scholarly journals Type VI Aplasia Cutis Congenita: Bart’s Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Ferit Kulalı ◽  
Ahmet Yagmur Bas ◽  
Yusuf Kale ◽  
Istemi Han Celik ◽  
Nihal Demirel ◽  
...  

Bart’s syndrome is characterized by aplasia cutis congenita and epidermolysis bullosa. We present the case of a newborn male who developed blisters on the mucous membranes and the skin following congenital localized absence of skin. Bart’s syndrome (BS) is diagnosed clinically based on the disorder’s unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis. The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow-up examinations showed complete healing. We emphasized that it is important to use relatively simple methods for optimal healing without the need for complex surgical interventions.

2020 ◽  
Vol 8 (10) ◽  
pp. 4701-4709
Author(s):  
Stuti Sharma ◽  
Meenakshi Pandey

Introduction: The word Asrigdara explains about prolonged, cyclic or acyclic excessive menstrual bleed-ing in Ayurveda. 9-14% of women in their reproductive age lose 80 ml of blood in each cycle. It is the most common cause of Anemia. Traditionally, Heavy Uterine Bleeding is managed with medical therapy and surgical intervention with associated side effects. But due to limitation of medical therapy as well as surgical interventions, it becomes the necessity of time to find out a permanent, easy, effective and less side effect producing care which can be easily administered and accepted by the patient. Objective: To evaluate the clinical efficacy of Kutajashtaka Avaleha orally and combination of Kutajashtaka Avaleha and Yashtimadhu Ghrita matra basti in combating the signs and symptoms of Asrigdara. Materials and Methods: 56 patients were selected from the Stri Roga and Prasuti Tantra OPD, All India Institute of Ayur-veda, New Delhi and randomly divided into 2 groups. Group A was administered with Kutajashtaka Ava-leha orally regularly for the period of 3 months with the doses of 5gm BD after meal with water, while in Group B, Combination of Yashtimadhu Ghrita Matra Basti (60 ml for 7 days in 3 consecutive cycles after meal through rectal route after 7th day of menses) for 3 months and Kutajashtaka Avaleha was given and the follow up period of 1 month without medication was same for both the groups. Results: Kutajashtaka Avaleha orally alone and Combination of Yashtimadhu Ghrita Matra Basti & Kutajashtaka Avaleha oral both gave statistically highly Significant results (P<0.001) but with better results when the treatment was combined and Markedly Improved (51-75% relief) the signs and symptoms. Conclusion: Combination of Yashtimadhu Ghrita Matra Basti & Kutajashtaka Avaleha oral gave more statistically significant results than Kutajashtaka Avaleha orally alone. No adverse effects were reported during the entire trial period.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Öznur Özalp ◽  
Nelli Yıldırımyan ◽  
Canan Öztürk ◽  
Burak Kocabalkan ◽  
Göksel Şimşek Kaya ◽  
...  

Abstract Background Leukocyte- and platelet-rich fibrin (L-PRF) is an autologous matrix scaffold which regulates inflammation by stimulating cytokines and growth factors that are involved in the immune response. L-PRF is suggested as a viable adjunctive method to surgical interventions due to its advantages on tissue healing. This study aims to retrospectively evaluate the adjunctive role of L-PRF in surgically treated medication-related osteonecrosis of the jaws (MRONJ) patients. Methods Between January 2012 and December 2020, patients with AAOMS stage II and III MRONJ lesions, who were treated surgically with adjunctive use of L-PRF in the authors’ institution were enrolled. Surgical interventions consisted of either marginal resection or sequestrectomy with peripheral ostectomy (SPO) or curettage and L-PRF application. Medical records of these patients were retrospectively reviewed and healing was assessed according to certain parameters including mucosal closure and presence of infection, exposed bone, fistula or radiologic markers of disease progression for a minimum of 12 months. Results Thirteen patients (7 women and 6 men) with an average age of 72.4 years (± 10.61, range 54–84) were included in the study, nine of whom had AAOMS stage III and four stage II MRONJ. Three patients had a marginal resection, nine patients had sequestrectomy with peripheral ostectomy (SPO) and one patient underwent a curettage procedure. All marginal resection and six SPO patients showed complete healing while four patients, who had SPO or curettage experienced incomplete healing. Mean follow up was 20.1 ± 18.29 months. Conclusion The use of L-PRF may be a favorable adjunctive option in the treatment of MRONJ owing to its favorable effects on tissue repair, ease of application, minimally invasive and cost-effective character and autogenous nature. Trial registration Retrospectively registered.


