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1.
Saudi Pharm J ; 32(6): 102055, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38699598

ABSTRACT

Acute myeloid leukaemia (AML) is characterized by uncontrolled proliferation of myeloid progenitor cells and impaired maturation, leading to immature cell accumulation in the bone marrow and bloodstream, resulting in hematopoietic dysfunction. Chemoresistance, hyperactivity of survival pathways, and miRNA alteration are major factors contributing to treatment failure and poor outcomes in AML patients. This study aimed to investigate the impact of the pharmacological p38 mitogen-activated protein kinase (MAPK) inhibitor SB203580 on the chemoresistance potential of AML stem cell line KG1a to the therapeutic drug daunorubicin (DNR). KG1a and chemosensitive leukemic HL60 cells were treated with increasing concentrations of DNR. Cell Titer-Glo®, flow cytometry, phosphokinase and protein arrays, Western blot technology, and reverse transcription-quantitative polymerase chain reaction (RT-qPCR) were employed for assessment of cell viability, half-maximal inhibitory concentration (IC50) determination, apoptotic status detection, cell cycle analysis, apoptosis-related protein and gene expression monitoring. Confocal microscopy was used to visualize caspase and mitochondrial permeability transition pore (mPTP) activities. Exposed at various incubation times, higher DNR IC50 values were determined for KG1a cells than for HL60 cells, confirming KG1a cell chemoresistance potential. Exposed to DNR, late apoptosis induction in KG1a cells was enhanced after SB203580 pretreatment, defined as the combination treatment. This enhancement was confirmed by increased cleavage of poly(ADP-ribose) polymerase, caspase-9, caspase-3, and augmented caspase-3/-7 and mPTP activities in KG1a cells upon combination treatment, compared to DNR. Using phosphokinase and apoptosis protein arrays, the combination treatment decreased survival Akt phosphorylation and anti-apoptotic Bcl-2 expression levels in KG1a cells while increasing the expression levels of the tumor suppressor p53 and cyclin-dependent kinase inhibitor p21, compared to DNR. Cell cycle analysis revealed KG1a cell growth arrest in G2/M-phase caused by DNR, while combined treatment led to cell growth arrest in S-phase, mainly associated with cyclin B1 expression levels. Remarkably, the enhanced KG1a cell sensitivity to DNR after SB203580 pretreatment was associated with an increased upregulation of miR-328-3p and slight downregulation of miR-26b-5p, compared to DNR effect. Altogether, these findings could contribute to the development of a new therapeutic strategy by targeting the p38 MAPK pathway to improve treatment outcomes in patients with refractory or relapsed AML.

2.
Int J Surg Case Rep ; 121: 109935, 2024 Jun 22.
Article in English | MEDLINE | ID: mdl-38941733

ABSTRACT

INTRODUCTION AND IMPORTANCE: Adamantinomas are rare, low-grade, malignant skeletal tumors accounting for only 0.33-0.48 % of primary malignant bone tumors. CASE PRESENTATION: An 11-year-old boy with adamantinoma of the ulna was treated with en bloc resection, kryptonite bone cement, and plate fixation. The surgery resulted in marked pain relief and good functional recovery. No evidence of recurrence was observed for 5 years postoperatively, and the Musculoskeletal Tumor Society score was 93. CLINICAL DISCUSSION: This case of an 11-year-old previously treated for an aneurysmal bone cyst (ABC) illustrates the complexity of diagnosing adamantinoma, especially in atypical locations like the ulna. The successful use of en bloc resection and synthetic bone graft highlights the importance of accurate diagnosis and advanced surgical methods in achieving favorable outcomes in pediatric orthopedic oncology. CONCLUSION: Ulnar adamantinomas are rare and can be difficult to diagnose. The successful treatment of this tumor, as described in this case report, can help guide the management of similar cases in the future.

