Neonatal cholestasis in Thai infants.

The objective of this study was to study etiologies and outcome of neonatal cholestasis in Thai infants. The medical records of infants aged less than 3 months with the diagnosis of neonatal cholestasis in Department of Pediatrics, Siriraj Hospital from 1993 to 2004 were retrospectively reviewed. The etiologies were diagnosed by history, physical examination, and proper investigations. There were 252 infants, including 135 males (53.6%) and 117 females (46.4%). The etiologies of cholestasis were idiopathic neonatal hepatitis (INH) 23%, extrahepatic biliary atresia (EHBA) 22.2%, total parenteral nutrition (TPN)-related cholestasis 18.3%, infection 9.9%, endocrine causes 6%, choledochal cyst 5.6%, Down syndrome 4.4%, hemolytic anemia 1.6%, and miscellaneous causes 9.1%, respectively. TPN-related cholestasis was increasingly found due to advance management of critically ill premature infants. Inborn error of metabolism were suspected in 8 patients (3.21%). Seventeen cases (6.7%) developed cholestasis during the first week of life due to hemolytic anemia, intrauterine infection, hypoxia and others. During the 3 month follow-up period, 6 cases died of progressive dysfunction of liver and one case with idiopathic neonatal hepatitis died from intracranial bleeding from vitamin K deficiency. In conclusion, INH and EHBA are the most common causes of neonatal cholestasis. Due to advance management and nutritional support in critically ill premature infants, TPN-related cholestasis is found more often. Inborn error of metabolism related to neonatal cholestases is uncommon in Thai infants. Overall short-term prognosis of neonatal cholestases is good.

Slide tracheoplasty for severe congenital long segment tracheal stenosis in infants: surgical and anesthetic management.

Congenital long segment tracheal stenosis is the rare occurrence of an intrinsic narrowing of the trachea due to a complete cartilagenous ring. It is difficult to manage and can be life threatening especially when these patients who are usually neonates or infants have the pathological pattern of a long segment and have to come for surgical correction. Despite many technical reports on how to correct this anormaly, currently, the technique of "slide tracheoplasty" is claimed to be the most successful with the good immediate and long-term outcomes. But because of the rare and life threatening disease, so we were encouraged and write this report about the disease and its management. These are the case series reports of 4 infants with a history and diagnosis of severe long segment congenital tracheal stenosis who needed a difinite surgical repair. The authors decided to use the surgical technique of "slide tracheoplasty" with successful outcome. In two of the cases, patients needed cardioplumonary bypass support during the surgical repair. All of these patients did well after the operation except one patient with a history of congenital heart disease (tetralogy of Fallot) who needed an emergency surgical repair and was reoperated upon with pericardial patch. In this reports the authors did not find any benefit from tracheostomy. Also, details of surgical and anesthetic procedure were discussed with the conclusion that the surgical technique of slide tracheoplasty should be the surgical of choice for the management of congenital long segment tracheal stenosis.

Persistent hyperinsulinemic hypoglycemia of infancy: experience at Siriraj Hospital.

BACKGROUND: Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of recurrent or persistent hypoglycemia in early childhood. Conventionally, pancreatectomy (Px) has often been recommended to control hypoglycemia. However, PHHI can be managed successfully by intensive medical treatment to avoid pancreatectomy. METHOD: Data from 10 infants (8M, 2F) with PHHI were retrospectively analyzed. RESULTS: Eight patients (80%) developed symptoms within 72 hours after birth (early-onset). Six patients (60%) underwent 85 per cent-95 per cent Px due to failure of medical treatment. Two patients who underwent less than 95 per cent Px required second Px (97% and 99%). One patient developed permanent diabetes mellitus and malabsorption. Hypoglycemia could be successfully managed by medication alone in four patients (40%). Of these, three patients had early-onset neonatal hypoglycemia. Medication could be discontinued in three patients (75%). Three of ten patients (30%) had delayed development. Pancreatectomies and/or the diagnosis of PHHI were made late for these patients. One of these three children also developed epilepsy. CONCLUSIONS: Patients with PHHI frequently require pancreatectomy which commonly results in long-term complications especially diabetes mellitus and malabsorption. Our data suggest that PHHI can be managed successfully with an intensive medical regimen even in patients with early-onset hypoglycemia. Although medical management is very laborious for the family and physician, it should be applied until euglycemia is accomplished. Moreover, the early diagnosis of PHHI and the successful hypoglycemic control are very necessary to prevent permanent neurologic sequelae.