Portal Hypertension in Children: Investigating Umbilical Catheterization in the Neonatal Period.

Portal cavernoma is a major cause of extrahepatic portal hypertension (EHPH) in children. It is a serious condition, due to the frequency and severity of digestive hemorrhages secondary to the rupture of esophageal varices (EV). Neonatal umbilical catheterization is a significant risk factor for the development of portal vein thrombosis (PVT) and portal hypertension. We report a case of a five-year-old male who presented with upper gastrointestinal (GI) bleeding on ruptured esophageal varices resulting from a portal cavernoma, complicating neonatal umbilical vein catheterization. This case illustrates the risk of severe vascular complications, particularly portal hypertension that can result from neonatal umbilical vein catheterization.

Albinism and Primary Immunodeficiency in Infants: A Case Study of Griscelli Syndrome.

Griscelli syndrome (GS) type II is a rare hereditary disorder characterized by partial albinism, immunodeficiency, and the subsequent development of hemophagocytic syndrome (HPS). Herein, we present a case involving a four-month-old infant admitted to our facility due to a prolonged fever complicated by HPS. The diagnosis of GS type 2 was established based on a constellation of clinical and laboratory findings: consanguinity, familial history of early infectious fatalities, ocular-cutaneous hypopigmentation, characteristic silvery hair sheen, onset of HPS, and notably, the pathognomonic appearance upon microscopic examination of a hair sample. The absence of giant granules within nucleated cells helped exclude Chediak-Higashi syndrome.

Compartment Syndrome: An Uncommon Twist in Childhood Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) also known as rheumatoid purpura is the most common vasculitis in children. This condition affects small blood vessels, predominantly targeting the skin, digestive system, joints, and kidneys. Short-term prognosis mainly depends on abdominal complications, while long-term prognosis is mainly determined by the severity of kidney involvement, which occurs in about 35% of cases. Although uncommon, other organs such as the lungs, heart, or nervous system may also be affected. Compartment syndrome of the hand and forearm is a very rare complication of HSP. To our knowledge, only two cases have been reported in the literature. We describe the case of a four-year-old child who presented with rheumatoid purpura complicated by compartment syndrome of the hand and forearm successfully managed through emergency fasciotomy.