3.
Cureus ; 16(3): e56743, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38650790

ABSTRACT

Cerebral palsy (CP) is a neurodevelopmental disorder that affects motor function and is often accompanied by secondary musculoskeletal issues. Severe scoliosis, a lateral curvature of the spine over 40 degrees, poses a significant challenge for individuals with CP, impacting their mobility and overall well-being. While the association between scoliosis and gastrointestinal complications is acknowledged, the occurrence of colonic volvulus with necrosis in the context of CP and severe scoliosis is rare and complex. This case report emphasizes the importance of clinical awareness in managing gastrointestinal complications in patients with CP and severe scoliosis. An 11-year-old female presented with gastroenteritis and a concurrent viral upper respiratory tract infection. She experienced complications such as greenish vomiting, hematemesis, abdominal distention, and constipation. The patient has a medical history of epilepsy and was diagnosed with quadriplegic CP at four months old due to viral meningitis. She is currently on anti-epileptic medications and receives regular follow-ups with neurology. Severe lumbar scoliosis of more than 50 degrees Cobb angle is also noted. Physical examination revealed dehydration, bilious content in nasogastric tube (NGT) aspiration, tender abdomen, and an empty digital rectal examination. Some laboratory findings showed elevated levels of erythrocyte sedimentation rate (ESR), prothrombin time (PT), blood urea nitrogen (BUN), and sodium, while albumin levels were decreased, and white blood cell (WBC) count was mildly elevated. Abdominal computed tomography (CT) with contrast showed a distended ascending colon with air and swirling of the mesentery. The distal half of the large bowel was not dilated, and fecal matter was present. The small bowel appeared to be collapsed, and there was moderate free fluid in the peritoneal cavity, indicating colonic volvulus involving the proximal large bowel. The patient underwent surgery, which involved deflating and removing the distended colon, resecting the gangrenous colon, and performing an ilio-sigmoid anastomosis to restore gastrointestinal continuity. Postoperatively, the patient received close monitoring in the pediatric intensive care unit (PICU), received total parenteral nutrition (TPN) for five days, gradually progressed feeding, and showed overall improvement in her condition. In conclusion, this case report highlights a rare occurrence of colonic volvulus in a patient with CP and severe scoliosis. It emphasizes the complex relationship between neurological and musculoskeletal disorders in gastrointestinal complications. A multidisciplinary approach is important for optimal management. It shows the importance of musculoskeletal factors in patients with neurological conditions. Overall, it contributes to the medical literature and emphasizes tailored management strategies for gastrointestinal issues in such patients.

4.
J Family Community Med ; 31(2): 116-123, 2024.
Article in English | MEDLINE | ID: mdl-38800786

ABSTRACT

BACKGROUND: Hypertension (HTN) is a common chronic health problem with many complications and high morbidity rates. This study aimed to describe the HTN pathway, to assess the performance of screening and registration programs, to explore the challenges and suggest solutions for those challenges. MATERIALS AND METHODS: This study was conducted in primary care centers in the Aseer region, Saudi Arabia, at the end of 2022. The study consisted of three parts namely: Screening for HTN, registration of known hypertensive patients and opinions of representatives of Primary Healthcare Centers (PHCs) on challenges to the implementation of HTN pathway and suggestions for overcoming these challenges. Three Google forms were developed by the investigators to achieve the objectives of this study. The first two forms were completed by doctors and nurses at each PHC and reviewed by the leader of HTN pathway, and the third form completed by a representative of each PHC. SPSS version 26 was used for data management and analysis. Chi-square test was used to determine association between categorical variables; binary logistic regression analysis was performed to determine the correlates of being hypertensive and having good control of HTN. RESULTS: A total of 159,243 individuals were screened for HTN, 55% of whom were females and 94% were Saudis. The prevalence of HTN was 13%; 70% were overweight or obese and 14% had diabetes. The total registered number of patients was 55,628; 50% had good HTN control. Major challenges were inadequate health coaches, care coordinators, laboratory and radiology facilities, lack of coordination with hospitals, and ineffective appointment system. CONCLUSION: This study revealed that the current HTN pathway was successful with regard to screening and registration of patients with HTN. Many challenges need an executive plan with SMART objectives to optimize the care for HTN patients in the region.

