It has been suggested that cortisol deficiency may be responsible for the cholestasis. Although congenital hypopituitarism was reported as a possible cause of neonatal cholestasis, congenital adrenal hyperplasia with neonatal cholestasis is a very rare report in the literature. We experienced a case presenting with neonatal cholestasis associated with congenital adrenal hyperplasia. This case provides a clinical evidence supporting the notion that cortisol has an important physiological role in bile acid synthesis and transport.
Adrenal Hyperplasia, Congenital* ; Bile ; Cholestasis* ; Hydrocortisone ; Hypopituitarism

PURPOSE: The aim of this study was to identify the clinical features and natural history of dietary protein induced proctocolitis (DPIPC) and to detect the causative foods of DPIPC, and to evaluate the effect of elimination of the foods on the course of the disease. METHODS: Between March 2003 and July 2004, data from 30 consecutive patients with DPIPC who were followed for over 6 months, was reviewed. The diagnostic criterion used for DPIPC was an increase in the number of eosinophils in the lamina propria (> or =60 per 10 high-power fields). In breast feeding mothers, 5 highly allergenic foods were eliminated from the maternal diet for 7 days, namely, allergenic food groups such as dairy products, eggs, nuts and soybean, fish and shellfish, and wheat and buckwheat. We observed the disappearance or appearance of hematochezia after elimination or challenge with the offending foods. RESULTS: Before diagnosis infants were breast-fed (93.3%) or formula-fed (6.7%). Mean age at symptom onset was 11.5+/-5.1 (5~24) weeks, and mean age at diagnosis was 17.8+/-9.5 (8~56) weeks. Duration from symptom onset to diagnosis was 6.3+/-6.7 (0~36) weeks. Mean peripheral blood eosinophil count was 478+/-320 (40~1,790)/mm3 and eosinophilia (>250/mm3) was observedin 90.0% of patients. None of patients were found to have an increased serum IgE level. Of 15 patients that received sigmoidoscopy, nodular hyperplasia with erosion was observed in 93.3%. Of 27 patients whose mother ate the diet eliminated the 5 food groups, hematochezia diappeared in 74.1% of patients. Offending foods were identified as dairy products (37.5%), wheat and buckwheat (27.5%), fish and shellfish (20.0%), nuts and soybean (7.5%) and eggs (7.5%). A free maternal diet without patient's clinical symptoms was achieved at 29.4+/-8.7 (9~44) weeks of patient's age, and a free baby diet without blood in stools was achieved at 37.5+/-9.7 (12~56) weeks of age. CONCLUSION: DPIPC commonly occurs in exclusively breast-fed babies. Elimination of the above-mentioned 5 hyper-allergenic food groups from the maternal diet for 7days enables the detection of the offending foods. DPIPC is a transient disorder and 96.0% of patients can tolerate the offending foods at 12 months of age.
Breast Feeding ; Dairy Products ; Diagnosis ; Diet* ; Dietary Proteins* ; Eggs ; Eosinophilia ; Eosinophils ; Fagopyrum ; Gastrointestinal Hemorrhage ; Humans ; Hyperplasia ; Immunoglobulin E ; Infant ; Mothers ; Mucous Membrane ; Natural History* ; Nuts ; Ovum ; Proctocolitis* ; Shellfish ; Sigmoidoscopy ; Soybeans ; Triticum

PURPOSE: Cow's milk protein-induced enterocolitis (CMPIE) is a symptom complex of vomiting and/ or diarrhea caused by delayed hypersensitivity and may result in serious complications. This study was undertaken to identify high risk factors to facilitate the early recognition of CMPIE. METHODS: We reviewed the data of 101 patients, aged 15 to 45 days, admitted due to vomiting and/ or diarrhea between 2003 and 2004. After excluding 13 patients absolutely breast-fed and 2 patients transferred from other hospitals with the impression of CMPIE, the 86 study subjects were divided into three groups based on the underlying etiologies; CMPIE, infectious and non-infectious group. RESULTS: CMPIE was diagnosed in 11 patients (12.8%). On admission, failure to gain weight (P= 0.003), hypoalbuminemia (P=0.003), peripheral leukocytosis (P=0.015), and metabolic acidosis (P=0.014) were more significant in the CMPIE group than in the others. Multiple logistic regression analysis showed that the independent predictors of high risks for CMPIE were failure to gain weight < 10 g/day (OR, 10.25[95% CI, 1.62-65.06]) and serum hypoalbuminemia < 3.5 g/dL (OR, 9.18[95% CI, 1.69- 49.74]). Cow's milk challenges were performed in the 11 CMPIE patients; vomiting (81.8%), abnormal stool test (80.0%), peripheral leukocyte count and absolute neutrophil count (ANC) increase (100.0%) (P< 0.05), and enteropathy (100.0%). CONCLUSION: CMPIE is not a rare clinical disease in early infancy. The high risk factors of CMPIE were identified as follow: failure to gain weight below 10 g/day, hypoalbuminemia on admission and a rapid decrease during admission. Cow's milk challenge test with endoscopic duodenal biopsy was helpful to confirm CMPIE.
Acidosis ; Biopsy ; Diarrhea ; Enterocolitis* ; Humans ; Hypersensitivity, Delayed ; Hypoalbuminemia ; Leukocyte Count ; Leukocytosis ; Logistic Models ; Milk* ; Neutrophils ; Risk Factors* ; Vomiting