The Association of Leukocyte Adhesion Defect Type I and Persistent Hyperinsulinemic Hypoglycemia of Infancy in a Saudi Arabian Family

Suliaman, Fawzi; Jabbar, Mohammed Abdul

doi:10.1080/08880010290097206

Cited by 5 publications

(4 citation statements)

References 13 publications

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“…Bone marrow transplantation has proven to be the most effective, as well as curative, therapy for the disease with matched 22 or mismatched human leukocyte antigen I antigen donors 23 . Most of the cases of reported transplantation were performed during the first 2 years of life 21–24 . However, not all patients with severe LAD are candidates for early bone marrow transplantation 25 .…”

Section: Discussionmentioning

confidence: 99%

Periodontal Manifestation of Leukocyte Adhesion Deficiency Type I

Dababneh¹,

Alwahadneh

Hamadneh³

et al. 2008

Journal of Periodontology

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Section: Discussionmentioning

confidence: 99%

Periodontal Manifestation of Leukocyte Adhesion Deficiency Type I

Dababneh¹,

Alwahadneh

Hamadneh³

et al. 2008

Journal of Periodontology

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“…As the disease is very rare, only a few cases where more than one offspring is affected are reported in the literature 4 . Recurrent and chronic skin ulceration, devoid of any pus formation, along with marked neutrophilia and finding reduced CD18 on cytometry confirmed the diagnosis in both brothers.…”

Section: Figurementioning

confidence: 82%

A curious case of two brothers with recurrent chronic skin ulceration

et al. 2020

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From regional lymphadenopathy in primary syphilis to worsening lymphadenopathy during a Jarisch-Herxheimer reaction, lymphatic involvement is one of the most characteristic features of syphilis. 3 As secondary syphilis results in part from lymphatic dissemination of Treponema pallidum, 3,4 it is plausible that this stage of syphilis could present with lesions in a sporotrichoid pattern, as seen in this case.We posit that two features may help differentiate sporotrichoid secondary syphilis from other sporotrichoid conditions: location on the head and neck and concurrent classic findings of syphilis. In the current case, the sporotrichoid pattern involved the postauricular, occipital, and posterior cervical lymph nodes. These lymph nodes are among the most commonly affected in secondary syphilis, 4 unlike other sporotrichoid conditions which usually involve lymph nodes of the extremities. 1 The other clue in this case was the presence of mucosal erosions and condyloma lata, both of which are known manifestations of secondary syphilis. 3,4 From 2014 to 2018, the number of cases of primary and secondary syphilis in the United States increased by 71.4%. 5 Given this rising incidence, we suggest that secondary syphilis be added to the sporotrichoid differential diagnosis, especially if lymph nodes of the head and neck are involved and classic features of syphilis are simultaneously present.

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“…5,[12][13][14] Mutations of SUR1 have been found in Ashkenazi Jews and in residents of Saudi Arabia. 7,15,16 Other cases have described a milder, dominantly inherited form of hyperinsulinism that is not linked to the SUR1 locus but is thought to be a mutation in the glucokinase gene. 17,18 A third group of described cases have an unusual combination of congenital hyperinsulinism and hyperammonemia.…”

Section: Hyperinsulinemic Hypoglycemia In Infantsmentioning

confidence: 99%

Persistent hyperinsulinemic hypoglycemia in infants

Markham

2003

Newborn and Infant Nursing Reviews

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Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) is the most common cause of recurrent hypoglycemia in neonates and infants. It is a disorder of glucose homeostasis characterized by unregulated hyperinsulinemia and profound hypoglycemia. PHHI may be differentiated from other causes of hypoglycemia by demonstrating the persistence of inappropriately high insulin levels regardless of blood glucose concentration. Prompt recognition and treatment of PHHI is critical because uncorrected hypoglycemia in the newborn period is associated with permanent damage to the central nervous system and subsequent mental retardation. The aim of therapy is to maintain euglycemia to protect the developing brain from possible damage. Despite recent advances in medical treatment, subtotal pancreatectomy is often necessary. This article will review the function of insulin, glucagon, and somatostatin, pathophysiology of hyperinsulinism, clinical manifestations, differential diagnosis, management, and reported neurologic outcomes of newborns and infants with PHHI.

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The Association of Leukocyte Adhesion Defect Type I and Persistent Hyperinsulinemic Hypoglycemia of Infancy in a Saudi Arabian Family

Cited by 5 publications

References 13 publications

Periodontal Manifestation of Leukocyte Adhesion Deficiency Type I

Periodontal Manifestation of Leukocyte Adhesion Deficiency Type I

A curious case of two brothers with recurrent chronic skin ulceration

Persistent hyperinsulinemic hypoglycemia in infants

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