5.
Polymers (Basel) ; 14(2)2022 Jan 11.
Article in English | MEDLINE | ID: mdl-35054684

ABSTRACT

The aim of this study was to evaluate the properties of new dental formulations containing eugenyl-2-hydroxypropyl methacrylate (EgGMA) monomer, as restorative dental material, in terms of their degree of photopolymerization and cytotoxicity. The target model composites (TBEg0, TBEg2.5, and TBEg5) were prepared by mixing 35% organic matrix (TEGDMA/BisGMA (50/50 wt%) of which 0, 2.5, and 5 wt%, respectively, were replaced with EgGMA monomer) with 65% filler (silanized hydroxyapatite (HA)/zinc oxide (ZnO2), 4:3 by weight). The vinylic double-bond conversion (DC) after light-curing was studied using Fourier transform infrared technique whereas cell viability was in vitro tested using primary human gingival fibroblasts cells over 7 days by means of AlamarBlue colorimetric assay. The obtained data were statistically analyzed using ANOVA and Tukey post-hoc tests. The results revealed no significant difference in DC between TBEg2.5 (66.49%) and control (TBEg0; 68.74%), whereas both differ significantly with TBEg5, likely due to the inhibitory effect of eugenol moiety at high concentration. The cell viability test indicated that all the composites are biocompatible. No significant difference was counted between TBEg2.5 and TBEg5, however, both differed significantly from the control (TBEg0). Thus, even though its apparent negative effect on polymerization, EgGMA is potentially safer than bisphenol-derived monomers. Such potential properties may encourage further investigations on term of EgGMA amount optimization, compatibility with other dental resins, and antimicrobial activity.

6.
Radiol Case Rep ; 16(6): 1574-1579, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33995747

ABSTRACT

Anomalous origin of vertebral arteries is a rare vascular anomaly and mostly discovered as incidental findings during computed tomography angiogram , magnetic resonance angiography or digital subtracted angiogram of the aortic arch and cerebral vessels. Herein, we present an extremely rare case of a 31-year-old female who presented with headache after emotional trauma. A conventional cerebral angiogram showed anomalous origin of the right vertebral artery. This finding was incidentally discovered, and it is of utmost importance for future head and neck endovascular interventions to avoid inadvertent arterial injury.

7.
Transplant Cell Ther ; 27(10): 851.e1-851.e6, 2021 10.
Article in English | MEDLINE | ID: mdl-34126277

ABSTRACT

The recommended therapy for severe aplastic anemia (SAA) in younger patients with a matched sibling donor (MSD) is allogeneic hematopoietic cell transplantation (allo-HCT). A number of conditioning regimens and protocols have been used for these patients. Here we report a homogeneous cohort of SAA patients receiving a uniform transplantation protocol. This study is a retrospective analysis of 82 consecutive patients with SAA who underwent MSD allo-HCT at a single center. The median duration of follow-up for survivors was 100 months, the 10-year overall survival (OS) was 87.5%, and the 10-year event-free survival was 75.3%. The OS was 97.4% for "mobilized" bone marrow (BM) graft recipients and 78.9% for "nonmobilized" BM graft recipients (P = .01. The cumulative incidence of acute graft-versus-host disease (GVHD) was 25.6%, that of chronic GVHD was 27.16%, and that of graft failure was 16.2%. Recipient age ≥30 years and transplantation at >6 months after SAA diagnosis were associated with a increased risk of events. In the presence of a fully matched sibling donor, allo-HCT with a mobilized BM graft and fludarabine-cyclophosphamide conditioning is an efficacious and safe approach. Early transplantation is associated with a better outcome, emphasizing the importance of not delaying transplantation in these patients. Prospective trials are needed to determine the optimal regimen.


Subject(s)
Anemia, Aplastic , Adult , Anemia, Aplastic/therapy , Cyclophosphamide/therapeutic use , Humans , Prospective Studies , Retrospective Studies , Siblings , Vidarabine/analogs & derivatives
8.
Leuk Res Rep ; 14: 100220, 2020.
Article in English | MEDLINE | ID: mdl-32817817

ABSTRACT

Pro-myelocytic acute leukemia (APL) is characterized by the proliferation of cells blocked at promyelocytic stage and ATRA is the choice of initial treatment because of the APL sensitivity to this compound. In this case study we report a 28-year-old man who presented to the Emergency Department with epistaxis, petechial rash, and fever. Laboratory tests revealed the presence of high white blood cell count with 60% blasts and evidence of coagulopathy. The diagnosis was confirmed later as APL. Because of the delayed transfer to the reference center and unavailability of ATRA initial treatment, the patient received isotretinoin, a related compound. The treatment was successfully implemented in the initial management of acute pro-myelocytic leukemia as patient condition improved. isotretinoin could be used as an alternative therapy for ATRA whenever the latter is not available. further research is needed to establish the appropriate doses and to assess the potential risk of differentiation syndromes.

