Two cases of Kallmann syndrome associated with empty sella

Kallmann syndrome (KS) is a developmental disease characterized by the association of isolated hypogonadotropic hypogonadism and anosmia/hyposmia. We report an unusual presentation of two females with KS and empty sella. These females, aged at 20 and 29-year-old, presented primary amenorrhea with prepubertal estradiol and low gonadotropin levels. No other significant clinical signs were observed. Empty sella was observed on MRI in both cases. Sequencing of FGFR1 gene, recently implicated in autosomal form of KS, was performed and one splicing mutation (IVS14 + 1G > A) was identified in one patient.     

Does Physicians’ Case Volume Impact Inpatient Care Costs for Pneumonia Cases?

Summary Background Increasing physician case volumes are documented to reduce costs and improve outcomes for many surgical procedures but not for medical conditions such as pneumonia that consume significant health care resources. Objective This study explored the association between physicians’ inpatient pneumonia case volume and cost per discharge. Design The design was a retrospective, population-based, cross-sectional study, using National Health Insurance administrative claims data. Setting The setting was Taiwan. Participants The participants were a universal sample of 270,002 adult, acute pneumonia hospitalizations, during 2002–2004, excluding transferred cases and readmissions. Measurements Hierarchical linear regression modeling was used to examine the association of physician’s volume (three volume groups, designed to classify patients into approximately equal sized groups) with cost, adjusting for hospital random effects, case severity, physician demographics and specialty, hospital characteristics, and geographic location. Results Mean cost was NT$2,255 (US$1 = NT$33 in 2004) for low-volume physicians (≤100 cases) and NT$1,707 for high-volume physicians (≥316 cases). The adjusted patient costs for low-volume physicians were higher (US$264 and US$235 than high- and medium-volume physicians, respectively; both P < .001), with no difference between high- and medium-volume physicians. High-volume physicians had lower in-hospital mortality and 14-day readmission rates than low-volume physicians. Conclusions Data support an inverse volume–cost relationship for pneumonia care. Decision processes and clinical care of high-volume physicians versus low-volume physicians should be studied to develop effective care algorithms to improve pneumonia outcomes and reduce costs.     

Unusual case of camptocormia triggered by lumbar-disc herniation

A 21-year-old male patient with low back pain and marked forward bending was presented. The exaggerated lumbar flexion was preventing him to stand in erect posture but disappeared while lying. The symptoms had begun after he had lifted a heavy object. Straight-leg-raising test could not be performed properly because of the exaggerated pain. The light-touch sense was decreased on L5 and S1 dermatomes. There was no loss of muscle strength. The deep-tendon reflexes were normal. Plain graph showed mild narrowing in the L4–5 and L5–S1 intervertebral spaces. Lumbar magnetic resonance imaging revealed disc protrusions in L4–5 and L5–S1 levels. During his stay in the department, the patient was given tizanidine and tramadol, and physical therapy was performed. A paravertebral intramuscular injection with lidocaine was applied. Moreover, the patient was referred to psychiatrist for evaluation regarding his medical history of conversive seizures and possible efforts for secondary gain. No response was obtained from all the treatments. The final diagnosis was camptocormia triggered by lumbar-disc herniation. He was applied supportive psychotherapy, psychoeducation regarding secondary gain, strong suggestions to improve posture, positive reinforcement, and behavioral therapy. His postural abnormality resolved and disappeared completely with mild pain. Electronic supplementary material The online version of this article (doi:) contains supplementary material, which is available to authorized users.     

