Recurrent Hyperparathyroidism Caused by Benign Neoplastic Seeding: Two Cases of Parathyromatosis and a Review of the Literature

Background : Parathyromatosis is defined as small nodules of hyperfunctioning parathyroid tissue scattered in the soft tissues of the neck and/or mediastinum. Parathyromatosis may be primary, it may be aimed for when auto-transplanting parathyroid tissue in secondary hyperparathyroidism, and it may occur after surgery for primary hyperparathyroidism (pHPT). In the latter cases parathyromatosis poses a diagnostic and therapeutic challenge. Methods : To illustrate the clinical problem of parathyromatosis as a rare cause of recurrent disease after an operation for pHPT we describe two patients and performed a review of the literature for contributing factors. Results : Two patients, previously treated for pHPT and having undergone multiple neck explorations had at their final operation numerous small nests of benign parathyroid tissue scattered throughout connective tissue of the neck. These findings concur with various previous cases reported in the reviewed literature.

Conclusions : These cases illustrate that meticulous handling of parathyroid adenomas during surgical excision is of the utmost importance. Regardless of which operating technique is utilised, great emphasis must be placed on precautions towards ensuring the complete and above all the intact removal of the affected gland without capsular rupture. As parathymatosis is caused by seeding, which although extremely rare, it might very well result in the need for extensive reexploration.     

LASIK versus PRK for high astigmatism

Purpose

To compare the refractive outcomes of laser in situ keratomileusis (LASIK) and photorefractive keratectomy (PRK) for myopic astigmatism of 3 diopters (D) or more.

Study design

Retrospective matched comparative study.

Methods

This study include consecutive myopic patients (SE 0 to –10 D) undergoing LASIK or PRK between 2007 and 2016 with astigmatism of 3 to 6 D, and postoperative follow-up of at least 30 days for LASIK and 60 days for PRK, compared outcomes of LASIK and PRK eyes.

Results

The LASIK and PRK groups comprised 175 eyes of 175 patients each, with median follow-up of 39 and 139 days, respectively (P?<?0.001). Mean preoperative manifest astigmatism was –3.35?±?0.46 and –3.42?±?0.51 D (P?=?0.92), postoperative SE was –0.43?±?0.55 and –0.16?±?0.64 D (P?<?0.001), and arithmetic astigmatism was –0.59?±?0.46 and –0.88?±?0.60 D (P?<?0.001), for the LASIK and PRK groups, respectively. Fifty-seven and 64.0% eyes had postoperative SE within?±?0.5 D of emmetropia (P?=?0.19), and 57.7 and 38.8% eyes were within 0.5 D of attempted astigmatic correction (P?<?0.001) for the LASIK and PRK groups, respectively. More PRK eyes were overtreated regarding both SE and astigmatism than LASIK eyes (P?<?0.001). The efficacy and safety indices were close to 1.0 in both groups. The surgically induced astigmatism, magnitude of error, index of success, correction index and flattening index were all better in the LASIK group.

Conclusion

Both LASIK and PRK achieve good outcomes in high astigmatism. LASIK achieved mild superiority over PRK.

     

Frequency of hand movements as a possible diagnostic tool for parkinsonian bradykinesia. Proposal of a simple bedside test

Neurological Sciences - Bradykinesia, dysrhythmia, and decrement in hand movements (HM) are core symptoms of Parkinson’s disease (PD). The maximal rate of repetitive rhythm-preserving HM can...     

Systemic granulomatosis and hypercalcaemia following intravesical bacillus Calmette-Guérin immunotherapy

Abstract.  Schattner A, Gilad A, Cohen J (Kaplan Medical Center, Rehovot; and Hebrew University-Hadassah Medical School, Jerusalem, Israel). Systemic granulomatosis and hypercalcaemia following intravesical bacillus Calmette–Guérin immunotherapy (Case report). J Intern Med 2002; 251: 272–277.     

Myotonia in DNM2-related centronuclear myopathy

Centronuclear myopathy (CNM) is a rare hereditary myopathy characterized by centrally located muscle fiber nuclei. Mutations in the dynamin 2 (DNM2) gene are estimated to account for about 50 % of CNM cases. Electromyographic recordings in CNM may show myopathic motor unit potentials without spontaneous activity at rest. Myotonic discharges, a distinctive electrical activity caused by membrane hyperexcitability, are characteristic of certain neuromuscular disorders. Such activity has been reported in only one CNM case without a known genetic cause. We sequenced the DNM2 gene and the genes associated with myotonia (CLCN1, SCN4A, DMPK and ZNF9) in a sporadic adult patient with CNM and myotonic discharges. Sequencing the entire coding region and exon–intron boundaries revealed a heterozygous c.1106g-a substitution in exon 8, resulting in a R369Q change in the DNM2. Sequencing the CLCN1, SCN4A, DMPK and ZNF9 genes ruled out mutations in these genes. This is the first report of DNM2-related CNM presenting with myotonia. The diagnosis of CNM should be considered in patients with myotonic discharges of an unknown cause.     

