A Rare Cause of Rectovaginal Fistula in Early Infancy: It is in the Genes!

Acquired Rectovaginal Fistula (RVF) is rare in infants. Interleukin10/ Interleukin 10 receptor deficiencies are monogenic disorders presenting as aggressive forms of infantile onset inflammatory bowel disease with perianal abscess and fistula. Genetic studies assist in confirming the diagnosis. We present a two month old infant with rectovaginal fistula, severe colitis, failure to thrive and recurrent infections in whom colonoscopy revealed irregular colonic ulcers, and genetic studies confirmed an IL10RB mutation. Hematopoietic Stem cell transplantation is the definitive therapy for this disorder which the child underwent. We report this infant with an acquired RVF with extraintestinal features due to IL10RB mutation to highlight the importance of thinking beyond the local anatomy and looking into the genetic domain.

Socioeconomic Burden of Rising Methamphetamine-Associated Heart Failure Hospitalizations in California From 2008 to 2018.

BACKGROUND: Methamphetamine-associated cardiomyopathy/heart failure (MethHF) is an increasingly recognized disease entity in the context of a rising methamphetamine (meth) epidemic that most severely impacts the western United States. Using heart failure (HF) hospitalization data from the Office of Statewide Health Planning and Development, this study aimed to assess trend and disease burden of MethHF in California. METHODS: Adult patients (≥18 years old) with HF as primary hospitalization diagnosis between 2008 and 2018 were included in this study. The association with Meth (MethHF) and those without (non-MethHF) were determined by meth-related International Classification of Diseases-based secondary diagnoses. Statistical significance of trends in age-adjusted rates of hospitalization per 100 000 adults were evaluated using nonparametric analysis. RESULTS: Between 2008 and 2018, 1 033 076 HF hospitalizations were identified: 42 565 were MethHF (4.12%) and 990 511 (95.88%) were non-MethHF. Age-adjusted MethHF hospitalizations per 100 000 increased by 585% from 4.1 in 2008 to 28.1 in 2018, while non-MethHF hospitalizations decreased by 6.0% from 342.3 in 2008 to 321.6 in 2018. The rate of MethHF hospitalization increase more than doubled that of a negative control group with urinary tract infection and meth-related secondary diagnoses (7.82-fold versus 3.48-fold, P<0.001). Annual inflation-adjusted hospitalization charges because of MethHF increased by 840% from $41.5 million in 2008 to $390.2 million in 2018, as compared with an 82% increase for all HF hospitalization from $3.503 billion to $6.376 billion. Patients with MethHF were significantly younger (49.64±10.06 versus 72.20±14.97 years old, P<0.001), predominantly male (79.1% versus 52.4%, P<0.001), with lower Charlson Comorbidity Index, yet they had longer length of stay, more hospitalizations per patient, and more procedures performed during their stays. CONCLUSIONS: MethHF hospitalizations increased sharply during the study period and contributed significantly to the HF hospitalization burden in California. This emerging HF phenotype, which engenders considerable financial and societal costs, calls for an urgent and concerted public health response to contain its spread.

A case report of a child with probable drug resistant tuberculous pericarditis with a review of challenges involved in diagnosis, treatment and follow up of children with DR-TB pericarditis.

BACKGROUND: There are unique challenges in the diagnosis and management of multi drug resistant tuberculosis (MDR-TB) in children. It is difficult to obtain confirmatory microbiological diagnosis in TB pericarditis. It is essential to differentiate between drug sensitive and drug resistant forms of TB as it has a major bearing on the regimen used, and inappropriate TB treatment combined with steroid use for pericarditis can lead to deterioration. With lack of samples, the treatment decision relies on the drug resistance pattern of the close contact if available. Therapeutic challenges of MDR-TB management in a child involve use of toxic drugs that need to be judiciously handled. We report a 2 years 4 months old male child who was diagnosed with TB pericarditis and treated based on the resistance pattern of his mother who was on treatment for pulmonary MDR-TB. CASE PRESENTATION: This 2 years 4 months old male child was diagnosed with TB involving his pericardium. Getting him started on an appropriate regimen was delayed due to the difficulty in establishing microbiological confirmation and drug susceptibility. He was commenced on a regimen based on his mother's drug resistance pattern and required surgery due to cardiac failure during the course of his treatment. He successfully completed 2 years of therapy. CONCLUSIONS: This child's case demonstrates that despite unique challenges in diagnosis and management of drug resistant extra pulmonary tuberculosis in children, treatment of even complex forms can be successful. The need for high suspicion of MDR-TB, especially when there is close contact with pulmonary TB, careful design of an effective regimen that is tolerated by the child, indications for invasive surgical management of pericarditis, appropriate follow-up and management of adverse effects are emphasised.

