Health-related quality of life outcomes in children after congenital heart disease surgery in low-middle-income countries: a systematic review and meta-analysis.

Introduction: Improved treatments for children with congenital heart disease (CHD) have led to a growing interest in long-term functional outcomes such as health-related quality of life (HRQOL). Studies on HRQOL in children with CHD have yielded contradictory results. In this study, we aimed to perform a systematic review and meta-analysis to analyze the effect of surgery on HRQOL outcomes in children with CHD in low-income and middle-income countries. Methods: A comprehensive search for articles was performed using the Medline (PubMed), Scopus, and Embase databases from their inception to September 5, 2023. Studies reporting QOL outcomes in children <18 years and published in English were included. Results: Of the 1239 records screened, 10 studies, including 1721 participants, were included in the study. The overall QOL was significantly better in the control group than in the children who underwent surgery for CHD (P=0.04, standard mean difference of -0.62, 95% CI: -1.2 to -0.04), and the overall QOL was significantly better in the children with CHD after surgery than before surgery (P=0.05, standard mean difference of -0.56, 95% CI: -1.11 to -0.01). Conclusion: The QOL of children from low-income and middle-income countries who undergo surgery for CHD is significantly poorer than that of controls in all dimensions except the emotional domain. Meanwhile, surgery has the greatest impact on improving the physical domain in children with CHD after surgery. Strategies to improve HRQOL in this subgroup of patients should be further investigated.

Richter supraumbilical hernia managed with invagination: a case report.

Introduction and importance: Richter's hernia is an incarceration of the anti-mesenteric border of a segment of bowel through an abdominal wall defect. It primarily affects elderly individuals but can occur at any age, with a slightly increased incidence in females. The increase in laparoscopic and robotic-assisted procedures has led to a rise in Richter's hernias. Case presentation: A 40-year-old male with a history of laparoscopic cholecystectomy and kidney transplantation presented with a 4-day history of supraumbilical swelling and abdominal pain. The swelling was irreducible and accompanied by mild tenderness, and local signs of inflammation were exhibited. Intraoperatively, a 1.5 cm hernia defect was found, with the sac containing omentum and a portion of bowel segment for which invagination with serosal closure with the Mayo double-breasting technique was done. Clinical discussion: Richter's hernia presents with abdominal discomfort, bloating, nausea, and vomiting, with a notable feature being the delayed onset of symptoms due to its partial involvement of the bowel wall. Diagnosis can be achieved through a computed tomography (CT) scan or intraoperative exploration. Management of Richter hernia is contingent upon the patient's clinical condition, physical examination, and suspicion of strangulation. Conclusion: Diagnosis of Richter's hernia demands higher suspicion, particularly in patients with predisposing factors like a history of minimally invasive surgery. Prompt surgical intervention is crucial for reducing mortality and enhancing prognosis, with invagination alone being adequate if ischaemia is confined and mesh placement is unnecessary.

Hepatic angiomyolipoma masquerading as abdominal pain: A case report.

INTRODUCTION AND IMPORTANCE: Hepatic angiomyolipoma (HAML) is a rare liver tumor composed of blood vessels, smooth muscle, and fat cells. HAML occurs across a wide age range, with symptoms including abdominal discomfort, bloating, and weight loss. Diagnosis is challenging due to varied imaging appearances, but histopathological examination supplemented by immunohistochemical analysis, particularly using HMB-45, is definitive. CASE PRESENTATION: A 33-year-old man presented with a two-year history of right upper quadrant abdominal pain, occasionally relieved with analgesics but worsening over the past month and a half. Examinations revealed a soft, non-distended abdomen with a palpable liver. Laboratory tests, including viral markers and tumor markers were normal. Contrast-enhanced CT revealed a well-defined oval mass in liver segment III with heterogeneous enhancement leading to provisional diagnosis of HAML. The patient underwent a successful en bloc excision with no intraoperative or postoperative complications. CLINICAL DISCUSSION: Surgical resection is recommended for symptomatic cases or inconclusive biopsies, with stringent follow-up necessary due to the potential for recurrence and association with other malignancies. CONCLUSION: HAML may present with prolonged nonspecific abdominal symptoms. CT imaging aids in diagnosing cases with abundant fatty tissue. En bloc tumor excision proves safe and effective in treating symptomatic presentations.

Incidentally discovered intestinal malrotation during evaluation for blunt abdominal trauma: A case report.