SVEP1 plays a crucial role in epidermal differentiation

SVEP1 is a recently identified multidomain cell adhesion protein, homologous to the mouse polydom protein, which has been shown to mediate cell‐cell adhesion in an integrin‐dependent manner in osteogenic cells. In this study, we characterized SVEP1 function in the epidermis. SVEP1 was found by qRT‐PCR to be ubiquitously expressed in human tissues, including the skin. Confocal microscopy revealed that SVEP1 is normally mostly expressed in the cytoplasm of basal and suprabasal epidermal cells. Downregulation of SVEP1 expression in primary keratinocytes resulted in decreased expression of major epidermal differentiation markers. Similarly, SVEP1 downregulation was associated with disturbed differentiation and marked epidermal acanthosis in three‐dimensional skin equivalents. In contrast, the dispase assay failed to demonstrate significant differences in adhesion between keratinocytes expressing normal vs low levels of SVEP1. Homozygous Svep1 knockout mice were embryonic lethal. Thus, to assess the importance of SVEP1 for normal skin homoeostasis in vivo, we downregulated SVEP1 in zebrafish embryos with a Svep1‐specific splice morpholino. Scanning electron microscopy revealed a rugged epidermis with perturbed microridge formation in the centre of the keratinocytes of morphant larvae. Transmission electron microscopy analysis demonstrated abnormal epidermal cell‐cell adhesion with disadhesion between cells in Svep1‐deficient morphant larvae compared to controls. In summary, our results indicate that SVEP1 plays a critical role during epidermal differentiation.     

Synaptic pathways in neural microcircuits

The functions performed by different neural microcircuits depend on the anatomical and physiological properties of the various synaptic pathways connecting neurons. Neural microcircuits across various species and brain regions are similar in terms of their repertoire of neurotransmitters, their synaptic kinetics, their short-term and long-term plasticity, and the target-specificity of their synaptic connections. However, microcircuits can be fundamentally different in terms of the precise recurrent design used to achieve a specific functionality. In this review, which is part of the TINS Microcircuits Special Feature, we compare the connectivity designs in spinal, hippocampal, neocortical and cerebellar microcircuits, and discuss the different computational challenges that each microcircuit faces.     

Prostatic involvement by transitional cell carcinoma in patients with bladder cancer and its prognostic significance

To study the importance of prostatic involvement by transitional cell carcinoma (TCC) in patients with bladder cancer, we examined the entire prostates by whole-mount sections from 214 radical cystoprostatectomy specimens for detailed patterns of involvement by TCC and correlated the results with lymph node metastasis and patients' survival. Prostatic involvement by TCC was detected in 69 (32%) of 214 cases. Among them, 30 (43%) patients had carcinoma in situ (CIS) and the other 39 (57%) were invasive TCC. Carcinoma in situ occurred in either prostatic urethra (n = 6, 20%) or, more commonly, in prostatic ducts/acini (n = 14, 47%), and in a combination of prostatic urethra and ducts (n = 10, 33%). Ten (26%) of the invasive TCC resulted from direct penetration from the primary tumor in the bladder, and the remaining 29 (72%) cases arose from prostatic urethra/ducts, of which 11, 13, and 5 invaded the lamina propria, prostatic stroma, and periprostatic or seminal vesical tissue, respectively. Both prostatic TCC involvement and nodal metastasis were highly significant prognostic factors for patients' survival and the survival significance of prostatic TCC involvement still existed regardless of lymph node status. Furthermore, the presence of prostatic CIS and degrees of prostatic invasion are associated with nodal metastasis and survival. Patients with prostatic CIS or urethral lamina propria invasion had a similar, but higher incidence of lymph node metastasis and lower long-term and 5-year survival than those patients without prostatic involvement. Similarly, prostatic stromal invasion and periprostatic/seminal vesical invasion had a similar, but much higher nodal metastasis and worse survival than patients with only prostatic CIS or urethral lamina propria invasion. In summary, presence of prostatic TCC involvement and levels of involvement are significant prognostic factors in patients with bladder cancer.