Treatment modalities for granulomatous mastitis, seeking the most appropriate treatment with the least recurrence rate: a systematic review and meta-analysis.

BACKGROUND: Granulomatous mastitis (GM) is a rare, benign, inflammatory breast disease with an unknown etiology that predominantly affects women of reproductive age. The definitive treatment of GM is currently controversial; an appropriate therapeutic strategy has yet to be identified, and the disease's high recurrence rate remains. This study aims to determine the recurrence rate for each GM treatment strategy to identify the most appropriate treatment modality. METHODS: The search for relevant articles was undertaken using three international databases, including Medline, Scopus, and Web of Science. Articles published in English until the end of 2021 evaluating the recurrence rate of GM were included. Using Stata 13.0, the pooled incidence and 95% confidence interval (CI) for the recurrence rate were determined. RESULTS: Sixty-five eligible studies were included in our study. The recurrence rates of systemic steroid use, topical steroid use, antibiotic use, methotrexate use, observation, drainage, excision, antibiotic use and surgery, steroid use and surgery, antibiotic and steroid use, methotrexate and steroid use were 24% (95% CI: 21-27%), 11% (95% CI: 6-21%), 18% (95% CI: 14-22%), 13% (95% CI: 7-22%), 11% (95% CI: 7-17%), 65% (95% CI: 50-78%), 13% (95% CI: 10-16%), 23% (95% CI: 14-36%), 7% (95% CI: 5-11%), 11% (95% CI: 6-18%), and 4% (95% CI: 2-8%), respectively. Drainage had the highest recurrence rate, while combined methotrexate and steroid treatment had the lowest rate. CONCLUSION: The optimal treatment strategy for GM depends on the disease's severity, consequences, and the patient's features. The study results indicate that combination therapy is preferable for minimizing the risk of relapse and reducing treatment complications.

Acute fatal ventricular arrhythmia induced by severe hyperkalemia in a toddler with decompensated methylmalonic acidemia.

BACKGROUND: Methylmalonic acidemia is a very rare genetic metabolic disease. Patients with isolated methylmalonic acidemia typically present with acute alterations of consciousness, failure to thrive, anorexia, vomiting, respiratory distress, and muscular hypotonia. Despite the evidence-based management, affected individuals experience significant morbidity and mortality. Hyperkalemia is one of the unusual complications of methylmalonic acidemia. CASE PRESENTATION: In this paper, we describe a 4-year-old Persian boy with methylmalonic acidemia who developed life-threatening arrhythmia following severe hyperkalemia and metabolic acidosis. Emergent management of the condition was successfully carried out, and the rhythm changed to normal sinus rhythm by effectively reducing the serum potassium level. We discuss the possible etiology of this lethal condition and describe its management on the basis of the available evidence. CONCLUSION: During metabolic decompensation in methylmalonic acidemia, frequent blood gas and electrolyte testing to prescribe and adjust therapy and annual echocardiogram and electrocardiogram screening are essential.

The clinical outcomes of xenografts in the treatment of burn patients: a systematic review and meta-analysis.

BACKGROUND: Although autografts are not feasible in patients with extensive burn wounds, allografts and xenografts can be used for temporary coverage. In this systematic review and meta-analysis, we compared the outcomes of xenografts and the standard treatment of burn wounds. METHODS: International online databases were searched for English articles comparing xenografts with routine treatment in the burn patients. The random-effects model was used to estimate standardized mean differences (SMD) or odds ratios (OR) with a 95% confidence interval (CI). RESULTS: From a total of 7144 records, 14 studies were included in our review after screening by title and abstracts followed by full-texts. No significant difference in hospital stays was found between the mammalian xenografts and control groups (SMD [95% CI] = - 0.18 [- 0.54-0.18]). The mean number of dressing changes was significantly lower in both mammalian xenografts compared to the controls (SMD [95% CI] = - 1.01 [- 1.61-- 0.41]) and fish xenografts compared to controls (SMD [95% CI] = - 6.16 [- 7.65-- 4.66]). In the fish xenografts, re-epithelialization time was significantly lower compared to controls (SMD [95% CI] = - 1.18 [- 2.23-- 0.14]). CONCLUSIONS: Xenografts showed a significantly lower number of dressing changes and fish xenografts showed significant benefit in re-epithelialization compared to routine treatment. The beneficial results of xenografts suggest further research in the use of different types of xenografts in patients with extensive burn.