Phagocytosis of co-developing neutrophil progenitors by dendritic cells in a culture of human CD34<Superscript>+</Superscript> cells with granulocyte colony-stimulating factor and tumor necrosis factor-α

Tumor necrosis factor-alpha (TNF-alpha) has been shown to induce the differentiation of CD34(+) cells toward dendritic cells (DCs). We have previously shown that DCs are co-generated from human CD34(+) cells during erythroid or megakaryocytic differentiation in the presence of TNF-alpha, and those DCs are able to stimulate autologous T cell proliferation. The aim of this study was to learn whether the co-stimulation of granulocyte colony-stimulating factor (G-CSF) and TNF-alpha would generate neutrophil progenitors and DCs together from human CD34(+) cells, and if this was the case, to clarify the phenotypic and functional characteristics of these DCs. When highly purified human CD34(+) cells were cultured for 7 days with G-CSF alone, the generated cells predominantly expressed a granulocyte marker, CD15, and then differentiated into neutrophils after 14 days of culture. The addition of TNF-alpha with G-CSF markedly decreased the number of CD15(+) cells without affecting the total number of cells during 7 days of culture. Almost one third of the generated cells were positive for CD11c and CD123. Furthermore, CD11c(+) cells were found to phagocytose CD15(+) cells and were able to induce allogeneic, but not autologous, T cell proliferation in the mixed lymphocyte reaction (MLR). On the other hand, the CD11c(+) cells generated by TNF-alpha and cytokines capable of inducing erythroid differentiation were able to stimulate autologous T cells. There was a difference in the expression of CD80, CD83 and CD86 among CD11c(+) cells induced by G-CSF plus TNF-alpha and those generated by interleukin-3, stem cell factor, and erythropoietin plus TNF-alpha. These results indicate that the co-stimulation of human CD34(+) cells with G-CSF and TNF-alpha induces the phagocytosis of co-developing neutrophil progenitors by DCs, and the stimulatory effects of these DCs on autologous T cells is different from that of DCs generated from CD34(+) cells during erythroid differentiation.     

Early heparinization decreases the incidence of left atrial thrombi detected by intracardiac echocardiography during radiofrequency ablation for atrial fibrillation

INTRODUCTION: We reviewed our experience in managing intracardiac ultrasound-detected left atrial thrombus and analyzed the impact of the timing of heparin therapy on thrombus incidence. METHODS AND RESULTS: We identified 508 patients undergoing ablation procedures for atrial fibrillation in which intracardiac ultrasound was used. All patients received unfractionated heparin during the procedure: 31 patients before the first transseptal puncture (preTS1), 257 between the first and second transseptal punctures (TS1-TS2), and 220 following both punctures (postTS2). By using intracardiac echocardiography (ICE), thrombus was detected in 30 of these 508 patients (5.9%). Of these, 29 were in the left atrium and constituted our study group. In 21 patients, the thrombi were successfully aspirated from the left atrium using strong suction through the transseptal sheath. All patients in whom thrombi were aspirated did well without neurological event or death. When patients received heparin therapy either preTS1 or TS1-TS2, there was a significant decrease in the occurrence of ICE-detected left atrial thrombus compared with those who received heparin postTS2 (0 of 31 patients (0%) preTS, 9 of 257 (3.5%) TS1-TS2, and 20 of 220 (9.1%) postTS2; (preTS1 vs postTS2, p = 0.01; preTS2 [preTS1 and TS1-TS2] vs postTS2, p < 0.001). CONCLUSION: Early administration of intravenous heparin, specifically before transseptal puncture, decreases the incidence of left atrial thrombi.     

Endoscopic submucosal dissection for cancers of the remnant stomach after distal gastrectomy