INTRODUCTION AND IMPORTANCE: Intestinal malrotation is a congenital abnormality predominantly diagnosed in children, with only a few cases reported in adults. Patients may be incidentally identified during unrelated surgical procedures or postmortem examinations. It is crucial to promptly recognize this condition to prevent severe complications such as bowel ischemia and potential fatality. CASE PRESENTATION: A 40-year-old male presented to the Emergency Department after a child jumped on his abdomen with complaints of acute left upper quadrant abdominal pain progressing to be generalized. Examination showed pallor, abdominal tenderness without guarding or rigidity, and intact bowel sounds. Preoperative diagnostic tools revealed intestinal malrotation confirmed during the laparotomy, prompting the performance of Ladd's procedure to address the malrotation. CLINICAL DISCUSSION: Disruption in the normal embryological development of bowel is the cause of intestinal malrotation. The role of additional surgery especially in patients with asymptomatic disease related to malrotation is debated. CONCLUSION: Intestinal malrotation is rare in adults and often found incidentally during evaluation for unrelated medical conditions. Timely identification and surgical intervention usually result in positive outcomes. Our case underscores the incidental discovery of malrotation during the evaluation of blunt abdominal trauma, treated with Ladd's procedure. This is particularly significant due to geographical constraints associated with the patient's rural origin, as untreated malrotation could lead to complications in future occurrences.

Prevalence and Clinical Types of Tremor in Multiple Sclerosis and its Associated Disability: A Systematic Review.

Objective: To describe the state of literature regarding prevalence, clinical types of tremor in Multiple Sclerosis and associated disability. Background: Tremor has long been recognized as an important symptom of multiple sclerosis. This can be intention and postural tremor that affects the upper limbs. Patients with multiple sclerosis who experience tremor of any severity typically retire early or lose their jobs due to disability. Methods: This systematic review was performed up to September 9, 2022. Article selection was performed by searching the MEDLINE (PubMed) and EMBASE electronic bibliographic databases. The search strategy was not limited by study design but only for articles in the English language. Results: A total of nine full-text articles were included in the analysis. Six studies were cross-sectional studies; one each was a prospective observational study, a case-control study, a community-based cohort. The prevalence of tremor in the multiple sclerosis (MS) population among studies ranged widely, between 12.5% and 68.9%. The presence of severe tremor ranged from 3% to 33%. Younger age was a significant predictor of tremor in two studies. The most common tremor subtype was action tremor. Upper extremities were the most common site involved in the majority of our studies, followed by head and neck. Conclusions: Prevalence of tremor ranged from 12.5% to 68.9% in the MS population with severe tremor being an infrequent complication. Severity of tremor correlated with increasing disability. Upper limb action tremor was the most common with rare occurrences of resting and rubral tremor.

Abdominal Wall Dyskinesia in a Child Presenting as Belly Dancers' Syndrome: A Case Report.

Belly dancer's dyskinesia or syndrome is a rare condition characterized by involuntary, undulating, infrequent diaphragm movements. The etiologies for this disorder include nervous system disorders (peripheral or central), drug-induced, psychological, or idiopathic. This article describes a 10-year-old boy with an underlying psychological stressor who suddenly experienced involuntary abdominal wall movements after salbutamol nebulization. After a detailed history, physical examination, and abdominal ultrasound that revealed rapid rhythmic diaphragm movements, the child was diagnosed with salbutamol-induced belly dancer's dyskinesia with an underlying psychological problem. These movements subsided with medical and psychological therapy for two weeks. Belly dancer's dyskinesia is a complex disorder that is difficult to diagnose but can be managed with medical treatment and psychological counseling alone in a few patients. In contrast, in other cases, surgical intervention may be required. Keywords: case reports; dyskinesias; salbutamol.

Guillain-Barre Syndrome following lower segment caesarean section under spinal anesthesia: A case report.

Symptoms of Guillain-Barre Syndrome (GBS) may be mistaken for typical puerperal changes, delaying diagnosis. Surgery and anesthesia may be triggers for GBS with an overall increase in pro-inflammatory cytokines in the postpartum period. We report a unique case of GBS in the postpartum period who made a good recovery with supportive measures.

Paraparetic Guillain-Barre syndrome: An uncommon diagnosis of acute flaccid paralysis of the lower limbs.

Apart from the usual differentials of transverse myelitis and cord compression, paraparetic GBS should be considered when sudden, flaccid paralysis of the lower limbs occurs, as prompt diagnosis and management can minimize sequel and unnecessary procedures. We do report a case wherein we managed a similar situation without the use of an immunomodulatory therapy.