Author(s):  
Elika Ridelman ◽  
Paulette I Abbas ◽  
Beth A Angst ◽  
Justin D Klein ◽  
Christina M Shanti

Abstract Introduction Pediatric hand friction injuries caused by running treadmills are ever more prevalent due to the increasing presence of home exercise equipment. These and other related injuries have received recent attention in the media, leading to mass product recalls in some cases. These friction injuries are similar to deep thermal burns and these patients are usually cared for in the pediatric burn unit at our institution. We sought to describe our recent experience managing 22 patients with these injuries. Methods Patients were queried from our urban pediatric burn center database. After IRB approval, a retrospective chart review of all patients admitted for a treadmill-related hand injury over a 5-year period was performed. Data collected included patient demographics, initial treatment methods (surgical vs. non-operative), type of dressing used, hospital length of stay (LOS), and compliance with follow-up. We collected patient-centered outcomes included the time to healing and the need for subsequent surgical and/or non-surgical interventions to treat the sequelae of these injuries. Results Our center treated 22 hand friction injuries caused by treadmills over the 5-year period. Five patients (23%) underwent early surgical intervention (&lt;30 days from injury), with a median time from injury of 7 days (IQR 1.75-13.5). This cohort required additional interventions to treat postoperative sequelae with 2 (40%) requiring additional surgery and 3 (60%) managed non-surgically. Of the 17 (77%) who were initially treatednon-operatively , 5 (29%) healed completely with dressing changes only and required no further treatment. Eleven (65%) had injury-related sequelae and were managed successfully with non-surgical interventions. Only 1 patient (6%) required scar modification by z-plasty. Average LOS was 14 hours and median number of weekly follow-up visits was 4 until complete healing. Conclusion Given the excellent outcomes observed in patients treated without acute surgical intervention in this case series, non-operative non-operative management appears to be feasible, safe and may reduce the need for operative intervention. These injuries, although small, are deep and require a prolonged period of dressing changes and aggressive therapy including post-healing time commitment to scar management in order to achieve best outcomes. Our study supports the need for increased community awareness of this type of injury.


1982 ◽  
Vol 56 (5) ◽  
pp. 711-715 ◽  
Author(s):  
Kamel F. Muakkassa ◽  
Robert B. King ◽  
David B. Stark

✓ The management of congenital scalp and skull defects, as generally advocated, is surgical. The authors report such a case that was treated conservatively. At her 3-year follow-up review, the patient's scalp and skull defects and other associated cutaneous defects were fully reconstituted. Such a nonoperative approach, while rarely reported, emphasizes the natural course that some of these lesions may follow. The literature on aplasia cutis congenita is briefly reviewed.


2012 ◽  
Vol 16 (6) ◽  
pp. 523-531 ◽  
Author(s):  
Gregory J. Velat ◽  
Steve W. Chang ◽  
Adib A. Abla ◽  
Felipe C. Albuquerque ◽  
Cameron G. McDougall ◽  
...  

Object Intramedullary, or glomus, spinal arteriovenous malformations (AVMs) are rare vascular lesions amenable to resection with or without adjuvant embolization. The authors retrospectively reviewed the senior author's (R.F.S.'s) surgical series of intramedullary spinal AVMs to evaluate clinical and radiographic outcomes. Methods Detailed chart and radiographic reviews were performed for all patients with intramedullary spinal AVMs who underwent surgical treatment between 1994 and 2011. Presenting and follow-up neurological examination results were obtained and graded using the modified Rankin Scale (mRS) and McCormick Scale. Surgical technique, outcomes, complications, and long-term angiographic studies were reviewed. Results During the study period, 20 patients (10 males and 10 females) underwent resection of glomus spinal AVMs. The mean age at presentation was 30 ± 17 years (range 7–62 years). The location of the AVMs was as follows: cervical spine (n = 10), thoracic spine (n = 9), and cervicothoracic junction (n = 1). The most common presenting signs and symptoms included paresis or paralysis (65%), paresthesias (40%), and myelopathy (40%). Perioperative embolization was performed in the majority (60%) of patients. Pial AVM resection was performed in 17 cases (85%). Angiographically verified AVM obliteration was achieved in 15 patients (75%). At a mean follow-up duration of 45.4 ± 52.4 months (range 2–176 months), 14 patients (70%) remained functionally independent (mRS and McCormick Scale scores ≤ 2). One perioperative complication occurred, yielding a surgical morbidity rate of 5%. Three symptomatic spinal cord tetherings occurred at a mean of 5.7 years after AVM resection. No neurological decline was observed after endovascular and surgical interventions. No deaths occurred. Long-term angiographic follow-up data were available for 9 patients (40%) at a mean of 67.6 ± 60.3 months (range 5–176 months) following AVM resection. Durable AVM obliteration was documented in 5 (83%) of 6 patients. Conclusions Intramedullary AVMs may be safely resected with satisfactory clinical and angiographic results. The pial resection technique, which provides subtotal AVM nidus resection, effectively devascularized these lesions, as confirmed on postoperative angiography, without violating the spinal cord parenchyma, thereby potentially reducing iatrogenic injury.


Author(s):  
Jaume Trapé ◽  
Maria Sala ◽  
Fina Franquesa ◽  
Josep M. Ordeig ◽  
Josep M. Soler-Bel ◽  
...  