9.
Adv Med Educ Pract ; 11: 781-790, 2020.
Article in English | MEDLINE | ID: mdl-33117050

ABSTRACT

BACKGROUND: Little is known about the impact of diabetes mellitus (DM) continuing education (CE) programs on the knowledge, attitudes, and practices of primary healthcare physicians (PHPs) in the Arab world. Accordingly, we aimed to evaluate the effectiveness of a diabetes CE program on the knowledge, attitudes and practices of PHPs in Aseer region, Saudi Arabia. METHODS: This was a quasi-experimental study using a pre-test and post-test design. PHPs attended a three-day diabetes CE session and completed a standardized questionnaire before and after the training session. Also, their practices were assessed by reviewing the records of patients with DM before the CE program and three months later. RESULTS: A total of 51 PHPs completed the CE program. The sample had a mean (± SD) age of 33.8 ± 6.0 years, and 72.5% were male. The mean knowledge score increased from 14.33 (± 3.37) to 17.61 (± 2.57) (p < 0.001), and the rate of good knowledge increased from 39 (76.5%) before to 51 (100.0%) after (p < 0.001). There was no significant difference in the mean attitude scores before and after the intervention (3.79 vs 3.86; p = 0.10), respectively. Overall, PHPs' practices related to glycosylated hemoglobin estimation (p = 0.004), foot care (p = 0.02), diet (p < 0.001), exercise (p <0.001), and weight assessment (p < 0.001) significantly improved following the intervention. CONCLUSION: The CE program for PHPs was effective in addressing knowledge gap of PHPs and in improving their practices towards quality patient care.

10.
Exp Clin Transplant ; 17(5): 702-705, 2019 10.
Article in English | MEDLINE | ID: mdl-29633931

ABSTRACT

Refractory antiphospholipid syndrome represents a challenge for preventing thrombosis that may occur despite adequate anticoagulation and immunomodulation therapy. Here, we report a 35-year-old male patient who presented with variable venous thromboembolic events. Autologous hematopoietic stem cell transplant was performed after conditioning with cyclophosphamide and antithymocyte globulin. Five years after transplant, the treatment continued to show a successful outcome in preventing new thrombotic events. Autologous hematopoietic stem cell transplant represents a chance for cure from antiphospholipid syndrome despite the paucity of reported data so far.


Subject(s)
Antiphospholipid Syndrome/surgery , Hematopoietic Stem Cell Transplantation , Adult , Humans , Male
11.
Acta Cytol ; 52(2): 169-77, 2008.
Article in English | MEDLINE | ID: mdl-18499989

ABSTRACT

OBJECTIVE: In the current study, we explore the diagnostic parameters and pitfalls in the follow-up of 123 cases of Pap smears diagnosed as high-grade atypical squamous cells (ASC-H) at our institution. STUDY DESIGN: A computer database search was performed from the archives of the Ottawa Hospital Cytopathology Service for cases diagnosed with ASC-H between January 2003 and July 2005. RESULTS: Follow-up of the 123 cases of ASC-H showed high grade squamous intraepithelial lesion (HSIL) in 73 patients (59.4%), low grade squamous intraepithelial lesion (LSIL) in 11 (8.9%), immature squamous metaplasia in 23 (18.7%), reactive squamous cell changes in 12 (9.8%), benign glandular lesions (endocervical atypia, degenerated glandular cells) in 2 (1.6%) and atrophy in 2 (1.6%). In our study, 83 patients were younger than 40 years (67.4%), with biopsy-proven HSIL found in 54 patients (65.1%). The remaining 40 patients (32.6%) were older than 40 years of age, and follow-up biopsies showed HSIL in 19 patients (47.5%). CONCLUSION: In our study, 59.4% of the cases that were diagnosed cytologically as ASC-H were found to have HSIL on subsequent biopsies. This correlation was stronger in patients below the age of 40 years (65.1% vs. 47.5%). The cytopathologic feature most strongly associated with HSIL was the presence of coarse nuclear chromatin (84%).


