Reduced sodium/proton exchanger NHE3 activity causes congenital sodium diarrhea.

Congenital sodium diarrhea (CSD) refers to an intractable diarrhea of intrauterine onset with high fecal sodium loss. CSD is clinically and genetically heterogeneous. Syndromic CSD is caused by SPINT2 mutations.

[Warren shunt combined with partial splenectomy in children with extra-hepatic portal hypertension, massive splenomegaly and severe hypersplenism].

Introduction: Extra-hepatic portal vein obstruction (EHPVO) is one of the most often causes of portal hypertension in children.

Objective: Establishing the importance of shunt surgery in combination with partial spleen resection in selected pediatric patients with EHPVO, enormous splenomegaly and severe hypersplenism.

Methods: Distal splenorenal shunt (DSRS) with partial spleen resection was performed in 22 children age from 2 to 17 years with EHPVO.

Warren shunt combined with partial splenectomy for children with extrahepatic portal hypertension, massive splenomegaly, and severe hypersplenism.

Purpose: To establish the importance of shunt surgery combined with partial resection of the spleen for selected pediatric patients with extra-hepatic portal vein obstruction (EHPVO), enormous splenomegaly and severe hypersplenism. Severe hypersplenism is often refractory to treatment with endoscopic sclerotherapy or band ligation and shunt surgery; however, to our knowledge, this is the first such study to be published.

Methods: Distal splenorenal shunt with partial resection of the spleen was performed in 16 of 60 children treated for EHPVO in the Gastroenterology Department of our hospital.

Erythrocytic transglutaminase inhibition hemolysis at presentation of celiac disease.

Celiac disease (CD) is a common autoimmune condition. Previously it was considered to be a rare childhood disorder, but is actually considered a relatively common condition, present at any age, which may have multiple complications and manifestations. Hematological disorders of the disease are not uncommon.

[Bilobular pancreas: another variant of the divided pancreas?].

Background: Pancreas divisum is the most common anomaly of the pancreas. This anomaly has been known as a possible cause of recurrent pancreatitis.

Case Report: We performed computerized tomography (CT) of the abdomen in 5 children in whom a divided pancreas was confirmed using endoscopic cholangiopancreatography.

Infantile polyarteritis nodosa presenting as hyponatraemic hypertensive syndrome.

Unlabelled: The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo-old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic-range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polyarteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non-aneurismal changes such as arterial cut-off, arterial tapering stenosis and nephrogram perfusion defect.