Chronic Hypoxemia in a Child: Thinking Outside the Box.

Background: Chronic hypoxemia is generally attributed to primary cardiac or pulmonary entities. Case characteristics: A 9-year-old boy presenting with cyanosis, clubbing and hypoxemia, without icterus or hepatosplenomegaly. Cardiovascular and respiratory system examinations were normal. Outcome: He was diagnosed as type IB Abernethy malformation, a rare cause of hepatopulmonary syndrome. Message: Pediatricians should consider hepatopulmonary syndrome in the differential diagnosis of chronic hypoxemia, even in the absence of jaundice or hepatosplenomegaly.

Xanthogranulomatous Pyelonephritis (XGPN) in a Child with Multiple Ureteric Calculi.

Xanthogranulomatous pyelonephritis (XGPN) is an unusual suppurative granulomatous reaction to chronic renal infection, often in the presence of chronic obstruction, characterised histologically by granulomatous reaction with giant cells and foamy histiocytes1. XGPN is rare in children2. We report a case of XGPN, in a child with multiple lower ureteric calculi. An 11 year old male child presented to us, with features suggestive of acute pyelonephritis of two months duration.complete evaluation including contrast CT of the abdomen a diagnosis of XGPN was made. As the renal unit in question was non functional, he was treated successfully by subcapsular nephrectomy. This case is presented to emphasise the fact that, it is very important to diagnose XGPN early and differentiate it from chronic pyelonephritis. Prolonged antibiotic therapy and surgery is invariably required to relieve the obstruction and to eradicate the infection in patients with XGPN.

Congenital Right Intrathoracic Stomach Secondary to Hiatus Hernia in an Infant.

A four month old, female infant presented with acute onset respiratory distress and persistent, non-bilious vomiting for one day. The initial chest radiograph showed two large, fluid filled structures in the right hemi thorax. An upper GI contrast study showed right intrathoracic stomach with hold-up of barium in the pylorus. A diagnosis of congenital right intra-thoracic stomach with organo-axial torsion was made and patient underwent laparotomy and repair of the hiatal defect. The child is asymptomatic on follow up. The case is reported for unusual symptomatic presentation on early infancy.

Estimation of hydrogen sulphide in the human lymphocytes.

Hydrogen sulphide (H2S), a signaling gasotransmitter and a potent vasorelaxant is endogenously produced by the enzymes cystathionine-beta-synthase (CBS) and cystathionine-gamma-lyase (CSE). CBS is a predominant source of H2S in the central nervous system, while CSE is the major H2S producing enzyme in the brain and other nervous tissues. Though the expression of these enzymes in the blood lymphocytes is known, H2S formation in the lymphocytes has not been reported so far. In the present study, H2S levels in the lymphocytes of healthy control subjects were estimated, after suitable modifications in a routine method [Stipanuk M H & Beck P W (1982) Biochem J 206, 267-277] used for detecting tissue levels of H2S. In this method, homocysteine (Hcys) due to its higher solubility was used as the substrate in place of L-cysteine and NaOH was used in place of zinc acetate to increase the entrapment of H2S in the central well. A mean H2S level of 11.64 +/- 6.36 microM/min/mg protein was detected in the lymphocytes of 8 subjects (mean age, 24 +/- 2; 2 male, 6 female). The modified method was found to be more sensitive for H2S estimation in human lymphocytes. As endogenous H2S is reported to be involved in the pathogenesis of various cardiovascular and pulmonary diseases, the levels of H2S in lymphocytes can be a marker of the endogenous tissue levels.