Life-threatening mesenchymal hamartoma of the chest wall in a neonate.

Mesenchymal hamartomas of the chest wall are unusual tumours diagnosed in neonates. They mostly resolve spontaneously hence conservative management has been advocated. Some compress vital structures in the thoracic cavity or bleed warranting surgical intervention. We present a neonate with mesenchymal hamartoma of the chest wall presenting as unilateral multifocal lesions with life threatening complications. He responded well to surgical intervention and was successfully discharged.

Challenges in a unique presentation of congenital dengue with congenital heart disease.

Vertical transmission of dengue has been documented and is associated with diverse presentations in newborns. Care of such babies requires meticulous support to maintain homeostasis until the baby recovers. We present a neonate diagnosed with congenital dengue, born to a mother diagnosed with dengue on the second day after delivery. Baby was diagnosed by serological tests, underwent appropriate management in neonatal intensive care but was noted to have had coexistent persistent ductus arteriosus and pulmonary hypertension complicating pneumonia and seizures. Management of the baby was complicated by persistent haematological derangements caused by dengue and haemodynamic alterations caused by the heart disease.

Clinical Characteristics and Outcome of Methamphetamine-Associated Pulmonary Arterial Hypertension and Dilated Cardiomyopathy.

OBJECTIVES: This study sought to characterize patients with methamphetamine-associated pulmonary arterial hypertension (MA-PAH) and cardiomyopathy (MA-CMP), to compare with MA controls (MA-CTL), users with structurally normal hearts, with the aim of identifying risk factors for these conditions. BACKGROUND: MA-PAH and MA-CMP are 2 poorly understood cardiac complications in MA users. METHODS: We retrospectively studied the clinical characteristics and outcomes of 50 MA-PAH, 296 MA-CMP, and 356 MA-CTL patients, whom we evaluated between 2010 and 2017. RESULTS: After a median follow-up of 20.0 months (interquartile range [IQR]: 7.6 to 42.6 months), all-cause mortality was 18.0% for MA-PAH, 15.2% for MA-CMP, and 4.5% for MA-CTL group (p < 0.001). More women (58%) were in the MA-PAH group than in the MA-CMP (14%; p < 0.001) and MA-CTL (42%; p = 0.028) groups, whereas the MA-CMP group was predominantly male (86% vs. 58% in the MA-CTL group; p < 0.001). More MA-CMP patients had hypertension (p < 0.001) or alcoholism (p < 0.001) than MA-CTL patients. Logistic regression analyses identified male sex, alcoholism, and hypertension as independent factors associated with MA-CMP with the following respective adjusted odds ratios (OR) of 3.791 (95% confidence interval [CI]: 2.508 to 5.730), OR of 2.959 (95% CI: 2.084 to 4.203), and OR of 2.111 (95% CI: 1.486 to 2.999), whereas female sex was the only factor associated with MA-PAH. CONCLUSIONS: Both MA-PAH and MA-CMP patients carried significant disease burden and mortality risk. Male sex, hypertension, and alcoholism were strongly associated with MA-CMP, whereas female sex and other unknown factors may influence development of MA-PAH. This study adds to the understanding of MA-associated cardiac complications and highlights directions for future investigation.

Peripheral neuropathy in a diabetic child treated with linezolid for multidrug-resistant tuberculosis: a case report and review of the literature.

BACKGROUND: Extensively drug-resistant (XDR) tuberculosis (TB) and multidrug resistant (MDR)-TB with additional resistance to injectable agents or fluoroquinolones are challenging to treat due to lack of available, effective drugs. Linezolid is one of the few drugs that has shown promise in treating these conditions. Long-term linezolid use is associated with toxicities such as peripheral and optic neuropathies. Diabetes mellitus (DM), especially when uncontrolled, can also result in peripheral neuropathy. The global burden of DM is increasing, and DM has been associated with a three-fold increased risk of developing TB disease. TB and DM can be a challenging combination to treat. DM can inhibit the host immune response to tuberculosis infection; and TB and some anti-TB drugs can worsen glycaemic control. A child experiencing neuropathy that is a possible complication of both DM and linezolid used to treat TB has not been reported previously. We report peripheral neuropathy in a 15-year-old boy with type 1 DM, diagnosed with MDR-TB and additional resistance to injectable TB medications. CASE PRESENTATION: The boy was treated with a linezolid-based regimen, but after 8 months developed peripheral neuropathy. It was unclear whether the neuropathy was caused by the DM or the linezolid therapy. He had clinical improvement following cessation of linezolid and was declared cured following 21 months of treatment. Following completion of treatment, nerve conduction studies demonstrated significant improvement in neuropathy. CONCLUSIONS: To the best of our knowledge, this is the first case of peripheral neuropathy reported in a diabetic child on long-term linezolid therapy for tuberculosis. This case study underlines the importance of stringent follow-up for side effects of linezolid, especially when associated with co-morbidity such as DM that increases the chances of adverse effects. The presence of both DM and TB should alert a physician to strive for optimal glycaemic control to minimize the risk of complications as well as optimizing the chances of recovery from TB. Our case report shows the need for close and frequent monitoring for neuropathy to enable early intervention and thereby a favourable outcome in children who may otherwise suffer a long-lasting, debilitating, and painful neuropathy.

