Pseudo-Bartter syndrome in an infant without obvious underlying conditions: A case report.

Pseudo-Bartter syndrome (PBS) develops owing to renal or extrarenal chloride loss, leading to hypokalemic alkalosis. Whereas most adult cases result from diuretic/laxative abuse, many infantile cases occur secondary to cystic fibrosis. Rarely, infantile PBS is caused by renal salt loss with anomalies of the kidney/urinary tract or genetic disorders, such as Dent disease. Here, we report the case of a 10-mo-old girl with a one-month history of decreased formula intake and 5.6% body weight loss. She showed typical laboratory findings as PBS, including hypokalemia (2.7 mEq/L) and high levels of bicarbonate (32.7 mEq/L) with a plasma renin activity of 399 ng/mL/h. With minimum supplementation of potassium and sodium, an improvement in body mass index, from -1.13 SD to +0.52 SD, with complete resolution of laboratory data was obtained in approximately one month. No causative mutations were identified in candidate genes for Bartter-Gitelman syndrome. Due to profound hypochloruria (< 15 mEq/L), PBS of renal origin was unlikely. In addition, extrarenal chloride loss did not seem to be the case, because the patient never manifested gastrointestinal symptoms. Therefore, we speculate that a temporary decrease in chloride intake, coupled with the putative genetic/epigenetic disadvantage of chloride retention, such as a subtle renal leak, may be responsible for the PBS in our patient.

Development of fatty acid calcium stone ileus after initiation of human milk fortifier.

We report a case who was born with extremely low birth weight infant and had experienced abdominal operation for necrotizing enterocolitis, eventually developed ileus due to fatty acid calcium stones after giving human milk fortifier. He had developed necrotizing enterocolitis on day 30 of his age, such that we performed enterectomy and ileostomy. He could not tolerate enteral feeding fully, because intestinal fistula infection was repeated. Although we administered hindmilk, he grew up slowly and he suffered cholestasis as well. We performed end-to-end anastomosis to prevent fistula infections on day 87. After this operation, breast milk feeding volume was increased easily. However, we started to add HMF of half-strength on day 124, because his body weight gain remained very poor. And we confirmed to intensify the ratio of HMF full-strength on day 128. After that his abdomen had distended on day 131. As there is no effect of conservative therapy to occlusive ileus, we did emergency laparotomy on day 139. Intestinal calculi were impacted at anastomic portion. Although all stones were removed, he died on 144 days due to disseminated intravascular coagulation and renal failure. Calculi analysis revealed that all of them were fatty acid calcium stones. There is no report about like our case. We speculate that the construction of fatty acid calcium result from either high concentration of calcium/phosphorus or rapid increase in the fortification. We could have prevented this case happened by slower increment of fortification.

Mastitis is associated with IL-6 levels and milk fat globule size in breast milk.

BACKGROUND: From animal studies, it is known that mastitic inflammation of the mammary lobes can produce proinflammatory cytokines and can damage the milk fat globule (MFG). OBJECTIVE: To investigate, in women, whether MFG and interleukin (IL)-6 differences are observed between mastitic milk (MM) and healthy milk (HM) of a mother. METHODS: MM was obtained from the specific nipple pore leading to the mastitic lobe of 17 women; HM was obtained from the other breast. Milk sampling occurred at days 0 (pre-treatment), 1, and 2 (post-treatment). MFG size and IL-6 were measured by laser light scattering and enzyme-linked immunosorbent assay, respectively. We analyzed MFG and IL-6 differences between HM and MM, whether any differences occurred over time with treatment, and whether differences were observed between mothers with systemic symptoms (fever/malaise, Group A) or without systemic symptoms (Group B). RESULTS: On day 0, MM had higher MFG size (P < .01) and IL-6 levels (P < .001) than HM. This difference significantly decreased over time with treatment for both MFG size (P < .01) and IL-6 (P < .05). On day 0, Group A mothers had significantly larger MFG size (P < .01) and IL-6 (P < .001) than Group B. CONCLUSIONS: MM contains larger MFG and higher IL-6 levels than milk from the healthy breast. This difference is larger if accompanied by systemic symptoms of mastitis (fever/malaise). These changes decreased over time with treatment. Therefore, early initiation of appropriate treatment may be useful in limiting the processes that contribute to alterations in MFG size and IL-6.

The freeze-thaw process and long intervals after fortification denature human milk fat globules.

Although mother's milk is the optimal nutrition for preterm infants, the amount of protein, calcium, and phosphorus become inadequate for the growth of preterm infants, such that fortification is recommended when enteral feeding is established. Recently, intestinal obstruction due to calcium soap formation has been reported. All the reported cases were fed fortified thawed human milk. It has not been elucidated how human milk fortifier reacts with milk fat globules (MFGs) in thawed human milk. Therefore, we investigated whether freezing durations and time between fortification and enteral feeding denatured MFGs. Six samples of preterm mothers' milk of each type (fresh unfrozen, 1-month frozen, and 12-month frozen) were analyzed. Fortifier was not added to the control. The samples were measured at 1, 12, and 24 hours after fortification. The MFG size distribution of each milk sample was measured. The freezing process enlarged the mode diameter (4.0 ± 0.4, 4.3 ± 0.7, and 4.5 ± 0.9 in fresh unfrozen milk, 1-month frozen, and 12-month frozen milk, respectively, p < 0.001). Fortification itself did not significantly alter the MFG size in thawed mother's milk. However, a time lag of 12 hours after fortification enlarged the MFG size and resulted in smaller surface area per unit (3.6 ± 0.2, 3.3 ± 0.1, and 3.3 ± 0.1 m(2)/g, fresh unfrozen, 1-month frozen, and 12-month frozen, respectively) compared with 0 hours of time lag (3.8 ± 0.2, 3.5 ± 0.1, and 3.4 ± 0.2 m(2)/g, fresh unfrozen, 1-month frozen, and 12-month frozen, respectively). Not only the freeze-thaw procedure but also time lag after fortification affected MFG size. We recommend using fortified thawed human milk within 12 hours.

On-line statistical segmentation of a non-speech auditory stream in neonates as demonstrated by event-related brain potentials.

The ability to statistically segment a continuous auditory stream is one of the most important preparations for initiating language learning. Such ability is available to human infants at 8 months of age, as shown by a behavioral measurement. However, behavioral study alone cannot determine how early this ability is available. A recent study using measurements of event-related potential (ERP) revealed that neonates are able to detect statistical boundaries within auditory streams of speech syllables. Extending this line of research will allow us to better understand the cognitive preparation for language acquisition that is available to neonates. The aim of the present study was to examine the domain-generality of such statistical segmentation. Neonates were presented with nonlinguistic tone sequences composed of four tritone units, each consisting of three semitones extracted from one octave, for two 5-minute sessions. Only the first tone of each unit evoked a significant positivity in the frontal area during the second session, but not in the first session. This result suggests that the general ability to distinguish units in an auditory stream by statistical information is activated at birth and is probably innately prepared in humans.