Multiple endocrine neoplasia type IIa: a case report.

The authors reported a twelve year and four-month old girl who had prolonged fever for 2 weeks. Physical examination revealed a painless enlarged thyroid gland with firm consistency. Hyperparathyroidism was suspected because of hypercalcemia, hypophosphatemia, high level of serum alkaline phosphatase, and decreased density of long bones. Thyroid scan showed a cold nodule of the left upper lobe which subsequently proved to be a medullary thyroid carcinoma by high serum thyrocalcitonin level and pathological examination. Her 24-hour urinary vanillyl mandelic acid was in the normal range, and abdominal ultrasonography demonstrated normal adrenal glands. Multiple endocrine neoplasia type IIa (MEN IIa) was diagnosed by medullary thyroid carcinoma and hyperparathyroidism. However, the fully developed syndrome is characterized by the combined occurrence of medullary thyroid carcinoma, primary hyperparathyroidism, and pheochromocytomas. This syndrome is a rare, complex, and potentially lethal disease so early recognition and family screening are very important.

Cholesterol ester storage disease: a reported case.

Cholesterol ester storage disease is a rare autosomal recessive disease which is characterized by accumulation of cholesterol esters and triglycerides in the hepatocytes and other tissues. A 5-year and 4-month old Thai female with hepatosplenomegaly and hypercholesterolemia was diagnosed to have this disease by light and electron microscopic studies of the liver tissue from open biopsy. Early diagnosis and treatment with appropriate drug can help the patient by delaying the consequent complications. Genetic counselling and simplified explanation of the disease are a benefit to the patient's family.

Intestinal anisakiasis: first reported case in Thailand.

A case of intestinal anisakiasis is reported. The patient came with the symptoms of acute abdominal obstruction. The diagnosis was obtained by identification of the parasite in the tissue sections of the resected segment of the small intestine. This case appears to be the first reported case in Thailand.

A histopathologic study of 61 cases of peripheral intrahepatic cholangiocarcinoma.

The histopathological study of 61 cases of peripheral intrahepatic cholangiocarcinoma was reported. Of the 5 autopsied livers and 13 surgically resected livers, 89 per cent showed massive gross appearance, 94 per cent contained Opisthorchis viverrini or showed evidence of previous opisthorchiasis. Gall stones were found in 7 cases (11.5%), common bile duct stones were found in one case. The tumors were classified into well differentiated, less differentiated and uncommon types (adenosquamous, anaplastic and mucinous) which were found in 14.66, 70 and 13.66 per cent respectively. Of the 61 liver specimens, 92 per cent produced mucin and 53 per cent had more than one histological types. The important routes of intrahepatic spreadings were lymphatic involvement (90%) and permeation into the portal connective tissue (80%). One case of combined hepato-cholangiocarcinoma was observed in a cirrhotic liver containing Opisthorchis viverrini and positive HBsAg.

Type IV glycogen storage disease: first reported case in Thailand.

Glycogen storage disease type IV is rare. The disease is characterized by widespread storage of a polysaccharide similar to amylopectin. An autopsy case of a 6-year-old Thai boy with cirrhosis and deposits in the liver, heart, muscle and central nervous system was described along with histochemical and electron microscopic findings.

Dengue haemorrhagic fever with unusual manifestations.

A retrospective study on 18 cases of DHF presented with jaundice and neurological signs which were considered unusual manifestation of DHF reveals that the causes or contributing factors are multifactorial. Most commonly found associated conditions were prolonged shock with metabolic acidosis and severe DIC that lead to hypoxia/ischaemia and resulted in both hepatic and brain dysfunction. Gross haemorrhage in the brain was noted in 6 of the 10 fatal cases while brain oedema was noted in 3 cases. Electrolyte disturbance such as hyponatremia could be another cause of brain oedema. It is certain from this study that there is no pathological evidence of encephalitis. Hepatic dysfunction found in associated with jaundice and encephalopathy is possibly caused by toxic substances, drugs and/or associated with underlying liver conditions. Reye's or Reye's-like syndrome was postulated in one case.