Subject(s)
Cervix Uteri/pathology , Diagnostic Errors/prevention & control , Neoplasms, Squamous Cell/pathology , Uterine Cervical Dysplasia/pathology , Uterine Cervical Neoplasms/pathology , Adult , Aged , Biopsy , Cell Nucleolus/pathology , Chromatin/pathology , Colposcopy , Cytoplasm/pathology , Female , Follow-Up Studies , Humans , Metaplasia , Middle Aged , Neoplasms, Squamous Cell/classification , Neoplasms, Squamous Cell/genetics , Ontario , Papanicolaou Test , Terminology as Topic , Uterine Cervical Neoplasms/classification , Uterine Cervical Neoplasms/genetics , Vaginal Smears , Uterine Cervical Dysplasia/classification , Uterine Cervical Dysplasia/genetics
12.
Diagn Cytopathol ; 35(2): 91-5, 2007 Feb.
Article in English | MEDLINE | ID: mdl-17230567

ABSTRACT

Positive diagnosis of metastatic prostate adenocarcinoma (PAC) can be made by microscopic examination of the cytologic specimens and immunostaining for prostate-specific antigen (PSA) and prostate acid phosphatase (PAP). Immunohistochemical markers have been known to display negative, weak, or focal staining in poorly differentiated PAC and in patients with prior hormonal and/or radiation therapy. The purpose of this study is to characterize the cytopathology of metastatic PAC as it has not been documented in large series. Fifty cases of metastatic PAC with cytological specimens consisting of 41 fine-needle aspiration biopsies (FNAB), 6 pleural fluid aspirates, and 3 catheterized urine samples were reviewed and correlated with the surgical specimens and the clinical charts. Immunostaining for PSA, PAP, cytokeratin AE1/3, cytokeratin 7 (CK7), cytokeratin 20 (CK20), vimentin, and carcinoembryonic antigen (CEA) was done. Mean patient age was 77 +/- 8 yr; serum PSA, 4.1 +/- 2.3; and primary PAC Gleason score, 8.1 +/- 1.5. Cytologically, the specimens consisted of cell clusters or cell sheets with overlapping uniform hyperchromatic nuclei with or without nucleoli. Twelve cases were not reactive to PSA and PAP and 44 cases displayed negative immunoreactivity to both CK7 and CK20. Carcinoid-like lesions and small cell carcinomas were seen in 4 cases and were misdiagnosed as nonprostatic origin based on the following features: negative immunoreactivity to PSA and PAP with or without positive reactivity to CEA, and different histopathological features when compared with the primary PAC. In addition to the frequency of high-grade PAC, awareness of the negative immunoreactivity to PSA and PAP, the discrepancy in the histopathological patterns between the primary and secondary tumors, especially the frequent neuroendocrine differentiation, are helpful features for the diagnosis of metastases of prostatic origin.


Subject(s)
Adenocarcinoma/pathology , Prostatic Neoplasms/pathology , Adenocarcinoma/secondary , Adenocarcinoma/urine , Aged , Aged, 80 and over , Biopsy, Fine-Needle , Humans , Immunohistochemistry , Male , Middle Aged , Pleural Effusion/pathology , Prostatic Neoplasms/urine
13.
J Biomed Mater Res A ; 105(7): 1876-1886, 2017 Jul.
Article in English | MEDLINE | ID: mdl-28266173

ABSTRACT

Adverse local tissue reactions (ALTR) are the primary cause of failure of metal on metal (MoM) hip implants, and fewer but not negligible number cases of nonmodular metal on polyethylene (MoP) implants. In this study, we analyzed 17 cases of MoP ALTR, and equal number of MoM, by histological observation, cobalt and chromium concentration in serum and synovial fluid and cytokine analysis in ALTR tissues. ALTRs in MoP are highly necrotic, affecting larger areas than MoM ALTRs. Degenerative changes in blood vessels' wall were seen in all MoP ALTRs. The concentration of cobalt and chromium was higher in synovial fluid but lower in serum of MoP patients compared to MoM patients. Elevated concentrations of chemokines were observed in ALTR tissues. We conclude that ALTRs in MoP systems are highly necrotizing lesions that seem to have a similar development to ALTRs in MoM. Alteration of vessels wall seems to have a role in the tissues necrosis, as well as the elevated concentration of cobalt and chromium in synovial fluid of MoP patients. Chemokines may be involved in the pathogenesis of ALTR and constitute possible diagnostic targets. © 2016 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 105A: 1876-1886, 2017.


