Recurrent Soft Tissue Infections Associated With Burosumab Therapy in X-Linked Hypophosphatemic Rickets.

X-linked hypophosphatemic rickets (XLH) is a genetic disorder characterized by elevated fibroblast growth factor 23 (FGF23), resulting in renal phosphate wasting and inadequate bone mineralization. Burosumab, a monoclonal antibody that inhibits FGF23 activity, has shown promise in improving renal phosphate reabsorption and clinical outcomes in XLH patients. However, the potential side effects of burosumab, particularly its impact on immune function and susceptibility to infections, remain a subject of concern. In this case report, we describe a 57-year-old male individual with XLH who experienced recurrent soft tissue infections while receiving burosumab therapy. The infections included an olecranon abscess, a cervical retropharyngeal phlegmon with a sternocleidomastoid abscess, and suprapubic cellulitis, all of which were treated with antibiotic therapy. Following discontinuation of burosumab therapy, the patient did not experience further soft tissue infections. These observations suggest a potential association between burosumab therapy and an increased risk of soft tissue infections. Mechanistically, disruption of the FGF23-Klotho signaling axis may lead to impaired humoral immunity mediated by B lymphocytes and compromised innate immune response mediated by macrophages. Further investigation is warranted to better understand the immunological effects of burosumab and its implications for infectious complications in XLH patients.

Zoster Sine Herpete: Confirmatory Diagnosis Using Varicella-Zoster Virus DNA Polymerase Chain Reaction Analysis of Intact Skin Scrapings.

Zoster, commonly known as shingles, is a syndrome caused by the reactivation of the varicella-zoster virus (VZV) that results in characteristic vesicular eruptions in a unilateral dermatomal distribution. Timely diagnosis of zoster sine herpete (ZSH), a variant of shingles without the typical vesicular rash, can be challenging due to the absence of visual cues and limited application of traditional diagnostic methods. Recent case reports have demonstrated the utility of VZV DNA polymerase chain reaction (PCR) analysis of involved skin exudates for confirming ZSH. This report presents a case of acute ZSH involving the left trigeminal nerve, where the diagnosis was confirmed using VZV DNA PCR analysis of skin scrapings. The patient initially presented with radicular pain and was treated for presumed shingles, but persistent neuropathic pain persisted. The confirmatory VZV DNA PCR analysis of the involved skin scraping sample aided in establishing the diagnosis of ZSH. The case highlights the potential of using intact skin scrapings for VZV DNA PCR in confirming ZSH. Prompt initiation of antiviral therapy is crucial for minimizing the duration and severity of radicular pain in ZSH cases. Larger studies are needed to further evaluate the utility of VZV DNA PCR analysis for accurately diagnosing ZSH.

Severe Ketamine-Induced Uropathy Associated With Cholangiopathy Requiring Percutaneous Nephrostomy.

Ketamine-induced uropathy (KIU) is becoming more prevalent as ketamine abuse becomes widespread. Recently, a plethora of reports have highlighted the association between recreational ketamine abuse and its deleterious effects including uropathy and cholangiopathy. However, there are hardly any reports that demonstrate the management of severe KIU in young people. We report a case of an Asian female in her late 20's with a 10-year history of ketamine abuse who presented to the emergency department with sharp, epigastric pain for two weeks. An evaluation revealed acute kidney injury and transaminitis with bilirubinemia. CT scan showed bladder and ureteral wall thickening with hydroureteronephrosis. The patient underwent percutaneous bilateral nephrostomy tubes placement, improving urine output and with acute kidney injury resolution. Repeat renal/bladder US showed near complete resolution of bilateral hydronephrosis. This case documents one of the first few documented cases of severe ketamine-induced uropathy with cholangiopathy necessitating bilateral nephrostomies in the continental U.S.

Thyrotoxic Periodic Paralysis: The Peril of Potassium Replacement.

