Congenital self-healing Langerhans cell histiocytosis with pulmonary involvement: spontaneous regression.

Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by eruption of multiple, discrete and red-brown papules and nodules which may increase in size and number during the first few weeks of life with spontaneous regression. Systemic signs are usually absent except for occasional mild hepatomegaly. The authors report a case of CSHLCH associated with multiple lung cysts with spontaneous regression. A healthy full term male infant presented at birth with numerous diffuse discrete dark-red papules sized 0.2-1.0 cm scattered on the eyelids, temporal areas of the scalp, neck, palms and soles. Histologic findings were mixed inflammatory infiltration of numerous histiocytes. Immunochemistry findings were S-100 and CDla positive consistent with CSHLCH. Hepatomegaly and multiple lung cysts were detected at 1 month old. Since he was healthy, no medication was given except for close monitoring. At 2 months of age, hepatomegaly was resolved but the liver had sclerosing change. Skin lesions regressed completely at the age of 4 months. Lung cysts were markedly improved at 7 months old and completely resolved at 1 year old. Spontaneous regression in CSHLCH with lung involvement has never been reported in Thailand.

Nutritional support in pediatric patients undergoing bone marrow transplantation.

BACKGROUND: Children undergoing bone marrow transplantation (BMT) are prone to develop severe gastrointestinal (GI) complications and metabolic imbalance which consequently impair their nutritional status. Nutritional support is an important adjunctive treatment during BMT. OBJECTIVE: To assess GI complications, metabolic complications and nutritional outcome of children undergoing BMT with nutritional support intervention. METHOD: Retrospective study of 20 children (median age 6.8 years, 11 males) undergoing BMT at Ramathibodi Hospital from March 1995 to July 2000 was conducted. Their medical records were reviewed. RESULTS: The patients underwent autologous (n = 9) and allogenic BMT (n = 11). Median z-scores of weight for age, height for age and weight for height were 0.06 +/- 1.93, -0.55 +/- 1.18 and 0.48 +/- 1.94, respectively. Nineteen patients had vomiting for 9.8 +/- 5.5 days. Eighteen patients developed diarrhea for 9.6 +/- 7.2 days. The durations of vomiting and diarrhea, as a percentage of total hospital days, were 33.5 +/- 16.3 per cent and 30.4 +/- 17.0 per cent, respectively. There were no differences between the patients with autologous and allogenic BMT regarding these durations. All patients needed enteral and/or parenteral nutrition support for 21.0 +/- 7.7 days except for one patient who could take adequate oral intake. The duration of enteral nutrition support was not significantly different between the groups but the duration of parenteral nutrition support was significantly longer in the allogenic group. Metabolic complications were hypokalemia, hypophosphatemia and one case of arrhythmia secondary to hypomagnesemia. All patients developed febrile neutropenia but none developed catheter-related sepsis. The length of hospital stay was 30.5 +/- 10.2 days. The median z-score of weight for height on the day of discharge was 1.08 +/- 2.03. CONCLUSION: Children undergoing BMT usually have GI symptoms of vomiting, diarrhea and mucositis as well as metabolic imbalances such as hypokalemia, hypophosphatemia and hypomagnesemia. Despite these complications, their nutritional status could be restored by proper nutritional support.

Neonatal lupus erythematosus: clinical manifestations and management.

The authors report 6 cases of neonatal lupus erythematosus (NLE) who were seen at Ramathibodi Hospital from 1993 to 2000. The female to male ratio was 1:5. Cutaneous lesions were the major manifestation in all cases. Other clinical manifestations were thrombocytopenia, hepatosplenomegaly and mild elevation of liver enzymes. Skin rashes mostly erupted at 3-6 weeks old. None had a complete heart block but one had abnormal electrocardiograph (ECG) changes compatible with Wolff-Parkinson-White syndrome (WPW). Four of six patients had thrombocytopenia. All of the abnormalities resolved spontaneously except thrombocytopenia. Three of six needed blood transfusion to replace blood loss from gastrointestinal bleeding. Intravenous immunoglobulin (IVIG) 2 g/kg was given in 3 cases with good response in two of three cases. Platelets rose rapidly and maintained at a normal level within 24-48 hours. Combined therapy with corticosteroid 2 mg/kg was given to 1 case with good outcome. Telangiectasia was the most common sequelae especially in patients who had periorbital lesions resembling raccoon's eyes. The authors conclude that IVIG in the dose of 1 g/kg for 1-2 days is an effective treatment for NLE with severe thrombocytopenia especially when corticosteroid is contraindicated.