Subject(s)
Chromium , Coated Materials, Biocompatible/administration & dosage , Cobalt , Hip Prosthesis/adverse effects , Polyethylene/adverse effects , Synovial Fluid/metabolism , Aged , Chromium/pharmacology , Chromium/toxicity , Cobalt/pharmacokinetics , Cobalt/toxicity , Female , Humans , Male
14.
Cardiovasc Pathol ; 14(6): 324-6, 2005.
Article in English | MEDLINE | ID: mdl-16286042

ABSTRACT

An 83-year-old man with chronic lymphocytic leukemia (CLL) for 10 years presented with dyspnea and hypotension. Blood investigations and electrocardiogram were consistent with acute myocardial infarction. The patient deteriorated quickly and died shortly thereafter. At autopsy, there was severe atherosclerosis of the coronary arteries and an inferolateral left ventricular wall myocardial infarct. Microscopy showed that CLL involved the nodes, liver, spleen, bowel, and kidneys. The coronary artery walls were infiltrated with leukemia cells invading the tunica media and the atheromatous plaque. Infiltration of the coronary arteries by CLL is not common and the possible role in coronary syndromes is discussed.


Subject(s)
Coronary Artery Disease/etiology , Coronary Artery Disease/pathology , Leukemia, Lymphocytic, Chronic, B-Cell/complications , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Myocardial Infarction/etiology , Aged, 80 and over , Flow Cytometry , Humans , Immunohistochemistry , Male , Myocardial Infarction/pathology
15.
J Pediatr Surg ; 46(4): 636-639, 2011 Apr.
Article in English | MEDLINE | ID: mdl-21496530

ABSTRACT

PURPOSE: The purpose was to study the impact of Down's syndrome (DS) in the diagnosis, management, and outcomes of patients with Morgagni hernia (MH). METHODS: Twenty-two (22) patients with MH treated at a tertiary center were retrospectively studied for history, findings, associated anomalies, referral diagnoses, hospital admissions, radiological procedures for diagnosis, age at which operated on, operative procedure, complications, and recurrences. Eleven did not have DS (group 1); 11 others had associated DS (group 2). The ages at operation were compared in the 2 groups using the Mann-Whitney test. More than 3 hospital admissions for symptoms and signs relevant to MH before a diagnosis of MH were considered a "delayed diagnosis." RESULTS: Twenty-two patients (7 females, 15 males) aged 3 months to 10 years were seen. They presented with respiratory distress (n = 16), with vomiting (n = 5), with intestinal obstruction (n = 1), by serendipity (n = 2), and with recurrence from another hospital (n = 1). The mean age of group 1 was 14.5 months, and that of group 2 was 29.18 months; the difference was not significant (P = .621). Nine patients of group 2 were "delayed diagnosis" compared with 2 in group 1. Both delays from group 1 had severe associated anomalies. All patients underwent operative correction (17 open and 5 laparoscopic repairs). Two had recurrences, one operated on by the open method by us and another laparoscopically by the Lima technique at another center. Both had DS. Both were reoperated on by the open method. CONCLUSIONS: The diagnosis of MH may be strikingly delayed when associated with DS or other severe congenital anomalies. Morgagni hernia should be strongly considered in patients with DS admitted repeatedly for chest infections. Chest x-rays in 2 planes may avoid misdiagnosis of MH. Both open and laparoscopic methods have proven satisfactory as operative treatment of MH. Recurrences were seen in patients with DS, which may be corrected by laparotomy or laparoscopically. We feel that resecting the sac and approximating the posterior lip of the defect to the anterior abdominal wall, whether in open or laparoscopic methods, may give stronger repairs, which may avoid recurrence.