Bochdalek hernia presenting as recurrent fever and bicytopenia in a pediatric patient: A rare case report.

Bochdalek hernia is a rare condition characterized by the displacement of abdominal contents into the thoracic cavity. Due to the nonspecific nature of symptoms, prompt diagnosis, and management by emergency care providers can be challenging. Treatment of a Bochdalek hernia typically involves the reduction in the herniated contents back into the abdominal cavity. In this case report, we present the case of a 1-year-old girl who presented to the emergency department with a fever and bicytopenia. Further evaluation revealed a Bochdalek hernia, which was successfully managed with surgical intervention. This case highlights the importance of considering a Bochdalek hernia in the differential diagnosis of patients presenting with recurrent nonspecific symptoms (fever and bicytopenia).

Idiopathic granulomatous mastitis: A case report and literature review.

Key Clinical Message: Idiopathic granulomatous mastitis (IGM) is a challenging chronic inflammatory disease in diagnosis with unknown etiology. Although the most appropriate treatment protocol has not yet been identified, prednisolone was used in our patient as an effective and practical choice in the treatment of IGM. Abstract: Idiopathic granulomatous mastitis (IGM) is a chronic inflammatory disease of the breast and mimics disorders such as breast cancer and breast abscess. Due to the uncommon of this disease, there is no definitive etiology, or treatment. A 38-year-old woman presented with a 3-week history of painful right retro-areolar mass. She had no history of breast trauma and a family history of breast cancer. She had a history of breastfeeding her second child for 12 months in the past year. Diagnostic tests and investigations led to the IGM diagnosis. Therefore, the patient was successfully treated with a course of corticosteroids, but after 2 months, during treatment, she developed Brucellosis. Despite the patient's Brucella infection and treatment with anti-Brucella drugs, prednisolone as an anti-inflammatory corticosteroid therapy was influential in the treatment of IGM.

An HIV-positive woman with massive brain lesion due to toxoplasmosis: A case report.

Key Clinical Message: Toxoplasmosis-related huge brain lesions may require decompressive craniectomy and lesion excision to avoid brain damage. In this situation, injectable cotrimoxazole is a better choice for treatment. Abstract: Toxoplasma gondii is a worldly distributed obligate intracellular protozoa. Toxoplasmosis is a prevalent opportunistic infection in HIV-infected people, but it was rarely recorded prior to the identification of HIV infection. Here, we report a toxoplasmosis brain lesion in an Iranian HIV-positive patient. A 45-year-old woman with a complaint of malaise was referred to the Valiasr Hospital in Arak city. In her past clinical history, the patient had a history of anemia, deep vein thrombosis (DVT), and positive HIV. The patient was informed of the diagnosis of massive brain toxoplasmosis as a definite diagnosis. The patient was then taken to the operating room for a left decompressive craniectomy, during which the ensuing brain lesion was excised. After a few days, she was discharged from the hospital in good condition and without any complications.

Comparison of child abuse history in patients with and without overactive bladder: a case-control study.