AbstractDiagnosing patients with signs or symptoms suggestive of cancer is difficult. Serum tumor markers (TM) may be useful, but it is known that a range of pathologies other than cancer can increase their concentrations and so TM data must be interpreted with caution. The aim of this study is to determine the diagnostic accuracy of TMs in patients with signs or symptoms of cancer.We prospectively studied 234 patients seen at rapid diagnostic units who presented signs or symptoms suggestive of cancer. Ninety patients had wasting syndrome, 74 had pulmonary symptoms and 70 other presentations. CYFRA21-1, CEA, CA19-9, total bilirubin and creatinine were determined. The final diagnosis was obtained after 6 months’ follow-up. Patients were classified according to the absence (group A) or presence (group B) of abnormal bilirubin or creatinine.Of the 234 patients studied, 103 (44.0%) had tumors diagnosed. Cut-off points for each TM were calculated for a specificity of 100%. For the total group, the values were CYFRA21-1, 15 μg/L, CEA, 43.8 μg/L and CA19-9, 7428 KU/L, with an overall sensitivity of 46.6%. For group A (n=142), the following cut-off points were established: CYFRA21-1, 7.8 μg/L, CEA, 13.8 μg/L and CA19-9, 101 KU/L, obtaining a sensitivity of 68.6%. For group B (n=92), the values were the same as for the whole group, and a sensitivity of 42.4% was achieved.We conclude that TMs can aid diagnosis in these patients with signs or symptoms suggestive of cancer. Their sensitivity can be improved by using different cut-off points in the presence and absence of renal and hepatic dysfunction.


2021 ◽  
pp. 1-6
Author(s):  
Andrea Scotti ◽  
Elisa Benanti ◽  
Federica Augelli ◽  
Franz Wilhelm Baruffaldi Preis

<b><i>Introduction:</i></b> Aplasia cutis congenita (ACC) is a rare congenital abnormality characterized by the absence of a portion of skin at birth which most commonly involves the scalp and can affect the galea, the pericranium, the bone, and the dura mater. It can be an isolated condition or associated with other disorders. <b><i>Case Report:</i></b> We present a case of ACC with a large defect of the scalp and the underlying bone treated with the use of Integra® Dermal regeneration template. At 5 months of follow-up, the wound is completely healed and the bony defect greatly reduced. Contraction of the area of alopecia was observed. <b><i>Discussion:</i></b> Several surgical and conservative options have been described to treat this congenital condition: advanced dressing, skin graft, local flaps, free flaps, and other methods. In our case, we used Integra® Dermal templates which provide a barrier for infections, promote cellular activity for a rapid vascularization, and improve healing.


2021 ◽  
Vol 15 (1) ◽  
pp. 37-42
Author(s):  
Maryam Alizadeh Chamkhaleh ◽  
Samileh Noorbakhsh ◽  
Mohammad Vafaee-Shahi ◽  
Aina Riahi ◽  
Negar Hajinasab ◽  
...  

Background: One of the important causes of childhood febrile illness is meningitis. It causes bacterial, viral, fungal, parasitic, and non-infectious agents. Pediatric bacterial meningitis is one of the most important causes of infant mortality, especially in premature infants. This study aimed to identify the outcome and mortality of children with meningitis. Methods and Materials: All children with meningitis in the Pediatric Department of Rasool Akram Hospital from December 23, 2007, to December 16, 2017, were included. Signs and symptoms (fever, consciousness, neck rigidity, and seizure) were collected and Cerebrospinal fluid analysis in children was made. Based on these findings, patients were divided into two groups, which include bacterial and non-bacterial meningitis. Then we recalled all families and examined them to discover the child’s outcome and complications at least after two years. Results: During the ten-year study period, 202 children were included in the study. Patients aged less than 12 months were found to be more frequently affected. Fever was the most common presentation (83.4%) followed by seizure (51.5%) and vomiting (49%). 119 (58.9%) of the patients were male and 83 (41.1%) were female. Definite bacterial meningitis was the final diagnosis in 35.6% (72) of patients (mean age 34.1± 48.3 months), and non-bacterial meningitis was diagnosed in 64.4% (130) of cases (mean age 46.3± 52.4 months). The most common organism in bacterial meningitis (by all methods) was Streptococcus pneumonia. Among children, 187 (92.1%) were survivors and 15 (%7.9) were non-survivors. Neurological sequelae such as motor deficit and epilepsy were identified in 9 (4.8%) and 5 (2.6%) subjects, respectively, and that they were all caused by bacterial meningitis. There was no death or neurological sequelae observed during follow-up in children with non-bacterial meningitis. Gender, age, signs and symptoms of patients at the time of admission were not significantly different between the bacterial and non-bacterial meningitis groups (p>0.05). Hydrocephalus, CSF characteristic, morbidity and mortality were significantly different between the bacterial and non-bacterial meningitis groups. Conclusion: This study showed that elevated LDH and low glucose levels in CSF are characteristic of bacterial meningitis. Increased LDH is significantly related to death in children (P-value > 0.001) with meningitis. Beneficial empirical antibiotics and sufficient follow-up by parents cause good prognosis in children with meningitis. Also in a child with fever and seizure, we should think of meningitis, even though in the absence of positive neurological examinations. It seems that increased CSF LDH, elevated CSF protein and age under one year are significant mortality risk factors in children with meningitis.


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