Subject(s)
Abnormalities, Multiple/diagnosis , Down Syndrome/diagnosis , Early Diagnosis , Laparoscopy/methods , Child , Child, Preschool , Diagnosis, Differential , Diagnostic Errors , Female , Follow-Up Studies , Hernia, Diaphragmatic/diagnosis , Hernia, Diaphragmatic/surgery , Hernias, Diaphragmatic, Congenital , Humans , Infant , Male , Recurrence , Retrospective Studies , Time Factors
16.
Indian J Pediatr ; 76(5): 489-93, 2009 May.
Article in English | MEDLINE | ID: mdl-19390801

ABSTRACT

OBJECTIVE: Para esophageal hiatal hernia is a rare childhood condition and reported series have had scant number of children which makes diagnosis a challenge. The authors sought to study the presentation and the outcome of treatment of congenital para esophageal hernias (CPEH) over a period of 10 years from a single tertiary care hospital in Saudi Arabia. METHODS: The records of 9 patients presenting between 1997 and 2007, were retrospectively analyzed for demographics, presenting features, referral diagnoses, investigations, management including operative procedures, their outcome and follow-up. RESULTS: Nine patients (3 males and 6 females) aged between 8 days to 34 months were seen. Respiratory distress (n=6), vomiting (n=5) and frequent respiratory tract infections (n=3) were the most common presentations. Cyanosis (n=2), cough and excessive crying were the other important symptoms. The referral diagnoses in these patients included congenital Bochdalek's hernias, lung abscess, bronchogenic cyst, pneumatocoele, bronchiolitis, and pneumonias which reflected a misinterpretation of their clinical findings and chest X-rays. Seven of these patients had other associated congenital anomalies. Three had cardiovascular abnormalities and 2 had lesions of the central nervous system. A pair of siblings had Marfan's syndrome. All the patients had abnormal chest C-rays and an UGS (upper GI series) proved to be diagnostic in 8 patients. The CT scans done in 4 patients corroborated the findings of the UGS. A laparotomy was done on most patients (n=8) which comprised of reduction of the stomach, resection of the hernial sac, tightening of the hiatus and a gastropexy or a gastrostomy. One patient, who underwent thoracotomy died of surgical complications. Two others died of causes unrelated to the surgery. The remaining six operated patients have been followed up for a median of 3.5 years and are doing well. CONCLUSION: CPEH is uncommon in children, presented with respiratory tract symptoms and vomiting, and may be associated with Martan's syndrome. It should be considered in the workup of a child with vomiting or frequent chest infections. Abnormal chest X-rays may indicate the diagnosis and a subsequent UGS, is confirmatory. The present study found the aparotomya good approach for repair of the wide hiatus. A gastropexy and a floppy fundoplication were added to prevent reherniation and post operative reflux though given the small numbers it is not possible to determine the place of either of these procedures. CPEH may be frequently associated with other congenital problems which may impact survival.


Subject(s)
Hernia, Hiatal/congenital , Hernia, Hiatal/surgery , Laparotomy/methods , Thoracotomy/methods , Child, Preschool , Female , Follow-Up Studies , Fundoplication/methods , Gastroplasty/methods , Hernia, Hiatal/diagnostic imaging , Hernia, Hiatal/mortality , Humans , Infant , Infant, Newborn , Male , Postoperative Complications/mortality , Postoperative Complications/physiopathology , Registries , Retrospective Studies , Risk Assessment , Saudi Arabia , Severity of Illness Index , Survival Rate , Time Factors , Tomography, X-Ray Computed , Treatment Outcome
17.
J Pediatr Surg ; 44(5): 888-92, 2009 May.
Article in English | MEDLINE | ID: mdl-19433163

ABSTRACT

BACKGROUND: The "Nuss" procedure for repair of pectus excavatum is performed with increasing frequency worldwide. We describe a technique of performing relaxing or "kerf" incisions along the cartilaginous ribs at the point of maximal chest wall concavity to facilitate retrosternal dissection and to reduce chest wall tension. The effects on operative parameters and long-term changes in cardiopulmonary function and appearance were evaluated prospectively. METHODS: Thirty patients underwent a Nuss procedure for the period from 2001 to 2004. Fifteen patients underwent a modified Nuss (MN) with sternocostal "relaxing" incisions, and these were compared to 15 patients undergoing a standard Nuss (SN). Data were prospectively gathered and included the number of relaxing incisions, bar number, blood loss, duration of epidural analgesia, number of days to discharge, and preoperative and postoperative cardiopulmonary function measures. RESULTS: The average Haller index of the SN group was 3.9 +/- 0.7 vs MN group index of 4.6 +/- 1.6 (P < .05). The median number of relaxing incisions in the study group was 4.0 +/- 0.7 (range 3 - 8). There were no significant differences between the study groups in any of the operative parameters analyzed, including blood loss, days of epidural requirements or days to reach functional independence. Nor were there differences in the improvement in subjective appearance, complications, pulmonary function, cardiac output at rest, oxygen transport or minute ventilation at maximal exercise. Subjectively, bar positioning and the intraoperative visualization was easier with the relaxing incisions. CONCLUSIONS: The addition of sternocostal relaxing incisions to the standard Nuss procedure appears to facilitate retrosternal dissection and bar placement, but no changes in long-term function or cosmesis were noted. The use of relaxing incisions appears to be safe and may facilitate operative visualization of retrosternal structures.