Child abuse is a major global concern in terms of healthcare and social welfare. Child abuse is associated with numerous physical and mental health issues, including anxiety and depression. Overactive bladder (OAB) is a bladder storage functional disease defined by urine urgency with or without urge incontinence and is frequently accompanied by frequency and nocturia. This disorder's origin is not entirely understood. Since OAB can be caused by problems of nervous system maturation or behavioural disorders, its correlation with child maltreatment is possible. Objective: This study aimed to compare the occurrence of maltreatment in children with OAB to healthy children referred to Amirkabir hospital, Arak. Method: This study included 100 children with overactive bladder and 100 healthy children without overactive bladder (ages 5-12 years) as case and control groups, respectively. Children referred to paediatric clinic at Amirkabir hospital in Arak, were selected as participants. Child abuse domains including psychological/emotional, physical, and neglect were diagnosed using a standardized child abuse questionnaire answered by the children. Data were analyzed by SPSS version χ2 test, t-test, and Pearson's χ2 test. Results: The Prevalence of child maltreatment was significantly greater in the case group (31 cases) than in the control group (12 cases) (P<0.0001). The psychological/emotional domain of child abuse was observed in 19 case group participants and 4 control group participants (P=0.001), and the physical domain was observed in 29 case group participants and 11 control group participants (P<0.0001). Despite this considerable difference, 10 and 8 children in the case and control groups, respectively, scored positively for the neglect domain (P=0.112). Conclusion: Child abuse is considerably more common in children with OAB than in healthy children, especially in the psycho-emotional and physical domains, and it is possible to prevent and treat this condition by notifying parents. Children with OAB should also be subjected to child abuse screening.

Adolescence Onset Primary Coenzyme Q10 Deficiency With Rare CoQ8A Gene Mutation: A Case Report and Review of Literature.

Background: Primary deficiency of coenzyme Q10 deficiency-4 (CoQ10D4) is a heterogeneous disorder affecting different age groups. The main clinical manifestation consists of cerebellar ataxia, exercise intolerance, and dystonia. Case report: We provide a case of adolescence-onset ataxia, head tremor, and proximal muscle weakness accompanied by psychiatric features and abnormal serum urea (49.4 mg/dL), lactate (7.5 mmol/L), and CoQ10 level (0.4 µg/mL). Brain-MRI demonstrated cerebellar atrophy, thinning of the corpus callosum, and loss of white matter. Whole exome sequencing showed a homozygous missense mutation (c.911C>T; p.A304V) in CoQ8A gene which is a rare mutation and responsible variant of CoQ10D4. After supplementary treatment with CoQ10 50 mg/twice a day for 2 months the clinical symptoms improved. Conclusion: These observations highlight the significance of the early diagnosis of potentially treatable CoQ8A mutation as well as patient education and follow-up. Our findings widen the spectrum of CoQ8A phenotypic features so that clinicians be familiar with the disease not only in severe childhood-onset ataxia but also in adolescence with accompanying psychiatric problems.

HAV-induced acalculous cholecystitis: A case report and literature review.

Hepatitis A virus (HAV) has some life-threatening extrahepatic complications, such as acute acalculous cholecystitis (AAC). We present HAV-induced AAC in a young female, based on clinical, laboratory, and imaging findings, and conduct a literature review. The patient became irritable, which progressed to lethargy, as well as a significant decline in liver function, indicating acute liver failure (ALF). She was immediately managed in the intensive care unit with close airway and hemodynamic monitoring after being diagnosed with ALF (ICU). The patient's condition was improving, despite only close monitoring and supportive treatment with ursodeoxycholic acid (UDCA) and N-acetyl cysteine (NAC).

A Rare Case of Cerebrotendinous Xanthomatosis Associated With a Mutation on COG8 Gene.

Cerebrotendinous xanthomatosis (CTX) is a rare hereditary disease described by a mutation in the CYP27A1 gene, which encodes the sterol 27-hydroxylase enzyme involved in the synthesis of bile acid. Accumulation of cholesterol and its metabolite, cholestanol, in multiple body organs causes the symptoms of this disease. In addition, a mutation in the COG8 gene, which encodes a subunit of conserved oligomeric Golgi (COG) complex, causes another rare disorder attributed to type IIh of congenital disorder of glycosylation (CDG). We described a rare case of CTX disorder associated with a mutation on COG8 gene, which presented by unusual symptoms.

Streptococcus Viridans meningitis in an immunocompetent child: A case report.

In bacterial meningitis, the Viridans streptococci group is not considered a prevalent pathogen. In contrast, the S. viridans group may cause endocarditis and fatal infections in immunocompromised children and adults. We report a 5-year-old immunocompetent boy with signs of meningitis. The CSF tested positive for meningitis with streptococcus viridans.

A rare case of an immunocompetent patient with isolated pulmonary mucormycosis.