Hypokalemic periodic paralysis (HPP) is a clinical condition of sudden-onset, recurrent transient episodes of weakness caused by severe hypokalemia. Thyrotoxic periodic paralysis (TPP) is a specific subgroup of the HPP spectrum, where hypokalemia occurs in the setting of thyrotoxicosis, and the repletion of potassium must be performed with caution. A male in his second decade of life with hyperthyroidism non-adherent to methimazole presented with acute-onset bilateral lower extremity weakness. On physical examination, the patient had diffuse thyromegaly and tremors on outstretched hands. Bilateral lower extremity weakness and decreased reflexes were also noted, with preserved muscle tone and normal passive range of motion. Labs demonstrated hyperactive thyroid function and severe hypokalemia at 1.7 mEq/L, with U waves present on the electrocardiogram. In the intensive care unit, the patient received methimazole and propranolol for thyrotoxicosis and a total dose of 60 mEq/L of potassium replacement therapy. Despite the expected correction by 0.6 mEq/L, his follow-up potassium level dramatically increased to 5.7 mEq/L, resulting in the actual correction of 4.0 mEq/L. Within a few hours, the patient regained his baseline strength with a significant improvement in tremors. Patients with TPP present with acute-onset extremity weakness and severe hypokalemia, which reverses quickly with potassium repletion. Clinicians should not only treat thyrotoxicosis but also avoid overly aggressive repletion of potassium as this may lead to rebound hyperkalemia when the initial transcellular potassium shift is reversed.

Effect of Process Orientation on the Mechanical Behavior and Piezoelectricity of Electroactive Paper.

This paper reports the effect of process orientation on the mechanical behavior and piezoelectricity of electroactive paper (EAPap) made from natural cotton pulp. EAPap is fabricated by a casting and wet drawing of cellulose film after dissolving cotton with LiCl and DMAc solvent. During the fabrication, permanent wrinkles, a possible factor for performance deterioration, were found in the films. Finite element method was introduced to identify the formation mechanism behind the wrinkles. The simulation results show that the wrinkles were caused by buckling and are inevitable under any conditions. The tensile and piezoelectric tests show that the orientation dependency of the stretched EAPap gives the anisotropic characteristics on both mechanical and piezoelectric properties. In this research, the anisotropic elastic moduli and Poisson's ratios are reported. The piezoelectric charge constant of EAPap in the linear elastic is calculated. The piezoelectric charge constants of EAPap are associated with the alignment angle in the order of 45° > 0° > 90° due to the strong shear effect. The higher stretching ratio gives the higher piezoelectricity due to the alignment of the molecular chains and the microstructure in EAPap. The highest piezoelectric charge constant is found to be 12 pC/N at a stretching ratio of 1.6 and aligning angle of 45°.

Postoperative Pain Control by Intercostal Nerve Block After Augmentation Mammoplasty.

BACKGROUND: In breast augmentation with implant, there is severe pain due to damage from expansion of breast tissue and the pectoralis major. Therefore, the authors conducted this study to analyze the effectiveness of postoperative intercostal nerve block (ICNB) in reducing postoperative pain after breast augmentation with implant. METHOD: Forty-four female patients were enrolled in the study. Just before awaking from general anesthesia, 34 cases were injected with 0.2% ropivacaine to both third, fourth, fifth, and sixth intercostal spaces. We compared them (ICNB group) with the control group for VAS scores at the time of arrival in the recovery room, after 30, 60, and 120 min. RESULT: The average VAS scores per time of the control group and ICNB group were 7.1 ± 0.74 and 3.50 ± 1.81 at arrival time in the recovery room, 7.00 ± 0.67 and 3.03 ± 1.47 after 30 min, 5.50 ± 0.71 and 2.68 ± 1.49 after 60 min, and 4.60 ± 0.84 and 2.00 ± 1.35 after 120 min. VAS scores of two groups were significantly different at each time and decreased overall. Also, time and group effect of the two groups were significantly different, especially between 30 and 60 min. CONCLUSION: ICNB just before awaking from general anesthesia showed a statistically significant reduction in VAS score, and this means postoperative pain was reduced effectively and time to discharge could be shortened. Therefore, it can be a good way to reduce postoperative pain after augmentation mammoplasty with implant. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these evidence-based medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Treatment of the jaw-winking syndrome.

