Congenital hyperinsulinism: diagnostic and management challenges in a developing country – case report

نویسندگان

  • Cheri Mathews John
  • Prakash Agarwal
  • Suriyakumar Govindarajulu
  • Sandhya Sundaram
  • Senthil Senniappan
چکیده

Management of congenital hyperinsulinemia of infancy (CHI) is challenging. A 4-month-old female infant with persistent hypoglycemia and elevated insulin levels was diagnosed with CHI. Gallium-68 DOTANOC positron emission tomography/computed tomography (PET/CT) scan (68Ga-labeled [1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetic acid]-1-NaI3-octreotide) demonstrated focal disease in the body of the pancreas. Genetic studies indicated paternal inheritance, making focal disease likely. She was started on diazoxide therapy with partial improvement in blood glucose levels. Due to a suboptimal response to diazoxide and the likelihood of focal disease amenable to surgery, a laparoscopic subtotal pancreatectomy with preservation of the head of the pancreas was performed. The biopsy demonstrated diffuse hyperplastic pancreatic islet cells on immunohistochemistry, indicative of diffuse rather than focal disease. Paternal inheritance is a recognized indicator of focal disease. Gallium-68 DOTANOC PET/CT scan is the only available imaging modality in South India as 18F-L-dihydroxyphenylalanine (DOPA) PET/CT scan is not available at present. A laparoscopic approach reduces the postoperative recovery time and morbidity in such patients. The absence of 18F-L-DOPA PET/CT scan and the limited supply of diazoxide makes the management of this complex condition more challenging in developing countries.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

معرفی یک مورد نوزاد نارس مبتلا به هیپوگلیسمی پایدار ناشی از هیپرانسولینیسم مادرزادی

Congenital hyperinsulinism is a rare disorder can be associated with serious brain damage and neurodevelopmental delay in neonates. This disorder is an uncommon cause of neonatal hypoglycemia and most of the affected newborns are results of term pregnancies. In this paper, we report a case of persistent hypoglycemia due to congenital hyperinsulinism in a preterm neonate who also had hypertrophi...

متن کامل

Persistent Hyperinsulinemic Hypoglycemia of Infancy: A Rare Case with Multiple Anomalies

Background: Few cases of persistent hyperinsulinemic hypoglycemia of infancy (PHHI) have been reported, so far. The main concern in the management of PHHI is to prevent severe hypoglycemia, which can lead to coma, brain damage and mental retardation. Total or subtotal pancreatectomy is normally required for the infants, despite the availability of medical therapies. Case report: In this report...

متن کامل

Leadership challenges in health care organizations: The case of Iranian hospitals

Background: Effective leadership is a vital component of health care systems and has an extensive range of functions in improving organizational effectiveness and efficiency. This study aimed at exploring leadership challenges encountered by leaders in Iranian hospitals.    Methods: This qualitative study was conducted on a purposeful sample of 27 members of hospital management team ...

متن کامل

A novel case of compound heterozygous congenital hyperinsulinism without high insulin levels

BACKGROUND Congenital hyperinsulinism leads to unregulated insulin secretion and hypoglycemia. Diagnosis can be difficult and genetic testing may be warranted. CASE This patient initially presented at 11 months with seizure activity secondary to severe hypoglycemia. Her diagnostic evaluation included genetic studies, which confirmed congenital hyperinsulinism. A novel combination of mutations...

متن کامل

Diagnostic challenges in an atypical chest pain, Tietze’s syndrome: a case report in Northeast of Iran

Tietze’s syndrome is an inflammatory disorder, which frequently misdiagnosed as the severe life-threating problem. Herein, we reported a case of a 23-year-old male, who complained about the acute chest pain after pneumonia complication. After full examination, he diagnosed as Tietze’s syndrome. Since the precise diagnosis of Tietze’s syndrome has a great importance, our report may raise conscio...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 22  شماره 

صفحات  -

تاریخ انتشار 2017