No abstract

Acute post-streptococcal glomerulonephritis (APSGN) is the major cause of acute glomerulonephritis among children, especially in low- and middle-income countries. APSGN commonly occurs following pharyngitis due to the activation of antibodies and complements proteins against streptococcal antigens through the immune-complex-mediated mechanism. APSGN can be presented as acute nephritic syndrome, nephrotic syndrome, and rapidly progressive glomerulonephritis, or it may be subclinical. The management of APSGN is mainly supportive in nature with fluid restriction, anti-hypertensives, diuretics, and renal replacement therapy with dialysis, when necessary, as the disease is self-limiting. Congestive heart failure, pulmonary edema, and severe hypertension-induced encephalopathy might occur during the acute phase of APSGN due to hypervolemia. APSGN generally has a favorable prognosis with only a small percentage of patients with persistent urinary abnormalities, persistent hypertension, and chronic kidney disease after the acute episode of APSGN. Decreased complement levels, increased C-reactive protein, and hypoalbuminemia are associated with disease severity. Crescent formations on renal biopsy and renal insufficiency on presentation may be the predictors of disease severity and poor outcomes in APSGN in children.

Cardiorenal syndrome (CRS) type 1 is the development of acute kidney injury in patients with acute decompensated heart failure. CRS often results in prolonged hospitalization, a higher rate of rehospitalization, high morbidity, and high mortality. The pathophysiology of CRS is complex and involves hemodynamic changes, neurohormonal activation, hypothalamic-pituitary stress reaction, inflammation, and infection. However, there is limited evidence or guideline in managing CRS type 1, and the established therapeutic strategies mainly target the symptomatic relief of heart failure. This review will discuss the strategies in the management of CRS type 1. Six clinical studies have been included in this review that include different treatment strategies such as nesiritide, dopamine, levosimendan, tolvaptan, dobutamine, and ultrafiltration. Treatment strategies for CRS type 1 are derived based on the current literature. Early recognition and treatment of CRS can improve the outcomes of the patients significantly.

Cytomegalovirus (CMV) is the most common cause of congenital infection, affecting 1% of all live births. Intrauterine infection such as CMV infection is a risk factor for developing cerebral palsy. This study aims to investigate the association between congenital CMV infection and the development of cerebral palsy. A systematic literature search was conducted in PubMed, Web of Science and Ovid SP to identify relevant studies. The quality of studies was assessed using the Newcastle-Ottawa Scale. The random-effect model was used to calculate the pooled prevalence. The generic inverse variance method was used for statistical analysis. A total of 12 studies were included in this systematic review and meta-analysis. The overall pooled prevalence of cerebral palsy among patients diagnosed with congenital CMV infection was 26% (95% confidence interval (CI), 13-40%). The overall pooled prevalence of congenital CMV infection among patients with cerebral palsy was 10.9% (95% CI, 5-16%). Congenital CMV infection was significantly associated with the development of cerebral palsy in children. Routine follow-ups should be offered to screen for cerebral palsy.

No abstract

Rheumatic heart disease (RHD) is damage of the heart valves due to acute rheumatic fever (ARF) which results from the body's autoimmune response to Streptococcus pyogenes (group A Streptococcus bacteria) infection which is a throat infection. Acute rheumatic fever is one of the most important causes of cardiovascular morbidity and mortality in the developing countries although the incidence has decreased in North America, Europe and developed countries over the past 4 to 6 decades. The clinical symptoms of ARF which occurs approximately two to four weeks after S. pyogenes infection. The most common clinical manifestations of ARF are carditis and arthritis, followed by chorea and then subcutaneous nodules and erythema marginatum.

No abstract

Niemann-Pick disease type C (NP-C) is a severe neurovisceral lipid storage disease that results in the accumulation of unesterified cholesterol in lysosomes or endosomes. The clinical presentations of NP-C are variable which include visceral symptoms, neurologic symptoms and psychiatric symptoms. Psychosis is the most common psychiatric manifestation of NP-C and is indistinguishable from a typical psychosis presentation of schizophrenia. The common psychotic presentations in NP-C include visual hallucinations, delusions, auditory hallucinations and thought disorders. Psychosis symptoms are more common in adult or adolescentonset forms compared with pediatric-onset forms. The underlying pathophysiology of psychosis in NP-C is most probably due to dysconnectivity particularly between frontotemporal connectivity and subcortical structures.NP-C sometimes is mistaken for schizophrenia which causes delay in treatment due to lack of awareness and literature review. This review aims to summarize the relevant case reports on psychosis symptoms in NP-C and discuss the genetics and pathophysiology underlying the condition.