SARS-CoV-2 Infection Presenting as Acute Chest Syndrome in a Child With Hemoglobin SD-Los Angeles Disease: A Case Report and Review of Literature.

Hemoglobin D-Los Angeles is a variant of hemoglobin that can polymerize in the deoxygenated state. When co-inherited with Hemoglobin S (HbSD-Los Angeles disease) a severe sickling syndrome similar to HbSS can result. Corona virus infectious disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome-corona virus-2. It has been associated with acute chest syndrome (ACS) in individuals with sickle cell disease (SCD), but this complication has not previously been reported in patients with HbSD-Los Angeles. Dexamethasone has been shown to improve outcomes in non-SCD patients with severe acute respiratory syndrome-corona virus-2 pneumonia or acute respiratory distress syndrome; however, its use in SCD patients with ACS is controversial due to a reported increased risk of complications including vaso-occlusive painful episodes. Herein, we reported a patient with HbSD-Los Angeles and COVID-19-associated ACS whom we treated with dexamethasone without transfusion. The patient experienced a rapid recovery without sequelae from steroid use. To further evaluate the use of steroids, we conducted a literature review focusing on the management of pediatric SCD patients with COVID-19-associated ACS. We identified a total of 39 pediatric patients with SCD and COVID-19, of whom 21 (54%) had ACS. Packed red blood cell transfusion (n=11), exchange transfusion (n=4), or a combination of exchange transfusion and packed red blood cell transfusion (n=4) were the most frequently reported treatment, with hydroxychloroquine (n=5), remdesivir (n=1), and tocilizumab (n=1) also being reported. Three patients were treated with dexamethasone. All patients recovered and no adverse outcomes from steroid use were reported. Even though transfusion is considered the standard of care for children with ACS and steroids are not routinely recommended, our experience suggested that COVID-19-associated ACS may be an important exception, especially for patients who refuse transfusion or are in resource-poor nations where blood transfusions may not be readily available. Further studies are warranted to confirm these observations.

Case report: Refractory Evans syndrome in two patients with spondyloenchondrodysplasia with immune dysregulation treated successfully with JAK1/JAK2 inhibition.

Biallelic mutations in the ACP5 gene cause spondyloenchondrodysplasia with immune dysregulation (SPENCDI). SPENCDI is characterized by the phenotypic triad of skeletal dysplasia, innate and adaptive immune dysfunction, and variable neurologic findings ranging from asymptomatic brain calcifications to severe developmental delay with spasticity. Immune dysregulation in SPENCDI is often refractory to standard immunosuppressive treatments. Here, we present the cases of two patients with SPENCDI and recalcitrant autoimmune cytopenias who demonstrated a favorable clinical response to targeted JAK inhibition over a period of more than 3 years. One of the patients exhibited steadily rising IgG levels and a bone marrow biopsy revealed smoldering multiple myeloma. A review of the literature uncovered that approximately half of the SPENCDI patients reported to date exhibited increased IgG levels. Screening for multiple myeloma in SPENCDI patients with rising IgG levels should therefore be considered.

Profile Changes and Stability following Distraction Osteogenesis with Rigid External Distraction in Adult Cleft Lip and Palate Deformities.