Frequency and Associated Clinical Features of Functional Tricuspid Regurgitation in Patients With Chronic Atrial Fibrillation.

Significant functional tricuspid regurgitation (TR) can develop in some but not all patients with chronic atrial fibrillation (AF). This study sought to identify factors likely to be involved in determining the severity of TR in patients with chronic AF. In this retrospective cohort study of adult patients referred for transthoracic echocardiography for evaluation of AF between 2004 and 2015, we identified 170 patients with chronic AF in the absence of structural or known coronary heart disease. Patients were classified into nonsevere (89 patients) versus severe TR (81 patients) groups based on a comprehensive assessment of color Doppler, spectral Doppler, and morphologic parameters of the tricuspid valve and right side of the heart. Patients with severe TR were significantly older (76 ± 10 vs 70 ± 11, p <0.001), with smaller body surface area (1.7 ± 0.3 m2 vs 1.9 ± 0.23 m2, p = 0.001) and with female predominance (percentage of men 30% vs 57%, p <0.001). Although comorbidities, use of cardiovascular medications, and left-sided cardiac parameters were statistically indistinguishable between these 2 groups, right-sided cardiac dimensions, tricuspid valve tethering height, and tricuspid valve tethering area were significantly larger in the severe TR group. A comprehensive multivariate logistic regression model (model 1) identified the age, gender, right ventricular systolic pressure, right atrial volume index, and right ventricular end-diastolic area as independent factors associated with TR severity. A simplified logistic regression model using only clinical factors (model 2) confirmed the age, gender, and right ventricular systolic pressure as clinically relevant factors in relation to TR.

Dying from cardiac tamponade.

BACKGROUND: To determine the causes of cardiac tamponade (CT), focussing especially on haemopericardium (HP), as a terminal mode of death, within a 430,000 rural English population. METHODS: Our hospital mortuary register and, all postmortem reports between 1995 and 2004 inclusive, were interrogated for patients dying of CT or HP. The causes of CT/HP and selected morphological characteristics were then determined. RESULTS: 14,368 postmortems were performed in this period: of these, 461 patients died of CT. Three cases were due to non-haemorrhagic pericardial effusion. HP accounted for the remaining 458 cases of which, five were post-traumatic, 311 followed rupture of an acute myocardial infarction (RAMI), 138 after intra-pericardial rupture of dissecting ascending aortic aneurysms (RD3A) and four were due to miscellaneous causes.HP was more commonly due to RAMI. Men tended to die from RAMI or RD3A earlier than women. RAMI or RD3A were commoner in men <70 yrs, but more frequent in women after this.Two thirds of RAMI were associated with coronary artery thrombosis. Anterior free wall rupture was commonest overall, and in women, but posterior free wall rupture was commoner in men.The volume of intrapericardial blood in RAMI (mean = 440 ml) and RD3A (mean = 498 ml) varied between 150 and 1000 ml: intrapericardial blood volume was greater in men than in women dying from either RAMI or RD3A. CONCLUSION: At postmortem, CT is most often related to HP, attributable to either RAMI or intrapericardial RD3A. Post-traumatic and other causes of CT are infrequent.

Influence of vessel diameter on the efficacy of distal protection devices during saphenous vein graft intervention.

We sought to determine the influence of vessel diameter on the efficacy of distal protection devices during saphenous vein graft intervention. From the Filterwire EX Randomized Evaluation trial, in which patients who underwent saphenous vein graft stenting were randomized to distal protection with the GuardWire or FilterWire EX, outcomes in 572 patients were examined in vessel size tertiles. The 30-day composite incidence of major adverse cardiac events (MACEs) increased with vessel size and was 6.9%, 9.7%, and 14.9% in the smallest, middle, and largest tertiles, respectively (p = 0.04). MACE rates were relatively vessel size independent for the GuardWire but increased steadily with vessel size with the FilterWire EX. In the smallest tertile, MACEs were reduced by 71% with the FilterWire EX compared with the GuardWire (p = 0.05), with the devices showing similar event rates in the other tertiles.