特發性副甲狀腺機能低下症之一例

A Case of Idiopathic Hypoparathyioidism

謝榮森(Rong-Sen Hsieh)

《Acta Paediatrica Sinica》 9卷2期 (1968/06) Pp. 92-97

摘要

雖然早在1879年Sandström發現了副甲狀腺，但是對其功能的了解卻自1908年MacCallum, Voegtlin和1911年Erdheim開始，他們發現副甲狀腺截除術後會發生手足搐搦及低鈣血症。其後1924年由Hanson和1925年Collip的努力，證明利用副甲狀腺抽出物可以造成骨骼的病變、高磷尿症、高鈣尿症及其他的化學變化。關於副甲狀腺素對於骨髓及腎臓的詳細作用也陸續被發現，約在此時1926年Beumer和Falkenheim首先發現報告「特發性副甲狀腺機能低下症」病例，以後同樣病例報告陸續出現，文獻上的報告差不多100例左右，當然尚有未經報告的病例存在。臺北市立仁愛醫院小兒科在2月下旬看到一個類似病例，檢查的結果初步證明是「特發性副甲狀腺機能下症」，願提出讓各位參考，同時藉這個機會就過去報告的病例作簡單的檢討。

並列摘要

A 3years and 5 months old girl was admitted to the Taipei Municipal Jen-Ai-Hospital because of suffering from fine tremor on eyelids and mouth angles, and carpopedalspasm which made the patient unable to stand up for two days. These symptoms improved immediately after intravenous injection of 20 ml of Vitacal (2%calcium chloride solution), and the patient was hospitalized for further examination under an impression of ”Hypoparathyroidism”.
The patient had convulsive seizure and consciousness. disturbance at one year of age which recovered on the next day, but lethargic state had persisted for about one month and then eyelids tremor occurred off and on in the following one year. Physical growth and mental development were retarded, i.e. head control at 1 year of age; hand control at 1½ years; pull up to standing position at 2 years; but still unable to stand up by herself, walk alone, and speak.
Physical examination revealed, an underdeveloped and moderately nourished girl with apathetic facial expression tremors on the eyelids, mouthangles and tongue, and carpopedal spasm but in conscious state. Neither hyperpnea nor tachypnea was noted. Body length was 83.5cm; sitting height 53.5cm; head- girth 46cm and body weight 10.5kg. Nothing particular on the examination of the chest and abdomen. No abnormal neurological finding was detected after, intravenous injection of calcium chloride solution.
Laboratory studies done at that time revealed: BUN 26.5m/dl, Protein 8.7gm/dl, A/G 4.2/4.5, Serum Ca. 5.2mg/dl, inorganic P. 5.4mg/dl, K. 4.4mEq/L, Na. 143mEq/L, Cl. L02mEq/L, Alkaline phosphatase 2.9 B. S. U., Uric acid 15.2mg/dl. Urine and stool were normal. Calcium tolerance test devised by Howard et al. showed a marked increase of urinary inorganic phosphate with little change in serum inorganic phosphorus (See Table 1 in the text). Roentgenological examination of chest, abdomen, skull and long bones revealed no abnormal findings.
After discharge, the patient had the seizure characterized by carpopedal spasm, and fine tremor on the eyelids and jaw on June 29 and July 3. Again, these symptoms were cleared up as soon as intravenous injection of calcium chloride solution was given.
Although Ellsworth-Howard test has not been done owing to lack of parathormone ready for use, a diagnosis of ”idiopathic hypoparathyroidism” would be rather prefered than ”psudohypoparathyroidsm” in this case. Differential diagnosis among various causes of tetany was briefly discussed, and some controversial laboratory data found in this case were analysed and explained.