OBJECTIVES: The objective of this study is to analyze the hard and soft-tissue profile changes as well as the upper airway changes after distraction osteogenesis (DO) using rigid external distraction device in adult cleft lip and palate (CLP) patients. The study also evaluates the stability of the surgical result. MATERIALS AND METHODS: Three lateral cephalometric radiographs were taken: Predistraction (T1), postdistraction (T2), and 1 year after distractor removal (T3). The treatment changes (T1 vs. T2) and the stability (T2 vs. T3) were analyzed. The overall treatment changes after 1 year were also evaluated (T1 vs. T3). The lateral cephalograms were digitally analyzed with the help of software named Dolphin. STATISTICAL ANALYSIS USED: Wilcoxon Signed-Ranks test was used, and the probability value (P value) of 0.05 was considered as statistically significant level. RESULTS: Eleven adult patients with CLP were retrospectively analyzed. After distraction, there was a significant mean maxillary advancement of 14 mm (P < 0.01) from a T1 value of 73.54 ± 10.38 to a T2 value of 88.2 ± 10.49. The lower facial height and the incisor exposure were significantly increased. The nasolabial angle had a significant improvement of 24.5° (P < 0.01) from a T1 value of 56.6 ± 21.03 to a T2 value of 81.18 ± 14.4.The upper airway was significantly improved by 3.7 mm (P < 0.01) with a T1 value of 13.5 ± 3.8 to a T2 value of 17.2 ± 3.66. After 1-year follow-up, there was a significant maxillary relapse of 3.20 mm (P < 0.05) from a T2 value of 8.29 ± 6.84 to a T3 value of 5.09 ± 5.59. However, the soft-tissue profile and upper airway remained stable. CONCLUSION: The clinician should have an understanding of the related hard and soft tissues as well as airway changes which may assist him when planning for maxillary advancement for CLP patients with DO. There were significant improvements immediately after distraction, but during the 1-year follow-up, some relapse was seen. This stressed on the need for overcorrection of about 35%-40% for adult CLP patients.

Anaesthetic management of a patient with severe pulmonary arterial hypertension for renal transplantation.

We describe a patient with severe pulmonary arterial hypertension scheduled to undergo live-related renal transplantation. We emphasise on meticulous anaesthetic management and early renal transplantation to prevent the progression of disease which would become refractory to treatment, leading to right ventricular failure. Regional (continuous epidural) anaesthesia has been used as technique of choice, where the selective advantages of this technique have been put to good use.

Diffuse intrinsic pontine glioma biopsy: a single institution experience.

Tumor biopsy is rarely performed in diffuse intrinsic pontine glioma (DIPG) due to the presumed risk of surgical complications, although data on the surgery related morbidity of DIPG biopsy is sparse. We performed a retrospective review on 22 consecutive cases of DIPG diagnosed from 2002 to 2012 at Children's Hospital of Michigan, 15 of which underwent biopsy. Transient new or worsening neurological deficits were observed in three of 15 cases following surgery. No surgery related mortality or permanent deficit was observed, and the mean overall survival was 10.4 ± 3.8 months. Undergoing biopsy did not adversely affect the outcome.

Modification of the dingman mouth gag for better visibility and access in the management of cleft palate.

Palatal and pharyngeal surgeries often require wide visibility and access. Various mouth gags and retractors have been devised and many modifications suggested to optimize these surgeries. The Dingman mouth gag, one of the commonly used retractors, offers a lot of advantages in terms of good mouth opening, tongue retraction, self-retaining cheek retractors, and anchorage for sutures, but it has a main limitation in that it allows only limited visibility of the anterior palate and alveolus. Hence, a modification of the Dingman mouth gag is presented for better visibility of and accessibility to the anterior palate.

A Novel Sterol Isolated from a Plant Used by Mayan Traditional Healers Is Effective in Treatment of Visceral Leishmaniasis Caused by Leishmania donovani.

Visceral leishmaniasis (VL), caused by the protozoan parasite Leishmania donovani, is a global health problem affecting millions of people worldwide. Treatment of VL largely depends on therapeutic drugs such as pentavalent antimonials, amphotericin B, and others, which have major drawbacks due to drug resistance, toxicity, and high cost. In this study, for the first time, we have successfully demonstrated the synthesis and antileishmanial activity of the novel sterol pentalinonsterol (PEN), which occurs naturally in the root of a Mexican medicinal plant, Pentalinon andrieuxii. In the experimental BALB/c mouse model of VL induced by infection with L. donovani, intravenous treatment with liposome-encapsulated PEN (2.5 mg/kg) led to a significant reduction in parasite burden in the liver and spleen. Furthermore, infected mice treated with liposomal PEN showed a strong host-protective TH1 immune response characterized by IFN-γ production and formation of matured hepatic granulomas. These results indicate that PEN could be developed as a novel drug against VL.

