Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium

Background

Idiopathic nephrotic syndrome (NS) in children is classified as steroid sensitive or steroid resistant. Steroid sensitivity typically portends a low risk of permanent renal failure. However, some initially steroid-sensitive patients later develop steroid resistance. These patients with late steroid resistance (LSR) are often treated with immunosuppressant medications, but the effect of these additional drugs on the long-term prognosis of LSR is still unknown.

Methods

A retrospective chart review was performed on patients diagnosed with idiopathic NS and subsequent LSR during the 8-year study period from 2002 up to and including 2009, with a minimum of 2 years of follow-up. Primary outcome measures were proteinuria and renal function.

Results

A total of 29 patients were classified as having LSRNS. The majority of patients received treatment with calcineurin inhibitors and/or mycophenolate mofetil. Seven patients received three or more non-steroid immunosuppressant medications. Sustained complete or partial remission was achieved in 69 % of patients. Three developed end-stage renal disease, and all others maintained normal renal function. There were 13 episodes of serious adverse events, none of which were fatal or irreversible.

Conclusion

Most patients with LSRNS responded to immunosuppressive therapy by reduction or resolution of proteinuria and preservation of renal function. The results suggest that immunosuppressive treatment is a viable option in NS patients who develop LSR.     

A SIX-CENTRE INTERNATIONAL STUDY OF THE OUTCOME OF TREATMENT IN PATIENTS WITH CLEFTS OF THE LIP AND PALATE: THE RESULTS OF A CROSS-LINGUISTIC INVESTIGATION OF CLEFT PALATE SPEECH

During the period 1964–73, 286 patients were treated for mandibular fractures. 229 patients (80%) attended the follow-up examination. The length of observation was from 1 to 9 years. The treatment and the follow-up evaluations in the 229 patients are described. Early fracture treatment has been the objective, also in patients who have undergone severe cerebral traumas. Antibiotic prophylaxis was implemented in cases of compound fractures. Preservation of teeth and tooth buds in the line of fracture was attempted. Clinical infection occurred in 0.4% of the patients. At the follow-up examination 1 patient presented with a fracture displacement outside the condylar process. Neither malocclusion nor pseudarthrosis following fracture or after treatment were seen. Permanent sensory disturbances in the innervation area of the mental nerve following fracture occurred in 8%. Radiological examination of the teeth in the line of fracture revealed unnoticed apical bone lesions in 17% of 118 patients. In a further 23% there was a negative response to the vitality test. Follow-up control of mandibular fractures is advised at 12 to 18 months after treatment.     

Avoiding Medical Complications During the Refeeding of Patients With Anorexia Nervosa

Nutritional rehabilitation and weight restoration are key underpinnings of the treatment protocol for patients with anorexia nervosa. While their inherent state of malnutrition and weight loss is certainly not a healthy one, ironically, the very essence of the refeeding process, if done injudiciously, can also be unsafe for patients with anorexia nervosa. In this article we will provide a review of the major complications that may arise during refeeding, how best to avoid them, and how to treat them.     

Abdominal transplantation for unresectable tumors in children: the zooming out principle

Purpose

To present our experience in abdominal transplantations to manage unresectable abdominal neoplasms in children and to describe the role of extensive surgeries in such cases.

Methods

This is a retrospective study of 22 abdominal transplantations in 21 patients for abdominal tumors over 16 years. Transplantation techniques included liver transplant (LT), multivisceral transplant (MVTx), and intestinal autotransplant (IA). Follow-up intervals ranged from 0.3 to 168 months (median 20 months).

Results

LT alone was performed in 15 patients for primary malignant (11) and benign (4) liver tumors. Pathological classification included HB hepatoblastoma (6), HCC hepatocellular cancer (3), hepatic epithelioid hemangioendothelioma HEH (1), angiosarcoma (1), benign vascular tumors (3), and adenoma (1). IA was performed in four patients for lesions involving the root of the mesentery; tumors of the head of pancreas (3) and mesenteric hemangioma (1). MVTx was performed in 2 patients for malignancies; pancreaticoblastoma (1), recurrent hepatoblastoma (1), and in one patient as a rescue procedure after IA failure. Four of the eleven patients who underwent LT for malignant liver tumor had metastatic disease at presentation. Six of them died of recurrent neoplasm (3), transplant-related complications (2), and underlying disease (1). All LT patients who had benign tumors are alive with functioning grafts. All IA patients survived and are on an oral diet, with one patient requiring TPN supplementation. One of the three patients who underwent MVTx died of metastatic disease.