As one of the most lethal infections caused by fungi, pulmonary mucormycosis usually affects patients who are immunocompromised. Isolated pulmonary mucormycosis in immunocompetent patients is very rare.This report describes a 65-year-old immunocompetent man, who was first treated with antibiotics after being diagnosed with a lung infection, then after the lack of response to treatment, he was diagnosed with mucormycosis in further investigations. Finally, the patient died due to a lack of response to treatment. Despite its rarity, it is important to consider other differential diagnoses such as pulmonary mucormycosis if the pneumonic process does not respond to medical treatment.

Evaluation of the association between asthma and non-neurogenic urinary incontinence in children; a case-control study.

BACKGROUND: Asthma is the most common chronic disease in children. Asthma can lead to sleep disorders and psychiatric issues, which are often accompanied by urinary incontinence in children. Furthermore, several studies have shown a relationship between allergic diseases and urinary incontinence. This study aims to examine the association between asthma and non-neurogenic urinary incontinence. MATERIALS AND METHODS: This case-control study included 314 children over three years old referred to Amir Kabir Hospital; 157 with asthma and 157 without asthma. After explaining each urinary disorder in accordace with the International Children's Continence Society's definitions, parents and children were asked about their presence. The disorders included monosymptomatic nocturnal enuresis(MNE), nonmonosymptomatic nocturnal enuresis (NMNE), vaginal reflux (VR), pollakiuria, infrequent voiding, giggle incontinence (GI), and overactive bladder (OAB). The analysis was performed using Stata 16. RESULTS: The average age of the children was 8.19 ± 3.15 years. Patients with asthma (p = 0.0001) and GI (p = 0.027) had a considerably lower average age than patients without these disorders. Asthma and urinary incontinence, including NMNE, Infrequent voiding, and OAB, were significantly correlated (p = 0.017, 0.013, and 0.0001, respectively). Moreover, the association between MNE and asthma was significant in males (p = 0.047). CONCLUSION: Due to the relationship between asthma and urinary incontinence, children with asthma must be evaluated for the presence of urinary disorders and, if present, receive the proper treatment in order to improve their quality of life.

A novel variant of Paganini-Miozzo syndrome: a case report.

Paganini-Miozzo syndrome (MRXSPM) is a rare neurogenetic disorder that is transmitted by X-linked recessive inheritance. This is the third case reported of this disease in the world with a novel variant. A boy was referred due to the absence of neck holding and hand tremors. The examinations found facial anomalies. The brain magnetic resonance imaging (MRI) showed cerebral atrophy and diffused white matter, and irregularities were seen in his electroencephalogram (EEG). The echocardiography revealed a mid-muscular ventricular septal defect. A whole exome sequencing (WES) analysis revealed a novel variant (c.979C > T; p.Pro327Ser) of uncertain significance in the HS6ST2 gene indicating Paganini-Miozzo syndrome. The current case provides additional evidence that MRXSPM can be associated with various neurological and cardiac complications. It is essential to rule out other causes, such as metabolic and infectious diseases. EEG, MRI and WES analyses can help to make a definitive diagnosis.

An unusual case of a toddler with Canavan disease with frequent intractable seizures: A case report and review of the literature.

Canavan disease is a rare fetal inherited leukodystrophy, caused by accumulation of N-acetyl-aspartate in the brain. Here, we report a child presented with frequent intractable seizures and visual impairment. A 14-month-old female infant with a complaint of the absence of neck holding and generalized tonic-clonic seizures was referred to our hospital. Macrocephaly, setting sun eyes, tremor, and hypotonia were observed. Funduscopy showed optic atrophy. Our patient's flash visual evoked potential showed blindness. Her brain magnetic resonance imaging showed diffuse white matter in subcortical, basal ganglia, and dorsal pons. Electroencephalography showed diffuse slow and sharp waves. The genetic study detected a hemizygous mutation in the aspartoacylase gene. Our patient was diagnosed with Canavan disease and began anticonvulsant treatment. However, seizures were not under control. Then, her medications were discontinued, and clobazam and primidone were administered. In conclusion, starting clobazam and primidone may help prevent frequently intractable seizures in Canavan disease patients.