Wellens Syndrome: An Atypical Presentation With Burning Chest and Epigastric Pain.

Wellens syndrome is a pre-infarction stage due to the critical stenosis of the proximal left anterior descending artery. It is characterized by intermittent chest pain with classical ECG changes. The cardiac biomarker is within the normal limit or only slightly elevated in this condition. Early recognition and cardiac intervention are important to prevent adverse cardiac outcomes. We report this rare case of Wellens syndrome in a 70-year-old male with intermittent chest and epigastric pain associated with belching for five days. The patient presented characteristic T-wave changes, symmetric deeply inverted T-waves, in precordial leads (V2-V4). Cardiac biomarkers, including troponin, were negative. He underwent cardiac catheterization and found a clot in the proximal left anterior descending coronary artery, which required a catheter-directed thrombectomy and drug-eluting stent placement. It is important to recognize this condition early and referred on time from primary health centers to higher centers, especially in developing countries like the Maldives, since delays in transfer may lead to a serious outcome.

Kawasaki Disease Shock Syndrome: A Nine-Year-Old Girl With Atypical Presentation of Kawasaki Disease in Emergency Department.

Kawasaki disease (KD) is an acute rheumatological illness usually affecting children between six months and five years of age. It is a vasculitis syndrome of medium-sized vessels that has typical clinical characteristics such as fever, rash, cervical lymphadenopathy, conjunctivitis, and mucosal changes. However, sometimes, it may present with the features of shock when it is known as Kawasaki disease shock syndrome (KDSS). The actual etiology of this disease is still unknown. The primary treatment of this disease is aspirin and intravenous immunoglobulin (IVIG). The most common and serious complication of KD is cardiac complications which can be avoided by IVIG if given on time. KDSS is the other rare but serious early complication that can be presented to the ED as an initial presenting feature. Early diagnosis of KDSS in the ED and its treatment is very important to prevent early and late complications, including cardiac complications of this disease. Although the usual age group for this disease is under five years, here we have presented a rare case of KDSS in a nine-year-old female child.

Hepatitis B Seroprotection in Pediatric Nephrotic Syndrome.

OBJECTIVE: To study the prevalence of Hepatitis B seroprotection in children (>1 y) with nephrotic syndrome vaccinated against Hepatitis B vaccine as per the Universal Immunization Program schedule (0,6,10,14 wk); to compare the Hepatitis B seroprotection rates and anti-HBs titers among different phenotypes of nephrotic syndrome; to evaluate the association between Hepatitis B seroprotection status and the immunosuppressive agents; and to study the correlation between anti-HBs titres and proteinuria. METHODS: Hepatitis B serology and anti-HBs titers were analyzed in 100 children (age-1-18 y) with different clinical phenotypes of nephrotic syndrome (cases) and 100 healthy controls. RESULTS: The proportion of seroprotected children among the cases and controls was 37% (n=37) and 61% (n=61), respectively (P<0.04). The median (IQR) anti- HBs antibodies titers among the cases was 75 (62.5, 81) mIU/mL and 112 (56, 367) mIU/mL among the controls (P=0.001). The proportion of seroprotected children among the steroid sensitive nephrotic syndrome, steroid-resistant nephrotic syndrome and controls was 40% (n=28), 30% (n=9) and 61% (n=61), respectively (P<0.01). No differences in the anti-HBs titers between children receiving steroids versus steroids along with other immunosuppressants were found. Weak negative correlation was noted between proteinuria and protective titers (r = -0.155; P=0.039). CONCLUSIONS: Children with nephrotic syndrome, in general, and steroid-resistant nephrotic syndrome in particular, show poor seroprotection with Hepatitis B vaccination.

An unusual presentation of early congenital syphilis with annular configuration of blisters resembling "string-of-pearls".

Skin lesions in early congenital syphilis can show considerable variability. A 35-day-old infant presented with rhinitis, desquamation of palms and soles, pseudoparalysis of the right upper limb, and annular lesions with peripheral blistering over the chest and abdomen, resembling a "string-of-pearls." A diagnosis of congenital syphilis was made on the basis of radiologic evaluation and reactive Venereal Disease Research Laboratory (VDRL) in both mother and child. We report this case to highlight the unusual presentation of congenital syphilis with annular configuration of blisters, mimicking a "string-of-pearls."