Conclusions

Allo/auto transplantation for abdominal tumors is a valuable modality when conventional treatments fail or are not feasible.

     

Nicotine improves probabilistic reward learning in wildtype but not alpha7 nAChR null mutants,yet alpha7 nAChR agonists do not improve probabilistic learning

Cognitive impairments, e.g., reward learning, are present in various psychiatric disorders and warrant treatment. Improving reward-related learning could synergistically enhance psychosocial treatments and cognition generally. A critical first step is to understand the mechanisms underlying reward learning. The dopamine system has been implicated in such learning, but less known is how indirect activation of this system may affect reward learning. We determined the role of alpha7 nicotinic acetylcholine receptors (nAChR) on a probabilistic reversal learning task (PRLT) in mice that includes reward and punishment. Male alpha7 knockout (KO), heterozygous (HT), and wildtype (WT) littermate mice (n?=?84) were treated with vehicle, 0.03, or 0.3?mg/kg nicotine. Two cohorts of C57BL/6NJ male mice were treated with various alpha7 nAChR ligands, including the full agonists PNU282877 and AR-R-17779, the positive allosteric modulator CCMI, the partial agonist SSR180711, and the antagonist methyllycaconitine. All mice were then tested in the PRLT. Nicotine (0.3?mg/kg) significantly improved initial reward learning in alpha7 WT and HT mice but did not improve learning in KO mice, suggesting an involvement of the alpha7 nAChR in the pro-learning effects of nicotine. Neither alpha7 nAChR treatments (PNU282987, AR-R-17779, CCMI, SSR180711, nor methyllycaconitine) affected mouse PRLT performance however. Nicotine improved reward learning via a mechanism that may include alpha7 nAChRs. This improvement unlikely relied solely on alpha7 nAChRs however, since no alpha7 nAChR ligand improved reward learning in normal mice. Future assessments of the effects of other nAChR subtypes on reward learning are needed.     

Model minority stereotype and the diagnosis of alcohol use disorders: Implications for practitioners working with Asian Americans

This study investigated the priming effects of the model minority stereotype on 122 clinicians in training regarding their diagnostic accuracy on Asian Americans compared to Whites. It was hypothesized that clinicians in training would be less likely to diagnose Asian Americans with alcohol use disorder and would perceive them to have fewer clinical symptoms than Whites due to the model minority stereotype. Consistent with the hypotheses, clinicians in training were less likely to assign alcohol use disorder to Asian Americans compared to Whites, as well as to the unprimed condition versus the condition primed with the stereotype. Implications regarding cultural competence and future research are discussed.     

Supporting older people following out of hours discharge from the Emergency Department: An integrative review of the literature

Background

For many older people the emergency department (ED) is an important but sometimes difficult step in their healthcare journey. They often attend the ED with co and multi morbidities. Discharge home at evenings and weekends when post-discharge support services are limited can result in a delay or failure to follow through on their discharge plan leading to adverse health outcomes and in some cases, readmission to ED.

Objective

The aim of this integrative review was to identify and appraise the support available to older people following discharge from the ED out of hours (OOH).

Methods

For this review, out of hours referred to those times after 17.30 until 08.00 a.m. on Mondays to Fridays, all hours on weekends and public holidays. Whittemore and Knafl's (Journal of Advanced Nursing, 2005;52:546), framework was used to guide all stages of the review process. Articles were retrieved following a rigorous search of published works using various databases, the grey literature and hand search of the reference lists of the studies included.

Results

In total 31 articles were included in the review. These comprised systematic reviews, randomised control studies, cohort studies and surveys. Main themes identified included processes that enable support, support provision by health and social care professionals and telephone follow-up. Results identified a significant dearth of out of hours discharge research and a strong recommendation for more concise and thorough research in this important area of care transition.

Conclusion

Older person discharge home from the ED presents an associated risk as previous research has identified frequent readmission and periods of ill health and dependency. Out of hours discharge can be even more problematic when it may be difficult to arrange support services and ensure continuity of care. Further work in this area is required, taking cognisance of the findings and recommendations identified in this review.