Evaluation of Renal Function and Urinalysis in Children With Simple Febrile Convulsions.

Febrile convulsion (FC) is the most prevalent type of seizure in children. Febrile diseases have been associated with alterations in kidney function and urine indices. Therefore, in this study, renal function and urine analysis were examined in children with simple FC. The study comprised children with simple FC who were referred to the Amirkabir hospital in Arak between 2020 and 2021. Children were examined for urinalysis and kidney function by assessing the estimated glomerular filtration rate (eGFR). A total of 157 patients with FC were admitted. Hematuria, proteinuria, and pyuria were detected in 8.9%, 1.9%, and 5.1% of cases, respectively. The urine cultures of 2 children were positive. Moreover, it was found that in simple FC, eGFR decreases regardless of gender (P > .05), although kidney function decreases more in children older than 24 months. In conclusion, all children with FC must undergo renal function assessments. Furthermore, urinalysis and urine culture are advised to rule out infection.

Urinary tract infection caused by Edwardsiella tarda: a report of the first case in Iran.

BCKGROUND: Edwardsiella tarda, an Enterobacteriaceae family member, is prevalent in different aquatic settings and rarely infects humans. As a result of eating raw or undercooked seafood, humans become infected through their intestinal tracts. Extraintestinal infections have been reported infrequently, mostly in immunocompromised and chronically ill patients. CASE PRESENTATION: Our report describes a case of urinary tract infection caused by E. tarda in a 4-year-old female patient with a history of urinary tract infection and a complaint of fever, dysuria, and frequency. E. tarda was identified as the pathogen isolated from the urine culture. The patient's symptoms were alleviated after receiving ceftriaxone and then nitrofurantoin. CONCLUSION: This case demonstrates that even in immunocompetent patients, E. tarda can infect extraintestinal organs, including urinary tract. Our patient represents the first case of E. tarda infection in Iran and due to the fact that this pathogen is transmitted by aquatic animals, there is a possibility of infecting more aquatic animals and humans in Iran; therefore, the necessary precautions should be taken.

Cerebrovascular accident in a child with precursor B-cell acute lymphoblastic leukemia and coronavirus disease 2019: a case report.

BACKGROUND: Coronavirus disease 2019 can lead to rare but severe and life-threatening diseases in susceptible high-risk populations, including patients with immunodeficiency. A rare event in this report is stroke following COVID-19 disease in a patient with an immunocompromised background due to leukemia and anti-cancer treatments. CASE PRESENTATION: A 6-year-old iranian girl with precursor B-cell leukemia receiving vincristine therapy presented with fever and absolute neutrophil count < 500. Her severe acute respiratory syndrome coronavirus 2 polymerase chain reaction test was positive. During hospitalization, she had abrupt onset tachypnea, reduced O2 saturation, and generalized tonic-clonic seizures treated with phenytoin and levetiracetam. Right parietal lobe ischemia was found on a brain computed tomography scan, and the cerebrospinal fluid polymerase chain reaction test was positive for severe acute respiratory syndrome coronavirus 2. Several days later, she developed lower extremity paralysis and speech impairment, so speech therapy and physiotherapy were initiated. The patient also received dexamethasone, mannitol, heparin, and remdesivir. She was discharged with enoxaparin and levetiracetam. Chemotherapy resumed 2 weeks following discharge. Her speech and walking improved after 10 months of follow-up, and bone marrow aspiration showed total remission. CONCLUSION: Owing to the link between coronavirus disease 2019 and hematologic cancers with hypercoagulopathy and the tendency of patients with leukemia to have coronavirus disease 2019 complications, children with leukemia as well as suspected coronavirus disease 2019 must be hospitalized to prevent blood clot formation.

Post-COVID-19 splenic infarction in a patient with chronic atrial fibrillation: A case report.

We describe splenic infarction (SI), an infrequent condition, in an 82-year-old COVID-19 patient with chronic atrial fibrillation (AF). COVID-19 may cause thrombosis, and AF is a predisposing factor for splenic infarction. Suspicion of SI may be warranted in COVID-19 patients with abdominal pain, especially if a predisposing factor exists.