diagnosis

Polyneuropathy is a common neurologic condition with an overall prevalence in the general population of about 1%–3%, increasing to roughly 7% among people older than 65 years. Polyneuropathy has many causes, and can present in many different ways; thus, it requires a logical clinical approach for evaluation, diagnosis and management. We review the approach to evaluating a patient with polyneuropathy by highlighting important aspects of the history and neurologic examination. We focus on the role of diagnostic investigations for distal symmetric polyneuropathy (DSP), the most common subtype, and an approach to the symptomatic treatment of painful diabetic polyneuropathy (PDN). We draw on practice based guidelines, meta-analyses and systematic reviews, where
possible, as they represent the highest levels of evidence (Box 1).

Skin lesions of the face, trunk, and extremities are commonly seen in the pediatric population. Although most of these lesions are benign, they can be locally destructive or interfere with normal development. Recognition and diagnosis of these lesions allow for timely workup and referral; treatment, if needed; and facilitation of parental discussions. The purpose of this article is to review common pediatric skin and soft-tissue lesions-or "lumps, bumps, and birthmarks"-to assist with diagnosis, workup, and guidelines for referral to pediatric plastic surgery. [Pediatr Ann. 2023;52(1):e23-e30.].

PRACTICE CHANGER
CONSIDER A “NO-BIOPSY” APPROACH BY EVALUATING SERUM IMMUNOGLOBULIN (IG) A ANTI-TISSUE TRANSGLUTAMINASE (TTG-IGA) ANTIBODY TITERS IN ADULT PATIENTS WHO PRESENT WITH SYMPTOMS CONCERNING FOR CELIAC DISEASE (CD). AN INCREASE OF ≥ 10 TIMES THE UPPER LIMIT OF NORMAL (ULN) FOR TTG-IGA HAS A POSITIVE PREDICTIVE VALUE (PPV) OF ≥ 95% FOR DIAGNOSING CD WHEN COMPARED WITH ESOPHAGOGASTRODUODENOSCOPY (EGD) WITH DUODENAL BIOPSY—THE CURRENT GOLD STANDARD.

Importance: Pulmonary arterial hypertension (PAH) is a subtype of pulmonary hypertension (PH), characterized by pulmonary arterial remodeling. The prevalence of PAH is approximately 10.6 cases per 1 million adults in the US. Untreated, PAH progresses to right heart failure and death.

Observations: Pulmonary hypertension is defined by a mean pulmonary artery pressure greater than 20 mm Hg and is classified into 5 clinical groups based on etiology, pathophysiology, and treatment. Pulmonary arterial hypertension is 1 of the 5 groups of PH and is hemodynamically defined by right heart catheterization demonstrating a mean pulmonary artery pressure greater than 20 mm Hg, a pulmonary artery wedge pressure of 15 mm Hg or lower, and a pulmonary vascular resistance of 3 Wood units or greater. Pulmonary arterial hypertension is further divided into subgroups based on underlying etiology, consisting of idiopathic PAH, heritable PAH, drug- and toxin-associated PAH, pulmonary veno-occlusive disease, PAH in long-term responders to calcium channel blockers, and persistent PH of the newborn, as well as PAH associated with other medical conditions including connective tissue disease, HIV, and congenital heart disease. Early presenting symptoms are nonspecific and typically consist of dyspnea on exertion and fatigue. Currently approved therapy for PAH consists of drugs that enhance the nitric oxide-cyclic guanosine monophosphate biological pathway (sildenafil, tadalafil, or riociguat), prostacyclin pathway agonists (epoprostenol or treprostinil), and endothelin pathway antagonists (bosentan and ambrisentan). With these PAH-specific therapies, 5-year survival has improved from 34% in 1991 to more than 60% in 2015. Current treatment consists of combination drug therapy that targets more than 1 biological pathway, such as the nitric oxide-cyclic guanosine monophosphate and endothelin pathways (eg, ambrisentan and tadalafil), and has shown demonstrable improvement in morbidity and mortality compared with the previous conventional single-pathway targeted monotherapy.

Conclusions and relevance: Pulmonary arterial hypertension affects an estimated 10.6 per 1 million adults in the US and, without treatment, typically progresses to right heart failure and death. First-line therapy with drug combinations that target multiple biological pathways are associated with improved survival.