with progressive moderate to severe CKD. The objective of this study was to examine the clinical outcomes in children with moderate to severe CKD who were followed in the multidisciplinary Chronic Renal Insufficiency (CRI) clinic, which we started at the Children''s Hospital of Michigan in 2002. These patients were compared with a cohort of our CKD patients followed in a general nephrology clinic before the establishment of the CRI clinic. We evaluated outcome variables in these two cohorts of patients, including laboratory parameters, growth, and dialysis modality and access type at initiation of chronic dialysis.     

Psychosocial morbidity in Cushing disease: a study from India

The main objective of this article is to study the psychosocial profile of patients of Cushing disease (CD) in a developing country setting. Eighteen patients with CD underwent a cross-sectional assessment regarding their socio-demographic and clinical profile, life events, social support, coping, dysfunction, quality of life, and psychiatric morbidity. Twenty-two demographically group-matched healthy participants (free from psychological morbidity) acted as the control group. The CD group had predominance of females (71.5%) with mean age at onset of 20.38 (range 8–38) years, and mean duration of illness of 65.33 (range 4–260) months. Six subjects (i.e., GHQ positive group) scored positive on the General Health Questionnaire-12 giving a psychological morbidity rate of 33.33%, with one having an ICD-10 diagnosis. There was no difference between GHQ positive and GHQ negative groups on number of life events, social support, quality of life and dysfunction. However, GHQ positive group used significantly more of internalizing coping strategies. Psychological morbidity occurs in a significant percentage of patients with CD. Presence of psychological morbidity is associated with internalizing coping strategies.     

Bacterial indicators of pollution of the Douala lagoon, Cameroon: Public health implications

Background

Indiscriminate disposal of untreated wastes which are often heavily laden with sewage microorganisms some of which are pathogenic to humans into aquatic environments near cities could serve as potential dangers to human health.

Objective

A prospective study was undertaken to investigate the scope of potential bacterial pathogens and to assess the extent of pollution of the Douala lagoon.

Methods

A total of eighty water samples were collected fortnightly from the lagoon at five stations from March to October 2005 and analysed for heterotrophic bacterial densities, coliform counts, faecal coliform and faecal streptococcal counts. Bacteria were isolated and identified using standard microbiology and biochemical techniques.

Results

High heterotrophic bacterial counts (33 × 10⁵ − 161 × 10⁵ CFU/ mL), total coliform counts (1.8 × 10² − 2.4 × 10² CFU/100 mL), faecal coliform counts (2.2 × 10² − 2.4 × 10² CFU/ 100 mL) and faecal streptococcal counts (2.1 × 102 − 2.3 x 10² CFU/100mL were observed in all sampling stations. Eleven species of bacteria: Bacteroides fragilis, Proteus vulgaris, Klebsiella pneumoniae, E. coli, Enterococcus faecalis, Enterobacter aerogenes, Citrobacter freundii, Aeromonas hydrophila, Pseudomonas aeruginosa, Bacillus mycoides and Serratia marcesens, were frequently isolated.

Conclusion

The presence of potential bacterial agents such as Bacteroides fragilis, Pseudomonas aeruginosa, Aeromonas hydrophila, Klebsiella pneumoniae and E. coli in the lagoon may pose a serious threat to the health and well being of users of the Lagoon and calls for urgent intervention.     

Posterior Superior Mesenteric Artery (SMA) First Approach vs. Standard Pancreaticoduodenectomy in Patients with Resectable Periampullary Cancers: a Prospective Comparison Focusing on Circumferential Resection Margins

Background

The ‘SMA-first’ (P-SMA) pancreatoduodenectomy (PD) allows dissection directly on the right lateral aspect of superior mesenteric artery (SMA) which may decrease circumferential resection margin (CRM) positivity. This comparative study between standard PD (sPD) and P-SMA approach was planned focusing on CRM involvement.

Methods

This was a prospective study comparing consecutive patients with resectable periampullary cancers (PACA) undergoing PD using the standard or P-SMA approach. The perioperative outcomes and the CRM positivity rates (specimens analysed according to the standardized Leeds pathology protocol (LEEPP)) were compared.

Results

Overall, 39 patients (28 men; mean age 54 years; sPD 21, P-SMA 18) were included. Both groups were comparable with regard to demographic/tumour characteristics and perioperative outcomes. The P-SMA technique was significantly faster (321.1 ± 54.0 vs. 357.6 ± 55.8 min; p = 0.05). Though the mean tumour size (2.2 vs. 2.1 cm; p = 0.84) and T stage (T2 and T3) distribution were similar in both groups, lymph node yield was significantly higher in the P-SMA group (10.7 vs. 5.95; p = 0.001; mean 8 (2–21)). Though CRM positivity (margin <1 mm) occurred in 8 (21.1%), we did not find the P-SMA PD to yield significantly lower CRM positivity rates compared to the sPD (3/17 (17.6%) vs. 5/21(23.8%); p = 0.71). At a median follow-up of 28 months, fewer patients in the P-SMA PD group developed recurrence (2/15 vs. 5/19; p = 0.3) or died (3/15 vs. 7/19; p = 0.19), though this difference was not significant.

Conclusions

In patients with resectable PACA, P-SMA PD was significantly faster and yielded higher lymph node counts in the specimen but did not lower the rate of CRM positivity as determined by the LEEPP.