Asynchronous virtual visits for the follow-up of chronic conditions.

BACKGROUND: Both primary care and specialty care in many areas face access constraints. Tools to evaluate and engage patients with chronic disease, without having them present to the clinic, are needed. Asynchronous virtual care has been explored as one of the ways to deliver care more efficiently, yet this has not been integrated into a busy practice environment. This pilot study aims to assess the utility of a Web platform that allows patients with chronic disease to be evaluated for follow-up care, thereby avoiding an office visit. MATERIALS AND METHODS: Patients with 10 common chronic conditions were recruited into the study at a busy primary care clinic. Instead of booking an in-office follow-up visit, they were directed online to complete a questionnaire pertaining to their condition 7-28 days after their office visit. Their physician would review their responses and make treatment decisions, informing the patient online. Patient and physician satisfaction was measured using a validated Likert scale after each visit. RESULTS: Patients were satisfied with the Web site and process as a way to receive their follow-up care. Clinicians were satisfied in making clinical decisions with the information received via the Web site. The clinician time spent for the overall encounter was significantly shorter than for an in-person follow-up visit. CONCLUSIONS: Clinicians and patients are interested in tools that improve patient health, are convenient, and save time for both parties. Targeting patients with chronic illness and leveraging available technology to deliver the care are very satisfactory to both clinicians and patients. Asynchronous virtual visits for patients with chronic medical conditions are an effective way to evaluate and manage patients, while providing physicians significant time savings. These visits have the potential to reduce in-office follow-up visits across primary care, potentially improving access and reducing costs.

A 'pen' in the neck: an unusual foreign body and an unusual path of entry.

Penetrating injuries to head and neck region with varying objects have been reported in the literature. Majority of these injuries occur in interpersonal violence or bomb blasts or road traffic accidents. Despite the improvement in imaging technologies and surgical methods, penetrating injuries to head and neck with impacted foreign bodies are very challenging due to the proximity to vital structures and/or difficulties in accessing them for the removal. Following injury the normal anatomy could be altered because of edema or tissue destruction, which makes the diagnosis or retrieval more difficult. Parapharyngeal or prevertebral space is an unusual place for lodgment of foreign bodies and in these cases the usual point of entry is the oral cavity, cheek or neck. Here, we report a case of a ball point pen extending to the prevertebral region at the level of C1-C2 vertebrae from point of entry at the suprazygomatic region in the temporal fossa.

Rehabilitation of edentulous atrophic anterior mandible - the role of vertical alveolar distraction osteogenesis.

The rehabilitation of patients with edentulous alveolar ridge is always a challenge, more so in case of a long standing atrophic mandible. Mandible, the largest movable bone in the maxillofacial skeleton is associated with many soft tissue attachments which imparts dislodging forces to prosthesis. In addition to this, the rate of resorption of the mandibular ridge is four times that of the maxilla. These factors make the environment of the mandibular arch less favorable to complete denture stability and retention. An ideal solution would be to augment the atrophic alveolar ridge with native bone of the individual which shall eliminate the possible complications, associated with conventional ridge augmentation procedures. With advent of modern technology, and increased biological understanding, the principles of distraction osteogenesis are increasingly being applied to the craniofacial skeleton and have been found to be a viable option in augmenting the native alveolar bone in the mandible. Here the application of an indigenous stainless steel vertical alveolar distraction device to augment atrophic anterior mandibular ridge is assessed in six patients.

Mandibular conservation in oral cancer.