BACKGROUND: Marcus Gunn described for the first time a syndrome consisting of unilateral, congenital ptosis, and rapid exaggerated elevation of the ptotic eyelid during movement of the mandible to the contralateral side. METHODS: Here, clinical findings from the management of 20 patients with jaw-winking syndrome have been reviewed. Preoperative measurement of the ptotic degree and the levator function by Berke's method, and marginal limbal distance were all evaluated. Moreover, the amount of winking found in the upper eyelid on primary gaze was graded on a scale from I to III. The operation was performed on 20 patients under local anesthesia: 10 by unilateral levator resection and the other 10 by the frontalis muscle falp or orbicularis oculi muscle flap. RESULTS: The correction of blepharoptosis was possible without encountering severe complications. However, a moderated degree of jaw-winking, slight undercorrection, and transient lagophthalmos were all inevitable. CONCLUSIONS: The management of patients with jaw-winking syndrome is a challenging endeavor. Therefore, a comprehensive medical and ophthalmologic evaluation and a detailed history are mandatory before undertaking the successful treatment of patients with this syndrome.

Anthropometry of asian eyelids by age.

BACKGROUND: The normal morphologic and functional values of eyelids and orbits vary according to race, sex, and age. Unfortunately, there is a paucity of information related to these values in Asians, leading Asian surgeons to use statistical data from Caucasians. METHODS: The authors quantified and statistically analyzed nine morphologic and functional values in 234 Asian male and 264 Asian female subjects. RESULTS: Among adults, the mean value for the palpebral fissure was 27.0 +/- 1.8 mm in males and 26.8 +/- 1.7 mm in females in the horizontal dimension and 8.0 +/- 1.0 mm in males and 8.2 +/- 1.1 mm in females in the vertical dimension. The average slant of the palpebral fissure was 7.9 +/- 2.4 degrees in males and 8.8 +/- 2.3 degrees in females, and the average height of the opened upper eyelid was 12.4 +/- 2.4 mm in males and 12.0 +/- 1.9 mm in females. The average height of the double fold in the closed eye was 6.6 mm in males and 6.5 mm in females, and the average intercanthal distance was 38.4 +/- 3.0 mm in males and 38.2 +/- 2.8 mm in females. The average interpupillary distance was 64.6 +/- 2.9 mm in males and 63.6 +/- 2.9 mm in females. CONCLUSIONS: The peak level of growth in the vertical dimension of the palpebral fissure was reached between ages 10 and 13 years, that of the intercanthal distance between ages 14 and 16 years, and that of the horizontal dimension of the palpebral fissure between ages 17 and 19 years.

Comparison of levator resection and frontalis muscle transfer in the treatment of severe blepharoptosis.

BACKGROUND: The optimal surgical approach for blepharoptosis is dependent upon many factors, the most important being levator function. However, the preferred approach in severe blepharoptosis remains a matter of contention. METHODS: We investigated 130 patients with levator function between 2 and 4 mm who underwent corrective surgery for blepharoptosis between January 1990 and December 2004. There were 65 eyelids of levator resection performed in 50 patients and 105 eyelids of frontalis transfer performed in 80 patients. Postoperative results were evaluated, with an average follow-up period of 27 months. RESULTS: The average preoperative degree of ptosis was approximately 2.7 mm in cases treated with levator resection and 4.0 mm in cases treated with frontalis muscle transfer. The average postoperative level of ptosis was approximately 1.7 mm in levator resection and 2.1 mm in frontalis muscle transfer. The average degree of postoperative ptosis improvement was approximately 1.0 mm in levator resection and approximately 1.86 mm in frontalis muscle transfer. The most frequent complication of levator resection was undercorrection. Eyelid deformity due to excessive traction was more frequent in the frontalis muscle flap technique. CONCLUSION: Levator resection and frontalis transfer can effectively treat blepharoptosis patients with poor levator function. Frontalis muscle transfer should be performed more carefully in operation to avoid complications which too excessive contraction could cause for blepharoptosis patients with 2 approximately 4 mm of levator function. Also, some accessorial methods were regarded as necessary to prevent undercorrection in performing levator resection.