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Donohue syndrome (DS) is an extremely rare and usually fatal inherited disease resulted from mutations in the INSR (Insulin Receptor) gene and delineated by severe insulin resistance with fasting hypoglycemia, postprandial hyperglycemia, and facial dysmorphism. Optimal treatment of these cases is unclear and most DS cases die during the first two years of life. Herein, we introduce a case of leprechaunism due to the rarity of this syndrome (one case in every four million birth) revealed by clinical and laboratory findings.

 We present a 4-day old boy with an abnormal facial appearance, low birth weight who was admitted to the Neonatal Intensive Care Unit (NICU) due to poor feeding and jaundice. The patient had coarse facies, hypertrichosis, abdominal distention, genitomegaly, and acanthosis nigricans. Laboratory examinations revealed fasting hypoglycemia, postprandial hyperglycemia, and hyperinsulinemia. The diagnosis of Donohue Syndrome was characterized by the combination of dysmorphic features and biochemical results. Supportive care such as normalizing blood glucose and continuous feeding was initiated. He was discharged with good condition several days later but was admitted again at 6 months of age due to sepsis and then died.

According to the present case report, close monitoring of blood glucose as well as caring to prevent infection and sepsis is recommended.

Neonatal hyperbilirubinemia is a common problem that can be potential toxicity effects on the nervous system in infants، especially on the hearing ways. Regarding to hearing impairment leaves so negative effects on speech، language and cognitive development. The aim of this study was the assessment of the incidence of sensorineural hearing loss among icteric infants admitted to the hospitals of Gorgan، Iran in 2012.

In this longitudinal study، 223 infants were recruited by convenience sampling، that OAE screenings at the birth time was normal. Using Automated Auditory Brainstem Response (AABR) was performed for infants at the hospitalization and they were assessed after discharge، and with Auditory Brainstem Response (ABR) were confirmed.

The mean age of the infants on admission was 3. 84±4. 58 days and 55. 6% of them were female. The mean birth weight was 3431±218 grams and the mean gestational age was 39±1. 3 weeks. Serum total bilirubin levels in 61. 4% of infants were between 12-20 mg/dL and of 38. 6% were higher than 20 mg/dL. 13. 0% received conventional phototherapy، 72. 6% intensive phototherapy and 14. 3% of patients had exchange transfusions. Totally، 8 (3. 6%) of infants in admission had disturbance waves in AABR. Subsequent studies have shown improvement in 7 patients، but one patient problem still remains، and from other patients with normal admitted AABR in following 6 infants was impaired in ABR. Finally، 7 (3. 1%) of hearing loss were remains. There was a statistically significant difference between the hearing loss، serum total bilirubin level and total AABR impairment result on admission and final result (p<0. 05)، but there was no significant difference with variable of gender and kind of delivery (p<0. 05).

This study indicated that hyperbilirubinemia in the neonatal period can cause sensorineural hearing loss. Hence، regular screening in icteric newborns seems to be necessary.

Micropenis may be the most important sign of hypogonadism or pituitary hypofunction in neonatal period and after that. Since there is no data concerning the normal penile length and its distribution in different races in Iran، so، the aim of this study was to determine the penile length in Fars، Turkmen and Sistani newborns.

In this cross-sectional study، the stretched length of 427 male term infants (166 Fars، 129 Turkmen and 132 Sistani neonates) were assessed in the Dezyani affiliated hospital of Gorgan، Iran. Penile length measurements were done by a well-educated nurse in nursery ward in first 72 hours of their life by VYCON ruler. Penile length less than 23. 3 mm was defined as micropenis.

In this study the mean penile length was 32. 1± 3. 5 mm. Mean penile lengths in Fars، Turkmen and Sistani neonates were 31. 8±3. 9، 32. 3±3. 3 and 32. 3±3. 3 mm، respectively. There was no significant difference between races considering the mean penile length. One infant (0. 23 %) was diagnosed for micropenis.

The results of this study showed that ethnicity had no significant effect on the penile length in newborn infants and the prevalence of micropenis was low in the present study.