Surgery is one of the established modes of initial definitive treatment for a majority of oral cancers. Invasion of bony or cartilaginous structures by advanced upper aero-digestive tract cancer has been considered an indication for primary surgery on the basis of historic experience of poor responsiveness to radiation therapy [1]. The mandible is a key structure both in the pathology of intra-oral tumours and their surgical management. It bars easy surgical access to the oral cavity, yet maintaining its integrity is vital for function and cosmesis. Management of tumours that involve or abut the mandible requires specific understanding of the pattern of spread and routes of tumour invasion into the mandible. This facilitates the employment of mandibular sparing approaches like marginal mandibulectomy and mandibulotomy, as opposed to segmental or hemimandibulectomy which causes severe functional problems, as the mandibular continuity is lost. Accurate preoperative assessment that combines clinical examination and imaging along with the understanding of the pattern of spread and routes of invasion is essential in deciding the appropriate level and extent of mandibular resection in oral squamous cell carcinoma. Studies have shown that local control rates achieved with marginal mandibulectomy are comparable with that of segmental mandibulectomy. In carefully selected patients, marginal mandibulectomy is an oncologically safe procedure to achieve good local control and provides a better quality of life. This article aims to review the mechanism of spread, evaluation and prognosis of mandibular invasion, various techniques and role of mandibular conservation in oral squamous cell carcinoma.

Predictors of mandibular involvement in cancers of the oromandibular region.

PURPOSE: Invasion of the mandible by oral squamous carcinoma is not only a relative contraindication to mandible conservation but also an indicator of poor prognosis. This study looks at clinical, radiologic, and operative variables that may help in predicting mandibular bone involvement. PATIENTS AND METHODS: A prospective study was carried out to evaluate the mandibular involvement and its predictors in 51 cases of oral squamous carcinoma located in the mandibular region. All patients underwent segmental- or hemimandibulectomy. A detailed clinical examination was followed by radiologic assessment and operative assessment. Statistic analysis was carried out by chi(2) test (odds ratio [OR] with a significance level of 5%). Multivariate analysis was carried out by logistic regression analysis. RESULTS: Univariate analysis identified location of tumor on lower alveolus (OR = 8.5), sensory disturbances of inferior alveolar nerve (OR = 16.2), location of tumor within 1 cm of mandible (OR = 1.4), presence of findings on periosteal striping (OR = 2.0) like subperiosteal reaction (OR = 3.5), cortical expansion (OR = 8.8) and presence of pathologic fracture (OR = 2.3) as predictor of bone invasion. Grade of tumor (P = .05) and radiologic bone involvement (P = .02) were found to be significant independent predictors of pathologic bone involvement on multivariate analysis. CONCLUSIONS: It is possible to identify mandibular invasion in almost all cases of oral squamous carcinoma by combining clinical examination, radiologic findings, and findings on periosteal stripping. This helps surgeons to make an informed preoperative and intraoperative decision about mandibular conservation. However, one should be careful when evaluating bone involvement on periosteal stripping as this cannot be recommended as a method of choice due to fear of tumor dissemination and violation of oncologic principles.

Patterns of mandibular invasion in oral squamous cell carcinoma of the mandibular region.

BACKGROUND: Mandibular resections are routinely carried out for achieving a R0 resection for oral cancers. However, the need of mandibular resection to achieve this has always been questioned. The present study was carried out to define the pattern of mandibular involvement in carcinoma of the mandibular region. PATIENTS AND METHODS: A total of 25 consecutive patients who had undergone mandibular resection and were found to have mandibular invasion were studied in a prospective open fashion. After decalcification the specimens were serially sectioned at 1 cm interval to identify invasion of mandibular bone. Type of invasion, route of spread and host cell reactions were also recorded. RESULTS: The mandibular involvement was infiltrative in 14(56%) and erosive in 11(44%). It was cortical in 5(20%), marrow involvement was seen in 15(60%) while 5(20%) had spread through the inferior alveolar canal. Of the 25, 24(96%) lesions were located with in 1 cm of the mandible. CONCLUSION: The possibility of mandibular involvement is higher in patients where tumours are located with in 1 cm of the mandible. Involvement of mandible through the canal of inferior alveolar nerve in the present study was relatively high (20%). Therefore it is recommended that before a decision is taken to preserve the mandible it should be thoroughly screened for possible involvement.