BACKGROUND: Endoscopic submucosal dissection (ESD) of early gastric cancer is less invasive than surgical resection, and if technically feasible, it may result in less long-term morbidity than does incisional surgery. However, ESD is technically difficult in patients who have had a previous distal gastrectomy. OBJECTIVE: Our purpose was to retrospectively assess the results of ESD of early gastric cancer in the remnant stomach. DESIGN: Case series. SETTING AND PATIENTS: A total of 31 lesions in 30 patients with early remnant gastric cancer were treated with ESD at Okayama University Hospital, Tsuyama Central Hospital, Hiroshima City Hospital, Kagawa Prefectural Central Hospital, and Mitoyo General Hospital from March 2001 to January 2007. INTERVENTION: ESD. MAIN OUTCOME MEASUREMENTS: En bloc resection rate, complete resection rate, operation time, and complications. RESULTS: En bloc resection and complete resection were achieved in 30 (97%) and in 23 (74%) lesions, respectively. The median operation time required for ESD in the remnant stomach was 113 minutes (range 45-450 minutes). Perforation occurred in 4 (13%). The incidence of delayed bleeding requiring blood transfusion was 0%. LIMITATION: Short duration of follow-up. CONCLUSIONS: ESD is feasible in the remnant stomach but has a relatively high complication rate and should only be performed by experienced endoscopists.     

Fully automated diagnostic system with artificial intelligence using endocytoscopy to identify the presence of histologic inflammation associated with ulcerative colitis (with video)

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Does Maternal HIV Disclosure Self-Efficacy Enhance Parent–Child Relationships and Child Adjustment?

Nondisclosure of maternal HIV status to young children can negatively impact child functioning; however, many mothers do not disclose due to lack of self-efficacy for the disclosure process. This study examines demographic variations in disclosure self-efficacy, regardless of intention to disclose, and assesses the relationship between self-efficacy and child adjustment via the parent–child relationship among a sample of HIV+ mothers and their healthy children (N = 181 pairs). Mothers completed demographic and self-efficacy measures; children completed measures assessing the parent–child relationship and child adjustment (i.e., worry, self-concept, depression). Across demographics, few mothers reported confidence in disclosure. Results from covariance structural modeling showed mothers endorsing higher self-efficacy had children who reported better relationship quality, and, in turn, reported fewer adjustment difficulties; higher levels of disclosure self-efficacy also directly predicted fewer adjustment problems. Findings offer support for interventions aimed at providing mothers with skills to enhance confidence for disclosing their HIV status.     

Incorporating PROMIS Symptom Measures into Primary Care Practice—a Randomized Clinical Trial

Background

Symptoms account for more than 400 million clinic visits annually in the USA. The SPADE symptoms (sleep, pain, anxiety, depression, and low energy/fatigue) are particularly prevalent and undertreated.

Objective

To assess the effectiveness of providing PROMIS (Patient-Reported Outcome Measure Information System) symptom scores to clinicians on symptom outcomes.

Design

Randomized clinical trial conducted from March 2015 through May 2016 in general internal medicine and family practice clinics in an academic healthcare system.

Participants

Primary care patients who screened positive for at least one SPADE symptom.

Interventions

After completing the PROMIS symptom measures electronically immediately prior to their visit, the 300 study participants were randomized to a feedback group in which their clinician received a visual display of symptom scores or a control group in which scores were not provided to clinicians.

Main Measures

The primary outcome was the 3-month change in composite SPADE score. Secondary outcomes were individual symptom scores, symptom documentation in the clinic note, symptom-specific clinician actions, and patient satisfaction.

Key Results

Most patients (84%) had multiple clinically significant (T-score?≥?55) SPADE symptoms. Both groups demonstrated moderate symptom improvement with a non-significant trend favoring the feedback compared to control group (between-group difference in composite T-score improvement, 1.1; P?=?0.17). Symptoms present at baseline resolved at 3-month follow-up only one third of the time, and patients frequently still desired treatment. Except for pain, clinically significant symptoms were documented less than half the time. Neither symptom documentation, symptom-specific clinician actions, nor patient satisfaction differed between treatment arms. Predictors of greater symptom improvement included female sex, black race, fewer medical conditions, and receiving care in a family medicine clinic.

Conclusions

Simple feedback of symptom scores to primary care clinicians in the absence of additional systems support or incentives is not superior to usual care in improving symptom outcomes.

Trial Registration

clinicaltrials.gov identifier: NCT02383862.