Subject(s)
Funnel Chest/surgery , Thoracotomy/methods , Adolescent , Analgesia, Epidural , Blood Loss, Surgical , Cartilage/surgery , Child , Esthetics , Exercise Tolerance , Female , Heart Function Tests , Humans , Length of Stay/statistics & numerical data , Male , Minimally Invasive Surgical Procedures , Pain, Postoperative/drug therapy , Prospective Studies , Prostheses and Implants , Respiratory Function Tests , Thoracoscopy , Young Adult
18.
J Pediatr Surg ; 42(10): 1780-4, 2007 Oct.
Article in English | MEDLINE | ID: mdl-17923216

ABSTRACT

Surgery for pheochromocytoma differs from that of other tumors owing to the potential release of catecholamines, which may lead to severe intraoperative hemodynamic changes. The present standard of care for resection of adrenal pheochromocytoma has become a laparoscopic approach for surgical excision. Extra-adrenal pheochromocytoma is a very rare entity, especially in the pediatric age group; the utility of the laparoscopic approach is not established in this population. We present a case report of a child with hormonally active extra-adrenal pheochromocytoma originating in the organ of Zuckerkandl that was resected laparoscopically. We found the laparoscopic approach gave excellent exposure, allowing for proper identification of the tumor's origin and its relation to surrounding structures; a complete resection with excellent control of the feeding blood vessels was performed. Herein we present the details of this case and a review of the relevant literature. After our initial experience we can recommend laparoscopic exploration for similar cases of suspected extra-adrenal pheochromocytoma as an appropriate tool to identify extension of the disease and estimate resectability. Proper patient preparation and monitoring are critical for success.


Subject(s)
Abdominal Neoplasms/surgery , Laparoscopy/methods , Para-Aortic Bodies/pathology , Pheochromocytoma/surgery , Abdominal Neoplasms/blood supply , Abdominal Neoplasms/diagnosis , Abdominal Neoplasms/metabolism , Adolescent , Adrenergic alpha-Antagonists/administration & dosage , Catecholamines/metabolism , Female , Humans , Hypertension/etiology , Hypertension/prevention & control , Intraoperative Complications/prevention & control , Para-Aortic Bodies/metabolism , Pheochromocytoma/blood supply , Pheochromocytoma/diagnosis , Pheochromocytoma/metabolism , Prazosin/administration & dosage , Preanesthetic Medication
19.
J Pediatr Surg ; 40(2): E15-7, 2005 Feb.
Article in English | MEDLINE | ID: mdl-15750910

ABSTRACT

Intrathoracic stomach is a rare and serious congenital abnormality. The anomaly may be complicated by gastric volvulus and can lead to ischemic gastric infarction in the neonate. If diagnosed antenatally, neonatal management can be planned in advance so as to reduce morbidity. This anomaly must be differentiated from the more common congenital diaphragmatic hernia, as associated pulmonary hypoplasia is common in the latter and rare with gastric herniation. We report an infant born to a mother with Marfan's syndrome with the antenatal diagnosis of intrathoracic stomach. The ultrasound and magnetic resonance imaging features of this congenital abnormality are described. A review of the literature would indicate that this is the first case report of gastric volvulus diagnosed in utero.


Subject(s)
Hernia, Hiatal/diagnostic imaging , Marfan Syndrome/complications , Stomach Volvulus/diagnostic imaging , Thorax/abnormalities , Ultrasonography, Prenatal , Adult , Female , Gastrostomy , Hernia, Hiatal/congenital , Hernia, Hiatal/etiology , Humans , Infant, Newborn , Magnetic Resonance Imaging , Pregnancy , Pregnancy Complications , Stomach Volvulus/congenital , Stomach Volvulus/etiology , Thorax/diagnostic imaging
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